Surgical Pathology - English Course

Marius Florin Coro
Assoc. Professor, MD, Ph.D.

University of Medicine and Pharmacy of Trgu Mure
The First Surgery Clinic of Mure Clinical County Hospital

Surgical Pathology

2013

About the course of surgical pathology
Authors notes

The book is addressed especially to the students in the fourth year of general medicine,
but it might be also useful to residents and young specialists who wish to refresh their
knowledge about surgical diseases.
The main goals of the book are to provide fundamental knowledge about the specialty of
general surgery and surgical pathology, to help students in understanding the pathophysiology
of potential surgical diseases, to provide information required for diagnosis of diseases that
require surgical treatment and also to make students to understand the main methods of
surgical treatment and their physiological bases.
The book covers the entire necessary topics, being based on academic curricula.
Given the large amount of information, I tried to edit the book in a more easily
digestible or scholastic form, as a course with many pictures and graphics, which are more
suggestive than thousand of words are.
Sources of inspiration have been multiple, especially based on those found on the internet
from books and articles, but also information is based on my own experience and that of my
surgical staff. Therefore, without claiming an absolute originality, the curse is a compilation of
data from many sources (books are written from books). I tried keeping updated data with the
latest news about the diagnosis and treatment of surgical diseases. Most pictures are from the
casuistic of the surgical clinic where I work.
Because in my teaching experience I have noticed that many students have important
lacks in knowledge about anatomy, each chapter regarding specific organ pathology begins
with a reminder of the anatomy of that region. Surgery without anatomy is unthinkable! Each
surgical procedure is based on solid knowledge of anatomy. I emphasized the aspects of
surgical anatomy, which differs somewhat from conventional descriptive anatomy, with
reference to practical aspects.
Since the course contains basic information, I strongly encourage students to read from
other sources too, especially from bibliography required for the license and residency exams.
Information included in course will be completed and detailed during the internship,
when students will learn and practice the surgical cases presentations.
I will be very grateful if students will give me a feedback if they will find errors or
inadvertences in the course, and I assure them that I am open to any suggestion to improve it.

Assoc. Prof. Marius Coro, MD, Ph.D.
University of Medicine and Pharmacy Trgu-Mure, Romania
Email: corosmarius@yahoo.com

Surgical Pathology

Table of Contents

Chapter Title Page
Introduction . 1
Surgical Pathology of the Esophagus... 7
Surgical Pathology of the Stomach and Duodenum. 45
Bariatric Surgery.. 109
Surgical Pathology of the Small Intestine 127
Surgical Pathology of the Large Bowel... 151
Rectal Cancer... 203
Anoperineal Pathology. 217
Surgical Pathology of the Appendix 240
Surgical Pathology of the Pancreas.. 263
Surgical Pathology of the Liver... 299
Surgical Pathology of the Biliary Tree. 332
Peritonitis.. 364
Intestinal Occlusions 374
Acute Mesenteric Ischemia.. 388
Upper Gastrointestinal Bleedings. 394
Abdominal Trauma... 417
Abdominal Wall Pathology.. 445
Surgical Pathology of the Thyroid Gland. 495
Breast Pathology... 520
Surgical Pathology of the Arteries... 562
Surgical Pathology of the Lower Limb Veins. 584
Indications for Splenectomy. 604
Thoracic Traumas. 612
Pleuropulmonary Surgical Pathology... 640

INTRODUCTORY COURSE
About General Surgery
J ust like other teachers would say that their specialty is the most important, so I
would also say that surgery is probably the most important medical specialty in saving
lives and cure many illnesses.
General surgery is a very vast medical specialty and specialists in this field should
acquire multiple skills to treat an enormous variety of diseases. For a better cure, a
number of other surgical subspecialties have emerged from surgery specialty, focused
on certain areas dealing with a well-defined group of diseases belonging to different
systems or organs.
Almost all organs, systems or tissues, have a pathology that requires surgical
solution as the only method of cure or in combination with other methods.

Basic skills of general surgery are necessary to any doctor or nurse regardless their
specialty. The third year college students, in classes of semiology and surgical practice
acquire most of these skills. Starting with rules of asepsis and antisepsis, any doctor
should know the basic methods of rescue and life support, which are learned for the first
in classes of surgery. Maneuvers as injections, hemostasis, airway release,
cardiopulmonary resuscitation, and thoracocentesis should be known by any doctor.
These are only few aspects that make surgery one of the most important medical
specialties.
J ust think how many young patients would die because of acute appendicitis in the
absence of surgical treatment!
1

Common Features of Surgery
1. Surgery is an invasive method of treatment - The main feature is the
invasiveness, which often involves incisions of the skin and/or mucous
membranes, total or partial removal of tissues, organs or pathologic processes or
implantation of different kind of devices or prosthesis. That is the main reason
why many patients are afraid to seek medical attention postponing the
consultation as much as possible, situation that leads to late presentation in
advanced stages of the disease. Patients are afraid of pain, scars, mutilation. In
nowadays, surgery became less and less invasive, less mutilating if the disease is
detected in early phases of evolution. Minimal invasive procedures (eg.
laparoscopy, surgical endoscopy) have been developed followed by a very rapid
healing and less postoperative complications.
2. Surgery is a risky specialty. The vital risk - Surgery is an extreme solution of
treatment applied in certain cases when other methods fail. Surgery represents a
trauma for the body and the body reacts to it in different ways trying to maintain
the homeostasis. The vital risk represented by operative and postoperative
mortality rate markedly depends on preoperative health status of the patient. In
young patients without other co-morbidities, this risk is very low (about zero to
one percentage) but unfortunately, in elderly the risk is higher depending on the
associated illnesses (cardiovascular, pulmonary, diabetes, etc.). To reduce this
risk, patients must be thoroughly assessed before operation and other co-
morbidities should be adequately treated. In many cases, the risk is not due to
the surgery itself but to the underlying disease. The vital risk depends mostly on
patients general condition but also on possible surgical complications. Surgery
is applied only in those cases when patient would die in the absence of surgical
therapy. That is a reason why the patient, relatives and the surgeon should
assume this vital risk. Do not promise and never give to the patient or relatives
the feeling that the operation has no risks. The patient must be correctly
informed about all risks.
3. Anesthetic risk - Surgery is performed under anesthesia, which may be local,
regional, spinal or general anesthesia. Anesthesia has its own risks, which are
cumulated to those of surgical procedure. The type of anesthesia applied
depends on anesthesiologist considering the patients biological status, but
minimal surgical interventions are carried out under local anesthesia performed
by the surgeon itself. Even the local anesthesia is burdened by vital risk
considering the possible anaphylactic reaction to anesthetic solution. Patients
must be carefully tested for allergic reactions before applying the anesthesia.
Despite of all precautions there are cases of sudden death during maneuvers of
anesthesia.
4. Septic risk - Surgery means incisions, incisions mean solution of continuity in
skin or mucosa representing a gate of entrance for germs with the consequence
of local or general (spread) infections. This is the reason why surgeons
especially, and all the staff working in surgery, must comply with all rules of
asepsis. There are operations with a higher risk of infection represented
especially by those performed on the digestive tract, which is populated by many
germs especially anaerobic. Development of new generations of antibiotics is of
real help but these must be used with discernment not to select resistant strains.
Wound suppuration results in prolonged hospitalization, increased costs, vicious
scars, and incisional hernias.
2

5. Risk of contamination - This risk endangers both the patients and the surgeon.
Surgical team has the risk of contamination by various mechanisms (accidental
wounds, splash in the eye, etc.) with the patient's biological products, which may
contain pathological germs such as HIV, hepatitis viruses, bacteria, TB,
Echinococcus, and other microorganisms. On the other hand, the patients have
also the risk to be contaminated with germs from surgical team or more
frequently from other patients in so-called nosocomial infections.
6. Surgery produces irreversible effects - In most cases, surgery produces
irreversible effects on bodys anatomy and physiology. Removal of organs, part
of them or tissues is not desired by surgeon and is performed just in certain cases
because every organ has its well-defined role in the organism. There are rare
cases when normal considered organs are removed just to prevent development
of serious illnesses (eg. bilateral mastectomy in patients with positive BRCA, or
oophrectomy in advanced breast cancer treatment). Most of these changes do not
impair the patients quality of life (cholecystectomy, appendectomy, etc.) or
their function may be compensated by drugs (eg. drugs containing thyroid
hormones after total thyroidectomy). Some anatomical changes are intentionally
produced to improve the patients condition (eg. gastric sleeve or gastric by-pass
for morbid obesity). Surgery may produce changes in patients psychological
status and fortunately, in most cases changes are good. Some irreversible
changes are very apparent especially in plastic surgery influencing deeply the
patients psyche. When changes are made, especially on the patients demand
for esthetic purposes, all precaution must be taken and all the possible
complications should be explained to the patient prior to operation. Other
permanent changes will affect negatively the patients psyche and quality of life
(eg. total mastectomy, rectal amputation, etc.) but these are performed just when
necessary.
7. Surgery produces immediate results - Unlike other specialties, surgery produces
immediate results being very efficient in life saving and healing.
8. Surgery is spectacular - Based on the above statement, yes surgery is
spectacular and represents a source of inspiration for many pictures or movies.
Almost every artistic movie concerning the medical activity is based on surgery
and in very rare cases, or never, on other non-spectacular specialties.
9. Surgery is stressful - Surgery presumes in many cases emergency and
emergency is stressful. Surgery is not for everyone ! Surgery is a specialty for
those physicians who are mentally balanced and stable, with a good health
condition being able to resist for long periods of time standing, for those able to
work in team, for those who can afford to miss more time from home and
family, for those able to perform duties at any hour of the day or night, for those
who like the challenge, for those who do not like monotony, for those willing to
constantly improve and be open minded and up-to-date with medical knowledge
and surgical procedures. If all these conditions are met, then surgery is not
stressful for surgeon. The most stressful is decision making the surgeon must
choose the right moment and the right attitude to save the patient. For optimum
results, vast knowledge is necessary but doubled by experience. In surgery better
to learn from others mistakes than from yours. In surgery, from ecstasy to
agony is just a step. Even if the surgery went perfectly, unexpected
complications may occur. Stress does not end with operation, it becomes even
more intense in postoperative period, but stress is counterbalanced by
3

satisfaction of life saving. Not always, the surgeon can guide his actions based
on protocols. There are many cases when improvisations are necessary, making
surgery an art.
10. Surgery presumes work in team - For best results, well-trained teams are
necessary. Depending on operation type, two or three surgeons, a scrub nurse,
anesthesiologist, and auxiliary staff form the team (totally about 7-8 persons in
an operating room). Every member of the team should know its responsibilities.
11. Surgery has a high judicial risk - Surgery is one of the specialties with the
highest judicial risk because it is an invasive method burdened by risks
including that vital and produces irreversible effects o patients. Surgeons are
increasingly facing multiple civil liability claims from their patients. Malpractice
insurance in Romania unfortunately does not cover compensations for moral
damages. Most often patients sue doctors for the inconvenience caused by
surgery due to lack of communication between doctor and patient. Surgeons
have to take every precaution to prevent such situations. It should be reserved
enough time for discussion with the patient and relatives to explain in detail the
benefits of surgery but also its limitations and possible complications. Patients
must sign informed consent before surgery although this document is not a legal
guarantee that the doctor will not be sued. Another very important precaution is
that the patient's medical records should be very well documented even with
intraoperative films or pictures. Surgeons must be increasingly aware of the
importance of maintaining patient medical records. These are the most important
legal documents that can help the doctor in the conflict with the patient.
12. Surgery is a costly specialty -To perform operations of high performance,
minimal invasive with minimum damages to the patient, special and very costly
devices and instruments are necessary. In addition, the consumption of sanitary
materials during operation and after it is high because all these materials are
disposable. There are cases when minimally invasive surgery allows the patient
to be discharged the same day or the day following surgery, with low costs, but
there are serious cases that require prolonged hospitalization with very high
costs.
Other important aspects in surgery:
1. Never forget that the patient is the most important for us doctors!
2. There are no illnesses outside the patients! Do not forget the patient! We will
treat the patient not the disease!
3. We do not operate for the sake of work or for science! We dont make
experiences on patients!
4. The treatment must be adapted to each patient! Patients are different and may
have more or less associated diseases, which must be considered.
5. There are no pure surgical illnesses! In most cases, we combine surgical
treatment with other kind of treatments (medical, oncological, etc.) Those
illnesses for which the principal solution is surgery take part from the family of
surgical pathology.
6. The basic principle in surgery is: PRIMUM NIL NOCERE ! a Latin phrase
that means "First, do no harm.
7. The surgeon is ethically obliged to maintain the confidentiality of the clinical
record and, in principle, access to the record by third parties should be restricted.
4

General Aspects Concerning Surgical Diseases
Regardless the organ affected, the pathology may be represented by:
Malformations
Injuries (trauma)
Infections Inflammations
Degenerative illnesses
Tumors
o Benign
o Malignant
Phases of surgical management are:
The diagnosis which is based on:
o History
o General and local examination
o Investigations
Preoperative preparation
Surgical treatment
Postoperative care
Evaluation of late results (outcome)

5

6

Surgical Pathology of the Esophagus
SURGICAL PATHOLOGY OF THE ESOPHAGUS

1. Surgical anatomy
2. Esophageal syndrome
3. Achalasia
4. Esophageal diverticula
5. Esophageal cancer
6. Esophageal varices

1. Surgical Anatomy
The esophagus is a segment of the digestive tract being represented by a musculo-
membranous tubular organ, located between the pharynx and the stomach. Its average
length is 25 cm and its diameter is about 1.5-2 cm.
The esophagus has three segments depending on which anatomical region it
crosses: cervical, thoracic and abdominal. (Figure 1) It also has two main curves: one
in sagital plane, with convexity oriented posterior, and the other with convexity oriented
to the right in frontal plane. (Figure 2) These curves guide the surgical approach to the
left or to the right. Thus, in cervical region, the approach is preferred on the left side.
For the first two thirds of intrathoracic esophagus the approach is easier via the right
thoracotomy, because the esophagus is located at the right of the aorta, whereas the
lower third of intrathoracic esophagus is easier accessed via a left thoracotomy.
The esophagus has three physiological straits important in the development of
postcaustic strictures: (Figure 3)
Cricoidian strait - at 15 cm from the dental arch
Bronchoaortic strait - at 25 cm from the dental arch
Diaphragmatic strait - at 40 cm from the dental arch

On the posterior aspect of the cervical esophagus, at level of cricoid cartilage,
there is a weakness zone of the esophageal wall between the lower edge of the inferior
pharyngeal constrictor muscle and the upper edge of the crico-pharyngeal muscle. It is
the Leimers triangle of weakness (also known as the Killian's dehiscence) (Figure 4),
which is important in the development of cervical esophageal diverticulum known as
the Zenker's diverticulum.

7
Anatomical relations
Cervical esophagus
This segment, with a length of about
5 cm, lies next to the cricoid cartilage
(the 6
th
cervical vertebra) and enters
the chest (through the upper chest
aperture) behind the jugularis notch
of the sternum (C5-6 - T2).
Trachea (the membranous part), the
thyroid and parathyroid glands and
the recurrent laryngeal nerves
represent anterior relations.
Behind the esophagus, there is the cervical spine.
Lateral to the esophagus are the common carotid artery and the left thoracic
duct.
Thoracic esophagus - with a length of about 18-20 cm, is located in the posterior
mediastinum
In the upper mediastinum, it lies between the trachea and the spine. Then it
descends downward and to the right, behind the aortic arch toward the posterior
mediastinum.
Anterior it has relations with the trachea, right bronchus, pericardium, left atrium
and diaphragm.
Posterior relations are with aortic arch, intercostal arteries, thoracic duct and
hemiazygos vein and the diaphragmatic portion of the aorta.
On its left side, the esophagus borders the aorta, the subclavian artery, the left
thoracic duct, the recurrent laryngeal nerve and the left pleura.
On the right side, it comes in contact with the pleura and the right hemiazygos
vein. The right vagus nerve has a trajectory behind the esophagus while the left
is located in front of it. Thoracic duct has an upward trajectory initially on the
right side of the esophagus then, from the fourth thoracic vertebra, it passes
behind, toward the left side.
Abdominal esophagus - of 1-2.5 cm length, is located between the esophageal hiatus
and cardia.
Anterior is covered by peritoneum and it comes in contact with the left livers
lobe and the left vagus trunk.
Posterior it has relations with the posterior vagus nerve and the aorta through the
left diaphragmatic pillar.
Lateral it has relations with the diaphragmatic pillars.
Between the esophagus and the diaphragm, there are interposed connective
tissue (the membrane of Leimer-Bertelli Treitz) and muscle fibers, which are
drawn from the diaphragm and dissolve into the esophageal wall (Rougets
muscle).
Knowledge about anatomical relations of the esophagus are very important for
surgeons during esophageal surgery and to understand what organs and anatomical
structures could be invaded or compressed by expansive processes (tumors, diverticula,
etc.) and what would be the consequences.

8
The structure of the esophagus
Esophagus has four layers: external (adventitia), muscular layer (tunica
muscularis), submucosa (tela submucosa), and the internal esophageal lining (tunica
mucosa).
Esophageal muscles consist of two layers, one internal with circular fibers and the
other external with longitudinal fibers. External longitudinal layer in the upper portion
of the esophagus is divided into two fascicles, one anterior that is fixed to the cricoid
and the other posterior that continues with the pharyngeal muscles. As they descend, the
two fascicles merge into an even layer. This particular positioning of the longitudinal
layer in the initial portion of the esophagus delineates between the inferior pharyngeal
and cricopharyngeal muscles a "V" shape of low resistance area of the esophageal wall,
where only circular muscular fibers are present (triangle of Leimer or Killian
dehiscence). In its upper portion, the cervical esophagus has striated muscular fibers and
gradually, as it descends, these turn into smooth muscles. Circular muscular fibers are
coiled so that their synergistic contraction with that of the longitudinal fibers causes a
peristaltic wave that pushes the bolus toward the stomach. Of particular importance is
the antireflux mechanism of the lower esophageal sphincter (LES) whose damage
causes a variety of pathology (achalasia, esophageal reflux disease, etc.).
Submucosa contains blood and lymphatic vessels, nerve fibers and mucous
glands. It consists of loose connective tissue that allows dissection between the
muscular and mucosa layers during esophageal myotomy.
Esophageal mucosa has an appreciable thickness with longitudinal folds that
allow the lumen expansion. It consists of three layers: stratified squamous epithelium,
lamina propria and muscularis mucosae, better expressed in the lower portion of the
esophagus.
Vascular supply
Arteries - Esophagus is the weakest vascularized segment of the whole digestive
tract. This aspect is important for two reasons:
1. Because of the weak vascularization the esophageal anastomoses are at high risk
of dehiscence, and
2. It explains why stripping of the esophagus (esophagectomy) can be performed
with a minor blood loss.
Arterial supply of the esophagus comes from branches of the following arteries:
inferior thyroid, subclavian, esophago-bronchial (directly from the aorta), intercostal
arteries, the own esophageal arteries, gastric coronary and diaphragmatic arteries.
Veins - there are two venous plexuses: submucous, and periesophageal. (Figure 5)
The blood from the upper esophagus is drained into
the azygos veins, and from the lower portion into the
coronary gastric veins and portal system. (Figure 6) The
esophagus is one of the places where the two venous
systems: systemic (caval) and portal, are interconnected
(natural shunts) explaining the development of esophageal
varices in case of portal hypertension.
Lymphatics - two plexuses collect the lymph:
muscular and submucous. The eyes of the lymphatic
network are very elongated, which explains the spread
of tumoral cells along the esophageal wall far (6 cm)
9
from the primary tumor, giving the possibility of 2-3 concomitant esophageal
tumors. Lymphatics drain into the next lymph node stations: cervical, paratracheal,
posterior mediastinal, of gastrohepatic ligament and celiac. (Figure 7)

Innervation is sympathetic (from
paravertebral, celiac and semilunar ganglia) and
vagal. There are two intramural plexuses:
Auerbach and Meissner important in
esophageal contractions for swallowing.
Alterations of these structures will lead to
esophageal motor dysfunction explaining for
example the achalasia.
Physiology
The main function of the esophagus is in
swallowing. (Figure 8) Factors contributing to
the swallowing process are gravity and
esophageal peristaltic waves. There must be
coordination between peristaltic waves and opening of the cardia. The LES (low
esophageal sphincter) opens on a pressure of 20 cm water column. Missing of this
coordination will lead to esophageal functional syndromes such as achalasia.
2. Esophageal Syndrome
Causes of this syndrome can be either organic or/and functional, and includes:
1. Dysphagia (difficulty in swallowing). Sometimes, dysphagia may have a short
onset and evolution especially when functional disorders are the cause. If there
are anatomical lesions, dysphagia is usually progressive manifested initially for
solids and then for fluids. In achalasia, the reverse situation exists, the so-called
"paradoxical dysphagia.
2. Pain located retrosternal and in epigastrium.
3. Regurgitation. Not to be confused with vomiting. The content of regurgitation
is represented by undigested food coming from esophagus not from the stomach
like in vomiting. It may occur early in upper stenosis, or late in lower stenosis.
4. Hypersalivation (sialorrhea) is caused by the vagus nerves irritation produced
by esophageal distension.
10
3. Achalasia (Cardiospasm)
Achalasia is represented by three elements: the incomplete lower esophageal
sphincter (LES) relaxation when the alimentary bolus reaches at this level, increased
LES tone, and aperistalsis (lack of peristaltic waves) of the esophagus.
History. Sir Thomas Willis, in 1672, was the first who described the disease. In 1881
von Mikulicz described the disease as cardiospasm to demonstrate that it is due to a
functional disorder not to a mechanical one.[1,2]
Epidemiology [2-6]
The prevalence is of less than 1/10.000, and the incidence between 0.03 and
1/100.000 inhabitants per year.
The sex ratio is: male/female = 1/1
The most affected ages are between 25 and 60 years. Less than 5% of cases
occur in children.
Achalasia seems to be less common among some Asian and African populations.
Causes
1. Idiopathic (the cause is not known). Perhaps a viral infection results in
myenteric plexus inflammation that leads to an autoimmune response with
subsequently destruction of the inhibitory myenteric ganglion cells resulting in
the clinical syndrome of idiopathic achalasia. [1]
2. Trypanosoma Cruzii infection (Chagas disease)
3. Tumors in this area
4. Degeneration of vagus nerves nuclei
5. Pharmacological disorders of chemical mediators from the neuromuscular
endplate in the lower esophageal sphincter (LES)
Three criteria are defining the achalasia:
1. The spasm of the lower esophageal sphincter
2. Abnormal esophageal peristalsis
3. Weak esophageal contractions or nonexistent, aperistaltic, biphasic or
multiphasic contractions.
Morphopathology
The dimension of abdominal esophagus is normal but the thoracic esophagus is
much dilated and the dilatation does not depend on the duration of disease.
The esophageal wall may be very thin with a bold or thin (atrophic) mucosa,
with esophagitis.
The cardia has no modifications.
The intramural Meissners plexuses are altered, especially in the lower part of
the esophagus.
Physiopathology
Food accumulates in the esophagus
leading to its expansion and dilatation.
After a while, the food manages to pass into
the stomach according to the Hurst law.
According to this law, the LES will open
only when the intra-esophageal pressure is
higher than the LES contraction force of 20
11
cm of water column. When the intra-esophageal pressure lowers, the LES will close
again and the food cannot pass into the stomach. (Figure 9)
Symptoms. Commonly, achalasia appears in patients with neuropathic disorders, in
hypothyroidians, addicts, and others, usually after strong emotions. Characteristic for
achalasia are:
The insidious debut
The capricious even paradoxical dysphagia
Regurgitation of undigested foods
Retrosternal pains due to esophagus distension
Sialorrhea (hyper salivation)
Fetid halitosis
Dyspnea
Palpitations
Complications
Pulmonary complications are the most frequent as a consequence of
regurgitation.
Bleeding from ulcerations of the esophageal mucosa.
Malignization is more frequent then in the absence of the disease. It seems that
intra-esophageal fermentation leads to the formation or maintenance of a chronic
esophagitis with a high risk of malignization.[7]
Diagnosis
The main investigation for diagnosis
is the esophagography or the
continuous fluoroscopy with
contrast agent (barium swallow). On
radiograph, the excessive dilatation
of the esophagus above the LES,
with the absence of normal
peristaltic movements can be seen.
The appearance of the esophagus is
like a bird beak or similar to
sigmoid colon. An air-fluid level
is often present. (Figure10)
Esophagoscopy (endoscopic
examination) shows the
permeability of the cardia. Biopsies are necessary only in case of suspicious
lesions.
Esophageal manometry (esophageal motility study) measures the esophageal
muscles tonus during swallowing. Lack of LES relaxation and the absence of
peristaltic movement are revealed by this examination.
Differential diagnosis
In most cases, imagistic investigations are very suggestive and confident for
diagnosis but other conditions should be excluded such as:
1. Cancer of the lower third of the esophagus. In this case, the esophagus is not as
much dilated as in achalasia and there is an anfractuous, rigid filling defect.
Endoscopy and biopsy are essential for diagnosis.
2. Benign esophageal strictures especially after caustic or acid solutions ingestion
12
3. Schatzki ring - a diaphragm-like localized narrowing in the lower esophagus
5. Esophageal compression coming from neighborhood pathological processes
Treatment may be: pharmacologic, endoscopic or surgical.
Conservative treatment (pharmacotherapy and endoscopic) is represented by:
Nitrites and calcium channel blockers (nifedipine). These drugs produce a
relaxation of the LES. The success rate is about 10% of cases.[8] This treatment
is applied especially to elderly in whom other invasive procedures are risky.
Botox injections. The botulinum toxin is injected by endoscopic approach into
the muscular layer. The toxin produces paralysis of nerves inducing muscular
relaxation in the LES region. Its success rate is of 30%-75%.[8,10,11] Results
may last from 6 month to 1 year.[9,11] It can induce inflammatory local reactions
that make more difficult a subsequent operation. The procedure is also indicated
in elderly.[10]
Pneumatic dilatation of LES. (Figure 11) During this maneuver, a balloon is
introduced endoscopically into the esophagus in the LES region, and then
gradually inflated producing the disruption of several muscle fibers. Its success
rate is of 42%-85%.[8,11,12] More then 50% of patients require more then one
session of dilatation.[8] Perforation rate is about 0-18%.[8,13] Subsequent gastro-
esophageal reflux rate is about 25%.[8]
Surgical treatment is recommended
when other conservative methods have
failed.
The standard operation is the Hellers
procedure (extramucosal cardiomyotomy).
(Figure 12) During this procedure, the
muscular layer of the inferior part of the
esophagus is cut (myotomy). Myotomy
extends upwards on the esophagus over a
distance of 5-6 cm above the cardia and
downwards on the stomach over a distance of
1.5-2.5 cm, followed by a partial
fundoplication to prevent gastroesophageal
reflux. The operation can be performed in
classical way by open approach (laparotomy
or thoracotomy), but in nowadays the
minimal invasive approaches (laparoscopy or
thoracoscopy) are preferred. The operation
relieves symptoms in 85%-95% of patients,
and the incidence of postoperative reflux is
10%-15%.[8,11,14,15]
In laparoscopic approach, initially the
peritoneum that covers the anterior surface of
the abdominal esophagus is cut usually using
the hook (taking care not to damage the
anterior vagus nerve) and then the esophagus
is prepared on its anterior surface toward the
13
mediastinum for at least 6 cm. This maneuver presumes the widening of the esophageal
hiatus, which predisposes to gastroesophageal reflux. Then, the esophageal muscular
layer is divided longitudinally (usually using scissors) along the plan offered by the
submucosa. The division is continued downwards along the cardia and on a distance of
about 1-2 cm on the stomach wall. Dissection at this level is more difficult with higher
risk of perforation of the gastric mucosa. Once myotomy completed, the mucosa layer is
inspected carefully to observe any damages that can be easier highlighted by
introducing methylene blue dye into the esophagus and stomach. To keep the myotomy
open, some surgeons suture the sectioned muscular edges to the diaphragmatic pillars.
After that, a fundoplication antireflux procedure is usually associated. The most often
performed is the Dors procedure in which a flap of the gastric fundus is sutured over
the myotomy. The most serious complication is perforation of the mucosa layer that can
be managed intraoperatively by defect suture, protected by the gastric patch.
Unrecognized, this complication will lead to peritonitis requiring laparotomy.
The Sauerbruch procedure, esogastrostomy, (Figure 13) is rarely applied and the
risk of consecutive gastroesophageal reflux is generally higher.
The Sweet-Kazanski procedure (Figure 14) performs the resection of the superior
pole of the stomach followed by esogastrostomy and is rarely applied.

In advanced stages of the disease, with severe alterations of esophageal function,
with massive dilatation, cardia fibrosis and frequent lung complications due to
aspiration, esophagectomy is required followed by esophagoplasty using the stomach,
colon or small intestine.
References
1. Park W, Vaezi MF. - Etiology and pathogenesis of achalasia: the current understanding. - Am. J. Gastroenterol. 2005;
100(6):1404-1414.
2. Allaix ME - Achalasia - Medscape reference, Emedicine - http://emedicine.medscape.com/article/169974-overview
3. Mayberry JF. - Epidemiology and demographics of achalasia. - Gastrointest. Endosc. Clin. N. Am. 2001; 11:235-248.
4. Ho KY, Tay HH, Kang JY. - A prospective study of the clinical features, manometric findings, incidence and prevalence
of achalasia in Singapore. - J. Gastroenterol. Hepatol. 1999; 14:791-795.
5. Epocrates online - https://online.epocrates.com/u/2923872/Achalasia/Basics/Epidemiology
6. Mayberry JF, Atkinson M. - Studies of incidence and prevalence of achalasia in the Nottingham area. - Q. J. Med. 1985;
56:451-456.
7. Cecconello I, Zilberstein B, Ishioka S, Pollara WM, Lemos AM, Venco FE. - Precancerous esophageal lesions. - Dig.
Dis. Sci. 1986; 31(10supl):80S.
8. Allaix ME - Achalasia - Medscape reference - http://emedicine.medscape.com/article/169974-treatment
9. The Merk Manual For Health Care Professionals (online edition) -
http://www.merckmanuals.com/professional/gastrointestinal_disorders/esophageal_and_swallowing_disorders/motility_
disorders.html
10. Dughera L, Battaglia E, Maggio D, et al. - Botulinum toxin - Treatment of oesophageal achalasia in the old old and
oldest old: a 1-year follow-up study. - Drugs Aging 2005; 22:779-783.
11. Pohl D, Tutuian R. - Achalasia: an Overview of Diagnosis and Treatment - Journal of gastrointestinal and liver diseases
2007; 16:297-303.
14
12. Vela MF, Richter JE, Khandwala F, et al. - The long-term efficacy of pneumatic dilatation and Heller myotomy for the
treatment of achalasia. - Clin. Gastroenterol. Hepatol. 2006; 4:580-587.
13. Tarun S, Andreas A. - Balloon Dilatation of Esophageal Strictures/Achalasia - Semin. Intervent. Radiol. - 2004;
21(3):149155.
14. Patti MG, Arcerito M, De Pinto M, et al. - Comparison of thoracoscopic and laparoscopic Heller myotomy for achalasia.
- J. Gastrointest. Surg. 1998; 2:561-566.
15. Anselmino M, Zaninotto G, Costantini M, et al. - One-year follow-up after laparoscopic Heller-Dor operation for
esophageal achalasia. - Surg. Endosc. 1997; 11:3-7.

15
4. Esophageal Diverticula
Diverticulum is a sacciforme dilatation of the esophageal wall with various
locations, at different levels, which communicates with the esophageal lumen through
an opening of various calibers. (Figure 15) The main locations are cervical and thoracic.
Based on physiopathological criteria, there are two main types of esophageal
diverticula: diverticula of pulsion or of pressure, and diverticula of traction.

A. Cervical Diverticulum - Zenkers Diverticulum
Zenkers diverticulum (Figure 16) is a diverticulum of pressure or pulsion, which
results from the posterior herniation of the hypopharyngeal mucosa near the junction to
the esophagus. Two main factors acting at the level of Leimer's triangle contribute to the
occurrence of the diverticulum:
1. The natural weakness of this zone,
and
2. The increased pressure on this
region during swallowing due to the
lack of relaxation (spasm) of the
cricopharyngeal muscle when the
alimentary bolus arrives.
Zenker's diverticulum frequently
associates with hiatal hernia and gastro-
esophageal reflux disease.[1] The
diverticulum develops slowly. Its size
varies from a bean to an apple or even to a
head of a fetus, determining compressions
against the esophagus.[2]

16
History
In 1877, Friedrich Albert von Zenker, professor of pathology at Erlangen -
Germany University, described it first. At the beginning of the twenty century, Killian
demonstrated that this diverticulum is produced through a weak zone of the pharyngo-
esophageal wall (the triangle of Leimer or Killians dehiscence). In 1886 Wheeler is the
first doctor who performed a resection of this kind of diverticulum.
Epidemiology [3,4]
Prevalence: 0.01-0.2%.
It affects mostly adults over 60 years old
Slight predominance in males
Extremely rare in Asia and Africa
Symptoms depend on the development stage and are represented by:
Deglutition disorders, progressive dysphagia without pain (90% of patients),
regurgitations, sialorrhea and fetid halitosis.
Cough and hoarseness may also appear, caused by compression on laryngeal
nerves.
Regurgitations are produced mainly when the patient is leaning forward, the so-
called shoelace sign.
Sometimes the diverticulum can be observed as a tumor bulging in the lateral
region of the neck. In 90% of cases it bulges on the left side.[3]
Complications
Weight loss
Pneumonia of aspiration
Ulceration and perforation
Esotracheal fistula
Bleeding
Esophageal stenosis
Hoarseness
Malignant transformation - squamous epithelioma [5]
Diagnosis is based on radiological investigations: barium swallow (Figure 17) and
fluoroscopy. The endoscopic examination has the risk of perforation.
Differential diagnosis should include:
1. Achalasia
2. Esophageal tumors
3. Esophageal strictures
4. Esophagitis
5. Aneurysm of aorta
6. Mediastinal and pulmonary cysts
Commonly the confusion is made
with other fluid-air formations of
intrapulmonary or mediastinal collections
(abscesses, cysts, hydatid cyst).
Treatment
Treatment is conservative in elderly with co-morbidities and in small size
diverticulum. Surgical treatment is indicated in large diverticulum with intense
symptoms and in case of complications.
17
There are two types of approaches:
A. The classical open surgery, through the left cervical approach. Possible
operations are:
1. Diverticulectomy, represented by the excision of the diverticular sac. (Figure 18)
2. Myotomy, applied especially in small diverticulum. The surgeon performs a
myotomy of the cricopharyngeal muscle and partially of the inferior pharyngeal
constrictor muscle. Myotomy leads to rapid improvement of swallowing
disorders and the disappearance of diverticulum. The operation can be
performed in local anesthesia. (Figure 19)
3. Myotomy associated with diveticulopexy is the operation that associates
myotomy with the fixation (suturing) of the diverticulum up to the prevertebral
fascia. (Figure 20)
B. The endoscopic approach
1. Using an EndoGIA stapler, the lower edge of diverticular orifice, represented by
the cricopharyngeal muscle, is cut and simultaneously stapled resulting a "V"
shape wider opening joining the two cavities: esophageal and diverticular. The
procedure takes 15-20 minutes and the patient is discharged in the same day.
(Figure 21)
2. Using the laser, the principle is the same but the risk of fistula formation is
higher.

B. Diverticula with Thoracic Location (Figure 22)
Thoracic diverticula are less frequent than cervical ones. Depending on their
location along the esophagus they can be classified as peribronchial diverticula
(located near the trachea bifurcation) and epidiaphragmatic (epiphrenic) diverticula
(located near the diaphragm). These diverticula can be diverticula of pulsion, especially
those peribronchial, and diverticula of traction, those epiphrenic.
18
Commonly, they are of small dimension
causing few symptoms. Frequently are associated
with other esophageal affections. Only those
diverticula with severe symptoms or
complications (esobronchial fistula) have a
surgical indication. For peribronchial diverticula,
the surgical approach is via a right thoracotomy
whereas for epiphrenic location diverticula via the
left thoracotomy.
Diverticula of traction have thoracic
location being produced by traction exerted on
esophagus by adhesions with surrounding organs
as the result of inflammatory processes (pleurisy,
mediastinal adenopathy, etc.). There are no
symptoms, the condition being discovered
incidentally. Radiologic aspect is characteristic of cones with the tip oriented upward.
This type of diverticula does not require any treatment.
References
1. Morales-Divo C, Jecker P, Lippert B, Mann WJ. - Extraesophageal reflux in patients suffering from Zenker's
diverticulum. - W.J. HNO. 2007; 55(7):546-50.
2. Sturdza VR, Sturdza M. - Zenker's diverticulum. - Rev. Med. Chir. Soc. Med. Nat. Iasi. 1989; 93(4):759-61.
3. Stafford ND, Moore-Gillon V, McKelvie P. - Handedness and the side on which pharyngeal pouches occur. - B.M.J.
1984; 288:815816.
4. Jill DSouza - Zenkers Diverticulum. - Grand Rounds Presentation, University of Texas Medical Branch, Department of
Otolaryngology , May 28, 2010 (http://www.utmb.edu/otoref/grnds/divert-zenk-100526/divertic-zenk-100526.pdf)
5. Bradley PJ, Kochaar A, Quraishi MS. - Pharyngeal pouch - carcinoma: real or imaginary risks? - Ann. Otol. Rhinol.
Laryngol. 1999; 108:10271032.

19
5. Esophageal Cancer
Esophageal cancer is the most deadly cancer of the alimentary tract, even more
than pancreatic cancer. It ranks on the forth place after gastric, colon and rectal cancer,
as frequency. The main features of esophageal cancer, which makes it one of the most
lethal cancers, are:
1. It is very extended in surface and depth
2. It is difficult to treat
3. Treatment is followed by modest results
Epidemiology [1,2]
There are two main histological types of esophageal cancer: squamous and
adenocarcinoma. Squamous cell carcinoma is most frequently encountered in Asian
countries, the Middle East and in South Africa. In recent years the incidence of
adenocarcinoma has increased dramatically especially in Western countries. Based on
race, squamous cancer incidence is three times higher in black people, while
adenocarcinoma is more common in whites. Geographical distribution shows an
incidence of 20-30 times higher in China and Asia in general than in the U.S. and
Europe. Distribution by sex groups shows a three to five-fold higher incidence in males
than in the females. The average age of patients who develop esophageal cancer is 69-
70 years. Only two thirds of treated patients survive at 2 years and about 15% to 20% of
survive at least 5 years after diagnosis.[3]
Etiology and risk factors
The exact etiology of esophageal cancer is unfortunately unknown. Despite great
efforts in elucidating the disease, we follow the empirical observations, which establish
risk factors associated with esophageal cancer. Their presence does not mean that the
individual will develop esophageal cancer, but
the probability is higher. The following are the
risk factors most often incriminated:
1. Age over 55 years
2. Smoking and alcohol abuse
3. Hypoproteic, hypocaloric and rich in
fats diet
4. Gastroesophageal reflux and Barrett
esophagitis
5. Achalasia
6. Plummer-Vinson syndrome
7. Tylosis (palmoplantar keratoderma)
9. Benign stenosis
10. Local radiations
Morphopathology
Based on its location, esophageal cancer
can be classified as: (Figure 23)
Of the upper third
Of the mid third (thoracic)
Of the lower third

20
Based on histological appearance, it is classified as:
Squamous cell epithelioma (mostly spinocellular)
Glandular epithelioma (adenocarcinoma)
Other rare forms of cancer: sarcoma (<1%), lymphoma (1%), small-cells
carcinoma (2%), mucoepidermoid carcinoma (<1%), adenocystic carcinoma
(<1%) and spindle cell carcinoma (5%).[4]
Squamos cell epithelioma is mostly encountered in the upper part of the esophagus. Its
microscopic aspect is initially as a plate and then, exophytic and polypoid forms may
appear.
Adenocarcinoma, located usually in the lower part of the esophagus, is encountered
mostly in high-developed western countries. It represents more than 50% of all
esophageal cancers. From histological point of view is very similar to the gastric cancer
and is developed almost every time on a base of Barrett ulcer. Approximately 1% of
patients with Barrett ulcer will develop cancer within one year without treatment.[5]
Monitoring the patients with Barrett ulcer is indicated because:
1) There is no evidence that the medical treatment will vanish the risk of cancer,
and
2) Diagnosed in early stages, cancer can be cured.
Cancer extension. Tumoral cells spread in surface and depth invading
surrounding tissues and organs (trachea,
bronchus, aorta, pleura, etc). An important
feature is that local spread may be far away
from the original site due to enlarged
lymphatic network eyes, so multifocal
tumors may be encountered over a distance
of 5-6 cm from the originating tumor.
Lymphatic spread (Figure 24) goes towards
regional lymph nodes being an important
prognostic factor and also limiting the
curative operations.
Staging is of important value for
treatment indications and from prognosis
point of view. The stage is important to be
established before the beginning of
treatment.[6] Unfortunately this goal is
difficult to achieve because the esophagus
is an organ which is difficult to explore.
Many investigations are necessary to
evaluate the penetration of the tumor
through the esophageal wall (T stage) and for lymph nodes involvement (N stage). The
most useful investigations are:
Esophagoscopy with multiple biopsies
Endoluminal ultrasound examination
CT scans and MRI scans
Thoracoscopy and laparoscopy
PET (positron emission tomography) scanning with 18-fluorodeoxyglucose
has been recently incorporated into the staging evaluation of esophageal
21
cancer. This noninvasive test is more sensitive than CT for detecting distant
metastases.[7]
Even with all these investigation, often a correct stadialization can be achieved
only during operation. Keep in mind that this type of cancer may be multifocal.
Classification. TNM classification - American Joint Commission on Cancer (AJCC)
Staging for Esophageal Cancer
M stage (metastases): T stage
MX: Distant metastasis cannot be
assessed
TX: Primary tumor cannot be assessed
T0: No evidence of primary tumor
M0: No distant metastasis Tis: Carcinoma in situ
M1: Distant metastasis T1: Tumor invades lamina propria (T1a)
or submucosa (T1b) Tumors of the lower thoracic
esophagus: T2: Tumor invades muscularis propria
M1a: Metastases in celiac lymph nodes T3: Tumor invades adventitia
M1b: Other distant metastases T4: Tumor invades adjacent structures
Tumors of the midthoracic esophagus: N stage
M1a: Not applicable NX: Regional lymph nodes cannot be
assessed M1b: Nonregional lymph nodes and/or
other distant metastases N0: No regional lymph node metastasis
N1: Regional lymph node metastasis Tumors of the upper thoracic
esophagus: N1a: One to three nodes involved
M1a: Metastases in cervical nodes N1b: Four to seven nodes involved
M1b: Other distant metastases N1c: More than seven nodes involved

Stages
Stage 0 - Tis, N0, M0
Stage I - T1, N0, M0
Stage IIA - T2, N0, M0, T3, N0, M0
Stage IIB - T1, N1, M0, T2, N1, M0
Stage III - T3, N1, M0, T4, any N, M0
Stage IV - Any T, any N, M1
Stage IVA - Any T, any N, M1a
Stage IVB - Any T, any N, M1b
Skinner WNM classification modified by Ellis. (Table 1) (Figure 25)[8] This
classification is more adapted to reality and prognosis.
W stage represents the grade of wall
penetration
22
W0 - tumor limited to the
mucosa without penetrating the
muscular lining
W1- tumor passes muscularis
of mucosa, but did not cross the
esophageal muscles layer
W2 - tumor extended to
adventitia and neighboring organs
N stage represents lymph node metastases
N0 - without metastases
N1 - less than five lymph nodes are involved
N2 - more than five lymph nodes are involved
M represents distant metastases
M0 - no metastases
M1 metastases are present


Table 1 - Skinners WNM classification modified by Ellis
Stage W N M
0 W0 N0 M0
I W0
W1
N1
N1
M0
M0
II W1
W2
N1
N0
M0
M0
III W2 N1 M0
IV W0
any W
N2
any N
M0
M1
Symptoms. The onset is insidious and that is the main reason why many patients are
diagnosed in advanced stages of evolution. The patient with esophageal cancer usually
is a man in the 6
th
- 7
th
decade of age, smoker, with the habit of alcohol consumption,
who complains of retrosternal discomfort in early phases and then of all symptoms of
the esophageal syndrome. The main symptoms are:
1. Dysphagia is the main symptom being permanent, progressive, and generally
has a short duration of evolution. At first manifested for solid foods and then for
liquids and finally complete when the patient can no longer feed and die of
starvation.
2. There is an important weight loss
3. Retrosternal and epigastric pain
4. Superior digestive bleedings
5. Other symptoms from invaded or compressed organs such as hoarseness,
hematemesis, hemoptysis, esotracheal or esobronchial fistula, which are
characteristic for the period of complications due to loco-regional extension,
usually meaning the overcoming of curative surgery stage.
Physical examination reveals an asthenic patient, underweighted, with nicotine skin
impregnation. Particular attention should be given to supraclavicular lymph nodes
palpation. The presence of lymph node metastases at this level contraindicates surgery
with radical intent. Physical signs are poor especially in the early stages.
Workup
The most important is endoscopy because of direct visualization and the
possibility of performing biopsies. (Figure 26)

Radiological (barium swallow) investigation is also very important to establish
the precise location and relations with surrounding organs. It is important to assess the
shape of the stomach, which could be used for esophagoplasty. Axial or marginal
stenosis, with rigid wall and lacunar aspects, are the radiological features. (Figure 27)
Endoluminal ultrasound is important for T or W stage. (Figure 28)
CT and MRI scan for tumoral extension.
23
Bronchoscopy is useful to reveal the penetration of the trachea and bronchial tree.

Differential diagnosis should include benign stenosis, achalasia, diverticula, and reflux
esophagitis.
Treatment of esophageal cancer is complex and multimodal - mainly surgical plus
preoperative radio-chemotherapy in patients over 75 years for tumor conversion, also
applied in the postoperative period. Recent studies show that using either chemotherapy
or chemoradiotherapy before surgery is better than surgery alone.[6,9,10] Other
modalities of treatment are :
Laser tumor vaporization
Electrocoagulation
Application of stents
Preoperative preparation. The patient must be carefully investigated and
prepared before surgery. Besides the above-mentioned investigations, required for
primary diagnosis, further investigations are needed. Routine laboratory tests including
blood group and proteinemia will be performed. Cardiac function will be explored
establishing the necessary medication. Respiratory function will also be explored by
spirometry, since thoracotomy and manipulation of the lung reduce the ventilation
capacity of the lungs. Hepatic and renal function will also be evaluated. Gastroscopy (if
possible) and contrast imaging will explore the stomach to assess its availability for
esophagoplasty. The patient should undergo preoperative mechanical bowel preparation
by purgation (washout) considering that the colon may be used for esophagoplasty.
Electrolyte and protein deficits will be corrected. If the level of proteins is low, below
3.4 g%, the risk of anastomotic fistulas and infections is very high. In cachectic patients,
the best approach is to perform a preoperative feeding jejunostomy for enteral nutrition.
Jejunostomy may be useful in postoperative period or may be a definitive solution in
unresectable cases.
Following these investigations, the patient will be classified in one of the
anesthesia risk groups according to the scale ASA (American Society of
Anesthesiologists). (Table 2)
Table 2 - American Society of Anesthesiologists (ASA) Risk Classification
I A normal healthy patient
II A patient with mild systemic disease
III A patient with severe systemic disease
IV A patient with severe systemic disease that is a constant threat to life
V A moribund patient who is not expected to survive without the operation
VI A declared brain-dead patient whose organs are being removed for donor purposes
24
Surgical treatment. Some important aspects should be considered before
operation:
1. Most patients are elderly with associated illnesses
2. They are hypoproteic and cachectic through inanition
3. Many of them have altered respiratory function which contraindicates the
thoracotomy
There are two types of operations:
1. Curative (the intention is to cure by removing the tumor and corresponding
lymph nodes) , and
2. Palliative (the intention is only to prolong life and ensure a better quality of
life without removing the tumor. There are cases when the tumor can be
removed but the operation still has a palliative character due to the tumoral
spread.
In choosing the type of operation that will be applied some aspects should be
considered:[11]
Location of the tumor
Age of the patient
Biologic status
Tumor extension
Intraoperative staging
Location at the superior third of the esophagus
Tumors in this region are mostly squamous, being encountered more frequently in
women. Tumors located at the entrance in the thoracic cavity are frequently
inoperable due to early invasion of the trachea and great vessels.
Location at the thoracic esophagus
Those located in the superior part are frequently inoperable due to rapid invasion of
the surrounding organs. They are operable only if do not penetrate the esophageal
wall and are not spread in more than four lymph nodes.[12] Chemotherapy and
radiotherapy can be indicated as a first step for tumor conversion.
Location at the abdominal esophagus and cardia
Majority of them are adenocarcinomas and are suitable for en-block resection.
Radical intention surgery (curative) consists in tumor ablation by resection in
oncological limits associated with loco-regional lymph
nodes excision and reestablishment of the digestive
tract continuity.
Palliative surgery is represented by operations of
necessity when tumors are evidently unresectable or
the patients cannot be operated. Palliative solutions
are:
Palliative resections
By-passes
Stent application
Different kinds of stomas for alimentation
(gastrostomy, jejunostomy) (Figure 29)

25
Criteria for palliation are:
1. Age over 75 years
2. Recurrent laryngeal nerve palsy
3. Horner syndrome (compression on sympathetic chain)
4. Spinal cord persistent pain
5. Diaphragmatic relaxation (phrenic nerve palsy)
6. Malign pleural effusion
7. Respiratory obstruction or esobronchial fistula
8. Length of the tumor more than 9 cm
9. More than 20% weight loss
10. Multiple lymph node metastases and metastases at distance
11. Impaired ventilatory function with FEV1 <1.25 L (FEV1 is the volume of air
exhaled in one second during a forced maximal exhalation)
12. Others
Extension of the resection. Considering the extensive local spread of tumor, resection
should be performed at least at 10 cm above the tumor and that means, in almost all
cases, total esophagectomy and esophageal reconstruction using the stomach, colon or
small intestine. Resection and approaches depend on tumor location.
A. Tumors with cervical location
To remove the tumor and reestablish
the digestive tract continuity, three surgical
approaches are required. (Fig 30)
Cervical approach - The esophagus is
discovered in the cervical region and tumor
resectability is assessed. If resectable, the
esophagus will be sectioned above the tumor
in oncological limits associated with regional
lymphadenectomy. (Figure 31) If necessary,
laryngectomy will be performed and
tracheostomy also.
Right thoracic approach - The
esophagus will be dissected and separated
from surrounding organs through a right
posterolateral thoracotomy.
Abdominal approach - The stomach
(gastric tube) or the colon is prepared to be
ascended in the cervical region where it will
be anastomosed with the esophagus. (Figure
32)
When the tumor of cervical esophagus is unresectable, surgery consists in:
Feeding gastrostomy
Tracheostomy when the larynx is involved in the tumoral process
For the thoracic and abdominal location of tumors, the following operations may
be applied:
1. Resection en-block with curative intention
2. Palliative resections
3. Other palliative operations
Feeding gastrostomy, feeding jejunostomy, esophageal by-pass
26

B. Curative resection for thoracic location of tumors
Two or three approaches are necessary for this location of the tumor. (Figure 33)
The operation starts with the thoracic approach, via a right posterolateral
thoracotomy, and the assessment of the tumor resectability. If the tumor is resectable,
the esophagus and lymphatic tissue are
prepared and separated from neighboring
structures. If an intrathoracic anastomosis
is planned, in case of a lower
intrathoracic location of the tumor (Ivor
Lewis operation), the cervical approach
is not further necessary. If a cervical
anastomosis is planned, the thoracotomy
is closed and two drainage tubes are
placed into the pleural cavity, and then
follow the abdominal and cervical
approaches. If the tumor is not resectable,
a palliative operation is performed.
The operation continues with the abdominal approach. In this stage the
abdominal metastases are assessed and the modality of esophageal reconstruction using
the stomach or the colon. In this phase, it is still possible to abandon the
esophagectomy. The stomach or colon is prepared for esophagoplasty.
The cervical approach is used when a gastric tube or the colon, replaces the
entire esophagus, the anastomosis being performed in the cervical region. The
esophagus is sectioned in the cervical region. From this point, there is no possibility to
return - esophagectomy and esophagoplasty must be performed. The stomach or the
colon is ascended in the cervical region in orthotopic position and the anastomosis is
performed reestablishing the continuity of the digestive tract.

C. Curative resections for tumors of the abdominal esophagus
The operation starts with the abdominal approach. The resectability is assessed
and if the stomach is appropriate to be used for esophagoplasty. There are two main
possibilities for continuing the operation:
1. By transhiatal approach, as in Orringer Akiyama procedure, or
2. By right thoracotomy, as in Ivor Lewis operation
27
There are other possibilities too, but less used. Operations may be performed by
laparoscopic approach, or by thoracoscopic approach. Advantages of thoracoscopic
esophagectomy are:[13-15]
The numbers of lymph nodes removed are comparable with thoracotomy
A better approach to the subclavian and supradiaphragmatic regions
Less blood loss
Smaller incisions without rib resection
Faster recovery
Transhiatal esophagoplasty (Orringer Akiyama operation) (Figure 34)
There are two approaches: the first is the abdominal and the second is cervical.
The abdominal esophagus is prepared first and then the thoracic esophagus by blunt
dissection using fingers closely applied to the esophagus through the diaphragmatic
hiatus. The esophagus is separated by surrounding organs. Bleeding is minimal due to
the poor vascularisation of the
esophagus.
First, through the left cervical
incision the cervical esophagus is
found and isolated. Next, it is dissected
downwards using the same blunt
dissection until both hands meet in the
mediastinum. After that, the esophagus
is transected in the cervical region and
stripped out through the hiatus. (Figure
35) In most cases, pleural lesions will
not appear.
The greater curvature of the stomach is used for the new esophagus. The lesser
curvature is resected along with the esophagus. Resection can be performed more easily
using staplers. (Figure 36) As both vagus nerves are resected, a pylorotomy is needed to
facilitate the gastric emptying (after vagotomy the pylorus becomes hypertonic). The
arterial supply of the gastric tube will be ensured only by the gastroepiploic arteries,
which must be carefully protected against damages.
The gastric tube is ascended orthotopically in the cervical region where the
esogastric anastomosis is performed. (Figure 32)

When the stomach is not suitable for esophagoplasty (small stomach, previous
operations on stomach, etc.), the most frequently used organ is the colon (ascending or
transverse colon especially). The colon should be mechanically prepared before
operation. When the right colon is used, the ileocolic and the right colic vessels are
resected and appendectomy is also performed. The arterial supply will be ensured by the
middle colic artery or left colic artery. The cecum is ascended to the cervical region
28
either in orthotopic position or via retrosternal route. The transverse colon is resected
and the proximal end is anastomosed to the stomach. The distal end is closed and the
digestive tract is reestablished by ileotransversostomy. The ascending colon will be in
isoperistaltic position facilitating the swallowing. (Figure 37)

IvorLewis operation (Figure 38)
In this type of operation, the lower part of the esophagus and the upper part of the
stomach are resected, and the anastomosis is performed inside the right thoracic cavity.
The operation presumes a double approach: right thoracotomy and laparotomy.
First, through the thoracic approach the esophagus is dissected and resected above
the tumor. Next, through the abdominal approach, the upper part of the stomach is
resected too. The stomach is then ascended into the right hemithorax and the
anastomosis between the two organs is performed. Finally, the right pleural cavity is
drained and drains are sealed under water (Bulau drainage). Pylorotomy is needed
because the both vagus nerves are transected.
The removed esophagus may be replaced by other parts of the digestive tract such
as the stomach, colon (right or transverse most often), or small intestine. Small intestine,
even has a very good vascular supply, is rarely used due to the short mesentery, which
does not allow ascending it up to the cervical region.
In these kinds of operations, preserving a good vascular supply of the digestive
segment used for esophagoplasty is of utmost importance to prevent necrosis or
anastomotic leaks.

29
Palliative operations
In cases when the esophageal tumor is still resectable, but radical criteria are not
met, a palliative resection can be performed if the procedure is not too risky for the
patient in the hope that its life quality will be improved. During these operations,
extensive lymph node excision is not intended, but removing the obstacle by esophageal
resection. Unfortunately, these interventions are encumbered by a high percentage of
complications and a high rate of early postoperative mortality.
By-pass operations to avoid the tumoral obstacle are rarely performed due to the
bad prognosis.
If the patient is above the surgical resources, a feeding gastrostomy (Figure 39) or
jejunostomy (Figure 40) will be performed, avoiding death by starvation.
Less invasive, the endoscopic procedures are the best solution in advanced
esophageal cancer. If the tumor completely obstructs the esophageal lumen, an
endoscopic laser can be used to vaporize the tumor and drill a tunnel through which a
self-expandable stent is introduced. (Figure 41)
Possible postoperative complications
They are represented by general complications (especially pneumonia) that may
occur after any major abdominal or thoracic intervention but there are certain local
complications that deserve special attention:
Recurrent nerves paralysis appears in a percentage of 3-16% after transhiatal
procedure. In 50% of cases, they are temporary. This complication can be avoided
by keeping the dissection next to the esophagus without using electrocautery and
excessive traction. This complication predisposes to postoperative pulmonary
infections due to glottis closure disorders during swallowing and cough.
Anastomotic leaks incidence varies widely: 3-39%.[16,17] The consequences of
intrathoracic fistula are often dramatic and fatal. Cervical anastomotic fistula is
more likely to heal spontaneously. Effective drainage and nutrition by jejunostomy
are key elements in healing. In case of thoracic large fistulas reintervention is
required in most cases but is burdened by a high mortality, especially if
reintervention is delayed and the patient enters in advanced sepsis.[18] Recently
implantation of a self-expanding stent as a primary treatment to seal the leak became
the new trend with good results.[19, 20]
Anastomotic stenosis occurs at a rate of 20-40% and requires dilatation.[21-23]
Stenosis is more frequent after mechanical anastomosis. It appears at about three
months after surgery, manifested by progressive dysphagia. Generally, it requires 3-
5 dilatations. Late stenosis occurrence is caused, in the vast majority of cases, by
tumor recurrence.
Chilothorax, is produced by the rupture of the thoracic duct during esophagectomy.
It is a rare complication (2%). If drainage exceeds 1500 ml of lymph daily,
reintervention and thoracic duct ligation are required.[24]
Prognosis
Although great progresses have been made over the past 30 years in developing
methods for early diagnosis, survival rates remain very low. Only 35-45% of patients
survive at 2 years after surgery with radical intent, only 25% at 5 years, and only 5% at
7 months after palliation.[11]

30

References
1. Kollarova H, Machova L, HorakovaD, Janoutova G, Janout V. - Epidemiology of Esophageal Cancer. - An Overview
Article - Biomed Pap. Med. Fac. Univ. Palacky Olomouc Czech Repub. 2007; 151(1):1728.
2. Oesophageal cancer incidence statistics - Cancer Research UK - http://www.cancerresearchuk.org/cancer-
info/cancerstats/types/oesophagus/incidence/uk-oesophageal-cancer-incidence-statistics
3. Esophagus Cancer - American Cancer Society - http://www.cancer.org/acs/groups/cid/documents/webcontent/003098-
pdf.pdf
4. Shields TW, LoCicero J, Ponn RB, Rusch VW. - Less Common Malignant Tumors of the Esophagus. - Lippincott
Williams & Wilkins. 2005; pp. 23252340.
5. Barrett's Esophagus - The University of California, San Francisco - http://bariatric.surgery.ucsf.edu/conditions--
procedures/barrett%27s-esophagus.aspx
6. Ajani J, D'Amico TA, Hayman JA, Meropol NJ, Minsky B. - National Comprehensive Cancer Network - Esophageal
cancer. Clinical practice guidelines in oncology. - J. Natl. Compr. Canc. Netw. 2003; 1(1):14-27.
7. Ahmed A, David J. A, Thomas R. - Esophageal Cancer -
http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/hematology-oncology/esophageal-cancer/
8. Ellis FH Jr, Watkins E Jr, Krasna MJ, Heatley GJ, Balogh K. - Staging of carcinoma of the esophagus and cardia: a
comparison of different staging criteria. - J. Surg. Oncol. 1993; 52(4):231-235.
9. Cancer.Net Guide - Esophageal Cancer - Treatment - Printed October 4, 2012 from http://www.cancer.net/cancer-
types/esophageal-cancer/treatment
10. Medical Research Council Oesophageal Cancer Working Group. - Surgical resection with or without preoperative
chemotherapy in oesophageal cancer: a randomised controlled trial. - Lancet 2002; 359:1727-1733.
11. DAmico TA. - Surgery for Esophageal Cancer. - Gastrointest. Cancer Res. 2008; 2(4 Supplement 2):S6S9.
12. Ando N. - Future perspectives on the standardization of surgical treatment for esophageal cancer. - Nihon Geka Gakkai
Zasshi 2003; 104:390-394.
13. Low DE. - Open versus minimally invasive esophagectomy: what is the best approach? Frame the issue. - J. Gastrointest.
Surg. 2011; 15:497-499.
14. Kunisaki C, Kosaka T, Ono HA, Oshima T, Fujii S, Takagawa R, Kimura J, Tokuhisa M, Izumisawa Y, Makino H,
Akiyama H. - Significance of thoracoscopy-assisted surgery with a minithoracotomy and hand-assisted laparoscopic
surgery for esophageal cancer: the experience of a single surgeon. - Endo. I. J. Gastrointest. Surg. 2011; 15:1939-1951.
15. Bresadola V, Terrosu G, Cojutti A, Benzoni E, Baracchini E, Bresadola F. - Laparoscopic versus open gastroplasty in
esophagectomy for esophageal cancer: a comparative study. - Surg. Laparosc. Endosc. Percutan. Tech. 2006; 16:63-7.
16. Atkins BZ, Shah AS, Hutcheson KA, Mangum JH, Pappas TN, Harpole DH Jr, D'Amico TA. - Review Reducing
hospital morbidity and mortality following esophagectomy. - Ann. Thorac. Surg. 2004; 78(4):1170-6.
17. Turkyilmaz A, Eroglu A, Aydin Y, Tekinbas C, Muharrem Erol M, Karaoglanoglu N. - The management of
esophagogastric anastomotic leak after esophagectomy for esophageal carcinoma. - Dis. Esophagus 2009; 22:119-126.
18. Martin LW, Hofstetter W, Swisher SG, Roth JA. - Management of intrathoracic leaks following esophagectomy. - Adv.
Surg. 2006; 40:173-190.
19. Schweigert M, Dubecz A, Stadlhuber RJ, Muschweck H, Stein HJ. - Treatment of intrathoracic esophageal anastomotic
leaks by means of endoscopic stent implantation. - Interact. Cardiovasc. Thorac. Surg. 2011; 12:147-151.
20. Feith M, Gillen S, Schuster T, Theisen J, Friess H, Gertler R. - Healing occurs in most patients that receive endoscopic
stents for anastomotic leakage; dislocation remains a problem. - Clin. Gastroenterol. Hepatol. 2011; 9(3):202-210.
21. van Heijl M, Gooszen JA, Fockens P, Busch OR, van Lanschot JJ, van Berge Henegouwen MI. - Risk factors for
development of benign cervical strictures after esophagectomy. - Ann. Surg. 2010; 251(6):1064-1069.
31
22. Honkoop P, Siersema PD, Tilanus HW, Stassen LP, Hop WC, van Blankenstein M. - Benign anastomotic strictures after
transhiatal esophagectomy and cervical esophagogastrostomy: risk factors and management. - J. Thorac. Cardiovasc.
Surg. 1996; 111(6):1141-1146.
23. Rice TW. - Anastomotic stricture complicating esophagectomy. - Thorac. Surg. Clin. 2006; 16(1):63-73.
24. Mine S, Udagawa H, Kinoshita Y, Makuuchi R. - Post-esophagectomy chylous leakage from a duplicated left-sided
thoracic duct ligated successfully with left-sided video-assisted thoracoscopic surgery. - Interact. Cardiovasc. Thorac.
Surg. 2008; 7:1186-1188.

32
6. Esophageal Varices
Esophageal varices are dilated veins in the esophageal wall under the mucosa
layer, produced by the increased pressure in portal vascular system. Wolf has first
described them in 1928. Due to the gravitational forces, varices are located mainly in
the lower third of the esophagus. Varices are also frequently found in the upper portion
of the stomach. (Figure 42)
Portal vein, with a caliber of 6-8 mm,
forms by the confluence of the superior
mesenteric vein with the splenic vein behind the
head of the pancreas. The blood flow into the
portal vein is about 1.5 l/min representing one
forth from cardiac output. Portal vein supplies
75% of blood volume that passes through the
liver and 60% of its oxygen.[1] Normal portal
vein pressure is 5 mm Hg.[2] The first
consequences of pressures above this value are
splenomegaly and thrombocytopenia. The
esophageal varices are a later complication that appears at values above 10-12 mmHg in
portal pressure.[3] The rupture risk of varices is 9% at a pressure of 13 mmHg and it
rises up to 72% at a pressure of 17 mmHg.
The main complication of varices is bleeding. When bleeding occurs, the patient
becomes a major emergency case. Almost 50% of patients will develop bleeding from
varices. Even though gastric varices are bleeding less frequently than esophageal, the
hemorrhage is more severe and associated with a higher mortality rate. Mortality from
ruptured esophageal varices in the last few decades has decreased from 42% in 1981 [4]
to 15-20% in present days.[5,6] Bleeding will stop spontaneously in about 40-70% of
cases.[3] However rebleeding will occur in 40% of cases in the next 72 hours. Every
bleeding episode will shadow more and more the prognosis. If patients survive the
variceal rupture, there is approximately a 70% risk that they will have a further bleed
within the following 2 year.[7] The high mortality rate comes from some factors such as:
Liver insufficiency
Sepsis
Exsanguination
Brain edema
Complications of the anemia
Superior digestive bleeding is the most difficult to treat when is due to cirrhosis
and portal hypertension. Fast evolution, high frequency of bleeding events, jaundice,
ascites and neuropsychiatric manifestations are factors of severe prognosis.
Association between cirrhosis and peptic (gastric, duodenal) ulcer is well known
and raises the risk because the cirrhotic patient will bleed two times more frequently
than the patient without ulcer and three times more than those with ulcer but without
cirrhosis. Hematemesis in patients with liver cirrhosis may occur from three main
sources:[8]
1. Esophageal varices - 50% of cases
2. Gastric varices - 30% of cases
3. Gastro-duodenal ulcer - 9% of cases
However, association of the main three sources has been encountered.

33
Causes of portal hypertension are:
Prehepatic (portal vein obstruction)
Congenital atresia or stenosis of portal vein
Portal vein thrombosis
Splenic vein thrombosis
Extrinsic compression (tumors)
Hepatic
Cirrhosis
Congenital liver fibrosis
Idiopathic portal hypertension
Schistosomiasis
Posthepatic
Budd-Chiari syndrome
Constrictive pericarditits
Arterio-portal fistula
Increased splenic flow
Clinical aspects
Objectives of clinical examination in a patient with portal hypertension are:
1. To determine the cause of portal hypertension
2. To assess the liver functional reserve
3. To define the portal venous anatomy and hemodynamic status assessment
4. To determine the source of GI bleeding (if present).
History of chronic alcoholism, hepatitis or exposure to hepatotoxic agent raises
the suspicion of cirrhotic etiology of portal hypertension. In cirrhotic patient the
following aspects may be noticed:
1. Distended abdomen, ascites, splenomegaly and hepatomegaly
2. Visible venous network even periumbilical (the jelly fish head aspect or
"caput medusa")
3. Cruveilhier-Baumgarten murmur, heard in epigastric region produced by
collateral connections between the portal system and repermeabilized
umbilical vein
4. Spider angiomata
5. Gynecomastia, testicular atrophy and disappearance of men pattern hair in
men
6. Carmine lips and palmar erythema
7. Hemorrhoids
8. Jaundice
9. Encephalopathy manifestation
10. Other signs
The patient usually is brought by ambulance in emergency at surgical services
presenting hematemesis and melena. The general condition of the patient rapidly
declines due to the loss of volume and important signs encephalopathy appear (memory
loss, disorientation, tremors, and dizziness going to coma). Encephalopathy is caused by
the increased amonemia due to the impaired liver detoxification function of the
ammonium and other toxic substances resulting from digestion of blood in intestines.
Causes that determine the onset of bleeding from varices are the increased portal
pressure and esophageal mucosa ulcerations due to gastroesophageal reflux in most
34
cases. In other cases esophageal varices rupture is caused by injuries produced by
poorly chewed solid foods.
Laboratory data - reveal the liver impaired function and immunosuppression.
Accompanying hypersplenism in cirrhosis produces a moderate pancytopenia.
Anemia indicates a bleeding from varices, hemolysis, simple chronic malnutrition, or
bone marrow suppression associated with alcoholism.
Association of hyperaldosteronism with vomiting or diarrhea may increase fluid
and electrolyte imbalance with hyponatremia, hypokalemia, metabolic alkalosis, and
prerenal azotemia.
Coagulopathy is usually the consequence of deficiency of hepatic synthesis of
clotting factors, so that prolonged prothrombin time reflects the degree of liver
insufficiency. Similarly, liver failure, both the chronic and acute, is directly proportional
to the total bilirubin, and the degree of hepatocellular necrosis can be assessed by
amino-transferases values, consistently elevated in cirrhosis and chronic viral hepatitis.
1. Albumin levels are low
2. Decreased values of coagulation factors (II, V, VII, IX, and X)
3. Hyperamonemia
4. Thrombocytopenia, leukopenia, neutropenia
5. Anaemia
6. Aminotransferases (AST and ALT), bilirubin, are elevated
7. Others
To integrate the above factors into a predictive score of liver functional reserve
Child-Pugh introduced a score scale (modified by Turcotte) that allows prediction of
variceal bleeding and liver cirrhosis mortality. (Table 3 and 4)

Tble 3 - Child-Pugh classification of cirrhosis [9-11]
Factor Units 1 2 3
Serum bilirubin mol/L
mg/dL
< 34
< 2.0
34-51
2.0-3.0
>51
>3.0
Serum albumin g/dL >3.5 3.0-3.5 < 3.0
Prothrombin time Seconds prolonged
INR
0-4
< 1.7
4-6
1.7-2.3
>6
>2.3
Ascites None Easily controlled Poorly controlled
Hepatic encephalopathy None Minimal Advanced

Table 4 - Cirrhosis classes
Points Class One year survival Two year survival
5-6 A 100% 85%
7-9 B 81% 57%
10-15 C 45% 35%
The higher the score, the worse the prognosis is. [3]
Diagnosis of esophageal varices
Most frequently esophageal varices are diagnosed in emergency conditions during
the first episode of bleeding when endoscopic examinations are indicated to establish
the etiology of bleeding.
In nowadays, esophageal varices are diagnosed most frequently by endoscopic
investigation (esophagoscopy) and more rarely by barium swallow (esophagography). It
is important to assess the location (esophagus and/or stomach) and the size of varices.
(Table 5) Esophagoscopy visualizes directly the esophageal varices and also can be a
method of applying various treatments such as sclerotherapy and ligation. The
35
investigation is not riskless and is contraindicated in certain situations (myocardial
infarction).
Table 5 - Endoscopic aspects and classification of esophageal varices [12]
Grade Endoscopic aspect
I. Dilated veins (< 5mm) limited to the mucosa
II. Straight dilated veins (> 5 mm) bulging in lumen
III. Tortuous large veins which partially obstruct the lumen
IV. The lumen is completely obstructed by enlarged veins and there are cherry red spots and red
sign with a high risk or rupture
Abdominal ultrasound investigation is a harmless investigation but it can not
directly observe the varices. On the other hand it offers important information such as:
1. The morphology and dimensions of the liver and spleen
2. Vascular information: portal, mesenteric and splenic veins diameters,
presence of portal cavernoma, repermeabilization of umbilical vein, etc.
3. Presence of ascites
4. Duplex Doppler Sonography can detect the direction flow of blood stream in
the portal system.
CT and MRI scans give extra information about anatomical structures and the
morphology of the portal system and the aetiology of portal hypertension. These are
used mainly when a portosystemic shunt is intended to be performed or in case of liver
transplant.
Angiographic examinations offer extra information very useful in obscure cases
of portal hypertension.
1. Splenoportography - rarely used in nowadays
2. Selective arteriography of the superior mesenteric artery - venous phase
3. Transhepatic portography
4. Hepatic phlebography
Imagistic criteria for portal hypertension diagnosis are: [13]
1. Portal vein diameter >13mm (57% sensitivity, 100% specificity)
2. Superior mesenteric and splenic veins diameter >10 mm
3. Gastric vein diameter >4mm
4. Recanalization of umbilical vein >3mm (Cruveilhier-von Baumgarten
syndrome)
5. Lack of respiratory increase of splanchnic vein diameter (80%
sensitivity, 100% specificity)
6. Esophageal and gastric varices
7. Hepatofugal venous flow (82% sensitivity, 100% specificity)
8. Portal cavernoma
9. Splenomegaly
10. Ascites
Differential diagnosis usually includes the followings:
a. Causes of portal hypertension [3]
1. Schistosomiasis
2. Severe congestive heart failure
3. Hemochromatosis
4. Wilson disease
5. Autoimmune hepatitis
6. Portal/splenic vein thrombosis
7. Sarcoidosis
36
8. BuddChiari syndrome
9. Chronic pancreatitis
10. Hepatitis B
11. Hepatitis C
12. Alcoholic cirrhosis
13. Primary biliary cirrhosis (PBC)
14. Primary sclerosing cholangitis (PSC)Esophagus carcinoma
b. Causes of upper digestive bleeding
1. Superior vena cava obstruction
2. Klippel-Trenaunay and Parkes-Weber - syndromes (rare)
3. Gastro-duodenal ulcer
4. Hemorrhagic gastritis
5. Gastric cancer
6. Mallory-Weis syndrome
7. Diverse coagulopathies
8. Other causes of hematemesis
The diagnosis is quite easy in the presence of other symptoms and signs of
cirrhosis but must be confirmed by endoscopy.
Treatment
Treatment of esophageal varices is necessary due to the high risk of bleeding
followed by an increased risk of mortality. Therapeutic arsenal is vast and it has been
enriched in recent years with the acquisition of knowledge about the pathophysiology of
portal hypertension. The arsenal is represented by:
1. Pharmacotherapy
2. Endoscopic procedures
Sclerotherapy
Rubber bad ligation
Injection of bovine thrombin
Application of hemoclips
Argon plasma coagulation
3. Balloon tamponade with Sengstaken-Blakemore or Linton-Nachlas sonde
4. Transjugular intrahepatic portosystemic shunt (TIPS)
5. Surgical portosystemic shunts
Unfortunately, all these methods are used only to eliminate the danger of one of
the most important complications of portal hypertension, but the main cause remains. In
addition, some therapeutic methods are encumbered by high risks and side effects. As
most cases of portal hypertension are due to liver cirrhosis, liver transplantation is
currently the best solution in cases where suitable.
Therapeutic approaches can be classified into several categories such as:
In emergency conditions
Hemostasis by compression using the Sengstaken- Blakemore or Linton-
Nachlas tube
Surgical hemostasis - gastrotomy, haemostatic sutures, gastrorraphy.
Pharmacological therapy
In elective conditions
Surgical decrease of portal pressure (portosystemic shunts, azygoportal
disconnection)
TIPS (Transjugular Intrahepatic Portosystemic Shunt)

37
Endoscopic treatment
Rubber band ligation
Sclerosing substances (Sodium Tetradecyl Sulfat, Cyanoacrylat)
Coagulation with Argon plasma
Maintenance treatment
Lifestyle changes
Diet
Liver transplant
Another classification is:
1. Primary prophylaxis
2. Secondary prophylaxis - the therapy that prevents relapses of bleeding
I. Primary prophylaxis
Primary prophylaxis is considered the first therapy that prevents bleeding from
esophageal varices. In the same category enter the surgical portosystemic and
transjugular shunts, sclerotherapy and variceal ligation, but because of the risks and side
effects, especially of shunts, in nowadays, the pharmacotherapy is considered as a first
intention in primary prevention.
Cirrhotic patients should be examined endoscopically every 2-3 years and those
with high-risk of esophageal varices bleeding will benefit from primary prevention
methods.
The most used drugs are beta-blockers. Propranolol, blocking the beta-receptors,
will induce an alpha-adrenergic overactivity with the consequence of splanchnic
vasoconstriction and thus reducing the portal flow and pressure. Also reduces cardiac
output. Use of beta-blockers can reduce the risk of bleeding by 45% and deaths by 50%
from variceal bleeding. Propranolol dosage should induce a decrease of heart rate by
25% but not less than 55-60 beats / min.
When the effect of beta-blockers is unsatisfactory (20%) or there are
contraindications (20%), long-acting nitrates can be used. They produce vasodilatation
with decreased venous return and post-sinusoidal resistance and thus, consecutive
decrease of portal pressure. In high doses, produce a decrease of tension that will induce
splanchnic vasoconstriction with the consequent decrease in portal pressure.
Endoscopic ligation is the only invasive procedure recommended as primary
prophylaxis especially for varices with high risk of bleeding.
II. Treatment of superior digestive bleeding in emergency condition
The primary goal is to stop the bleeding and rebalance the patient (procedures are
performed simultaneously). Actions:
A. Rapid clinical evaluation of the patient
1. Collect biological specimens (blood, urine)
2. Clinical assessment (blood pressure, heart rate, temperature, blood
oxygenation, central venous pressure, diuresis, etc.)
3. A central venous catheter insertion for fluids administration and other drugs
4. Urinary catheter
5. Orotracheal intubation if the patient is unconscious
38
6. Nasogastric tube insertion to evaluate blood loss and for gastric lavage (not
recommended by some authors because it may produce rupture of the
varices) [14]
B. Resuscitation
1. If blood transfusion is not available in the first moment, intravenous albumin
in saline solution or saline will be administered in patients without ascites or
other signs of hepatic decompensation
2. Once the fluid balance restored, solution with low sodium (dextrose 5%) is
administered
3. Fresh frozen plasma is administered to patients requiring massive blood
transfusions. Hematocrit must be brought and maintained at the minimum of
30%
4. Calcium is also administered
5. At values < 50.000/ml platelets transfusions are administered
6. Gastric emptying, enemas and administration of lactulose are useful for
decreasing the amonemia
7. Nonabsorbable oral antibiotics (Neomycin 4x1 g/day for a week) should be
given for the same purpose and to reduce bacterial and endotoxin
translocation
8. The patient will receive treatment with vitamin K
C. Measures to stop the bleeding from varices
1. Pharmacotherapy
Octreotide acetate (Sandostatin) - by inhibiting the release of vasodilators
hormones (glucagon) indirectly produces vasoconstriction in splanchnic territory with
the consequent decrease in portal flow. It should be administered in dose of 50
micrograms for 5-10 min followed by a maintenance dose of 50 micrograms/hour for 3-
5 days.
Vasopressin (Pitressin) - posterior pituitary hormone, produces direct
vasoconstriction of vessels on splanchnic territory being able to reduce 60-75% of
variceal bleeding by reducing portal flow. It may produce several side effects caused by
vasoconstriction in other areas (heart, intestines, etc.). It is given in doses of 20 units for
20-30 min, and then 0.2 to 0.4 units/min. Along with vasopressin, nitroglycerin is
administered to counteract the vasoconstrictor effects on the myocardium.
Octreotide or vasopressin may be discontinued at 24 hours after cessation of
bleeding.
2. Hemostasis by balloon tamponade
The most commonly used is the Sengstaken-Blakemore tube. (Figure 43) The
method has a success rate of 90% in stopping bleeding but is very unpleasant for the
patient and has certain risks (pulmonary aspiration, esophageal rupture, esophageal
mucosa injury and ulceration, etc.) with a mortality rate of 5-22%. [15,16]
The tube can be used for gastric lavage and monitoring of any bleeding from
associated peptic ulcer.
Linton-Nachlas tube has one pear-shaped balloon and is indicated for bleeding
from gastric varices.
Balloons must be deflated at 24 hours after inflation to prevent damages to
esophageal mucosa. Even if the bleeding stopped, the tube will remain in place when
39
endoscopic methods are not available. The balloons can be re-inflated if bleeding
reoccurs.

3. Endoscopic procedures
Endoscopic procedures for definitive hemostasis should be applied as soon as
possible if bleeding can be controlled by pharmacotherapy.
Sclerotherapy and ligation have a success rate higher than hemostasis with
balloon tamponade. Both methods are effective, but ligation is encumbered by fewer
complications as sclerotherapy (bleeding, perforation, necrosis, stenosis, pleural
collections, etc.).
Concomitant use of Vasopressin and Octreotide increases the success rate in these
maneuvers.
4. Hemostatic surgery
If bleeding could not be stopped by tamponade or/and pharmacotherapy, or the
hospital does not have endoscopy facility, or bleeding could not be solved by repeated
endoscopic maneuvers, hemostatic surgery is required.
The less extensive operation is exploratory laparotomy followed by longitudinal
gastrotomy and hemostasis by ligation of sight bleeding varices usually located in the
fornix or around the cardia.
Other more laborious maneuvers such
as portal-systemic shunts or azygoportal
disconnection are burdened by high mortality
in emergency. However, when there are no
other solutions, they can also be performed
in emergency condition too.
Sugiura-Futagawa procedure,
introduced in 1967, is a non-shunting
operation to treat bleeding from esophageal
varices. Esophageal transection is associated
with devascularization of left esophageal and
gastric border starting intra-thoracic from the
lower pulmonary vein and continuing intra-
abdominal perigastric associated with
ligation of short gastric vessels, splenectomy,
40
vagotomy and pyloroplasty. (Figure 44) Bataglia and collaborators perform a
transection of the esogastric junction and reestablish the digestive continuity using a
circular stapler. Devascularization of the half top of the gastric body and the last 10 cm
of the thoracic esophagus is also performed. An antireflux fundoplication is performed
and splenectomy is associated in case of severe hypersplenism.
5. TIPS (Transjugular Intrahepatic Portosystemic Shunt)
A better alternative to surgical solution is TIPS, which can be a preceding
procedure in liver transplantation. (Figure 45)
Initially, under fluoroscopic guidance, a catheter is inserted by transjugular
approach through a suprahepatic vein,
transhepatic into an important branch of the
portal vein. Then, the transhepatic path is
dilated with a balloon and finally a metal
stent is inserted. The stent keeps the path
open and connects the portal vein to the
systemic circulation. The shunt reduces the
pressure in the portal vein and
consecutively in varices. The method is
burdened by risks of immediate
complications (hematoma, liver capsule
rupture, pulmonary edema, etc.), and late
complications such as portal
encephalopathy (20-35%), shunt stenosis
and thrombosis, and recurrent bleedings.[17]
The mortality risk of the procedure is only
10% which is a major advantage over
surgery.
III. Secondary prophylaxis
Bleeding relapse occurs in approximately 70% of cases in the first year after the
first episode. With each recurrence, the risk of death is higher, which is why the
secondary prophylaxis measures are necessary. These measures include pharmacologic
treatment and invasive techniques performed in elective conditions. These procedures
are represented by endoscopic variceal ligation or sclerotherapy and surgical methods
(TIPS, azygoportal disconnection) described above.
Vascular surgical procedures to reduce portal pressure
Since the introduction of portacaval shunt by Whipple in 1945, portal
hypertension surgery has evolved steadily.
Contraindications for this kind of surgery are:
1. Jaundice associated with ascites, edema and liver atrophy, or
2. Jaundice associated with ascites and increased amonemia.
Such surgery is applied as secondary prevention only after the failure of other less
invasive methods for the prevention of bleeding from esophageal varices. However,
besides liver transplantation, these methods are the only ones giving a fair chance of
survival.
The aim of these operations is to decrease the portal pressure by derivation of the
portal blood into the systemic circulation via a shunt. In most cases, a beneficial effect
41
on ascites in cirrhosis is obtained. The portacaval shunt may have some negative effects
on the brain (portal encephalopathy) that should be considered. Throughout history, a
wide variety of shunts has been developed, fact that proves that none is ideal. In
principle, there are two types of shunts: troncular and radicular.
A. Troncular shunts: (Figure 46)
1. End-to-side portacaval shunt (TANSINI)
2. Double portacaval shunt (Mc DERMOTT)
3. Side-to-side portacaval shunt (ECK)
The disadvantage of these types of shunt is the decrease of portal flow toward the
liver, accelerating the development of liver failure.
B. Radicular shunts (Figure 47)
1. Distal splenorenal shunt (WARREN) - most often used
2. Proximal splenorenal shunt after splenectomy (BLAKEMORE-
LINTON)
3. End-to-side mesentericocaval shunt (BOGORAZ, DRAPPANAS)
4. End-to-side cavomesenteric shunt (MARION)
5. Mesentericocaval shunt in "H" with graft (DRAPANAS-BANCU-
PAPAI),
6. Coronarocaval shunt (INOGUCHI)
7. Splenorenal shunt with interposition and ligation of splenic vein before
flowing into the portal vein (NAGASUE)
8. Portarenal shunt (left renal vein is implanted in the portal, mesenteric or
splenic vein)
9. Omfalocaval shunt
10. Splenocaval shunt (ASADA)
11. Splenosuprarenal shunt

42

Warren procedure: The distal end of the splenic vein is connected to the left renal vein
and the left gastric vein is disconnected from the portal vein. The results are:
1. The blood reflux from the portal vein to the gastric veins lowers
2. The blood flows from the varices through the splenic (portal circulation)
vein into the left renal vein (systemic circulation)
3. Pressure in the varices lowers
4. The blood flow to the liver is maintained through the portal vein
Portal hypertension surgery is a palliative surgery. Its primary aim is to stop the
bleeding. It has an increased rate of postoperative morbidity and mortality. Most
common postoperative complications are:
1. Portal encephalopathy
2. Rebleeding
3. Shunt thrombosis
Liver transplantation remains the surgical option for cure. (Figure 48)

43
44
References
1. Curtney S. Liver - Chapter 48 - Surgery: Basic Science and Clinical Evidence, second edition. 2008 Springer, edited by
Jeffrey A. Norton, Philip S. Barie, Ralph R. Bollinger, Alfred E. Chang, Stephen Lowry, Sean J. Mulvihill, Harvey I.
Pass, Robert W. Thompson.
2. Burroughs AK. - CHAPTER 9, The Hepatic Artery, Portal Venous System and Portal Hypertension: the Hepatic Veins
and Liver in Circulatory Failure - Sherlocks Diseases of the Liver and Biliary System, Twelfth Edition. Edited by James
S. Dooley, Anna S.F. Lok, Andrew K. Burroughs, E. Jenny Heathcote. - Published 2011 by Blackwell Publishing Ltd.
3. Esophageal varices - World Gastroenterology Organisation practice guideline. June 2008 -
http://www.worldgastroenterology.org/assets/downloads/en/pdf/guidelines/18_treatment_e_varices_en.pdf
4. Graham DY, Smith JL. - The course of patients after variceal hemorrhage. - Gastroenterology 1981; 80:800-809.
5. Chalasani N, Kahi C, Francois F, Pinto A, Marathe A, Bini EJ, et al. - Improved patient survival after acute variceal
bleeding: a multicenter, cohort study. - Am. J. Gastroenterol. 2003; 98:653-659.
6. Carbonell N, Pauwels A, Serfaty L, Fourdan O, Levy VG, Poupon R. - Improved survival after variceal bleeding in
patients with cirrhosis over the past two decades. - Hepatology 2004; 40:652-659.
7. McKay Rebecca, Webster NR. - Variceal bleeding. - Oxford Journals Medicine, BJA: CEACCP - 7(6):191-194
http://ceaccp.oxfordjournals.org/content/7/6/191.full
8. Odelowo OO, Smoot DT, Kim K. - Upper gastrointestinal bleeding in patients with liver cirrhosis. - J. Natl. Med. Assoc.
2002; 94(8):712715.
9. Harrison's Manual of Medicine 17/e - September 15, 2009.
10. Child CG, Turcotte JG. - Surgery and portal hypertension. In: The liver and portal hypertension. Edited by CG Child.
Philadelphia: Saunders 1964:50-64.
11. Pugh RN, Murray-Lyon IM, Dawson JL, Pietroni MC, Williams R. - Transection of the oesophagus for bleeding
oesophageal varices. - Br. J. Surg. 1973; 60(8):646649.
12. Spech HJ, Wrdehoff D. - Classification of esophageal varices - endoscopic and clinical aspects. - Leber Magen Darm
1982; 12(3):109-14.
13. Yong H. Hahn - Portal hypertension - CHORUS Collaborative Hypertext of Radiology - 2 February 1995 Last updated:
1 September 2006, http://chorus.rad.mcw.edu/doc/00863.html
14. Ritter DM, Rettke SR, Hughes Jr. RW, Burritt Mary F, Sterioff S, Ilstrup DM. - Placement of Nasogastric Tubes and
Esophageal Stethoscopes in Patients with Documented Esophageal Varices - Anesth. Analg. 1988; 67:2833.
15. Cook D, Laine L. - Indications, technique, and complications of balloon tamponade for variceal gastrointestinal bleeding.
- J. Intensive Care Med. 1992; 7(4):212-218.
16. Conn HO, Simpson JA. - Excessive Mortality Associated With Balloon Tamponade of Bleeding Varices. - A Critical
Reappraisal - JAMA 1967; 202(7):587-591.
17. Freedman AM, Sanyal AJ, Tisnado J. - Complications of transjugular intrahepatic portosystemic shunt: a comprehensive
review. - Radiographics 1993; 13(6):1185-210.

Surgical Pathology of the Stomach and Duodenum
SURGICAL PATHOLOGY OF THE STOMACH AND
DUODENUM

1. Surgical anatomy
2. Peptic ulcer
A. Gastric ulcer
B. Stress ulcers
C. Duodenal ulcer
3. Benign tumors of the stomach and duodenum
4. Gastric cancer
5. Gastric sarcomas
6. Gastric volvulus
7. Operated stomach pathology

1. Surgical Anatomy
The stomach
The stomach is the most dilated portion of the digestive tract, being located
between the esophagus and the small intestine. It is an intra-peritoneal organ. Its
capacity is about of 1000-1500 ml.
The stomach has several ligaments, anatomical structures more or less loose,
which give it certain mobility. (Figure 1) The most fixed parts of the stomach are the
cardia, which continues the esophagus, and the pylorus, continuing with the duodenum.
Excessive laxity of these ligaments may cause gastric volvulus.
The stomach has two faces (anterior and posterior), two curves (lesser and
greater) and two openings (cardia and pylorus). (Figure 2) On the lesser curvature of the
stomach the lesser omentum is inserted and on the greater curvature, the greater
omentum.

Behind the stomach there is a virtual cavity named bursa omentalis which
communicates with the peritoneal cavity through the Winslow hiatus (epiploic
foramen). This aspect is important because the cavity may become the place of different
kind of collections during pathological processes (retrogastric abscess during peritonitis,
pancreatic pseudocyst in acute pancreatitis, etc.). (Figure 3) Small intestines may enter
the foramen of Winslow and incarcerate (the internal hernia of Treitz).
45
The lesser curvature has two segments: one vertical and one horizontal separated
by the angular notch. The greater curvature starts from the angle of Hiss, continues with
gastric fornix and then describes an arch parallel to the lesser curvature, toward the
pylorus. The cardia, maintains its configuration and position due to some anatomical
structures: the membrane of Laimer Treitz, Bertelli, the Rouget muscle, left gastric
artery, vagus nerves and continuity with the esophagus. At level of cardia there is a
complex system acting like an unidirectional valve that prevents gastroesophageal
reflux, protecting the esophagus from the corrosive effects of gastric juice. This system
is represented by the angle of Hiss, the low esophageal sphincter, the right
diaphragmatic pillar and the Gubaroff valve. The lower opening, the pylorus
(sphincteric muscle), can be recognized by the presence of pyloric vessels (the vein of
Mayo).
Relations with surrounding organs are important especially in case of gastric
tumors, which can penetrate these organs (pancreas, colon, mesocolon, spleen and
liver). (Figure 4)

The anterior surface of the stomach, easily accessible, comes against the chest,
the abdominal wall, and the left and quadrate lobe of the liver. The posterior surface,
more difficult to surgical exploration, represents the anterior wall of bursa omentalis
through which the stomach comes in relation with the pancreas, splenic vessels, spleen,
left kidney and left adrenal gland and the transverse mesocolon.
Arterial supply
The stomach has a very good vascular supply. (Figure 5) It has four arterial
sources, all coming from the celiac trunk. These arteries form two vascular arcades
along the two gastric curves. The main arterial sources are:
1. Left gastric artery (coronary) - from the celiac trunk
2. Right gastric artery (pyloric) - from the hepatic artery
3. Left gastroepiploic artery - from the splenic artery
4. Right gastroepiploic artery - from the gastroduodenal artery
In addition to these sources, should also be mentioned the short gastric arteries
from the splenic artery and the diaphragmatic inferior artery, which irrigates the gastric
fornix.
Venous drainage
Gastric veins have the same routes as the arteries and they flow into the portal
system. Left and right gastric veins flow directly into the portal vein, the left
gastroepiploic via the splenic vein, and the right gastroepiploic vein via the superior
mesenteric vein.
46

Lymphatic drainage
The stomach has a very well represented lymphatic network. There are two
lymphatic networks: one submucous that drains the lymph through the muscular layer
toward the other plexus located in subserosa layer, from where extragastic lymphatic
vessels are starting.
There are four areas of lymphatic drainage of the stomach: (Figure 6)
1. Area I- the superior region of the lesser curvature from where the lymph is
drained towards the lymph nodes located around the gastric artery and
celiac trunk and toward the right paracardial lymph nodes.
2. Area II- the antral region of the lesser curvature is drained towards
suprapyloric and hepatic pedicles nodes.
3. Area III- the superior region of the greater curvature is drained towards
the lymph nodes of left gastro-epiploic artery, spleen hilum and left
paracardial nodes.
4. Area IV- the antral region of the grater curvature is drained towards the
lymph nodes of the right gastro-epiploic artery and subpyloric.
Knowing the lymphatic drainage of the stomach is important, to be able to
perform radical oncological operations that
besides removing the tumoral area should also
perform regional lymphadenectomy according to
the lymphatic drainage map.
Initially, Japanese specialists [1] described
16 groups of lymph nodes draining the stomach,
of particular importance in lymphatic excision in
surgical treatment of gastric cancer. (Figure 7)
1. Right paracardial
2. Left paracardial
3. Along the lesser curvature
4. Along the grater curvature
5. Right suprapyloric
6. Subpyloric
7. Along the left gastric artery
8. Along the common hepatic artery
9. At celiac trunk
10. From splenic hilum
47
11. Along the splenic artery
12. In the hepato-duodenal ligament
13. Retroduodenopancreatic
14. At the origin of the superior mesenteric artery
15. Along the mid colic artery
16. Para-aorto-caval
Later, other groups were added, such as:
17. On the anterior surface of the pancreatic head
18. Along the inferior margin of the pancreas
19. Infradiaphragmatic
20. In the esophageal hiatus of the diaphragm
110. Paraesophageal in the lower thorax
111. Supradiaphragmatic
112. Posterior mediastinal
Innervation
Knowledge about gastric innervation is
important to understand various physiological
processes of motility and gastric secretion, very
useful in gastric surgery, especially in peptic
ulcer.
Sympathetic innervation is derived from
spinal segments T5-T10 through the great
splanchnic nerve, celiac ganglia and from fibers
of perivascular plexuses.
The two vagus nerves (anterior and
posterior) provide parasympathetic innervation.
The left or anterior vagus can be found on
the front of the esophagus. It often splits into
branches at this level, so it rarely can be found as a single trunk. The right or posterior
vagus is located right behind the esophagus, in front of the aorta. (Figure 8)
Vagus nerves innervate the digestive tract up to the transverse colon. Lesions of
these nerves will affect more or less the function of these organs.
Vagus nerves ramify into branches such as: direct branches, hepatobilliary
branches and two motor nerves (Latarjet). Cutting the Latarjet nerves will results in
atony of the antrum (" the gastric mill") and permanent spasm of the pylorus.
Gastric wall structure
The stomach has the thickest wall of all segments of the digestive tract. (Figure 9)
From outside to inside the layers are:
1. Serosa - represented by the visceral peritoneum.
2. Muscular layer - composed of three sublayers: longitudinal (outer), circular
(mid) and oblique (inner).
3. Submucosa - composed of loose connective tissue, full of vascular,
lymphatic and nervous (Meissner) plexuses. Vascular plexuses are
particularly well represented as a true "sponge filled with blood."
4. Mucosa - forms longitudinal and transverse folds (rugae). Gastric glands,
simple or ramified, tubular type, are named after the region where they are
48
found: cardial, fundic, and pyloric. Antral mucosa cells have endocrine
secretory function the best represented being the gastrin-secreting G cells.

This special structure of gastric wall (resistant serosa, thick muscular layer and
the well-represented vascular supply) makes it a particularly good material for
anastomosis with low risk of developing anastomotic fistulas.
Types of cells encountered in the mucosa layer are:
1. Mucus producing cells
2. Parietal cells - producing HCl
3. Zymogene cells - producing pepsin
4. Endocrine cells
G cells producing gastrin being best represented in the antral mucosa
ECL (enterochromaffin-like cells) producing histamine
Delta cells producing somatostatin
Others
Physiology
The stomach has motor and secretory functions, particularly important in the early
stages of digestion.
Motor function allows the storage of foods, their kneading and mixing with
digestive juices and their progression into the duodenum. The stomach is normally
completely emptied after 3-4 hours. Motor activity of gastric emptying ("antro-pyloric
pump") is ensured by gastric antral peristaltic waves with a regularity of 3/minute. The
function is controlled by vagus and sympathetic autonomic nerves and by some
hormones.
The secretory function of the stomach is exocrine and endocrine.
Exocrine function is represented by the gastric juice, with intense acid pH,
secreted in volume of 1.5-3 L/24 hours. It is composed of hydrochloric acid, enzymes
(pepsinogen) and mucus.
Digestive enzymes are:
1. Pepsin, a proteolytic enzyme secreted in the inactive form of pepsinogen.
2. Labferment (gastric rennin, from the gastric secretion of newborn babies)
is involved in casein coagulation.[2]
3. Gastric lipase, which acts on short chain fatty acids, being important in
infants.
4. Cathepsin, a proteolytic enzyme that plays a role especially in infants.[2]
5. Gelatinase, is a proteolytic enzyme that hydrolyses gelatin with more
intense activity than that of pepsin.
6. Other enzymes are carbonic anhydrase, lysozyme and gastric urease.
49
The gastric juice is a colorless, clear to slightly opalescent fluid, with a very low
pH because HCl content. It contains 99% water and 1% organic and anorganic solids.
Gastric mucus is secreted by epithelial and mucous cells of the fundic and pyloric
glands. It provides physical protection against ingested particles and prevents direct
contact between the HCl and digestive enzymes, and the underlying mucosa. The roles
of HCl are:
1. Activates the pepsinogen to pepsin
2. Breaks down food proteins in more digestible forms of acidoproteins
3. Solubilization of the nucleoproteins and collagen
4. Converts Fe3+ to absorbable Fe2+
5. Antiseptic role - due to its corrosiveness, the HCl kills any pathogens. HCl
is so strong that the stomach lining would be digested in the absence of the
protective role of the mucus
6. Stimulates the Delta cells producing somatostatin
Intrinsic factor (Castle), secreted by parietal cells, is involved in the absorption of
vitamin B12 (Castle extrinsic factor).
Endocrine function. (Figure 10) The stomach wall secretes several polypeptides
with hormonal role, of which the best known are gastrin and somatostatin.
Gastrin is secreted in the antrum. It acts on enterochromaffin-like cells in
the gastric corpus to release histamine, which stimulates parietal cells to
produce acid.
Somatostatin has a role in regulating acid secretion and gastrin secretion.
Regulation of gastric secretion A complex neuro-endocrine mechanism
regulates the secretion in three phases:
1. The cephalic phase - in this phase, as much as 20-50 % of the maximal
acid response to a meal is produced, and the vagal mechanism plays the
principal role.
2. The gastric phase - in this phase, the endocrine regulation is the most
important but also the vago-vagal reflexes mediate acid response to
distension.
3. The intestinal phase - represents 5 - 10% of response by cholinergic neural
regulation and by endocrine regulation through gastrin, secretine and
cholecystokinin.
Vagus nerves play an important role in the regulation of acid secretion, gastrin
release, and histamine production. On this fact is based the vagotomy as a method of
treatment of peptic ulcer, which was introduced in practice by Dragstedt in 1943.
The cephalic phase of acid secretion occurs even before food enters the stomach.
Secretion is stimulated by the sight, smell, thought, or taste of food. When appetite is
depressed, this part of the cephalic reflex is inhibited. From the cortex, stimuli are
transmitted to the amygdala and hypothalamus and through the dorsal motor nuclei of
the vagi to the vagus nerves.
Important role in this stimulation have the excitatory action of thyrotropin-
releasing hormone (TRH) as well as the serotonin containing neurons.
50

The duodenum
Duodenum is the initial portion of the small intestine being the most fixed part. It
extends between pylorus and duodeno-jejunal flexure (Treitz angle). (Figure 11)
Duodenum is a secondary retroperitoneal organ being covered by peritoneum (the
coalescence fascia of Treitz). Due to its retroperitoneal position, it is difficult to access
during operation.
Duodenum has a horseshoe shape being divided into four parts:
1. Top (D1) - duodenal bulb
2. Descending (D2)
3. Horizontal (D3)
4. Ascending (D4)
The duodenum is neighboring with many organs: (Figure 12)
D1 with: the liver, gallbladder and liver pedicle
D2 with: the liver, gallbladder, right kidney, transverse mesocolon, right
colon and intestinal loops
D3 : anterior with the root of mesentery (superior mesenteric artery and
vein) and posterior with the aorta
D4 with: gastric antrum, jejunal loops and bursa omentalis

51
The structure of the duodenal wall
There are four layers: serosa, muscular, submucosa, and mucosa.
Circular folds (Kerkring) are missing in the upper portion of the duodenum.
Brunner glands, specific to the duodenum, are more common in D1. Secretion of these
glands is clear, viscous and alkaline (pH 8.2-9.3). In the mucosa, Lieberkuhn intestinal
glands are also present.
Although duodenal vascularization is quite rich, the retroperitoneal duodenal wall
is not a good material for sutures and anastomoses being at high risk of fistula because
of: the lack of visceral peritoneum (except D1), the poor developed of muscular layer
and the bilio-pancreatic content, which is intensive peptic.
Physiology
Inside the duodenum, foods are mixed with the biliary and pancreatic secretion
necessary for digestion. The alkaline duodenal content (bile and pancreatic juice)
neutralizes the acid content of the stomach.
Secretin and cholecystokinin hormones are released from cells of the duodenal
epithelium as a response to acidic and fatty stimuli when gastric chyme reaches into the
duodenum.
Secretin is a hormone with regulatory effect on the pH of the duodenal
contents via the control of gastric acid secretion and buffering with
bicarbonate from pancreas.
Cholecystokinin causes the release of digestive enzymes and bile from the
pancreas and gallbladder, respectively. It also acts as a hunger suppressant.
References
1. Japanese Classification of Gastric Carcinoma - 2nd English Edition - Japanese Gastric Cancer Association - Gastric
Cancer, 1998; 1:10-24.
2. Kelly EJ, Newell SJ, Brownlee KG, et al. - Gastric acid secretion in preterm infants. - Early Human Dev. 1993; 35:215-
220.

52
2. Peptic Ulcer
Peptic ulcer represents a local anatomical expression of a general disease. It is
mostly located in the stomach or duodenum, and less often in another segment of the
digestive tract. It is represented by an ulceration located initially on the mucosa and then
penetrating gradually all the other layers of the wall.
For many years, peptic ulcer was considered the result of faulty lifestyle,
particularly in terms of stress and diet, but modern theories associate ulcers with
bacterial infection and the use of drugs.
Gastric ulcer and duodenal ulcer was termed under the generic name of "ulcerous
disease" or gastro-duodenal ulcer. As knowledge progressed, it was found that gastric
ulcer and duodenal ulcer are two distinct diseases, which have many similarities but also
differences. For this reason, the two conditions will be treated separately.

A. Gastric Ulcer
Epidemiology
The incidence increases steadily with age, the tip being reached in the 6
th
decade
of life. The ratio between men and women is 2/1 but in India is higher. Only in Japan,
the gastric ulcer is more common than duodenal ulcer.[1-3]
Etiopathogenesis
The predominant role in the etiology of gastric ulcer is the less efficiency of local
defense factors resulting in an imbalance between aggression and defense factors of the
gastric mucosa.
Gastric mucosal defense factors are:
The viscous alkaline mucus
The tight connection between gastric epithelial cells
The renewal of epithelium lining every 3 days
Aggression factors are:
Acid-peptic hypersecretion - is less important than for duodenal ulcer
Gastric motility disorders with gastric stasis or delayed emptying
Duodenal-gastric reflux which impairs the mucus barrier
Helicobacter pylori infection, is less important in the genesis of gastric
ulcers than in the duodenal ulcer.[4] The bacteria colonize the antral region
of the stomach, causing chronic active gastritis (type B). This chronic
gastritis results in increased secretion of gastrin and therefore the
hydrochloric acid and peptic secretion too. In addition, H. pylori has a direct
ulcerogenic effect by releasing cytotoxic enzymes such as phospholipase and
proteases and also because of its spiral shape and flagella. The presence of
genes associated with cytotoxic effect (cag A) was isolated in 60% of cases.
Exogenous factors, such as: treatment with nonsteroidal anti-inflammatory
drugs (NSAIDs), intra-arterial chemotherapy, cortisone, excessive use of
laxatives. Non-steroidal anti-inflammatory drugs block the formation of
local prostaglandins responsible for the production of protective mucus by
blocking the cyclooxygenase 1 (cox 1) essential in the production of these
prostaglandins. Therefore, new non-steroidal anti-inflammatory drugs act
only on cox-2, less important in the secretion of these prostaglandins.
53
Glucocorticoids lead to atrophy of all epithelial cells, but it seems that their
role is still minor.
Smoking and stress
Zollinger Elisson syndrome
Genetic factors - blood group O seems to be more relate to duodenal
ulcer.[5,6]
Morphopathology
Gastric ulcer appears as a lack of substance in the gastric mucosa, usually single
but may be multiple and may be located anywhere from the cardia to the pylorus. Ulcer
margins are perpendicular and associated with chronic gastritis signs.
Depending on its depth, we can distinguish acute and chronic ulcers.
Acute ulcer may be represented either by a gastric erosion (exulceratio
simplex) limited to the mucosa, being of small size, which heals without
scars, or by a larger ulcer (about 1 cm diameter) that penetrates all gastric
layers, surrounded by swelling and redness, which heals leaving a visible
scar.
Chronic ulcer reaches 2-5 cm in diameter, has a round or oval shape, with
well-defined edges where there is always a chronic inflammatory infiltrate.
Because of this inflammatory reaction, the stomach may become adherent to
adjacent organs facilitating ulcer progression representing the ulcer
penetration. During the active phase, the ulcer has four zones: inflammatory
exudate, fibrinoid necrosis, granulation tissue and fibrous tissue. Fibrous
base of the ulcer may contain vessels with thin walls or thrombosis.
Classification
The most frequent location is the lesser curvature. Then follows, in order: anterior
wall, posterior wall, pre-pyloric region and grater curvature. Almost half of gastric
ulcers are located in the antral region.
Johnsons classification of gastric ulcers depending on location and secretory
status: (Figure 13) [6]
Type I: ulcer with high location on the upper part of the lesser curvature.
Acid secretion is reduced to normal value. Gastritis and duodenal reflux is
common. The incidence is 50-60% of patients.
Type II: located on the lesser gastric curvature and associated with pyloric
or duodenal ulcer. The level of gastric secretion may be normal or increased
and gastric emptying is delayed. It is considered secondary to duodenal ulcer
and appears in 23-25% of cases.[6]
Type III: ulcer located in antrum, pre-pyloric, which acts as a symptomatic
duodenal ulcer. It is associated with acid hypersecretion.
To this classification, Conter and Kauffman added two new types: type IV, ulcer
on the lesser curvature near the gastroesophageal junction and type V, ulcer at any
location, after consumption of aspirin or NSAIDs.
54

Symptoms
In recent years, symptoms have lost much of specificity so that the clinical picture
is no longer as characteristic as it was described in classical treaties. The evolution is in
acute episodes interrupted by asymptomatic periods.
The main symptom is the pain, usually with epigastric location, but other
locations are possible depending on lesions position. The pain is as if burning,
cramping, twisting or stabbing, induced or exacerbated by food ingestion and relieved
by evacuation of the stomach. Nausea and vomiting appear inconsistent in disorders of
gastric evacuation. Vomiting often has a calming effect on pain. Retrosternal heartburn
occurs mainly in ulcers located in a higher position or in those associated with
gastroesophageal reflux. Other symptoms are related to complications.
Diagnosis
Diagnosis relies on three elements: anamnesis, physical examination and
investigations. Physical examination offer poor elements for positive diagnosis. The
most important are investigations especially those endoscopic.
X-ray with contrast (barium or water-soluble
substances) may indicate the diagnosis in 80% of
cases. The direct radiological sign is the niche (ulcer
crater) that appears as a filling defect on gastric
contour and takes several forms: small, triangular,
pedunculated. The niche may be of medium size,
large with three levels inside: barium, fluid and air
(the Haudek niche) or giant. After evacuation, the
contrast remains inside the niche as a "persistent
spots." Sometimes, ulcerous niche is surrounded by
a halo of edema giving the aspect of target sign(en
cocarde). Indirect radiological signs are represented
by deformation of the lesser curvature, a ring of
spastic contraction of the greater curvature in
ulcerous region ("finger showing the lesion") and
the convergence of mucosal folds toward the ulcer.
(Figur
base
[7-9]
e 14)
Gastroscopy is the main investigation for
diagnosis. It directly visualizes the ulcer and allows
lesion biopsy sampling. (Figure 15) A single biopsy
sampling has an accuracy of 70% for malignant
ulcer, while seven biopsies taken from the ulcer
and margins increase the accuracy to 99%.
55
Differ
pter,
but th
ain such as duodenal ulcer, pancreatitis, biliary colic, hiatal
Evolu
ars, of the pharmacologic treatment, made the healing of gastric ulcer to be
the ru
ons of gastric ulcer may be acute or chronic and can occur at any stage

sis when ulcers are located in pre-
es more to the
ociated chronic gastritis, rather than ulcer formation itself.
Treat
response to
medic nt means that the patient is a possible candidate for surgery.
T
rgery may be an immediate emergency or performed in
-6 weeks did not lead to ulcer
er at the second or more event or
ia
o deformation of the stomach or duodenal bulb
er health care
ential diagnosis
The most difficult is to differentiate a benign ulcer from a malignant one. Gastric
cancer has radiological and endoscopic features that will be described in other cha
e main criterion of differentiation is the histology after endoscopic biopsies.
Based only on symptoms, differential diagnosis should include other diseases
causing upper abdominal p
hernia, angina and others.
tion and complications
Gastric ulcer is a chronic disease evolving for years. Remarkable progresses in
the last ye
le.
Complicati
of development.
1. Bleeding - appears in about 40% of patients, and is more serious and
refractory than that from duodenal ulcer. It may take the form of occult
bleeding or hematemesis, and/or melena.
2. Perforation - appears often as the first manifestation of the disease (onset by
complication). Especially ulcers on the anterior wall of the stomach perforate.
3. Penetration - is a particular form of perforation that appears especially when
ulcers are located on the posterior wall. The most frequent organ involved is
the pancreas but may be also the colon.
4. Stenosis - can occur either as a pyloric steno
pyloric region, or as a mid gastric stenosis.
5. Malignization - it is estimated that the percentage of malignization of gastric
ulcers is about 1-3%, and rarely a malignant ulcer can be differentiated from a
primary gastric cancer.[10-12] Tendency to malignancy relat
ass
ment
Conservative treatment of gastric ulcer uses the same drug groups as in
duodenal ulcer. Healing of ulcers should always be confirmed by endoscopy and always
compared with previous examinations. Any failure or delay in patients
al treatme
Surgery
he indications of surgical treatment can be classified as absolute and major.
1. Absolute indications refer to complicated gastric ulcer, regardless what the
complication is. Su
elective conditions.
2. Major indications include: [13]
Cases when proper medical treatment for 3
healing or at least a 60% reduction in size
Mild or moderate bleeding ulc
accompanied by chronic anem
Perforated and covered ulcer
Chronic ulcers, leading t
Gastric ulcer in elderly
Associated with duodenal ulcer
Those patients who do not have access to a prop
56
The objectives of surgical treatment in gastric ulcer are:
astric emptying and lavage are performed in cases
of ass
osing the level of gastric resection.
Ty
ior gastric resection)
ection of half of the stomach)
erior pole of the stomach (superior gastric resection)
y). It is
applied especially in cases associated with acid hypersecretion and stress ulcers.

1. Ulcer ablation (gastric resection)
2. Breaking the pathogenic chain, and
3. Restoration of digestive tract continuity as physiologically as possible
Preoperative patient preparation presumes correction of fluid, electrolytic,
hemodynamic and metabolic imbalances, and treatment of visceral (cardiovascular,
liver, kidney, lungs) dysfunctions. G
ociated outlet gastric stenosis.
Location of ulcer is the basic criterion in cho
pes of resection most often used are: (Figure 16)
1. Antrectomy
2. Resection of two inferior thirds of the stomach (infer
3. Hemigastrectomy (res
4. Subtotal gastrectomy
5. Resection of the sup
6. Total gastrectomy
Vagotomy is a procedure used to decrease the acid-peptic secretion, and is always
associated with an emptying method of the stomach (pyloroplasty or antrectom

Restoration of digestive tract continuity, in case of inferior gastric resection, can
be achieved by either anastomosis of the remaining stomach to the duodenum
(anastomosis Bilroth I type) (Figure 17) or to the first jejunal loop (Billroth II type)
brought to the stomach as an "omega" or "Y" (Roux) loop in front of the transverse
colon or through the mesocolon. (Figure 18) The gastro-duodenal anastomosis most
commonly used is the end-to-end anastomosis between the duodenum and the partially
sutured gastric stump (Pean-Billroth I) but there are many other possibilities.
57

Types of operation depending on ulcer type:
Type I of gastric ulcer located on the lesser curvature and accompanied by
hyperacidity requires distal gastric resection, including the ulcer, followed by
gastrointestinal Billroth I or Billroth II anastomosis (variants Pean, von
Haberer, Roux, Hoffmeister-Finsterer, Reichel-Polya, Balfour, etc).
Type II of gastric ulcer is located on the lesser curvature being associated with
duodenal ulcer, hyperacidity and delayed gastric emptying. Surgical
recommendation is bilateral truncal vagotomy associated with antrectomy and
gastro-duodenal or gastro-jejunal anastomosis.
Type III of gastric ulcer with pyloric or pre-pyloric location associated with
hypersecretion is best suited to vagotomy with antrectomy.
Type IV of gastric ulcer, located under the cardia, is best suited to a "saddle" or
"scale" gastric resection including the ulcer (Pauchet resection), followed by a
gastro-duodenal, or better gastro-jejunal anastomosis. Superior gastric resection
(Sweet) followed by esogastrostomy is rarely performed and encumbered by a
high rate of gastro-esophageal reflux. Kelling-Madlener procedure is
represented by truncal vagotomy and antrectomy
with gastric ulcer (type IV) left in situ. It must be
accompanied by multiple biopsies to ensure that
lesion is benign.
Type V of gastric ulcer, iatrogenic, usually
responds favorably to medical treatment, surgery
being indicated only in case of complications.
Surgical treatment of complications is almost similar
to that of duodenal ulcer complications.
A. In case of perforated ulcer:
1. Simple suture of the ulcer or suture with omental
pach (Opel, Graham) (Figure 19)
58
2. Ulcer excision and suture
3. Gastric resection including the ulcer
4. Truncal vagotomy plus antrectomy including the ulcer
Histological examination of the resected stomach is mandatory. A malignant
histopathological finding requires in most cases reintervention to perform a more radical
gastric resection.
B. In case of mid gastric stenosis, the stenotic part of the stomach will be
excised, followed by end-to-end gastro-gastric anastomosis.
C. Gastric bleeding from a single ulcer usually is treated by gastric resection
including the ulcer associated or not with vagotomy. Bleeding from stress ulcers
respond well in most cases to conservative treatment but there are rare cases when even
total gastrectomy is necessary to stop the bleeding.
B. Stress Ulcers
(See also the chapter of upper gastrointestinal bleeding)
Stress ulcers are special clinical forms of ulcers appeared on a normal mucosa due
to extreme stressful situations: multiple trauma, burns, major insufficiencies
(respiratory, liver, kidney), prolonged surgery, brain damage, etc.
There is a redistribution of blood circulating mass, which decrease gastric
irrigation, inducing an "acute" decrease of defense capacity of the gastric mucosa.
Lesions may be single or multiple (erosive gastritis). Always ulcers are "acute"
not surrounded by inflammatory reaction. Upper gastrointestinal bleeding is the
manifestation. Usually it is slow, intermittent, in rare cases heavy and fast.
Stress ulcer treatment is prophylactic (avoiding causal conditions) and curative
consisting in gastric emptying (nasogastric tube), local hemostatics, proton pomp
inhibitors, and replacement of lost blood. When these conservative measures prove
ineffective, surgical hemostasis including vagotomy and in rare cases even total
gastrectomy become necessary.
References
1. Kurata JH, Haile BM. - Epidemiology of peptic ulcer disease. - Clin. Gastroenterol. 1984; 13(2):289-307.
2. Watanabe Y, Kurata JH, Kawamoto K, Kawai K. - Epidemiological study of peptic ulcer disease among Japanese and
Koreans in Japan. - J. Clin. Gastroenterol. 1992; 15(1):68-74.
3. Lam SK. - Differences in peptic ulcer between East and West. - Baillieres Best Pract. Res. Clin. Gastroenterol. 2000;
14(1):41-52.
4. Suerbaum S, Michetti P. - Helicobacter pylori infection. - N. Engl. J. Med. 2002; 347(15):1175-1186.
5. Beasley WH. - Blood Groups of Gastric Ulcer and Carcinoma. - Br. Med. J. 1960; 1(5180):1167-1172.
6. Johnson D. - Gastric ulcer: classification, blood group characteristics, secretion patterns and pathogenesis. - Ann. Surg.
1965; 162(6):996-1004.
7. Sanjeeb Shrestha, Daryl Lau - Gastric ulcers http://misc.medscape.com/pi/android/medscapeapp/html/A175765-
business.html
8. Llanos O, Guzmn S, Duarte I. - Acuracy of the first endoscopic procedure in the differential diagnosis of gastric lesions.
- Ann. Surg. 1982; 195(2):224-226.
9. Yeowon Choi, Hyo Sun Choi, Woo Kyu Jeon, Byung Ik Kim, Dong Il Park, Yong Kyun Cho, Hong Joo Kim, Jung Ho
Park, and Chong Il Sohn - Optimal Number of Endoscopic Biopsies in Diagnosis of Advanced Gastric and Colorectal
Cancer - http://dx.doi.org/10.3346/jkms.2012.27.1.36 - J. Korean. Med. Sci. 2012; 27:36-39.
10. Finsterer H. - Malignant Degeneration of Gastric Ulcer. - Proc. R. Soc. Med. 1939; 32(3):183-196.
11. Rubin E, Reisner H. - Essentials of Rubins Pathology 5Th Edition 2008.
12. Hansson LE, Nyrn O, Hsing AW, Bergstrm R, Josefsson S, Chow WH, Fraumeni JF, Adami HO. - The Risk of
Stomach Cancer in Patients with Gastric or Duodenal Ulcer Disease. - N. Engl. J. Med. 1996; 335:242-249.
13. Jamieson GG. - Current Status of Indications for Surgery in Peptic Ulcer Disease. - World J. Surg. 2000; 3(24): 256-258.

59
C. Duodenal Ulcer
Epidemiology
Duodenal ulcer occurs more frequently in adulthood, being about four times more
common than gastric ulcer. The maximum incidence is between the forth and fifth
decade of live, and the ratio between men and women is 4/1-6/1.
The incidence of duodenal ulcer decreased in last decades due to the development
of new antisecretory drugs, but still remains at 1-2%.[1] In nowadays, admission in
hospital is required only in complicated cases of duodenal ulcer. Duodenal ulcer never
turns malignant.
Etiopathogenesis
Determinant (endogenous) factors:
Gastric acid hypersecretion
Decrease of duodenal mucosal defense capacity
Helicobacter pylori infection is now recognized as a major factor for the
development of ulcerous disease and an important risk factor in gastric
cancer. The presence of bacteria was found in 90% of patients with duodenal
ulcer.[2,3] The lifetime risk of peptic ulcer in a person infected with H. pylori
ranges from 3 % in the United States to 25% in Japan.[4,5]
Predisposing (exogenous) factors:
External factors incriminated in duodenal ulcer: smoking, alcohol
consumption, poor nutrition, chronic intake of inflammatory drugs,
psychological factors
Genetic factors are also important because the disease frequently occurs in
members of the same family and in those with O (I) blood type.[6,7]
Morphopathology
Duodenal ulcer appears as a single or
multiple ulcerations located with predilection in
the duodenal bulb (D1).
The ulcer crater has an inflammatory
reaction around it, which over the time turns into
fibrosis, specific for the callous ulcer.
In case of healing, the ulcer leaves a white
scar, as a star, visible macroscopically. The
process of fibrosis can lead to duodenal
deformation and appearance of stenosis and
pseudodiverticula. (Figure 20)
Symptoms
The main symptom is the epigastric pain manifested as a burning discomfort,
cramping or twisting. Usually it occurs at 1-2 hours after ingestion of food. It can
appear during night or morning and it gives the feeling of "hunger pain". The pain
relieves after the ingestion of milk or alkali. The picture above is the so-called the
small periodicity in duodenal ulcer (pain-ingestion-relief-pain). Symptoms usually are
exacerbated in spring and autumn - the great periodicity or seasonal periodicity. As
the disease progresses, the character of pain may change.
Vomiting is not characteristic for uncomplicated duodenal ulcer, but when it
occurs, it has a pain-relieving effect.
60
Other possible symptoms are characteristic for complicated forms.
Signs
Physical examination is usually negative, but it could reveal tenderness on
palpation in the epigastrium and duodenal point.
Phenotypic appearance of the patient suffering from duodenal ulcer is usually of
an asthenic, brown-haired person, with a disorganized diet, smoker and possible
alcoholic and coffee consumer (ulcerous aspect patient").
Workup
The barium swallow may reveal the peptic
ulcer by direct and/or indirect imagistic signs. The
direct sign is the niche, which appears as a filling
addition or as a suspended spot surrounded by a halo.
(Figure 21) Indirect signs are: the convergence of the
mucosa folds, rapid evacuation of the duodenum and
duodenal deformity in old scar, when it takes the form
of "clubs", "hourglass", "hammer", etc. Barium
swallow can make the diagnosis in only 50% -60% of
cases, but this percentage may reach up to 95% for
ulcers larger than 1 cm and when dual contrast is
used.[8]
Endoscopy is the standard method of diagnosis
of duodenal ulcer, with specificity over 90%. (Figure
22)
Differential diagnosis should include other
conditions associated with epigastric pain.
A. Digestive diseases to be differentiated from
a duodenal ulcer are:
Gastric ulcer and cancer - the pain does not
have the character of small periodicity and
seasonality and it appears immediately after
food ingestion. Gastric biopsy is very
important.
Acute gastric volvulus - the pain is intense, with abdominal distension, intense
nausea the patient being not able to vomit, and the nasogastric tube cannot be
introduced into the stomach (Borchordts triad). Chronic volvulus gives more
discrete symptoms, with less pain, associated with the feeling of early satiety,
stroscopy with biopsy and specific
ound shows the gallstones. Murphy maneuver reveals pain in
the cystic point.
dysphagia and dyspnea.
Gastric TB and syphilis - there are permanent epigastric pains in a patient with
history of tuberculosis or syphilis. Ga
laboratory tests will make the difference.
Biliary diseases - biliary colic occurs after certain foods, is located in the right
upper quadrant with radiation to the scapulovertebral space; is not calmed after
alkaline and antacid intake but is calmed after antispasmodic drugs; it is often
associated with nausea and vomiting and sometimes with jaundice and fever.
Abdominal ultras
61
Acute pancreatitis - the pain is localized in the epigastrium with transverse
radiation; it does not respect the small and great periodicity; appears after an
excessive consumption of food and/or alcohol, and is accompanied by nausea
and vomiting. The general status is altered going up to state of shock.
Ultrasound and CT scan reveal changes in pancreas, serum and urinary amylases
are generally elevated.
Chronic pancreatitis - the epigastric pain is still present, but without the small
and great periodicity, does not calm after alkali or food intake, in a patient with a
history of recurrent acute pancreatitis, digestive disorders and often diarrhea.
Ultrasound examination and CT can reveal the presence of Wirsung duct
dilation and pancreatic lithiasis.
Pancreatic cancer - is associated with constant stabbing epigastric pain with
radiation to the middle of the back, calmed in the "Mohammedan position. The
patient presents progressive weight loss. In cephalic location, verdinic jaundice
and Courvoisier-Terrier sign are present. Ultrasound and CT scan reveal the
tumor.
Ascending and transverse colon tumors - are manifested by pain rather colicky
accompanied by diarrhea and/or constipation. Often patients are anemic with
progressive weight loss. Irigography and colonoscopy with biopsy can establish
the diagnosis.
Abdominal angina - there is a mesenteric ischemia in patients with atrial
fibrillation, atherosclerosis, and history of previous thromboembolism. The pain
appears postprandial, located mainly around the umbilicus, with impaired
general condition, flatulence, vomiting and bloody stools, with the possibility of
intestinal infarction and evolution to death. The diagnosis is difficult, often
established only by laparotomy. Selective mesenteric angiography can make the
diagnosis.
B. Neighborhood disorders :
Renal colic - the pain is intense, colicky, located in lumbar region radiating into
inguinoscrotal (labial) region, accompanied by nausea, vomiting, dysuria,
polakyuria and hematuria. Giordano sign is positive. Ultrasound may reveal
dilatation of the upper urinary tract and stones. Atispastic drugs may calm down
the pain.
Hiatal hernia - the pain is located mainly retrosternal as heartburn and
regurgitation is often associated due to gastroesophageal reflux. Cardiac
arrhythmias and dyspnea are other complaints. Barium swallow in
Trendelemburg position and esophagoscopy will make the diagnosis.
Internal hernia - intense colicky abdominal pain followed by occlusive
phenomena with vomiting. Most cases are intraoperative surprises.
Posterior inferior myocardial infarction - there are intense epigastric pains
sometimes difficult to distinguish from an abdominal disease. This condition
must be considered in any intense epigastric pain, especially in predisposed
patients because of its ability to progress to death. ECG, cardiology specialty
examination and laboratory tests can rule out a heart attack.
Basal pneumonia and pleurisy - pain is usually located at one hemithorax or
hypochondrium, accompanied by respiratory events (cough, dyspnea) and fever,
62
and has nothing in common with alimentation. Fluoroscopic examination reveals
pleuro-pulmonary changes.
Diseases of the spine - can generate pains in epigastric or hypochondrium region
misleading the diagnosis. Postural pain usually is enhanced by certain
movements and reduced by anti-inflammatory and muscles relaxant drugs.
Other systemic diseases that can mimic symptoms of a duodenal ulcer: diabetes,
saturnine colic, tabes, porphyria, but in clinical practice they are rarely
encountered.
Particular anatomoclinical forms
Postbulbar ulcer is an ulcer located on the descending part (D2) of the
duodenum. In most cases is difficult to diagnose either by endoscopic or
radiologic examinations due to its lower location. It may evolve with
periulcerous inflammatory reaction penetrating the Vaters ampulla or even the
hepatic pedicle. It often complicates with bleeding, stenosis and penetration into
the pancreas.
Double or multiple ulcers, also known as "kissing ulcer", are severe forms,
burdened by high rate of bleeding and perforation. Ulcers are located in mirror
on the anterior and posterior duodenal wall.
Duodenal ulcer associated with gastric ulcer is quite common.
Giant duodenal ulcer can attain considerable size, with a diameter up to 6 cm of
the crater, penetrating deep into the pancreas giving permanent pain and weight
loss.
Duodenal ulcer in elderly frequently occurs due to local age-specific circulatory
disorders and chronic intake of inflammatory drugs. Clinical manifestations may
be atypical.
Zollinger-Ellison syndrome is a condition characterized by the appearance of
multiple ulcers in the digestive tract, the acid-peptic hypersecretion being caused
by increased levels of gastrin produced by a gastrinoma. In most cases,
gastrinoma is located in pancreas but other locations are possible: duodenal wall,
stomach wall, liver or spleen. It has an incidence of 1%, most commonly
affecting patients between 30 and 60 years. The clinical picture is that of
duodenal ulcer, but with some particularities: pain is extended, intense, does not
diminish after intake of milk and alkali nor after specific medication, vomiting is
usual and very abundant, diarrhea and steatorrhea are present. Diagnosis is based
on dosing the blood gastrin level, which is found to have much higher values
than normal. CT or MRI scans rarely can highlight the tumor (gastrinoma).
Evolution and complications
Complications may occur at any time and are represented by:
1. Bleeding
2. Perforation
3. Stenosis
4. Penetration
Treatment of duodenal ulcer
Conservative treatment has two goals: relief the pain and heal the lesion. These,
can be achieved by:
63
1. Administration of the latest generation of antacid drugs represented by
proton pump inhibitors (PPI) such as Pantoprazolum, Esomeprasolum,
Omeprazole, etc.
2. Digestive mucosa protective drugs containing aluminum, bismuth, etc.
3. Eradication of Helicobacter pylori (Amoxicillin, Clindamycin, etc.)
4. Diet
Surgical treatment
If anyone should consider removing half of my stomach to cure a small ulcer in
my duodenum I would run faster than he - Charles E. Mayo, 1927. Surgery has become
increasingly rare, practically addressed to complicated cases. The objectives of surgery
are:
1. Decrease the acid secretion
2. Removal of the lesion when possible
3. Ensure gastric drainage as physiological as possible
The decrease of gastric acid secretion can be achieved by three surgical
procedures:
1. Parasympathetic denervation - eliminates the vagus nerve stimulation of
acid secretion
2. Gastric resection more or less extensive - decreases the parietal cell mass
3. Antrectomy - decreases the secretion of gastrin
Surgical vagotomy cuts off the vagal innervation of the gastric wall in order to reduce
gastric secretion. It was introduced in practice as safe procedure for peptic ulcer disease
by Dragstedt in 1943 in North America.[9] Popular up the 90
s
, vagotomy was
increasingly less used after the discovery by Barry Marshall and Robin Warren of the
fact that Helicobacter pylori is responsible for most cases of ulcer. There are four types
of vagotomy: (Figure 23)
1. Truncal vagotomy - the two vagus nerves are cut at esophagus level before
branching. The operation can be performed by abdominal or thoracic approach.
Truncal vagotomy has a destructive effect because it interrupts the
parasympathetic innervation of the digestive tract from the stomach to the
transverse colon. Intestines will still work but with some difficulty.
2. Selective vagotomy (Jackson-Franksso, Grifith 1960) - cuts off the vagus nerves
after separation of the hepatobiliary branches.
Because truncal and selective vagotomy interrupt the motor fibers of Latarjet
nerves, inducing gastric atony and spasm of the pylorus, these types of
procedures should be associated with a procedure that ensures gastric emptying.
3. Supraselective vagotomy (highly selective, parietal cell vagotomy - Amdrup
1969) - cuts off the vagal branches strictly near the gastric wall so that the other
branches, including Latarjet nerves, remain integer. No associated procedure of
gastric emptying is necessary.
4. Posterior vagotomy associated with anterior sero-myotomy (Taylors operation)
spares the anterior vagus and its motor branches.
64

Gastric emptying procedures associated to vagotomy are:
1. Operations addressed to the pylorus: (Figure 24)
Extramucosal pylorotomy (Fredet procedure) - pyloric muscle is
sectioned respecting the integrity of the mucosa.
Pylorotomy-pyloroplasty - the pylorus is sectioned including the
mucosa and then sutured in a manner that enlarges the pyloric
opening (Heineke-Mikulicz, Finney, Mayo, Strauss, Horsley, Chabal,
procedures etc.).
Pylorectomy-pyloroplasty - a partial excision of the pylorus including
the ulcer is performed (Wangensteen, Judd-Starr, Mayo, etc.).
2. Antroduodenostomy - an extrapyloric communication between the antrum
and duodenum is performed (Villard-Jaboulay, Pean, Burlui procedures).
(Figure 25)
3. Gastroenteroanastomosis (GEA) - in nowadays are used for gastric drainage,
as palliative operation in cases of inoperable tumors with antro-duodenal
stenosis (stenosing antral gastric cancer, pancreatic cancer, Vaters
ampulloma, renal cancer penetrating into the duodenum, etc.). (Figure 26)
4. Antrectomy

65

Vagotomy associated with antrectomy is currently considered the most
appropriate method of surgical treatment of duodenal ulcer. Restoration of digestive
continuity is performed by a Billroth I or II type of anastomosis. (Figure 27)

Complications of Duodenal Ulcer
A. Bleeding
This complication represents a major problem of health. It appears in 20-50% of
cases [10-12] being the main cause of mortality (6-14% at 30 days).[13-15]
Modalities of onset can be dramatic with hematemesis or melena or insidious. It
may be the first manifestation of the disease. It was estimated that approximately one-
third of patients with a bleeding ulcer would develop recurrent bleeding in the following
1-2 years without testing for, and eradication of H. pylori.[16]
Most often, the erosion occurs on the posterior wall of the duodenal bulb where
the ulcer erodes the pancreaticoduodenal artery.
Symptoms are related to acute anemia: pallor, sweating, thirst, restlessness,
faintness, tachycardia and hypotension.
Bleeding manifests as:
1. Hematemesis
2. Hematochezia
3. Melena
66
Hematemesis is a serious symptom accompanying heavy bleeding. In massive
bleeding undigested blood may appear in stool as fresh clots (hematochezia). A systolic
blood pressure less than 80 mm Hg is an indicator of severity. Laboratory emphasizes
the decrease of hematocrit and hemoglobin, which is evident after 24 hours of initial
bleeding due to the initial hemoconcentration.
Diagnosis is based on digestive endoscopy, which may reveal more than 90% of recent
or ongoing bleeding.
Hematemesis - will be differentiated from nasopharyngeal or teeth bleeding,
hemoptysis, and other causes of upper gastrointestinal bleeding: esophageal
varices, gastric ulcer, gastric tumors, esophageal ulcer, gastritis, vaterian
ampulloma, hiatal hernia, Mallory -Weiss syndrome.
Melena and hematochezia will be differentiated from small intestine tumors,
colorectal polyps and cancer, colic angiodysplasia, congenital disorders,
treatment with drugs based on bismuth, aluminum or iron.
Treatment
Upper gastrointestinal bleeding is a surgical emergency, the patient being
admitted to a department of surgery, and depending on the severity of bleeding, in the
intensive care unit for better monitoring. When hemostasis can be achieved by
conservative methods or by endoscopy, the patient may be transferred to the
gastroenterology department.
The main purposes of treatment are to stop bleeding, rebalance the patient and
prevent rebleeding. Treatment may be pharmacologic, endoscopic or surgical.
General measures: ensure adequate venous access (central venous catheter) for
administration of fluids and for measuring the CVP (central venous pressure),
nasogastric aspiration to monitor bleeding and gastric lavage, urine probe to monitor
urine output, blood pressure monitoring, pulse oximetry, etc.
Fluid rebalancing by replacing lost blood, crystalloid and macromolecular
solutions.
Antacids (80 mg PPI in bolus over 30 minutes, followed by a continuous infusion
of 8 mg/h administered for 3 days) and hemostatics (Adrenostazin, K vitamin,
Calcium).[17,18]
Somatostatin and its analogue Octreotide reduce splanchnic blood flow, inhibit
gastric acid secretion and have a cytoprotective effect. They can be used as adjunctive
therapy before and after endoscopy.[19] Somatostatin is used in dose of 250 micrograms
iv bolus followed by hourly administration of the same dose infusion for 3-7 days.
Octreotide dose is 50-100 micrograms in bolus followed by 25 micrograms per hour for
3 days.
Gastric aspiration with lavage and medication can stop the bleeding if it is not
produced by an important vessel (gastroduodenal artery). Emptying the stomach is very
important in hemostasis. Lavage is performed with cold, alkaline and hemostatic
solutions (adrenostazin) until clarification of lavage fluid.
Nasogastric aspiration is important for:
1. Diagnosis purposes. The absence of blood in the stomach in a patient with
melena raises the suspicion of a postbulbar ulcer or other sources located
downstream the duodenum.
67
2. Monitoring the amount of stomach content and thus the flow of bleeding and
haemostatic treatment efficiency.
3. Stomach emptying to facilitate endoscopic examination.
4. Therapeutic reasons. Gastric emptying is the primary condition for
successful hemostasis. Gastric lavage is a maneuver that requires a lot of
patience from the therapist because it may take several tens of minutes
depending on the degree of filling of the stomach with blood clots.
Maneuver continues until the lavage fluid clarifies.
Contraindications if gastric lavage:
1. Benign or malignant esophageal stenosis
2. Patients with recent ingestion of corrosive agents - risk of perforation
3. Patients with craniofacial trauma with fracture of the cribriform plate - risk
of intracranial penetration of the tube
4. Comatose patients - gastric intubation is indicated only after airway
prosthesis to prevent aspiration
5. Patients with stroke because the effort induced by sensation of vomiting may
increase brain damage
6. Patient refusal
Endoscopic hemostasis is the most effective method of definitive nonsurgical
hemostasis. It can be performed in various ways: by thermo-coagulation, Argon-
coagulation, laser-coagulation, sclerosing substances (Polidocanol) or using hemoclips.
The use of adsorptive powder (Hemo-spray) is a promising treatment. The rate of
definitive hemostasis following thermo-coagulation or clips is almost identical (81.5%
vs. 81.2%). A 2 mm diameter artery is considered as the limit to endoscopic therapy.[20]
Embolization is another possibility to obtain hemostasis. Terminal vascular
branches are easier to embolize than gastro-duodenal artery.
Surgical treatment
Indications:
A. Absolute
1. Failure of conservative therapy (5- 25%) [15,21]
2. Hemodynamic instability despite sustained resuscitation (more than three
units of blood transfused)
3. Bleeding relapse after initial stabilization (up to two attempts at
endoscopic hemostasis)
4. Shock associated with recurrent bleeding
5. Small persistent bleeding, requiring more than three units of blood per
day
6. Association to bleeding of another complication
7. Patient with evident cardiac or cerebral vascular affection
8. Patient older than 60 years
B. Relative
1. A rare blood group or difficult typing
2. Refusal of transfusion
3. An associated gastrinoma
4. Future need for major surgery (heart transplant, kidney)
5. Patients who can not meet an adequate lifestyle and disease treatment

68
Surgical treatment includes the following possibilities:
1. Hemostasis by vascular suture (pylorotomy, suture the bleeding vessel
from a posterior penetrating duodenal ulcer, followed by vagotomy and
pyloroplasty). (Figure 28) Leaving in place the ulcer is encumbered by
the risk of recurrent bleeding.
2. Antrectomy associated to truncal vagotomy followed by Bilroth I or II
reconstruction.
3. Truncal vagotomy associated with pylorotomy-pyloroplasty.
4. Inferior gastric resection followed by Bilroth I or II reconstruction.
Postoperative mortality in bleeding ulcers is about 10%.

B. Perforation
Despite all progresses in medical treatment of peptic ulcer, perforation remains
one of the most important complications burdened by a high (8-42%) mortality risk.[22-
24]
The first cases of perforation were reported in 1817 by Traves, and in 1884,
Mikulicz first described treatment methods.[25] In the past, the incidence of perforation
was about 10% but it decreased after introduction of modern antacid drugs.
Helicobacter pylori is involved in 70-92% of all perforated duodenal ulcers. The
second most common cause (40-50%) of perforated duodenal ulcer is the ingestion of
anti-inflammatory non-steroidal (AINS) drugs. The least common cause is pathologic
hypersecretory status, such as Zollinger-Ellison syndrome.[25]
In half of cases, the perforation takes place in the free peritoneal cavity, when the
ulcer is located on the anterior duodenal wall. In about 40-50% of cases the perforation
is sealed (covered) by neighboring organs: the liver, gall bladder or omentum, as a
defense reaction of the body that tries to isolate the pathologic process.[25]
Clinical picture is dominated by symptoms and signs of generalized peritonitis. First,
there is a chemical and then bacterial peritonitis develops. The onset is sudden with
intense epigastric pain (as a knife stabbing") that can migrate into the right iliac fossa
and then quickly generalized. Initially epigastric pain and then located in the right iliac
fossa, lead to confusion with acute appendicitis. The pain may radiate to shoulder (Kehr
sign) because of the diaphragmatic peritoneum irritation, transmitted along the phrenic
nerve.
General signs may be fever, tachycardia, pallor, superficial tachypnea and signs
of acute dehydration. After several hours symptoms fade away, as hypovolemic shock
sets up. Abdominal wall contraction is replaced by abdominal distension, nausea and
69
vomiting are frequent, bowel movements are absent and hypotension and tachycardia
are increasing gradually.
Without any treatment, in most cases the patient will die.
Signs
The patient is immobilized by pain in fetal position (mobilization worsens the
pain), with peritonitic face in later stages (pallor, cold sweat, sunken eyes).
Abdominal muscle contraction is characteristic ("board-like rigidity " with visible
muscles reliefs) and the abdomen does not participate to the respiratory movements.
Mandel sign is positive (pain produced by abdominal wall percussion) and on
auscultation, bowel sounds are abolished as ileus is installed. Blumberg sign is positive
and liver dullness may miss (due to pneumoperitoneum). Douglas sac is very painful at
digital rectal examination.
Investigations
The pathognomonic sign on plain abdominal
radiograph is the pneumoperitoneum (intraperitoneal free
air) visible under the diaphragm in standing position
(Figure 29) or located periumbilical (Judins sign) in
supine, present in about 75-80% of cases.[25] In doubtful
cases a water-soluble (never barium) gastroduodenogram
will show the leak from the duodenum.
Ultrasound examination may reveal fluid in the
peritoneal cavity.
Acute pancreatitis
Appendicitis
Acute cholecystitis
Intestinal infarction
Myocardial infarction
Regarding the differential diagnosis, its importance is diminished by the need for
emergency surgery whatever the cause of peritonitis. However, the differential
diagnosis from a heart attack is very important when pneumoperitoneum is missing.
In rare cases, perforation can occur in closed peritoneal pouches (because of
inflammatory adhesions), the clinical picture being that of an intraperitoneal abscess. In
a limited number of cases, due to a good local reactivity, neighboring organs may cover
the perforation. Posterior duodenal ulcer perforation is represented by penetration into
the pancreas or liver pedicle. A penetrating ulcer changes its initial clinical picture so
that the pain becomes constant and refractory to treatment.
Treatment of perforated duodenal ulcer is surgical performed in emergency condition
with one exception: for recent perforated ulcer (less than 4-6 hours from onset) when
conservative treatment may be tried. Wangensteen first described this type of treatment
in 1935 and Taylor confirmed observations in 1946. A nasogastric tube is placed and a
continuous aspiration is performed. All biological parameters will be corrected and
broad-spectrum antibiotics will be administered. These measures, in most cases, will
lead to perforation sealing by neighborhood organs.[25-27]
In the first 6 hours after perforation, laparoscopic approach may be tempted but
after this interval, the classic approach is indicated.
70
Surgical therapeutic possibilities are:
1. Suture of the perforation with or without omental patch and a thorough
abdominal lavage. (Figure 19) For sealing (patching) the perforation, the
omentum can be used (technique falsely attributed to Graham, as originally
described by Cellan-Jones in 1929) [28] as well the falciform ligament. In
nowadays suture of perforation, either by open or laparoscopic approach,
followed by treatment for eradication of H. pylori, is considered the best
solution.[29-34] If the ulcer persists after this treatment, the following
surgical methods may be applied:
2. Vagotomy associated with pyloroplasty or pylorectomy-pyloroplasty.
3. Antrectomy associated with vagotomy and gastro-duodenal anastomosis
(Pean-Bilroth I).
4. Gastric resection with Bilroth I, II or Roux type of reconstruction.
Risk factors affecting prognosis are: delayed treatment (> 24 hours), preoperative
shock (BP < 100 mmHg), and concurrent serious medical illness. When all three
conditions are met, the mortality rate is close to 100%.
Generalized peritonitis will be treated by copious lavage and drainage of the
peritoneal cavity. Broad-spectrum antibiotics will be administered and the eradication
treatment for H. pylori will be started.
C. Stenosis (Narrowing and obstruction)
The incidence of stenosis affecting gastric emptying is about 5-10% of untreated
patients.
Location of stenosis may be the pylorus, duodenal bulb or the postbulbar region.
There are two forms of stenosis actually representing evolutionary stages:
1. Functional stenosis. There is a pyloric spasm accompanied by edema and
inflammation. This may be a reversible stage under medical treatment.
2. Organic stenosis. The ulcer is transformed in fibrous scar tissue due to
hyperplastic processes. This stage is irreversible having a surgical indication
for treatment.
The stomach changes, in an effort to adapt its functions, to the new conditions. In
the first stage, the stomach increases its tone in an attempt to overcome the obstacle. As
the lesion develops, the stomach enters the stage of compensated stenosis with gastric
hypersecretion and increased movements. In the last phase of gastric asystole, the
stomach is more dilated and atonic reaching down into the pelvis.
Symptoms are determined by the evolutionary stage. Thus, the initial clinical stage will
be dominated by intermittent colicky epigastric pains, which ameliorate after vomiting.
In compensated stage, the pain diminishes but becomes permanent and is accompanied
by a feeling of fullness in the epigastrium. Vomiting is rarer but becomes abundant,
effortlessly and contains ingested food without bile content. Patients still complain of
constipation. Decompensated phase is characterized by the appearance of edemas
caused by severe cachexia and denutrition.
Clinical examination reveals epigastric bulging and sometimes gastric peristaltic
waves, may be observed through the abdominal wall (Waltons sign). On palpation,
gastric clapotement can be heard (gastric splashing produced by repeated dippings with
the fingers over the stomach, which may be heard usually with the unaided ear).
71
Laboratory findings highlight the dehydration and electrolyte imbalance, and in
advanced stages, creatinine and urea values are increased. Frequent vomiting containing
HCl with loss of Cl and K will induce an increase in plasma bicarbonates as a
compensatory phenomenon, leading to the metabolic hypokalemic and hypochloremic
alkalosis associated with hypocalcemia (Darrow syndrome).
Diagnosis is based on:
Endoscopy - the stenosis cannot
be passed by the endoscope and the
stomach contains a high quantity of
fluid and food.
Radiological examination with
contrast highlights significant distension
of the stomach, which takes the form of
a "sink, often plunging under the iliac
crests. The contrast substance "falls" in
gastric liquid of stasis as "snowflakes.
(Figure 30)
Differential diagnosis should include other conditions that can cause difficulties in
gastric emptying, as follows:
1. Antral stenosing cancer - sometimes the tumor is palpable and suggestive
radiographic appearance on barium swallow and endoscopy with biopsy
make the diagnosis.
2. Pancreatic tumor penetrating the duodenum - usually there is an associated
mechanical jaundice and bilious vomiting. Ultrasound and CT scan show the
tumor.
3. Gastric ptosis - although X-ray shows a big stomach, the discharge is present
especially after right lateral decubitus. Epigastric pain appears in orthostatic
position. Endoscopy does not find any stenosis.
4. Chronic gastric volvulus - there is a suggestive radiographic appearance at
barium swallow.
5. Gastroduodenal mucosal prolapse - the diagnosis relies on gastrography
with contrast substance and endoscopy.
Treatment
Conservative treatment, in the functional phase, consists in continuous gastric
aspiration, gastric lavage with hypertonic solutions, and administration of antacid and
atispastic drugs, anti-inflammatory (cortisone) drugs, fluid, electrolytic and protein
rebalancing.
More than 75% of patients require surgery, which is identical with the surgical
treatment of the duodenal ulcers. (Figure 31-32)
A good preoperative preparation is needed to correct imbalances. A nosogastric
tube will be inserted and gastric lavage will be performed for two or three days. This
maneuver will evacuate the undigested food and will increase the tonicity of the
stomach. Hypoproteinemia will be corrected by total parenteral nutrition in the first
days. Surgery should not be postponed too much because the overall condition of the
patient might get worsen.
72

D. Penetration
Ulcer penetration is actually a perforation in an area of the duodenum covered by
surrounding organs. Penetration occurs most frequently in ulcers located on the
posterior wall toward the pancreas. Penetration may also occur in hollow organs such as
colon, gallbladder and common bile duct and fistulas between duodenum and these
organs may appear.
If in his penetrating evolution, the ulcer meets blood vessels these are eroded with
the consequent bleeding. Gastro-duodenal artery is most often interested in this process.
Clinical picture is characterized by changes of pain character with appearance of
symptoms from penetrated organs, increased frequency of upper gastrointestinal
bleeding and ulcer resistance to proper treatment.
Diagnosis is based on radiologic and endoscopic explorations.
Treatment is only surgical. In most cases, the ulcerous crater cannot be removed being
represented by the penetrated organ itself. Duodenal resection is made under the crater
which remains outside the digestive tract (it is excluded). Restoration of digestive
continuity is achieved by Bilroth I, Bilroth II or Roux anastomosis.
References
1. Wang YR, Richter JE, Dempsey DT. - Trends and outcomes of hospitalizations for peptic ulcer disease in the United
States, 1993 to 2006. - Ann. Surg. 2010; 251(1):51-58.
2. Paptheodoridis GV, Sougioultzis S, Archimandritis AJ. - Effects of Helicobacter pylori and nonsteroidal anti-
inflammatory drugs on peptic ulcer disease: A systematic review. - Clin. Gastroenterol. Hepatol. 2006; 4:130-142.
3. Suerbaum S, Michetti P. - Helicobacter pylori infection. - N. Engl. J. Med. 2002; 347(15):1175-1186.
4. Feldman RA. - Epidemiologic observations and open questions about disease and infection caused by Helicobacter
pylori. - In: Achtman M, Suerbaum S, eds. Helicobacter pylori: molecular and cellular biology. - Wymondham, United
Kingdom: Horizon Scientific Press, 2001:29-51.
5. Schlemper RJ, van der Werf SD, Biemond I, Lamers CB. - Seroepidemiology of gastritis in Japanese and Dutch male
employees with and without ulcer disease. - Eur. J. Gastroenterol. Hepatol. 1996; 8:33-39.
6. Doll R, Drane H, Newell AC. - Secretion of blood group substances in duodenal, gastric and stomal ulcer, gastric
carcinoma, and diabetes mellitus - Gut. 1961; 2(4):352-359.
73
7. Edgren G, Hjalgrim H, Rostgaard K, Norda R, Wikman A, Melbye M, Nyrn O. - Risk of Gastric Cancer and Peptic
Ulcers in Relation to ABO Blood Type: A Cohort Study. - Am. J. Epidemiol. 2010;172(11):1280-1285.
8. Kill J, Andersen D. - X-Ray Examination and/or Endoscopy in the Diagnosis of Gastroduodenal Ulcer and Cancer. -
Scand. J. Gastroenterol. 1980; 15(1):39-43.
9. Dragstedt LR, Owens FM. - Supradiaphragmatic section of vagus nerves in the treatement of duodenal ulcer. - Proc. Soc.
Exp. Biol. Med. 1943; 53:152.
10. Ohmann C, Thon K, Hengels KJ, Imhof M. - Incidence and pattern of peptic ulcer bleeding in a defined geographical
area. DUSUK Study Group. - Scand. J. Gastroenterol. 1992; 27(7):571-581.
11. Lee YC, Liou JM, Wu MS, Wu CY, Lin JT. - Eradication of Helicobacter Pylori to Prevent Gastroduodenal Diseases:
Hitting more than One Bird with the Same Stone. - Therap. Adv. Gastroenterol. 2008; 1(2):111-120.
12. Van Leerdam ME - Epidemiology of acute upper gastrointestinal bleeding. - Best Pract. Res. Clin. Gastroenterol. 2008;
22:209-224.
13. Sadic J, Borgstrm A, Manjer J, Toth E, Lindell G. - Bleeding peptic ulcer - time trends in incidence, treatment and
mortality in Sweden. - Aliment Pharmacol. Ther. 2009; 15;30(4):392-398.
14. Del Piano M, Antonia Bianco M, Cipolletta L, Zambelli A, Chilovi F, Di Matteo G, Pagliarulo M, Ballar M, Rotondano
G. - The "Prometeo" Study: Online Collection of Clinical Data and Outcome of Italian Patients With Acute Nonvariceal
Upper Gastrointestinal Bleeding. - J. Clin. Gastroenterol. 2013; 47(4):e33-37.
15. Holster IL, Kuipers EJ. - Management of acute nonvariceal upper gastrointestinal bleeding: Current policies and future
perspectives. - World J. Gastroenterol. 2012; 18(11):1202-1207.
16. Laine LA. - Review Helicobacter pylori and complicated ulcer disease. - Am. J. Med. 1996; 100(5A):52S-57S;
discussion 57S-59S.
17. Keyvani L, Murthy S, Leeson S, Targownik LE. - Pre-endoscopic proton pump inhibitor therapy reduces recurrent
adverse gastrointestinal outcomes in patients with acute non-variceal upper gastrointestinal bleeding. - Aliment
Pharmacol. Ther. 2006; 24(8):1247-1255.
18. Barkun AN. - The role of intravenous proton pump inhibitors in the modern management of nonvariceal upper
gastrointestinal bleeding. - Drugs Today (Barc) 2003; 39 Suppl A:3-10.
19. Choi CW, Kang DH, Kim HW, Park SB, Park KT, Kim GH, Song GA, Cho M. - Somatostatin adjunctive therapy for
non-variceal upper gastrointestinal rebleeding after endoscopic therapy. - World J. Gastroenterol. 2011; 17(29):3441-
3447.
20. En-Ling Leung Ki1, James Y W - New Endoscopic Hemostasis Methods. - Clin. Endosc. 2012; 45(3):224-229.
21. Van Leerdam ME, Vreeburg EM, Rauws EA, Geraedts AA, Tijssen JG, Reitsma JB, Tytgat GN. - Acute upper GI
bleeding: did anything change? Time trend analysis of incidence and outcome of acute upper GI bleeding between
1993/1994 and 2000. - Am. J. Gastroenterol. 2003; 98(7):1494-1499.
22. Hart AR, Todd CJ. - Epidemiology of duodenal ulcer perforation: a study on hospital admissions in Norfolk, United
Kingdom - Digestive and Liver Disease, 2002; 34(5):322-327.
23. Kocer B, Surmeli S, Solak C, Unal B, et al. - Factors affecting mortality and morbidity in patients with peptic ulcer
perforation. - J. Gastroenterol. Hepatol. 2007; 22(4):565-570.
24. Noguiera C, Silva AS, Santos JN, Silva AG, Ferreira J, Matos E, Vilaa H. - Perforated Peptic Ulcer: Main Factors of
Morbidity and Mortality. - World J. Surg. 2003; 27(7):782-787.
25. Andrew G Hill - Management of perforated duodenal ulcer - Surgical Treatment: Evidence-Based and Problem-Oriented.
- Holzheimer RG, Mannick JA, editors. - Munich: Zuckschwerdt; 2001. http://www.ncbi.nlm.nih.gov/books/NBK6880/
26. Crofts TJ, Park KG, Steele RJ, Chung SS, Li AK. - A Randomized Trial of Nonoperative Treatment for Perforated Peptic
Ulcer. - N. Engl. J. Med. 1989; 320(13):970-973.
27. Berne TV, Donovan AJ. - Nonoperative Treatment of Perforated Duodenal Ulcer. - Arch. Surg. 1989; 124(7):830-832.
28. Cellan-Jones CJ. - A rapid method of treatment in perforated duodenal ulcer. - B.M.J. 1929; 36:1076-1077.
29. Graham RR. - The treatment of perforated duodenal ulcers. - Surg. Gynecol. Obstet. 1937; 64:235-238.
30. Lui FY, Davis KA. - Gastroduodenal perforation: maximal or minimal intervention. - Scand. J. Surg. 2010; 99(2):73-77.
31. Siu WT, Leong HT, Law BK, Chau CH, Li AC, Fung KH, Tai YP, et al.- Laparoscopic repair for perforated peptic ulcer:
a randomized controlled trial. - Ann. Surg. 2002; 235(3):313-319.
32. Lunevicius R, Morkevicius M. - Management strategies, early results, benefits, and risk factors of laparoscopic repair of
perforated peptic ulcer. - World J. Surg. 2005; 29(10):1299-1310.
33. Lunevicius R, Morkevicius M. - Systematic review comparing laparoscopic and open repair for perforated peptic ulcer. -
Br. J. Surg. 2005; 92(10):1195-1207.
34. Bhogal RH, Athwal R, Durkin D, Deakin M, Cheruvu CN. - Comparison between open and laparoscopic repair of
perforated peptic ulcer disease. - World J. Surg. 2008; 32(11):2371-2374.

74
75
3. Benign Tumors of the Stomach
Benign tumors of the stomach represent only 5-20% of all gastrointestinal tumors
but in recent decades, there is an increasingly incidence primarily due to their more
frequent diagnosis by modern endoscopic methods of investigation.[1,2] Tumors may
have multiple and uncertain etiology, being incriminated factors such as: genetic,
familial, chronic inflammation, megaloblastic anemia, smoking, and alcohol. Although
benign, some of them have a malignant potential so that early diagnosis and proper
treatment are essential in healing.
Tumors may be unique or multiple, pedunculated or sessile and can be located in
any area of the stomach, being rare in the fornix. Tumors can develop from any layer of
the gastric wall and usually remain confined to the tunica which has generated it.[3]
Almost 90% of duodenal tumors are benign. Duodenal tumors located in the D2
and D3 segments are malignant in approximately 50% and the D4 tumors are
represented by adenocarcinoma in 80-90% of cases.
Histopathological classification [2]
Stomach
A. Epithelial:
Hyperplastic Polyps
Adenomatous Polyps
B. Mesenchymal:
Leiomyoma
Leiomyoblastoma
Neurogenic
Vascular
Lipoma
C. Others:
Inflammatory Pseudotumors
Peutz-Jeghers Polyps
Cystic Tumors

Duodenum
A. Epithelial:
Adenoma (tubular, villous,
tubulovillous)
Brunners Gland Adenoma
B. Mesenchymal:
Leiomyoma
Leiomyoblastoma
Lipoma
Vascular
Fibroma
Neurogenic
C. Others:
Familial Adenomatous Polyposis
Gardners Syndrome
Peutz-Jeghers Syndrome
Duodenal Gangliocytic
Paraganglioma
Symptoms
Most patients will not have any symptoms for a long period. Symptoms are
produced by tumoral complication, being represented mostly by upper gastrointestinal
bleeding and gastric outlet obstruction. Nonspecific symptoms as abdominal pain,
nausea, and weight loss may be present. Periampullary tumors can manifest with
jaundice, cholangitis, and pancreatitis. On physical examination, the bulky tumors may
be palpable.
Diagnosis
Endoscopic examinations (gastroduodenoscopy and endoscopic ultrasound) are
the most important because they directly visualize the tumor and are able to perform
biopsies. In certain cases, endoscopic procedures (stenting) may be applied as an
intermediate or definitive treatment.
CT and MRI scans are important in assessing the degree of tumoral penetration
and spread outside the digestive wall.
The most common benign tumors in the stomach are polyps (75%) and
leiomyomas. In the duodenum, the most common benign lesion is adenoma followed by
leiomyomas and lipomas.
Polyps can be classified as hyperplastic adenomatous polyps, papillary adenoma,
inflammatory fibroid polyp, hyperplastic polyps and pseudopolyps. Macroscopically
there are multiple small tumors, developed in the lining. Polyps develop from cells
located at the neck portion of gastric glands and cells of the sheath epithelium.
Adenomas develop from glandular cells of the gastric mucosa and Brunner glands.
Histological examination of the polyp reveals the presence of a vascular axis covered by
mucosa with hyperchrome cells and elongated nuclei. Quite often, in some sections,
anarchic mitosis or malignant transformation can be found. Hyperplastic polyps are
usually small, multiple, spread evenly across the surface of the gastric mucosa. Gastric
polyps have an increased risk of malignancy (10% for adenomatous and 1-2% for
hyperplastic polyps).[4] Although, only polyps over 2 cm diameter were considered at
high risk of malignant transformation, biopsy examinations showed the presence of
neoplastic cells even in polyps of 5 mm diameter.[4]
Fibromas are composed of abundant connective fibers centered on vessels
especially in the posterior gastric wall.
Leiomyomas occur more frequently in men aged between 50 and 70, with
predilection in gastric corpus (40%) and antrum (25%). Approximately 10-20% of
leiomyomas of the small intestine are located in the duodenum. They are not
encapsulated and are difficult to distinguish from malignant tumors. Usually, they are
asymptomatic but may be associated with anemia due to bleeding caused by ulceration
of the mucosa. The risk to turn malignant is considered at 12%.[4]
Gastric lipomas are tumors with an incidence of approximately 1-3% of all
benign gastric tumors and are usually located in the antrum. Most tumors are located in
submucosa layer manifested by bleeding consecutive to mucosa ulceration.[4]
Myxomas, hemangiomas and lymphangiomas are rare.
Schwannomas, developed from nerve fibers of the stomach may reach
considerable size. They represent 4% of all benign gastric tumors and 5% of the bowel.
Histological appearance is characteristic of vortices formed by anastomosed fusiform
cells separated by a conjunctive stroma. Frequently, tumors have a pedicle, which
confers mobility so that they can be confused with other abdominal tumors. In about
40% of cases are complicated by bleeding and occlusion.[4]
Particular clinical forms are the gastric polyps in genetic syndromes: familial
adenomatous polyposis, Peutz-Touraine syndrome and Gardner syndrome. Other
particular forms are the periampullary duodenal tumors, usually villous or tubulovillous
adenomas with a high tendency to malignant transformation.
Treatment
Until recently, it was thought that polyp dimension is an important criterion for
their removal (larger than 2 cm) considering also the histopathological outcome of the
biopsy. Currently, size is not a criterion for indication of removal, just histopathological
outcome is important.
Therapeutic possibilities are multiple, ranging from simple endoscopic removal to
pancreatoduodenectomy. Most tumors can be removed by minimal invasive techniques
such as endoscopic resection, laparoscopy or combination of these two.
Benign gastric polyps are removed endoscopically in most cases. Gastric
resection is necessary only when polyps are numerous or there is a suspicion of
malignant transformation. Patients with polypectomy will be followed up annually for
solitary polyps or every six months if there were multiple polyps. Unique and small
duodenal polyps can be solved by endoscopic resection.
76
Leiomyomas, lipomas and Schwannomas usually require surgical resection within
safe limits, which can be performed by open surgery or by laparoscopic approach.
Periampullary tumors raise special problems of surgical tactic, the surgeon having
to choose between a laborious operation such as the cephalic pancreatoduodenectomy
(which appears to be exaggerated for a benign lesion and often encumbered by risks)
and a smaller one, which is papillectomy, but even this one is not free of risks.[5]
References
1. Angelescu N. - Tratat de patologie chirurgical - Ed. Med. Buc. 2001;Vol.I-II.
2. Goh PMY, Lenzi JE - Benign tumors of the duodenum and stomach - Surgical Treatment: Evidence-Based and Problem-
Oriented. Holzheimer RG, Mannick JA, editors. - Munich: Zuckschwerdt; 2001.
3. Halpin R, Thomson S, Catterall N, Haffejee A. - Smooth muscle tumors of the stomach: clinicopathological aspects. - J.
R. Coll. Surg. Edinb.1993; 38:23-27.
4. Orlowska J, Jarosz D, Pachlewski J, Butruk E. - Malignant transformation of benign epithelial gastric polyps. - Am. J.
Gastroenterol.1995; 90:2152-2159.
5. Schoenberg M, Treistschke F, Harada N, Beger H. - Benign tumor of the ampulla of Vater: surgical treatment and
prognosis. - Eur. J. Surg.1998; 164:765-770.

77
4. Gastric Cancer
The stomach is one of the most common sites of cancer in the digestive tract,
ranking second after colon and rectum. Gastric cancer mortality rates have remained
relatively unchanged over the past 30 years, and gastric cancer continues to be one of
the leading causes of cancer-related death.[1] The first gastric resection was performed
by Billroth in 1891, and the first resection for cancer belongs to Schlatter in 1897.
Despite the abundance of articles on gastric cancer, the only who have made
contributions in terms of improved postoperative outcomes were those related to early
diagnosis of disease (early cancer) by screening of Japanese researchers.
Epidemiology
Up to 2-3 decades ago, the stomach cancer ranks first in the hierarchy of digestive
cancers before colorectal cancer. As knowledge about etiopathogenesis and treatment
methods developed, gastric cancer incidence decreased constantly. However, stomach
cancer was the fourth most common malignancy in the world in 2008, with an estimated
989,600 new cases. Approximately 72% of new cases occurred in developing countries.
Stomach cancer incidence rates vary widely across countries, ranging from less than
1/100,000 inhabitants in areas such as Botswana to about 62/100,000 in Korea for men
and from less than 1/100,000 in Botswana to about 26/100,000 in Guatemala for
women. In general, the highest incidence rates are in Asia (particularly in Korea, Japan,
and China) and many parts of South America, and the lowest rates are in North America
and most parts of Africa with the exception of Mali and Western Sahara.[2]
Age most affected is 50-60 years. Gastric cancer occurs rarely under the age of 30
and is two to three times more common in men than in women. After the age of 40,
there is a linear increase of carcinogenic risk. In United States the mean age at time of
diagnosis is 72 years, the lifetime risk of gastric cancer is approximately 1% and that of
dying from gastric cancer is 0.6%.[3]
Gastric cancer mortality, although decreasing, remains high. It is the third leading
cause of cancer death in men and the fifth leading cause in women. The disease
prognosis remains reserved and the 5-years survival rate in Europe is about 25%. In
Japan, the five-year relative survival has increased with 20% (up to 50% survival)
between 1975 and 1999.[2]
Etiopathogenesis
Etiology of gastric cancer is obscure. It is recognized that on a genetic
predisposition basis, a number of predisposing factors may lead to the development of
cancer.
Predisposing factors that have a certain role in the genesis of gastric cancer are:
1. Helicobacter pylori infection
2. Certain gastric diseases
3. Diet
4. Socio-economic conditions
Infection with Helicobacter pylori seems to increase the risk of gastric cancer by
three to six times, in association with gastric mucosal atrophy. More than 50% of new
stomach cancer cases are attributed to H. pylori infection.[4-7] H. pylori infection is
linked to gastric lymphomas and regression of such tumors often follows its
eradication.[8-10]

78
Gastric diseases:
1. Atrophic gastritis appears to be the most important in antral cancer genesis
2. Gastric ulcer has a potential for malignancy, especially in locations of the
lesser curvature
3. Gastric adenomatous polyps, especially those larger then 2 cm in diameter,
but malignancy was also found in smaller polyps
4. Intestinal metaplasia, Menetrier disease, Zollinger-Ellison syndrome
5. Pernicious anemia associated with atrophic gastritis caused by an
autoimmune condition, increases two to three times the risk of gastric cancer
[11,12]
6. Previous gastric resection - Balfour was the first surgeon who in 1922 made
the correlation between gastric cancer and previous operations on the
stomach for benign diseases. Patients with distal gastric resection and/or
vagotomy (mostly for duodenal ulcer) in their history have a four to seven-
fold greater risk of developing gastric cancer on the remaining stump, caused
by the duodenal-gastric reflux.[13-16] The average time between resection
and cancer onset is 20-25 years.
7. Gastroesophageal reflux disease (GERD) is doubling in the risk of gastric
cardia adenocarcinoma.[17] The risk of cardia cancer remains high even after
antireflux surgery.[18] People diagnosed with Barrett's esophagus have an
eighteen-fold risk of cancer.[19]
In 8% to 10% of cases, gastric cancers have an inherited familial component [20]
(Li-Fraumeni syndrome, hereditary nonpolyposis colon cancer, familial adenomatous
polyposis and Peutz-Jeghers syndrome).
Diet is considered a major factor in gastric cancer. This does not necessarily refer
to some kind of food but to the way of food preservation and their nitrate content, which
the body turns into nitrites and then into nitrosamines. Smoked conserved foods have a
carcinogenic role compared to those preserved by cold. Smoked foods, salted meat or
fish and pickled vegetables are mostly incriminated. High fruit and vegetable intake is
associated with a reduced risk of gastric cancer. Mediterranean-style of diet reduces the
risk of gastric cancer.[21]
Smokers have a 50% to 60% increased risk for stomach cancer compared to
nonsmokers.[22-24]
Morphopathology
Location of gastric cancer is compared to the long axis of the stomach.
Approximately 40% of cases develop in the lower (antral) part of the stomach, 40% in
the middle (corpus), 15% in the upper part (cardia/fundus) and almost 10% comprise
more than one region.
Macroscopic aspect (Borrmanns classification 1926):
Type I: polypoid
Type II: fungoid
Type III: ulcerative
Type IV: infiltrative
Unclassifiable
A very common classification is that of Marson and Dawson:
Vegetative with variable extent, located mostly in the fundus and body of
stomach
79
80
Ulcerative of different sizes, with predominant antral location
Infiltrative with the disappearance of mucosal folds, which can occur in
any location. Linitis plastica is a special aggressive form of cancer,
which gradually infiltrates the entire stomach.
A widely accepted classification is that proposed by Lauren in 1965 who
described a simplified system: [25]
1. Diffuse gastric cancer (70% of cases),
2. Intestinal gastric cancer (30% of cases), and
3. Others
Diffuse type is found more frequently in young female patients. It is associated
with blood type A. Tumoral cells are poorly differentiated, with signet-ring cells.
Transmural and lymphatic spread are more common. Metastases occur early and there is
a worse overall prognosis. In contrast to the intestinal-type tumors, gastritis is
uncommon.[26]
Intestinal type has a progression of carcinogenesis similar to colon cancer. Initial
environmental exposures, such as infection with H. pylori, result in a chronic superficial
gastritis. There is then a progression from atrophic gastritis to intestinal metaplasia,
dysplasia, and finally cancer. Intestinal-type tumors are more common in elderly
males.[3]
Morphological and clinical classification of gastric cancer:
1. "in situ" - limited to the epithelium, without exceeding the basal
membrane
2. "early" (early gastric carcinoma) - limited to the mucosa and submucosa,
regardless of size and presence or absence of metastasis. Superficial or
early cancer is an important prognostic factor.
3. "invasive" - extended beyond the submucosa
Endoscopic appearance is classified as:
1. Type I - exophitic, corresponds to a malignant polyp
2. Type II - superficial, can be raised, flat and bumpy
3. Type III - ulcers
Each of these types may be microscopically:
1. well differentiated
2. medium or less differentiated
3. undifferentiated (mucus-secreting)
Histopathological classification (WHO)
A. Epithelial tumors
1. intraepithelial neoplasia -adenoma
2. carcinoma
o Adenocarcinoma (95%)
intestinal type
diffuse type (plastic linitis)
o papillary adenocarcinoma
o tubular adenocarcinoma
o mucinous adenocarcinoma
o carcinoma with signet ring cells
o adenosquamuos carcinoma
o squamous cell carcinoma
o small cell carcinoma
o undifferentiated carcinoma
o Others
3. carcinoid (well differentiated
neuroendocrine tumor)
B. Non-epithelial tumors
o Leiomyoma
o Schwannomas
o granular cell tumor
o Glomic tumor
o Leiomyosarcoma
o gastrointestinal stromal tumors (GIST)
benign
uncertain malignant, potential
malignant
o Kaposi's sarcoma
o Other
C. Malignant lymphomas
o cell lymphoma of MALT type marginal zone
o mantle cell lymphoma
o large cell lymphoma Diffuse B
D. Others

Spread of gastric cancer
1. By contiguity, successively in all gastric wall layers, and neighboring organs:
liver, pancreas, colon, kidney, etc.
2. Through the lymphatic vessels towards the lymph nodes. There are four areas
of lymphatic drainage of the stomach, described in the chapter about anatomy.
3. Through the blood vessels to the liver, lungs, adrenal glands, ovary, thyroid,
etc., where metastases occur.
4. Intraperitoneal, after penetrating the serosa layer, inducing ovarian tumors
(Kruckenberg) or peritoneal carcinomatosis and ascites.

Linitis plastica, known as Brinton's disease, represents a morphological variant of
diffuse cancer invading all the layers of the stomach. It is not associated with H. pylori
infection or chronic gastritis. It is characterized by a thick, rigid stomach wall caused by
diffuse infiltration of poorly differentiated tumoral cells and extensive fibrosis.[27,28]
Staging of gastric cancer [29]
Key features and changes to the recommendations to the TNM gastric cancer staging
system for the 7th edition includes the following:
Tumors arising at the esophagogastric junction, or arising in the stomach 5 cm or less
from the esophagogastric junction and crossing the esophagogastric junction, are
staged using the TNM system for esophageal carcinoma. The revised gastric cancer
staging system applies to tumors arising in the more distal stomach and to tumors
arising in the proximal 5 cm but not crossing the esophagogastric junction.
T categories have been harmonized with T categories of the esophagus and small and
large intestine, with T2 defined as a tumor that invades the muscularis propria, and T3
defined as a tumor that invades the subserosal connective tissue. T4 is now defined as a
tumor that invades the serosal (visceral peritoneum) or adjacent structures.

T-primary tumor (Figure 33)
TX Primary tumor cannot be assessed
T0 No evidence of primary tumor
Tis Carcinoma in situ: intraepithelial tumor without invasion of the lamina propria
Tl Tumor invades lamina propria, muscularis mucosae, or submucosa
o Tla Tumor invades lamina propria or muscularis mucosae
o T1b Tumor invades submucosa
T2 Tumor invades muscularis propria
T3 Tumor penetrates subserosal connective tissue without invasion of visceral
peritoneum or adjacent structures. T3 tumors also include those extending into the
gastrocolic or gastrohepatic ligaments, or into the greater or lesser omentum, without
perforation of the visceral peritoneum covering these structures
T4 Tumor invades serosa (visceral peritoneum) or adjacent structures
o T4a Tumor invades serosa (visceral peritoneum)
o T4b Tumor invades adjacent structures such as spleen, transverse colon, liver,
diaphragm, pancreas, abdominal wall, adrenal gland, kidney, small intestine, and
retroperitoneum
N, lymph nodes
NX Regional lymph node(s) cannot be assessed
NO No regional lymph node metastsis
81
Nl Metastasis in 1 to 2 regional lymph
nodes Stages based on these parameters:
Stage 0 Tis N0 M0
Stage IA Tl N0 M0
Stage IB T2 N0 M0
Tl Nl M0
Stage IIA T3 N0 M0
T2 Nl M0
Tl N2 M0
Stage IIB T4a N0 M0
T3 Nl M0
T2 N2 M0
Tl N3 M0
Stage IDA T4a Nl M0
T3 N2 M0
T2 N3 M0
Stage IIIB T4b N0 or NI M0
T4a N2 MU
T3 N3 M0
Stage mC T4b N2 or N3 M0
T4a N3 M0
Stage IV Any T Any N Ml

N2 Metastasis in 3 to 6 regional lymph
nodes
N3 Metastasis in 7 or more regional
lymph nodes
M-Metastases
MX-metastases can not be assessed
M0- no distant metastases
M1-with distant metastases
In United States, approximately 24% of
diagnosed stomach cancers are localized, but
32% are spread to lymph nodes and 32% are
associated with metastases.[3]
Histopathological grading:
GX - degree of differentiation can not
be assessed
G1 - well-differentiated tumors
G2 - moderately differentiated tumors
G3 - poorly differentiated tumors
G4 - undifferentiated tumors

Symptoms
Symptoms of gastric cancer are poor and nonspecific in early stages, leading to
delayed diagnosis. Symptoms are dependent on age, location, extent and type of tumor.
Symptoms are more intense when tumors are located at stomach openings: pylorus and
cardia.
The development of gastric cancer is divided into three clinical stages: the onset,
the status period and the terminal stage. The onset is insidious characterized by vague
dyspeptic disorders, nausea, anorexia, stomach fullness, weight loss. Pain is continuous
or intermittent, occurring early postprandial. Vomiting or food ingestion does not calm
the pain as in duodenal ulcer. On contrary, food intake may exacerbate the pain (as in
gastric ulcer). Anorexia is a consistent symptom, progressive, often selective, first to
meat and then to fat. Vomiting occurs when the tumor is located at gastric openings. In
pyloric location vomiting appears late after food intake, whereas in case of cardial
location dysphagia is the main symptom and vomiting is manifested as regurgitation. In
evolution, vomiting becomes more frequent, and may contain dark digested blood in
small quantities. Upper gastro-intestinal bleeding is rarely massive and less important
than in peptic ulcer.
82
Patients with linitis plastica will complain of early satiety due to loss of stomach
distensibility.
Almost 10% of patients have only signs of neoplastic impregnation (Savitki) -
loss of appetite, pallor, fatigue, asthenia, weight loss, decreased work capacity.
Less than 10% of patients will experience a complication of cancer: cardia or
pylorus stenosis, upper gastrointestinal bleeding or perforation with peritonitis.
Physical examination. In first stages of the disease, physical signs are not conclusive.
Classical findings such as Virchows supraclavicular node, Sister Mary Josephs
periumbilical node, Blumers shelf, Krukenbergs tumor, hepatomegaly, jaundice,
ascites and cachexia are present only in very advanced incurable stages.
Clinical signs of inoperability of gastric cancer (even if palliative operations are
possible) are:
1. Epigastric palpable tumor
2. Positive Virchows lymph node confirmed by biopsy (metastasis in the
left supraclavicular lymph node)
3. Carcinomatous ascites (shifting dullness on percussion), confirmed by
cytological examination of fluid extracted by puncture
4. Palpable tumors in Douglas sac, by rectal or vaginal examination
(Blumers sign) or Krukenbergs ovarian tumors
5. Metastatic skin tumors in the abdominal wall or around the umbilicus
(Sister Mary Joseph's lymph node)
6. Hepatomegaly due to metastases (confirmed by ultrasound) often
associated with jaundice and cachexia
Particular anatomo-clinical forms
Antropyloric cancer is the most common location; it rapidly invades the
pylorus producing gastric outlet obstruction.
Tumors with proximal location (cardial) clinically manifest as an
esophageal syndrome.
Tumors located at corpus, fundus and curvatures have a long clinical
latency period, so the diagnosis is often delayed and tumors can reach
significant size being palpable.
Linitis plastica reduces the gastric capacity, giving the patient the feeling
of early satiety and vomiting.
Workup
Gastroscopy is the most important examination for diagnosis because it visualizes
the lesion and also can perform multiple biopsies.
X-ray barium swallow may offer extra information.
Pathognomonic signs are the lack of filling with barium
cancerous niche and the rigidity of the gastric wall. (Figure
34) In plastic linitis, the stomach is transformed in a rigid
tube with complete disappearance of peristaltic movements.
In Japan, radiologic investigation is used as a screening
method. Special techniques permit the diagnosis of different
morphological types of early cancer described by the Japan
Research Society of Gastric Cancer such as small polyps
(type I), superficial lesions that are minimally elevated (type
IIa), or flat (type IIb), slight depressions (type IIc), or shallow
83
ulcers (type III).[30]
Other additional useful investigations are:
Abdominal ultrasound, useful especially for liver metastases and ascites
Endoscopic ultrasound to assess the degree of wall penetration and perigastric
lymph node involvement
CT and MRI scan to evaluate the relations with other surrounding organs and
metastases
Endoscopic ultrasound and CT or MRI examinations are the current primary
staging modalities for gastric cancer.
PET (Positron Emission Tomography) scan for detecting recurrence,
determining prognosis, and measuring therapy response
Laparoscopy is also considered a method of diagnosis. In 12% to 52% of
patients felt to be appropriate for gastric resection, laparoscopy can discover
metastatic lesions thus avoiding unnecessary laparotomies.[31,32]
Laboratory:
Anemia
Hypoproteinemia
High erythrocyte sedimentation rate (ESR)
Gastric hypo-anacidity
High levels of CEA (carcinoembryonic antigen), CA 19-9, and others.
However, only one-third of all patients have abnormal CEA and/or CA19-9
levels.[33]
Complications
Possible complications of gastric cancer are occult or gross bleeding, pyloric
stenosis, perforation, tumor necrosis, phlebitis and metastases.
Gastric cancer perforation at clinical presentation is rare but still possible (2-4%
of cases) affecting mostly patients in advanced stages of cancer evolution (T3 and T4).
Perforation can occur in early gastric cancers also, emphasizing the importance of
biopsy and frozen section analysis during emergency operations for perforated gastric
ulcers.[3]
Prognosis
While remarkable progress in terms of diagnosis and treatment of gastric cancer
were made, improving outcome depends only on early detection of the disease. In
Japan, double-contrast barium, as a screening tool, was introduced since the 1960s and
since 1983, a mass screening program was implemented. The consequence was a
dramatic decrease in gastric mortality.[3] The Japanese 5-year survival rate is 40% to
60%, compared with only 20% in other developed countries. In early stages of "early
cancer, survival rates are over 90% at 5 years.[34] Even though, it is considered that
mass screening programs should be applied only to countries with high incidence of
gastric cancer.[3]
Treatment
Treatment of gastric cancer is complex, multimodal: surgical and oncological, but
surgery remains the most important.

84
Surgical treatment
Surgery for gastric cancer can be radical or palliative. Operations may be
performed in emergency, for cancer complications, or in elective conditions, when the
operation is scheduled.
Contraindications for radical surgery are related to the presence of peritoneal
carcinomatosis or distant metastases including liver, although if they are present, limited
excision can be performed.
Before operation, a good evaluation and preparation of the patient are necessary.
In certain cases colon preparation is also indicated, because advanced gastric tumors
located on corpus and grater curvature may penetrate the transverse colon or mesocolon,
and thus, associated colon resection may become needed.
Operations can be performed by classic (open) approach (laparotomy) or by
laparoscopic approach.
There are multiple types of possible laparotomies. (Figure 35) Choosing one of
them depends on tumor location and surgeons preference, but in most cases, xipho-
subumbilical laparotomy is used.

Radical surgery performs a complete removal of the tumor by partial or total
gastrectomy, or even extended if necessary to neighborhood viscera. In addition,
omentectomy and regional lymphadenectomy are necessary. (Fig 36)
Gastric resection must respect a distance of 6 cm from the intraluminal macroscopic
visible margin of the tumor.[35]
In patients with more than five positive
lymph nodes, there was no relationship between
margin status and survival.[3]
Depending on tumor location, there are
more possibilities for gastric resection: (Figure
37)
1. Inferior gastric resection in most cases
represented by a subtotal gastrectomy is
applied for tumors located on antrum.
2. Superior gastric resection is rarely
applied. It may be performed when the
tumor is located under the cardia. The
consecutive esogastrostyomy is burdened
by a high incidence of gastroesophageal
reflux, which is very difficult to treat.
85
Pylorotomy must be associated to
this procedure because both vagus
nerves are cut. Most surgeons
prefere instead, a total gastrectomy
for this location of tumor. Special
problems of surgical tactic raises
the tumor extended to cardia. In
this case the surgical solution may
be represented by a partial
esogastrectomy with intrathoracic
esogastrostomy, which implies abdominal and thoracic approaches.
3. Total gastrectomy is applied in many cases when the tumor is located above the
gastric angle on the lesser curvature or on the gastric corpus.
Gastric resections may be associated with resection or removal of other organs
such as splenectomy and partial pancreatectomy, when the tumor penetrates the
pancreas, or resection of the transverse colon when the tumor penetrates the colon or the
transverse mesocolon.
Regional lymphadenectomy. The Japanese classification of regional
lymphadenectomy is based on 16 principal lymph node stations, grouped into three
levels according to tumor location. (Table 1) A good lymphadenectomy requires
removal of N1 and N2 stations. (Figure 38)
Table 1 - gastric lymph node stations [36]
Location of primary tumor Station Lymph nodes location
Superior third Middle Inferior third
1 Right paracardial N1 N1 N2
2 Left paracardial N1 N3 M
3 Greater curvature N1 N1 N1
4sa Greater curvature - short gastric vessels N1 N3 M
4sb Greater curvature - left gastroepiploic N1 N1 N3
4d Greater curvature - right gastroepiploic N2 N1 N1
5 Suprapyloric N3 N1 N1
6 Subpyloric N3 N1 N1
7 Left gastric artery N2 N2 N2
8a Anterior to hepatic artery N2 N2 N2
8p Posterior to hepatic artery N3 N3 N3
9 Celiac trunk N2 N2 N2
10 Spleen hilum N2 N3 M
11p Splenic artery proximal to spleen N2 N2 N2
11d Splenic artery distal to spleen N2 N3 M
12a Hepatoduodenal lig. left N3 N2 N2
12b,p Hepatoduodenal lig. posterior N3 N3 N3
13 Retropancreatic M N3 N3
14v Superior mesenteric vein M N3 N2
14a Superior mesenteric artery M M M
15 Middle colic artery M M M
16al Aortic hiatus M M M
16a2,b1 Middle paraaortic N3 N3 N3
16b2 Caudal paraaortic M M M

86
Regional lymphadenectomy is classified as:
1. D1 - Removal of a minimum 15 lymph nodes located at less than 3 cm from
the tumor en bloc with the greater omentum and stomach;
2. D2 - Removal of at least
25 nodules, located at
more than 3 cm from
tumor boundaries
including also the omental
bursa and lymph nodes of
the celiac trunk,
hepatoduodenal, splenic
and retroduodenal nodes.
3. D3 - Removal of at least
25 nodules.
The benefits of very extended
(D2, D3) lymphadenectomy
resections are unproven considering
the postoperative morbidity and
survival rates, but the modern splenic
preservation D2 resection is an
acceptable procedure.[3,37]
Restoration of digestive
continuity is achieved by gastro-
jejunal anastomosis in case of inferior
gastric resection, or esojejunostomy
for total gastrectomy, or
esogastrostyomy for the superior
gastric resection. (Figure 39)
Treatment of linitis plastica, in
the absence of peritoneal
carcinomatosis, is represented by
total gastrectomy and adjuvant
therapy, but prognosis is very poor.
Palliative surgery confers a
small survival benefit, but is very
important in terms of quality of life
and prevention of complications. It is
indicated in patients who do not meet
the criteria for radical surgery. Types
of palliative surgery are:
1. Palliative gastric resection
2. Internal derivations
(gastroenteroanastomosis,
biliodigestive anastomosis)
3. Feeding stomas
(gastrostomy, jejunostomy),
or diversion stomas (colostomy).
87
Endoscopic resection of gastric mucosa affected by neoplastic process is
technically feasible, but it is indicated only in the early stages of gastric cancer when the
tumor does not exceed the mucosa and there are not associated lymph node metastases.
Postoperative complications
Early postoperative complications
Possible general complications are the same as in any operated patient and are
represented more frequently by thrombophlebitis, pulmonary embolism, pneumonia,
myocardial infarction, cardiac decompensation, psychosis, etc. Preventing them is
possible in a high percentage of patients by a thorough preoperative investigation and
treatment of comobirdities, by early postoperative ambulation, administration of
anticoagulant drugs, antibiotics and other very important nursing measures.
Local complications occur at a rate of about 25% of operated patients, the most
dangerous being the anastomotic leaks (5-10%), especially of the esojejunal
anastomosis.[38,39] It seems that hand sewn anastomosis is at higher risk of leakage than
mechanical anastomosis.[40,41] Once diagnosed, the anastomotic leakage (associated
with general peritonitis and possible mediastinitis) presumes, in most cases,
reintervention to repair the anastomosis or to establish a new anastomosis but,
unfortunately, there is a high postoperative mortality rate (10-40%) in these cases.[42-44]
In recent years, new endoscopic stenting methods have been developed with a better
outcome. A covered stent is introduced endoscopically at anastomosis site producing by
self expanding the occlusion of the leak and this might be an alternative therapeutic
option for the treatment of intractable postoperative anastomotic leak after radical
gastrectomy.[42,45]
Duodenal stump fistula is also a possible complication but it appears in rare cases.
It is manifested by bilious discharge on the subhepatic drainage tubes. If there are no
signs of peritoneal irritation, general condition is not altered, and there is no evidence of
subhepatic collection on ultrasound examination, reintervention is not mandatory and
the drainage tube will function as an external directed duodenal fistula, with the
opportunity of spontaneously healing.
Another possible complication after total gastrectomy with distal
splenopancreatectomy is pancreatitis and pancreatic fistula manifested by right upper
quadrant pain, hiccups respiratory disorders, fever, sepsis, and gray purulent discharge
on drainage tubes. In addition to appropriate medical treatment of acute pancreatitis,
pancreatic fistula is more likely to close spontaneously after a few weeks especially if
drainage tubes are in the right place. Otherwise, reintervention is necessary to evacuate
the abscess and to reposition the drains near the pancreatic stump.
Other local complications such as wound infections, hematoma, evisceration,
incisional hernia and so on, have nothing special from other types of operation.
Late postoperative complications
Stenosis of the anastomosis may appear but it can be managed either by
endoscopic balloon dilatation or by stenting.
Side effects after subtotal or total gastrectomy that affect quality of life can be
weight loss, dumping syndrome, anemia, iron deficiency and osteomalacia (the
softening of the bones caused by defective bone mineralization). After resection of the
superior part of the stomach, the intrinsic factor secretion is eliminated and vitamin B12
deficiency with pernicious anemia thus follows in 2 to 7 years.

88
Measures for prevention of gastric cancer
1. Eat mostly foods with protective role: fresh vegetables, fruits, milk, corn, etc.
2. Use correct cooking techniques, food preservation and storage.
3. Avoid common fat fried or reheated, smoked, canned, and pickled vegetables.
4. Avoid smoking and passive inhalation of cigarette smoke.
5. Avoid excessive consumption of alcoholic beverages, especially concentrated
alcohol.
6. Periodically health checks by medical specialty examinations (radiology,
endoscopy and biopsy) especially in people at high risk (gastric ulcer and
stomach surgery in history).
Gastric Stump Cancer
Gastric stump cancer is defined as a gastric carcinoma occurring after gastric
surgery for benign diseases. It should not to be confused with the relapse of gastric
carcinoma after partial gastrectomy for gastric cancer.
The pancreaticobiliary reflux into the gastric stump, produced by operations for
peptic ulcer, was incriminated for this type of cancer [46,47], but many studies now
suggest that the relationship between surgery for peptic ulcer and cancer is probably due
to the presence of H. pylori in the stomach of the operated patients.[3] The relative risk
in patients who underwent such operations is approximately three-fold higher after 30
years from initial operation.
The main problems in this group of patients are the resectability rate and survival
rate which, in many cases are very low (40% respectively 10%) except in Japanese
series where authors reported a resectability rate of 90% and a 5 year-survival rate of
40%.[3]
Early Gastric Cancer
Early gastric cancer (EGC) was fist described in 1962 by the Japanese Endoscopy
Society and it represents a stage of the gastric cancer, which is limited to the mucosa
and submucosa layers, independent of lymph node metastasis. Because in Japan there is
a well-implemented screening program for gastric cancer, this type of cancer represents
40%-50% out of all cases of gastric cancer at time of presentation. In western countries,
this percentage is only about 15-18%.[3] In most cases the cancer is associated with
peptic ulcer disease and is located in the antrum on the lesser curvature of the stomach.
Lymph nodes are found positive in 10-29% of cases. The 5-year survival rate is about
85-90%.
Early gastric cancer can be managed with minimally invasive techniques.
Endoscopic mucosal resection is used widely in Japan. Contraindications for endoscopic
resection are large, poorly differentiated tumors, which invade the submucosa.
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44. Markku VI, Reijo H, Pekka L, Veikko R, Sauli JV. - Total and proximal gastrectomy in the treatment of gastric
carcinoma: A series of 305 cases. - World J. Surg. 1981; 5(2):249-255.
45. Fernndez A, Vila JJ, Vzquez S, Gonzlez-Portela C, de la Iglesia M, Lozano M, Toscano E. - Self-expanding plastic
stents for the treatment of post-operative esophago-jejuno anastomosis leak. A case series study. - Rev. Esp. Enferm.
Dig. 2010; 102(12):704-710.
46. Northfield TC, Hall CN. - Carcinoma of the gastric stump: risks and pathogenesis. - Gut 1990; 31:1217-1219.
47. Lundegardh G, Adami HO, Helmick C, Zack M. - Risk of cancer following partial gastrectomy for benign ulcer disease.
- Br. J. Surg. 1994; 81:1164-1167.

91
5. Gastric Sarcomas
Sarcomas are malignant tumors of mesenchymal origin representing about 1-3%
of malignant tumors of the stomach, with an increased malignancy. The most frequent
way of spreading is via the blood vessels. They can be localized or diffuse, intra- or
extra-gastric, pedicled or sessile, fast growing and with high tendency to bleeding and
perforation. Gastric tissue sarcomas are classified by provenance in:
1. lymphomas
2. leiomyosarcomas
3. fibrosarcomas
4. neurosarcomas
5. angiosarcomas
Leiomyosarcomas represent 25% of all gastric sarcomas developing from
muscular gastric layers. Their location may be inside or outside the stomach and may
have a pedicle. During evolution, they ulcerate and bleed. Histologically consist of
elongated muscle cells with abundant cytoplasm with large nuclei. Symptoms depend
on location of tumor, its size and the presence of ulceration. Often is diagnosed during a
bleeding episode. Diagnosis is difficult because there are no pathognomonic signs. Even
endoscopic biopsy may be negative due to submucosal location of the tumor.
Fibrosarcomas develop from connective tissue within submucosa layer, where
otherwise they remain confined. They can be pedicled or sessile and large forms
become ulcerated. Histologically they are composed of elongated cells arranged in
plaques or of spherical and polygonal cells. The only effective treatment is surgical.
Malignant Schwannomas are rare, 90% of them being benign. They are
developed from nerve tissue and can take two forms of evolution: a very malignant one,
rapidly evolving and the other one with reduced malignancy that can get healing even
after simple excision.
Angiosarcomas have the starting point the blood vessels. Those typical are rarely
located in the stomach, and those atypical are represented by Kaposi's sarcoma and
hemangiopericytoma.
o Kaposi's sarcoma is characterized by the presence of multiple visceral lesions,
rarely gastric, preceded or followed by characteristic skin lesions. The
incidence is higher following renal transplantation and immunosuppression,
and in AIDS patients. When the lesion is present on the stomach, it appears as a
round ulcerated growth without affecting gastric peristalsis and without
stiffening the gastric wall. Frequently are hemorrhagic. Of more than 450
reported cases, only eight instances have been documented until 2002.[1] The
therapeutic approach to gastrointestinal bleeding includes injection therapy,
heat coagulation, sclerotherapy, H2 blocker, sucralfate, and general supportive
care. Surgical excision, angiographic embolization, and systemic chemotherapy
are also considered choices of treatments.[1]
o Hemangiopericytoma occurs as a proliferation of peripheral cells located in
the arteriovenous capillary network (Rougiet pericytes). Their appearance is of
tumors formed by clews of capillary outside the basement membrane,
sometimes forming hematic cysts on the stomach wall. Tumors betray their
presence by gastrointestinal bleeding. Treatment is surgical. Until 1995, only
29 cases were reported.[2]
92
Gastric Lymphoma
Gastric lymphoma can be primitive or secondary in a systemic disease (Hodgkin's
disease, Brill-Symmers disease) and may be reticulosarcoamas or lymphosarcomas.
However, the stomach is the most common extranodal site of lymphoma, representing
4-20% of all extranodal lymphomas [3] and the most common site in the gastrointestinal
tract.
Gastric lymphomas are encountered mostly at ages over 50 and two to three times
more frequently in males than in females.[4]
Morphopathology
Most gastric lymphomas are developing from the so-called mucosa-associated
lymphoid tissues (MALT), which usually develop after chronic inflammation induced,
by H. pylori infection. The association between H. pylori chronic gastritis and MALT
lymphoma has been confirmed in large population based studies.[5-7]
The origin of majority of gastric MALT lymphomas is the B-cells non-Hodgkins
type (NHLs). Other rare forms of gastric lymphomas are non-MALT type, and in rare
cases, tumors may be of T cell origin.
Grading has been classified into low, intermediate and high grade.
Macroscopically, tumors are large, developed primary in the submucosa layer. In
1/3 of cases, the lesions are larger than 10 cm at time of presentation. Usually invasion
of serosa precedes the mucosa invasion and metastases in regional lymph nodes (63%)
precede the visceral invasion. In 5-23% of cases it affects the whole gastric body or
there is a multifocal location.[8]
Risk factors:
o Helicobacter pylori
o Long-term immunosuppressant drug therapy
o HIV infection
Symptoms
In most patients symptoms are vague and nonspecific debuting with 4-10 months
prior the diagnosis. Upper abdominal pain and early satiety are the most common
symptoms. Other symptoms may be nausea, vomiting, weight loss, abdominal fullness,
night sweat, jaundice, fever and dysphagia. In advanced cases, hematemesis and melena
are present, manifested especially by occult bleeding and gastric obstruction.
Frequently the tumor is palpable in the epigastrium.
Diagnosis
Preoperative diagnosis of lymphoma is important because it allows an accurate
assessment of patients for staging with a high therapeutic importance.
The diagnosis can be established only by histological examination and
immunohistochemical staining of tissue samples, which are typically obtained by biopsy
at the time of endoscopy. Other possibility is the biopsy during the laparoscopic
exploration of the peritoneal cavity.
Imaging investigations such as CT scans and endoscopic ultrasound are useful to
stage disease. Thoracic X-ray examination, CT scan, MRI and ultrasound examination
permit assessment of nodal involvement located above and below the diaphragm, and
extension of the tumor outside the stomach.
An elevated LDH level may be suggestive of lymphoma.
93
Staging: [3]
Staging Ann Arbor Classification (according to Musshoffs criteria)
o IE - Lymphoma limited to the stomach
o IIE - Abdominal extension from primary location
IIE1 - Involvement of stomach and contiguous lymph nodes
IIE2 - Involvement of stomach and noncontiguous subdiaphragmatic
lymph nodes
o III - Involvement of stomach and lymph nodes on both sides of diaphragm
o IV - Hematogenous spread (stomach and one or more extralymphatic organs
or tissues)
The Following subscripts may be added E=extranodal, S=splenic,
A=asymptomatic, B=symptomatic.
Modified Blackledge staging system for gastrointestinal lymphomas (the 5th
International Conference on Malignant Lymphoma) [9]
o Stage I - Tumor confined to gastrointestinal tract without serosal
penetration:
Single primary site
Multiple, non-contiguous lesions
o Stage II - Tumor extending into abdomen from primary site:
Nodal involvement
II1 Local (gastric/mesenteric)
II2 Distant (paraaortic/paracaval)
o Stage IIE - Penetration of serosa to involve adjacent structures:
Enumerate actual site of involvement, e.g. stage IIE
(pancreas), stage IIE (large intestine), stage IIE
(post-abdominal wall)
Perforation/peritonitis
o Stage IV - Disseminated extranodal involvement or a gastrointestinal-tract
lesion with supradiaphragmatic nodal involvement
No stage III
Treatment of malignant lymphomas is complex being represented by surgery, radio-
and chemotherapy.
Surgery is indicated in the presence of complications and it should be as
conservative as possible. Surgery is represented by subtotal or total gastrectomy and in
certain cases associated with extensive resection of neighboring organs if these are
invaded by tumor. It requires removal of regional lymph nodes.
Chemotherapy and radio-chemotherapy are indicated as adjunctive therapy not
only to treat but also to prevent relapses in patients at high risk or those with negative
prognostic factors. Adjunctive treatments diminish the importance of tumoral
infiltration of resection margins, aspect that becomes less important in the development
of tumor recurrence, so that an incomplete resection will not influence the survival in
patients undergoing such a complex treatment. This is the reason why limited operations
are preferred to those extensive considering also the better quality of life. Recent studies
have shown the importance of liver biopsy during surgery, while splenectomy does not
seem to be mandatory, as long as resection had a curative character and was
accompanied by standard lymphadenectomy.[9]
Surgery is not indicated in stages III or IV.[9]

94
Prognosis
Survival at 5 years in resectable cases is between 40% and 70%, depending on
lymph nodes status. However, the prognosis is better than in other gastric tumors.[9]
Gastric Carcinoid
The original description of carcinoid tumors was made by Langhans, in 1867.
Gossett and Massion in 1914 showed the affinity of certain granules in the cytoplasm of
the cells for silver salts. These were designated argentaffin cells and hence the lesions
were called argentaffinomas.[10] The term "carcinoid" was introduced by Oberndorfer
[11] first in 1907 and in 1923 Askanazy [12] reported the first gastric carcinoid.[13]
Carcinoid tumors are neuroendocrine tumors developed from fundal
enterochromaffin-like (ECL) cells of Kulchitsky, which are considered neural crest cells
situated at the base of the crypts of Lieberkuhn's glands. They either develop as a result
of chronic stimulation of enterochromaffin like cells by high concentrations of serum
gastrin or may occur sporadically.[14-16]
Epidemiology
The incidence of gastric carcinoid tumors have steadily increased over the last 50
years from 0.3% to 1.8% among all gastric cancers representing 8.7% of all enteric
carcinoids.[17] Reasons of this situation may be the improvement of diagnostic
procedures and the increasingly use of gastric acid inhibitors that induces higher levels
of gastrin secretion.
Gastric carcinoid can occur at any age between 20 and 90 years, being
encountered with a slightly higher frequency in women compared to men.
The occurrence of carcinoid tumors of clinical importance according to the
location of origin: [18]
28.5% small intestine
5% appendix
14% rectum
28% bronchial system of the lungs
5-7% colon
4% stomach
1% pancreas
>1% liver
8% other
Symptoms
Patients with gastric carcinoid tumors have nonspecific symptoms represented by
pain or a palpable abdominal mass. These types of tumors secret serotonin and other
histamine like substances being able to induce the carcinoid syndrome when the level of
secreted serotonin and histamine exceeds the metabolizing capacity of monoamine
oxidase present in the liver and lung.[10] Over 85% of patients are asymptomatic and
only 10% present carcinoid syndrome manifested primarily by transient erythema and
diarrhea.
Classification of gastric carcinoid tumors: [19,20]
Type I (75%) - Associated with chronic atrophic gastritis with or without
pernicious anemia. In autoimmune gastritis, a progressive destruction of
parietal cells leads to atrophy, intestinal metaplasia, and hypergastrinemia.
95
Type II (5%) - In Zollinger Ellison syndrome, especially in patients with
multiple endocrine neoplasia type I (MEN1).
Type III (20%) - Sporadic tumors, not associated with hypergastrinemia,
usually located in gastric antrum and corpus, reaching large even ulcerated
with a more aggressive evolution (survival at 5 years under 50%).
Macroscopic aspect is of a well-circumscribed polypoid formation covered by
mucosa, or rather larger infiltrating the whole gastric wall, which on the section surface
has a yellow-gray appearance.
Histological types are: [20]
I - tumor composed of G cells (gastrinoma) - usually solitary lesions located
in the antrum
II - tumor composed of enterochromaffin-like cells (ECL) - are the most
common; usually multiple; located in the gastric fundus; small; rarely
metastasizing or lethal; do not cause hypersecretion syndrome; may regress
spontaneously or after antrectomy [21,22]; are usually treated by local
excision or by endoscopic approach.
Carcinoid tumors are much less aggressive than adenocarcinomas having a
reduced capacity to metastasize. The incidence of metastases depends on tumor size,
being less than 15% in tumors smaller than 1 cm but can reach over 95% in tumors
exceeding 2 cm.
Tumoral invasion produces via the lymphatic and venous system. Regional
metastases are common, but distant metastases are rare.
Diagnosis
The diagnosis of carcinoid tumors is mainly based on gastroscopy and histological
examination of tissue samples. The carcinoid syndrome diagnosis is based on symptoms
and biochemical dosing of 5-HIAA (5-hydroxyindoleacetic acid) in urine, resulting
from degradation of serum serotonin or CGA (cromogranina a), which seems to be even
more specific.
Treatment of choice is tumors removal, either by endoscopic approach in small tumors
(<10 mm) or surgically (antrectomy or even total gastrectomy with proper lymph node
dissection) in larger, complex or multiple tumors.[23]
Chemotherapy proved to be the most effective although encouraging results are
obtained in only 20-30% of cases. To treat carcinoid syndrome, Somatostatin analogues
are used.[24,25]
Ongoing endoscopic surveillance is required every six-twelve months since
recurrence rate remains high.
Prognosis is excellent compared to other forms of malignancy. The average 5-year
survival rate for all types of gastric carcinoma is 64.3% (98% for type I) in localized
forms, 29.9% with regional, and 10% with distant metastases.[26,27] Long survivals
have been reported even in the presence of metastases.
References
1. Cheng-Hui Lin, Chao-Wei Hsu, Yan-Jen Chiang, Kwai-Fong Ng - Cheng-Tang Chiu, MD. - Esophageal and Gastric
Kaposi's Sarcomas Presenting as Upper Gastrointestinal Bleeding- Chang Gung Med. J. 2002; 25(5).
http://memo.cgu.edu.tw/cgmj/2505/250506.pdf
2. Guadagni S, Gianfelice F, Pistoia MA, et al. - A case of gastric hemangiopericytoma. - Minerva Chir. 1995; 50(7-8):693-
698.
96
3. Al-Akwaa AM, Siddiqui N, Al-Mofleh IA. - Primary gastric lymphoma. - World J. Gastroenterol. 2004; 10(1):5-11.
http://www.wjgnet.com/1007-9327/10/5.asp
4. Danzon A, Belot A, Maynadi M, Remontet L, Dupont AC, Carbonnel F. - Incidence and survival of gastric non-
Hodgkin's lymphoma: a population-based study from the Association of the French Cancer Registries (FRANCIM). -
Acta Oncol. 2009; 48(7):977-983.
5. Farinha P, Gascoyne RD. - Helicobacter pylori and MALT lymphoma. - Gastroenterology 2005; 128:1579-1605.
6. Montalban C, Norman F. - Treatment of gastric mucosa associated lymphoid tissue lymphoma: Helicobacter pylori
eradication and beyond. - Expert Rev. Anticancer Ther. 2006; 6:361-371.
7. Parsonnet J, Hansen S, Rodriguez L, et al. - Helicobacter pylori infection and gastric lymphoma. - N. Engl. J. Med, 1994;
330 (18):1267-1271.
8. Kitamura K, Yamaguchi T, Okamoto K, Ichikawa D, Hoshima M, Taniguchi H, Takahashi T. - Early gastric lymphoma:
a clinicopathologic study of ten patients, literature review, and comparison with early gastric adenocarcinoma. - Cancer.
1996; 77(5):850-857.
9. Speranza V, Lomanto D. - Primary gastric lymphoma.- Surgical Treatment: Evidence-Based and Problem-Oriented.
Holzheimer RG, Mannick JA, editors. Munich: Zuckschwerdt; 2001. http://www.ncbi.nlm.nih.gov/books/NBK6966/
10. Kaur S, Goyal R, Juneja H, Sood N, Bajaj P. - Carcinoid Of The Stomach -A Rare Tumour. - Ind. J. Radiol. Imag. 2006;
16(4):545-547.
11. Oberndorfer S. - Karzinoide Tumoren des Dunndarms. - Frankf. Z. Pathol. 1907; 1:425-429.
12. Askanazy M. - Zur Pathogenese der Magen-krebse und uber inhren gelegentlichen Ursprung aus angeboren epithelialen
Keimen in der Magenwand. - Dtsch. Med. Wochenschr. 1923; 49:49-51.
13. Abby Mulkeen, Charles Cha - Gastric carcinoid. - Curr. Opin. Oncol. 17:1-6. 2004 Lippincott Williams & Wilkins.
14. Berger MW, Stephens HD. - Gastric carcinoid tumors associated with chronic hypergastrinemia in a patient with
Zollinger - Ellison syndrome. - Radiology 1996; 201:371-373.
15. Hkanson R, Sundler F. - Proposed mechanism of induction of gastric carcinoids: the gastrin hypothesis. - Eur. J. Clin.
Invest. 1990; 20 Suppl 1:S65-71.
16. Solcia E, Rindi G, Silini E, Villani L. - Enterochromaffin-like (ECL) cells and their growths: relationships to gastrin,
reduced acid secretion and gastritis. - Baillieres Clin. Gastroenterol. 1993; 7(1):149-165.
17. Modlin IM, Lye KD, Kidd M. - A 50-year analysis of 562 gastric carcinoids: small tumor or larger problem? - Am. J.
Gastroenterol. 2004; 99(1):23-32.
18. A Review of Carcinoid Cancer - July 2012 (updated) - http://www.carcinoid.org/content/review-carcinoid-cancer
19. Rindi G, Luinetti O, Cornaggia M, et al. - 3 Subtypes of gastric argyrophil carcinoid and the gastric neuroendocrine
carcinoma a clinicopathological study.- Gastroenterology 1993; 104:994-1006.
20. Kloppel G, Anlauf M. - Epidemiology, tumour biology and histopathological classification of neuroendocrine tumours of
the gastrointestinal tract. - Best Pract. Res. Clin. Gastroenterol. 2005; 19:507-517.
21. Gergics P, Dabasi G, Csoregh E, et al. - Regression of metastatic gastric carcinoid associated with atrophic gastritis and
after octreotid treatment. - European Congress of Endocrinology 2007, Budapest, Hungary, Endocrine Abstracts;
(2007)14:485.
22. Odashima M, Otaka M, Jin M, Horikawa Y, Matsuhashi T, Ohba R, Mimori N, Koizumi S, Kinoshita N, Takahashi T,
Watanabe S. - Rapid Regression of Multiple Gastric Carcinoid Tumors with Hypergastrinemia and Atrophic Gastritis
after Renal Transplantation. - Dig. Dis Sci. 2008; 53(3):865-866.
23. Kurt Borch, Bo Ahrn, Hkan Ahlman, Sture Falkmer, Gran Granrus, Lars Grimelius - Gastric Carcinoids Biologic
Behavior and Prognosis After Differentiated Treatment in Relation to Type. - Ann. Surg. 2005; 242(1):64-73.
24. Kvols LK, Moertel CG, OConnell MJ, Schutt AJ, Rubin J, Hahn RG. - Treatment of the malignant carcinoid syndrome:
evaluation of a long-acting somatostatin analogue. - N. Engl. J. Med.1986; 315:663-666.
25. Kvols LK. - Metastatic carcinoid tumors and the malignant carcinoid syndrome. - Ann. N. Y. Acad. Sci.1994; 733:464-
470.
26. Modlin IM, Kidd M, Latich I, Zikusoka MN, Shapiro MD. - Current Status of Gastrointestinal Carcinoids. -
Gastroenterology 2005; 128:1717-1751.
27. Adachi Y, Shiraishi N, Kitano S. - Modern treatment of early gastric cancer: review of the Japanese experience. - Dig.
Surg. 2002; 19:333-339.

97
6. Gastric Volvulus
Gastric volvulus means partial or complete twisting of the stomach around of its
one axis. (Figure 40) The most frequently used classification is that proposed by
Singleton: [1]
1. Organoaxial (59% of cases)
2. Mesentericoaxial (29% of cases) and
3. Combined
Another classification:
1. Subdiaphragmatic, or primary (one
third of all cases) which is not
associated with diaphragmatic defects,
and
2. Supradiaphragmatic, or secondary,
associated with diaphragmatic defects.
The degree of twisting varies between 180 degree and 360 degree.
ble loss of blood supply (ischemia) and possible gastric wall necrosis,
c volvulus can be classified as:
type 2 - congenital or acquired
Etiolog
ost common complication of
nias
intragastric pressure by intrinsic or extrinsic benign or malignant
ow insertion of esogastric junction
Symp
ccompanied by early satiety, nausea and vomiting or simply
dyspe
en, with rapid
inability to vomit
apidly progressive
as sharp chest pain
radiating to the left side of the neck, shoulder, arms, and back.[2]
Consequences of this twisting are:
1. Varia
and
2. Gastric occlusion
According to etiology, gastri
1. type 1 - idiopathic, or
2.
y
Determinant factors:
1. Excessive laxity of the stomach fixing ligaments
2. Hiatal hernias - gastric volvulus is the m
paraesophageal her
3. Gastric adhesions
4. Other rare causes
Contributory factors:
1. Increased
obstacles
2. Motility disorders of the stomach
3. L
toms
In chronic cases, the clinical picture is dominated by epigastric pain that occurs
shortly after meals, a
ptic symptoms.
In cases of acute onset, symptoms are of an acute surgical abdom
progress to shock. In these cases, Borchardt describes the clinical triad:
1. Early vomiting followed by incoercible nausea and
2. Epigastric pain and distention r
3. Failure to insert a gastric tube
Intra-abdominal gastric volvulus manifests as a sudden severe epigastric or left
upper quadrant pain. Intra-thoracic gastric volvulus manifests
98
Diagnosis is based, in addition to clinical picture, on radiological exploration. In acute
forms, abdominal X-ray reveals epigastric fluid-air levels or an epigastric large gaseous
distension, or a fluid-air level in the left thorax above the diaphragm. In chronic forms,
barium swallow will highlight the shape and position changes of the stomach. In many
cases, this examination reveals the cause of the volvulus. CT scan with contrast is also
very useful in diagnosis.
Differential diagnosis should include acute cholecystitis, peptic ulcer, acute
pancreatitis, acute myocardial infarction, gastric perforation, etc.
Treatment
Surgery may be performed in emergency in acute cases, or it may be postponed
and performed in elective condition in chronic or intermittent cases.
Intraoperative aspects: (Fig 41)
Torsion of the esophagus
Duodenum displaced to the
left
Clockwise torsion of the
stomach
The great omentum covers
the stomach
Hemorrhages on the stomach
surface
The principles of surgical treatment are:
1. Decompression,
2. Reduction, and
3. Prevention of recurrence.
Tanner described the surgical
options for repair, including: [2,3]
1. Diaphragmatic hernia
repair
2. Simple gastropexy
3. Gastropexy with division of
the gastrocolic omentum
4. Partial gastrectomy
5. Fundoantral
gastrogastrostomy, and
6. Repair of the diaphragmatic relaxation
Gastric resection is indicated when gastric wall viability is affected. When gastric
walls are viable, gastropexy (the anterior wall of the stomach is fixed by sutures, with or
without gastrostomy tube, to the abdominal wall) is the method that prevents recurrence
of the volvulus. (Figure 42) Fundoplication is performed in case of hiatal hernia.
Other causing pathologies should be treated in the same operation: hiatal hernia,
extrinsic or intrinsic compressions, etc.
Endoscopic reduction can be attempted in patients with multiple co-morbidities
who are poor candidates for surgery.[2]
Mortality rates reach 80% for untreated and 15-20% with appropriate treatment in
acute cases, and 0-13% in chronic cases.[4,5]

99
References
1. Singleton AC. - Chronic gastric volvulus. - Radiology. 1940; 34:53-61.
2. William W Hope - Gastric Volvulus - Medscape reference, Emedicine http://emedicine.medscape.com/article/2054271-
overview
3. Tanner NC. - Chronic and recurrent volvulus of the stomach with late results of "colonic displacement". - Am. J. Surg.
1968; 115(4):505-515.
4. Palanivelu C, Rangarajan M, Shetty AR, Senthilkumar R. - Laparoscopic suture gastropexy for gastric volvulus: a report
of 14 cases. - Surg. Endosc. 2007; 21(6):863-866.
5. Katkhouda N, Mavor E, Achanta K, Friedlander MH, Grant SW, Essani R, et al. - Laparoscopic repair of chronic
intrathoracic gastric volvulus. - Surgery. 2000; 128(5):784-790.

100
101
7. Postgastrectomy Syndromes
Under this generic name, a number of specific late complications affecting 20%
of patients after gastric surgery are gathered. Vagotomy and ablation or bypass of the
pylorus are the most significant factors contributing to postgastrectomy syndromes.[1]
Gastric surgery may result in mechanical and functional disorders by two
mechanisms:
1. Mechanical disorders of digestive transit are represented by: stenosis,
efferent or afferent loop syndrome, bilio-gastric or esophageal reflux, all of
which are relatively easy to correct by surgical procedures.
2. Functional disorders, occurring in patients who received the correct surgery
but cannot adapt to changes in local physiology (dumping syndrome,
postprandial hypoglycemia, metabolic disturbances, diarrhea), which are
more difficult to treat, surgery in these cases assuming a secondary role.
Complications of gastric surgery: [2]
Esophageal
1. Gastroesophageal reflux
2. Dysphagia
Gastric
1. Delayed gastric
emptying
2. Bezoars
3. Anastomosis stenosis
4. Stoma inflammation
5. Gastric stump gastritis
6. Recurrent peptic ulcer
7. Gastric stump cancer
8. Afferent loop syndrome
9. Efferent loop syndrome
Small intestine
1. Postvagotomy diarrhea
2. Dumping syndrome
3. Bacterial contamination
syndrome
4. Pancreatic insufficiency,
celiac disease, lactase
deficiency
5. Weight loss and
malabsorption of:
1. Metals
2. Folic Acid
3. Vitamin B12
4. Calcium
5. Fats
6. Anemia
Gall bladder
1. Gallstones
All these conditions occurring late after gastric surgery have some common
characteristics:
Most cases occur after operations for duodenal ulcer
The condition is not influenced by the stage of the initial disease for what the
patient was operated
Disturbances are more intense when surgery produced profound
modification of the regional anatomy
Many postoperative complaints are often associated, and may be represented
by local, general or mixed symptoms
A. Functional disorders of the esogastric junction
Reflux esophagitis is caused by the alteration of the antireflux mechanisms at
level of cardia, followed by reflux of the gastric content into the esophagus and
consecutive esophagitis. This complication can occur after:
Surgical interventions that remove the cardia such as upper and total
gastrectomy,
Lower gastric resections because the Hiss angle becomes widened or is
abolished,
Any other gastric surgery after which the stomach evacuation is impaired.
The onset of symptoms may be rapid or after a period up to 5 years after surgery.
The clinical picture is dominated by heartburn, acid or bilious regurgitation, belching.
Initially, symptoms have a postural character appearing mainly in supine position. Then,
symptoms become permanent caused by local morphological changes of the reflux
esophagitis, and can be associated with anemia and dysphagia subsequent to repeated
bleeding and esophageal stenosis.
Diagnosis is based on radiological examination including Trendelemburg
position, endoscopic examination, manometry of the esophagus and pH-measurements.
Treatment may be prophylactic and curative. Surgical prophylaxis of esophagitis
should be performed during the primary operation.[3] Prophylaxis means a correct
surgical technique avoiding unnecessary detachments and mobilization of the stomach
during distal resection and vagotomy. Another method to prevent biliopancreatic
content of the duodenum to reach up into the esophagus is to remove the duodenum
from the normal digestive route, aim that can be achieved by restoring the digestive
tract after gastric resection using a gastro-jejunal anastomosis, either with a Roux-en-Y
loop or with an omega loop with Braun fistula. Curative surgery is indicated when
symptoms are severe and do not respond to usual therapeutic methods (postural
drainage, antisecretory drugs, etc.). The surgical procedure depends on the previous
operation. After inferior gastric resection, a fundoplication can be performed or the
gastroduodenal anastomosis is transformed into a gastrojejunal anastomosis using a
Roux-en-Y intestinal loop. If the initial operation was a superior gastric resection,
possible alternatives are pylorotomy and antro-jejunostomy with a Roux-en-Y loop.
After total gastrectomy, the aim is to divert the duodenal content far away from the
ascending intestinal loop that goes to the esophagus and the procedure depends on what
kind of montage was used initially.
Early postoperative dysphagia is usually caused by intraoperative trauma of the
esophagus and periesophageal hematoma. Causes of late postoperative dysphagia are
esophagitis and esophageal anastomotic stenosis.
B. Anastomosis disorders
In this category are included late complications resulting from inflammatory
reactions present at site of anastomosis and clinically manifested by superior digestive
bleedings and occlusive phenomena.
Gastritis is the inflammation of the gastric stump generally caused by duodeno-
gastric reflux. The pathogenesis may be represented by either chemical alkaline
irritation or microbial colonization of the gastric mucosa. Morphological aspects can be
hypertrophic or atrophic gastritis or even ulcerative gastritis. Symptoms are represented
by pain, belching, and vomiting, postprandial fullness and in about 20% of cases by
upper gastrointestinal bleeding. The diagnosis is established by endoscopy, which
reveals the edematous mucosa with hyperemia or ulcers. Treatment in most cases is
conservative using medication. Severe bleedings that cannot be resolved by medication
or endoscopic procedures require a surgical solution. This consists in either a total
gastrectomy or other different kinds of gastrojejunal montages (Roux-en-Y
gastrojejunostomy) accompanied by vagotomy.[4,5]
Anastomotic inflammation is caused, in many cases, by nonabsorbable sutures.
A reject type reaction appears around the suture threads with ulceration of the mucosa
or even polypoid mucosal hypertrophy that can interfere with the flow through the
102
stoma. Symptoms are uncharacteristic represented by dyspepsia and small and repeated
gastrointestinal bleedings. Treatment is usually surgical and consists in resection of the
anastomosis with restoration of digestive continuity associated with vagotomy. In some
cases, visible sutures can be removed by endoscopic procedures.
Stenosis of the anastomosis. Benign stenosis is encountered especially after
gastroduodenal anastomosis and is the result of an inflammatory intrinsic or extrinsic
process. Causes of stenosis are usually a wrong indication of surgical procedure or a
defective technique. Symptoms are of a high intestinal occlusion with early postprandial
stomach fullness sensation and vomiting, phenomena that occur months or years after
surgery. Diagnosis relies on radiological and endoscopic investigations, which should
always differentiate a benign stenosis from a malignant one. Treatment may be
prophylactic by respecting a correct indication and surgical technique. Surgical
solutions are represented by resection of the anastomosis and reestablishing the
digestive continuity using another type of anastomosis, usually a gastrojejunostomy on
Roux-en-Y jejunal loop, with or without vagotomy.
C. Mechanical dysfunctions
Afferent loop syndrome occurs less frequently in nowadays because surgical
tactic was modified. It appears after Bilroth II type operation (gastric resection and end-
to-side gastrojejunal anastomosis) being caused by the accumulation of secretions in the
duodeno-jejunal loop located upstream the anastomosis.
The syndrome may have an acute or a chronic clinical form. The acute form
occurs several weeks after surgery and is due to mechanical occlusion of the intestinal
loop (adhesions, internal hernia and torsion). The marked distension of the afferent loop
can cause duodenal stump dehiscence with subsequent fistula and peritonitis. The
chronic form is characterized by intermittent accumulation of secretions in the duodeno-
jejunal loop, being periodically discharged into the gastric stump, producing vomiting.
Causes of this condition can be mechanical, in most cases, represented by technical
deficiencies or incomplete extrinsic obstruction, or functional due to duodenal
dyskinesia.
The clinical syndrome is manifested in two phases: in the first there is an early
postprandial accumulation of secretion into the afferent loop that produces an epigastric
painful sensation of distension with general signs (tachycardia, pallor, sweating),
followed by the second phase manifested by vomiting and general signs resolution.
Diagnosis is based on radiological and endoscopic explorations that always must
be corroborated with clinical data. The barium swallow examination reveals the
persistence of the contrast in the afferent loop.
Treatment is surgical having an absolute indication in acute forms. The solution is
represented by the Braun fistula between the afferent and efferent intestinal loop and
removal of the obstacle. Other solution is deconstruction of the existing gastrojejunal
anastomosis and reconstruction of a new anastomosis when the duodenum is
reintroduced in its normal circuit (transformation of Bilroth II operation in Bilroth I
operation) associated or not with vagotomy or by performing a Roux-en-Y montage.
(Fig 43) Depending on the etiology, there are many other surgical choices.
103

Efferent loop syndrome is a rare condition caused by the obstruction of the
efferent loop or defective anastomosis. The clinical picture is represented by symptoms
of high intestinal obstruction with early postprandial vomiting. Diagnosis is confirmed
by imagistic investigation with soluble contrast. Treatment consists in reoperation and
in many cases by reconstruction of the anastomosis or just removing the obstacle.
Postoperative internal hernias are also less common and are mainly produced
by the penetration of an intestinal loop through a breach of the transverse mesocolon
(Petersen hernia) when transmesocolic gastrojejunal anastomosis was performed. The
herniated loop may become incarcerated producing intestinal occlusion or even
intestinal necrosis with peritonitis. The onset may be acute with sudden intense
epigastric pain followed by the abolishment of the intestinal transit and vomiting, or
progressive with chronic intermittent painful phenomena. In most cases, the diagnosis is
established during operation. The solution is represented by reposition of the herniated
intestinal loop in its natural position and closure of the mesocolon breach. If the
intestine is necrotic, it must be resected and the digestive continuity is restored by
enetro-enteral anastomosis.
D. Functional disorders
1. Early postprandial syndrome called " dumping syndrome"
It may occur after any type of intervention but mostly after an operation that
produces a profound change of the local anatomy. Most frequently appears after Billroth
II type operations and less frequently after vagotomy with pyloroplasty or GEA
(gastroentro- anastomosis). It also appears more frequently after surgery for duodenal
ulcer than for gastric ulcer or cancer. Women are more commonly affected.
Clinical picture
Symptoms are represented by the feeling of rapid fullness after meals, nausea,
even vomiting, belching, abdominal cramps and diarrhea.
General symptoms are generalized sweating, weakness, dizziness with pallor or
redness of the face, and palpitations. These phenomena may have variable intensities.
Symptoms are closely related to diet and appear especially after hypertonic food
intake. The explanation may be that local hyperosmolarity induces a massive bowel
fluid influx, decreasing the circulating blood volume and its redistribution.
Simultaneously a number of vasoactive substances (serotonin and kinines) are released
being responsible for general symptoms.
Diagnosis is based on clinical symptoms. Symptoms can be induced by oral
administration of hypertonic glucose solution (100-150 ml glucose 50%).
104
The treatment is prophylactic and curative.
Dumping syndrome prevention refers mainly to making an accurate indication
and the best surgical technique.
Curative treatment may be medical and surgical.
Conservative treatment is the first choice and it can solve up to 90% of cases.
Conservative measures include medical, dietary and behavioral therapy. The basis of
conservative treatment is the adjustment of the diet: small and frequent meals, avoiding
carbohydrates and hypertonic solutions (milk, sweet solutions), and postprandial rest.
The patient can itself remove from diet foods, which cause symptoms. The only drug
that has shown consistent efficacy is Sandostatin.
Surgery is applied only in 10% of cases and is indicated in case of medical
treatment failure in severe forms, extended over 6-12 months, leading to denutrition.
There are no standard procedures. The
procedure depends on which type of operation
was previously performed and its goal is to slow
down the gastric emptying.
The best results were obtained using an
anastomosis with Roux-en-Y intestinal loop, but
if the initial operation was a gastric resection
with gastrointestinal anastomosis by Roux, the
solution is an interposition of an anisoperistaltic
intestinal loop (Soupault-Bucaille operation).
(Figure 44)
2. Late postprandial syndrome or late dumping syndrome
Consists in dumping like symptoms that appear late, at 2-4 hours, after food
ingestion and which disappear after carbohydrate intake. The mechanism is represented
by late hypoglycemia as a result of excessive stimulation of the pancreas by
postprandial hyperglycemia consecutive to an accelerated absorption of carbohydrates.
The only treatment applied is that hygienic-dietetic by reducing carbohydrates in the
diet, and if symptoms appear, the patient will ingest one teaspoon of sugar. However, in
time this condition can lead to pancreas exhaustion with the development of diabetes
mellitus.
E. Other syndromes consecutive to gastric surgery
Weight loss and malabsorption occur in 30-60% of cases and are more common
after gastric resections associated with vagotomy. The cause is represented mainly by
the diminished gastric capacity that induces rapid satiety and also by many adverse
effects after truncal vagotomy (diminished gastric motility and emptying, lower gastric
acid secretion, increased gastric tone, augmented release of gastrointestinal hormones
such as cholecystokinin and gastrin, impaired release of insulin, etc.). [6] However,
patients with a syndrome of severe malnutrition and persistent diarrhea require further
investigation.
Anemia usually develops after gastric surgery. In most cases, an iron deficiency
anemia can be caused by:
Pre-existing anemia
Insufficient compensation of intra and postoperative blood loss
Postoperative gastrointestinal bleeding
105
Iron malabsorption due to decreased gastric acidity, and removal of the
duodenum (which is the place for absorption of all forms of iron) from the
normal digestive circuit.
Some patients may have anemia secondary to folate malabsorption. Vitamin B12
deficiency anemia can occur to either gastric resection that removes the gastric segment
that secret the intrinsic factor or secondary to infection or, rarely caused by pancreatic
insufficiency. In patients with pernicious anemia, urinary excretion of vitamin B12
(Schilling test) will reach normal values after administration of intrinsic factor
associated with low doses of vitamin.[7]
Gallstone disease is more common after truncal vagotomy than after other types
of vagotomy. The cause is the consecutive hypotonia of the gallbladder and decreased
bile flow.
Recurrent peptic ulcer represents the relapse of the ulcer after an operation
performed for ulcerous disease. In literature, the term is also used for relapses of ulcer
after H. pylori infection eradication.[8] This type of pathology is more rarely
encountered in nowadays as the peptic ulcer itself is more and more rare and more
rarely treated by surgeons.[8] Better understanding the pathology of peptic ulcer with H.
pylori as the principal cause and development of new generation of proton pump
inhibitor drugs, dramatically decreased the number of patients requiring surgical
solution for peptic ulcer.
Recurrence of peptic ulcer appears most often after duodenal ulcer surgery but
relapses after H. pylori eradication are more frequent after gastric ulcers (2.3%).[8]
Development of suture materials (absorbable) also contributed to decrease of the
incidence of this condition.
The highest rates of recurrence (30-35%) are encountered after simple
gastroenteroanastomosis (GEA) and the lowest after vagotomy associated with
antrectomy (under 1%). The general recurrence rate is 1-5%.[9] In simple
gastroenteroanastomosis, the secretory status of the stomach remains unchanged and the
acid comes in direct contact with the jejunal mucosa without being neutralized by the
alkaline content of the duodenum where as after vagotomy associated with antrectomy
the acid secretion is very diminished as a result of abolishment of cephalic phase of
gastric secretion and also the stimulating gastrin secretion is decreased.
Pathogenesis of recurrent peptic ulcer is similar to the primary peptic ulcer,
determinant role having the peptic acid hypersecretion and H. pylori infection.
Increased residual acidity denotes either a technical deficiency, a deficiency of surgical
indication or an abuse of ulcerogenic drugs. Ulcers caused by an endocrine mechanism
are usually multiple.[9-11]
Recurrent ulcer is found in most cases in the immediate vicinity of the
anastomosis between the stomach and duodenum or jejunum, but it can be found even
in the anastomosis if nonabsorbable sutures were used. Ulcer quickly penetrates the
digestive wall penetrating into adjacent structures.
Recurrent ulcer symptoms are atypical. Pain is less characteristic than in primary
peptic ulcer, being localized especially around the umbilicus having nothing in common
with food intake and is not calmed by antacids. Nausea and vomiting occur in the
presence of stenosis being less abundant as stomach capacity is reduced. Often recurrent
ulcers are manifested only by complications: gastrointestinal bleeding, perforation,
fistulas.
106
Digestive contrast radiography may reveal ulcerative niche, more difficult to
assess due to changes of the regional anatomy. It may also reveal ulcerative stenosis,
presence of fistulas or some technical deficiencies of the surgical procedure. The
decisive role in diagnosis has the endoscopic investigation. By endoscopy the ulcer can
be directly visualized, biopsies can be performed and unresorbable sutures can be
removed.
Laboratory investigations might be useful to asses the hormonal status especially
for gastrin levels, but also for other hormones and H. pylori presence.
Diagnosis of recurrent ulcer remains a difficult one. The most important
diagnostic tools remain endoscopy and hormonal tests.
Conservative treatment may be applied in the first stages of uncomplicated
recurrent ulcers. Therapeutic principles and methods are the same as for primary
duodenal peptic ulcer. About 50% of these patients are candidates for surgery and
surgery has a success rate of 70%.[9]
Surgery is indicated in complicated cases, for those who do not have a good result
after conservative treatment and also in cases when hypergastrinemia is found.[10]
Surgery aims are to remove the ulcerous part of the digestive tract, to complete the
initial operation in order to reduce the gastric secretion and to reestablish the continuity
of the digestive tract. Types of surgery depends much on primary operation: [9]
1. After truncal vagotomy without gastric resection (pylorotomy), a
hemigastrectomy or antrectomy will be performed
2. After gastric resection with vagotomy, a re-vagotomy may be indicated
because in many cases the initial vagotomy was incomplete
3. After insufficient gastric resections, a gastric re-resection may be performed
associated with vagotomy
Unfortunately there are cases (but rare) when even after surgical reintervention
the ulcer relapses, even with a good medical therapy, so total gastrectomy becomes the
only solution.[11]

References
1. Eagon JC, Miedema BW, Kelly KA. - Postgastrectomy syndromes. - Surg. Clin. North. Am. - 1992; 72(2):445-465.
2. Schwartz SI. - Principles of Surgery, Mc Graw - Hill Book Comp;1994
3. Ermolov AS, Dzhumabaev SU, Urinov AIa, Kharitonov LG. - Postoperative reflux esophagitis and its sequelae. - Vestn.
Khir. Im. I. I. Grek. 1994; 152(5-6):33-35.
4. Van Heerden JA, Phillips SF, Adson MA, McIlrath DC. - Postoperative reflux gastritis. - Am. J. Surg. 1975; 129(1):82-
88.
5. Sawyers JL, Herrington JL Jr, Buckspan GS. - Remedial operation for alkaline reflux gastritis and associated
postgastrectomy syndromes. - Arch. Surg. 1980; 115(4):519-524.
6. John G. Kral - Effects of truncal vagotomy on body weight and hyperinsulinemia in morbid obesity. -Am. J. Clin. Nutr.
1980; 33:416-419.
7. Deller DJ, Witts LJ. - Changes in the blood after partial gastrectomy with special reference to vitamin B12. I. Serum
vitamin B12, haemoglobin, serum iron, and bone marrow. - Q. J. Med.1962; 31:71-88.
8. Miwa H, Sakaki N, Sugano K, et al. - Recurrent peptic ulcers in patients following successful Helicobacter pylori
eradication: a multicenter study of 4940 patients. - Helicobacter. 2004; 9(1):9-16.
9. Stabile BE, Passaro E Jr. - Recurrent peptic ulcer. - Gastroenterology, 1976; 70(1):124-135.
10. Turnage RH, Sarosi G, Cryer B, Spechler S, Peterson W, Feldman M. - Evaluation and management of patients with
recurrent peptic ulcer disease after acid-reducing operations: a systematic review. - Journal of Gastrointestinal Surgery,
2003; 7(5):606-626.
11. Setlacec D, Popovici A, Medianu D, Horvat T. - Recurrent postoperative ulcer. - Revista de Chirurgie Oncologie
Radiologie ORL. Oftalmologie Stomatologie Chirurgie, 1981; 30(5):351-364.

107
108

Bariatric Surgery
BARIATRIC SURGERY

The word "Bariatric" was derived from a Greek word Baros meaning heavy
and Iotrics meaning medical treatment.
Bariatric surgery, or obesity surgery, includes several surgical procedures applied
to obese patients to lose weight and thereby improve their health condition. This kind of
surgery is not applied for aesthetic reasons.
Even though obesity is not a primary disease of the digestive system, this topic is
included in this volume because the vast majority of bariatric surgical procedures are
applied on the stomach.
Obesity
Background
Body weight is a feature that determines the individual physical appearance,
mental health, and quality of life.
Obesity is currently defined as an excess accumulation of body fat so that it can
affect the health. Obesity is the result of a positive energy balance protracted over time,
due to an increase in caloric supply and/or a decrease in caloric expenditure.[1]
Humans have been described as being among the fattest of all mammals.[2] In
fact, mammals embrace a wide range (1%-45%) of body mass across a wide range of
body size, and humans from pre-industrial societies are lying roughly within the middle
of the range for their weight.[3]
The ability to accumulate energy reserves, as fat in conditions of abundant food
was an evolutionary advantage necessary for survival in conditions of insufficient or
lack of food, so the chance of survival of obese individuals was higher during famine.
This tendency to store fat, however, is inadequate in today's developed societies, which
ensure a stable food supply.
From other point of view, we must not forget that until recently, obesity was
considered as a sign of distinction. Relevant are the paintings of Rubens, and other
artists, where overweight is the symbol of beauty. Opinion about obesity in nowadays,
has changed radically, not only from aesthetical point of view, but from medical as well.
Today is a certain relationship between obesity and diseases with the highest
mortality rate such as cardiovascular diseases, dyslipidemia, diabetes etc. Thus, 85-95%
of diabetics have been or are obese and over 60% of dyslipidemia patients are obese.
Obesity is rather a health problem then a problem of image. It becomes a major
cause of morbidity and mortality through diverse diseases (cardiovascular disease
diabetes and joint pain, etc.).
Obesity, first emerged as a major public health problem in western populations,
but rapidly has spread to countries undergoing nutritional transition and became the
primary global nutritional problem.[4] In the last decade, obesity and overweight have
become a global problem - according to the World Health Organization (WHO), back in
2005, approximately 1.6 billion adults over the age of 15 were overweight, at least 400
million adults were obese and at least 20 million children under the age of 5 years were
overweight. Experts believe that if the current trends continue by 2015, approximately
2.3 billion adults will be overweight and more than 700 million will be obese. The
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Bariatric Surgery
extent of the obesity problem causes serious consequences for individuals and
government health systems.
Formulas to establish the degree of obesity and risk of associated illnesses
Classically, obesity was considered as being the overcoming of the ideal weight
by 15 - 20% calculated after the Brocas index (Weight=Height-100). A more exact
formula to calculate the ideal weight was introduced by Lorentz in 1029:
Weight = Height - 100 [(Height - 150) x 0.25]
The formula is complicated and therefore not much used in practice.
Nowadays, the most commonly used is the Body Mass Index (BMI), for
classifying people's weight relative to an ideal weight for their height. Adolphe Quetelet
was who proposed this index formula and it was termed the Body Mass Index in 1972
by Ancel Keys.[5]

Obese is usually accepted the individual, whose BMI exceeds 30 kg/m2 as to
make differentiation from the overweight, whose BMI is between 25 to 29.9 kg/m2.
(Table 1)
Although BMI does not
directly measure bodys fat
percentage, it is still useful in
assessing body weight relative to
height. It is not a perfect indicator
from this point of view. For
example there are people,
especially men, whose high
weight, compared to height, is due
to muscular mass not to fat.
BMI is considered a lower
predictor of cardiovascular and
metabolic events compared to
abdominal (waist) circumference or waist-hip index, but currently remains the most
widely used epidemiological tool.
Table 1 BMI classification [6]
Category BMI Mass (weight)
Severe underweight
Underweight
< 16.5
16.5 to 18.5
< 53.5 Kg
53.5 - 60 Kg
Normal 18.5 - 25 60 - 81 Kg
Over weight 25 - 30 81 - 97 Kg
Obese Class I
Obese Class II
Severe obesity
Morbid obesity
Super-obese
Hyper-obese
30 - 35
35 - 40
40 - 45
45 - 50
50 - 60
> 60
97 - 113 Kg
113 - 130 Kg
130 - 146 Kg
146 - 162 Kg
162 - 194 Kg
>194 Kg

Waist circumference is especially useful in normal or overweight patients
categorized on the BMI scale. At BMIs greater than or equal to 35, waist circumference
has little added predictive power of disease risk beyond that of BMI. It is therefore not
necessary to measure waist circumference in individuals with BMIs greater than or
equal to 35.[7] A high waist circumference is associated with an increased risk for type 2
diabetes, dyslipidemia, hypertension, and cardiovascular
diseases (CVD) in patients with a BMI in a range
between 25 and 34.9 kg/sqm.[8,9] "The longer the belt,
the shorter the life!" (Figure 1)
Cardiovascular risk associated to waist index: [10]
1. Low risk <95 cm for men and <80 women
2. Medium risk 94-102 cm
3. High risk> 102 cm

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Bariatric Surgery
Waist-to-hip ratio is an index which provides information on fat distribution, and
it seems to correlate better than BMI with cardiovascular risk.[11] It is used mainly to
classify obesity as gynoid (gluteo-femoral type) or android (abdominal type). (Figure 2)
Persons with abdominal obesity, "apple-shaped", are at higher risk of metabolic and
cardiovascular diseases than those with "pear-shaped" bodies who carry more weight
around the hips.

Based on the index there are two clinical forms of obesity: gynoid and android.
Gynoid type obesity (Rubensian type) is seen more frequently in women, but it
can occur in male adults and children of both sexes. Skeletal muscles are poorly
developed, and adipose tissue is localized in the lower parts of the body, the lower
abdomen, flanks, hips, thighs, knees, calves. Patient moves with difficulty, tiring easily
and quickly gain weight. Consumes fewer calories, but weakens hard. Complications
such as respiratory, heart, bones, muscles, etc., occur rapidly. Some forms retain more
water and salt and do not respond to diet or diuretic therapy. A special form is the
Dercum cellulite, in which body fat infiltration is painful (adiposis dolorosa) and it has a
hereditary character.
Android type obesity (Falstaff type - Sir John Falstaff is a fictional character who
appears in three plays by William Shakespeare as a companion to Prince Hal, the
future King Henry V.) is found more frequently in men. Adipose tissue develops in the
upper part of the body, neck, shoulders, upper abdomen. The musculature is strong and
adipose tissue less developed than in the previous form. These, are people who always
complain of hunger and they eat more. Complications such diabetes, hyperuricemia,
dyslipidemia and atherosclerosis are the most frequent.
None of the formulas presented so far indicate, in fact, what is the percentage of
fat in the body. A more precise and individualized way to determine the ideal weight is
a test to assess the percentage of fat in the body. Fat percentage is an index that takes
into account that body fat percentage is generally 10% higher in women for the same
BMI and that body fat percentage is higher at more advanced ages even if weight
remains the same. Test accuracy is 4%. Formula is:
Fat % = 1.2 * BMI + 0.23 * age 5.4 10.8 * sex (where, sex =0 for woman and
sex=1 for man) The higher the fat percentage, the higher the risk of metabolic diseases.
(Table 2)
Table 2 Risk category according to fat percentage
Risk category Men Women
High < 5% < 10%
Low 5 - 15% 10 - 23%
Normal values < 20% < 26%
Moderate risk 20 - 24% 26 - 31%
High risk 25% or above 32% or above
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Bariatric Surgery
Utility of obesity indexes. Formulas represent the foundation for medical interventions
and nutrition in obesity. There are situations when medical interventions are
significantly based on these formulas: for example the decision to establish an enteral
nutrition, can objectively be sustained by calculating an index called Buzby. Body mass
index is used in some protocols as a landmark in establishing therapeutic attitudes (for
example, most surgeons do not perform obesity surgery for MBI below 40). Nutritional
risk index, called index Buzby, is used to stratify degrees of nutritional state of the
patient. The formula is: NRI (Nutritional Risk Index) = 1.519 x serum albumin (g / l) +
41.7 x (current weight / usual weight). The interpretation is:
1. NRI 97.5: no denutrition
2. NRI 83.5 - 97.5: moderate denutrition
3. NRI 83.5: severe denutrition
Dysmetabolic Syndrome
The metabolic syndrome is a cluster of the most dangerous heart attack risk
factors: diabetes and elevated fasting plasma glucose, abdominal obesity, high
cholesterol and high blood pressure.[12,13] Recently, the IDF (International Diabetes
Federation) issued a new set of criteria definition focusing on abdominal obesity as a
mandatory element of the syndrome. Dysmetabolic syndrome is represented by:
Abdominal obesity (defined by abdominal circumference > 94 cm for European
men and > 80 cm for European women - for other specific ethnic values, see the source
document) plus at least two of the four elements listed below:
1. High triglycerides (>150 mg/dL), or specific treatment for this type of
dyslipidemia
2. Low HDL-cholesterol (<40 mg/dl in men and <50 mg/dL), in women or
specific treatment for this type of dyslipidemia,
3. High blood pressure (systolic>130 mmHg or diastolic> 85 mmHg), or
treatment for previously diagnosed hypertension,
4. Elevated fasting glucose level (>100 mg/dL), or previously diagnosed type 2
diabetes.
Anatomy of the adipose tissue
Since childhood, there is a difference in terms of body fat and its distribution
between the two sexes prevailing in girls. Differences are maintained in terms of the
number of adipocytes (fat cells), which is higher in women. The number of fat cells
once established (possibly hereditary), remains permanently. The ratio between the
number and volume of fat cells causes two types of obesity: a form of hypertrophic
and another of hyperplastic obesity. Certain hormones also affect the body distribution
of fat. Androgen (testosterone) and glucocorticoids determine a prevalent distribution of
fat in the upper part of the body (android obesity) whereas the estrogen in the lower part
(gynoid obesity).
Hyperplastic obesity is represented by an increased number of adipocytes,
depending on the food received in childhood. The number of adipocytes in
adults is fixed. It stabilizes around the age of 20 to 23 years.
Hypertrophic obesity occurs in adults caused by reduced physical activity
without a proportional reduction of food intake. With age, physical activity
reduces and the caloric surplus is turned into triglyceride reserves, increasing
the volume of adipocytes.
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Bariatric Surgery
Besides hyperplastic and hypertrophic obesity, there are the mixed forms of
obesity, which associate an increased number of adipocytes with an increase in volume
that occurs at any time during life. It is more common in women.
Losing weight means lowering the volume, not the number of adipocytes, which
is a fixed value.
Adipose tissue is not an inert tissue; it regulates the uptake of fatty acids and
circulating triglycerides, the endogenous synthesis of glycerides starting from glucose,
the catabolism of fatty acids, the release of fatty acid into circulation, etc.
Regarding the feelings of hunger and satiety, regulation is done by:
Glucose (hunger during hypoglycemia and sensation of fullness during
hyperglycemia)
Digestive factors (glucose entering the duodenum is followed by a decrease
in hunger)
Neurological factors that act on the hypothalamus and cortex. Endogenous
obesity is the expression of a neuroendocrine disease.
Body fat is classified as:
1. Subcutaneous fat, and
2. Visceral (intra-abdominal or central fat)
In women the amount of fat is higher (about 1/5) but is mainly subcutaneous and
less visceral than in males. Visceral fat has its origins in brown adipose tissue,
incorporating a large amount of blood vessels and a high density of mitochondria, which
are very active metabolically. Visceral fat generates heat. It acts as a reservoir of fatty
acids, which are rapidly released into circulation for oxidation processes (combustion).
Problems arise when the fat exceeds a certain amount and constant release too
many fatty acids in portal circulation. As a consequence, hyperinsulinemia occurs
preventing normal suppression of glucose release from the liver and causing insulin
resistance (type 2 diabetes), increase of triglycerides and high-density lipoprotein
suppression (HDL) - See metabolic syndrome.
Epidemiology
Obesity is a problem that currently affects civilized world, where 25% to 50% of
population suffers from overweight or obesity.[14] (Figure 3) It is a medical and social
problem, reaching epidemic proportions worldwide. In 2008, more than 1.4 billion
adults, 20 years and older, were overweight. Of these, over 200 million men and nearly
300 million women were obese. More than 40 million children under the age of five
were overweight in 2010. Obesity medical costs account for 2-7% of total medical costs
in developed countries.[15]
In USA, 65% of adults are overweight or obese, and obesity represents the second
cause of preventable death. Direct health costs attributable to obesity have been
estimated to $52 billion in 1995 and $75 billion in 2003.[16]
It is generally believed that overweight is less prevalent than undernutrition in the
developing world, particularly in rural areas, and that it is concentrated in higher
socioeconomic status (SES) groups.
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Fig. 3 Global prevalence of obesity
The highest rate of obesity has been reported in the Pacific Islands and the lowest
rates have been seen in Asia. The rates in Europe and North American are generally
high, while the rates in Africa and Middle Eastern countries are variable.[17,18]
Obesity etiology
The vast majority of authors agree that the positive energy balance between
excessive food intake and reduced energy expenditure is the primary cause of obesity.
In a small number of cases, the high consumption of foods is due to genetic, medical, or
psychiatric disorders. Obesity is caused by a complex interaction between the
environment, genetic predisposition, and human behavior. The main causing factors are:
1. Diet
2. Lifestyle
3. Genetic factors
4. Socioeconomic factors
5. Medical and psychiatric diseases
6. Intestinal flora
7. Others
Diet is the principal factor in the development of obesity. It acts through the
composition of the macronutrients and eating behavior. Nutrients with high calorie
count per gram affect the accumulation of excess energy; fats are deposited in the
adipose tissue with about 96% efficiency. Food taste is also important; it is easy to eat
too much palatable foods.
Eating behavior: eating disorders such as consumption of large quantities of food
but not very frequently or excessive consumption in the late afternoon or evening
(night eating syndrome) predispose to obesity.[1,19]
Physical activity: this is the most variable element of the energy balance,
representing from 20 to 50% of the total energy expenditure. In western countries, there
is a close correlation between low levels of physical activity and obesity. The risk of
obesity is more than 5 times greater in children who watch television for five hours or
more per day, compared to those who watch it for less than two hours.[20]
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Bariatric Surgery
Social influences: population studies confirm the influence of the society in
which we live on the development of obesity. The body weight trend differs greatly
between people who emigrate to rich, western societies and those with the same genetic
patrimony who remain in their conditions of origin. There is a tendency to gain weight
after marriage and as parity increases. Environmental factors are a very important
component in the genesis of the behaviors that aid excess weight and are among those
called into question to explain the global epidemic obesity.
Genetic factors seem to be important having a role equal to 40% of cases.[1] The
ascertainment that obesity is present in entire families could appear to prove the genetic
hypothesis. Up to now, researchers verified about forty genes or loci in different
chromosomes apparently linked to the obese phenotype or, in any case, connected to the
amount of adipose tissue. The main genetic syndromes associated with obesity are the
'Trader-Willi syndrome" and the "Laurence-Moon-Bardet-Biedl syndrome" but other 25
conditions that associate obesity with genetic mutations have been described.
Educational level: the incidence of obesity is significantly greater in persons with
an elementary level of education or no formal education compared to graduates (15.4%
against 4.4%).[1,21,22]
Other factors
Easy access to palatable foods
Car culture
Mechanized manufacturing
Insufficient sleep
Psychological factors, meaning refuge in food satisfaction after a failure or
different emotional delusions
Endocrine disorders, which interfere with lipid metabolism
Decreased variations in ambient temperature
Lowering the percentage of smokers (smoking reduces appetite)
Excess of drugs that can lead to weight gain
Pregnancy in advanced ages
Positive natural selection of individuals with higher BMI
Expanding distribution of ethnic groups and other population groups that
tend to obesity
Associative tendency of obese people (marriages, etc.)
Neurobiological mechanisms implicated in obesity
The discovery of leptin in 1994 has elucidated a number of other hormonal
mechanisms involved in hunger, obesity, and peripheral insulin resistance. A number of
other mediators such as ghrelin, orexin, PYY 3-36, adiponectin, adipokines, etc., have
been discovered. It is believed that a deficiency of leptin is associated with obesity but
there was found that many obese have high levels of this mediator due to resistance
developed against this mediator. Leptin acts on the arcuate nucleus of the hypothalamus
so that the deficiency or resistance to this mediator leads to overeating, and represents
possible mechanism in some genetic or acquired forms of obesity.
Ghrelin is a 28 amino acid peptide hormone, structurally similar to motilin,
produced mainly by the stomach and, to a lesser degree, by other tissues.[23,24] Its
activity is mediated by the growth hormone (GH) activating the GH-secretagogue
(GHSla) receptors which are concentrated at the hypothalamic-hypophyseal level.[25]
Ghrelin also has many other endocrine effects. Ghrelin, unlike leptin has an orexic
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Bariatric Surgery
effect. The body weight gain caused by ghrelin is due to an increase in fat mass without
changes in skeletal musculature.[26]
Consequences of obesity:
1. Longevity is reduced by 12-15 years
2. Hypertension
3. Dyslipidemia
4. Diabetes type 2 with all its
consequences
5. Atherosclerosis, coronary artery
disease
6. Vascular accidents (stroke, heart
attack)
7. Gall bladder problems (lithiasis)
8. Osteoarthritis
9. Sleep apnea
10. Cancer (endometrial, breast,
prostate, colon)
11. Social discrimination
12. Lack of strength and energy
13. General malaise
14. Immobility

15. Headaches
16. Swelling of the legs
17. Varicose veins
18. Hemorrhoids
19. Chronic gastritis
20. Reflux esophagitis
21. Nervous system disorders: insomnia,
increased appetite for food, increased
thirst, vegetative disorders
22. Impaired potency
23. Affecting the menstrual cycle
24. Infertility (female extra pounds, make
the pregnancy difficult and sometimes
causes abortions)
25. Hydro-saline metabolism disorders
26. Complications after infectious diseases
27. Susceptibility to pneumonia
Recommendations:
Obese patients should be evaluated by a medical specialist (because obesity should be
considered a disease) to provide necessary and relevant information, to make the right
choice of treatment.
Available methods in treating the obesity and its consequences are:[27,28]
Diet - Reduce caloric intake by 500 to 1,000 kcal per day to produce a
weight loss of 1 to 2 lb per week. Whatever diet is recommended, do not
forget that what matters are calories! (1200 kcal/day for women and 1500
kcal/day for men are considered as normal intake). Nutritional needs should
take into account the individuals age, sex, height. The diet should last about
six months. Then, follows a period of 12-18 months, in which the loss should
be maintained. Only after that, a new diet starts again. To combat obesity
both, dietary and behavior attitude toward the physical activity should be
changed. Food should provide the principal nutrients, but also trace elements
(Ca, Mg, Na, K, etc.) and vitamins. Usually it is a mild hypocaloric diet,
with reduced fat (especially saturated fat) and salt, rich in fiber.
Physical activity - Obese patients should start with moderate levels of
physical activity (e.g., brisk walking) for 30 to 45 minutes, three to five days
per week. All patients should accumulate at least 30 minutes or more of
moderate intensity physical activity on most, and preferably, all days of the
week.
Changing lifestyle - Use multiple strategies (e.g., combine cognitive
restructuring, self-monitoring, social support, stimulus control and stress
management).
SPA
Alternative medicine (acupuncture)
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Bariatric Surgery
Medication - Always drug therapy is associated with diet and exercise.
Anns Rule: start medication only after 2-3 months of efforts with diet and
exercise.
Surgery
Treatment of related diseases
The objectives of these recommendations are:[29]
1. To reduce body weight: 10% of body weight in six months
2. To maintain the weight loss over time
3. To prevent further weight gain
It is necessary to establish a management strategy for the obese patient according
to its BMI:[1]
BMI 25-29.9 - hypocaloric diet, physical exercise
BMI 2.5-29.9 associated with co-morbidities - hypocaloric diet, physical
exercise, possible pharmacological therapy
BMI 30-34.9 - integrated intervention consisting of hypocaloric diet,
pharmacotherapy, behavior therapy, physical exercise
BMI 35-39.9 associated with co-morbidities - integrated intervention as per
preceding point associated with possible surgical treatment
BMI > 40 - integrated intervention and surgical treatment
Benefits of weight loss
Decreases the risk of type 2 diabetes
Lowers blood pressure
Improves lipid metabolism
Reduces the risk of sleep apnea
Reduces symptoms of osteoarthritis
Treatment
Scientific studies have shown that conservative treatment, alone cannot provide a
significant long-term weight loss in patients with severe obesity. It has been shown that
vast majority of patients regain lost weight over the next five years, causing depression,
anxiety, and irritability. Despite the fact that conservative methods are mostly doomed
to failure in obtaining sustainable weight loss, is still better to give the patient the
chance to benefit from these methods before reaching at surgical therapy, because the
diet should still be respected in postoperative period. These patients require multiple
investigations, multidisciplinary checkups, expensive treatments and a continuous
monitoring.
The aim of treatment for morbid obesity should be the improving of health
condition by obtaining a lasting weight loss. Fluctuations in weight by caloric restriction
should be avoided.
Surgical treatment
BARIATRIC SURGERY represents the surgical maneuvers that are applied to
obese patients in order to normalize their weight status, not including removal of fat or
other cosmetic surgical techniques.
Following the recommendations of consensus conferences, obesity surgery should
be reserved for patients with BMI> 40 or BMI> 35 in patients with associated co-
morbidities. Surgical treatment should be considered after failure of conventional
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Bariatric Surgery
therapy of obesity: diet and behavior modification, physical activity and
pharmacotherapy.
Evaluation of the subject requires the assessment of the degree of weight gain, the
level of health risk and the entity of the motivation to lose weight. The following points
should be considered:[1]
the reasons that inspire weight loss,
the history of previous attempts, whether successful or not,
the presence of support from family, friends and in the work
environment,
the subject's knowledge about the causes of obesity,
the attitude towards physical activity,
the ability to engage in physical activities,
the availability of time for treatment,
economic considerations.
Contraindications
1. Psychiatric disorders
2. Adrenal and thyroid disease
3. Chronic inflammatory pathology of the digestive system
4. Alcoholism and drug addiction
Bariatric surgery is the only treatment for severe obesity for which there is
scientific evidences regarding mortality reduction.
Effects of bariatric surgery on the main endocrine and metabolic alterations
caused by obesity. The majority of data in the literature concerns insulin. In fact,
bariatric surgery significantly reduces insulin resistance, insulin secretion and the
connected alterations in glucose tolerance and lipid metabolism, most likely because of
the reduction in adipose mass. It is also believed that weight loss re-establishes normal
GH secretion in the obese person.
Conditions for the best result of bariatric surgery are:
1. Surgery should be performed by an experienced surgeon who works in a
suitable environment
2. Hospital environment must ensure a multidisciplinary pre-and postoperative
approach, with postoperative follow-up for life
3. The patient must be well informed, highly motivated and with minimal
operative risks
4. Patient selection must be made by a multidisciplinary team internist,
endocrinologist, surgeon, psychiatrist and nutritionist
5. Postoperative dietary supervision is required.
Surgical procedures may be:
Restrictive - reduce the food intake. The role of gastric resection is to induce
early satiety, to limit the food intake and so inducing the weight loss. Restrictive
procedures are:
o 1. Intragastric balloon
o 2. Gastric banding
o 3. Vertical banded gastroplasty
o 4. Gastric sleeve
Malabsorptive - reduce food absorption surface in terms of food consumption
almost unchanged. Weight loss is based on fat and protein malabsorption. These
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Bariatric Surgery
procedures improve lipid status and glucose control in patients with
dyslipidemia and diabetes. On the other hand, it may produce Iron (Fe)
deficiency anemia, deficiency of fat-soluble vitamins (A, D, E, K), bone
demineralization and very smelly flatulence due to steatorrhea.
Mixed procedures - restrictive and malabsorptive
o 5. Gastric bypass
o 6. Biliopancreatic diversion (Scopinaro)
o 7. Duodenal switch (Marceau)
All these procedures (except intragastric balloon, which is an endoscopic
procedure) can be performed by open surgery or laparoscopic approach, but almost all
cases are performed in nowadays by laparoscopic approach due to its well-known
advantages, being a minimal invasive procedure.
1. Intragastric balloon procedure (Figure 4)
The procedure is based on lowering the stomach capacity after introducing of a
foreign body (balloon) into the stomach that induces thus the feeling of fullness. The
first experiences were performed in the 80s, but the results were disappointing due
complications. In 1986, Gau introduced the use of a silicone
balloon that could be inflated with saline solution, this
technique being used in nowadays. The procedure is
reversible.
The balloon is placed endoscopically into the stomach
and filled with saline dyed with methylene blue. About 400-
700 ml of solution is introduced, the balloon occupying about
40% of stomach capacity. Antisecretory medication is
associated. The balloon must be removed after a maximum
period of six months. Unfortunately, after removal, the lost
weight is generally regained. The weight loss is 25%-40% of extra pounds.
The procedure can be useful for preparing the patient for a later bariatric surgery,
reducing the anesthesiological and surgical risk thanks to the achieved weight loss.
Contraindications of the procedure are:
previous surgery on the stomach
severe esophagitis or gastritis
peptic ulcer
hiatal hernia >5 cm
hemorrhagic diathesis
drug or alcohol addiction
treatment with anticoagulants or cortisone
pregnancy
psychiatric disorders
Possible complications are nausea and vomiting, colicky pains, gastric ulceration
and perforation, bowel occlusion due to balloon disinflation and balloon rupture.
2. Gastric banding (Figure 5) is also a gastric restrictive procedure, which may be
reversible. It is performed by laparoscopic approach. An adjustable band is set around
the stomach just below the cardia and inflated in order to produce a narrowing and thus
dividing the stomach into two segments. The superior segment has a very limited
capacity (20-30 ml) inducing a very rapid satiety after food intake. There are several
types of rings (bands) but the most used are those of silicone, equipped with a port that
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Bariatric Surgery
allows inflating the ring to produce the desired narrowing of the stomach. The ring is
inflated with saline solution, under fluoroscopic guidance.
The procedure was widely applied, but because of its poor results and possible
complications, is less applied in nowadays, when other methods (gastric sleeve) proved
to have better results.
The procedure is indicated for patients with BMI of 40-50. The weight loss is
about 50% of extra pounds but in 20% of
cases, it has no apparent effect.
Complications may appear in up to 20% of
cases being represented by: ring slippage,
ulceration and perforation of the stomach,
intragastric migration of the band,
enlargement of the upper segment of the
stomach, and port infection.
The main contraindication for
adjustable gastric banding is the
nonacceptance on the patients part of
dietetic restrictions, with lack of compliance
with the long-term follow-up and behavioral disorders.
3. Vertical banded gastroplasty (Figure 6)
This procedure is a restrictive one and weight loss is caused by early satiety
consecutive to reduction of the functional volume of the stomach. Two procedures are
combined in this operation. Initially the stomach is partially divided into two segments
using two types of staplers: circular and linear. The upper segment will represent the
new small stomach (gastric pouch), which will communicate with the other segment of
the through a narrow outlet created by the circular stapler. The narrow communicating
part of the stomach is then calibrated using a
ring represented by a nonadjustable band
used also to prevent gastric dilatation of this
segment.
Early postoperative complications are
represented by those common to any
operation (pulmonary embolism, respiratory
insufficiency, sepsis, etc.) or specific, like
bleeding, perforations, pouch-emptying
delays. The most frequent late postoperative
complication is caused by poor patient
compliance and intolerance to solid foods,
with excessive vomiting, outlet stenosis,
gastroesophageal reflux, gastric pouch
dilatation and weight loss failure.
If weight loss after this operation is unsatisfactory, it can be easily transformed
into a gastric Roux-en-Y bypass operation.
4. Gastric sleeve procedure (Figure 7) is an irreversible procedure based on the same
principle of reducing the capacity of the stomach that induces rapid satiety.
This procedure was originally published by Marceau in 1993 as a restrictive part
of the duodenal switch operation. It is widelly used in Europe.
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Bariatric Surgery
It is a simple procedure performed by laparoscopic approach. The stomach is
divided into two parts using linear staplers. The larger part of the stomach, represented
by the greater curvature, gastric fundus and a part of the body, is removed. The
remaining part of the stomach is represented by a narrow gastric tube (sleeve) formed
by the lesser curvature of the stomach and the antrum. Special devices such as LigaSure
and staplers with hemostatic strips simplified very much this operation (the mean
operating time is 60-90 minutes).
The procedure does not affect digestion.
The weight loss is about 60% of extra pounds
in the first year. The patient may regain weight
after 4-5 years due to the enlargement of the
remaining stomach.
Gastric sleeve may represent the first
stage in treating super- or hyper-obese patients.
This operation will ensure a weight loss, which
will make more confortable the next operation
(gastric bypass or duodenal switch) and will
reduce the anesthesiological and surgical risks.
A second step procedure is proposed after a
mean of 12-16 months if the patient regains
weight or after stabilization of weight loss.
The main indications of this procedure
are:
BMI of > 40
Patients who are not in appropriate physical condition to undergo gastric
bypass surgery or other more radical weight loss surgeries
Patients who cannot return for the follow-up visits required by gastric
banding
Males with android obesity
Age > 60 years
Intraoperative unexpected technical or anatomical difficulties during more
radical intended operations
Adolescents
Down staging ASA score as a first step of other more radical bariatric
procedure
The advantages of sleeve gastrectomy are as follows:[1]
The stomach is reduced without loss of function
The chance of ulcer occurrence is minimized
Pyloric preservation prevents dumping
It requires only few days of hospitalization
It provides an effective first-stage procedure for super obese patients
It is useful in patients with disorders such anemia or Crohns disease
No foreign materials are left inside the body
5. RouxenY gastric bypass (RYGBP) (Figure 8) is a more radical operation, which
combines two principles: that of gastric volume reduction with that of malabsorption
produced by the reduction of intestinal absorption of alimentary nutrients. There is also
an important hormonal mechanism, which contribute to the weight loss.
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Bariatric Surgery
In 1966, Mason, starting from observations that patients operated with gastric
subtotal resection for peptic ulcer had a rapid postprandial satiety and had lost weight,
began the restrictive surgery for obesity using gastric bypass.
This is the bariatric operation most spread
in USA.
The restrictive procedure is achieved by
dividing the stomach into two segments: the upper
one, the gastric pouch, which will represent the
new stomach of small volume (15-20 ml) and
the lower one, which will be left on site. The
gastric pouch will be anastomosed to a Roux-en-
Y jejunal loop ascended in supramesocolic region
in front of the transverse colon or retrocolic
through the transverse mesocolon. This will be the
alimentary limb of the Y loop. The jejunum will
be transected at a distance of about 50 cm from
the duodenal Treitz angle (this will be the
biliopancreatic limb) and reinserted into the
jejunal loop (which will be ascended) at about 100
cm from the resection line of the intestine. Distances where the jejunum will be divided
and reinserted may vary according to many factors but especially considering the BMI
of the patient. Therefore, the smaller the gastric pouch and the more distal the intestinal
segment anastomosed to it, the more effective the procedure is.
The effects of this operation will be:
Restrictive - rapid satiety after food intake due to the small capacity of
the gastric pouch
Malabsorptive - food will meet the biliopancreatic juice and digestion
will start only after the alimentary limb of the Y intestinal loop (the
common loop). In this way digestion will be impaired and the absorption
as well.
Hormonal - exclusion of the food transit of the stomach, in particular of
the fundus and duodenum, where ghrelin (orexic hormone) is produced,
would lead to the stimulation of the secretion of ghrelin which, through
an override inhibition, would result in the suppression of ghrelin
production, with the effect of decreasing the feeling of hunger and
relative reduction in food intake and frequency of meals.[1] It is still
unclear whether or not and how ghrelin plays a role in this complex
hormonal mechanism.
Indications:
BMI>50
Failure of a previous restrictive bariatric procedure
Metabolic disorders: RYGBP is more likely to be effective in patients
with metabolic disorders in the presence of significant imbalances, where
malabsorption is the only possible solution and gives a guarantee of long
term success (for example, dyslipidemia of particular significance)
Eating disorders: patients with reduced compliance with diets
Sweet eaters: a restrictive operation is not effective for these patients but
in RYGBP procedure there is a reduced sweet intake over the long term,
especially when dumping syndrome is present
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Bariatric Surgery
The procedure is more laborious and burdened by possible intraoperative
complications then the previous presented operations.
Early postoperative complications are represented by:
Anastomotic dehiscence with an incidence that varies from zero to 7%, is
the most severe complication, being the major cause of death
Intestinal obstruction
Digestive hemorrhages from anastomotic bleeding
Peritonitis caused by intestinal perforation undiagnosed during operation
The most frequent late postoperative complications are stenosis of the
gastrojejunal anastomosis, anastomotic ulcer and intestinal obstructions caused by band
adhesions or internal hernias.
6. Biliopancreatic diversion (Scopinaro procedure) (Figure 9)
It is a mixed restrictive and malabsorptive method. Stomach volume is reduced by
distal gastrectomy. Digested food absorption is reduced by intestine anastomosis close
to the cecum. Bile and pancreatic juice will be in direct contact with food only in the
terminal portion of the intestine (the last 50 cm of ileum). A long (250 cm) Roux-en-Y
limb is used for reconstruction, where the enetroenteral anastomosis is performed at 50
cm distance from the cecum. Cholecystectomy is associated in most cases because of
the risk of developing gallstones.
With this operation a greater weight loss is obtained (80% of surplus) but with the
risk of hypoalbuminemia, hypovitaminosis and
dumping syndrome.
The essential weight loss is based on the
limitation of intestinal absorption. The absorption
of fats being conditioned by the presence of bile
salts, takes place only in the common limb (the
last 50 cm). Proteins and starches are absorbed in
the entire intestine. Mono and disaccharides, short
chain triglycerides and alcohol, which do not
require digestion, continue to be absorbed after
this procedure.
Unfortunately, there are many side affects
after this type of operation such as increased
frequency of bowel movements (two to four per
day) with foul-smelling stools and flatulence.
Dumping syndrome with diarrhea is also frequent.
Late specific complications include:
Anemia - the exclusion from the alimentary limb of the primary iron
absorption site inevitably leads to hypoferremia. Oral supplementation
with iron and folates reduces anemia incidence.
Post anastomotic ulcer - responds well to medical treatment and has a
low tendency to recur if the operated subject refrains from smoking and
alcohol.
Gastrojejunal anastomosis stenosis requires balloon dilatations or
surgical revision with deconstruction of the existing gastrojejunal
anastomosis.
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Bariatric Surgery
Bone demineralization - the duodenum and the proximal jejunum are the
sites of choice for calcium absorption.
Protein malnutrition - hypoalbuminemia, anemia, edemas, asthenia, and
alopecia are the most serious specific late complication and usually
require two to four weeks of parenteral nutrition.
Peripheral neuropathy requires administration of B complex and E
vitamins.
Biliopancreatic diversion is unanimously
considered the most effective operation for
obesity. Like any other powerful weapon, it can
be very dangerous if used incorrectly.[1]
7. Duodenal Switch (Marceau Procedure)
(Figure 10) is a variation of biliopancreatic
diversion.
The main differences consist in preserving
the antropyloric region to prevent dumping
syndrome and a longer digestive intestinal loop
for a better digestion and absorption. It is
considered a hybrid technique, which combines
the restrictive effect of gastric sleeve with that of
moderate intestinal malabsorption. The
alimentary loop has a length of 250 cm and the
common loop of 100 cm. The procedure primarily
induces malabsorption of fats due to the diversion of bile and pancreatic enzymes to the
distal portion of the alimentary limb (common limb).
Surgical treatment of type 2 diabetes (metabolic surgery)
The first intention of bariatric surgery was to treat obesity but over years,
remarkable metabolic effects, especially the improvement of type 2 diabetes, were
observed. Almost every obese patient with type 2 diabetes who underwent such surgery
had normalized their glucose levels and thus they were able to quit pharmacological
treatment.[30-33] Further, non-obese diabetic patients, who underwent gastric surgery for
diverse causes had noticed a better glycemic control.[34-36] These observations were
also confirmed by animal experiments.[37]
Improvement of glycemic control observed especially after gastrointestinal
bypass procedures (Roux-en-Y gastric bypass and biliopancreatic diversion are the most
effective procedures in controlling diabetes) offered the opportunity to treat and
understand type 2 diabetes and thus, surgery becomes a viable alternative to
conservative treatment of type 2 diabetes especially in obese patients. The antidiabetic
effect of bariatric surgery is also long lasting.
It is not known exactly yet all the mechanisms by which these interventions cure
type 2 diabetes, but there are several theories that have been confirmed by experiments.
Further research on this topic is needed.
The evolution of bariatric surgery to metabolic surgery for type 2 diabetes and
metabolic syndrome is represented by the change in title of American Society of
Bariatric Surgery to American Society of Metabolic and Bariatric Surgery.

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Bariatric Surgery
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nonobese type 2 diabetic patients: an interventional study with duodenal-jejunal exclusion. Obes. Surg. 2009;
19(8):1077-1083.
36. Cohen RV, Schiavon CA, Pinheiro JS. - Duodenal-jejunal bypass for the treatment of the type 2 diabetes in patients with
body mass index of 22-34kg/m(2):a report of 2 cases. Surg. Obes Relat Dis. 2007; 3(2):195-197.
37. Rubino F, Marescaux J. - Effect of duodenal-jejunal exclusion in a non obese animal model of type 2 diabetes mellitus: a
new perspective for an old disease. Ann. Surg. 2004; 239:1-11.

Surgical Pathology of the Small Bowel
SURGICAL PATHOLOGY OF THE SMALL BOWEL

1. Surgical anatomy
2. Intestinal volvulus
3. Intussusceptions
4. Crohn disease
5. Meckels diverticulum pathology
6. Tumors of the small intestine
1. Surgical Anatomy
The small intestine is the longest segment of the digestive tract extending
between pylorus and ileocecal valve, occupying most of the peritoneal cavity. Its length
is about 4-7 meters, with variations between 3 and 10 meters, depending on the tonicity
and constitutional type. It develops from the primitive intestine of endodermic origin,
which, communicates with the umbilicus during intrauterine life through the
omfaloenteric canal, which is completely reabsorbed
at birth.
The small intestine is composed of three
segments: duodenum, jejunum and ileum.
The intestinal diameter is of 2-4 cm. Intestines
form 15 to 16 U shaped loops of which the first 8 to
10 are horizontally positioned, belonging to the
jejunum, and the remaining in vertical position,
belonging to the ileum. (Figure 1)
The small intestine is neighboring with the
majority of abdominal viscera: superior with the
transverse colon and mesocolon, inferior with the
pelvic organs (bladder, rectum, uterus), to the right
with the ascending colon, to the left with the
descending colon, posterior with the retroperitoneal
organs and anterior with the greater omentum.
Except of duodenum, the small intestine is
provided with mesentery.
Mesentery is a dependence of the posterior
peritoneum through which the intestines are fixed to
the posterior abdominal wall. The root of mesentery
has a fix base of 15-18 cm length and it extends
obliquely from the duodenojejunal flexure (L2
vertebra) to the cecum. Inside the mesentery, there are
vessels (branches of superior mesenteric artery and
veins), nerves and lymphatics, all of them covered by
fatty tissue.
The distal portion of the mesentery is much
longer than the proximal one and forms folds. Its
greater length in the last ileal loops explains why
127
these loops are commonly involved in the right side inguinal and femoral hernias.
(Figure 2)
The duodenum is the first, the
shortest (25 cm), the widest and the most
fixed part of the small intestine. The
duodenum begins at pylorus, on the right
side, and ends at duodenojejunal junction, on
the left side. The duodenojejunal flexure is
fixed by the attachment of the suspensor
muscle of duodenum (Treitz). It has four
segments. The second, the third and part of
the fourth segment are retroperitoneal
segments. The pancreatic duct and the
common bile duct are emptying into the
second duodenal segment, at level of the
major duodenal papilla where the sphincter
of Oddi is present. (Figure 3)
The jejunum and the ileum.
Although these two segments appear similar, there are a few distinguishing features: the
jejunum is thicker and has plicae circulares, while the ileum has more Peyer's patches.
The small bowel enters the cecum at the ileocolic junction where there is the ileocecal
valve.
and inflammation of the diverticulum may simulate acute appendicitis.
Vascu
mportant condition for a safe
anasto
roach the intestine. From the last arcade,
vessel
rging into the
superior mesenteric vein (SMV), a tributary of the portal vein. (Figure 5)

Occasionally, a remnant of the vitelline (vitellointestinal or omphaloenteric) duct
persists in adults as an ileal diverticulum (Meckel's diverticulum), which is located at
50-100 cm from the ileocolic junction. The diverticulum may contain ectopic gastric or
pancreatic tissue,
larization
The small bowel is the best
vascularized segment of the digestive tract,
which is an i
mosis.
The main sources of arterial supply of
the duodenum are the inferior
pancreaticoduodenal artery and the superior
pancreaticoduodenal artery (from celiac
trunk through other arteries). For jejunum
and ileum the source of arterial supply is the
superior mesenteric artery (SMA) emerging
from the abdominal aorta below the celiac
trunk. (Figure 4) Each of those 12-16
primary branches, emerging from SMA,
divides into two branches, which unite with adjacent branches forming a series of
arterial arcades (arches). It may be up to five arterial arcades (in the longest portion of
the mesentery) diminishing in size as they app
s enter the intestinal wall as vasa recta.
Venous drainage originates from mucosa and submucosa network, from where it
forms a subserosal network from which emerge 7-8 venous trunks conve
128
Lymphatic vessels, originate from lacteals, in the intestinal villi, which drain into
the lymphatic plexuses. The lymphatic plexuses drain into lymphatic vessels between
the layers of the mesentery and then sequentially through three groups of lymph nodes:
1. Juxta-intestinal lymph nodes (close to the intestinal wall),
2. Mesenteric lymph nodes (scattered among the arterial arcades), and
3. Central superior nodes (along the proximal part of the SMA).
Efferent lymphatic vessels from these nodes drain into the superior mesenteric
lymph nodes. In the lower part of the ileum, there are the Peyer's patches, which are
aggregated lymphoid nodules. (Figure 6)
Carcinoma of the intestine can spread to the liver via the portal vein as well as via
lymphatics.

Small intestine innervation originates in the celiac and mesenteric artery plexuses,
from where fibers are distributed through the perivascular network to the myenteric
plexus (Auerbach) and submucous plexus (Meissner).
Small intestine structure
Inside, the small intestine has circular valves (Kerkring's plicae) with an initially
height of 7-10 mm, which decrease gradually towards the ileum, covered by intestinal
villi that gives the soft appearance of the mucosa. All these elements significantly
increase the contact surface with the intestinal content for a better absorption of
nutrients.
The small intestine has four layers: serosa, muscular, submucosa and mucosa.
Mucosa is the best represented; it occupies two thirds of wall thickness. Mucosa
epithelium is composed of enterocytes, Goblet cells, Paneth cells, stem cells,
enterochromaffin cells, and undifferentiated cells.
The main functions of the small intestine are digestion and absorption.

129
2. Intestinal Volvulus
Intestinal volvulus represents a distinct clinical form of intestinal obstruction
characterized by the twisting of the whole intestine or just a segment of it, at least 360
around its mesenteric axis.
Classification
The pathological process can involve the entire intestine, the total volvulus,
which frequently occurs in children. In these cases it is often involved the right colon as
well (common mesentery). In segmental volvulus, only a segment of the bowel is
twisted, and it occurs mainly in adults, with predilection in men. (Figure 7)
Another classification based on etiopathogenesis is:
1. Primary volvulus, mainly due to congenital anomalies (common mesentery).
2. Secondary volvulus, caused by extrinsic obstructions or compressions (adhesion
bands, tumors, etc.).
Morphopathology
The aspect of the intestinal segment and its
mesentery, interested in the pathological process,
is very similar to the aspect of the mesenteric
infarction. In the initial phase, the intestine is
enlarged, with thickened wall, edematous and red-
purple color. Then, the segment becomes brown,
inert, non-peristaltic, looking like a "dead leaf"
and has gangrenous areas or macroscopic
perforations. (Figure 8) At the base (root) of the
twisted bowel, there is a ring of torsion where the
most serious lesions are found, caused by
compression and ischemia. The fluid in peritoneal
cavity looks bloody with fetid smell.
Clinical picture
The clinical picture is dominated by
symptoms of intestinal occlusion installed more or
less acutely, followed by rapid onset of the
hypovolemic shock. Symptoms are represented by
violent pain, which does not respond to treatment.
Intestinal transit stops being accompanied by
nausea and vomiting. On inspection, asymmetrical
abdominal distension can be observed.
Workup
Although, there are no pathognomonic
signs for volvulus of small intestine, plain abdominal X-ray can be useful in
highlighting signs of occlusion, with multiple fluid-air levels.
Diagnosis
The diagnosis of certainty in most cases is not possible. The preoperative
diagnosis will be that of intestinal occlusion, which presumes surgical intervention. The
supposition of volvulus is based mainly on the sudden onset of violent continuous
abdominal pain localized around the umbilicus in a patient with possible previous
130
abdominal surgery and rapid progress to intestinal occlusion and hypovolemic shock. In
most cases, the final diagnosis is established during operation.
The differential diagnosis includes other causes of acute surgical abdomen: acute
pancreatitis, mesenteric infraction, peritonitis, etc.
Treatment
Treatment is exclusively surgical and of extreme emergency. It will be preceded
by a short and intense rebalancing continued intra- and postoperative. Surgery may be
conservative or radical. Conservative treatment means that the intestines will not be
resected. This is possible only in forms with recent onset, where the twisted intestine is
still viable but sometimes it is difficult to assess the viability. After detorsion, the
intestine is washed with lukewarm saline, and lidocaine is infiltrated in the mesentery.
After these maneuvers, a viable intestine restores it motility and resume normal color in
few minutes. A special attention must be given to the root of the twisted intestine where
lesions are more important.
To prevent relapses of the volvulus, enteropexy (surgical fixation of a segment of
intestine to the abdominal wall) or enteroplicatin (adjacent loops of intestine are sutured
to each other) can be performed. When the intestinal segment is compromised,
segmental resection is the solution followed by entero-entero anastomosis. The total
volvulus raises difficult problems for surgical attitude and unfortunately in most cases is
fatal.

131
3. Intussusceptions
Intussusception is produced when a segment of intestine invaginates into the
adjoining intestinal lumen, causing bowel obstruction. (Figure 9)
Epidemiology
Intussusception occurs mostly in children and
rarely in adults. Two thirds of children with
intussusception are younger than 1 year.
Intussusception is the most common cause of
intestinal obstruction in patients aged 5 months to 3
years.
Overall, male-to-female ratio is approximately
3:1. With advancing age, gender difference becomes
marked. In patients older than 4 years, the male-to-
female ratio is 8:1.[1]
Classification
There are two types of intussusceptions:
1. Primary or functional (idiopathic), which occurs on an unaffected bowel. There
are no evident causes of the intussusception.
2. Secondary or organic, when a mechanical obstacle (polyp, tumor, stenosis, etc.),
is the cause of intussusception.
Morphopathology
An intestinal intussusception, once started, tends to progress due to intestinal
peristalsis. Invagination process will stop at some length because of the tension in the
stretched mesentery.
Intussusception may occur in any intestinal segment, but it mostly appears at
ileocecal valve, where the terminal ileum invaginates into the ascending colon, favored
by the larger diameter of the colon. (Figure 10)
The part that prolapses into the other is called
the intussusceptum, and the part that receives it is
called the intussuscipiens.
There are two types of invaginations:
1. Simple, with 3 cylinders, and
2. Complex, with 5 or 7 cylinders
At the neck of intussusception, venous stasis
and swelling of the invaginated loop occur, which
will result in vascular elements compression,
responsible for the intestinal necrosis.
Clinical picture
Symptoms differ according to whether intussusception occurs in newborn,
infants, older children or adults.
In infants, intussusceptions begin suddenly with violent paroxysmal abdominal
pain; the infant becomes agitated, screams out, shakes hands and feet, and has a wry
face. The infant refuses food, or vomits the last meal. The bowel movements can be
abolished or bloody diarrhea may appear. The initial crisis lasts 5-10 minutes, and then
132
follows a lull lasting for variable time, and finally the resumption of crisis follows with
the same intensity or higher. Commonly the cycle consists of two or three such crises.
In older children and adult, the clinical picture is very different, abdominal
symptoms being much weakened and prolonged. The clinical picture often is that of
intestinal occlusion and the diagnosis is established during operation. At these ages,
three forms of clinical picture of the intestinal intussusception may appear:
1. Acute form, manifested as an intestinal occlusions
2. Subacute form, evolving with intermittent painful crises, interrupted by long
periods of lull
3. Chronic form, with typical attenuated symptoms, the clinical picture being
dominated by bowel disorders (constipation alternating with diarrhea)
The classic triad of signs and symptoms is represented by vomiting, abdominal
pain, and passage of blood per rectum. In infants, symptoms are more intense, and the
suspicion of intussusception is more common.
On abdominal palpation, the intussusception can be felt as a thickened "sausage
shaped" intestinal loop. Digital rectal examination reveals the presence of blood in
stool.
Workup
Abdominal plain radiograph will show a
typical pattern of intestinal obstruction. Contrast
radiography with swallowed barium and irigography
(barium enema) could identify the intussusception
but the barium is a problem for surgery if a resection
of the bowel will be needed. Radiological
appearance at barium (or other contrast material)
enema is the stop of the contrast substance, taking
the form of dome, cup, or trident, and form of
cockade in orthoroentgenograd aspect. In infants, the
mild barium enema carried out under the screen, can
reduce the intussusception.
Ultrasound (US) examination is preferred to
barium enema, because intussusception can be
identified in most cases by this harmless
investigation.[2-5] The components of an
intussusception produce characteristic appearances
on US scans. These appearances include the multiple
concentric ring sign (onion or target sign) and
crescent-in-doughnut sign on axial scans and the
sandwich sign and hayfork sign on longitudinal
scans.[6] (Figure 11) Ultrasound may also be useful
in diagnosing intestinal ischemia because the color
Doppler facility can reveal the mesenteric blood
flow.[7-9]
Diagnosis
In infant, intussusception diagnosis is based on the abdominal pain crises
associated with palpation of the invaginated intestine and/or the presence of blood on
digital rectal examination. In older children and adults, diagnosis is more difficult. In
133
many cases, symptoms can be confusing. In fact, only 30%68% of children with
clinical findings suggestive of intussusception are shown to have this condition.[6]
Therefore, it is desirable to use diagnostic tools such as ultrasound, which is the less
uncomfortable for the baby. Ultrasound has a sensitivity of nearly 100% and a
specificity around 90% in prospective studies.[3,4]
In most cases, the adult patient is operated with the diagnosis of bowel
obstruction, the intussusception being an intraoperative surprise.
Intussusception is a disease with an acute evolution encumbered by the
occurrence of severe complications, which endanger the patients life. Evolution to
spontaneous resolution of invagination is possible especially in infants, but is quite
exceptional and is encumbered by a large percentage of relapses. Unresolved intestinal
intussusception in the first 5-6 hours evolves to occlusion and intestinal necrosis with
subsequent perforation and generalized peritonitis. Untreated cases result in death
within a few days.
Treatment
Treatment may be conservative or surgical.
Conservative treatment is indicated in children as soon as possible after the
diagnosis and consists in air contrast enema (pneumatic reduction) that increases the
pressure in the intestine and may cause the return of the intestine to its normal position
(reduction). In most cases (46% to 80%), the procedure is successful.[10-13] The
procedure is carried out under fluoroscopic or ultrasound guidance.
Surgical treatment of an intussusception in
infants is indicated if there are signs of shock,
intestinal necrosis, or perforation (peritonitis
signs), or if the intussusception is irreducible. If
the affected intestine is viable, surgical treatment
is represented by a simple intestinal reduction
followed by enteropexy to prevent the relapse. If
the intestine is not viable, or has a dubious
viability, intestinal resection of the affected
segment is performed, followed by restoration of
the digestive continuity. (Figure 12)
The prognosis is very serious in untreated cases or excessively delayed, and is
directly correlated to the earliness of the intervention. With an early diagnosis and
adequate treatment, mortality rate is still about 1%.[14] Recurrence rate is estimated at
10% of cases.[10]
References
1. Felix C Blanco - Intussusception - Medsacape reference, Emedicine. http://emedicine.medscape.com/article/930708-
overview#a0156.
2. Pracros JP, Tran-Minh VA, Morin de Finfe CH, Defrenne-Pracros P, Louis D, Basset T. - Acute intestinal
intussusception in children: contribution of ultrasonography (145 cases). - Ann. Radiol. 1987; 30:525-530.
3. Verschelden P, Filiatrault D, Garel L, Grignon A, Perreault G, Boisvert J, Dubois J. - Intussusception in children:
reliability of US in diagnosis--a prospective study. - Radiology 1992; 184:741-744.
4. Bhisitkul DM, Listernick R, Shkolnik A, Donaldson JS, Henricks BD, Feinstein KA, Fernbach SK. - Clinical application
of ultrasonography in the diagnosis of intussusception. - J. Pediatr. 1992; 121:182-186.
5. Shanbhogue RL, Hussain SM, Meradji M, Robben SG, Vernooij JE, Molenaar JC. - Ultrasonography is accurate enough
for the diagnosis of intussusception. - J. Pediatr. Surg. 1994; 29(2):324-327; discussion 327-8.
6. del-Pozo G, Albillos JC, Tejedor D, Calero R, Rasero M, de-la-Calle U, Lpez-Pacheco U. - Intussusception in Children:
Current Concepts in Diagnosis and Enema Reduction - RadioGraphics 1999; 19:299-319.
134
7. Lam AH, Firman K. - Value of sonography including color Doppler in the diagnosis and management of long-standing
intussusception. - Pediatr. Radiol. 1992; 22:112-114.
8. Lagalla R, Caruso G, Novara V, Derchi LE, Cardinale AE. - Color Doppler ultrasonography in pediatric intussusception.
- J. Ultrasound Med. 1994; 13:171-174.
9. Kong MS, Wong HF, Lin SL, Chung JL, Lin JN. - Factors related to detection of blood flow by color Doppler
ultrasonography in intussusception. - J. Ultrasound Med. 1997; 16:141-144.
10. Liu KW, Maccarthy J, Guiney EJ, Fitzgerald RJ . - Intussusception-current trends in management. - Archives of Disease
in Childhood, 1986; 61:75-77.
11. Hilal A, MacMahon P, Cosgrove JF. - Outcome of acute intussusception in a regional paediatric centre. - Ir. Med. J.
2002; 95(2):58-59.
12. Blanch AJ, Perel SB, Acworth JP. - Paediatric intussusception: epidemiology and outcome. - Emerg. Med. Australas.
2007; 19(1):45-50.
13. Kaiser AD, Applegate KE, Ladd AP. - Current success in the treatment of intussusception in children. - Surgery 2007;
142(4):469-475; discussion 475-477.
14. Parashar UD, Holman RC, Cummings KC, Staggs NW, Curns AT, Zimmerman CM, Kaufman SF, Lewis JE, Vugia DJ,
Powell KE, Glass RI. - Trends in intussusception-associated hospitalizations and deaths among US infants. - Pediatrics
2000; 106(6):1413-1421.

135
4. Crohn's Disease (Regional Enteritis)
Crohn's disease represents a necrotizing granulomatous nonspecific inflammation
of the bowel. Although originally described only for the terminal ileum, it was
subsequently observed in any intestinal segment, affecting even the colon. Crohn's
disease and ulcerative colitis are frequently referred to as inflammatory bowel disease
(IBD).
The etiology is unknown. There are many hypotheses concerning the etiology. Bacterial
or viral infections and allergic reactions were most frequently promoted. It is considered
that Crohn's disease is caused by interactions between environmental, immunological
and bacterial factors in genetically susceptible individuals.[1-4]
Epidemiology
According to age, the disease occurs more often in the second or the third decade
of life and regarding sexual distribution, there is a slight preponderance in women.[5]
Approximately 3.6 million people in Europe and the USA suffer from BID.[6]
Crohn's disease affects 400,000 - 600,000 people in North America. Prevalence
estimates for Northern Europe have ranged from 27 to 48 per 100,000 inhabitants.[7-9]
Morphopathology
The most common affected site is the terminal ileum, on the last 30-40 cm, and
the colon followed by the anorectum. Perianal disease varies between 1476%.[10]
Jejunal location is a rarity. (Figure 13)
The appearance of the affected intestinal
segment differs as the disease is in the acute or
chronic phase.
In acute phase, the affected loop is red-purple,
swollen, stiff, and covered by fibrin deposits.
Intestinal wall is thickened and brittle. The limit to
the normal intestine is more or less clear, but the real
limit of the lesion exceeds the apparent macroscopic
limit.[10] The mesentery has an inflammatory process,
edema and enlarged lymph nodes. The mucosal
appearance is usually heterogeneous. Lesions of
different sizes are simultaneously present. The
mucosa may appear normal or may show multiple
small (12 mm in size) punctiform, rounded nodules,
or superficial erosions known as aphthoid lesions.[10]
In chronic phase, the affected segment looks like a cardboard being hypertrophied
and rigid with lipomatous sclerosis. Due to the bowel wall thickening, the lumen is
much narrowed. The corresponding mesentery is retracted and together with the
affected intestine can adhere to the neighboring organs gaining a pseudotumoral
appearance. Between these adhesions, chronic abscesses may be present. Over a period,
the mucosa erosions become confluent and transform into longitudinal ulcers
(serpiginous ulcers). The combination of longitudinal and transverse ulceration in an
edematous mucosa induces a characteristic "cobblestone" aspect. Ulcerations are more
common on the mesenteric border of the small intestine.[10]
The affection has a great tendency to fistulization in neighboring viscera or to the
skin.
136
Symptoms
Clinical picture may vary as the disease is in acute or chronic phase.
In the acute form, occurring mostly in children, the clinical picture is that of an
acute appendicitis associated with diarrhea. Fever may be also present.
The chronic form, most common, can evolve from acute form or may evolve from
the beginning as a chronic form.
Depending on predominant symptoms, there may be several clinical pictures:
A form that mimics ulcerative colitis manifested by right iliac fossa pain
occurring at 5-6 hours after ingestion of food, accompanied by watery and even
bloody diarrhea.[11]
The occlusive form, that evolves as a chronic occlusion with Konig syndrome in
the right iliac fossa, and finally as a complete occlusion. In stenosing forms,
vomiting and nausea may indicate the beginning of small bowel obstruction.
Pseudotumoral form is characterized by the presence of a right iliac fossa
tumoral mass with inflammatory phenomena associated to chronic pain and
subocclusive symptoms. This form may develop external fistulas in the
abdominal wall or perineum.
Crohn's disease can also cause a variety of systemic symptoms represented by:
growth failure in children, fever, and weight loss. In addition to systemic and
gastrointestinal involvement, Crohn's disease can affect many other organs such as eyes
(uveitis), joints arthritis, (ankylosing spondylitis), skin (erythema nodosum, pyoderma
gangrenosum), bones (osteoporosis, clubbing fingers), blood (autoimmune hemolytic
anemia) endocrine system and others.[12]
Workup
Laboratory tests may reveal hyperleukocytosis and anemia in severe forms, rarely
eosinophilia. Testing for Saccharomyces cerevisiae antibodies (ASCA) and
antineutrophil cytoplasmic antibodies (ANCA) has been evaluated to identify
inflammatory diseases of the intestine.[13,14]
Barium follow through X-ray is useful when
the disease involves only the small intestine.
Irigography shows the narrowing of the affected
segment and upstream dilatation.
The most useful tool in diagnosis is
colonoscopy with exploration of the last ileal
segment. Endoscopic mucosal appearance of
"cobblestone"-like is seen in approximately 40% of
cases. (Figure 14) The gastroenterologist can also
perform biopsies from affected area.
CT and MRI scans are useful for evaluating intra-abdominal complications of
Crohn's disease, such as abscesses, small bowel obstructions, or fistulas.
Ultrasonography - the main finding is a marked thickening of the intestinal wall
of up to 15 mm. A wall thickness more than 3 mm should be considered as a sign of
disease.[15]
Diagnosis
Clinical suspicion of Crohn's disease must be verified by histopathological
examinations.
137
Differential diagnosis, in acute forms includes the acute appendicitis and in
chronic forms ulcerative colitis, intestinal tuberculosis and intestinal tumors.
Evolution
Crohn's disease has a long evolution, with successive acute and subacute episodes
with an increased tendency to chronicity.
Complications are possible at any stage of evolution and consist of stenosis with
occlusion, perforations, internal or external fistulization. Fistulas have a chronic
evolution leading to patient weakening. Individuals with small bowel Crohn's disease
are at higher risk for small intestinal cancer and those with colon localization have a
relative higher risk for developing colon cancer.[16]
Treatment
Although, at present time, there is no cure for Crohn's disease, if it is diagnosed
early, the disease can be treated obtaining long remissions. The evolutionary form of the
disease determines medical or surgical therapeutic indication.
Certain lifestyle changes, including dietary adjustments can reduce symptoms.[17-
19] Medication used to treat symptoms of Crohn's disease include aminosalicylic acid,
prednisone, and immunomodulators such as azathioprine, mercaptopurine,
methotrexate, infliximab, adalimumab, certolizumab and natalizumab. Hydrocortisone
should be used in severe attacks of Crohn's disease.[20,21]
Surgery is indicated only in cases with complications such as obstructions,
fistulas and/or abscesses, or if the disease does not respond to drugs. Resection of a
large portion of the bowel is the only attitude that has some chances of cure but it will
lead to short bowel syndrome (watery diarrhea, minerals depletion, hypovitaminosis,
weight loss, anemia, etc.). Perforation suture and stomas are prohibited, as they will not
heal. After the first surgery, the disease usually shows up at the site of the resection, or
in other locations (another resection may be necessary within five years). Penetrating
type of the disease, a long duration of operation and handsewn anastomoses are factors
that increase the risk of postoperative intra-abdominal septic complications in Crohn's
disease.[22]
In rare cases, patients with Crohn's disease, especially those with postoperative
short bowel syndrome, are considered for intestinal transplantation. This operation
carries a high risk of death and of complications, including rejection of the new
intestine.[23,24]
Prognosis
Crohn's disease is a chronic condition but the mortality rate is relatively low.
However, it is associated with an increased risk of small bowel and colorectal
carcinoma. These patients have a much higher risk of small bowel cancer than general
population. Risk factors are represented by young age at onset, prolonged evolution,
smoking, and diffuse form of illness. Colorectal cancer is 5 times more frequent than in
the general population.[25]
References
1. Cho JH, Brant SR. - Recent Insights into the Genetics of Inflammatory Bowel Disease. - Gastroenterology 2011;
140(6):17041712.
2. Dessein R, Chamaillard M, Danese S. - Innate Immunity in Crohns Disease. - Journal of Clinical Gastroenterology,
2008; 42:S144147.
3. Stefanelli T, Malesci A, Repici A, Vetrano S, Danese S. - New Insights into Inflammatory Bowel Disease
Pathophysiology: Paving the Way for Novel Therapeutic Targets. - Current Drug Targets 2008; 9(5):413418.
4. Marks DJ, Rahman FZ, Sewell GW, Segal AW. - Crohn's disease: An immune deficiency state. - Clinical reviews in
allergy & immunology 2010; 38(1):2031.
138
5. Karlinger K, Gyrke T, Mak E, Mester A, Tarjn Z. - The epidemiology and the pathogenesis of inflammatory bowel
disease. - Eur. J. Radiol. 2000; 35(3):154-167.
6. Engel MA, Khalil M, Neurath MF. - Highlights in inflammatory bowel disease--from bench to bedside. - Clin. Chem.
Lab. Med. 2012; 50(7):1229-1235.
7. Crohn's disease From Wikipedia, the free encyclopedia
8. Bernstein CN, Wajda A, Svenson LW, MacKenzie, A, Koehoorn M, Jackson M, Fedorak R, Israel D et al. - The
Epidemiology of Inflammatory Bowel Disease in Canada: A Population-Based Study. - Am. J. Gastroenterol. 2006;
101(7):15591568.
9. Lotfus EV Jr . - Clinical epidemiology of in ammatory bowel disease: incidence, prevalence, and environmental in
uences. - Gastroenterology 2004; 126:15041517.
10. Geboes K. - Histopathology of Crohns disease and ulcerative colitis. -
http://www.forpath.org/workshops/0405/Crohn_s_disease_and_ulcerative_colitis.pdf
11. Baumgart DC, Sandborn WJ - Crohn's disease. - The Lancet. (2012).
http://www.thelancet.com/journals/lancet/article/PIIS0140-6736%2812%2960026-9/fulltext
12. Danese S, Semeraro S, Papa A, Roberto I, Scaldaferri F, Fedeli G, Gasbarrini G, Gasbarrini A. - Extraintestinal
manifestations in inflammatory bowel disease. - World J. Gastroenterol. 2005; 11(46):72277236.
13. Kaila B, Orr K, Bernstein CN. - The anti-Saccharomyces cerevisiae antibody assay in a province-wide practice: accurate
in identifying cases of Crohn's disease and predicting inflammatory disease. - Can. J. Gastroenterol. 2005; 19 (12):717
721.
14. Bernstein CN, Orr K, Blanchard JF, Sargent M, Workman D. - Development of an assay for antibodies to
Saccharomyces cerevisiae: Easy, cheap and specific for Crohn's disease. - Can. J. Gastroenterol. 2001; 15(8):499-504.
15. Kim Nylund, Svein degaard, Trygve Hausken, Geir Folvik, Glen Arslan Lied, Ivan Viola, Helwig Hauser, Odd-Helge
Gilja - Sonography of the small intestine. - World J. Gastroenterol. 2009; 15(11):1319-1330.
16. Ekbom A, Helmick C, Zack M, Adami H. - Increased risk of large-bowel cancer in Crohn's disease with colonic
involvement. - Lancet 1990; 336 (8711):357359.
17. Neuman MG, Nanau RM. - Inflammatory bowel disease: role of diet, microbiota, life style. - Transl. Res. 2012;
160(1):29-44.
18. Lambert B, Lemberg DA, Leach ST, Day AS. - Longer-term outcomes of nutritional management of Crohn's disease in
children. - Dig. Dis. Sci. 2012; 57(8):2171-2177.
19. Berni CR, Terrin G, Borrelli O, Romano MT, Manguso F, Coruzzo A, D'Armiento F, Romeo EF, Cucchiara S. - Short-
and long-term therapeutic efficacy of nutritional therapy and corticosteroids in paediatric Crohn's disease. - Dig. Liver
Dis. 2006; 38(6):381-387.
20. Cottone M, Renna S, Orlando A, Mocciaro F. - Medical management of Crohn's disease. - Expert Opin. Pharmacother.
2011; 12(16):2505-2525.
21. Dignass A, Van Assche G, Lindsay JO, et al. - European Crohn's and Colitis Organisation (ECCO). - The second
European evidence-based Consensus on the diagnosis and management of Crohn's disease: Current management. - J.
Crohns Colitis 2010; 4(1):28-62.
22. Kanazawa A, Yamana T, Okamoto K, Sahara R. - Risk factors for postoperative intra-abdominal septic complications
after bowel resection in patients with Crohn's disease. - Dis. Colon Rectum 2012; 55(9):957-962.
23. Ruiz P, Kato T, Tzakis A. Current status of transplantation of the small intestine. - Transplantation 2007; 83(1):1-6.
24. Middleton SJ, Jamieson NV. - The current status of small bowel transplantation in the UK and internationally - Gut
2005; 54(11):16501657.
25. Ptrascu Tr, Catrina E, Doran H, Mihalache O, Bug C, Degeratu D, Predescu G. - Implicatii chirurgicale ale
localizrilor intestinale n boala Crohn. - Chirurgia 2009; 104 (6):705-714.

139
5. Meckels Diverticulum Pathology
Meckels diverticulum is a true diverticulum of
the small intestine located on the antimesenteric
border of the ileum, at 50-100 cm away from the
ileocecal valve. (Figure 15) It appears with a
frequency of 2%, being more common in men.[1-3] It
represents the remnants of the proximal end of the
embryologic yolk stalk (the omphaloenteric, or
vitelline duct) which normally obliterates completely
by the 7
th
week of gestation.[3]
The diverticulum has a conical or cylindrical
shape, of 5-10 cm length and does not have its own
mesentery. It may be free in the peritoneal cavity or may be fixed to the umbilicus.
In the mucosa layer, heterotopic areas of gastric (60-85%), pancreatic (5-16%)
and rarely colic cells, may exist.[3-5]
Meckels diverticulum may be the site or the cause of many pathological
processes such as ulceration and bleeding, torsion with necrosis and perforation,
inflammation, obstruction with occlusion, tumors, and it can be involved in incarcerated
hernias (Littre hernia).[6,7]
The Meckel's rule of 2's: [2]
1. 2% of the population are born with a Meckel's diverticulum
2. Only 2% of those with a Meckel's manifest clinical problems
3. Usually located 2 feet proximal to the terminal ileum and the
diverticulum is usually 2 inches long
4. Symptoms commonly manifest at age 2 years
Meckel's diverticulitis
It is an inflammatory acute or subacute process very similar to acute appendicitis.
Pathological forms are identical: catarrhal, phlegmonous and gangrenous, with or
without perforation.
From clinical point of view, two aspects may draw the attention to a diverticulitis:
1. The pain located around the umbilicus associated with abdominal muscles
contraction, and
2. The occlusive symptoms, which appear more frequently than in appendicitis.
Preoperative diagnosis however is rare, patients being operated in most cases with
diagnosis of acute appendicitis. Therefore, whenever intraoperative appearance of the
appendix does not explain the clinical symptoms,
exploration of the last meter of the ileum is
recommended to find a possible Meckel's
diverticulitis.
Treatment is represented by diverticulectomy,
and resection of Meckels diverticulum should be
large enough to include the intestinal wall around the
base, or a segmental resection of the intestine
(enterectomy) may be applied. (Figure 16) In some
obese patients exploration through the right iliac fossa
incision (Mc Burney incision) is much difficult than
140
in laparoscopic approach.
Diverticulum ulcer
Ulceration of the diverticular mucosa is produced by acid or alkaline secretion
from the ectopic gastric or pancreatic cell islets.
The main manifestation is represented by bleeding but also perforation with
generalized peritonitis is possible. Symptoms are represented by intermittent pain
around the umbilicus associated with bleeding through the rectum as melena (black
stool) or even fresh blood in case of abundant bleeding. In perforation, symptoms of
general peritonitis are present.
In most cases, diagnosis is made during operation when laparotomy is performed
for serious gastrointestinal bleeding or peritonitis. For diagnosis of digestive bleeding,
gastroscopy and colonoscopy are usually performed first, but these will only rule out the
gastric or colonic cause of hemorrhage. A noninvasive method to diagnose the source of
bleeding might be a technetium-99m (99mTc) pertechnetate scan, also called Meckel's
scan which detects ectopic gastric mucosa.[4] This scan has 95% specificity and 85%
sensitivity. Angiography might identify briskly bleeding.[8]
Diverticular ulcer treatment is exclusively surgical and consists in
diverticulectomy.
Intestinal obstruction
Meckels diverticulum obstruction ranks second after bleeding in terms of
diverticular complications and represents about 5% of all causes of intestinal occlusions.
Mechanisms of occlusion are:
Luminal obstruction caused by tumors, enteroliths or foreign body
Diverticulum strangulation may appear if a very long diverticulum is wrapped
around the intestinal loop
Diverticulum volvulus, which can be only diverticular, or enterodiverticular
Invagination (intussusception) which is only diverticular at the beginning, but
as progression continues, it becomes ileoileal and then ileocolic
Incarceration, when a Meckels diverticulum is constricted in an inguinal
hernia, forming the Littr hernia
Clinical picture is dominated by abdominal pain and vomiting.
Treatment consists in diverticulectomy or segmental enterectomy.
References
1. Keljo DJ, Squires RH Jr. - Anatomy and Anomalies of the Small and Large Intestines. In: Feldman M, Scharschmidt BF,
Sleisenger MH, Klein S (eds). Sleisenger & Fordtran's Gastrointestinal and Liver Disease, 6th edition. 1998,
Philadelphia: W. B. Saunders Company, pp. 1426-1428.
2. Yamamoto LG. - Bowel Obstruction With Intra-Intestinal Sand. - Radiology Cases In Pediatric Emergency Medicine,
1996, Volume 5, Case 19 www.hawaii.edu/medicine/pediatrics/pemxray/v5c19.html
3. Merck Manual of Diagnosis and Therapy 18th edition - Robert S. Porter, Justin L. Kaplan, (Editors) - Merck Sharp &
Dohme Corp.2011, The Merck Manual Online -
http://www.merckmanuals.com/professional/gastrointestinal_disorders/diverticular_disease/meckels_diverticulum.html#
v894862
4. Martin JP, Connor PD, Charles K. - Meckel's diverticulum. - Am. Fam. Physician 2000; 61 (4):10371042.
5. Pariza G, Mavrodin CI, Ciurea M. - Complicated Meckel's diverticulum in adult pathology. - Chirurgia (Bucur) 2009;
104(6):745-748.
6. Sagar J, Kumar V, Shah DK. - Meckel's diverticulum: A systematic review. - Journal of the Royal Society of Medicine
2006; 99(10):501505.
7. Johnston AO, Moore T. - Complications of Meckel's diverticulum. - British Journal of Surgery Society Ltd., 1976;
63(6):453454.
8. Mattei P. - Fundamentals of Pediatric Surgery. - New York, NY: Springer Science+Business Media, LLC., 2011.
141
6. Tumors of the small intestine
Small bowel tumors (Figure 17) occur in patients over 40 years old, the benign
forms being 10 times more common than the malignant ones. Their common feature is
that they do not have any specific symptoms, only 1 of 10 patients being symptomatic,
aspect that delays much the diagnosis.
Intestinal tumors may be benign or malignant.

A. BENIG TUMORS
Etiology
The etiology is unknown. In some forms, there is a clear genetic determinism
(Peutz-Touraine-Jeghers and Gardner syndrome) but for the others there are many
theories, which partially explain the etiology (food theory of proliferative agents,
infectious theory, theory of biliopancreatic juice action, etc.).
Morphopathology
Benign tumors are represented by:
Adenoma is the most common benign tumor of the small intestine. It appears
under three pathological aspects: polypoid, insular and of Brunners glands.
Polypoid adenoma, whether tubular or villous type, can be unique or
multiple, located mainly in the proximal portion of the intestine. It may reach
or even exceed three cm in diameter. It can be pedunculated or sessile. The
risk of malignant transformation is lower than that of colic polyps. More
commonly villous polyps, especially sessile forms, larger than four cm, turn
malignant.[1] Insular adenoma develops from ectopic pancreatic tissue
islands, causing endocrine syndromes such as Zollinger-Ellison syndrome,
Werner-Morrison syndrome, hypoglycemic syndromes. Brunnerian adenoma
is the most frequent benign tumor present in the duodenum, located in the
proximity of the papilla. Its dimension is below one cm and is usually
asymptomatic.
Leiomyoma occurs at ages between 40 and 70 years. It develops either from
the muscularis mucosae or from the muscular layer of the intestine. The
tumor reaches sizes up to 2 cm, and can be located intraluminal, submucosal
or subserosal.
Fibroma develops from connective tissue. It may be pedunculated or sessile,
usually less than 2 cm in diameter and grows in submucosa layer.
142
Lipoma represents 8-20%. Rarely multiple, is located mainly in the distal
ileum or ileocecal valve. It may be associated with mesenteric or epiploic
lipomas (Odelberg).
Hamartoma appears as solitary or multiple polyps, in 10% of cases being
associated with Peutz-Touraine-Jeghers syndrome.
Benign neurogenic tumors represent 1% and can be schwannomas,
ganglioneurinomas or neurofibromas.
Hemangiomas represent 7% and can be teleangiectasia, capillary
hemangiomas and cavernous hemangiomas.[2,3]
Lymphangiomas represent 2% and can be simple or cavernous. It can be
manifested by hypoproteinemia in the absence of proteinuria and with
normal hepatic function, because of excessive loss of plasma protein into the
alimentary tract. The syndrome is encountered in small children and
infants.[4]
Clinical picture
Symptoms of benign tumors depend on their location, volume and their
development relative to the intestinal lumen. Endoluminal development of tumors
causes obstruction or intussusception. Intraparietal tumors produce intestinal stenosis
and tumors developed into the subserosa layer may produce intestinal volvulus. Colicky
pains of variable intensity, gastrointestinal bleeding and intestinal occlusion represent
the most frequent symptoms.
Physical examination reveals no changes unless tumors reach very large volume
becoming accessible to palpation as mobile tumors.
Workup
Radiological examination of the small intestine is difficult. Plain radiography
shows fluid-air levels when intestinal occlusion is present. Contrast radiologic
examination may highlight the site of intestinal obstruction or intussusception.
Enteroclysis is a fluoroscopic X-ray of the small intestine. The contrast material is
administered directly into the duodenum through a tube and images are taken in real
time as the contrast moves through the intestines.
Endoscopic examination has become more commonly used in exploring small
intestine. Double-balloon enteroscopy, also known as push-and-pull enteroscopy was
developed by Hironori Yamamoto in 2001.[5] It is the first endoscopic technique that
allows the entire gastrointestinal tract visualization in real time.[6]
Ultrasound examination is a harmless, cheap, portable, flexible investigation that
can reveal intestinal tumors. The method has limitations in obese patients and with
intestinal air impairing image quality. The small intestine is the most difficult part to
examine of the gastrointestinal tract because of its length and tortuous course.
Wireless capsule endoscopy is a relatively new noninvasive method of
examination, developed especially for the small intestine and obscure causes of
intestinal bleeding. One of the drawbacks of the method is that often is difficult to
discern the exact anatomic location of the lesion because the small bowel looks similar
through-out its length.[7-9]
CT scan is widely used for diagnosis with a specificity of 97%.
Laparoscopy and laparotomy are invasive methods of diagnosis.

143
Diagnosis
In many cases, diagnosis is difficult due to the lack of specificity of symptoms
and patients are operated for complications such as occlusion or gastrointestinal
bleeding.
Diagnosis may be suggested by the followings:
Periumbilical intermittent colicky pains associated with uncharacteristic
dyspeptic disorders
Recurrent subocclusive symptoms
Gastrointestinal bleeding of unclear etiology
Palpable abdominal tumor
In certain locations such as duodenum, the histopathological diagnosis is very
important and it will dictate the surgical attitude. Endoscopic biopsy should be
performed whenever is possible.
Differential diagnosis is made based on the dominant symptom (pain, obstruction,
hemorrhage and palpable abdominal tumor).
The evolution of these tumors is long and complications may be the first clinical
manifestation. Possible complications are intestinal occlusion (by obstruction,
intussusception, volvulus or tumoral stenosis), perforation with generalized peritonitis
and rarely spontaneous rupture of the tumors pedicle accompanied by bleeding.
Malignant degeneration is possible in some of these tumors.
Treatment
Treatment of benign tumors of the small intestine is exclusively surgical. Surgery
is represented in most cases by more or less extended intestinal resection. Surgical
attitude depends on tumor's location. Tumors located in duodenum raises problems of
surgical tactic. Some tumors in this location can be excised but others necessitate
cephalic pancreaticoduodenectomy. For tumors located in the lower part of the ileum,
right hemicolectomy may be required. Since, in most cases, at time of operation
histopathological diagnosis is unknown, the surgical attitude should be an aggressive
one.
Prognosis is excellent in almost all cases but it depends also on histopathological
features of the tumor.
B. MALIGNANT TUMORS
Malignant tumors of the small intestine represent only 2% of all gastrointestinal
cancers.[10-12] Malignant tumors may be classified as primary and secondary, and from
histological point of view as epithelial tumors (adenocarcinomas) and non-epithelial
tumors (sarcomas). (Table 1) There are around 40 different histological subtypes of
small intestinal cancers, the most common being adenocarcinoma, lymphoma, sarcoma
and carcinoid tumors.[13]
Adenocarcinomas represent 30%-50% and predominantly affect older patients
whereas sarcomas affect younger patients. The prognosis for malignant tumors of the
small intestine is poor for carcinomas (5 years relative survival less then 30%), better
for lymphomas and sarcomas, and the best for carcinoid tumors.[13]

144
Table 1 - WHO histological classification of malignant tumors of the small intestine
Primary tumors
Epithelial tumors
Intraepithelial neoplasia (dysplasia)
associated with chronic inflammatory
diseases
o Low-grade glandular
intraepithelial neoplasia
o High-grade glandular
intraepithelial neoplasia
Carcinoma
o Adenocarcinoma
o Mucinous adenocarcinoma
o Signet-ring cell carcinoma
o Small cell carcinoma
o Squamous cell carcinoma
o Adenosquamous carcinoma
o Medullary carcinoma
o Undifferentiated carcinoma
Carcinoid (well differentiated endocrine
neoplasm)
o Gastrin cell tumor, functioning
(gastrinoma) or non-functioning
o Somatostatin cell tumor
o EC-cell, serotonin-producing
neoplasm
o L-cell, glucagon-like peptide and
PP/PYY producing tumor
Mixed carcinoid-adenocarcinoma
Gangliocytic paraganglioma
Others
Non-epithelial tumors
Lipoma
Leiomyoma
Gastrointestinal stromal tumor
Leiomyosarcoma
Angiosarcoma
Kaposi sarcoma
Others
Malignant lymphomas
Immunoproliferative small intestinal
disease (includes -heavy chain
disease)
Western type B-cell lymphoma of
MALT
Mantle cell lymphoma
Diffuse large B-cell lymphoma
Burkitt lymphoma
Burkitt-like /atypical Burkitt-lymphoma
T-cell lymphoma
o enteropathy associated
o unspecified
Others
Secondary tumors

Adenocarcinomas
Adenocarcinomas are the most common malignant tumors of the small intestine
(30%-50%) localized with predilection in duodenum (54%) around the ampulla of
Vater, and more rarely in jejunum (28%) and ileum (18%).[14]
As with almost any cancer, etiology is unknown but there are some predisposing
factors or underlying conditions that increase the risk of adenocarcinomas such as
Crohn's disease, celiac disease, ulcerative colitis and familial adenomatous
polyposis.[15] It is not very clear if elevated body mass index (BMI), cigarette use and
alcohol consumption increase the risk of small bowel cancer.[16-19]
Clinical picture of small bowel adenocarcinoma is related to the size and location
of the tumor. Tumor located in jejunum and ileum, manifests with periumbilical colicky
pains accompanied by nausea, vomiting, weight loss, asthenia, and intermittent
obstructive episodes. Chronic bleeding manifests as iron-deficiency anemia, but
massive bleeding are rare. In large tumors a mobile palpable mass can be felt.
Complications may be represented by intestinal occlusion and perforation with
generalized peritonitis. In duodenal location, tumors can induce jaundice due to biliary
obstruction.
The diagnosis can be established by the same investigations presented at benign
tumors of the small intestine or by laparoscopy or laparotomy.
Adenocarcinoma develops from the Lieberkuhn glands and includes the entire
intestinal wall, accompanied by a fibrous reaction that gives its schirous aspect.
Carcinomas may be polypoid, infiltrating or stenosing. Duodenal carcinomas are usually
145
more circumscribed. Associated mesentery can be infiltrated by the tumoral process and
it contains the satellite lymphadenopathy (lymph node metastases), which sometimes
exceed the initial tumor size. With time, the tumor adheres to the neighboring organs.
The routes of tumoral spread are lymphatic, hematogenous and peritoneal.
TNM classification:
T - Primary Tumor
TX - Primary tumor cannot be assessed
T0 - No evidence of primary tumor
Tis - Carcinoma in situ
T1 - Tumor invades lamina propria or submucosa
T2 - Tumor invades muscularis propria
T3 - Tumor invades through muscularis propria into subserosa or into non-peritonealized
perimuscular tissue (mesentery or retroperitoneum) with extension 2 cm or less
T4 - Tumor perforates visceral peritoneum or directly invades other organs or structures
(includes other loops of small intestine, mesentery, or retroperitoneum more than 2 cm and
abdominal wall by way of serosa; for duodenum only, invasion of pancreas)
N - Regional Lymph Nodes
NX - Regional lymph nodes cannot be assessed
N0 - No regional lymph node metastasis
N1 - Regional lymph node metastasis
M - Distant Metastasis
MX - Distant metastasis cannot be assessed
M0 - No distant metastasis
M1 - Distant metastasis
Stage Grouping
Stage 0 -Tis N0 M0
Stage I - T1 N0 M0, T2 N0 M0
Stage II - N0 M0, T4 N0 M0
Stage III - Any T N1 M0
Stage IV - Any T Any N
Grading of small intestinal carcinomas: well, moderately and poorly
differentiated, or high- and low-grade.
Treatment is represented by surgery and adjunctive therapy. In most cases, a
curative resection is possible. In cases with extensive local disease or metastases to
multiple distant lymph nodes and/or to the liver, or in case of peritoneal carcinomatosis
patients are considered incurable. Surgery is represented by wide enterectomy
associated with regional lymphadenectomy in jejunal location and ileocolectomy in ileal
location of the tumor. In duodenal location a pancreaticoduodenectomy (Whipple
procedure) will be performed. Patients who are considered incurable may undergo
palliative resections or bypass procedures. Small bowel adenocarcinoma is generally
considered radioresistant.[14] For chemotherapy, 5-fluorouracil is the most common
agent used, either as a single agent or in combination with other agents.
The five-year survival rates for resectable lesions range between 40% and
60%.[11,14,20]
Lymphosarcomas
These tumors arise predominantly from the lymphatic structures of the terminal
ileum and frequently are lymphomas of mucosa-associated lymphoid tissue (MALT).
Lymphomas constitute a significant proportion (30-50%) of all malignant small
intestine tumors.[15] The majority of intestinal lymphomas involving the small bowel
are B-cell lymphomas of MALT type, including both low-grade and aggressive types.
The macroscopic aspect is that of a vegetant (fungating) tumor, multiple or infiltrative.
146
Symptoms are not specific, but aggressive tumors, such as T-cell lymphoma or
Burkitt lymphoma, may present as a large intra-abdominal mass or acutely with
intestinal perforation. The diarrhea is the result of steatorrhea, and a protein-losing
enteropathy can be seen. Peripheral edema, tetany and clubbing are observed in as many
as 50% of patients.[15]
Treatment of small bowel lymphoma is represented by surgery and adjunctive
radio-chemotherapy. At present, the best treatment is limited resection of the tumor,
followed by postoperative radiotherapy. The cure rate is approximately 75% for stage
IE patients, even for those with aggressive histological types.[21] Chemotherapy is
reserved for advanced-staged tumors.
Low-grade B-cell lymphomas have the best prognosis and T-cell lymphomas the
worst. Adverse prognostic features included perforation, high-grade histology, multiple
tumors and advanced stage.[22]
Sarcomas of the small intestine
Sarcomas are mesenchymal tumors and account for approximately 14% of
malignant small intestinal tumors having a more even distribution throughout the small
bowel compared to adenocarcinomas.
Clinical picture has nothing special.
There are many histopathological types.
1. GIST (gastrointestinal stromal tumors) - are rare tumors of the gastrointestinal
tract. The median age of diagnosis is approximately 60 years, with the annual incidence
estimated at 1020 cases per million inhabitants.[23-25] About 70% occur in stomach,
20% in small intestine and less than 10% in esophagus. These tumors start in special
cells found in the intestinal wall, called the interstitial cells of Cajal (ICCs), or in very
early cells that can develop into Cajal cells. These cells are part of the autonomic
nervous system, being sometimes called the "pacemakers" of the gastrointestinal tract.
GISTs are quite different in their treatment and prognosis from other gastrointestinal
tumors.
Smaller tumors are usually located in the muscular layer of the intestinal wall.
Large tumors grow, mainly outward, from the bowel wall. Metastases in lymph nodes
are uncommon (<10%) and thus imaging usually shows absence of lymph node
enlargement.[26] Clinical picture is represented by nonspecific abdominal pain,
subocclusive symptoms, gastrointestinal bleeding and distant metastases especially in
liver, omentum and peritoneum. Factors that correlate with malignancy are tumor size
over five cm, mitotic count > 5 per 50 HPF, dense cellularity, and mucosal invasion.
Nevertheless, all GIST tumors should be considered to have malignant potential.
For diagnosis PET, CT, and MRI scans are useful in differentiating
nonmetastasizing from malignant GISTs. Generally, the definitive diagnosis is
established by histopathological examination from a biopsy sample, or the resected
specimen. Small intestinal GISTs are in 95% positive for KIT (CD117) and usually for
CD34, and a subset (30-50%) are positive for smooth muscle actin.[15,26] Other
spindle cell neoplasm arising from the gastrointestinal tract including lipoma,
schwannoma, hemangioma, leiomyoma, and leiomyosarcoma are typically CD117-
negative.
Treatment is represented by surgical excision (enterectomy). Regional
lymphadenectomy is not necessary. The development of specific tyrosine kinase
inhibitors, such as imatinib mesylate, has led to a breakthrough in the treatment of
147
advanced GIST with a significant improvement in survival, with overall response rates
in excess of 80%.[23] It is foreseeable that more agents (Regorafenib, Masitinib,
Crenolanib, Motesanib, PTK787/ZK222584, mTOR inhibitors, Hsp90 inhibitors, etc.)
with novel mechanisms of action and targeting different pathways, will be studied for
GIST therapy.[27]
This type of tumors has a high tendency of recurrence.
2. Leiomyosarcomas are rare in the small intestine, and can be identified
immunohistochemically.
3. Angiosarcomas are represented by a proliferation of atypical endothelial cells
manifested especially by gastrointestinal bleedings. It has a poor prognosis (mean
survival time after diagnosis is 6 month).[28]
4. Kaposi sarcomas may involve small intestine, either the mucosa only or more
extensively. It is a common manifestation of the acquired immune deficiency syndrome
(AIDS). Most lesions are clinically silent but can produce a protein-losing
enteropathy.[29] For patients with single lesions, excisional biopsy often provides
adequate treatment. Simple excision is also appropriate for resectable recurrences.[30]
5. Liposarcomas are rare tumors of the small intestine.
Carcinoid tumors
Carcinoid tumors represent well-differentiated neoplasms of the diffuse endocrine
system. Other neuro-endocrine tumors of the small intestine are small cell carcinomas
(poorly differentiated endocrine neoplasms) and malignant large cell neuroendocrine
carcinomas. This tumor has endocrine activity secreting different kind of hormones
(serotonin, bradykinin, substance P and other vasoactive substances) which may
produce the so-called carcinoid syndrome manifested by flushing, diarrhea,
bronchoconstriction, and cardiac disease.
Carcinoid tumors account for some 35%-42% of neoplasms in the small intestine,
most of which occur in the ileum and rarely in the duodenum. The average annual
incidence rate is about one case per 100,000 inhabitants [13] but with an occurrence rate
of 1 case per 300 autopsies.[31]
Tumoral cells commonly spread to loco-regional lymph nodes, paraaortic lymph
nodes and to the liver. With distant spread, especially to the liver, carcinoid syndrome
can develop. Patients with the syndrome almost invariably have hepatic metastases.
Clinical picture is common to other small intestinal tumors manifested by
intermittent abdominal pains, and in more advanced cases, by intermittent intestinal
occlusion and a palpable abdominal mass. Most patients with carcinoid tumors do not
develop carcinoid syndrome. Only 40-50% of patients experience the syndrome.
In the presence of carcinoid syndrome, the diagnosis is easier. The main
investigation is represented by urinalysis which in patients with carcinoid syndrome,
shows high levels of urinary 5-Hydroxyindoleacetic acid (5-HIAA), usually more then
five times the normal values in a 24-hour.
Because carcinoid tumors are well vascularized, noncontrast CT scan is used to
highlight the tumor. In addition, OctreoScan may highlight the tumor. For this
examination, Ocreotide labeled with a radioactive isotope is injected. Because carcinoid
tumors have Somatostatin receptors, the radiolabeled Ocreotide binds to the tumor and
radiography thus shows the tumor.
148
Treatment is represented mainly by surgical removal of the tumor (segmental
resection and lymphadenectomy). Primary tumors should be resected, even in the
presence of distant metastases to prevent future intestinal obstruction. Early diagnosis
can potentially lead to a cure by surgical resection of the primary tumor. In addition,
liver metastases in patients with carcinoid syndrome should be resected, cauterized, or
ablated, because this usually results in a dramatic relief of symptoms. For inoperable
patients with symptomatic liver metastases another possibility is represented by hepatic
artery ligation or chemoembolization.
Pharmacological treatment is represented by Somatostatin analogs, which have
both anti-tumor effects as well as symptom palliation. Peptide-radio-receptor treatment
(PRRT) using radio-labeled peptides which binds to Somatostatin receptor is a useful
anti-tumor treatment.[32] Although symptomatic relief and stabilization of tumor growth
for various periods of time are observed in many patients treated with Somatostatin
analogs, tumor regression is rare. Currently, there is no other powerful antiproliferative
agent available for carcinoids.[33]
Postoperative long-term monitoring is required. This involves blood markers
every 6 months for the first 3 years and then yearly thereafter.
Prognosis of carcinoid tumors is the best among all small bowel cancers. The
relative 5-years survival rate is about 80%.[34]
References
1. Bremer EH, Battaile WG, Bulle PH. - Villous tumors of the upper gastrointestinal tract. Clinical review and report of a
case. - Am. J. Gastroenterol. 1968; 50(2):135-143.
2. Kim YS, Chun HJ, Jeen YT, et al. - Small bowel capillary hemangioma. - Gastrointest. Endosc. 2004; 60(4):599.
3. Ramanujam PS, Venkatesh KS, Bettinger L, et al. - Hemangioma of the small intestine: case report and literature review.
- Am. J. Gastroenterol. 1995; 90(11):2063-2064.
4. Walker-Smith JA, Reye RD, Soutter GB, Kenrick KG. - Small intestinal lymphangioma. - Arch. Dis. Child. 1969;
44(236):527532.
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1918.

Surgical Pathology of the Large Bowel
SURGICAL PATHOLOGY OF THE LARGE BOWEL

1. Surgical anatomy
2. Investigation methods of the colon
3. Megacolon
4. Colonic polyps
5. Colon cancer

1. Surgical Anatomy
The colon, or the large bowel, extends between the ileocecal valve of Bauhin, and
the rectum, describing a frame around the small intestines. Its length is about 1.6-1.7 m,
with large variations from individual to individual, and it has a capacity of 2-3 L.
It has a minor role in digestion and absorption but a more important role in
evacuation of waste.
The wall of the colon is much thinner than that of the small bowel and its arterial
supply is also poorer, aspects that make colon surgery more difficult, raising special
problems of surgical tactic and technique. Surgery of the colon is dependant of its
vascularization. The anastomotic risk is higher than for other segments of the digestive
tract.
Another important aspect is that the content of the colon is very septic, reason
why a good preoperative preparation is necessary in most cases. Septic risk is much
higher in colon surgery.
From anatomical point of view, the colon is divided into four segments: (Figure
1A)
1. Ascending colon (cecum, and ascending colon)
2. Transverse colon (hepatic flexure, transverse colon and lienal flexure)
3. Descending colon
4. Sigmoid colon
From surgical point of view, there are only two segments: the right colon and the
left colon. (Figure 1B)

151
This segmentation is also in accordance with the embryology, physiology,
vascularization and innervation of the colon. A functional sphincter, so called Cannon
Boehm sphincter, which may be observed on some contrast enemas, separates these two
segments.
The right colon extends from ileocecal valve until the 2/3 portion of the
transverse colon.
The transverse and sigmoid colon are
intraperitoneal organs, being completely covered
by visceral peritoneum. Both segments have
mesocolon called transverse mesocolon and
sigmoid mesocolon (mesosigma) and are the best
vascularized segments of the colon. (Figure 2)
They are very mobile and sometimes have an
appreciable length. These are important features
from clinical and surgical points of view. Due to
excessive mobility, tumors located on these segments can penetrate other anatomical
structures located far from the normal anatomical position of the colon. For example,
there are cases when tumors of the transverse colon penetrate the urinary bladder or the
uterus (colon ptosis). Excessive mobility may cause pathological processes such as
sigmoid volvulus. Furthermore, the length of these segments allows resection of a large
portion of the colon and tension free anastomosis. Having a better vascularization and
being covered by peritoneum, offer a good material
for anastomosis with lower risk of anastomotic
leak.
The ascending and descending colon are
secondary retroperitoneal organs, wrapped by
peritoneum only on the anterior surface due to the
coalescence process leading to formation of Toldt I
and Toldt II fascia, thus explaining the easy
extension of cancer on the posterior wall and rapid
penetration into surrounding tissues.(Figure 3)
In some cases, the cecum has an excessive
mobility that could lead to volvulus.
The right colon is more superficial and has a much larger caliber than the left
colon, fact that allows the development to larger dimensions of tumors in the absence of
occlusive symptoms. The left colons lumen is not so large, and tumors cannot grow to
large dimensions because they rapidly induce stenosis and obstruction. The left angle of
the colon is the highest and deepest located segment of colon being the most difficult to
explore.
External appearance of the colon is characteristic being different from that of the
small intestine. The haustra of the colon are small pouches caused by sacculation, which
give the colon its segmented appearance. The taenia coli run the length of the large
intestine. Because the taenia coli are shorter than the intestine, the colon becomes
sacculated between the taenia, forming the haustra.
The colic wall structure is composed of the same existing layers throughout the
digestive tract (mucosa, submucosa, muscular, subserosa and serosa or adventitia) but
with different features and organization. The outer muscular layer consists of
longitudinal fibers concentrated in three bands called taenia coli (mesocolic, free and
omental).
152
Arterial supply (Figure 4)
There are two main arterial sources: the
superior mesenteric artery (SMA) for the right
colon and the inferior mesenteric artery (IMA) for
the left colon.
Branches of the SMA are:
1. Ileocolic artery
2. Right colic artery
3. Middle colic artery
Each of these arteries divides into two
branches (ascending and descending) which join
together to form a marginal arch described by
Riolan at a distance of 5-7 mm from the colon
border. From this arch, emerge the vasa recta,
which also divide into anterior and posterior
branches.
Branches of the IMA are:
1. Left colic artery
2. Three sigmoid arteries, which join
together to for more consecutive
arches
3. Superior rectal artery
Venous drainage (Figure 5)
For the right colon, homonymous veins
along the arteries collect the venous blood and
then via the superior mesenteric vein (SMV) it
flows into the portal vein toward the liver.
Venous drainage of the left colon is
tributary to the inferior mesenteric vein (IMV)
through the homonymous veins. The IMV flows
into the splenic vein under the Treitz angle of the
duodenum and behind the pancreas.
Through the venous system, tumoral cells
metastasize (spread) to the liver and that is why
they are the first vascular anatomical elements
ligated and resected during radical oncological
operations.
Lymphatic drainage (Figure 6)
For the right colon, lymphatic drainage
starts from the lymphatic parietal network and
goes through four lymph nodes stations: epicolic
lymph nodes (located on the wall of the colon),
pericolic lymph nodes (along the marginal
vascular arch), intermediate lymph nodes (along the colic arteries and veins) and
superior mesenteric lymph nodes (at the origin of SMA).
153
For the left colon, lymphatic drainage follows the same four stations: epicolic,
pericolic, intermediate and central (at the root of the IMA).
Lymphatic drainage from the entire colon and proximal two-thirds of the rectum
goes to the paraaortic lymph nodes, which then drain, into the cisterna chyli.
Innervation
The right colon innervation is vegetative and sensitive. Autonomous innervation
is ensured by sympathetic efferent fibers originating in T5-T12 spinal nerves, and
parasympathetic from vagus nerves fibers. Sensitive innervation is ensured by fibers
coming from the T10-T11 spinal ganglia. Sympathetic innervation of the left colon
originates in the superior hypogastric and lumbar sympathetic plexuses.
Parasympathetic fibers originate in the sacral parasympathetic nerves.
Anatomical relations of the colon (Figure 7) Knowledge about anatomical relations of
the colon are very important giving the possibility to explain possible complications and
symptoms when colon tumor penetrates the adjacent organs. Furthermore, these
relations must be known by surgeon in order to avoid damages to the neighboring
anatomical structures during various interventions on the colon.
Thus, the most important relations of the right colon are the posterior relations
with the right ureter and duodenum. When suspected, tumoral invasion of these organs
must be verified preoperatively by different kind of investigations such as intravenous
urography, upper gastrointestinal endoscopy, computed tomography, etc. Other
anatomical structures that have relations with the posterior wall of the right colon are
the right kidney, right testicular or ovarian vessels, iliohypogastric, ilioinghinal and
lateral femoral cutaneous nerves. The hepatic flexure of the colon comes in direct
contact with the liver and right adrenal gland. The cecum has posterior relations with
external iliac vessels. The entire right colon comes in contact with small intestines,
greater omentum and abdominal wall which could be invaded by the tumoral process.
Transverse colon has posterior relations with small intestines, pancreas,
duodenum, and stomach. In case of a very long colic loop, transverse colon may reach
down into the pelvis (colon ptosis) and thus tumors can invade organs such as bladder,
uterus and annexes or even the sigmoid colon. Most frequently, the stomach and
pancreas are invaded by tumoral process located on this colic segment.
The left colon, through its splenic flexure has close relations with the left kidney,
pancreatic tail, spleen, and stomach. Descending colon has posterior relations with the
left ureter, spermatic or ovarian vessels, and left iliohypogastric, ilioinghinal and lateral
femoral cutaneous nerves. Sigmoid colon due to its mobility conferred by mesosigma
comes in contact with pelvic
organs (uterus, appendix,
bladder). The left colon has
also relations with small
intestine, the greater
omentum and abdominal
wall.
All these organs or
anatomical structures can be
invaded by tumors located
on colon, in many cases
requiring complex surgical
procedures to remove
154
affected structures along with a part of the colon in order to achieve oncological
radicality.
Physiology
The large intestine is mainly responsible for storing waste, absorbing water,
maintaining the water, minerals and vitamins (vitamin K) balance. It contains nearly 60
varieties of microflora, bacteria (predominantly anaerobes) which have a role in
fermentation, production of some nutrients, acid-base balance, and to prevent
proliferation of harmful bacteria. The right colon is functionally the colon of
fermentation, with role of water absorption, while the left colon serves mainly to store
and evacuate feces.
The entire colon is crossed by food in 20 hours. The swallowed barium reaches
the cecum in 3-6 hours after ingestion, the right colic flexure after 7-8 hours, the left
colic flexure after 9-12 hours, the sigmoid after16-20 hours and rectal ampulla between
24-48 hours.
2. Investigation Methods of the Colon
Most investigating procedures for colon are invasive and encumbered by certain
risks (1.9% complications), especially colon perforation (1/800 colonoscopies).[1,2]
The vast majority of these can be performed in ambulatory conditions.
Endoscopic investigations have become the main methods of diagnosis in colic
pathology. Unlike other imaging methods, they provide direct visualization of lesions
and allow the collection of tissue fragments for diagnostic purposes. In nowadays,
endoscopy is also the main approach for removal of colon polyps. Endoscopic
examination (as well radiological) implies a completely emptied colon. Endoscopic
methods are represented by rectosigmoidoscopy and colonoscopy (pancolonoscopy).
Rectosigmoidoscopy is a limited colonoscopy which investigates the colon up to
about 60-80 cm from the anus, and can be performed on an outpatient basis (does not
require hospitalization). It takes about 6-10 minutes. The examination is often sufficient
for screening purposes, given the fact that the vast majority of colic pathology is located
on the left colon.
Colonoscopy is a more laborious procedure (it takes 15-30 minutes) and the
endoscope progresses until the cecum. It can be performed in hospitalized patients but
also on an outpatient basis. Frequently the examination is performed in intravenous
general anesthesia. Iatrogenic colonic perforation is a serious but rare (0.12%)
complication of colonoscopy.[3]
Barium enema or irigoscopy, (Figure 8) is the fundamental radiological method
for investigating colon diseases. Double contrast barium enema (air is insufflated after
evacuation of barium) is a fine examination that can identify changes on the inner
surface of the colon.
Virtual colonoscopy or CT colonoscopy (Figure 9) is a three-dimensional
reconstruction of a computed tomography of the colon using special software. At the
beginning of the procedure, the colon is filled with carbon dioxide or air with a tube
introduced into the rectum.
155

Compared to barium enema, CT colonoscopy is more accurate, shorter in time,
with fewer complications, less radiation exposure, preferred by patients, and reveals
extracolonic lesions in 5% to 10% of cases.[4] It is faster then conventional colonoscopy
(10-15 minutes) and does not need sedation of patient.[5] The main drawback, compared
to endoscopic procedures, is that it cannot perform biopsies and also it is almost 10
percent less successful in detecting polyps than colonoscopy. It is possible that polyps
smaller than 10 mm in diameter to be missed by this method.
Other investigations are performed especially for liver metastases and tumor
relations with the surrounding organs.
Abdominal ultrasound reveals liver metastases and ascites, but also some colic
tumors can be observed. Intraoperative ultrasound is used especially for detecting liver
metastases.
Simple or contrast CT or MRI
scans are especially useful in advanced
forms of colic tumors and may reveal
penetration of neighboring structures.
(Figure 10)
PET scan and PET/CT are used
for detection of distant metastases.
Urography and cystoscopy are
indicated when there is a suspicion of
tumoral penetration into the uretres or urinary bladder.
Colon capsule endoscopy (CCE) represents a noninvasive technology that allows
visualization of the colon without requiring sedation, insufflation, or radiation.[6] The
colon wireless capsule measures 31 mm x 11 mm. It has dual cameras that enable it to
take pictures from both ends.[7] A second-generation of colon capsule endoscopy
system (PillCam Colon 2) (CCE-2) was developed to increase sensitivity for colorectal
polyps detection. The procedure might be a valid alternative to conventional
colonoscopy in selected cases such as patients refusing conventional colonoscopy or
with contraindications to colonoscopy, or when colonoscopy is incomplete.[8]
References
1. Kavic SM, Basson MD. - Complications of endoscopy. - Am. J. Surg. 2001; 181:319-332.
2. Gatto NM, Frucht H, Sundararajan V, Jacobson JS, Grann VR, Neugut AI. - Risk of perforation after colonoscopy and
sigmoidoscopy: A population-based study. - J. Natl. Cancer. Inst. 2003; 9(5):230236.
3. Lning TH, Keemers-Gels ME, Barendregt WB, Tan AC, Rosman C. - Colonoscopic perforations: a review of 30,366
patients. - Surg. Endosc. 2007; 21(6):994-997.
156
4. Stevenson G. - Colon imaging in radiology departments in 2008: goodbye to the routine double contrast barium enema. -
Can .Assoc. Radiol. J. 2008; 59(4):174-182.
5. Virtual Colonoscopy - Mayo Clinic. "Virtual colonoscopy is typically faster than traditional colonoscopy. A scan of your
colon takes about 10 minutes. Expect the entire virtual colonoscopy procedure to take 20 to 30 minutes."
http://en.wikipedia.org/wiki/Virtual_colonoscopy
6. Spada C, Hassan C, Munoz-Navas M, Neuhaus H. et al. - Second-generation colon capsule endoscopy compared with
colonoscopy. - Gastrointest. Endosc. 2011;7 4(3):581-589.
7. Spada C, Riccioni ME, Hassan C, Costamagna G. - PillCam capsule endoscopy for the diagnosis of colonic diseases. -
Recenti Prog. Med. 2010; 101(6):227-231.
8. Riccioni ME, Urgesi R, Cianci R, Bizzotto A, Spada C, Costamagna G. - Colon capsule endoscopy: Advantages,
limitations and expectations. Which novelties? - World J. Gastrointest. Endosc. 2012; 4(4):99-107.

157
3. Megacolon
Megacolon represents an abnormal dilatation of the colon often accompanied by a
paralysis of the peristaltic movements of the bowel. Dolichocolon represents an
abnormal length of the colon. The combination of these two conditions is called
megadolichocolon (abnormal long and dilated colon).
Classification
According to its etiology, megacolon can be classified as:
Congenital
Idiopathic
Acquired
Toxic
Symptomatic
Congenital megacolon
The disease is also known as aganglionic megacolon or Hirschsprung's disease.
The disease is named after Harald Hirschsprung, a Danish physician who first described
two infants who died of this disorder in 1888.[1]
Etiopathogenesis: it is a congenital disorder of the colon in which ganglion cells of the
myenteric or Auerbach's plexus, of the terminal sigmoid and rectum walls are absent.
The length of bowel that is aganglionic varies. In most cases the distal part is affected
(the short-segment form) but there are cases when aganglionosis extends proximal to the
sigmoid.[2] Total colonic and total intestinal aganglionosis also occur. The macroscopic
aspect reveals a narrowing of the affected colon and distension of the superjacent,
containing giant fecaloma. Histological examination reveals the absence of the
intramural ganglionic plexus.
Hirschsprung's disease is frequently associated with other malformations or
genetic mutations such as Down syndrome in 2-15%.[3-5]
Epidemiology
Hirschsprung's disease occurs in approximately 1% of 5000 live births being
more frequently encountered in males and often has a familial character.[5,6]
Symptoms
Symptoms appear few days after birth and go through several successive periods:
In neonatal period, the newborn fails to pass meconium within 24-48 hours
after birth;[7] the abdomen becomes distended associated with pain and
postprandial vomiting, which, are signs of low intestinal incomplete or
chronic obstruction.
In infant period, the child has a big belly giving the grotesque appearance,
and also the same complaints as above, especially chronic constipation since
birth, are present.
In the period of childhood and adolescence, constipation and abdominal
distension are persisting; defecation is possible only with enema (1 stool at
5-10 days).
On rectal digital examination, the rectal ampulla is found empty! This aspect is
important for differential diagnosis.
158
Diagnosis is based on clinical picture, with constipation and important abdominal
distension, plain abdominal radiography, that shows important and permanent
pneumatic distension of the entire colon, (Figure 11) barium enema that shows a
narrowed segment of the colon and superjacent massive distension, (Figure 12) and
sigmoidoscopy with biopsy: histopathology reveals the aganglionic tissue.[8] For a
correct technique, a full-thickness rectal biopsy is required.

Evolution: untreated the mortality rate in the neonatal period is about 60-70%. Those
who survive have a less serious evolution.
The general condition is influenced and may take two clinical forms: of chronic
toxemia (toxins from the colon enter the vascular system) and a permanent restrictive
type of respiratory insufficiency. The appearance is that of an underweight child, always
tired, pale, inert, with tachypnea and polypnea, and in advanced stages even cachectic.
Rarely acute occlusive episodes appear. Colonic perforation is also a rare (2%)
complication.[5] Enterocolitis may appear in 10%-26% of cases manifested by diarrhea,
being the main source of mortality and morbidity in Hirschsprung's disease.[5,9-11]
Treatment is purely surgical. If occlusion does not appear, operation is not indicated
under the age of one year. Instead, diet is recommended to ensure the intestinal transit
and also small repeated enemas.
Surgical treatment consists of resection of the abnormal segment of the colon and
restoration of normal transit. Usually the first step is a diverting loop colostomy above
the affected zone, or diverting loop ileostomy, procedures that allow the colon
emptying. After a period, when the child is in a better condition, colon resection
followed by different types of anastomosis can be performed. Contraindications to a
one-stage procedure include:[7]
massively dilated proximal bowel
severe enterocolitis
perforation
malnutrition
inability to accurately determine the transition zone by frozen section

159
Types of operations:
Swensons operation, resects the aganglionic segment of the colon and rectum
followed by a pull-through technique (the anal sphincter is preserved and the normal
colon is pulled through the anal canal). The anastomosis is a coloanal type.
Duhamel operation does not remove the rectum. After resection, the normal colon
is pulled through the posterior wall of the rectum (in the presacral space) and through
the anus. It preserves a portion of the rectum for its normal function in defecation
process.
Transanal Soaves procedure consists in removing the mucosa and submucosa of
the rectum and pulling the ganglionic bowel through the aganglionic muscular cuff of
the rectum.
Transanal endorectal pull-through procedure represents the latest development in
the concept of the minimally invasive surgery for Hirschsprungs disease. De la Torre-
Mondragon and Ortega-Salgado described the procedure in 1981.[12] An endorectal
mucosectomy is performed starting at 0.5 cm proximal to the dentate lines and extends
into the intraperitoneal rectum. The muscular sleeve is divided circumferentially at 3 to
4 cm proximal to the dentate line, exposing the intraperitoneal rectum and allowing full-
thickness mobilization of the rectosigmoid colon out of the anus. Aganglionic colon
segment is resected, and the normal colon is pulled down to anastomose with the distal
end of anorectal mucosa.[13]
Georgeson first described the laparoscopic approach for the surgical treatment of
Hirschsprung's disease in 1999.[14]
For patients with long-segment Hirschsprung's disease, surgical procedures that
bypass the aganglionic colon are used yet preserving the absorptive surface area and
allow for proper growth and nutritional support.
Recently, the usefulness of stem cells to regenerate the enteric nervous system
into the aganglionic intestine is being actively investigated.[15-17]
Idiopathic megacolon
It appears in children aged between three and seven years and causes are
unknown. Symptoms are represented by chronic constipation, moderate abdominal
distension. On rectal examination, the rectal ampulla is filled with feces. Barium enema
shows a distension of the entire colon without narrowing of any segment. Histology
reveals the presence of normal ganglia cells of myenteric plexus.[18,19] In the absence of
occlusion, the treatment is strictly conservative with an adequate diet rich in fibers,
small enemas, laxatives and psychotherapy.
Acquired megacolon
In Central and South America, infection with trypanosoma cruzi (Chagas disease)
can lead in one-third of cases to the destruction of digestive tract ganglia cells producing
a clinical picture of a congenital megacolon installed in adulthood. Destruction of
autonomic system cells may eventually result in megaesophagus, megacolon and
accelerated dilated cardiomyopathy.[20] The parasite can be detected by microscopic
examination of fresh anticoagulated blood. In the early, acute stage, symptoms are mild
and usually produce no more than local swelling at the site of infection. The initial acute
phase is responsive to antiparasitic treatments, with 6090% cure rates. After 48
weeks, individuals with active infections enter the chronic phase of Chagas disease that
160
is asymptomatic for 6080% of chronically infected individuals through their
lifetime.[21,22] Antiparasitic treatment is most effective early in the course of infection.
Other conditions may induce megacolon in adults such as schizophrenia, cerebral
atrophy, spinal injury, Parkinson disease, myxedema, amyloidosis, scleroderma, some
drugs, etc. Rectal examination reveals the rectal ampulla filled with feces. Treatment
addresses the underlying disease, with the careful use of enemas and laxatives or
purgatives.
Toxic megacolon
Toxic megacolon is a potentially lethal condition and means a nonobstructive
colonic dilatation larger than 6 cm and signs of systemic toxicity (fever, tachycardia,
leukocytosis, dehydration, hypotension, confusion).[23]
Toxic dilatation of the colon may occur in Crohn's disease but is more common in
ulcerative colitis. This condition is considered a severe form of ulcerative colitis
presenting additional colonic distension, due to severe inflammation.[23] Although
recognized as a complication of ulcerative colitis, toxic megacolon may occur in other
types of colitis, volvulus, diverticulitis and colon cancer. Risk factors in the
development of severe colitis include infection with C. difficile, malignancy,
immunosuppression, chemotherapy, renal failure, radiation colitis, etc.
Rapid distension of the colon may cause the following symptoms: painful
abdominal distension, fever, dehydration, tachycardia and cardiovascular shock.
Perforation with subsequent peritonitis is the most frequent complication.
Treatment addresses the underlying disease. It consists of reducing colonic
distension to decrease the risk of perforation, correcting dehydration through
administration of fluids and electrolyte solutions and treating toxemia and risk factors.
The mortality rate for toxic megacolon decreased in the past few decades from 20% to
4% due to early recognition and diagnosis, improved general and surgical treatment. If
perforation occurs, the mortality rate is approximately 20%.[25]
Symptomatic megacolon
It is secondary to a chronic organic barrier, represented by some incomplete
stenosis of the anal canal or rectum. Treatment is purely surgical, consisting of
removing the obstacle.

Dolichocolon is another pathological entity of the colon different from
megacolon, represented by a very long colon, which is not enlarged. This condition
affects especially the mobile segments of the colon: the transverse and sigmoid colon.
Chirays typical triad dominates the clinical picture and is represented by constipation,
abdominal distension and pain. Sometimes, the long and mobile colic segment may
become volvulated producing intestinal occlusion. Diagnosis relies on barium enema
that shows a long colic loop and a delay in evacuation of barium (after 2-5 days).
Nonsurgical treatment is common in these cases (proper diet, laxatives
/purgatives). In case of occlusion (volvulus), surgery is required (segmental colectomy).

161
References
1. John Ruhrh - Harald Hirschsprung 1830-1916. - A note on the history of hypertrophy of the colon. - Arch. Pediatr.
Adolesc. Med. 1935; 50(2):472-475.
2. Amiel J, Sproat-Emison E, Garcia-Barceo M, Lantieri F, Burzynski G, Borrego S, Pelet A, Arnold S, Miao X, Griseri P,
Brooks AS, Antinolo G. et al. - Hirschsprung disease: associated syndromes and genetics: a review. - J. Med. Genet.
2008; 45:1-14.
3. McKusick-Nathans Institute of Genetic Medicine - Hirschsprung and Down Syndrome - Johns Hopkins Medicine -
http://www.hopkinsmedicine.org/geneticmedicine/Clinical_Resources/Hirschsprung/Downsyndrome.html
4. Victor J. Bishop - Down Syndrome and Hirschsprung Disease -
http://www.riverbendds.org/index.htm?page=hirschin.html
5. Menezes M, Corbally M, Puri P. - Long-term results of bowel function after treatment for Hirschsprung's disease: a 29-
year review. - Pediatr. Surg. Int. 2006; 22(12):987-990.
6. Fiorino K, Liacouras CA. - Congenital aganglionic megacolon (Hirschsprung disease). In: Kliegman RM, Behrman RE,
Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap
324.3.
7. Steven L Lee, Shant Shekherdimian, Jeffrey J DuBois - Hirschsprung Disease - Medscape reference, Emedicine -
8. Kessmann J. - Hirschsprung's Disease: Diagnosis and Management. - Am. Fam. Phys. 2006; 74:1319-1322.
9. Pini Prato A, Rossi V, Avanzini S, Mattioli G, Disma N, Jasonni V. - Hirschsprung's disease: what about mortality? -
Pediatr. Surg. Int. 2011; 27(5):473-478.
10. Sariolu A, Tanyel FC, Bykpamuku N, Hisnmez A. - Clinical risk factors of Hirschsprung-associated enterocolitis.
I: Preoperative enterocolitis. - Turk. J. Pediatr. 1997; 39(1):81-89.
11. Teitelbaum DH, Qualman SJ, Caniano DA. - Hirschsprung's disease. Identification of risk factors for enterocolitis. -
Ann. Surg. 1988; 207(3):240-244.
12. De la Torre-Mondragon L, Ortega-Salgado JA. - Transanal endorectal pull-through for Hirschsprungs disease. - J.
Pediatr. Surg. 1998; 33:1283-1286.
13. Teeraratkul S. - Transanal one-stage endorectal pull-through for Hirschsprung's disease in infants and children. - J.
Pediatr. Surg. 2003; 38(2):184-187.
14. Georgeson KE, Cohen RD, Hebra A, et al. - Primary laparoscopic-assisted endorectal colon pull-through for
Hirschsprung's disease: a new gold standard. - Ann. Surg. 1999; 229(5):678-82; discussion 682-3.
15. Almond S, Lindley RM, Kenny SE, Connell MG, Edgar DH. - Characterisation and transplantation of enteric nervous
system progenitor cells. - Gut. 2007; 56(4):489-496.
16. Metzger M, Caldwell C, Barlow AJ, Burns AJ, Thapar N. - Enteric nervous system stem cells derived from human gut
mucosa for the treatment of aganglionic gut disorders. - Gastroenterology, 2009; 136(7):2214-2225
17. Thapar N. - New frontiers in the treatment of Hirschsprung disease. - J. Pediatr. Gastroenterol. Nutr. 2009; 48 Suppl
2:S92-4. Review.
18. Gattuso J, Kamm M, Talbot I. - Pathology of idiopathic megarectum and megacolon. - Gut. 1997; 41(2):252257.
19. Meier-Ruge WA, Mller-Lobeck H, Stoss F, Bruder E.- The pathogenesis of idiopathic megacolon - Eur. J
Gastroenterol. Hepatol. 2006; 18(11):1209-1215.
20. Louis V Kirchhoff - Chagas Disease (American Trypanosomiasis). - Medscape reference, Emedicine.
21. Bern C, Montgomery SP, Herwaldt BL, et al. - Evaluation and treatment of chagas disease in the United States: a
systematic review. - JAMA, 2007; 298 (18):21712181.
22. Rassi A, Rassi A, Marin-Neto JA. - Chagas disease. - Lancet, 2010; 375 (9723):13881402.
23. Jalan KN, Sircus W, Card WI, et al. - An experience of ulcerative colitis. I. Toxic dilation in 55 cases.- Gastroenterology
1969; 57(1):68-82.
24. Moulin V, Dellon P, Laurent O, Aubry S, Lubrano J, Delabrousse E. - Toxic megacolon in patients with severe acute
colitis: computed tomographic features. - Clin. Imaging 2011; 35(6):431-436.
25. Deepika Devuni - Toxic Megacolon - Emedicine Medscape, Updated: Aug 3,
2012(http://emedicine.medscape.com/article/181054-overview#aw2aab6b2b4)

162
4. Colonic Polyps
Polyps are tumors that may occur on the inner surface of the entire
gastrointestinal tract, mostly in the colon and rectum and have a potential of malignant
transformation. The word derives from the Latin "polypus" which means "multiple
legs". (Figure 13)
Polyps may be of different sizes and shapes (sessile or
pedunculated) (Figure 14); they are congenital or acquired,
benign or malignant, symptomatic or asymptomatic, singular
localized or multiple, spread throughout the entire colon.
Colonic polyps can occur as part of inherited polyposis
syndromes. In this case, their number is greater and the risk
for malignant transformation is much greater than with
isolated colonic polyps. Polyposis syndromes are hereditary
conditions that include familial adenomatous polyposis
(FAP), hereditary nonpolyposis colorectal cancer
(HNPCC)/Lynch syndrome, Gardner syndrome, Turcot
syndrome, Peutz-Jeghers syndrome, Cowden disease,
familial juvenile polyposis, and hyperplastic polyposis.[1]
These syndromes should be taken into account when
polyps are detected in young patients, in case of two or more
polyps detected, when colic cancer is detected in young
patients (under the age of 40) and when there are
extraintestinal manifestations associated to polyps. In these patients, even genetic
counseling is important, considering the hereditary transmission, and the high
possibility of malignant transformation.[2,3]
Epidemiology
Contradictory statistics for the incidence of polyps have been reported. Autopsy
studies performed in US suggest that about 30% of middle-aged or elderly individuals
have colonic polyps.[1] Other studies reported an incidence of 51%-69%, and that the
incidence increases with advancing years up to 88% in centenarians.[4,5] Estimated
prevalence of asymptomatic polyps in the general population ranges from 1.6% to12%,
while in population over 70 years, may reach 40%.[6-9] Because the highly prevalence
with increasing age, they confer an important predisposition to colon cancer.
The risk for cancer development depends on the size of the polyp, villous
histology, and its association with polyposis syndromes. In FAP, cancer inevitably
develops 10-20 years after the initial appearance of colonic polyps.[1]
Males seem to have a moderately higher incidence of polyps compared to
females.[10]
Classification. There are two main types of polyps from histopathological point of
view: non-neoplastic and neoplastic polyps. The most important difference between
these two groups is that the non-neoplastic polyps rarely turn malignant where as
neoplastic polyps are considered the precursors of colon cancer.
1. Non-neoplastic polyps
Hyperplastic polyps
Submucosal polyps represented by benign lymphoid polyps
Inflammatory polyps
Hamartomatous polyps
163
2. Neoplastic polyps
Adenomatous polyps
Carcinoid
Polyps of the connective tissue (lipomas, leiomyomas, hemangiomas)
Polyps can be:
Unique (solitary polyp)
Multiple (200-100)
In a diffuse polyposis (> 100)
Endoscopic classification
1. Sessile polyps - The polyps have a large base of implantation on the colic
wall.
2. Pedunculated polyps - The polyps have a more or less long stalk by
which they are attached to the colic inner surface. These polyps are very
mobile. They can be easily removed by endoscopic procedure.
3. Flat polyps - The height of the polyp is less than one-half its diameter.
4. Depressed polyps - these are particular lesions, which appear to harbor
high-grade dysplasia or be malignant even if small.
Features of histopathological types
Non-neoplastic polyps
1. Hyperplastic, is the most common histological form (about 90% of all
epithelial polyps). There is no dysplasia. There is an increase in number of
cells leading to enlargement of the tissue. Polyps are located mainly in the
recto-sigmoid being less then 0.5 cm in diameter, of nipple-like shape. Pure
hyperplastic polyps have no malignant potential. They are usually
asymptomatic and are discovered incidentally during endoscopy. Treatment
after biopsy confirmation may be endoscopic excision or follow-up.
2. Benign lymphoid polyps are often considered as inflammatory polyps, but
some authors consider them congenital malformations or hamartomas, since
these polyps are sometimes associated with familial polyposis.[11-13] Most
of them are sessile being encountered more commonly in men in the third or
fourth decade of life. The size varies from a few millimeters to 3 cm in
diameter. Microscopically are composed of relatively normal lymphoid
tissue.[14] May be asymptomatic or may present common symptoms of
polyps: bleeding, abdominal pain, intussusceptions. The main feature of this
type of polyp on double contrast radiograms is the presence of a "spot" in the
center of the polyp. Frequently regress spontaneously, but their removal is
recommended for diagnosis. Do not turn malignant.
3. Inflammatory polyps (pseudopolyps) occur because of regenerating
processes of ulcerated colic mucosa. They do not have malignant potential.
4. Hamartomatous polyps (term introduced by Albrecht in 1904) are
frequently found in congenital diseases such as Peutz-Jeghers syndrome,
Cronkhite-Canada syndrome, and Cowden disease. These polyps contain a
mixture of tissues. Even though these polyps themselves have no malignant
potential, patients with the syndrome have an increased risk of developing
carcinomas. Polyps do not have a tendency to excessive growth and after
adolescence, growth ceases.
164
o Juvenile polyps or of retention occur mainly in young children (2-4
years), and rarely in adolescents and adults. The polyp is composed
of hamartomatous epithelial retention formed by cystic dilated glands
filled with mucus and inflammatory debris. These types of polyps
were first described by Verse in 1908.[15] Most juvenile polyps are 1-
2 cm diameter large, have a long (1-2 cm) stalks, red granular or
glistening surface. Cystic cavities may be present. Polyps manifest
around the age of 5-6 years, when bleeding occurs, loss of mucus,
diarrhea, abdominal pain and, rarely, invagination or rectal prolapse.
Pedicle torsion can occur followed by bleeding and elimination of the
polyp with feces. Polyps do not turn malignant, but frequently may
be associated with adenomatous polyps, requiring their removal.[16-
18]
Familial Juvenile Polyposis (FJP) has an autosomal dominant
inheritance with germline mutation in SMAD4 gene chromosome
18q21.1 or in the gene BMPRA1A. Juvenile polyposis syndrome is
clinically diagnosed if any one of the three following findings is
present:[19]
1. More than five juvenile polyps of the colorectum
2. Multiple juvenile polyps throughout the gastrointestinal tract
3. Any number of juvenile polyps and a family history of
juvenile polyps
More juvenile polyps are present over the entire digestive tract and
there is a high risk of developing digestive cancer (gastric, colon,
rectal). The condition may be associated with Osler-Weber-Rendu
syndrome. In this case, the screening colonoscopy starts at age of 15
and the treatment is represented by colectomy (even total) for colonic
polyps. When the family-specific mutation is known, it is appropriate
to perform molecular genetic testing on at-risk family members in the
first to second decade of life to identify those who will benefit from
early surveillance, intervention and genetic counseling.
o Peutz-Jeghers syndrome (PJS) is an autosomal dominant condition
in which numerous hamartomatous polyps are present along the
entire gastrointestinal tract (small intestine, large intestine, stomach,
and duodenum). Polyps can also occur in the larynx, trachea, and
bladder. Jan Peutz, a Dutch physician, described the syndrome in
1921. Mutations are present in the serine/threonine kinase gene
STK11 [20-24] on chromosome 19p13.3 locus in approximately 70%
of familial cases and 30-70% of sporadic cases.[2] The incidence in
US is around 1 to 300,000 cases. In 95% of cases, polyps are
associated with brown pigmentation of the lips, mouth, perioral,
perinasal, periocular and perianal skin. Rarely exostosis, ovarian
tumors and polyps of the bladder are associated. Polyp's histology is
characterized by extensive smooth muscle arborization throughout
the polyp. This may give the lesion the appearance of
pseudoinvasion, because some of the epithelial cells, usually from
benign glands, are surrounded by smooth muscle.[25] Colic polyps
represent 25% of all polyps with diameters from a few millimeters to
3 cm. Anemia, rectal bleeding, abdominal pain, obstruction, and/or
intussusception are common complications. Malignant degeneration
165
is rare (2-3%) but the risk of developing cancer is 15-18 times higher,
compared with that of general population.[25,26] The cumulative risk
of developing any cancers associated with PJS in patients aged 15-64
years is 93%.[25,27] Breast cancer in patients with PJS may occur at a
younger age, and it may be also bilateral.[28] Genetic counseling
should be provided for these patients. Patients with Peutz-Jeghers
syndrome usually undergo numerous surgeries for both
gastrointestinal and extraintestinal problems during their lives.
o Cowden syndrome is an autosomal dominant condition (a mutation
in the PTEN gene on arm 10q) [29] consisting of multiple intestinal
hamartomas associated with hyperkeratotic skin and gingival lesions.
These patients are at high risk of breast, brain, thyroid, prostate and
skin cancer. Disease's name comes from the family in which it was
first reported.[30] Approximately 300 cases have been reported.[31]
Women reached the age of 40 develop breast cancer at a rate of 50-
75%.
o Ruvalcaba-Myhre-Smith syndrome is an autosomal dominant
disease characterized by macrocephaly, genital pigmented lesions,
subcutaneous and visceral lipomas, hemangiomas and intestinal
hamartomas. It is a rare condition.
o Cronkhite-Canada syndrome is a sporadic non-inherited condition
characterized by diffuse hamartomatous polyposis of juvenile type
associated with nail dystrophy, alopecia and skin hyperpigmentation.
Weight loss, diarrhea and malnutrition are also associated. Most
patients are older than 50 years at the time of presentation. The
etiology of the disease is unknown. The effectiveness of
corticosteroid therapy seems to support the involvement of the
immune system in pathogenesis. It is a rare disorder. At the end of
2002, only 467 cases have been reported in the world literature, 354
of which were reported by Japanese groups.[32] Mortality rate
exceeds 50% regardless of therapy.
Neoplastic polyps are:
5. Adenomatous polyps (tubular, tubulo-villous, and villous) are represented by
abnormal cellular proliferation, with protrusion into the lumen and account
for two thirds of the colonic polyps. Mostly are sporadic and occur over 60
years, and the incidence increases with age, or are associated with inherited
polyposis syndromes, occurring in young patients.
Size of the polyps can vary from small pedunculated to large sessile. Large
adenomas (> 9 mm) may be more common in African Americans who have
a higher risk of right sided colonic adenomas and may present with cancer at
a younger age (< 50 years) than Caucasians.[33]
Neoplastic polyps are considered a premalignant condition and need an
adequate treatment. Time required for development of cancer from polyps is
about 7-10 years. There is an increased incidence of adenomatous polyps
association with invasive carcinoma (one third of resected carcinomas and
two thirds of synchronous carcinomas are associated with at least one
polyp).[34,35] Adenomatous polyps double the risk of metachronous
carcinomas.[36]
166
Synchronous cancers are cancers developed simultaneously on different
segments of the colon.
Metachronous cancers are cancers developed in succession diagnosed at
least six months after diagnosis of previous adenoma.
The malignant potential of the adenomatous polyps is considered to be:[37-39]
1% for polyps smaller than 1 cm
10% for polyps of 1-2 cm
50% for polyps larger than 2 cm
Histologically, polyps are classified depending on the glandular pattern in:[40]
(Figure 15)
Tubular adenoma (80% of adenomas with 5% risk of malignancy),
which has a stalk with a central core of fibrovascular tissue, covered by
dysplastic colonic mucosa. Rarely are multiple.
Villous adenoma (5-15% of adenomas), which is the largest (1 to 10 cm)
polyp encountered mostly in the rectosigmoid region with a high
potential of malignant transformation (40% risk of malignancy).
Tubulo-villous adenoma (22% risk of malignant transformation).
Pathologic classification:[41]
Low-grade dysplasia: characterized by branching crypts lined by cells
with long, thin nuclei that begin to stratify, resulting in increased
nucleus-to-cytoplasm ratio and a loss of normal goblet cells.
High-grade dysplasia: There is no invasive malignancy, which means
that the muscularis mucosa is not penetrated by neoplastic cells. It
represents an intermediate step in the evolution from low-grade
adenomatous polyp to cancer. It is not associated with metastasis since
there are no lymphatic vessels in the lamina propria.
Stages of degeneration for an adenomatous polyp are:
Carcinoma in situ = does not exceed the basal membrane
Microcancer = does not exceed the muscularis mucosae
Invasive carcinoma = muscularis mucosae is invaded

167
Risk factors for cancer [42]
Adenomatous polyps > 1 cm in diameter
Villous histology (adenomatous polyps with > 25% of villous histological
component)
High-grade dysplasia - adenomas with high-grade dysplasia often coexist with
areas of invasive cancer in the polyp
Number of polyps: three or more polyps is a risk factor for development of
metachronous adenomas with advanced pathologic features
Clinical picture
Although most polyps are asymptomatic, they are often in a continuous growth
process (may require 10 years to double their diameter) so may end up producing
symptoms by inducing intussusception or intestinal ulceration with bleeding. Rarely do
they reach sufficient size or number to produce obstruction. Less commonly, large
polyps may secrete mucus enough to cause diarrhea or enteropathy with protein
depletion.
Symptoms of adenomatous polyps depend on the size, number, location, and
extent of villous component. Adenomatous polyps may be asymptomatic, their
identification being a result of routine endoscopic examinations, double contrast barium
enema or hemocult test during screening programs. Most frequent symptoms are
gastrointestinal bleeding, diarrhea and abdominal cramps. In severe forms of mucous
diarrhea, important metabolic disorders may appear because of fluid and electrolytes
loss.
Treatment
Symptomatic (unique or multiple) polyps are usually treated by endoscopic
removal, local resection or segmental colectomy. Asymptomatic polyps may also
require their removal.
It is mandatory for all polyps to find their histological type (by biopsy or
excision), and if they are neoplastic they must be properly removed, to eliminate the risk
of progression to invasive carcinoma.
For pedunculated polyps (pedicle less than 1.5 cm in diameter), the best treatment
is the endoscopic polypectomy. (Figure 16) Every removed polyp should be examined
histologically. Sessile polyps with a large base of implantation can be removed also by
endoscopic approach piece by piece in one or more sessions.

168
Sugarbaker et al.[43] described the postpolypectomy syndrome represented by
abdominal pain, distension, and fever. Symptoms are temporary and fade after a day.
Clinical picture suggests a colonic perforation but pneumoperitoneum cannot be
observed on abdominal radiograph. Symptoms are probably caused by the local
peritoneal irritation as a result of electrocoagulation resulting in a transmural burn
without perforation. Overall mortality rate is 0.05%.[44-46]
Possible complications of the endoscopic polypectomy are:
Perforation with the consequence of general peritonitis
Hemorrhage which can be early or late after 7-10 days
Segmental colectomy (by laparotomy or laparoscopic approach) is performed
when polyps are too big for endoscopic polypectomy or have a large base of
implantation, or they turned malignant.(Figure 17)
Current recommendations regarding colorectal cancer screening
Currently accepted screening modalities include annual fecal occult blood testing
plus or minus sigmoidoscopy every 5 years, flexible sigmoidoscopy every 5 years,
colonoscopy every 10 years, or double-contrast barium enema every 5 years.[42]
The decision to perform each follow up colonoscopy should depend on the
patients wishes, the presence of co-morbidities, the patients age, and the presence of
other risk factors. Risk can be stratified according to findings at baseline and refined at
each subsequent surveillance examination.[47]
1. Low risk
Patients with only one or two small (<1 cm) adenomas
Recommendation: no follow up, or five yearly until one negative
examination
2. Intermediate risk
Patients with three or four small adenomas or at least one >1 cm
Recommendation: three yearly surveillance up until two consecutive
negative examinations
3. High risk
If either of the following are detected at any single examination (at
baseline or follow up): >5 adenomas or >3 adenomas at least one of
which is >1 cm
Recommendation: an extra examination should be undertaken at 12
months before returning to three yearly surveillance
Familial Adenomatous Polyposis (FAP)
FAP is caused by a germline
mutation of the APC tumor suppressor
gene, located on band 5q21. It is an
inherited, autosomal dominant disease, with
penetrance of 80-100%, [48] represented by
numerous adenomatous polyps in the colon.
(Figure 18) Polyps may be present in other
segments of the digestive tract such as
stomach (49%) or duodenum (25%).[49]
Corvisart (1847) and Chargelaique (1859)
described the first cases in the mid-
169
nineteenth century. This syndrome should not be confused with other multiple
adenomas. The distinction is based on the number of polyps present in the large
intestine. When there are more than 100 polyps, we speak about a familial adenomatous
polyposis, but usually thousands of polyps are present.
The disease appears in childhood, and malignization is the rule (before 40
years).[50]
The incidence of FAP is about 1/10,000 births.
Polyps vary in number, size (1% of them exceeding 1 cm in diameter) and shape
(pedunculated or sessile). The favorite location is on the left colon.
Many patients are asymptomatic. The diagnosis often arises during screening of
high-risk patients. The onset of symptoms is insidious at any age manifested by
bleeding, anemia and diarrhea, which are the most common complaints, followed by
abdominal pain. Weight loss, anemia or intestinal obstruction suggests malignant
transformation. The average age when symptoms appear is around 20 years and the
average age of malignant transformation is 35 years.[50,51]
Diagnosis is established by colonoscopy and double contrast irigography. At the
time of diagnosis, two thirds of patients already have colorectal cancer. Genetic
diagnosis (detection of FAP gene) is routinely used in Western countries and represents
an important element in counseling protocol
on family planning.
Once the diagnosis has been
established, the entire gastrointestinal tract
will be explored endoscopically (mainly the
stomach), by barium swallow (used for the
small intestine) or by endoscopic video
capsule.
The only way to prevent malignant
transformation is to perform a total
colectomy before the age of 15-20 years. In
most cases treatment is represented by total
proctocolectomy (the colon and rectum are
removed) followed by permanent ileostomy
(Figure 19) or ileoanal anastomosis. For a
better contention, an ileal pouch is
performed in cases of ileoanal anastomosis.
(Figure 20) Another possibly is represented
by total colectomy with ileorectal
anastomosis associated with the removal of
all rectal polyps by electrocautery. In this
case, the rectum should be careful
monitored by repeated rectoscopy in the
following years.
Variants of FAP
Turcot syndrome is an
inherited autosomal recessive
transmitted disorder characterized by the association of adenomatous polyps
with tumors of the central nervous system (medulloblastomas and gliomas).
The syndrome is also called the "glioma-polyposis" syndrome.[52]
170
Gardners syndrome is a disease with autosomal dominant transmission,
with penetrance of 80 - 100%.[53] The disorder is localized to a small region
on the long arm of chromosome 5 (5q21-22).[54] The triad consisting of
intestinal polyposis plus bone tumors (osteomas) plus tumors of soft tissue
(desmoid tumours, epidermoid cysts, lipomas, dental abnormalities,
periampullary carcinomas and juvenile nasopharyngeal angiofibromas)
characterizes this condition.[55] Symptoms are represented by colicky
abdominal pains, diarrhea, rectal bleedings and anemia. Radiological and
endoscopic aspects are similar to those seen in familial adenomatous
polyposis. In addition, there are polyps in the small intestine (especially in
the duodenum) and malignant tumors in other organs (thyroid, adrenal
glands, Vaterian ampulloma, etc). Bone and soft tissue tumors often precede
gastrointestinal symptoms. Bone tumors occur mostly on the mandible,
sphenoid, maxillary, and long bones.[56,57] Discovery of bone or soft tissue
tumors necessarily leads to radiological and endoscopic investigations of the
digestive tract. Family history investigation is required. Definitive treatment
consists of total colectomy with ileorectal anastomosis, total
proctocolectomy with a continent ileostomy or ileoanal anastomosis with
ileal pouch.
Attenuated FAP represents a milder phenotypical FAP variant; less than
100 adenomas are presents and fewer extracolonic manifestations. There is
also a delayed onset of colorectal cancer (delayed by 10-20 years).[58]
Prophylactic colectomy with ileorectal anastomosis is recommended in most
patients.
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173
5. Colon Cancer
Colon cancer represents the location of the neoplastic process along the colic
frame, from the ileocecal valve to the rectosigmoid junction. Although, from anatomical
and ontogenetic points of view, the two portions of the large intestine, the colon and the
rectum are different, in most treaties, colon and rectal cancer are studied together as
colorectal cancer. There are many similarities of tumoral etiopathogenesis between the
two segments, but there are also significant differences regarding symptoms, methods of
investigation and especially of surgical treatment. Peculiarities of surgical treatment for
rectal tumors are dictated by the special anatomy of the rectum, which has very intimate
relations with pelvic organs, and by the intention of surgeons to preserve, whenever
possible, the anal sphincter during operations for rectal cancer.
Remember that there may be more simultaneous tumors located in different
segments of the colon and/or rectum, and these tumors are called synchronous tumors.
They usually occur as a result of malignization of colorectal polyps. Metachronous
cancer is the occurrence of another (or more) tumor along the colon (usually away from
the primary tumor) after a period of time (of 6 months) following the removal of the
primary tumor. These cases occur most often because of malignization of remaining
colic polyps. These tumors should not be confused with local recurrences caused by
multiplication of remnant tumor cells in the area of primary tumor.
If diagnosed in the early stages, colon cancer is curable offering high rates of long
survival.
Although the etiology of colorectal cancer is not yet known and treatment
methods remained the classical surgery and chemoradiotherapy, in recent years progress
have been made in different fields such as: better understanding of the molecular basis
of carcinogenesis, endoscopic investigation and less toxic treatments.
Incidence
About 608,000 deaths from colorectal cancer are estimated worldwide,
accounting for 8% of all cancer deaths, making it the
fourth most common cause of death from cancer.[1]
Of 57 million global deaths in 2008, 7.6 million
(13%) was caused by cancer in general. The most
important causes of cancer death in 2008 are shown in
the table below (Table 1) according to the WHO.[2]
Colorectal cancer mortality is declining in the
last 15 years (at an average rate of 1.6% per year) due
to modern methods of detection and better therapeutic
methods.[3]
Geographical spread. Colorectal cancer
incidence is variable depending on geographical, socio-economic profile, eating habits,
etc. Almost 60% of the cases occur in developed regions. Incidence rates vary 10-fold in
both sexes worldwide, the highest rates being estimated in Australia/New Zealand and
Western Europe, the lowest in Africa (except Southern Africa) and South-Central Asia,
and are intermediate in Latin America. In the United States, colorectal cancer is the
second cause of death after lung cancer. Estimated new cases and deaths from colon and
rectal cancer in the United States in 2012 was: 143,000 (103,170 colon and 40,290
rectal) and respectively 51,690 (colon and rectal combined).[4]
174
Gender and race. Colorectal cancer is the third most common cancer in men
(663,000 cases, 10.0% of the total) and the second in women (571,000 cases, 9.4% of
the total) worldwide. Incidence rates are substantially higher in men than in women
(overall sex ratio of the ASRs 1.4:1).[1] In US, colorectal cancer is the fourth most
common cancer in men, after skin, prostate, and lung cancer. It is also the fourth most
common cancer in women, after skin, breast, and lung cancer.[4]
Mortality rates are lower in women than in men, except in the Caribbean. There is
less variability in mortality rates worldwide (6-fold in men, 5-fold in women), with the
highest mortality rates in both sexes estimated in Central and Eastern Europe (20.1 per
100,000 for male, 12.2 per 100,000 for female), and the lowest in Middle Africa (3.5
and 2.7 respectively).
Colorectal cancer is the second most common cancer in US women of Hispanic,
American Indian/Alaska native, or Asian/Pacific islander ancestry, and the third most
common cancer in white and African American women.[3] There is also a higher
incidence and death from colon cancer in African Americans than in whites. Hispanic
persons have the lowest incidence and mortality from colorectal cancer.[5]
Age. The risk of cancer increases with age. More than 90% of cases are diagnosed
in patients over 50 years. The most commonly affected decade is 60-69 years. Over 70
years, 2/3 of digestive cancers belong to the large intestine. With increasing age, the
incidence of right colon cancer is higher.[6-9] The incidence of colon cancer in people
under 40 years varies between 2% and 4%.[10]
Topographic distribution. Over two thirds
of colorectal cancers are located in the left colon
and rectum.[11,12] (Figure 21) Some studies [9,13]
suggest a right ward shift in anatomical
distribution with increase in proximal colon
cancers whereas others deny it.[14,15]
Synchronous carcinoma incidence varies
between 1.5% and 8%. Benign polyps have a
frequency of 12-60% in patients with a single
cancer and 57-86% in those with synchronous
cancers.[10]
Pathogenesis
Colorectal cancer originates from the epithelial cells of the mucosa layer. Even
though, the etiology of colorectal cancer is still unclear, progresses have been made in
understanding the pathogenesis of this disease. In recent decades, researchers have
focused their attention on the genetic changes in cancer and adenomatous cells in
patients with sporadic or familial colic cancer. Advanced molecular biology techniques
were able to reveal tiny changes occurring at chromosomal level.
Most colorectal cancers develop from adenomatous polyps. Dysplastic changes
are not found in other types of polyps such as hyperplastic, inflammatory or
hamartomatous. There are many evidences supporting the hypothesis of cancer
development from adenomatous tissue.
Genetic modifications as well the molecular pathways are very complex and may
vary in each particular tumor. A few aspects are presented below.
Most frequently, cancer is induced by mutations in the Wnt signaling pathway
that artificially increase signaling activity. The Wnt signaling pathway is a network of
proteins that passes signals from receptors on the surface of the cell to DNA expression
175
in the nucleus. The mutations can be inherited or are acquired, and most probably occur
in the intestinal crypt stem cell.[16-19] The most commonly mutated gene is the APC
gene, which produces the APC protein. In the absence of this protein the -catenin
protein accumulates in excess and enters the nucleus where binds to DNA and activates
the transcription genes. Other mutations may be also present such as CTNNB1, AXIN1,
AXIN2, TCF7L2, or NKD1.[20] To induce the cancer other mutations are also
necessary. For example, the TP53 gene produces the protein p53 that monitors the cell
division and kill cells with Wnt pathway defects and, thus, functions as a tumor
suppressor that is involved in preventing cancer. Mutations of TP53 gene transform the
tissue from an adenoma into an invasive carcinoma. The DCC (Deleted in Colorectal
Cancer) gene, which produces a protein with the same name, was first discovered in a
colorectal cancer study in 1990. It is considered a tumor suppressor gene meaning that it
normally prevents cell growth.
Other genes stimulate the cell multiplication in the presence of growth factors.
These are oncogenes. For example, genes encoding the proteins KRAS, RAF, and PI3K,
can acquire mutations that result in over-activation of cell proliferation.
The c-MYC gene in normal cells, only expresses the gene when cells actively
divide whereas in cancer cells may express the gene in an uncontrolled fashion as the
result of genetic aberrations. The c-Myc protein or the c-MYC gene is overexpressed in
a wide variety of human cancers with 80% of breast cancers, 70% of colon cancer, 90%
of gynecological cancers, 50% of hepatocellular carcinomas.[21] This gene is currently
regarded as a central transcriptional oncogenic switch that regulates a large variety of
cellular functions through altering gene expression.
The chronological order of mutations is important. A primary KRAS mutation
generally leads to a self-limiting hyperplastic tumor, but if KRAS mutation appears
after a previous APC mutation the tumor progresses to cancer.[22]
Microsatellite instability has also an important role in tumor genesis.
Microsatellites are repeated sequences of DNA that everyone has. Defects in DNA
repair genes, however, can lead to these microsatellites gaining or losing length and
becoming unstable. This can lead to the colorectal tumors having a mutation rate that is
10 to 100 times higher than seen in other tumors.[23]
Comprehensive, genome-scale analysis has revealed that there are two types of
colorectal carcinomas: hypermutated and non-hypermutated tumor types.[24] The
hypermutated cancers are found mainly in the right colon and non-hypermutated could
be found throughout the colon and rectum. Studies have shown that people with
hypermutated tumors have a better prognosis than those whose tumors are mutated at
lower rates.[24]
Tumoral angiogenesis
Angiogenesis (Figure 22) is an important
process for growth, wound healing and
granulation tissue production and consists in the
development of new blood vessels. Given that
tumor proliferative process involves intense cell
proliferation, these cells need nutrient resources
that are brought by blood. For growth and spread,
the tumor needs new blood vessels to supply it
with nutrients. Tumoral cells secrete substances
that induce the development of new blood vessels.
This aspect is important in the fight against
176
cancer, and drugs that block angiogenesis substances secreted by tumoral cells have
been synthesized. A tumor that has no blood vessels cannot grow and finally die.
Angiogenesis also favors the transition from hyperplasia to neoplasia. Solid tumors,
smaller than 1 to 2 cubic millimeters are not vascularized. Oxygen and nutrients reach
the tumor by diffusion.
Angiogenesis stimulating substances produced by tumor are VEGF (vascular
endothelial growth factor) and bFGF (basic fibroblast growth factor).
Neovastat is an inhibitor of angiogenesis blocking receptor sites for the
angiogenic growth factor VEGF, which prevents endothelial cells from proliferating,
migrating, and organizing to form new blood vessels. Other similar drugs are
bevacizumab (Avastin), sorafenib (Nexavar), sunitinib (Sutent), pazopanib
(Votrient), and everolimus (Afinitor).[25-27]
Risk factors
The risk factor is something that increases the chance of developing a disease but
that does not mean that the patient will necessarily develop the disease. People with
certain risk factors are more likely to develop colorectal cancer. Studies have found the
following risk factors for colorectal cancer:
1. Genetic factors:
a. Familial adenomatous polyposis (FAP) is a rare (less than 1percent of
colorectal cancers), inherited condition caused by a change in a specific
gene called APC. The condition usually leads to colorectal cancer by age
40.
b. Gardner, Turcot and Oldfild syndromes, are familial polyposis
associated with other pathological disorders in different organs or tissues.
c. Peutz Jeghers syndrome
d. Hereditary nonpolyposis colorectal cancer (HNPCC) - Lynch syndrome.
It accounts for about three percent of all colorectal cancer cases.[28] In
individuals with this disorder, colon polyps occur at an earlier age than in
general population. Although the polyps do not occur in greater numbers
than in general population, they are more likely to become cancerous.
Lynch syndrome cancer risk is inherited in an autosomal dominant
pattern. The disorder is caused by a mutation in a mismatch repair genes
(MLH1, MSH2, MSH6, PMS1 and PMS2). Affected individuals have a
higher than normal chance of developing colorectal cancer, endometrial
cancer, and various other types of aggressive cancers, often at a young
age.[4]
Family members of people who have HNPCC or FAP can have genetic
testing to check for specific genetic changes.
e. Family history of colorectal cancer - Close relatives (parents, brothers,
sisters, or children) of a person with a history of colorectal cancer are
somewhat more likely to develop this disease themselves, especially if
the relative had the cancer at a young age. If many close relatives have a
history of colorectal cancer, the risk is even greater.
2. Underlying diseases
a. Inflammatory bowel disease. Persons with ulcerative colitis or Crohn
disease are at increased risk of developing colorectal cancer. In 1925,
Crohn and Rosenberg were the first who noticed the link between
177
ulcerative colitis and colic cancer. Ulcerative colitis is considered a
preneoplastic disease. Disease duration of 7-10 years and the onset at
young ages are additional risk factors. A Swedish study found that
approximately 40% of patients with ulcerative colitis developed colon
cancer especially when the disease started before the age of 15.[29] The
Crohn's disease has a risk of malignancy of about 4-20%.[30]
b. Previous operated colorectal cancer. A person, who has already had
colorectal cancer, may develop colorectal cancer a second time. In
addition, women with a history of cancer of the ovary, uterus
(endometrium), or breast are at a somewhat higher risk of developing
colorectal cancer.
c. Pelvic cancer post-irradiation. Patients who got irradiation on pelvic
region are eight times more likely to develop rectosigmoidian cancer
compared wit non-irradiated population. For women irradiated for
gynecological cancer this risk is 2 to 3.6 times higher.[10]
d. Sporadic colorectal polyps. They are common in people over age 50.
Most polyps are benign, but some (adenomas) can transform in cancer.
Finding and removing polyps may reduce the risk of colorectal cancer.
3. General factors
a. All men and women aged over 50. Colorectal cancer is more likely to
occur, as people get older. More than 90 percent of people with this
disease are diagnosed after age 50. The average age at diagnosis is 72.[4]
The risk population for colorectal cancer was classified into three grades.
1. A low-risk group:
a. All asymptomatic individuals over 45 years. Hemocult or Hemoquant
tests are indicated yearly.
2. A high-risk group:
a. Persons with family history of colorectal cancer. Regular colonoscopy is
indicated in people over 45.
b. Patients with a history of colon polyps. Follow up colonoscopy is
indicated every 1-3 years depending on the pattern of polyp.
c. Patients with a history of colorectal cancer. Follow up colonoscopy is
indicated every year after surgery.
d. Patients with history of inflammatory bowel disease. In these patients
annually colonoscopy is recommended.
3. A very high-risk group:
a. Familial adenomatous polyposis. Early diagnosis is based on detection
of genetic changes and treatment is proctocolectomy.
b. Hereditary nonpolyposis colorectal cancer. The treatment is also
represented by total colectomy.
Approximately 80% of cases of colorectal cancers are sporadic and occur in
people without apparent risks.
Over time, researchers have sought to find factors that cause or induce colorectal
cancer, factors that can be modified for primary prevention. Besides these factors,
others cannot be modified such as the age, race, genetic or familial factors.
178
Factors incriminated in the etiopathogenesis of colorectal cancer, which can be
modified, are:
1. Diet
a. Studies suggest that diets high in fat (especially animal fat) lead to
increased excretion of bile acids, which in animal studies, has been
shown to be tumor promoters, especially after changes in intestinal
bacterial flora.[31,32] Increased levels of secondary bile acids after
gallstone disease are believed to increase the risk of colorectal cancer [33]
but there is no positive association between previous cholecystectomy
and the risk of cancer.[33,34]
b. People who eat a diet very low in fruits and vegetables may have a higher
risk of colorectal cancer. The influence of vegetable fibers has been
extensively studied by Burkitt who popularized the idea that colic cancer
incidence is higher in western countries due to the lower fiber content in
diet.[35] Following researches concluded that dietary fibers from fruits
and vegetables would have a more important protective role than those
from cereals.[36,37] Vegetable fibers have a profilactic effect by several
mechanisms. The increased volume of the colon content reduces the
pathogenic factors contained in feces and decreases the bile acid
concentration, both by dilution and by absorption on vegetable fibers. A
higher concentration of fibers is associated with a reduction in colonic
anaerobic bacteria, with the consequent decrease in secondary bile acid
formation.
c. Calcium causes a reduction of epithelial proliferative response induced
by a diet rich in fatty acids and bile acids.[38]
d. Selenium is involved not only in colorectal carcinogenesis, but also in
other cancers such as prostate, lung, breast and ovary.[39,40]
Anticarcinogenic effect of selenium was proposed in 1973 following the
detection of low serum levels in patients with colorectal cancer. Meat
contains small quantities of selenium but rich sources are considered
grains and seafood.[41]
e. Vitamins such as A, C, D, E have a protective role by various
mechanisms especially by their antioxidant properties.
f. Carcinogenic substances produced by food preparation or conservation
can induce colon cancer. Roasted meat contains heterocyclic amines able
to induce colorectal cancer in animals.[42,43] Smoked foods have also a
carcinogenic effect. Vegetables and fruits may also contain polycyclic
aromatic hydrocarbons with carcinogenic role due to absorption of water
contaminated with residues of incomplete combustion of hydrocarbons.
g. Alcohol consumption. Most epidemiological studies have shown no
correlation between colic cancer and alcohol consumption. Some,
however, have found a positive correlation between alcohol use and
adenomatous polyps larger than 10 mm, suggesting that alcohol is a
factor that intervenes in the adenoma-cancer sequence.[44] In rectal
cancer, however, the correlation is quite evident especially for beer
consumption.[45]
Based on these facts, many different types of diets have been proposed, but
general recommendations are the followings:
179
To reduce dietary fat content at less than 30% of total caloric intake
value,
To eat a greater amount of vegetables, fruits, and cereals,
To reduced the consumption of alcohol,
To avoid smoked and fried foods.
2. Cigarette smoking: A person who smokes cigarettes may be at increased risk
of developing polyps and colorectal cancer.[46,47]
3. Ureterosigmoidostomy. Patients with such surgery are at increased risk of
cancer. Tumors appear around the anastomosis, with a latency period of less
than 20 years.[48-51]
Morphopathology
To grow up from 10 microns to 1 cm, a cancerous cell needs thirty divisions.
Considering that doubling time is 109 days, a 1 cm tumor evolves over nine years
before it can be detected as a small tumor. Even with a high growth potential of 34 days
of doubling time, the tumor will need at least 30 months to reach a diameter of one cm.
Tumor growth rate varies depending on its developmental stage. As the tumor increases
its volume, nutrition supply is more limited and hence there is a deceleration of tumor
growth.
Macroscopic appearance. There are four types of colorectal cancers. (Figure 23)
1. Ulcerative form (or endophytic/ulcerative) - the tumor looks like a crater
with irregular raised edges, often with necrotic base. Lesions tend to
infiltrate deep the colic wall increasing the risk of perforation.
2. Polypoid (vegetative or exophytic/fungating) form - the lesion protrudes into
the intestinal lumen having a cauliflower aspect. Sometimes the surface is
ulcerated (the ulcero-vegetative form). These types of tumors are located
most commonly in the cecum and ascending colon.
3. Infiltrative form - it is an annular tumor with circumferential involvement of
the colorectal wall and constriction of the lumen. The circular stenosing
lesion varies in size, with tendency to ulceration. It occurs most commonly
in the left colon, leading to intestinal occlusions. In the diffuse infiltrative
form, the cancer infiltrates the intestinal wall for at least 5-8 cm. It is rarely
encountered, being similar to the gastric linitis plastica.
4. The colloid form is described only by some authors. These tumors produce
large amounts of mucin that gives them a gelatinous appearance.

Pathways of dissemination
Colon cancer spreads by contiguity, through lymphatic or blood vessels, along
nerve fibers, into the peritoneal cavity by gravitation or by surgical insemination.
180
Intramural dissemination occurs in all directions, but especially in the transverse
direction, which explains the circular stenosing form of relatively small tumors.
The dissemination is produced through lymphatic permeation.
Transparietal dissemination is most intense in the central area of the tumor,
which is also its oldest part, leading to invasion of neighboring anatomical
structures.
Dissemination by gravitation occurs when the tumor exceeds the intestinal wall.
From the surface of the invaded serosa, tumoral cells are detach and seeded on
the parietal and visceral peritoneum resulting the peritoneal carcinomatosis
mainly in the Douglas bag (Blumer neoplastic infiltration) and ascites. This type
of dissemination may cause Krukenberg ovarian tumors.
Extramural lymphatic dissemination is produced by permeation and
embolization of lymphatic vessels and is the most common way of
dissemination. Lymph nodes metastases not always occur gradually and in
anatomical order. Some nodal stations can be avoided with more distant lymph
nodes invasion. This aspect motivates the removal of a large segment of colon
with regional lymphatic stations, even in the absence of detectable pathological
changes of lymph nodes, during operation.
Dissemination via venous blood system occurs
especially in less differentiated tumors. Liver
metastases are the most frequent being present in
one third to one-half of operated patients, but often
they are not detectable by macroscopic
examination. The next common sites of distant
metastases are the lungs (20%), brain, and bones.
(Figure 24) To prevent hematogenous spreading of
cancerous cells during operation, by handling the
tumor, vascular pedicles are ligated from the
beginning.
Intraluminal dissemination is also possible when
malignant cells or even tumoral fragments, can
detach spontaneously or by intraoperative handling,
and then seeded on the mucosa surface, especially
on bleeding scars, anastomosis line, post-
hemorrhoidectomy or other causes of mucosa
lesions.[52] That is the reason why the colon is
ligated upstream and downstream the tumor at the beginning of operation.
Staging of colon cancer
Many staging systems for colon cancer have been imagined, but it seems that
Dukes' modified by Astler-Coller (MAC) staging system is the most used by surgeons,
being practical and pretty well reflecting the tumor development stage. The system
proposed by Dukes in 1930 for rectal tumors has been modified over time by many
authors. In 1954, Astler and Coller proposed a change in the classification, so that it can
be extended to the intraperitoneal part of the rectum and colon.
The original Dukes classification was:
A: Tumor limited to the bowel wall
B: Tumor penetrating through the bowel wall
C: Regional lymph nodes metastasis
181
The MAC classification:
A: Tumor limited to the mucosa
B1: Tumor extending into but not through the muscularis propria
B2: Tumor penetrating through the serosa but no involvement of lymph nodes
B3: Tumor invades adjacent structures
C1: Same as B1 plus regional nodal metastasis
D: Distant metastasis
American Joint Committee on Cancer (AJCC) proposed the TNM classification:
Primary tumor (T):
Tx: Primary tumor cannot be assessed
Tis: Tumor involves only the mucosa. Also known as intramucosal carcinoma
T1: Tumor extends through the mucosa and into submucosa
T2: Tumor extends through the submucosa and into muscularis propria
T3: Tumor extends through the muscularis propria and into the subserosa but not to any
neighboring organs or tissues
T4: Tumor extends through the wall of the colon or rectum and into nearby organs or
tissues.
Regional lymph nodes (N):
NX: Regional lymph nodes not assessed
N0: No lymph node involvement is found
N1: Metastasis in 1 to 3 regional lymph nodes
N2: Metastasis in 4 or more regional lymph nodes.
Distant metastasis (M):
MX: Distant metastasis cannot be assessed (not evaluated by any modality)
M0: No distant metastasis
M1: Distant metastasis
Stage groupings: based on the patient's T, N, and M categories determined,
usually after surgery, the stage of tumor could be as in the following table. (Table 2)
Table 2 - TNM stage grouping of colon cancer and correlation with other
classification systems

Histopathological types and degrees of differentiation
The vast majority (90-95%) of colic cancers are adenocarcinomas. World Health
Organization (WHO) classified primary tumors of the colon and rectum as follows:
(Table 3) [53]
Table 3 - WHO histological classification of tumors of the colon and rectum
Epithelial tumours
Adenoma
Tubular
Villous
Tubulovillous
Serrated
Non-epithelial tumours
Lipoma
Leiomyoma
Gastrointestinal stromal tumour
Leiomyosarcoma
Angiosarcoma
Kaposi sarcoma
182
Intraepithelial neoplasia (dysplasia) associated
with chronic inflammatory diseases
Low-grade glandular intraepithelial
neoplasia
High-grade glandular intraepithelial
neoplasia
Carcinoma
Adenocarcinoma
Mucinous adenocarcinoma
Signet-ring cell carcinoma
Small cell carcinoma
Squamous cell carcinoma
Adenosquamous carcinoma
Medullary carcinoma
Undifferentiated carcinoma
Carcinoid (well differentiated endocrine
neoplasm)
EC-cell, serotonin-producing neoplasm
L-cell, glucagon-like peptide and
PP/PYY producing tumour
Others
Others
Malignant melanoma
Others
Malignant lymphomas
Marginal zone B-cell lymphoma of
MALT Type
Mantle cell lymphoma
Diffuse large B-cell lymphoma
Burkitt lymphoma
Burkitt-like /atypical Burkitt-
lymphoma
Others

Secondary tumors

Polyps
Hyperplastic (metaplastic)
Peutz-Jeghers
Juvenile

Mucinous adenocarcinoma means a type of adenocarcinoma, which contains
50% or more mucus. It is one of the most common types of colorectal cancers. Up to
95% of the colorectal cancers are mucinous adenocarcinoma. This variant is
characterized by pools of extracellular mucin that contain malignant epithelium as
acinar structures, strips of cells or single cells.[53] It is encountered mostly in young
patients and considered a type with a poor prognosis and reduced response to
chemotherapy.[54-56]
Signet-ring cell carcinoma. This is a variant of adenocarcinoma in which cells
have a typical aspect of signet-ring because the cytoplasm contains a large mucin
vacuole that displaces the nucleus. It is associated with a poor prognosis.[54]
Adenosquamous carcinoma is an unusual tumor with features of both squamous
carcinoma and adenocarcinoma. Pure squamous cell carcinoma is very rare in the large
bowel.
Carcinoid tumors are rare endocrine tumors usually located in the right colon as a
single bulky tumor. At time of diagnosis, 75% of them already have lymph node and
liver metastases and hence their poor prognosis.[57,58] Endocrine tumors are more
common in the rectum (54% of the cases), followed by the cecum (20%), sigmoid colon
(7.5%), rectosigmoid colon (5.5%) and ascending colon (5%).[53] Less than 5% of
patients present with the carcinoid syndrome.[59]
Malignant lymphoma of the colon represents approximately 10% of all primary
digestive tract lymphomas. It is less frequent than either gastric or small bowel
lymphomas. Almost all are located in the cecum having an ulcerovegetative and bulky
aspect with extension to the intestines. Most colorectal lymphomas occur in older
patients with acquired immunodeficiency syndrome (the majority of cases occur in
homosexual men). Ulcerative colitis, is a recognized predisposing factor.[60] Most
colorectal lymphomas involve the distal large bowel, rectum and anus.
Sarcomas are exceptionally rare. Gastrointestinal stromal tumors (GISTs) of the
colorectum are similar to those in the stomach and small intestine.
183
Grading
Regarding the degree of differentiation, there are two classification systems: that
of Broders and of Dukes. The first takes into account the percentage of differentiated
cells and classifies tumors in four grades: well differentiated, moderately differentiated,
poorly differentiated, and undifferentiated (anaplastic). Dukes classification considers
the cell arrangement, and has three degrees: Grade 1 includes the well-differentiated
tumors (tubular glands well shaped with the fewest mitosis and nuclear polymorphism),
Grade 3 includes the least differentiated tumors with only occasional glandular
structures with pronounced cellular pleomorphism and many mitoses, and Grade 2
which is between the two above.
According to WHO, degrees of differentiation are considered based on the
percentage of glandular structures present in the tumor:[53]
1. Well differentiated (grade 1) - lesions exhibit glandular structures in > 95% of
the tumor
2. Moderately differentiated (grade 2) - adenocarcinoma has 50-95% glands
3. Poorly differentiated (grade 3) - adenocarcinoma has 5-50% glandular structures
4. Undifferentiated (grade 4) - there are less than 5% glandular structures
Mucinous adenocarcinoma and signet-ring cell carcinoma by convention are
considered poorly differentiated (grade 3).
Screening methods
Knowing that the prognosis is closely related to the early diagnosis, for those
individuals at high risk of developing colorectal cancer, screening methods have been
imagined.
Hemocult test detects the occult intestinal bleeding. It uses a filter paper
impregnated with tincture of guaiac. A small amount of feces and hydrogen
peroxide are added on the paper. The color of the paper turns from yellow to
blue in the presence of peroxidase enzyme from the blood present in feces. The
method has a sensitivity of 75% but there are frequently false positive or
negative results (the C vitamin gives false negative, while aspirin and digested
blood from the stomach gives false positive results).
Haemo-Quant test uses the same principle but it is not influenced by the
dietary peroxidases.
Colonoscopy performed annually for high-risk category of individuals and every
five years after the age of 50 for low-risk category of population.
Carcino-embryonic antigen (CEA) is the most widely used serological marker,
being discovered by Philgold and Freedman in 1965. It is not specific for colon
cancer. High values of CEA can be found in other cancers too, such as
melanoma, lymphoma, breast, lung, pancreatic, gastric, thyroid, kidney, liver,
ovarian, bladder and cervical cancer. High CEA values can be also found in
some benign diseases, including bone inflammation, pancreatitis, and liver
disease. Smoking may increase CEA values above normal. CEA is used after
colorectal cancer surgery for early detection of recurrences or metastases. It is
repeated every 6 month in the postoperative period. If sustained increased levels
are detected, the probability of cancer recurrence is about 80-90% and a "second
look" operation may be indicated. Anyway, the utility of the second look
operation is questionable, given that less than 20% of recurrent tumors are
resectable and less than 5% with curative intention.[61-63]
184
Clinical picture
The clinical picture of colon cancer is not very specific and it varies very much
depending on the tumors stage of evolution. There are two main stages, namely: the
preclinical stage, in which patients have no symptoms although they are bearers of an
early colon cancer and the clinically manifested stage with various symptoms more or
less suggestive of a colon tumor. In this stage, patients frequently have symptoms of
tumoral complications such as gastrointestinal bleeding or low intestinal occlusion.
The best for prognosis is to detect colon tumors in the preclinical stage, but
unfortunately, the most patients are admitted to surgery at about 6-9 months after the
onset of symptoms because complaints are attributed to other benign conditions with
similar symptoms (enterocolitis, hemorrhoids, peptic ulcer, cholecystitis, etc.).
The onset is most frequently insidious with bowel disorder (diarrhea,
constipation, or alternating diarrhea-constipation), intermittent colicky pain, flatulence,
asthenia, and anemia. Sometimes, however, the onset may be acute with symptoms of
low intestinal occlusions or other complications such as bleeding, perforation, or
abscess formation.
The clinical manifested stage is dominated by bowel transit disorders plus signs
and symptoms related to tumoral complications (stenosis, bleeding, perforation, abscess
formation, penetration into neighboring organs and dissemination) and signs and
symptoms of neoplastic impregnation (the so-called Savitkis signs: weakness, fatigue,
loss of appetite decreased diminished intellectual capacity and sometimes fever).
Bowel disorders (present in 35% of patients) may manifest as diarrhea,
constipation or alternating constipation-diarrhea, the latter being the consequence of
intermittent discharge of feces collected above the obstacle represented by the tumor.
Other signs of dyspepsia such as gurgling and flatulence are associated. When tumor is
located near the ileocecal valve or at sigmoid colon, diarrhea may have a dysenteric
character. As the tumor increases, colon stenosis (especially on the left colon) or
obstruction (on the right colon) appear associated with symptoms of intestinal
obstruction manifested by intestinal transit stop for gases and feces. Vomiting occurs
later and in neglected obstruction, when it becomes fecaloid. The progression is toward
hypovolemic shock. Abdominal colicky pains accompany transit disorders most often.
Pain (present in 50% of patients) in early stage is vague and has a less precise
localization. Later, pains become colicky being associated with flatulence and nausea.
As the tumoral stenosis progresses, colicky pains increase in intensity reaching the peak
when complete occlusion is installed. A sudden very intense colicky pain can be the
result of complications such as intussusception or intestinal volvulus with rapid
evolution to occlusion. A constant pain in a particular region is a sign of local
complication such as tumor penetration into adjacent structures or tumor perforation
with abscess formation. The sudden intense abdominal pain with subsequent
generalization is a possible symptom of generalized peritonitis produced by diastatic
perforation. In advanced stages, pain may be produced by distant metastasis in various
regions (bone, chest, head, etc.).
Pallor associated with physical fatigue and weight loss are the following more
common signs and symptoms. The cause of pallor is the anemia produced by occult
bleeding especially in tumors of the right colon.
Pathological content of feces (blood, mucus, pus). The presence of blood in
stool is the most frightening sign for the patient. Bleeding with fresh blood is usually
present in rectal tumors, but can also occur in tumors located on the left colon.
185
Unfortunately, many times the presence of blood in stool is attributed to hemorrhoids or
anal fissures leading to delay in the correct diagnosis. Blood mixed with stool is a sign
that should raise the suspicion of colorectal cancer. Colonoscopy for diagnosis is
mandatory in this case. The dark blood (melena) occurs more frequently in right colon
tumors. Occult bleeding (present in 15% of cases) manifests only by anemia, fatigue
and dyspnea, frequently being encountered in ascending colon cancer. Anemia is often
the only symptom of cancer of the large intestine. There is a hypochromic and
microcytic anemia due to iron deficiency. Abundant mucus content of feces is
especially caused by tumors developed from adenovillous polyps. The presence of puss
in stool is more characteristic for advanced rectal cancer than for colon cancer. The
presence of puss in the stool can be explained by the evacuation of peritumoral abscess
content inside the colon.
In about 5% of cases, complaints are caused by metastases, the primary tumor
being silent. Symptoms can be: bone pain, pathological bone fractures, jaundice,
neurological signs, changes of personality, migrant thrombophlebitis and cutaneous
metastases (particularly the Sister Joseph's nodule). Colorectal cancer may be associated
with dermatologic diseases such as acanthosis nigricans, dermatomyositis, pemphigus
and pyoderma gangrenosum.
Complications
Untreated, the colon cancer progresses to death because of invasion,
dissemination, and complications.
Occlusion is the most common complication of colon cancer, occurring mainly
in the left colon tumors. Possible mechanisms by which the occlusion occurs are:
stenosis, encountered especially in the left colon tumors, luminal obstruction, especially
in vegetative bulky tumors of the right colon, torsion (volvulus), the sigma and cecum
may become volvulated, intussusception, especially ileocolic type, and penetration
when occlusion is caused by penetration into the small intestine. Untreated, the patient
will die from hypovolemic shock and/or generalized peritonitis caused by diastatic
perforation. Occlusion is a negative prognostic factor.
Penetration into the surrounding organs or tissues is the second most common
complication. Adhesion, more or less firm, to a neighboring organ does not necessarily
mean a tumor penetration. Often these adhesions are purely inflammatory, as confirmed
by histopathological examination. Penetration may complicate with intestinal
obstruction (small intestine, duodenum), ureter dilatation and hydronephrosis, or fistula
formation between the colon and other hollow organs such as bladder, uterus, intestines,
and stomach. Frequently the cancer penetrates into the retroperitoneum.
Perforation of the tumor is a
complication caused by the tumoral
ulceration that penetrates through the entire
colon wall thickness. Perforation into the
peritoneal cavity leads to generalized
stercoral peritonitis. The posterior
perforation results in a retroperitoneal
abscess, often difficult to be differentiated
from a periappendicular abscess or
diverticulitis. Perforation into a hollow organ
leads to an internal fistula. Diastatic
perforation means that perforation occurs at
distance, upstream the tumor, caused by
186
extreme distension of the colon with ischemic lesions of the intestinal wall. (Figure 25)
Infectious complications are mainly represented by peritumoral abscesses.
Hemorrhagic complications, although much rarer, is the second leading cause of
emergency admissions, as frequency, after intestinal occlusion.
Physical exam
On inspection, the pallor can be observed when anemia is present, and jaundice
appears in case of multiple liver metastases. Abdominal distension is present in cases of
intestinal obstruction. Symptomatic umbilical hernia may appear in carcinomatous
ascites.
On palpation, nothing can be found or just a mild pain at tumor site. Intense pain
associated with muscular defense is present in case of perforation. The tumor can be
palpated in approximately 15-20% of cases especially when is located on cecum or
sigmoid colon. When a tumoral mass is palpated in these regions, the differential
diagnosis should include the periappendicular block, kidney ptosis, retroperitoneal
tumors, and diverticulitis of the sigmoid colon. Multiple palpable tumoral masses
usually occur in case of peritoneal carcinomatosis.
On percussion, there are no pathological elements except the tympanic sound in
case of bowel obstruction and shifting dullness in case of ascites.
In most cases, auscultation of the abdomen does not reveal anything special. In
some cases, the steam spout sound caused by crossing the bowel content through the
narrowed zone can be heard (the Knig syndrome). Synchronous tumors can cause
these type sounds in succession (the Keberle syndrome). Sounds have a very high
intensity, so the other people in the room can hear them.
Digital rectal examination is mandatory in any clinical examination of the
digestive system and especially in suspected colorectal pathology. The presence of dark
blood on finger glove raises the suspicion of colon tumor. Peritoneal metastases in
Douglas bag can also be felt on digital rectal examination (the Blumer sign).
Clinical aspects depending on tumor location
1. Tumors located on the right colon have the following characteristics:
The macroscopic aspect is that of vegetative or ulcero-vegetative form.
Clinical picture is represented by anemia with pallor, physical fatigue, dyspnea,
inappetence, weight loss.
The tumor grows to large dimensions (5-15 cm in diameter) due to the much
larger lumen of the colon at this level, enabling its development without causing
alarming symptoms such as intestinal obstruction.
Usually the time elapsed between the onset of symptoms and surgery, is much
longer, and that is why in many cases tumors are found penetrating into the
adjacent anatomical structures. Special consideration should be given to the
penetration into the right ureter and duodenum, situations that require special
surgical tactics.
The palpable tumor can be confused with periappendicular block or right kidney
ptosis.
Complications are represented by penetration, ileocolic intussusception, and
volvulus of the cecum.
2. Tumors of the transverse colon have the following characteristics:
187
Tumors are of vegetative form but can be as well infiltrative.
The clinical picture is completely uncharacteristic being dominated by
symptoms belonging to neighboring organs pathology. Most often, the confusion
is made with peptic ulcer or chronic pancreatitis.
The length of the transverse mesocolon confers a particular mobility to the
tumor, which can reach even in the pelvis penetrating the bladder, or the uterus
with symptoms associated to the pathology of these organs. The pancreas and
the stomach are the most frequently invaded organs with the possibility of
developing gastrocolic fistula manifested by diarrhea, fecaloid vomiting, and
seriously impaired general condition. Tumors located in the hepatic angle of the
colon can penetrate the liver and extrahepatic bile ducts with mechanical
jaundice. Tumors of the splenic angle are usually smaller and produce stenosis
followed by intestinal occlusion.
3. Tumors of the left colon have the following characteristics:
This is the most common location of colon cancer. Almost 75% of left colon
tumors are located in the sigmoid colon.
Tumors are smaller, infiltrative, producing stenosis, and often complicated with
early intestinal obstruction facilitated by the narrower lumen of this segment of
colon.
Symptoms initially are represented by constipation and abdominal colicky pains,
followed then by diarrhea as a consequence of feces liquefaction upstream the
obstacle represented by tumor.
Bleeding is rarely heavy and usually exteriorized as fresh blood mixed with
feces. Perforation and peritumoral abscess raises difficult differential diagnosis
with perforated diverticulitis.
A long mesosigmoid confers a special mobility so that tumors located on
sigmoid loop can penetrate other structures such as bladder, uterus, ileal loops,
and cecum. Because the sigma has a more superficial location, compared with
other colon segments, the tumor can be palpated through the abdominal wall or
by rectal or vaginal tact when the sigmoid loop is fallen down in the Douglas
bag.
Workup
The most important investigation for colorectal cancer diagnosis is colonoscopy
(Figure 26) with biopsy. Colonoscopy has the advantages that directly visualizes
the tumor and can perform biopsies. Colonoscopy is not a simple procedure and
not free of any risks (perforation, bleeding) and requires a good preparation of
the bowel by purgation and even
sedation of the patient. Sigmoidoscopy
is an easier endoscopic investigation,
which does not require patient sedation
being useful for diagnosis of most
colorectal cancers considering that
two-thirds of tumors are located on the
left colon. It is also a good screening
investigation.
Barium enema is the second choice in
nowadays being applied especially in
188
cases where endoscopic investigation is contraindicated or is not possible for
other reasons. The colon must be prepared (completely evacuated) before
examination. Characteristic images
for colon tumor are lack of filling
with contrast, stenosis and the stop.
(Figure 27) Double contrast barium
enema uses barium, but also delivers
air into the colon to expand it. This
allows a better view of the contour of
the colon lining. Diagnosis of small
lesions is possible using this method
but depends heavily on the
experience and skill of the radiologist performing the investigation. The contrast
enema allows the visualization of the entire colic frame giving important
information about the shape and size of the colon and may highlight
synchronous tumors. Even though the patient more easily accepts it, the main
drawback is that it cannot directly visualize de tumor and cannot perform
biopsies. In addition, the result of the investigation depends on the experience of
the examiner.
Virtual colonoscopy is an easier way to highlight the colon tumors. In fact, it is a
CT scan of the abdomen where seriated images are processed using a special
software resulting a three dimensional aspect of the entire colon. The main
drawbacks of the method are: small lesions (less than 2 cm) may not be detected,
radiation of the patient (the method uses X-rays) and it cannot perform biopsies
so that the patient should undergo colonoscopy if lesions are found.
Endoscopic capsule is a new method of investigation of the entire digestive tract.
It is very convenient for the patient as it is not invasive. Unfortunately, the price
is still high. On the other hand, a specialist must carefully watch the entire film,
which can take several hours.
These investigations are designed for detecting the colorectal tumor, but there are
other very important investigations especially used to assess the relations of the
tumor with neighboring organs and its spread.
Abdominal ultrasound is a non-invasive investigation, which can highlight the
liver metastases and the presence of ascites. Other intra-abdominal co-
morbidities can be found using this investigation. Experienced examiners can
also detect the colon tumors.
Abdominal CT scan is recommended mainly for the purpose of differential
diagnosis with other abdominal tumors and for tumor relations with adjacent
structures highlighting the penetration. (Figure 28)
Intravenous urography is used when penetration into the ureter or bladder is
suspected. It highlights a possible solitary functioning kidney.
Upper digestive endoscopy is indicated in the presence of the suspicion that the
stomach or duodenum is penetrated by the tumor.
Chest radiography highlights the pulmonary metastases and associated
pathology.
Laboratory test are those commonly used for every surgical patient (complete
blood count, glucose, creatinine, urea, hepatic tests, coagulation tests, proteinemia,
blood group, etc.). Anemia is found in most patients especially in those with right colon
189
cancer. Even not specific for colon cancer, the
Carcino Embryonic Antigen (CEA), is
measured before operation because its value is
useful for assessing the radicality of the
surgery and for early detection of recurrences
and metastases. An ECG and other
investigations, depending on associated
pathology, are necessary in preoperative
period.
The positive diagnosis is based on the
triptych: patients history, physical
examination and investigations findings.
Patients history is of great importance. Not
only the data about the current medical history
are valuable but also those related to family
history. The presence of colorectal cancer in
relatives of first and second degree raises the
suspicion of a genetically modified
background and investigations will be targeted
in this direction. Total colonoscopy is
recommended in these cases to find any
synchronous tumors or polyps. Colicky
abdominal pain, with or without bowel
disorders, fatigue and weight loss are the most
common symptoms. Clinical examination, although in most uncomplicated cases does
not provide much information, must not be minimized because it can detect various
other associated conditions. The pallor, present mainly in right colon cancers, attracts
the attention. Colonoscopy directly visualizes the tumor and can perform biopsies for
histopathological diagnosis. The diagnosis is not complete without mentioning the
histopathological type and evolutionary stage, but preoperative staging is difficult
except when distant metastases are present (the forth stage in TNM classification or D
in MACs classification). The precise staging is possible only after getting the
histopathological result.
Differential diagnosis should include other conditions that may give similar clinical
picture depending on the tumor location.
Thus, for tumors located in the cecum and ascending colon the differential
diagnosis should include: periappendicular block, right kidney ptosis, Crohn's disease,
tumors of the abdominal wall, retroperitoneal tumors, ileocolic intussusceptions, cecum
volvulus, and anemia from other conditions.
For tumors located on the hepatic angle of the colon, differential diagnosis
includes: biliary stones, right kidney tumors, right liver lobe tumors, duodenal ulcer,
right renal colic and other conditions manifested by right upper quadrant pain.
For transverse colon tumors, the differential diagnosis is more difficult because of
the excessive mobility conferred by the long mesocolon. The following conditions
should be considered: gastric tumors, peptic ulcer, pancreatic tumors, acute and chronic
pancreatitis, retroperitoneal tumors, intestinal volvulus, Crohn's disease, utero-adnexal
diseases.
190
The splenic angle of the colon is the deepest segment. Differential diagnosis
includes: tumors of the pancreatic tail, splenic abscess and infarction, gastric tumors,
left adrenal gland tumors, left kidney pathology, retroperitoneal tumors, etc.
For the descending colon tumors most often diverticulitis will be considered, then
left kidney pathology and retroperitoneal tumors.
Due to the long mesosigmoid, tumors located on the sigma have a great mobility
with symptoms borrowed from nearby organs. Differential diagnosis will consider: left
reno-ureteral colic, left adnexal inflammatory disease or tumors, diverticulitis, sigmoid
volvulus, etc.
Generally, in any location of the cancer on the colic frame, differential diagnosis
should include other large bowel pathology such as colon polyps, ulcerative colitis,
Crohn's disease, synchronous tumors, enterocolitis, diverticulitis, etc.
In the presence of intestinal occlusion, even though the patient will undergo
surgery, the differential diagnosis between upper and lower occlusion is still important.
When blood is present in stool, other causes of digestive bleeding should be considered
such as peptic ulcer or gastric cancer, small bowel tumors, diverticulosis, ulcerative
colitis, angiodysplasia, hemorrhoids, anal fissure, overdosing of anticoagulant drugs,
etc.
Treatment
Colic cancer treatment is a complex treatment where the surgery has the principal
role. In addition to tumor excision patient will benefit from adjunctive oncological
treatment (chemo-immuno-radiotherapy).
Preoperative preparation. Before surgery, the patient must be brought in the
best physical and mental condition. Preoperative preparation is both local and general.
General preparation considers the associated illnesses, hydro-mineral imbalances,
anemia, hypoproteinemia, etc. Local preparation is addressed to the colon. The aim is to
evacuate completely the colon and to decrease its septicity. Colon emptying
(mechanical preparation) can be achieved by the so-called washout procedure.
The washout is a rapid way of colon emptying and presumes ingestion, on the day
before the operation, of a quantity of a fluid that causes watery diarrhea. The
composition of the ingested solution has been improved over the years so that it has
only a mechanically effect not being reabsorbed from intestines, does not produce
irritation, does not cause swelling of the intestinal wall, is not altered by intestinal flora,
and does not modify the biological parameters. Fortrans, Picoprep, Movicolon are the
best-known solutions.
Prevention of infectious complications is
achieved by administering pre-, intra- and
postoperative of a third generation cephalosporin
associated with antibiotics for anaerobic flora.
Preoperative mechanical preparation is possible
only in patients without occlusive complications. In
emergency conditions, the vast majority of patients are
hospitalized for intestinal obstruction, so this method is
not applicable. However, intraoperative anterograde
lavage of the colon can be performed. The saline
solution is introduced through a tube placed in the
cecum or ileum and then harvested through an opening
in the colon above the tumor. (Figure 29)
191
In recent years, there is a new trend in applying the ERAS (enhanced recovery
after colorectal surgery) principles, which advocate no colon preparation before
operation for better results and faster recovery of the patient.[64-66]
Surgery
Despite of all progresses in adjuvant therapy surgery remains the main treatment.
Surgical treatment of the colon cancer is well standardized. In curative intention
surgery, the colon tumor and the efferent lymph nodes are removed but unfortunately,
there are many advanced cases when only palliative surgery can be performed.
Treatment guidelines based on staging:[67]
1. Stage 0
a. Local excision or polypectomy
b. Colon resection for larger lesions and lymphadenectomy
2. Stage I (Dukes' A or B1)
a. Extended colon resection and lymphadenectomy
3. Stage II (Dukes' B, B2 or B3)
a. Extended colon resection and lymphadenectomy
b. Adjuvant treatment should be considered in these patients in two
situations:
i. In patients with colic cancers with a high degree of risk of
recurrence, such as those with tumor stenosis, perforated or
penetrating into adjacent structures (B3).
ii. In those patients who enter into a study of the effects of adjuvant
therapy, and will be closely monitored. For the other patients in
stage II, the adjuvant therapy has not brought certain benefits.
4. Stage III (Dukes' C1-C3). In this stage, there are present metastases in lymph
nodes (the number of involved lymph nodes is an important prognostic factor).
a. Wide surgical resection and lymphadenectomy
b. Postoperative treatment with chemotherapeutic agents and
immunostimulants
5. Stage IV (Dukes' D-Turnbull). Studies have shown that liver resections for
limited metastases may improve the prognosis of patients in this stage.
a. More or less extensive palliative resection or derivations (internal or
external)
b. Resection of the isolated metastases (liver, lung, ovarian)
c. Intra-operative intra-arterial chemotherapy in liver metastases
d. Palliative radiotherapy
e. Palliative chemotherapy
Lymphadenectomy is indicated even in small lesions because lymph nodes
metastases are always possible and sentinel lymph node biopsy is not yet a very well
standardized and widely applied procedure. On the other hand, during the operation, it is
almost impossible to assess the involvement of lymph nodes.

192
Tumor removal
Operations can be performed by open surgery or
by laparoscopic approach. In classic approach, after
laparotomy follows the local, regional, and general
inspection of the peritoneal cavity to assess the
resecability of the tumor and the presence of distant
metastases. A special attention will be given to the liver,
which will be thoroughly inspected and palpated,
because it is the most frequent site for metastases.
(Figure 30) In laparoscopic approach, initially the
pneumoperitoneum is achieved by introduction of
carbon dioxide, and then follows the exploration and
finally the tumor removal. In this case, the surgeon
relies only on the optical image because palpation is not
possib
-mentioned limits are exceeded. There are
two types of operations that can be applied:
nd
re extensive operations, applied especially for
synch
s, resection of colon,
and fi
ed from
the left side toward the right side. (Figure 31)
le.
The principles of tumor removal are represented by a more or less extended colon
resection associated with regional lymph nodes excision, followed by restoration of the
digestive tract continuity by various types of anastomoses. Generally, is considered that
resection should be performed at 5 cm under the tumor (downstream) and 15 cm above
it (upstream), but the surgery of the colon is dictated mostly by its vascularization. This
is the reason why in many cases the above
1. Curative operations, a
2. Palliative operations
A. Curative operations are aimed to completely remove the tumor along with
regional lymph nodes stations. The type of operation depends on tumor location. For the
right colon tumors, the operation is represented by the right hemicolectomy and for the
left colon tumors by the left hemicolectomy. Resections that are more limited are
segmental colon resections (Reibards operation) which can be applied when the tumor
is located on the mobile segments of the colon: the transverse colon or the sigma.
Subtotal and total colectomy, a
ronous tumors or polyposis.
Turbull introduced the no-touch isolation principle in the hope that it will prevent
further spread of tumoral cells during operation.[68] No-touch isolation means that the
tumor will be the last thing touched during the operation. It is believed that tumor
manipulation, by squeezing maneuvers, facilitates the s
the lymphatic and especially the blood vessels toward
the liver. This principle presumes: first ligation of
colon above and below the tumor to prevent
intraluminal spreading, then ligation and resection of
drainage veins to prevent spread over the venous
system, dissection of lymph node
pread of cancerous cells along
nally removal of the tumor.
Right hemicolectomy starts with the incision of
the posterior peritoneum along the right side of the root
of mesentery. The ileocolic, right colic and descending
branch of the mid colic vessels are resected and
ligated. The lymph nodes dissection is conduct
193
The classical right hemicolectomy presumes the removal of the right colon and
the first third of the transverse colon and also the last 20 cm of the ileum. The digestive
tract continuity will be restored by ileocolic anastomosis, the so-called
ileotransversostomy. The greater omentum will be divided up to the resection site of the
transverse colon. Then the gastrocolic ligament and the right phrenicocolic ligament are
also divided freeing up the hepatic angle of the colon. By this maneuver, the duodenum
is exposed and protected against damages. The mesocolon is transected and the
ascending colon is detached from the Told I fascia taking care of the right ureter. Then
the ileum and the transverse colon are transected and the right colon with corresponding
greater omentum is removed. The ileotransversostomy can be performed in various
ways: by side-to-side or end-to-side or end-to-end anastomosis. To prevent future
possible internal hernias, the breach between the mesentery and mesocolon is closed by
sutures. (Figure 32)

A variant of this operation is the extended right hemicolectomy in which the
transverse colon is resected in its left third (the first two thirds are removed). This
operation is applied when the tumor is located on the hepatic angle.
If the tumor penetrates other important organs such as the kidney, duodenum,
stomach, gallbladder, right hepatic lobe or pancreas, complex operations would be
necessary (right nephrectomy, gastric resection, cephalic duodenopancreatectomy, etc).
(Figure 33 and 34)

Left hemicolectomy. After ligation of the colon below and above the tumor, the
next maneuver will be the ligation and resection of the inferior mesenteric vein. This
vein can be easily discovered in the retroperitoneum, left to the Treitz angle of the
194
duodenum. Lymphatic dissection goes from the right side (th
root) toward the left side. (Figure 35) Depending on the
position of the tumor, the inferior mesenteric artery will be
resected or not. (Figure 36) For example if the tumor is
located on the sigmoid colon the artery should be resected
but when it is located on the splenic flexure, only the left
colic artery resection would be enough. Resection of the
inferior mesenteric artery at its root allows the removal of
the central lymph nodes. The colon resection is more or
less extended depending on tumor position and length of
the transverse colon. The continuity of the digestive tract
will be reestablished by colorectal anastomosis or
colocolic anastomosis. If the transverse colon is short, it
can be descended to the rectum using a more direct route
through the mesentery (transmesenteric). (Figure 36) The
most frequent possible intraoperative incident is the lesion
of the inferior pole of the spleen.
Tumors located on the s
e left side of the mesentery
igmoid loop can penetrate
other organs such as small intestines, bladder, uterus, or
adnexa, so that complex operations are often necessary (segmental resection of the
small intestine, partial resection of the bladder, adnexectomy or even total or subtotal
hysterectomy). (Figure 37)

Segmental resection of the colon is
applie
tumor
tal colectomy presumes the removal of almost the entire colon but usually
the sigma or a part of it is preserved. This type of operation can be applied for
d especially in elderly when the tumor is
located on the transverse or sigmoid colon.
This type of operation is not as radical as the
right or left hemicolectomy but still provides
good results. The mesocolon or mesosigma is
resected in a V shape. The continuity is
restored by colocolic anastomosis. (Figure 38)
Colorectal resection is applied when the
is located on the lower part of the
sigmoid colon. The sigma and the upper part
of the rectum are removed followed by colore
operation.
Subto
ctal anastomosis, the so-called Dixons
195
synchronous tumors located on the right and transverse or descending colon. The transit
is reestablished by ileosigmoidostomy. This operation may be also a solution in
stenosing tumors of the left colon with intestinal occlusion, with or without diastatic
perforation of the cecum. (Figure 39 and 40)

Total colectomy represents the
removal of the entire colon followed by
ileore
tases, their
removal is beneficial for the patient

by intestinal occlusion. The
first t e occlusion and to rebalance the patient. The
initial
after a short and intensive rebalancing. As it was mentioned
above
ctostomy. This is an extreme solution
applied especially in colon polyposis or
multiple synchronous tumors, located both
on the right and on the left colon. (Figure 41)
The attitude toward liver metastases
encountered during operation
In case of limited number of liver
metastases or clustered metas
prolonging survival.[69-71] Possible
operations are: metastasectomy (ablation of
metastases) liver resections, destruction of meta
physical methods (electrocautery, diathermy, ul
Surgical solutions in emergency
Most emergencies of colon cancer are represented
stases using chemical (ethanol) or
trasound).
arget of treatment is to remove th
treatment is always conservative. A nasogastric tube will be inserted for gastric
decompression, a central venous catheter for measuring the central venous pressure and
administration of fluids for hydro electrolytic and acid-base rebalancing, antispastic and
anti-inflammatory drugs to reduce the peritumoral edema, and repeated enemas will be
performed. In many cases, these conservative measures succeed in removing the
occlusion enabling a better preoperative investigation and preparation of the patient. In
this case, the emergency operation is postponed and it will be performed as in elective
cases.
If conservative methods do not succeed in resuming the intestinal transit, the
patient will be operated
, the first intention is to remove the occlusion, which is the best solution for
patient reanimation. Applying different surgical solutions depends much on patients
status (age, co-morbidities, and degree of health status impairment) and tumor location.
The minimal surgical interventions are represented by temporary external diversions:
196
colostomy or ileostomy performed upstream the tumor. There are many types of
colostomies but the most frequently applied are the loop colostomy or cecostomy.
(Figure 42) Loop colostomy is performed above the tumor, on the sigmoid or transverse
colon. Cecostomy or ileostomy is preferred when the tumor is located on the right
colon. Even though these procedures remove the occlusion, they represent only a
temporary solution. After a while, the patient must be reoperated to remove the tumor as
in elective operations. There are rare cases when colostomy or ileostomy represents the
definitive operation, namely when the tumor is unresectable. Another solution is
represented by subtotal colectomy especially in cases of splenic flexure colon cancer
with very dilated upstream colon, even with diastatic perforation.[72-74]

Hartmanns operation represents a seriated operation applied in emergency cases
with intestinal occlusion. The Hartmanns I procedure represents the removal of the
tumor
ed in patients with advanced stages of colon
cance rs or with distant metastases. Even if the tumor is
resect
occlusion,
al segment of the colon so that the distal end is closed and the proximal end is
exteriorized in terminal
colostomy avoiding thus the
anastomosis which is at high
risk of dehiscence in these
patients with very distended
colon. After a period from the
first operation (3- 6 months),
the continuity of the digestive
tract can be reestablished by a
second operation, the so-called
Hartmanns II operation.
(Figure 43)
B. Palliative operations
Palliative operations are perform
r, with unresectable tumo
able but the patient has distant metastases, the operation is still palliative. There
are also cases when, even if the radical operation is possible, from technical point of
view, the patients cannot withstand it because of the associated illnesses, and thus only a
palliative operation is the solution for that patient. The aim of these operations is to
prolong the patients life and to create conditions for a better quality of life.
In most cases, tumors are penetrating into other neighboring organs so that it
cannot be removed. To avoid further complications such as intestinal
197
differe
hese are the simplest palliative operations
with
ented especially by the terminal
am the

nt intestinal content to reach the suture line
the d
for tumors located
on the
major differences comp
nd energetic rebalanc
proph
ient is sent back to
o be started on the same day as operation
g high energy/high
nt kind of procedures are available which can be categorized as external
diversion and internal diversion operations.
External diversions are represented by colostomies (performed upstream the
tumor), ileostomy or cecostomy. (Figure 44) T
a low risk for the patient. External diversion can be performed for different
reasons.
1. Definitive external diversion. It is
repres
colostomy of Hartmanns stage I
operation or after rectal amputation for
rectal cancer. Other situations are
represented by unresectable tumors when
an upstream colostomy is performed to
resolve or prevent the occlusion.
2. Temporary external diversion. The
colostomy is performed upstre
tumor as a first stage operation resolving
the occlusion and then, after a while, the
performed and the colostomy is closed.
3. Colostomy or ileostomy of protection. These stomas are performed upstream a colo-
colic or colo-rectal anastomosis to preve
main operation of tumoral removal is
and thus preventing anastomotic dehiscence. These stomas are also temporary.
Internal derivations represent
anastomosis between different segments of
igestive tract in order to bypass an
unremovable obstacle (in this case
represented by the tumor). These operations
prevent intestinal occlusion and thus prolong
life.
The most frequent internal derivations
are ileotransversostomy
right colon, and ileosigmoidostomy for
tumors on transverse and descending colon.
(Figure 45)
Postoperative care
There are no ared to other surgeries on the digestive tract.
ing, pain relief drugs, thromboembolism Fluid, electrolytic, a
ylaxis (early mobilization, anticoagulants), antibiotherapy with broad spectrum
antibiotics, wound dressing, and other measures, represent postoperative care. Enteral
nutrition is resumed according to digestive tolerance.
ERAS (Enhanced recovery after colorectal surgery) principles:[64-66]
Nasogastric tube will be removed in recovery before pat
the ward
Daily physiotherapy and mobilization on day of surgery
Soft diet t
One liter of free fluid on the day of operation includin
protein nutritional supplements
198
Urinary catheter remains no longer than 24 hours following epidural removal
If patient has had epidural this should continue for a minimum of 48, aiming
stoperative complications are more frequent and
inal operations, due to the neoplastic disease, relatively
poor b
severe
clusion caused by intra-
abdom
tried to identify the specific and most significant
factors for colon cancer, establishing that the most important are: depth of
the tu
tly way to improve this prognostic.
(Table
Stage TNM Group Group Dukes Prognosis
for removal postoperative day 2
Discharge planning from postoperative day 2
Stoma care if necessary
In colon cancer surgery, po
severe than after other abdom
lood supply of the colon, and its septic content. Surgery of the colon is a risky
surgery (infectious, anastomotic and oncologic risk).
The most dangerous and life threatening
complication is the anastomotic dehiscence with
peritonitis and respiratory, heart, and
kidney complications. In most cases, reoperation
is necessary as soon as possible. Other possible
early complications are wound suppuration,
bleedings, evisceration, intestinal occlusion, colon
prolapse, and others.
Possible late complications are incisional
hernia, intestinal oc
inal adhesions, colostomy prolapse (Figure
46), local recurrence, and others.
Prognosis
A large number of studies
prognostic
moral penetration, number of lymph nodes with metastases, the degree of
histological tumor differentiation, blood or lymphatic vessels invasion and residual
tumor following surgery with curative intent.[75-77]
The higher stage of tumor, the more reserved is the prognosis, making early
detection through screening programs the best curren
4)
Table 4 - The five-year survival rates prognosis depending on colorectal stage. [78]
5 year survival
T1 N0 M0 Dukes A >90% Stage I

T2 N0 M0
T3 N0 M0 Dukes B 7 0-85% Stage II
T4 N0 M0 55-65%
a ny T N1 M0 Dukes C 45-55% Stage III
a N ny T 2, N3 M0 20-30%
Stage IV any T any N M1 Dukes D < 5%

199
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Rectal Cancer
RECTAL CANCER

1. Surgical anatomy of the rectum
2. Rectal cancer

1. Surgical Anatomy of the Rectum
The rectum is the portion of the digestive tract located between the sigmoid colon
and anal canal. The recto-sigmoid junction, measuring 2-6 cm, is considered by some
authors as part of the colon, whereas others include it in the rectal pathology. In this
area, colic taenias disappear forming a continuous layer and the circular muscle fibers
form a sphincter. The rectum is about 13 cm long with a variable caliber depending on
its fullness.
Anorectal junction is represented by the anorectal line, the dentate or pectinate
line, characterized by the presence of Morgagnis columns, which are located between
the anal valves. The anorectal line represents not only the boundary between the rectum
and anal canal, but also between the visceral and somatic innervation, between the
portal and caval system, and between the visceral and somatic lymphatic system at this
level. (Figure 1)
The rectum is divided in three segments:
2. The upper or pelvic segment, which is
covered by visceral peritoneum on its
anterior and lateral surface. It is the
intra-peritoneal segment.
3. The middle rectum, which is covered by
peritoneum only on its anterior aspect.
The peritoneum reflects on the uterus,
forming the bottom of the recto-uterine
sac of Douglas in women and the
bottom of recto-bladder sac in men.
4. Distal rectum, the third portion, is
entirely subperitoneal. Posterior there is
the mesorectum, which contains blood
vessels, lymphatic nodules and nerve
fibers. In radical surgery of the rectum
for cancer, the mesorectum is also
removed along with the rectum (total
mesorectal excision). Between prostate
and rectum, there is the fascia of
Denonvilliers, which facilitates dissection. In women there is a recto-vaginal fascia
ending down with the recto-vaginal septum described by Proust Barbilian.
Supportive anatomical elements of the rectum are represented by:
The pelvic floor formed by the levator ani muscles
Presacrate fascia of Waldeyer
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Rectal Cancer
Lateral ligaments of rectum which are formed by the condensation of pelvic
fascia representing the stalks or pedicles of the rectum
Fascia of Denonvilliers, which connects the rectum to the posterior surface of
the seminal vesicles and prostate
Pelvic peritoneum
Relations (Figure 2)
Anterior relations in man are represented by the prostate, seminal vesicles,
deferens ducts and urinary bladder, and through the pelvic peritoneum, by the
small intestines. In woman, anterior relations are with the vagina, uterus, adnexa
and small intestines.
Posterior relations are with the sacrum, coccyx, sacral vessels, sacral
sympathetic chains, branches of the 3
rd
and 4
th
sacral nerves, and the superior
hemorrhoidal vessels.
Lateral relations: the intraperitoneal segment has relations with the sigmoid,
small bowels, uterine adnexa, and the subperitoneal segment with the
hypogastric plexus and branches of internal iliac (hypogastric) vessels.

The anal canal is the terminal portion of the alimentary tract. It has a length of 2-
3 cm. It is a straight, the shortest and the most fixed part of the terminal bowel.
Anatomical aspects of the inner surface of the lower rectum and anal canal are
represented by:
Morgagnis columns, varying in number from 6 to 14
Anal valves (semilunar envelopes) with anal papillae
Anal sinuses or cripts
Pectinate or dentate line separating the anal canal from the rectum
Linea alba (white line) of Hilton, a delineation between the internal and external
anal sphincters
Anal musculature is represented by:
The internal anal sphincter (smooth muscle), a thickening of the rectal circular
muscular layer, which surrounds the anorectal junction. It ends at the white line.
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Rectal Cancer
The external anal sphincter (striated muscle), which surrounds the completely
the anal canal and consists of three parts: subcutaneous, superficial and deep.
The upper half of the anal canal is lined by mucosa and the lower half is lined by
stratified squamous non-keratinized epithelium. This explains the different kind of
histological types of cancers between those two segments.
Arterial supply of the rectum (Figure 3) is
represented by:
Superior rectal (hemorrhoidal)
artery, terminal branch of the inferior
mesenteric artery, which divides into
two branches
Middle rectal arteries, coming from
the internal iliac artery
Inferior rectal arteries, below the
levator ani muscles, come from the
internal pudendal artery
Middle sacral artery, arising from the
back of the aorta at one centimeter
above the bifurcation and goes in
front of the sacrum and coccyx
Rectal arteries anastomosize between
them, forming thus an intra- and extra-
parietal anastomotic system, sufficient to
restore the blood supply after a rectal
resection.
Rectal veins originate from rectal venous plexus located in the submucosa layer. From
this plexus, venules start crossing the muscular layer thus creating the rectal or
hemorrhoidal veins.
1. Superior rectal vein collects the blood from the rectal ampulla and flows into the
inferior mesenteric vein (portal
system).
2. Middle rectal veins are thin, leaving
the lower portion of the rectal
ampulla and flowing into the
internal iliac veins toward the
inferior cava vein.
3. Inferior rectal veins collect blood
from the lower rectum and anal
canal and flow into the internal
pudendal veins, which flow then
into the internal iliac vein (caval
system).
Thus, a communication, of important
clinical value, between the portal system
and caval system is established through
these rectal veins (see the portal
hypertension). (Figure 4)
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Rectal Cancer
There are four physiologic portacaval shunts:
1. Between the superficial and inferior epigastric veins, represented by the
paraumbilical veins. Dilatation of these veins gives the aspect of caput
medusae in portal hypertension.
2. Between the superior rectal vein and the inferior and middle rectal veins. In
portal hypertension, the consequence could be the developing of symptomatic
hemorrhoids.
3. Between small posterior veins of the colon and lumbar veins and renal venous
branches.
4. Between the azygos system (dependant to the superior cava vein) and the gastro-
esophageal veins belonging to the portal system with the consequence of gastro-
esophageal varices in case of portal hypertension.
The lymph is drained from the mucous and submucous plexuses towards three
pedicles:
1. Superior lymphatic pedicle which
has four lymph node stations:
a. The first station is located on the
posterior rectal wall, described by
Gerota
b. The second station located at the
bifurcation of superior
hemorrhoidal artery, described by
Mondor, also receiving short
lymphatic pathways from the
anus
c. The third station (Bacon) is
located in front of rectosigma at
the junction between
hemorrhoidal and sigmoidian
arteries, also known as the
"lymphatic mesenteric hilum "
d. The fourth station is located at the
origin of the left colic artery.
2. Lateral lymphatic pedicle includes all the lymph nodes of the pelvis, as follows:
a. Inferior lymph-node-station, located near the middle and lateral sacral
vessels
b. Lateral lymph-node-station, consisting of nodules along the middle
hemorrhoidal and hypogastric vein
c. Anterior lymph-node-station receiving lymph from recto-urethral muscle
3. Inferior lymphatic pedicle consists of lymphatics that drain into the groin lymph
nodes.
Innervation (Figures 6 and 7)
Sympathetic fibers of the rectum are derived from the first three lumbar spinal
segments, which pass through the sympathetic ganglion chains and leave them as
lumbar sympathetic nerve reaching the preaortic plexus. From here, there is an
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Rectal Cancer
extension along the inferior mesenteric artery called the inferior mesenteric plexus,
which reaches the lower portion of the rectum.
Presacral or hypogastric nerve comes from the aortic plexus and lateral lumbar
splanchnic nerves. The trunk thus formed is divided into two branches, which pass on
either side of the pelvis where branches meet the sacral parasympathetic nerves to form
the pelvic plexus. This innervates the lower rectum, anal canal, bladder and sexual
organs.
Parasympathetic innervation comes from erigens nerves, which originate from
sacral nerves 2, 3 and 4.
Innervation of the anal canal
Motor innervation
Internal sphincter has both sympathetic and parasympathetic nerve supply.
Sympathetic system has a stimulatory effect whereas the parasympathetic an
inhibitory effect of the sphincter.
External sphincter is innervated by the inferior rectal branch of the internal
pudendal nerve and by the perineal branch of the fourth sacral nerve.
Sensory innervation
The skin of the perianal region and anal canal is innervated by fibers of the
inferior rectal nerves. Sensory fibers are concerned with the reflex control of the
sphincters and with pain. The anal canal is very sensitive below the pectinate
line, so that external hemorrhoids may be very painful when complicated.

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Rectal Cancer
2. Rectal Cancer
The incidence, epidemiology, and etiopathogenesis of rectal cancer are the same
as for colon cancer. For the etiology of the anal cancer, some special features should be
highlighted. Squamous cell carcinoma (SCC) of the anal canal is frequently associated
with chronic HPV infection (sexually transmittable human papillomaviruses).[1-5] A
strong association with tobacco smoking has been established in women, but the role of
smoking in men is less clear.[1,5-7] Hemorrhoids, anal fissures, fistulae, abscesses and
Crohns disease of long duration in the anal region are no longer considered
predisposing factors.
The macroscopic aspect of the tumor can be ulcerative, infiltrative or vegetative.
Penetration into adjacent organs is rapid and more frequent as in colon cancer due
to the close relations with these organs.
From histopathological point of view, most cases are represented by
adenocarcinomas, (98%) but other forms are also possible, such as carcinoid (0.1%),
lymphoma (1.3%), sarcomas (0.3%). Epithelioma (squamous cell carcinoma) and
melanoma appear especially in anoperineal region.[1]
The TNM classification (AJ CC):
Primary tumor (T) (Figure 8)
TX - Primary tumor cannot be assessed or depth of penetration not specified
Tis - Carcinoma in situ (mucosal); intraepithelial or invasion of the lamina propria
T1 - Tumor invades submucosa
T2 - Tumor invades muscularis propria
T3 - Tumor invades through the muscularis propria into the subserosa or into
nonperitonealized pericolic or perirectal tissue
T4 - Tumor perforates the visceral peritoneum or directly invades other organs or
structures
Regional lymph nodes (N)
N1 - Metastasis in 1-3 pericolic or perirectal lymph nodes
N2 - Metastasis in 4 or more pericolic or perirectal lymph nodes
N3 - Metastasis in any lymph node along the course of a named vascular trunk
Distant metastasis (M)
MX - Presence of metastasis cannot be assessed
M1 - Distant metastasis

Adenocarcinomas of the rectum and anal canal are staged completely
differently. The T category of colorectal cancer is defined by extent through the wall,
208
Rectal Cancer
whereas the T category of anal canal cancer is defined by tumor size. The N category of
colorectal cancers is defined by number of affected nodes, whereas the N category for
anal cancers is defined by the location of affected nodes.[8]
Symptoms
The clinical picture of the rectal cancer depends on tumors development stage. In
the initial stage, the patient is asymptomatic for a long period. The tumor can be
discovered incidentally on endoscopic or rectal examination. In the phase of state,
symptoms are represented by:
Rectal bleeding which is the most frequent symptom (60% of cases). Other
pathological rectal discharge may be associated such as mucus and pus.
Confusion is often made with bleeding from hemorrhoids. Bleeding from
rectal cancer is rarely massive and the blood is mixed with stool. Sometimes
bleeding manifests as blood clots like a jelly or the fecal bolus is marked by
a trail of blood. Chronic bleeding will lead to anemia.
Pain is manifested initially as a local rectal embarrassment, intrarectal foreign
body sensation, or incomplete evacuation. In advanced stages, pain becomes
more and more intense associated with tenesmus.
Change in bowel habits is represented by diarrhea, particularly if the tumor
has a large villous component. Some patients experience a change in caliber
of the stool (thinner) and the large tumors can cause obstructive symptoms.
. In advanced stages, symptoms are represented by:
Intense pain - caused by sacral penetration
Colicky pain - caused by tumoral obstruction
Hypogastric and perineal pain - caused by penetration in the pelvic
organs
Anal incontinence
Recto-vaginal, recto-bladder or recto-uterine fistula
J aundice due to liver metastases
Neoplastic impregnation signs (asthenia, malaise, anemia, weight loss,
etc.)
Compressive edema of the legs and genital organs
Physical examination
Digital rectal examination is one of the most important methods of diagnosis in
rectal cancer and the cheapest. Remember that 75% of rectal tumors can be detected by
digital rectal examination! (tumors located up to 10-12 cm from anal verge). However,
one study considers that digital rectal examination is an inaccurate procedure and a poor
predictor for palpable rectal tumor.[9]
The following features of the tumor, found at rectal digital examination, should be
described:
The distance from the anal verge of the lower margin
The exact position on the rectal wall (anterior, posterior, lateral or circular)
Tumor dimensions (extension)
Tumor sensitivity
The surface of the tumor (ulcerated ?)
Tumor delimitation
Tumor mobility - very important to asses the penetration
Bleeding or not- look at the finger glove
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Rectal Cancer
Groin lymph nodes should be palpated because in lower location of the cancer,
these lymph nodes could be enlarged because of metastases.
In women, vaginal digital examination is of important value in assessing the
penetration of rectal cancer into the posterior wall of the vagina.
Investigations
Rectoscopy with biopsy is the most important investigation (for tumor with
higher location, rectosigmoidoscopy would be necessary).
Endorectal (transrectal) ultrasonography (EUS) (Figure 9) is useful for the
assessment of penetration through
the rectal wall, tumor relations with
surrounding organs and lymph
nodes metastases. It is 72-94%
accurate. [10-12]
CT or MR scan are performed for
the same reasons as above being
more precise but also more
expensive. Endorectal ultrasound
and magnetic resonance tomography
(MR) are the most adequate for
determining tumoral stage. MR is
highly accurate in predicting the
circumferential resection margin. Accurate node staging remains however
difficult with both EUS and MR. [13,14]
Abdominal ultrasound is useful especially for liver metastases and ascites.
Chest radiography is useful for detecting lung metastases.
Intravenous urography or cystoscopy is performed when a fistula with the
bladder or compression on ureters are suspected.
Carcinoembryonic antigen (CEA) test, even though is not specific for rectal
cancer, is performed in all patients. A baseline level is obtained before surgery
and then a follow-up level after surgery. If a previously normalized CEA begins
to rise (over 100 ng/mL) in the postoperative period, usually indicates metastatic
disease or local recurrence.
Differential diagnosis should include other anorectal diseases such as:
Hemorrhoids
Anal fissure
Ulcerative recto-colitis
Rectal syphilis
Crohn disease
Genitourinary diseases, and others
Treatment
The treatment is complex (multimodal): surgical and adjunctive oncotherapeutic.
The therapeutic priority depends on tumor location, stage and size.
In cancers located in the lower part of the rectum, initially preoperative
radiotherapy is indicated for tumoral reduction and then, after 4-6 weeks, follows the
surgery. Some authors advocate also for neoadjuvant chemotherapy for locally
advanced tumors.[15-17]
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Rectal Cancer
The operative technique varies depending on many factors among which tumor
location is primary. Other factors considered for the operative strategy are: tumoral
stage, age of the patient, other associated illnesses of the patient and the patient's
wishes.
The most important dilemma of the rectal surgery is to choose between a radical
operation and an anal sphincter preserving operation. Of course, surgeons will try
whenever possible to save the anal sphincter but without compromising oncologic
principles.
Surgical treatment implies:
1. the removal (excision) of the tumoral rectum, and
2. lymphadenectomy (total mesorectal excision) for tumors in stage II-III
Extension of resection. It was found that the tumor does not infiltrate more than 2
mm from the macroscopic edge. However, at least 2 cm below the tumor should be
respected as a distance of security. The lower limit of the resection is dictated by the
position of the tumor and the distance from the anal verge.
Considering that, the anal canal is 2-3 cm long, plus 2 cm of security zone,
results a distance of about 5 cm from the anal verge, up to the inferior margin of the
tumor. Under this limit, in most cases, the anal sphincter cannot be saved and a rectal
amputation is the major surgical indication. An important step forward in sphincter
preserving surgery was made by the
introduction of staplers for colorectal
anastomosis that enabled lowering as much
as possible the anastomosis line near the
anus.
In anorectal cancers, the only
operation indicated is abdominoperineal
resection (rectal amputation).
Types of operations
Radical operations (Figure 10)
Low anterior resection with colorectal
anastomosis (Dixons operation) with or
without temporary colostomy or
ileostomy for anastomosis protection.
(Figure 11)
Ultralow resection with peranal or
intersphincterian anastomosis (Parks
operation).
Abdominoperineal resection or rectal
amputation (Miles operation) with
definitive left iliac anus (terminal
colostomy). (Figure 12)
Pull-through operations (Babckoc,
Bacon, Mandache, Chiricuta). (Figure
13)
Hartmanns operation
Endoluminal excision (in early stages)

211
Rectal Cancer
Palliative operations
Colostomy, cecostomy, ileostomy
Palliative resection (Hartmann I step)
Approaches may be:
Classic - median laparotomy, or transsacral (rarely applied)
Laparoscopy
Endoluminal (endoscopic)

Anastomoses can be handsewn or mechanical performed by staplers. Resection of
the rectum and especially the colorectal anastomosis is more difficult in men because of
the tight pelvis. Erector nerves should be spared whenever possible to prevent the
impotence.[18,19] However, the whole mesorectum containing lymph nodes should be
excised, the so-called total mesorectal excision (TME). The cleavage plane (the holy
plane), facilitates the total mesorectal excision. (Figure 14) [20]

Stapled anastomosis
The evolution of mechanical suture technology experienced a continuous
improvement over the 40 years of history. The use of staplers facilitated the anastomosis
technique allowing a lower rectal resection and anastomosis, avoiding rectal
amputations in many patients. Staplers can be used for any anastomosis but they are the
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Rectal Cancer
most useful in rectal tumors. For sphincter preserving procedures, tumors should be
located above 5-6 cm from the anal verge. Resecting 2 cm below the tumor will leave
an anorectal stump of only 3-4 cm, the minimum length necessary for mechanical
colorectal anastomosis. For tumors located under 5-6 cm the options could be
represented by:
Abdominoperineal resection
Colo-anal anastomosis (intersphincteric)
Pull-through procedures
Technical details: the same principles as in conventional surgery must be
respected (anastomosis without tension and a good arterial supply of the colon) to
prevent anastomotic leaks. It is important to verify the integrity of anastomosis by
verifying the integrity of the tissue ring of the cut edges, direct visualization, hydro-
pneumo verification (the pelvis is filled with saline solution and air is insufflated
through the anus; if air bubbles occur in saline it means that the anastomosis is not
perfect sealed), palpation and endoscopic visualization. (Figure 15)

Advantages of mechanical suture are:
It gives the possibility to perform safe anastomosis in anatomical areas
difficult to reach where the handsewn anastomosis is either impossible or
very unsafe and burdened by high risks.
It can save many patients from rectal amputation with the consequence of the
handicap caused by permanent iliac anus especially in patients with rectal
tumors, in obese male patients with narrow basin.
It decreases the operation time
It improves the suture quality
It decreases the intraoperative blood loss
It decreases the intraoperative contamination
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Rectal Cancer
Postoperative complications after Low Anterior Resection
Anastomotic fistula is the most dangerous and life threatening due to septic
shock after peritonitis. To prevent peritonitis, temporary colostomy or
ileostomy can be performed.
Wound suppuration, bleedings, etc., are not so difficult to treat but may
endanger the patients life.
Intestinal obstruction mostly caused by adhesions or internal hernias.
Transient urinary dysfunction secondary to weakening of the detrusor
muscle.
Sexual dysfunction is more prominent and includes retrograde ejaculation
and impotence. In the past, this complication occurred in 5-70% of men, but
recent reports indicate that the current incidence is lower. [21,22]
Abdominoperineal resection - Rectal Amputation - Miles operation (Figure 16)
This type of operations presumes the removal of the entire rectum and anal
sphincter plus the mesorectum containing the lymph nodes. It is a mutilating operation
because the patient will remain with permanent iliac anus. The main indication is
represented by anorectal cancer or very low rectal cancer. Usually, the patient will
undergo radiotherapy for 6 weeks before operation.
The operations can be performed either by classical or by laparoscopic approach.
There are two approaches: the abdominal one, when the rectum and mesorectum are
prepared down to the levator ani muscles, and the perineal approach, when the
anorectum will be completely freed up and removed.

Complex operations
Unfortunately there are many cases with advanced cancers when the tumor
invades other organs being necessary multiple visceral resections (when possible) the
so-called complex operations.

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Rectal Cancer
Transanal excision of rectal tumor (Figure 17) can be performed only if: [23-26]
1. The tumor is in stage 0 or I (Tis, T1)
2. The tumor does not occupy more than one third of the circumference of the
rectum
3. There are no pathological lymph nodes (verified by endorectal ultrasound)
4. The tumor does not involve the anal sphincter
5. Preferably the tumor to be of polypoid form
6. Rectal wall is excised in its entire thickness at least at 1 cm away from the
lesion being followed or not by suture of the subperitoneal rectum.

References
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2. Stanley MA, Winder DM, Sterling J C, Goon PK. - HPV infection, anal intra-epithelial neoplasia (AIN) and anal cancer:
current issues. - BMC Cancer, 2012 8; 12:398.
3. Machalek DA, Poynten M, J in F, Fairley CK, Farnsworth A, Garland SM, Hillman RJ , Petoumenos K, Roberts J , Tabrizi
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4. Centers for Disease Control and Prevention (CDC). - Human papillomavirus-associated cancers - United States, 2004-
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5. Daling J R, Madeleine MM, J ohnson LG, Schwartz SM, Shera KA, Wurscher MA, Carter J J , Porter PL, Galloway DA,
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101(2):270-280.
6. Daling J R, Sherman KJ , Hislop TG, Maden C, Mandelson MT, Beckmann AM, Weiss NS. - Cigarette smoking and the
risk of anogenital cancer. - Am. J . Epidemiol. 1992; 135:180-189.
7. Frisch M, Glimelius B, Wohlfahrt J , Adami HO, Melbye M. - Tobacco smoking as a risk factor in anal carcinoma: an
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9. Ang CW, Dawson R, Hall C, Farmer M. - The diagnostic value of digital rectal examination in primary care for palpable
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10. Ren J H, Guo FJ , Dai WD, Han XJ , Ma N. - Study of endorectal ultrasonography in the staging of rectal cancer. - Chin.
Med. J . (Engl). 2012; 125(20):3740-3743.
11. Kav T, Bayraktar Y. - How useful is rectal endosonography in the staging of rectal cancer? - World J . Gastroenterol.
2010; 16(6):691-697.
12. Radovanovic Z, Breberina M, Petrovic T, Golubovic A, Radovanovic D. - Accuracy of endorectal ultrasonography in
staging locally advanced rectal cancer after preoperative chemoradiation. - Surg. Endosc., 2008; 22(11):2412-2415.
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13. J uchems MS, Aschoff AJ . - Current imaging for rectal cancer. - Chirurg. 2009; 80(4):274-280.
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15. Aliev II, Guliaev AV, Pravosudov IV, Karachun AM. - Current principles and approaches to the treatment for patients
with locally advanced rectal cancer. - Vopr. Onkol. 2012; 58(2):203-206.
16. Sauer R, Liersch T, Merkel S, Fietkau R, Hohenberger W, Hess C, Becker H, Raab HR, Villanueva MT, Witzigmann H,
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Clin. Oncol. 2012; 30(16):1926-1933.
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Anoperineal Pathology
ANOPERINEAL PATHOLOGY

1. Surgical anatomy of the anus
2. Anal fissure
3. Hemorrhoids
4. Anoperineal abscesses
5. Fistula in ano

1. Surgical Anatomy of the Anus
Muscles of the anal canal (Figure 1)
Internal anal sphincter is a circular muscle continuing the muscular layer of the
rectum, with a length of 2.5 cm and a thickness of 2-5 mm. Permanent spasm of the
sphincter plays an important role in the persistence of the anal fissure and its evolution
toward chronic fissure.
Longitudinal muscle layer of the anal canal represents an extension of the
homonymous rectal muscle and fibers of puborectalis muscle. Its fibers pass over the
lower portion of the external sphincter and insert on the perianal skin. These fibers
create conditions for spread of perianal infection and defines restricted areas whose
distension is responsible for the intense pressure and pain in these infections.
External anal sphincter. Some of its fibers insert posterior on the coccyx, and
anterior on the perineal middle point in men, or they mix with vaginal sphincter fibers in
women. The major difference between the two sphincters is of fibers type component:
smooth for the internal and striated for the external sphincter. Between the two
sphincters there is a virtual space called intersphincteric space, which is important
because it contains 4-8 apocrine glands that can cause infections. It also represents a
dissection plan in surgical interventions on sphincters. Anal glands may also be the site
of origin of adenocarcinoma.
The lowermost portion of the external
sphincter is traversed by a fan shaped
expansion of the longitudinal muscle, which
splits it up, into 8 to 12 discrete muscle
bundles.
Puborectalis muscle plays a key role in
maintaining the angle between the anal canal
and rectum, which is essential in maintaining
continence.
The dentate (pectinate) line is the most
important anatomical landmark, both from
morphological and surgical point of view. It separates:
Upward:
Columnar epithelium
Autonomous innervation (no sensitivity)
Portal venous system
Internal hemorrhoids (not painful)
217
Downward:
Stratified squamous epithelium
Spinal innervation (very sensitive)
Systemic circulation
External hemorrhoids (painful)
Above the pectinate line, the mucosa is thrown into 8 to 14 longitudinal folds
known as anal columns of Morgagni. Two adjacent columns are connected below at the
pectinate line by anal valves (Ball). Morgagni's crypts (or anal crypts, or sinuses, or
saccules of Harner) are small pockets between the lower ends of columns with the same
name. These sinuses may be of some surgical significance as some foreign bodies may
lodge into them with resulting infection or trauma.
Anal papillae are more often absent than present, but when present, they do not
usually arise from the free edges of the anal valves or crypts as some suppose. They
correspond usually to the rectal columns of Morgagni. The tips of the papillae
frequently project above the lower margins of the rectal columns.
The perineopelvic spaces (Figure 2)
Perineopelvic spaces are directly concerned in surgical therapy especially of
perianal abscesses and fistula in ano. The perineopelvic spaces are:
1. The perianal space surrounds the anus and the lower third of the anal canal.
Laterally it continues with the ischiorectal fossa. Posterior, it is designated as the
post-anal space.
2. The submucous space, located above the anorectal line contains the internal
hemorrhoidal plexus of veins. This space is particularly important in
hemorrhoids development.
3. The ischiorectal space, located on the both sides of the anal canal and below the
pelvic diaphragm. Its base is oriented downwards to the surface and the apex
upwards. It contains ischiorectal fat, inferior hemorrhoidal veins and nerves
crossing transversely. Posterior, it is
crossed by the perineal and
perforating branches (coetaneous) of
the pudendal plexus and anterior by
the posterior scrotal or labial vessels
and nerves.
4. The supralevator (pararectal)
spaces located above the levator
muscle and below the peritoneal
reflections of the abdominal cavity.
5. The retrorectal (presacral) space
lies posterior to the rectum and
anterior to the sacrum and coccyx.

218
PATHOLOGY
The prevalence of anal diseases in the general population is probably much
higher than that detected in clinical practice because most patients do not seek medical
care because of shame.[1] The common anoperianal lesions are presented in the table
bellow (Table 1)[2]
Table 1 - Anoperineal lesions

2. Anal Fissure
The anal fissure is a tear in the mucocutaneous layer of the anal canal. (Figure 3)
It affects men and women equally and both the young and the elderly.
Due to the extremely sensitive anoderm, fissure causes severe pain during and
after defecation.
Causes
Anal fissures are caused most frequently by trauma to the anal canal by a hard
stool or repeated episodes of diarrhea.
Other traumas that can cause anal
fissures are: introducing the anal
thermometer, enema cannula, ultrasound
probe, other foreign bodies, rectoscopy,
digital rectal examination and anal sex.
Other potential causes of fissures are:
Anal cancer
Crohn's disease (4% of patients
will have an anal fissure as the
first manifestation) [3]
Leukemia
Tuberculosis
Viral infections: cytomegalovirus
219
or herpes, syphilis, gonorrhea, Chlamydia, chancroid (Hemophilus ducreyi),
and human immunodeficiency virus (HIV)
Some drugs (antiparkinsonian) may induce constipation and others
(Nicorandil - vasodilator) directly anal ulceration.[4]
Fissures usually occur in stressed people or psychologically labile and sedentary.
Also, people who travel a lot (drivers, sales representatives, etc.) and do not have a
regular stool, are prone to constipation.
Location
The most common location of the anal
fissure is the posterior region (90%) in both
women and men. Anterior location is not as
frequent and more common in women (10%)
than in men (1%).[5] Multiple fissures are
also possible. Explanations for
predominantly posterior location of the anal
fissure are: the weaker muscles and the
poorer arterial supply in this region.[6] At the lower end of fissure a tag of skin may
form, called the sentinel pile. (Figure 4)
Pathophysiology
Most acute anal fissures heal spontaneously or with appropriate treatment but
there are cases that do not have any tendency to heal and rather become chronic. The
ulceration penetrates deeper and deeper through the layers of the anal canal and a series
of complications appear such as bleeding and intersphincteric abscess. The reason why
these fissures do not heal is a vicious circle: anal fissure induces a continuous spasm of
the internal anal sphincter muscle (smooth muscle) and spasm in turn maintains the
fissure. Because of the spasm, the muscle does not relax during defecation and fissure
open with each bowel movement.
There are two forms of anal fissure: acute and chronic (Figure 5)
Acute anal fissure appears as a tear of the anal skin continued into the anal canal
with slightly inflamed edges. Bleeding is frequent. It is accompanied by spasm of the
sphincter. In this stage, the fissure may be cured with conservative methods (ointments,
medication, etc.).
Chronic anal fissure is an ulcer like
lesion. It appears as a deep crater with
inflamed and hardened edges and a base
containing scar tissue or fibers of internal
sphincter. Sphincter spasm in advanced
forms is permanent due to muscle infiltration
with fibrous tissue and scar. Local
inflammatory changes can evolve to the
formation of abscesses with further
development of fistula. In most cases,
surgery is the only way to cure the fissure.
Symptoms
Pain and bleeding are the basic symptoms of anal fissure. The onset is sudden,
usually after a hard stool when the patient feels a sharp burning anal pain and observes
several drops of blood on toilet paper. The initial pain disappears but reappears with the
220
next stool. After defecation, the pain recurs with greater intensity and can last for
several hours. The pain can be so severe that patients are unwilling to have a bowel
movement, resulting in constipation and even fecal impaction. Moreover, constipation
can result in the passage of a larger, harder stool that causes further trauma and makes
the fissure worse. The pain also can induce dysuria, frequent urination, or the inability
to urinate. Ability to work is also impaired.
Anal bleedings are also possible, the patient observing the presence of bright red
blood.
Itching (pruritus ani), and a malodorous discharge may occur due to the discharge
of pus from the fissure.
Complications
Anal bleedings
Intersphincteric and perianal abscess. Anal fissures can be the starting point of
purulent collections in the anus and perianal region, manifested by increased
intensity constant pain, accompanied by fever and swelling of the perianal
region that becomes sensitive to touch.
Perianal fistulas
Mental and work capacity impairment. Even tendency to suicide have been
reported in some people with labile psyche.
Diagnosis
The diagnosis of anal fissure is not difficult being based on anal pain features and
clinical observations.
The diagnosis is
confirmed by a proctologic
examination that can be
performed in ambulatory
conditions. Complex anorectal
examination (digital and
rectoscopy) should be
performed in all cases, even
under anesthesia if necessary,
not to miss other more serious pathology than fissure (anal cancer, Cronhs disease,
ulcerative colitis, etc). (Figure 6)
Treatment
The goal of treatment for anal fissures is to break the vicious circle of anal
sphincter spasm that maintains the repeated tearing of the anoderm.
General measures
In acute fissures, medical therapy is successful in the majority of patients. Most
(80-90%) acute anal fissures will heal with conservative measures whereas only 40% of
chronic will heal with these methods.[7,8]
Preventing and treating constipation with proper diet with more vegetables and
fruits, with minimum 1.5 L of fluids intake per day, avoiding spicy foods, and
possibly mild laxative (stool softeners) administration. Enemas, glycerine
suppositories, and purgatives are prohibited.
Compliance with a strict local hygiene.
Sitz baths with warm chamomile tea are encouraged, particularly after bowel
movements, to relax the spasm and to increase the flow of blood to the anus.[9]
221
Local ointments with decontracturant, analgesic and epithelization effect.
Usually these ointments are used three times per day. Most often, ointments
containing nitroglycerin 0,2-0,4% are used.[10,11]
Other medicines if necessary (anti-inflammatory, decontracturant, anxiolytics,
laxatives, etc).
Treatment usually lasts three weeks. Some fissures are already healed after this
time. Sometimes, however, further treatment is required for another 3-4 weeks.
The patient should be warned that fissure could anytime reoccur if conditions that
led to it (constipation, diarrhea, etc.) continue.
Botulinum toxin
Botulinum toxin (Botox) relaxes (actually paralyses) muscles by preventing the
release of acetylcholine from the nerves that normally causes muscle cells to contract. It
has been used successfully to treat a variety of disorders associated with spasm of
muscles, including anal fissures. The toxin is injected into the internal anal sphincter.
The dose of Botox is not standardized. It varies from 2.5 to 20 units of toxin applied in
two locations (usually on either side of the fissure). Neither this type of treatment
ensures a definitive cure because fissures may reappear.[12,13]
Surgical treatment
It aims to stop the vicious circle fissure-contracture-fissure, by cutting the internal
anal sphincter. (Figure 7)
The anesthesia may be local, spinal, or
general according to the patient and doctor
preferences.
Partial lateral internal sphincterotomy
is the technique of choice for the treatment of
anal fissures.[14] In this procedure, the
internal anal sphincter is cut starting at its
distal most end, at the anal verge and
extending into the anal canal for a distance
equal to that of the fissure. The cut may
extend to the dentate line, but not farther.[15]
Percutaneous sphincterotomy
performs the sphincterotomy through a small
incision of the skin near the anal verge. In subcutaneous technique, the lower part of the
internal sphincter is cut by introducing the scalpel blade at the anal verge between the
anal c
ss
portant than the external one (which is not cut) in controlling the passage of stool.
anal mucosa and muscles.[16]
Following surgery, 90-100% of fissures heal.[17,18] Recurrence rates after this
type of surgery are low (0-10%) [19,20] and the risk of incontinence for stools following
surgery is also low. It is important to distinguish between short-term and long-term
incontinence. In the short-term (under six weeks), the sphincter is weakened by surgery,
so leakage of stool is sometimes possible. Long-term incontinence should not occur
after partial lateral internal sphincterotomy because the internal sphincter is le
im

222
References
1. Gopal DV. - Diseases of the rectumand anus: a clinical approach to common disorders. - Clin. Cornerstone. 2002;
4(4):34-48.
2. Gupta PJ . - A review of proctological disorders - European Review for Medical and Pharmacological Sciences 2006; 10:
327-335.
3. Williams DR, Coller J A, Corman ML, Nugent FW, Veidenheimer MC. - Anal complications in Crohn's disease. - Dis.
Colon. Rectum. 1981; 24(1):22-24.
4. Colvin HS, Barakat T, Moussa O, Babu H, Slaughter T, Palmer J G, Hinson FL. - Nicorandil associated anal ulcers: a
estimate of incidence. - Ann. R. Coll. Surg. Engl. 2012; 94(3):170-172.
n
93; 59(10):666-668.
ue? -
f chronic anal fissure.
20. Oueidat D. - A comparative study in anal fissure treatment. - J . Med. Liban. 1999; 47(3):164-168.

5. Petros J G, RimmEB, Robillard RJ . - Clinical presentation of chronic anal fissures. - Am. Surg. 19
6. Van Outryve M. - Physiopathology of the anal fissure. - Acta Chir. Belg. 2006; 1 06(5):517-518.
7. Wray D, Ijaz S, Lidder S. - Anal fissure: a review. - Br. J . Hosp. Med (Lond). 2008; 69(8):455-458.
8. Dhawan S, Chopra S. - Nonsurgical approaches for the treatment of anal fissures. - Am. J . Gastroenterol. 2007;
102(6):1312-1321.
9. Gupta PJ . - Effects of warmwater sitz bath on symptoms in post-anal sphincterotomy in chronic anal fissure--a
randomized and controlled study. - World J . Surg. 2007; 31(7):1480-1484.
10. Svendsen CB, Matzen P. - Treatment of chronic anal fissure with topically applied nitroglycerin ointment. A systematic
review of evidence-based results. - Ugeskr. Laeger. 2002; 164(33):3845-3849.
11. Ehrenpreis ED, Rubin DT, Ginsburg PM, Meyers J S. - Treatment of anal fissures with topical nitroglycerin. - Expert
Opin. Pharmacother. 2001; 2(1):41-45.
12. Yiannakopoulou E. - Botulinumtoxin and anal fissure: efficacy and safety systematic review. - Int. J . Colorectal Dis.
2012; 27(1):1-9.
13. Madalinski M, Kalinowski L. - Novel options for the pharmacological treatment of chronic anal fissure-role of botulin
toxin. - Curr. Clin. Pharmacol. 2009; 4(1):47-52.
14. Nelson RL, Chattopadhyay A, Brooks W, Platt I, Paavana T, Earl S. - Operative procedures for fissure in ano. -
Cochrane Database Syst Rev. 2011; (11):CD002199.
15. Lockhart-Mummery HE. - Fissure-in-ano. - In: Rob C, Smith R, editors. Operative Surgery. London: Butterworth; 1957.
pp. 113.
16. Boulos PB, Araujo J G. - Adequate internal sphincterotomy for chronic anal fissure: subcutaneous or open techniq
Br. J . Surg. 1984; 71(5):360-362.
17. Oh C, Divino CM, Steinhagen RM. - Anal fissure. 20-year experience. - Dis. Colon Rectum. 1995; 38(4):378-382.
18. Floyd ND, Kondylis L, Kondylis PD, Reilly J C. - Chronic anal fissure: 1994 and a decade later-are we doing better? -
Am. J . Surg. 2006; 191(3):344-348.
19. Abd Elhady HM, Othman IH, Hablus MA, Ismail TA, Aboryia MH, SelimMF. - Long-termprospective randomised
clinical and manometric comparison between surgical and chemical sphincterotomy for treatment o
- S. Afr. J . Surg. 2009; 47(4):11211-4.

223
3. Hemorrhoids (Piles)
Hemorrhoids are vascular structures
composed of veins, arterioles and
connective tissue, located in the rectal
submucosa (venous plexus), which have a
physiological role in anal continence, but
may become pathological with various
complications when they increase in
volume.
The anorectal canal is not like a pipe
with smooth inner surface. It has
overlapping folds (cushions) created by
physiological hemorrhoids contributing to
the tightness of the canal and so to gas
continence.
In gynecologic position, submucosal
venous cushions are located at hours 3, 7
and 11 on dial time, the favorite places where hemorrhoids usually occur. (Figure 8)
Frequency
Ten million people in the United States have hemorrhoids, which represents a
prevalence rate greater than 4%.[1] The true epidemiology of this disease is unknown
because patients have a tendency to use self-medication rather than to seek proper
medical attention.[2]
Etiology
The genetic factor is determinative. The main cause of hemorrhoids is a decreased
resistance of the venous walls, which become weaker leading to venous dilatations.
There is a genetic disorder of collagen and elastic fibers with decreased vein wall
elasticity. Usually it has a familial character and is frequently associated with other
diseases such as varicose veins and flat feet.
Predisposing factors are: [2-4]
Constipation: by forcing the passage of stool, the increased intra-abdominal
pressure causes increased pressure in the hemorrhoidal veins. There are
authors who do not agree that constipation is a risk factor.[5]
Diarrhea and laxatives are actually more important factors than constipation,
causing mucosa irritation.[4,5]
Occupational factors: prolonged standing or sitting (accountants, drivers,
pilots, etc).
Rectal cancer - symptomatic hemorrhoids
Increased intra-abdominal pressure
o Pregnancy
o Obesity
o Ascites
o Tumors
Increased venous pressure
o Portal hypertension (symptomatic hemorrhoids)

224
Classification
Hemorrhoids are divided into internal
(above the dentate line) and external (distal
or below the dentate line). Usually external
hemorrhoids are located under the perianal
skin. Mixed hemorrhoids appear when
internal high-grade hemorrhoids merge with
external hemorrhoids. (Figure 9)
Internal hemorrhoids are classified into
four grades:
Grade I: No prolapse.
Grade II: Prolapse upon defecation
but spontaneously reduce.
Grade III: Prolapse upon defecation
and must be manually reduced.
Grade IV: Prolapsed hemorrhoids,
which cannot be manually reduced.
Prolapsed hemorrhoids are internal
hemorrhoids that are so distended that they
are pushed outside the anus. (Figure 10)
Symptoms
Uncomplicated hemorrhoids are
asymptomatic.
Complications
Bleeding is the most frequent
complication and it is the major manifestation of the internal hemorrhoids. Bleeding is
the most frightening symptoms for the patient and leads him to the doctor. Blood is
usually bright red or dark red color and the patient notices it on toilet paper, on stool or
dripping in the toilet bowl. It is usually a terminal bleeding, blood is not mixed with the
stool but covering its surface.
Bleeding features are very important for differential diagnosis of hemorrhage
from other causes, especially colorectal cancer. Chronic bleeding will lead to iron
deficiency anemia.
Hemorrhoidal prolapse occurs in advanced stages of hemorrhoids (the fourth
stage). Prolapsed hemorrhoids may be complicated due to sphincter spasm which
squeezes them resulting in edema and venous thrombosis, with intense inflammatory
processes and pain. Hemorrhoidal prolapse may be partial or complete, circular.
Inflammation can progress to necrosis, ulceration, bleedings, and suppuration. (Figure
11)
Hemorrhoidal thrombosis occurs more frequently in the external hemorrhoids. It
is due to blood clotting in the hemorrhoidal veins (phlebothrombosis) and if
inflammatory processes are associated, it is called thrombophlebitis. The patient notices
a swelling on the anal ring that becomes increasingly painful. Pain intensity is variable
depending on the size of the associated thrombosis and inflammation. In most cases,
pain is continuous, lasting several days and affects the working capacity of the patient.
(Figure 11)
225
Thrombosed hemorrhoid may progress to regression and resorption with
decreasing volume and improved symptoms, or may progress to ulceration, bleeding,
and suppuration. With resolution of the thrombosis, the stretched anoderm persists as
excess skin or skin tags.

Diagnosis relies on proctologic examination and rectoscopy.
Examination begins with the examination of the perianal area. Gentle spreading
of the buttocks allows easy visualization of most of the anoderm; this includes the distal
anal canal. Anal fissures and perianal dermatitis are easily visible without internal
probing. Prolapse can be observed when the patient performs a Valsalva maneuver.
Digital examination of the anal canal can identify indurations or ulcerated areas,
also sensibility and anal sphincter tonus. Be sure to palpate the prostate in all men.
Because internal hemorrhoids are soft vascular structures, they are usually not palpable.
Anorectoscopy is mandatory for viewing internal hemorrhoids or other local
lesions.
Differential diagnosis of hemorrhoids includes other diseases of the anorectal region
that may cause pain, bleeding and itching.
Bleeding:
o ulcerative colitis
o anorectal cancer
o Crohn's disease
o anal syphilis
o anal fissure
o coagulation disorders
Pain
o anal fissure
o proctalgia fugax (or levator syndrome), a severe, episodic, rectal and
sacrococcygeal pain. It can be caused by cramp of the pubococcygeus or
levator ani
o anorectal tumors
o gynecological pathology
o coccydynia
Pruritus (itching)
o dry skin
o diabetes
o intestinal warms
o excessive washing, inadequate cleaning
o food irritants
o medication
o skin disorders, bacteria, fungus, etc
226
Treatment
It is preventive and curative.
Depending on the stage of hemorrhoids, the choice of the patient and physicians
experience, treatment can be surgical or non-surgical.
As with any other disease, if the patient presents to the doctor in early stages of
the disease, treatment will be much less aggressive, painless and can be performed in
ambulatory conditions. In advanced stages, or in case of serious complications, the only
option is surgical treatment.
Non-surgical methods of internal hemorrhoids are recommended especially in
less advanced stages (I, II and III). Among these are:
Rubber band ligation - the most widely used
Sclerotherapy
Laser or infrared photocoagulation
Cryotherapy
Rubber band ligation (Figure 12) is
performed on an outpatient basis. It is a
painless maneuver and a session usually
lasts less than 5 minutes. Blaisdell and
Baron described and refined ligation
therapy. The procedure is performed using a
tubular instrument connected to a suction
pump. The instrument is loaded with two
rubber rings. The internal hemorrhoid is
sucked into the tubular device and the two
rubber rings are downloaded at the root of
the hemorrhoid. Thus hemorrhoid
vascularization is interrupted inducing its
necrosis and detach within 5-7 days.
Remaining scar will heal completely in
about 2-3 weeks. The procedure is performed in several sessions depending on the
number and size of hemorrhoids. Elastic ligation is applied only to internal
hemorrhoids.
Surgery is indicated in advanced stages with complications of hemorrhoids and
for external hemorrhoids. The goal is to remove hemorrhoidal packages.
Indications for elective hemorrhoidectomy include the followings:[6]
1. Failure of medical and nonoperative therapy
2. Symptomatic third degree, fourth-degree, or mixed internal and external
hemorrhoids
3. Symptomatic hemorrhoids in the presence of a concomitant anorectal
condition that requires surgery
4. Patient preference, after discussion of treatment options with the referring
physician and surgeon
There are several methods of surgical treatment
Removal (hemorrhoidectomy)
Stapling (hemorrhoidopexy)
Blood clot (thrombus) removal. The external hemorrhoid is incised and
the blood clot removed.
Transanal hemorrhoidal dearterialization (THD) and rectal mucopexy
227
Operations can be performed under
local or general anesthesia, but in most cases
spinal or epidural anesthesia is preferred.
External hemorrhoidal thrombosis can
be treated by removing the blood clots and
excision of the underlying veins under local
anesthesia, (Figure 13) but remember that
acute thrombosis resolves spontaneously in
10-14 days, thus, a patient who presents late
and has diminishing pain should not be
operated.
Resection is usually reserved for patients with hygiene deficiency caused by
large skin tags, a history of multiple external
thromboses, or hemorrhoidal complications.
Milligan-Morgan Technique (Figure 14)
was developed by Drs. Milligan and Morgan
in the United Kingdom, in 1937.[7] The three
major hemorrhoidal cushions are excised. In
order to avoid stenosis, three pear-shaped
incisions are left open, separated by bridges
of skin and mucosa. This technique is the
most popular and is considered the gold
standard by which most other surgical
hemorrhoidectomy techniques are compared.
Ferguson Technique (Figure 15) was
developed in the United States, in 1952 by
Dr. Ferguson. This is a modification of the
Milligan-Morgan technique whereby the
incisions are totally or partially closed with
absorbable running sutures. The Ferguson
method has no advantage in terms of wound
healing because of the high rate of suture
breakage during bowel movement.[8]
Stapled Hemorrhoidopexy (Figure 16), also known as Procedure for Prolapse &
Hemorrhoids (PPH), Stapled Hemorrhoidectomy, or Circumferential Mucosectomy, is a
technique developed in the early 90's [9] that reduces the prolapse of hemorrhoidal
tissue by excising a band of the prolapsed anal mucosa using a circular stapling device.
This restores the hemorrhoidal tissue back to its original anatomical position.

228
Whitehead Hemorrhoidectomy (Figure 17) described in 1882 by the author, with
good results, but is responsible for the most serious postoperative complications: anal
stenosis, ectropion and anocutaneous sensitivity disappearance.[10] A circular sleeve of
anal mucosa with hemorrhoidal packages is excised, the skin being sutured to the rectal
mucosa. In Romania, the operation was
introduced and developed by Vercescu. In
1998, Wolff and Culp improved the
calibration technique with flaps sutured to
the dentate line that prevents stenosis of the
anal canal.[11] The method is successfully
used for irreducible hemorrhoidal prolapse
and can be achieved with a single
circumferential flap or two flaps (right and
left) prepared from anal mucosa after
dissection of the hemorrhoidal packages.
Radiofrequency ablation and suture fixation
of hemorrhoids, bipolar diathermy hemorrhoidectomy, LigaSure and Starion
hemorrhoidectomy with submucosal dissection are newer methods which use special
devices to remove the hemorrhoids with less intraoperative bleeding and postoperative
complications.[12-14]
Postoperative care
The patient is usually discharged on day 2 or 3 after surgery, but surgery may be
performed in terms of one-day surgery.
Recommendations after hospital discharge are:
Warm sitz bathes
Ointments with healing effect
Avoid constipation and diarrhea
Avoid spicy food
Paraffin oil to facilitate stool evacuation
Wound dressing is not always necessary
Control at 3-4 weeks after surgery including a digital rectal examination to
assess anal stenosis occurrence and patency of the anal sphincter.
Postoperative complications [15-17]
Early: severe postoperative pain lasting 2-3 weeks, wound infection, acute
urinary retention, hemorrhage, anal incontinence
Late: secondary hemorrhage, anal stenosis, ectropion, anal fissure, skin
tags, recurrence
References
1. J ohanson J F, Sonnenberg A. - The prevalence of hemorrhoids and chronic constipation. An epidemiologic study. -
Gastroenterology 1990; 98(2):380-386.
2. Varut Lohsiriwat - Hemorrhoids: Frombasic pathophysiology to clinical management. - World J . Gastroenterol. 2012;
18(17): 20092017.
3. Loder PB, KammMA, Nicholls RJ , Phillips RK. - Review Haemorrhoids: pathology, pathophysiology and aetiology. -
Br. J . Surg. 1994; 81(7):946-954.
4. Delc F, Sonnenberg A. - Associations between hemorrhoids and other diagnoses. - Dis. Colon Rectum 1998;
41(12):1534-1542.
5. J ohanson J F, Sonnenberg A. - Constipation is not a risk factor for hemorrhoids: a case-control study of potential
etiological agents. - Am. J . Gastroenterol. 1994; 89(11):1981-1986.
229
6. American Gastroenterological Association medical position statement: Diagnosis and treatment of hemorrhoids. -
Clinical Practice Committee, American Gastroenterological Association - Gastroenterology. 2004; 126(5):1461-1462.
http://faculty.ksu.edu.sa/Al-Amri/Guidelines/AGA%20statement%20dx%20hemorroids.pdf
7. Milligan ET, Morgan CN, J ones LE, Officer R. - Surgical anatomy of the anal canal and operative treatment of
haemorrhoids. - Lancet 1937; 11:11191194.
8. Agbo SP. - Surgical Management of Hemorrhoids. - J . Surg. Tech. Case Rep. 2011; 3(2): 6875.
9. Longo A. - 6th world congress of Endoscopy Surgery. Naples: Mundozzi Editore; 1998. - Treatment of haemorrhoidal
disease by reduction of mucosa and haemorrhoidal prolapse with a circular stapling device: A new procedure; pp. 777
784.
10. Maria G, Alfonsi G, Nigro C, Brisinda G - Whitehead's hemorrhoidectomy. A useful surgical procedure in selected
cases. - Tech. Coloproctol. 2001; 5(2):93-96.
11. Sands LR., Sands Dana R. - Ambulatory colorectal surgery - 2009 by Informa Healthcare USA, Inc.Informa Healthcare
is an Informa business.
12. Gupta PJ . - Radiofrequency ablation and plication of hemorrhoids. - Tech. Coloproctol. 2003; 7(1):45-50.
13. Andrews BT, Layer GT, J ackson BT, Nicholls RJ . - Randomized trial comparing diathermy hemorrhoidectomy with the
scissor dissection Milligan-Morgan operation. - Dis. Colon Rectum1993; 36(6):580-583.
14. Chen CW, Lai CW, Chang YJ , Chen CM, Hsiao KH. - Results of 666 consecutive patients treated with LigaSure
hemorrhoidectomy for symptomatic prolapsed hemorrhoids with a minimumfollow-up of 2 years. - Surgery 2013;
153(2):211-218.
15. Sayfan J . - Complications of Milligan-Morgan hemorrhoidectomy. - Dig. Surg. 2001; 18(2):131-133.
16. Wolff BG, Culp CE. - The Whitehead hemorrhoidectomy. An unjustly maligned procedure. - Dis. Colon Rectum. 1988;
31(8):587-590.
17. Mirzaei R, Mahjoubi B, Kadivar M, Azizi R, Zahedi-Shoolami L. - Anal sphincter injuries during hemorrhoidectomy: a
multi center study. - Acta Med. Iran. 2012; 50(9):632-634.

230
4. Anoperineal Abscesses
Etiology
Staphylococci, Streptococci, E. coli, Proteus and anaerobes such as clostridium
welchii and bacteroides are frequently responsible for these abscesses.
In 10-20% of cases, the site of entry of the infective organisms is obvious.
Perianal abscess may develop after:
Dorsal anal fissure
Anal hematoma
Thrombosed hemorrhoids
Following injection of a anesthetic solution or alcohol in perianal or
ischiorectal space in the treatment of perianal pain
Following injections (sclerotherapy) of the internal hemorrhoids
Injury to anal or rectal mucosa
As a complication of hemorrhoidectomy or sphincterotomy
Pathophysiology
Anorectal abscess is a cryptoglandular disease because infection of the anal crypts
followed by infection of the anal glands leads to the abscess formation. Cryptogandular
disease in acute stage presents as anorectal abscess while in chronic stages it presents as
fistula in ano.[1,2]
According to this theory, the first step
is the formation of the intersphincteric
abscess located between the internal
sphincter and the longitudinal
intersphincteric fibers, caused by the
infection of anal glands. Subsequently the
pus may force its way downward along the
longitudinal fibers to emerge at the anal
orifice as perianal abscess. Laterally it may
pass through the longitudinal muscles and
external sphincter to enter the ischiorectal
fossa and developing the ischiorectal abscess
or it may track upwards in the
intersphincteric space to produce a high
intermuscular abscess. If the pus tracks still
higher in the intersphincteric space it gives
rise to pelvirectal (or supralevator) abscess.
(Figure 18)
According to their location abscesses
may be: (Figure 19)
Intersphincteric
Submucous
Perianal
Ischiorectal
High intermuscular
Supralevator

231
Clinical picture
Perianal abscesses manifest with symptoms
and signs of an acute perianal inflammatory
process. Celsian signs: tumor, dolor, calor, rubor
and functio laesa are present.
Patient complains of pain, becoming more
and more intense. The patient cannot sit and in
most cases is feverish.
The classical signs of a perianal abscess
are: (Figure 20)
on inspection, a perianal swelling with
stretched, shiny, flushing skin
on palpation, a painful in duration with high local temperature. In advanced
stages, fluctuance may be felt. In more advanced stages, the ulcerated skin
with fistula and pus discharge appears.
When the abscess has a deep location, local signs are not so obvious.
On digital rectal examination a painful tender indurated bulge into the anal canal
on that side is felt. The maneuver is painful.
Natural evolution of perianal abscesses
Perianal abscesses progress to skin necrosis and outward fistulization with
incomplete abscess evacuation. As abscess evacuates, the internal pressure drops and
pain decreases. However, the abscess should be operated for a complete evacuation.
Intersphincterian abscess may spontaneously drain into the anal canal, or may
progress to other sites (perianal, submucous, ischiorectal, etc.).
Ischiorectal abscess evolves with symptoms of sepsis, or intrarectal fistulization is
possible in advanced stages.
Supralevator abscess, being very deep, is difficult to diagnose. Also evolves with
sepsis, and usually fistulization takes place into the rectum.[3]
In rare cases, the abscess can progress to a very serious form of necrotizing
fasciitis that extends to the genitals, the Fourniers gangrene.
Diagnosis
Diagnosis is usually easy and there is no need for special investigations. Clinical
examination of the perianal region and rectal examination are sufficient. In case of deep
abscess, (supralevator and ischiorectal) intrarectal ultrasonography and computed
tomography are useful in diagnosis.
Differential diagnosis should be made to other diseases that evolve with perianal
pain and sepsis:
Bartholins abscess
Hemorrhoidal thrombophlebitis
Douglas sac abscess
Gynecological inflammatory disease
Rectal tumors

232
Treatment
Usually patients are hospitalized in emergency condition.
The main treatment is surgery plus medication with broad-spectrum antibiotics at
first and then according to the antibiogram, inflammatory and pain relievers. Surgical
treatment consists of incision, evacuation, debridement, lavage and drainage or
swabbing with hydrogen peroxide, in spinal or general anesthesia. Packing may be
beneficial at the time of abscess drainage by providing hemostasis of the inflamed,
hypervascular abscess cavity.[39]
Perianal and submucous abscesses are easily incised. Incision is made in the
maximum fluctuance.
In case of ischiorectal abscesses, perianal skin incision is guided by the finger
introduced into the rectum to find the collection.
In case of intersphincterian abscess, pus is discharged spontaneously during anal
dilatation with the anuscope or after the internal sphincterotomy.
In case of supralevator abscess, the puss is evacuated into the rectum through an
incision of the rectal wall and then drained out with a transanal drainage tube.
Postoperative evolution
Dressings are applied each day. Wound will heal gradually by granulation but
often (7-40%) a perianal fistula will remain which should be operated over a period of 6
weeks.[2,4,5] If the wound is closed completely or the patient does not return for
treatment of perianal fistula, perianal abscess will recur with high probability over a
variable time.
Particular forms of perianal abscesses
Ischiorectal abscess
The only sign of inflammation may be
an induration of the perianal region. The
patient presents with pyrexia of obscure
origin without pain of any kind. Pelvis CT
scan and ultrasound examination are useful
in diagnosis.
Horse shoe abscess (Figure 21)
appears in neglected cases of ischiorectal
abscess when puss spreads from one side to
the other via the retroanal or preanal space
thus giving rise to a bilateral ischiorectal
abscess, the so-called horse shoe abscess.
This type of abscess is not uncommon
(10-11%) and in most cases, the infection
starts from a posterior anal crypt.
Fournier's Gangrene (Figure 22) is a
specific form of necrotizing fasciitis.
Classically, it involves the penis and
scrotum, but it also can affect genitalia in
females.[6] Often the underlying cause is
related to a perianal/ischiorectal abscess. The
tissue planes in the perineum and groin are
all connected and the aggressive agents of
233
destruction in necrotizing infections tend to spread along these planes. Pathognomonic
findings include a wide area of bruising and ecchymosis involving most of the gluteal
skin, crepitus, and skin changes over the base of the scrotum. Associated diseases that
compromise the immune system have been incriminated as necessary predisposing
factors for the development of Fournier gangrene. The following are common
predisposing co-morbidities:
Diabetes mellitus (cited most often)
Morbid obesity
Cirrhosis
Vascular disease of the pelvis
Malignancies
High-risk behaviors (eg. alcoholism, intravenous drug abuse)
Immune suppression due to systemic disease or steroid administration
Early aggressive treatment of the underlying conditions is essential. Surgery
consists in large debridement, copious wounds lavage with hydrogen peroxide, removal
of necrotic tissues and hyperbaric oxygen. Risk of death is around 16%, and is related to
the patient's condition at presentation.[6]
Supralevator abscess is a rare condition and the most difficult to diagnose.
The patient presents with septic status with high fever even pyrexia, with mild
continuous pelvic pains. On rectal examination, a bulge of the rectal wall may be felt
with the fingertip. When the abscess fistulizes into the rectum and stools contain a large
quantity of pus, the diagnosis becomes easier. CT scan reveals the collection in the
supralevator space. Surgical treatment consists of a transanal incision of the rectal wall
in the maximum fluctuance. A drainage tube is placed in the abscess cavity and
exteriorized through the anal orifice.
References
1. Parks AG. - Pathogenesis and treatment of fistula-in-ano. - Br. Med. J . 1961; 1:463469.
2. Whiteford MH. - Benign Anorectal Conditions. Perianal Abscess/Fistula Disease. - Clin. Colon Rectal Surg. 2007; 20(2):
102109.
3. Prasad ML, Read DR, Abcarian H. - Supralevator abscess: diagnosis and treatment. - Dis. Colon Rectum 1981;
24(6):456-461.
4. Vasilevsky CA, Gordon PH. - The incidence of recurrent abscesses or fistula-in-ano following anorectal suppuration. -
Dis. Colon Rectum1984; 27(2):126-130.
5. Henrichsen S, Christiansen J . - Incidence of fistula-in-ano complicating anorectal sepsis: a prospective study. - Br. J .
Surg. 1986; 73(5):371-372.
6. Eke N. - Fournier's gangrene: a review of 1726 cases. - Br. J . Surg. 2000; 87(6):718-728.

234
5. Perianal Fistula (Fistula-in-ano)
Perianal fistula is an abnormal connection (channel) between the anal canal and
the skin.
History [1]
References to fistula-in-ano date to antiquity. Hippocrates referred to surgical therapy for
fistulous disease.
The English surgeon John Arderne (1307-1390) wrote Treatises of Fistula in Ano; Haemmorhoids,
and Clysters in 1376, which described fistulotomy and seton use.
Historical references indicate that Louis XIV was treated for an anal fistula in the 18th century. In
the late 19th and early 20th centuries, prominent physician/surgeons, such as Goodsall and Miles,
Milligan and Morgan, Thompson, and Lockhart-Mummery, made substantial contributions to the
treatment of anal fistula.
In 1976, Parks refined the classification system that is still in widespread use.
Over the last 30 years, many authors have presented new techniques in an effort to minimize
recurrence rates and incontinence complications.
Despite 2500 years of experience, fistula-in-ano remains a perplexing surgical disease.
Frequency: The prevalence rate is 8.6 cases per 100,000 individuals. The male-to-
female ratio is 1.8:1. The mean age of patients is 38.3 years.[1,2]
Causes:
Primary
The most common cause is the obstruction of the anal gland that leads to
stasis and infection with perianal abscess formation, which was previously
operated, or with spontaneous fistulization to the skin surface.
Secondary
Iatrogenic (hemorrhoidal surgery)
Inflammatory bowel diseases (Crohn's disease more common than
ulcerative colitis)
Infections (viral, fungal or TB)
Malignancy
Radiation
Clinical picture
Patients often provide a reliable history of previous pain, swelling, and
spontaneous or planned surgical drainage of an anorectal abscess. Symptoms are:
Perianal discharge (puss) through the external opening of the fistula
Pain
Swelling
Anal bleeding
Diarrhea
Skin excoriation
Physical examination. An external opening that appears as an open sinus or
elevation of granulation tissue can be observed on the perianal skin. Spontaneous
discharge via the external opening may be apparent or expressible upon digital rectal
examination. (Figure 23)
Digital rectal examination may reveal a fibrous tract beneath the skin. It also
helps delineate any further acute inflammation that is not yet drained. Lateral or
posterior induration suggests deep postanal or ischiorectal extension. The sphincter tone
235
and voluntary squeeze pressure should be assessed before any surgical intervention to
delineate whether preoperative manometry is indicated.
Investigations
Anuscopy is usually required to
identify the internal opening.
Ultrasound, fistulography and MRI
investigations are not performed routinely
but they are helpful in highlighting an occult
cause of fistula recurrence. In recent years,
endoanal ultrasound (EUS) has been widely
used in the assessment of fistula and, in most
cases, shows the position of the internal
opening.[3]
When describing a fistula, it is
important to mention the following aspects:
Position of the skin opening on axial images (using the anal clock)
Distance of the opening to the anal verge
Secondary fistulas or abscesses
Several techniques have been described to help locate the trajectory of the fistula
and more important: to identify the internal opening. One of these is the instrumental
exploration of fistula tract. Care should be taken to not use excessive force and create
false passages. If internal orifice cannot be detected, blue dye or hydrogen peroxide may
be injected through the external orifice under direct visualization of the interior aspect
of the anal canal.
Sinus pilonidalis
Other causes of fistula in ano
Inflammatory bowel diseases (Crohn's disease more common than ulcerative
colitis)
Infections (viral, fungal or TB)
Malignancy
Classification
The most widely used is the Parks
classification, which distinguishes four
kinds of fistula: (Figure 24)[4]
1. Intersphincteric (25%)
2. Transsphincteric (69.2%)
3. Suprasphincteric (5%)
4. Extrasphincteric (3.8%)
The most common fistulas are the
intersphincteric and the transsphincteric.
The extrasphincteric fistula is uncommon
and only seen in patients who had multiple
operations. In these cases, the connection
with the original fistula tract to the bowel is
lost.

236
Other possible fistulas:
1. No perianal opening (external blind)
2. No internal opening (internal blind)
3. Complex fistulas - multiple, ramified tracts with or without multiple external
orifices.
A superficial fistula is a fistula which is not related to the sphincter or the perianal
glands and is not part of the Parks
classification. These are more often due to
Crohn's disease or anorectal procedures such
as hemorrhoidectomy or sphincterotomy.
Goodsalls rule (Figure 25)
This rule relates the external opening
of an anal fistula to its internal opening. It
states that the external opening situated
behind the transverse anal line will open into
the anal canal in the midline posterior. An
anterior opening is usually associated with a
radial tract. Anterior fistulas will have a
direct track into the anal canal. Posterior
fistulas will have a curved track with their internal opening lying in the posterior
midline of the anal canal.
An exception to the rule are anterior fistulas lying more than 3 cm from the anus,
which may have a curved track (similar to posterior fistulas) that opens into the
posterior midline of the anal canal.
Principles of treatment
Anal fistulas never heal spontaneously. The inner wall of the fistula develops
fibers and pyogenic membrane that does not allow spontaneous healing. Only surgical
treatment can heal the fistula. There are three main surgical procedures:
1. Fistulotomy (cutting/opening the fistula tract)
2. Fistulectomy (excision the whole fistula tract)
3. Seton placement
There are many other techniques including laser coagulation of the fistula tract,
sealing with fibrin glue, collagen plugs, etc.[3,5]
In simple cases, fistulotomy is preferred to fistulectomy because it does not
involve excision of a portion of the sphincteric muscle. Seton placement is rarely used
because it is a very painful procedure.
Fistulotomy (Figure 26)
A probe is passed into the fistula through the external opening. The overlying
skin, subcutaneous tissue, and internal sphincter muscle are divided with a scalpel
or electrocautery, thereby opening the entire fibrous tract, like a book. Curettage
is performed to remove granulation tissue in the tract base. Wound is dressed
daily to close from depth toward the surface.
237

Complete fistulectomy creates larger wounds that take longer to heal. The entire
fistula tract is excised with a perianal skin of 1-2 cm adjacent to the external
orifice. Daily dressing promotes internal healing before external closure. (Figure
27)
Seton placement (slow fistulotomy) - An elastic seton is applied through the
entire fistula and tied outside the anus. The seton will slowly cut the anal
sphincter from inside out with consecutive healing. (Figure 28)

Postoperative care
Sitz baths, analgesics, and stool bulking e are recommended. It is important to
ensure that the wound does not close prematurely, causing a recurrent fistula. Wound
healing usually occurs within 6 weeks.
Complications
Early postoperative
Urinary retention
Bleeding
Fecal impaction
Hemorrhoidal thrombosis
Delayed postoperative
Recurrence
Anal incontinence
Anal stenosis
Delayed wound healing

238
239
/Outcome and Prognosis
Following standard fistulotomy, the reported rate of recurrence is 0-18% and the
rate of any stool incontinence is 3-17%.[6-8] There are no major difference between the
various techniques as far as recurrence rates are concerned.[9]
There are cases with multiple recurrences or complex fistulas that sometimes
require making a temporary loop colostomy, to divert the feces from anorectum and to
allow healing after fistulectomy or fistulotomy.
To prevent recurrences several conditions are necessary:
Fistula tract must be removed entirely (fistulectomy) that is more difficult in
complex fistulas. Fistula track can be marked with methylene blue dye.
Internal orifice should be discovered
Wound healing must happen from the depth toward the surface
References
1. Dennis F Zagrodnik II. - Fistula-in-Ano - Medscape reference. http://emedicine.medscape.com/article/190234-overview
2. Sainio P. - Fistula-in-ano in a defined population. Incidence and epidemiological aspects. - Ann. Chir. Gynaecol. 1984;
73(4):219-224.
3. Helena Tabry, Farrands PA. - Update on anal fistulae: Surgical perspectives for the gastroenterologist. - Can. J .
Gastroenterol. 2011; 25(12):675680.
4. Ozkavukcu E, Haliloglu N, Erden A. - Frequencies of perianal fistula types using two classification systems. - J pn. J .
Radiol. 2011; 29(5):293-300.
5. Whiteford MH. - Benign Anorectal Conditions. Perianal Abscess/Fistula Disease. - Clin. Colon Rectal Surg. 2007;
20(2):102109.
6. Garcia-Aguilar J , Belmonte C, Wong WD, Goldberg SM, Madoff RD. - Anal fistula surgery. Factors associated with
recurrence and incontinence. - Dis. Colon Rectum1996; 39(7):723-729.
7. Vasilevsky CA, Gordon PH. - Results of treatment of fistula-in-ano. - Dis. Colon. Rectum1985; 28(4):225-231.
8. van Tets WF, Kuijpers HC. - Continence disorders after anal fistulotomy. - Dis. Colon Rectum1994; 37(12):1194-1197.
9. J acob TJ , Perakath B, Keighley MR. - Surgical intervention for anorectal. - Cochrane Database Syst. Rev. 2010;
(5):CD006319.

Surgical Pathology of the Appendix
SURGICAL PATHOLOGY OF THE APPENDIX

1. Surgical anatomy
2. Acute appendicitis
3. Chronic appendicitis
4. Tumors of the appendix
1. Surgical Anatomy
Appendix begins to develop in the fifth month of intrauterine life. It is a
cylindrical hollow organ, worm-like (hence the Latin name "appendix vermicularis"),
with a length ranging between 2 and 30 cm and a diameter of about 0.5 to 0.8 cm. It is
located at the bottom of the cecum (Figure 1) at the convergence of the three tenia coli
(omental, mesenteric and free). This last aspect is of particular importance in detecting
the base of the appendix through a small laparotomy. Another constant landmark is the
implantation of the appendix in cecum at approximately 2.5 cm medial and posterior to
the ileo-cecal valve. (Figure 2)

Histologically the appendicular wall is composed of the same layers as intestines
are. On the outside, it is covered by the visceral peritoneum. There are two muscular
layers: internal circular and external longitudinal derived from colic tenias. Submucosal
layer contains about 200 lymph follicles, hence the name of "abdominal tonsil" given to
the appendix. The maximum development of these follicles occurs by the age of 18-25
years, and after that, they regress. The lining of the appendix is represented by mucosa
with mucus-secreting epithelium. The appendicular lumen communicates with the
cecum through the appendicular ostium guarded by the Gerlach valve.
Abdominal topography of the appendix varies depending on position of the
cecum. In most cases, it is located in the right iliac fossa, but in case of a short
ascending colon it can be under the liver raising problems of differential diagnosis
(acute cholecystitis, duodenal ulcer, renal colic, colon tumor, etc.) or/and surgical
approach. On the other hand, when the ascending colon is too long, the appendix can be
located into the pelvis and its inflammation can be confused with an adnexal
inflammation. Diagnostic difficulties are also encountered in case of malrotation or situs
inversus, when the appendix is located in the left iliac fossa or left flank, in case of
appendicitis leading to confusion with diverticulitis or descending colon tumor.
240

The base of the appendix is always fixed
but its tip can be located in any position
("Gerotas compass"). The most common
positions are: behind the cecum (retrocecal),
subcecal, prececal, laterocecal (paracecal),
mesoceliac, preileal or retroileal (postileal).
[1,2](Figure 3) Special variants are that of
intramural or subserosal (the appendix is situated
in the colic wall thickness) and retroperitoneal,
which create particular difficulties in
intraoperative detection. In subserosal location,
the appendix can be found on digital palpation as
an endured cord in the cecum wall thickness.
The mesoappendix derives from ileal
mesentery being attached to the base of the cecum
and appendix. Sometimes, in women, it continues
with the ligament of Clado, a mesenteric fold
running from the broad ligament on the right side
to the appendix. In many cases, the mesoappendix
does not reach up to the tip of the appendix, this
being incriminated by some authors as the cause
of why the appendix becomes gangrenous and perforated most frequently at this level,
because of lack of vascularity.
Arterial vascularization of the appendix is provided by the appendicular artery,
usually single, derived from the ileocolic artery or branches. (Figure 4) Appendicular
vein drains into the ileocolic vein and then into the superior mesenteric vein. Lymphatic
drainage is towards lymph nodes along the appendicular artery, then ileocolic and
superior mesenteric artery. A secondary pathway is directly to the subpyloric lymph
nodes.
2. Acute Appendicitis
Despite progresses in antibiotic therapy and surgery, acute appendicitis remains a
surgical emergency being the main cause of admissions in emergency for acute surgical
abdomen. The only rational therapeutic approach remains the appendectomy. Untreated,
it causes serious complications that can lead to death (perforation, generalized
peritonitis). Therefore, early diagnosis and treatment are of utmost importance.
Acute appendicitis can create special problems of diagnosis and treatment in
atypical forms. Generally, one in every five appendicitis is misdiagnosed, especially in
women, and the incidence of normal (unmodified) appendix during appendectomy is
estimated at 10-40% of all cases.[3-7]
History
The appendix was probably recorded for the first time in Egyptian civilization
(3000 BC) during mummification. Although appendicitis was a common disease, only
at the beginning of the 9
th
century the appendix was identified as an organ capable of
producing disease.[8]
241
Up to 1850, there have been many interpretations concerning the causes that
induce the inflammation in the right iliac fossa. In 1827, Melier described several cases
of appendicitis at autopsy and formulated the idea that the appendix could be the cause
of death, an idea that conflicted with that of Dupuytrain, the most important surgeon of
the time. Further studies in England and Germany have confirmed that appendix may be
the potential source of disease and several publications have taken this subject after
1860.
In 1880, both Matterstock in Germany and With in Norway published works
indicating the appendix as a significant cause of inflammation of the right iliac fossa.
In 1886, Reginald Fitz, introduced the term of appendicitis and recommended the
early surgical treatment of disease. Since 1886, the spread of anesthesia as well the wide
acceptance of antiseptic concept, have paved the rapid implementation of this
recommendation.
In 1889, Chester McBurney described the migratory pain and the painful
abdominal point that bears his name, located at 4-5 cm away from the anterior-superior
iliac spine on the spino-umbilical line. He also stated, incorrectly, that this is the most
constant painful point in appendicitis. McBurney in New York and McArthur in
Chicago have introduced the oblique incision in the right iliac fossa, as a way of
approach for appendectomy in 1884.
In 1905, Murphy described the symptoms of appendicitis consisting of pain
followed by nausea, vomiting and right iliac fossa muscular contracture.
When Fitz described the acute appendicitis, the mortality rate of perforated
appendicitis was about 30%. With the introduction of penicillin in 1940, there was a
dramatic decrease in mortality rate, somewhere to 2% and further progress made in the
management of appendicitis brought this rate below 1%.
Incidence
The incidence is 7% - 10% in U.S. and Europe. In Asian and African countries is
lower probably due to dietary habits. Acute appendicitis occurs most commonly in the
second and third decade of life, but can affect any age. The highest incidence is seen in
10-19 years age group, where it reaches a frequency of 233 cases per 100,000 with a
prevalence for males (male / female ratio is of 1.4 - 1.7/1).[9,10]
Etiology
The infectious theory of Aschoff states that the cause of acute appendicitis is
bacterial infection. The microbial flora of the appendix is similar to that of the
ascending colon consisting of a variety of aerobic and anaerobic germs. However,
germs involved in producing acute appendicitis are the same as those that produce other
colic inflammations.
In acute non perforated appendicitis, bacteriological examination of peritoneal
fluid is positive in less than half of the cases, while in gangrenous or perforated
appendicitis the test is positive in 85%
of cases.[11] In 1938, Altemeier
demonstrated the polymicrobial
etiology of perforated appendicitis.
The most frequent aerobic and
anaerobic germs involved in the
etiopathogenesis appendicitis are
described in the table below. (Table 1)
Table 1: Main germs involved in producing
acute appendicitis
Aerobes Anaerobes
Escherichia coli Bacteroides fragilis
Streptococcus viridans spp. Bacteroides spp.
Pseudomonas aeruginosa Peptostreptococcus
Streptococcus Grup D Bilophila spp.
Enterococcus spp. Lactobacillus spp.
Fusobacterium spp.
242
Pathophysiology
There are two main theories:
1. Enterogenic theory: the obstruction of the appendix lumen is the
determining factor.
2. Hematogenic theory justifies appendicitis by blood dissemination of
bacteria usually during a respiratory infection.
Currently, it is widely accepted the role of appendicular lumen obstruction in
triggering acute appendicitis. This can occur due to either hyperplasia of lymph
follicles, to coprolits or other foreign body occluding the lumen. Hyperplasia of lymph
follicles is most frequent in young patients whereas in elderly the obstruction is usually
a consequence of fibrosis, coprolites or malignancies (1%) (carcinoid, adenocarcinoma
or mucocele). In endemic areas, intestinal parasites can also cause obstruction.
Lumen obstruction and bacterial overpopulation leads to accumulation of mucous
secretion, increasing the intra-luminal pressure with parietal compression leading to
lymphatic and then venous vessels obstruction. This creates conditions of acute
inflammatory reactions, the appendix becoming edematous and ischemic, leading to
bacterial translocation and even appendicular wall necrosis (gangrenous appendicitis).
Appendicular lumen obstruction causes distention that stimulates the free nerve
endings, the cause of pain located in epigastrium and of colicky pain in the right iliac
fossa (appendicular colic). Further distension by bacterial multiplication leads to
disturbances in venous and lymphatic circulation and triggers nausea, vomiting, along
with increased pain that becomes permanent and located in the right iliac fossa.
Without a proper treatment, the gangrenous appendix perforates into the
peritoneal cavity. This process can be fast producing a generalized peritonitis, or slow,
in which case, due to the inflammatory reaction of the greater omentum and visceral
peritoneum, either a localized peritonitis or an appendicular abscess will develop.
In the first 24 hours from onset, 90% of patients have only appendicular wall
inflammation or necrosis, but without perforations.
Morphopathology
There are three pathological stages of evolution:
1. Catarrhal appendicitis (congestive): the appendix is red-violet, turgid,
congested, with obvious vascular pattern. The mucosa is thickened with hyperemia and
areas of superficial ulceration and microscopic areas of necrosis.
2. Phlegmonous appendicitis (suppurative, purulent): the appendix is more
distended, under tension, often with uneven caliber, with the serosa's gloss lost, and
very brittle (it must be handled carefully because it may break). The tip of the appendix
is thicker. (Figure 5) Frequently turbid fluid is present in periappendicular area as a
consequence of peritoneal reaction. The mesoappendix is infiltrated and brittle. The
appendix can be adherent to the neighboring organs (false membranes). The content is
puss (empyema). At microscopic examination, the lymph follicles are destroyed with
their transformation into small abscesses.
3. Gangrenous appendicitis (necrotic): the appendicular wall has soft devitalized
areas of brownish color ("appearance of dead leaves"). (Figure 6) The mesoappendix is
intensely swollen with hyperemia, often with thrombosed vessels. In the peritoneum, an
intense fetid and hyperseptic fluid is present. Bacteriological examination of the fluid
reveals, in most cases, the presence of anaerobic germs (Clostridium perfringens,
Bacillus funduliformis) along with colibacillus and streptococcus.
243
Another pathological classification of acute appendicitis considers the evolution
to healing, chronicization or complications:

1. Edematous stage: acute appendicitis may show spontaneous regression or
progression to the next stage. The inflammatory process also affects the mesoappendix.
2. Purulent stage (phlegmonous): spontaneous regression is rarely present, the
appendix progressing towards perforation or rupture. Peritonitis may be present.
3. Gangrenous stage: spontaneous regression never appears. Peritonitis is always
present.
Another classification is:
1. Endogenous forms: catarrhal, phlegmonous, gangrenous appendicitis.
2. Exogenous forms: appendicular perforation.
The spread of germs into the peritoneal cavity with the consequence of localized
or generalized peritonitis can be achieved by transparietal diffusion or directly by
appendicular perforation.
In rapidly evolving forms, perforation causes generalized peritonitis (the one-step
peritonitis). In slow evolving forms, there is a localized peritonitis, the intestines,
greater omentum and other neighboring organs attempting to isolate the extension of the
inflammatory process. Thus, a periappendicular block results containing in its center
the appendix. This block is usually palpable. Antibiotics and local cold applications in
this stage can lead to regression of the inflammatory process. The appendix usually will
heal with fibrosis process and it will be removed after a few weeks.
In the absence of proper treatment, the periappendicular block usually progresses
to abscess formation, leading to peritonitis in two steps. The perforation of the appendix
in the center of the periappendicular block will develop a periappendicular abscess. In
this stage, surgical treatment consists only in evacuation of the puss by extraperitoneal
approach (to avoid spreading of puss into the peritoneal cavity) the so-called
oncotomy operation. A drain will be placed in the abscess cavity. The remaining
appendix usually heals with fibrosis and after a few weeks, it will be removed by
classical or laparoscopic approach.
Unrecognized and untreated, the periappendicular abscess will evolve with
increasing inside pressure leading to fistulization into the peritoneal cavity with the
consequence of generalized peritonitis in three steps. Other rare variants of fistulization
could be external (to the skin) or into a hollow organ (cecum, small intestine, sigmoid
colon or bladder).
Symptoms
In classic forms, the onset is acute, the main complaints being epigastric pain and
anorexia. Symptoms depend on patients age (infant, adult, elderly), the anatomical
position of appendix, and the morphopathological type (periappendicular block, abscess,
diffuse purulent peritonitis, or pseudotumoral appendicitis).
244
Histories of repeated identical symptoms or lasting more than 3-4 days are
elements that make us think of other possible disease than acute appendicitis.
Classic clinical presentation of acute appendicitis (appendix in normal position)
shows:
1. Pain is the major symptom. It is initially, located within the epigastrium or peri-
umbilical. After a period, ranging between 1 to 12 hours (typically 4-6 hours), it
descends in the right iliac fossa being exacerbated by effort, coughing or sudden
movements. It is not a colicky pain. Sometimes, this kind of progression is absent,
the pain starting from the beginning in right iliac fossa. Anatomical variations of the
appendix influence the location of pain: a retrocecal appendix produces a pain in the
right flank, a pelvic appendix will induce suprapubic pain, and a retro-ileal appendix
will produce pain by irritating the testicular artery and ureter.
2. Anorexia usually accompanies acute appendicitis and it is an early symptom. In
over 95% of cases, anorexia is the first symptom, followed by abdominal pain. If
vomiting appears before the pain then the diagnosis of acute appendicitis is
uncertain.
3. Nausea and vomiting occur in 75% of patients, but they are not heavy or lasting,
most patients presenting only one or two vomiting.
4. Bowel disorders. Most patients have constipation before the onset of pain. However,
diarrhea is also present in some patients, especially children, making bowel
disorders less useful in the diagnosis of acute appendicitis. In elderly pseudo-
occlusive appendicitis may occur with stop of the bowel passage and abundant
vomiting.
Clinical signs
General signs
General clinical status of the patient is not significantly altered in uncomplicated
acute appendicitis. The temperature is rarely higher than normal with a degree, and the
pulse is normal or slightly increased.
Besides the classic appendicitis, when the diagnosis is easy, there are many cases,
especially in elderly, when symptoms and signs are not at all characteristic. It is
therefore required a careful examination of the patient.
The patient will be examined in supine position with knees slightly bent. The
examination begins with inspection of the abdomen with particular attention to
respiratory movements. The presence of postoperative scars in the right iliac fossa not
always rules out an acute appendicitis. There are
cases of acute appendicitis developed on an
appendicular stump after an incomplete
appendectomy. Then, follows the superficial and
deep palpation, starting from the left iliac fossa
progressing counterclockwise to the right iliac fossa.
(Figure 7) Abdominal percussion is very important;
often the diagnosis of peritoneal irritation relies on
this maneuver. Auscultation has a limited value.
Digital rectal examination (and vaginal in women)
will not be neglected.
The patient may have an antalgic position most commonly in supine with the
right thigh bended on the pelvis.
245
In rare cases, a scleral jaundice (Cplescu-Coscescu sign) can be observed.
Local physical signs
On inspection: a slight "delay" of the abdominal wall respiratory movements in the right
iliac fossa can be observed. In more advanced stages, with peritoneal irritation,
immobility of this region as a reflex against pain is present. In perforated
appendicitis with generalized peritonitis, abdominal wall movements are
abolished and of course, other general and local signs of generalized peritonitis
(Hippocratic face, abdominal muscle contracture, diffuse pain, and stop of bowel
movements) are present.
Coughing sign (Kusnirenko). Asking the patient to cough it will have a
defense reflex placing hands in the painful region. In cases with less intense
peritoneal irritation, coughing maneuver may be applied during right iliac
fossa palpation (the Dunphy sign). The pain increases during cough in case of
appendicitis.
On superficial palpation: skin hyperesthesia in the right dermathomeres innervated by
the spinal nerves T10, T11, and T12 is common, but not required in any acute
appendicitis.
In typical cases of appendicitis, cutaneous hyperesthesia, as an expression of
localized peritoneal irritation is obvious (known as Dieulafoy sign).
Voskresenski maneuver: pain can be produced by slipping fingers over the
right iliac fossa covered by the patient's shirt.
Lanz sign: gliding a sensitivity test needle on the right iliac fossa and flank
skin surface, no longer produces reflex muscle contraction while in unaffected
areas this reflex occurs.
On deep palpation: the pain is the most intense
in McBurney's point or Iacobovici triangle
bordered by the spino-umbilical line, bi-
spinal line and the lateral edge of the right
abdominal muscle. (Figure 8)
Rovsing's sign is inconsistent, but very
specific for acute appendicitis:
applying a pressure on the left iliac
fossa will push the gas into the colon
towards the cecum that will produce
its distension followed by pain in the
right iliac fossa.
The right iliac fossa pain caused by
sudden decompression of the abdomen
after manual compression in another area, usually the left flank, represents the
Blumberg sign.
Defense of the abdominal wall muscles is proportional to the intensity of the
inflammatory process in appendicitis. At the onset, muscular defense, if
present, is usually voluntary. As the inflammatory process evolves, it
intensifies and becomes involuntary, muscle spasm turning into a localized
muscle contraction.
In later stages, an intensely painful tumor, poorly defined, in the right iliac
fossa may be palpated: the periappendicular block.
246
In generalized peritonitis, caused by appendix perforation, signs of
generalized peritoneal irritation are present.
Only 50% of patients have typical signs of an acute appendicitis. Variations of
appendix position produce variations in physical signs. Thus, in retrocecal
appendicitis, the anterior abdominal signs are less expressive and pain is more intense
in the right flank. When the appendix is located in the pelvis, abdominal signs may be
absent and the diagnosis can be overlooked if a digital rectal examination to highlight
pain of the Douglas bag bottom is not performed.
Psoas sign (Iavorski-Lapinski) indicates an irritating process in contact or
near this muscle (retrocecal or retroperitoneal appendicitis). Pain is produced
by iliopsoas muscle contraction when lifting the right leg in extension with
the patient in supine.
Obturator sign (Romberg) is the occurrence of hypogastric pain produced by
internal obturator muscle extension. The sign appears during the internal
rotation of flexed right thigh with the patient supine.
In paracecal and retrocecal position of the appendix, palpation of the right
iliac fossa with patient in left lateral decubitus may help to find easier the
region of maximum pain (the Mondor maneuver) especially in obese patients.
On percussion
Percussion above the iliac fossa is a maneuver that causes pain in classical
acute appendicitis and it is one of the most accurate signs of peritoneal
irritation (the "bell sign " of Mandel).
Binet sign represents the hypersonority on percussion over the cecum area
caused by its paresis and air distension. The Stokes's law says: "Any of smooth
muscle beneath an inflamed serosa layer enters into paresis".
On auscultation
Bowel sounds may be normal, increased in some forms of peritonitis
(associated with diarrhea), reduced or even abolished in appendicular
peritonitis.
Digital rectal examination may reveal tenderness of the Douglas bag, the so-called
Duglas scream" or the Proust sign, especially in cases of fluid collections at this level.
Classically, the local signs present in the right iliac fossa form the Dieulafoy triad:
1. Spontaneous and provoked pain
2. Skin hyperesthesia
3. Muscle contracture
Investigations
Laboratory
Usual investigations for suspected acute appendicitis are leukocytosis and
urinalysis. In classic cases of acute appendicitis rarely, other laboratory investigations
are needed for diagnosis. However, there are atypical forms of acute appendicitis in
which other investigations for differential diagnosis should be carried out.
Whyte Blood Cells Count (WBC) value ranges between 10.000-15.000/mm3.
Leukocytosis in pregnant women is not helpful because it is physiologic. WBC above
these values advocates for a complicated form of acute appendicitis (perforation). A
normal WBC does not exclude an acute appendicitis.
247
Urinalysis is mandatory to rule out a possible urinary infection or renal, ureteral
colic. Sometimes, however, white or red blood cells may be present in urinary sediment
as a result of irritation of the bladder in an acute appendicitis with pelvic location or of
the ureter in a retroperitoneal location of the appendicitis.
Abdominal ultrasound with moderate compression was considered as an effective
method in the diagnosis of acute appendicitis.[12-14] Other studies concluded that there
is no proven clinical benefit of ultrasound scanning of the appendix in the routine
clinical diagnosis.[15] The appendix is viewed as a blind intestinal loop without
peristalsis, originating in cecum. Ultrasound is considered suggestive if the appendix is
incompressible and has a diameter of 6 mm or greater and conclusive in the presence of
a cone of shadow generated by a coprolite, while an appendix of 5 mm or less excludes
the appendicitis.[16,17] Ultrasound offers important information regarding other
associated illnesses.
Abdominal plain radiography, used in the evaluation of acute surgical abdomen is
usually unnecessary in acute appendicitis. However, sometimes a perforated duodenal
ulcer mimics acute appendicitis, but pneumoperitoneum can be seen on the X-ray. A
radio-opaque appendicular coprolite is found exceptionally, but this is a very specific
sign in the presence of symptoms. Radiological examinations in patients with acute
appendicitis should be used in cases of uncertain diagnosis but surgical treatment should
not be delayed when clinical signs are inconclusive.
Chest radiography is recommended usually for exclusion of the lower lobe
pneumonia as a cause of abdominal pain.
Computed tomography. Although it is considered equally or more sensitive than
ultrasound,[18] computed tomography is more expensive and also produces irradiation.
It should be reserved for differential diagnosis of an appendicular block (colon tumor,
ovarian tumor, kidney ptosis, etc).
Scintigraphy with radiolabeled leukocytes (Indium-111-labeled or 99mTc
HMPAO-labeled), serves to reduce the false-positive laparotomy rate and to shorten the
clinical observation time in patients with acute appendicitis.[19,20]
Laparoscopy can be both a diagnostic method and an approach for surgery
(laparoscopic appendectomy, ovarian cystectomy, etc.).
Due to the large number of unnecessary appendectomies, proven by
histopathologic examinations, and the
difficulty of diagnosis especially in
children and pregnant women, there were
concerns to develop diagnostic scores. In
1986, Alvarado introduced a score from 1
to 10, assigning points based on signs,
symptoms and leukocytosis for patients
with suspected acute appendicitis. Using
this score, Alvarado recommended
operation for all patients with scores of 7
points or higher and monitoring of those
with scores of 5-6. (Table 2) The presence
of a high Alvarado score in adult males is
highly predictive of acute appendicitis,
however, in women of child bearing age
other causes of similar clinical presentation lead to a low diagnostic accuracy of the
score.[21-23]
Table 2 Alvarado score
Symptom/sign/test Score
Migratory pain 1
Anorexia 1
Nausea/vomiting 1
Muscular defense in right iliac fossa 2
Blumberg sign positive 1
Temperature (>37.3C) 1
Leukocytes >10.000/l 2
Neutrophils >75% 1
Total 10

248
In rare cases, the evolution is favorable toward remission under treatment with
antibiotic, anti-inflammatory, and symptomatic medication.
Untreated, in almost all cases, the evolution is unfavorable towards serious
general complications such as septicemia (often with bacillus funduliformis) and
pilephlebitis (the result of multiple liver abscesses), or local complications, developing
different types of peritonitis:
Localized peritonitis:
Localized plastic peritonitis also called "appendicular plastron" or
periappendicular block, which means a mass of appendicular adhesions,
occurs after 24-72 hours from the onset of symptoms and is accompanied by
pain and vomiting which then calm down, being followed by constipation
and anorexia. The temperature rises up to 38-39C and the pulse remains
rapid. On palpation in the right iliac fossa, a tumoral mass can be felt which
is painful with diffuse defined boundaries and adherent to the anterior
abdominal wall. Under conservative treatment with antibiotics, anti-
inflammatory and local cold applications the periappendicular block can
regress in few weeks. Appendectomy can be performed 2-3 months later.
Periappendicular abscess appears in the centre of the periappendicular block
when the appendix perforates followed by accumulation of puss. Signs and
symptoms of infection amplify: fever becomes oscillatory accompanied by
chills, anorexia intensifies, and leukocytes grow up around 20.000/mm3. In
the absence of surgical treatment, the abscess, in most cases, brakes into the
peritoneal cavity leading to generalized peritonitis. Treatment consists in
evacuation of the puss through an incision that avoids the peritoneal cavity to
prevent the spread of the infection inside the abdomen. The aim of this
operation called oncotomy is only to evacuate the abscess not to remove the
appendix. The appendix, or what has remained from it after the destructive
process, will be removed after few (3-6) months. Appendectomy is indicated
to prevent further new episodes of acute appendicitis.
Generalized peritonitis:
The peritonitis in one-step is the typical generalized peritonitis that occurs
when the appendix perforates into the peritoneal cavity.
Another type of peritonitis is the so-called peritonitis in two steps: the first
step represents the local peritonitis (peritoneal irritation) of the acute
appendicitis, followed by a quiet period (giving the feeling that the
pathological process is healing) and then suddenly the appendix perforates,
especially after administration of purgatives.
The three-step-peritonitis: the first step is the local peritonitis of the acute
appendicitis, the periappendicular abscess represents the second step, and the
third step is represented by the general peritonitis as a consequence of
abscess rupture into the peritoneal cavity.
The differential diagnosis of acute appendicitis largely overlaps the differential
diagnosis of acute surgical abdomen.
Positive diagnosis of acute appendicitis is based on clinical signs and laboratory.
There are rare cases in which appendectomy is contraindicated and generally, most
249
diseases confused with appendicitis have indication for surgical treatment or are not
worsen because of appendectomy. The most common error is the false positive
diagnosis of appendicitis, but the worse is to overlook the diagnosis.
The most common causes of the incorrect preoperative diagnosis (75% of all
errors) are in the reverse order of frequency: acute mesenteric lymphadenitis, no
pathological changes, pelvic inflammatory disease (PID), ovarian cyst torsion, rupture
of the ovarian follicles and gastrointestinal acute enteritis.
The differential diagnosis of acute appendicitis depends on three main factors:
position of appendix, stage of the disease (presence or absence of perforation) and the
age and sex of the patient.
Acute mesenteric lymphadenitis. Most commonly confused with acute appendicitis
in children, is almost constantly associated with upper respiratory tract infection. In
lymphadenitis, abdominal pain is more diffuse and muscular defense is not strictly
localized as in appendicitis, it is usually voluntary and rarely reaches to contracture.
The presence of generalized enlarged lymph nodes may settle the diagnosis. Few
hours of observation, since lymphadenitis is a self-limited disease is the standard
procedure but if the diagnosis remains uncertain, surgery is indicated if just to
specify the diagnosis.
Acute gastroenteritis. A common childhood disease can be easily distinguished from
an acute appendicitis. Viral gastroenteritis, is an acute self-limited infection of
diverse etiologies and is characterized by profuse watery diarrhea, nausea and
vomiting. Pains are colicky preceding diarrheal stools; abdomen is relaxed without
local signs.
Acute cholecystitis. Confusion is possible for a subhepatic located appendicitis.
Ultrasound can sometimes settle the diagnosis. The treatment is still surgical.
Perforated peptic ulcer. Perforated ulcer can mimic an acute appendicitis if the
stomach content flows via the right paracolic space into the iliac fossa and if
neighboring organs rapidly block the perforation. History and pneumoperitoneum
observed on plain abdominal radiography can rule out the acute appendicitis.
Meckel's diverticulitis. Inflammation of the Meckel's diverticulum produces the
same clinical picture as acute appendicitis. Preoperative differential diagnosis is not
necessary, just for academic purposes, since diverticulitis is associated with the
same complications as appendicitis and requires emergency surgery (resection of
diverticulum, a procedure that can be performed by the typical open approach used
for appendectomy or by laparoscopic approach.).
Ileo-cecal intussusception. Unlike diverticulitis, preoperative differential diagnosis
is very important due to the different treatment. The age of the patient is important:
appendicitis is extremely rare under 2 years while almost all idiopathic
intussusceptions occur at this age. Ileo-cecal intussusception typically occurs by an
apparent crisis of abdominal colic. Several hours after onset, blood can be noticed in
the stool. Sometimes, in right iliac fossa, a sausage-shaped tumor mass can be felt.
Later, as intussusception progresses, the right iliac fossa seems abnormally free.
Ileo-cecal intussusception in infants is treated by barium enema reduction (when no
signs of perforation are present), so differential diagnosis of acute appendicitis
should be clearly established as barium enemas application in acute appendicitis in
an infant may have catastrophic consequences.
Regional enteritis. Symptoms of regional enteritis such as fever, pain, and muscular
defense in the right iliac fossa, and leukocytosis, can be found as well in acute
250
appendicitis. Diarrhea and the absence of anorexia, nausea, and vomiting favor
differential diagnosis but are not sufficient to exclude an acute appendicitis. In many
cases, regional enteritis is diagnosed during the operation for a supposed
appendicitis.
Other conditions of the colon. Perforated diverticulitis of the ascending colon, or
more often of a long sigmoid loop set in the right iliac fossa, or perforated tumor of
the cecum may be indistinguishable from a perforated appendicitis and are usually
intraoperative surprises. History is of great importance in these cases.
Ureteral stones. A stone in the ureter near the appendix can simulate retrocecal
appendicitis. Radiation of pain to the labia, scrotum, or penis, the positive sign of
Giordano, hematuria and/or the absence of fever or leukocytosis suggest the renal
colic. Ultrasound examination or pyelogram usually confirms stones.
Urinary infection. Acute pyelonephritis, especially on the right side, can mimic
acute retrocecal appendicitis. Chills, pains in the costo-vertebral angle as well the
presence of bacteriuria in urinalysis allow differentiation of the two diseases.
Primitive peritonitis. It rarely mimics a simple acute appendicitis; in turn, symptoms
are similar to those of the secondary peritonitis caused by appendicular perforation.
Diagnosis is set by bacteriological analysis of peritoneal fluid obtained by puncture.
Monomicrobial flora indicates a primitive peritonitis and it will be treated
medically, whereas a polymicrobial flora suggests secondary peritonitis.
Henoch-Schonlein purpura (anaphylactoid purpura, or purpura rheumatica). This
syndrome usually occurs in 2-3 weeks after a streptococcal infection. Abdominal
pain may be prevalent but associated purpura and nephritic syndrome are usually
present.[24,25]
Gynecological pathology. Acute appendicitis diagnosis errors are most common in
young women. The most common gynecological diseases, which are labeled as
acute appendicitis, are pelvic inflammatory disease, ovarian follicle rupture, torsion
of ovarian cyst or tumor, endometriosis and ruptured ectopic pregnancy. In all these
cases, diagnostic laparoscopy plays an important role.
Other conditions occurring in both sexes and at any age: bowel perforation caused
by foreign body, mechanical bowel obstruction, mesenteric infarction, lower right
pleurisy, acute cholecystitis, acute pancreatitis and abdominal wall hematoma.
Clinical forms by age
Appendicitis in young children (up to 3 years) represents only 2%. Diagnosis is
difficult due to lack of information about history and difficult physical examination.
Combination of C-reactive protein (CRP) with leukocytosis and elevated ESR
allows the diagnosis of appendicitis in 96% of cases in children. The attitude of
choice is surgery in doubtful situations.
Appendicitis in the elderly: due to weaker reactivity, the onset is attenuated. The
disease remains unidentified until its complicated forms (pseudo-occlusive or
pseudo-tumoral) resulting in increased postoperative mortality. Often, the muscular
defense is absent even in the presence of a perforation with local peritonitis.
Differential diagnosis should include the cancer of the cecum (in appendicular
block), other types of colon cancer or diastatic perforation caused by a descending
colon cancer. Ultrasound, CT scan, and colonoscopy are very useful in these cases.
If intestinal occlusion is manifest, surgery should be performed anyway.

251
Clinical forms depending on severity
Toxic form with peritonitis occurs more frequently in children. General signs of
intoxication are dominant whereas abdominal signs are mild. The onset is sudden
with rapid alteration of general condition. The patient is shocked, pale, with
tachycardia and pulse-temperature dissociation.
Subacute form, manifested with attenuated symptoms, with periods of remission,
with sensitivity of appendicular points but without muscular defense.
Diffuse purulent peritonitis appears after an episode of acute appendicitis interrupted
by a brief period of quiet (the two-steps peritonitis). Differential diagnosis is
difficult especially with peptic ulcer perforation. Laparoscopic approach, whenever
possible, is the best solution for diagnosis.
Clinical forms by topography
Retrocecal appendicitis - digestive symptoms and signs are poor, muscle contracture
is not present because the appendix is not in direct contact with the anterior parietal
peritoneum. Microscopic hematuria may be present, contributing to confusion with
urinary disorders.
Subhepatic appendicitis may cause confusion with acute cholecystitis. Ultrasound
examination may settle the diagnosis.
Mesoceliac appendicitis (20% of cases) evolves as an occlusive syndrome with
fever from the beginning. Appendix can be located pre- or retroileal. The pain is
mostly located around the umbilicus.
Pelvic appendicitis is often interpreted as salpingitis, acute sigmoid diverticulitis or
urinary disorders. Rectal digital examination is painful; sometimes the inflamed
appendix can be felt in the Douglas sac. It can evolve to pelvic abscess that may
open spontaneously into the rectum, or more rarely in the vagina or bladder.
Left-sided appendicitis is rare (0.1%) and develops in association with two types of
congenital anomalies: situs inversus totalis and midgut malrotation.[26-28] The most
common confusion is with sigmoid colon diverticulitis, a left ureteral colic, or left
adnexitis. In case of total situs inversus abdominal and chest radiography can guide
the surgeon to this diagnosis (the heart and stomach air bubble on right side).
Appendicitis in the hernial sac may raise confusion with incarcerated hernia.
Other forms of appendicitis
Appendicitis in pregnant women. In early pregnancy, due to the associated nausea
and vomiting, appendicitis can be confused with an ectopic pregnancy, abortion
threat or pyelonephritis. The pain associated with fever and muscular defense should
raise the suspicion of appendicitis. In the last months of pregnancy, the diagnosis is
even more difficult due to the distended abdomen. The appendix may be pushed up
in a high position by the enlarged uterus. Given the enlarged gravid uterus,
abdominal access should be attained using an open technique.[29] Scheiber
performed the first laparoscopic appendectomy in pregnancy in 1990.[30]
Appendicitis in patients with AIDS / HIV infection evolves with classical clinical
picture, commonly without leukocytosis. However, concern for a possible
opportunistic infection etiology and a desire to avoid operating on these patients, the
correct attitude is a diagnostic laparoscopy followed by appendectomy if
appendicitis is found.[31]

252
Treatment
History
Amyan, an English army surgeon, reported the first appendectomy in 1735,
performed without anesthesia to a soldier with perforated appendicitis.[32] In England,
in the late nineteenth century, surgeon H. Hancock successfully performed the first
appendectomy. Later, American surgeon C. McBurney published a series of articles on
the diagnosis and treatment of acute appendicitis.[33]
Indications
Appendectomy by laparoscopic or classical approach is still the main method of
treatment in appendicitis. Surgical treatment of uncomplicated acute appendicitis is an
absolute indication.
Randomized trials on medical treatment with antibiotics in acute appendicitis
showed that despite an initial success rate of 63-95%, early recurrences occurred in 20-
35% of cases, and these were mainly complicated recurrent appendicitis.[34-36] Even
though antibiotic treatment appears to be a safe first-line therapy in unselected patients
with acute appendicitis, the high rate of relapse with complicated forms retained
surgical treatment as the main form of treatment. Despite the wide range of effective
antibiotics, antibiotherapy as the only form of treatment is used in very specific
situations.
Antibiotics in acute appendicitis are associated to surgery as prophylaxis of septic
complications. The use of antibiotic therapy and local ice pack applied on the right iliac
fossa, in diagnosed acute appendicitis, cannot guarantee that the appendicitis will not
progress to serious complications.
Contraindications of surgical treatment
There are no contraindications for appendectomy in acute appendicitis. There is a
single exception: the periappendicular block, in which case most surgeons prefer to
perform appendectomy later after the resolution of inflammatory process, after 2-3
months.
In suspected acute appendicitis, should be avoided the preoperative administration
of purgatives, enemas, and painkillers. Antispasmodic drugs are allowed being useful in
differential diagnosis with colicky pain of another cause.
In cases with diffuse appendicular peritonitis, operation can be delayed a few
hours to allow medical measures to correct electrolyte imbalances.
Anesthesia
Classical appendectomy can be performed in any type of anesthesia: local, spinal,
epidural, general intravenous or by orotracheal intubation. Laparoscopic appendectomy
requires general anesthesia by orotracheal intubation.
Surgical technique. The classical open approach (It will be described more
detailed because usually this is the first more important operation performed by surgical
residents during guards)
The objective of the intervention is to remove the affected appendix.
There are many possible types of incisions in the right iliac fossa, (Figure 9) but
the most common used is the McBurney incision that provides a direct approach, in the
vast majority of cases, to the classical location of the appendix.
253

Sequences:
Preparation of the operative field: skin disinfection is not limited to the right
iliac fossa but extended to the entire anterior abdominal wall, because there
are cases (generalized peritonitis) when the McBurney incision must be
converted into a more extended laparotomy.
Opening the peritoneal cavity: skin incision, subcutaneous hemostasis, cutting
the Scarpa's fascia, longitudinal incision of the external oblique muscle
aponeurosis, dissociation of internal oblique and transverse muscles fibers,
incision of fascia transversalis, opening the peritoneum and isolation of the
peritoneal cavity. On opening the peritoneal cavity usually, the first
anatomical element that appears in the operative field is the greater omentum
attracted there by the inflammatory process. Particular attention should be
paid to the peritoneal fluid that may be purulent, fetid, denoting a perforated
appendicitis. A turbid odorless liquid is not a sign of perforation.
Finding the appendix is the next step and not always a very easy task. The
exploration is performed with two anatomical forceps. Finding the cecum is
easier and then, following the tenia leads us to the base of the appendix. In
difficult cases digital exploration of the abdominal cavity helps in finding the
appendix, which is felt as an
increased consistency cord.
Appendectomy.(Figure 10) Once
the appendix found, the
appendectomy can be conducted in
an anterograde or retrograde way.
When the tip of the appendix is
easily found and available in the
operative field, the anterograde
appendectomy is the choice. The
mesoappendix containing blood
vessels is cut and ligated between
two forceps. The base of the appendix is ligated near the cecum. A purse
string is applied on the cecum serosa around the base of the appendix. The
appendix is cut and removed (this represents the septic phase of the operation
and care must be taken to avoid contamination of the operative field). The
remaining appendicular stump is sunken (clogged) into the cecum using the
purse string (some authors consider this maneuver as a stump inversion but it
is not a real inversion because the appendicular stump is just covered by
254
cecum serosa). Optionally, a supplementary Z sero-serous suture can be
applied. When cecum inflammation is associated, the so-called typhlitis, the
appendicular stump cannot be covered by cecum using that purse string
because cecum walls are thickened, cardboard like. On the other hand,
appendicular stump during laparoscopic appendectomy is never sunken into
the cecum. When the tip of the appendix cannot be found or is far away from
the operative field, the retrograde appendectomy will be performed. The
appendix base is found, isolated and ligated near the cecum. The appendix is
cut and the stump is covered or not by the cecum walls using a purse string.
The appendix is then isolated and removed cutting the mesoappendix.
Hemostasis is thoroughly verified. If necessary, a drainage tub will be left in
close to the cecum.
Closing the abdominal wound. After changing gloves, the surgeon proceeds to
abdominal wall closure in anatomical layers. The peritoneum and abdominal
muscles will be approximated using absorbable sutures. Then, the aponeurosis
of the external oblique muscles will be closed. The wound is copiously
washed using hydrogen peroxide and the skin edges will be approximated
using interrupted sutures. In case of an intense contamination of the wound
(gangrenous appendicitis, perforated appendicitis), the wound will be left
open to prevent wound suppuration especially caused by anaerobic germs.
After few days, when the risk of suppuration diminished, the wound can be
sutured (delayed primary wound suture).
When the aspect of the appendix does not justify symptoms (normal appendix),
the surgeon should explore the nearby organs: right ovary (ovarian cyst), right fallopian
tube (pyosalpinx, ectopic pregnancy), and the last meter of the ileum for a Meckel's
diverticulitis.
Appendectomy is generally considered a simple operation, but there are cases
when this intervention can be very difficult because of atypical position of the appendix
(subhepatic, retrocecal, subserous) and/or because the patient is obese. In such cases,
often a larger incision is required. Laparoscopic approach has a major benefit in these
cases.
In perforated appendix with generalized peritonitis, the recommended approach is
the median laparotomy that allows a thorough abdominal lavage and multiple drainage
of the peritoneal cavity.
Laparoscopic approach
Laparoscopic appendectomy was first performed in 1987, but controversies still
exist regarding the balance between advantages and disadvantages.
Advantages:[37]
It allows the visual exploration of the entire abdominal cavity
Reduced risk of wound infection and incisional hernia
Good postoperative evolution with rapid recovery
Rapid socio-professional reintegration
Prevents postoperative adhesions
Disadvantages:
Requires general anesthesia
Requires well-trained team
Requires expensive equipment
Increased risk of dissemination of infection (intraperitoneal abscesses)
255
Contraindications for laparoscopic approach are represented by hemodynamic
instability, first and last trimester of pregnancy, and lack of surgical expertise, but there
are cases when laparoscopy should be converted to open approach such as:[38]
Appendicular mucocele (to diminish the risk of appendicular rupture and
peritoneal spread with the consequence of pseudomyxoma peritonei)
Extensive intraperitoneal adhesions
Generalized peritonitis
Inability to visualize the appendix
Uncontrolled bleeding
Tumor of the appendix extending into base (and still the right
hemicolectomy can be performed by laparoscopic approach)
Unexpected diagnosis
Laparoscopic appendectomy is performed
under general anesthesia by orotracheal intubation.
The patient is laid in Trendelenburg position slightly
turned to the left. The anterior abdominal wall skin
is disinfected and isolated. The pneumoperitoneum
(CO2) is induced using a Veres needle introduced
through the umbilical incision. Three ports are
sufficient in most cases: a 10 mm optic port at the
umbilicus, a 10-12 mm working port in the left
lower quadrant and a suprapubic 5 mm port. (Figure
11) There are many variations regarding position of
ports but the most important thing is to respect the
triangulation. The peritoneal cavity is inspected and
the appendix is found. The same principle of
following the confluence of the three colic tenias will indicate the base of the appendix
in difficult cases. The mesoappendix is sectioned using LigaSure or Ultracision device
(hemostatic clips can also be used). The base of the appendix is ligated or clipped; the
appendix is cut and removed through the working trocar. The appendix can be
introduced into a bag and then removed, to avoid contamination of the abdominal wall.
If necessary, a drain tube will be placed near the cecum. Trocars are removed and
wounds sutured.
Intraoperative complications. The most common complication is bleeding from
mesoappendix. Rarely, the cecum or small intestines are injured especially in case of
periappendicular block. These lesions should be recognized and repaired; otherwise,
they will cause severe generalized peritonitis.
The removed appendix should be examined histologically given the fact that it
may contain neoplastic cells (carcinoma or carcinoid).
Therapeutic attitude toward the periappendicular block is that of active
expectation". The patient will be treated with broad-spectrum antibiotics and local ice
bag applications. Local signs, temperature and leukocytosis will be monitored daily. If
evolution is favorable, going to resorption, appendectomy will be performed in elective
conditions after few weeks. If evolution is unfavorable, going to abscess formation, the
patient will be operated and oncotomy will be performed (extraperitoneal approach with
evacuation and drainage of the abscess). The wound will not be closed.

256
Postoperative care
Depending on the severity of appendicitis and postoperative complications,
hospitalization varies between one day and several weeks. Patients will receive
painkillers, anti-inflammatory, antiemetics and antibiotics according to the severity of
appendicitis and complaints. Drainage tubes will be removed depending on how much
they drain. Usually patients are discharged on the third postoperative day with the
recommendation to avoid intense physical activity for 2-4 months, and skin stitches are
removed on day 7-8 after surgery.
Complications occur in 1-3% of cases without significant differences regarding
the type of approach (open or laparoscopic).
Infection remains the most common postoperative complication being manifested
by wound infection or intra-abdominal abscesses. The occurrence of infection depends
on the severity of appendicitis, the patient's age, its co-morbidities and the type of
closure of the abdominal wall. Generally, wound infection rates are accepted up to 5%
and less than 1% for intra-abdominal abscesses. Treatment of wound suppuration is
wide opening and lavage with antiseptic solutions, while for intra-abdominal abscesses
percutaneous drainage and antibiotics are preferred.
Stercoral fistula is mainly the consequence of technical fault in performing the
purse string on cecum, when the needle is inserted through the full thickness of the
cecum wall. It occurs mainly in young inexperienced operators. Another possible cause
is the decubitus produced by the drainage tube. The wound should be widely opened,
and if there are signs of generalized peritonitis, laparotomy is required followed by
suture of the cecal lesion. In certain cases, even right hemicolectomy is required to
resolve the case. When the lesion is isolated and fistula discharge is minimal,
conservative treatment of fistula is tempted, given the chance of spontaneous closure.
Intraperitoneal bleeding is usually caused by skidding of the appendicular artery
ligature. It requires reintervention for hemostasis.
Early intestinal obstruction is usually caused by intra-abdominal adhesions or
bowel volvulus around the drain tubes maintained for a long period. It is manifested by
bloating, stop of intestinal transit, and vomiting. It requires laparotomy with resolution
of the cause that led to occlusion.
Residual intra-abdominal abscesses are manifested by fever, leukocytosis and
diarrhea accompanied by tenesmus, and distended painful abdomen. Abscesses are
located mainly in the bottom of the Douglas bag (detected by digital rectal and
ultrasound examination), but can also be located between intestinal loops or under the
diaphragm, in which cases the diagnosis is more difficult.
Late postoperative complications are represented by recurrence of appendicitis on
a long appendicular stump in case of incomplete appendectomy [39] and incisional
hernia.
Prognosis
Although postoperative morbidity is around 10%, the mortality rate is very low
being less than 1%, higher in elderly (up to 5%).[33,40-42]
257
3. Chronic Appendicitis
Chronic appendicitis is represented by micro- and macroscopic lesions of the
appendix resulted after (repeated) inflammatory processes of moderate acute
appendicitis that evolved towards resolution.
The macroscopic appearance of appendix can be:
Without obvious changes
Sclero-hypertrophic. The appendix is enlarged, thickened, well
vascularized with infiltrated mesoappendix and enlarged lymph nodes.
Sclero-atrophic. The appendix is small or thin with areas of stenosis or
uniform caliber and thickened mesoappendix.
Atrophic processes affecting mucosa, submucosa and muscular layers often
dominate histopathology.
Along with appendicular lesions, there is a regional lymphangitis. Often, the
appendix is adherent to neighboring organs (cecum, ileum, the right adnexa, omentum,
mesentery, sigmoid colon, etc.). Multiple membranes (Jackson) sometimes cover the
appendix.
Symptoms and signs
There are no specific symptoms for chronic appendicitis. The clinical picture may
vary greatly, mimicking almost any abdominal pathology.
The main complaints are:
Abdominal pain mainly located in the right iliac fossa, flank, and right upper
quadrant. The pain has nothing specific.
Dyspeptic phenomena manifested by bloating, nausea, belching and
frequently constipation.
Neuropsychological complaints represented by fatigue, insomnia, headache.
Clinical examination usually reveals a diffuse abdominal tenderness with
moderate pain within the right iliac fossa. Otherwise, there are no other pathological
changes unless an associated pathology is present.
Diagnosis and treatment
Except for histopathology, no other investigation can specify with certainty the
diagnosis of chronic appendicitis.
The diagnosis of chronic appendicitis is actually made by exclusion of other
conditions that may cause the patients symptoms. Usually these patients have multiple
admissions to various medical services, especially gastroenterology, and numerous
investigations, which do not reveal any obvious changes justifying symptoms. Most
often patients undergo conservative treatment, that brings temporary relief of symptoms,
but finally they will reach in a surgical department. Thorough medical history may be
helpful in diagnosis.
Treatment is represented by appendectomy preferable by laparoscopic approach,
which offers the possibility of general visual exploration of the abdominal cavity. In
many cases, although appendectomy was performed, complaints may persist after
surgery, and is good for the patient to be informed preoperatively on that eventuality.
258
4. Tumors of the Appendix
Neoplastic lesions of the appendix are present in about 5% of the cases examined
histologically after appendectomy performed for acute appendicitis. Most of these
lesions are benign. Malignant appendicular tumors represent only 0.4% of the digestive
tract malignant tumors.[43,44]
Preoperative detection of these tumors is very rare and intraoperative diagnosis is
made in less than 50% of cases.
Appendectomy may be sufficient for many of these tumors such as appendicular
mucocele, most carcinoid tumors and other benign tumors.
Classification
A. Benign tumors:
Inflammatory pseudotumors (fibroblastic appendicitis)
Appendicular endometriosis
Others (hyperplasia or metaplasia of the mucosa, leiomyoma, neurinoma,
lipomas, angioma, etc.)
B. Benign tumors with malignant potential:
Appendicular carcinoid
Villous appendicular tumor (papillary or adenomatous)
Benign appendicular mucocele
C. Malignant:
Appendicular adenocarcinoma
Endocrine - malignant carcinoid
Appendiculare sarcomas (fibroblastoma, lymphocytoma,
lymphoblastoma)
Lymphoma
Malignant appendicular mucocele (pseudomyxoma)
Appendicular adenocarcinoma
It is a rare condition, found in about 0.2% of all cases of appendectomy.[43,45]
The maximum incidence is at age of 40-69 (as in colic cancer).
Two thirds of cases are symptomatic, patients being operated for an acute
appendicitis, an abdominal tumor or intestinal obstruction. Perforation may complicate
the clinical picture, occurring in up to 40% of cases, but perforation alone has little
effect on overall survival.[43,45,46] Evolving for a long period without any symptom, the
tumor is recognized usually during the operation for a supposed appendicitis or just
after histology examination. Sometimes the tumor can be detected on irrigography, CT
scan or ultrasound examination.
Treatment is represented by right hemicolectomy (the same as for the right colon
cancer).
Prognosis is similar to that of the colon cancer.
Appendicular carcinoid
Carcinoids are the most common tumors of the appendix.[43] The appendix was
the most frequent location of carcinoid in the digestive tract (approximately 20%) but in
last decades, the small intestine ranks first.[47,48] Appendicular carcinoid tumors are
found in 0.3 - 0.9 % of patients undergoing appendectomy.[49]
259
The tumor is more common in women, the maximum incidence being reached at
the 4
th
and the 5
th
decades of life.[48]
Tumoral cells have argentaffine granules (argentaffinoma). There are two well-
differentiated carcinoid cell types: EC cells - serotonin-secreting (more common) and L
cells - secreting enteroglucagon or peptide YY (rare). The origin of cells can be neural
or may develop from Kulchitsky-Masson cells from the bottom of Lieberkhn mucosal
glands. Another form of carcinoid is the "cup cells", called mucinous carcinoid or
adenocarcinoid or carcinoma with "Goblet" cells. It represents only 6% of all carcinoids
appearing especially in elderly and is more aggressive than classical carcinoid.
Frequently metastasizes to the ovary and on peritoneal surface.
In 71%-82% of cases, carcinoid tumors are located at the tip of the appendix
giving the appearance of "drum stick".[50,51] Tumor growth is slow and spread is late
even when the tumor penetrates the appendicular wall.
The vast majority of appendicular carcinoid tumors are nonsecreting and therefore
carcinoid syndrome (facial flash, hypertension, tachycardia, and diarrhea) is rare.
Usually patients are operated with the diagnosis of acute or chronic appendicitis.
Therapeutic attitude depends on the location of the tumor, its size, presence of
metastases plus histopathological and immunohistochemical features.[52-54]
If the tumor is located on the body or tip of the appendix, is less than 2 cm in
diameter, and is not associated with metastases, appendectomy is sufficient.
If the tumor is located at the base of the appendix, is larger than 2 cm and/or is
associated with metastasis, right hemicolectomy is the operation of choice.
Right hemicolectomy is also indicated when histopathological examination
shows the following characters:
o tumor is invasive
o mucin-producing tumor
o tumor originates in mucous cells
Prognosis is generally good. Overall survival at 5 years is 70% - 85% and cases
with metastases have a better prognosis than those with metastases from other types of
cancers.[47] The most frequent metastases are in liver, with a good indication of their
removal.
Appendicular mucocele
Mucocele is a rare appendicular tumor (0.3% of all appendectomies) represented
by a unique or multiple cystic dilatation of the appendix, with a mucoid content.
There are four histopathologic forms: simple appendiceal mucocele, mucocele
with epithelial hyperplasia, cystadenoma and cystadenocarcinoma; the last two
subgroups represent neoplastic forms.[55]
Benign mucocele is an accumulation of mucus produced by Goblet cells into the
appendicular lumen, caused by its obstruction. As volume increases, the tumor may
become palpable and complication such as rupture or twisting may appear. It is easy
highlighted by ultrasound examination. Treatment consists in classical appendectomy
with caution not to disseminate the tumor content by breaking the appendicular wall. As
long as there is no preoperative certainty of benign origin, most authors contraindicate
laparoscopic appendectomy in these cases because of the risk to break the appendix
during manipulation and intraperitoneal dissemination of cancer.
Malignant mucocele (one case of nine) actually represents a first degree of
mucous papillary adenocarcinoma. The mucus contains muciparous cells that can
260
disseminate after spontaneous or therapeutic manipulation in the peritoneal cavity,
causing peritoneal pseudomyxoma ("gelatinous disease of the peritoneum"). Treatment
consists in classic appendectomy, sufficient as long there are no signs of extravasation
in the peritoneal cavity. In cases of mucocele with dilated (larger than 2.0 cm),
appendicular base partial cecal resection including the site of implantation of the
appendix is recommended.[56] The appendix is sent to frozen sections examiknation. If
the result is positive for malignity, right radical hemicolectomy is recommended.
If there is a broken malignant mucocele, appendectomy is associated with
intraperitoneal hyperthermic chemotherapy (60 minutes at 42.5
0
C).[57] When the
peritoneal pseudomyxoma is present, appendectomy is recommended (not
hemicolectomy) associated with cytoreductive surgery and intraperitoneal
chemotherapy.[58] When there are concomitant mucinous tumors of the ovary,
adnexectomy and even hysterectomy are recommended.
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Surgical Pathology of the Pancreas
SURGICAL PATHOLOGY OF THE PANCREAS

1. Surgical anatomy of the pancreas
2. Acute pancreatitis
3. Cancer of the pancreas
1. Surgical Anatomy
The pancreas is a mixed gland. It has two components:
1. The exocrine component, composed of glandular acini and ducts of
pancreatic juice excretion, and
2. The endocrine component, represented by pancreatic insulin secreting
islands of Langerhans, and other pancreatic hormones, which are released
directly into the blood stream.
The length of pancreas is about 15-18
cm and it weights 70-90 g. The pancreas was
divided arbitrary in 5 segments: (Figure 1)
1. Head
2. Uncinate process
3. Neck (isthmus)
4. Body
5. Tail
The pancreas is located deep in a
retroperitoneal region, represented mainly by
the Luschka-Grgoire celiac region, bounded
by the diaphragm, the transverse mesocolon
with its root, T10-L1 vertebrae posterior and anterior by the lesser gastric curvature and
the upper portion of the duodenum.
Pancreas and duodenum are secondary retroperitoneal organs. Due to the
presence of the duodeno-pancreatic fascia of coalescence (Treitz), formed between the
parietal peritoneum and visceral peritoneum, the incision of this fascia allows duodeno-
pancreatic posterior mobilization (the Kocher maneuver) during surgery in this region.
The pancreas is located across, from duodenal horseshoe (to the right) towards the
hilum of the spleen (to the left), being a fixed organ.
Anatomical relations
The root of the transverse mesocolon attaches on the anterior surface of the
pancreas dividing it into two regions: one inframesocolic and the other supramesocolic.
The head and neck of the pancreas (Figure 2)
The head of the pancreas is attached to the 2
nd
and 3
rd
portion of the duodenum (the
duodenal horse shoe) by the biliopancreatic confluence which flows into the
duodenum through the Vaters ampulla at level of papilla major (the papilla minor is for
the Santorini duct). The common bile duct (choledocus) passes through the posterior
surface of the pancreas.

263
Because of intimate anatomical
relations, the cancer of the head of the
pancreas rapidly invades these structures (the
CBD and duodenum) inducing jaundice and
duodenal obstruction. Removing tumors in
this case presumes also the removal the
whole duodenum and a portion of the CBD.
Another important relation of the
pancreas in this region is that with the portal
vein which passes on the posterior aspect of
the pancreas between the neck and uncinate
process. Pancreatic cancer can also rapidly
invade this vein inducing prehepatic portal
hypertension and determining the
inoperability of the case. The inoperability is
also determined by the invasion of the
superior mesenteric artery (SMA) which
passes behind the pancreas to join the
superior mesenteric vein in the root of
mesentery. (Figure 3)
Posterior, the head of the pancreas is
bordered by the inferior cava vein.
Due to the proximity of the celiac
trunk, expansive processes localized in the
neck and body of the pancreas may compress
or invade the celiac plexus causing intense
pain radiating to the dorsal region.
Mohammedan praying position of the
patient reduces compression on the plexus and thus the pain.
The body of the pancreas (Figure 4)
The anterior surface of the pancreas is covered by the parietal peritoneum
derived from the peritoneal sheath of the transverse mesocolon. It is exposed to gastric
antrum and corpus through the bursa omentalis. This retrogastric space communicates
with the peritoneal cavity through the Winslow foramen. This is the space where
collections and effusions may accumulate during acute pancreatitis leading to the
development of pancreatic pseudocyst. Incision of the peritoneal sheath and capsule of
the pancreas will free up the parenchyma allowing its distension in edematous
pancreatitis to prevent ischemia and necrosis of the pancreas.
Tumors of the body of the pancreas may grow up to high dimensions, as they are
asymptomatic for a long period. They may invade the stomach but the opposite situation
is more frequent when gastric tumors or ulcers invade the pancreas.
Due to the proximity of mesocolon and transverse colon, in pancreatic cancer of
the body, these structures can be invaded also.
As growing, pancreatic tumors, compress the duodenum D3 and D4 leading to
high intestinal occlusion.
The left lobe of the liver may be also involved by the tumoral process located in
the upper part of the body of the pancreas.
264

The tail of the pancreas
This segment is located in the left upper quadrant being bordered anterior by the
stomach, posterior by the left kidney, inferior by the transverse colon and on the left
side by the spleen and splenic flexure of the colon.
Because of its very intimate relations with the splenic hilum, the tail of the
pancreas can be injured during splenectomy with the consequence of pancreatitis of the
tail.
Pancreatic tumors in this part of the pancreas can also grow to high dimension
without symptoms invading surrounding structures. Unfortunately, in most cases, even
though the tumor is resectable, at the time of operation the cancer is already spread,
with liver metastases. Invasion of the splenic vein determines segmental portal
hypertension.
The exocrine pancreas is a ramified tubuloacinar gland, structured in lobules separated
by interlobular connective tissue. Exocrine pancreatic secretion consists of:
Trypsin
Chymotrypsin
Carboxypeptidase
Amylase
Lipase
Cholesterol-esterase
Phospholipase
These enzymes contribute to food digestion of carbohydrates, proteins, and fats.
The pancreas secretes 500-800 ml of juice per day. The alkaline pH of pancreatic
juice results from secreted bicarbonate that serves to neutralize gastric acid and
regulates the pH of the intestine.
A network of ducts that carry the exocrine secretions into the duodenum
represents the ductular system.
Centroacinar ducts - cells of these ducts actively secret important quantities
of sodium bicarbonate
Intra- and interlobular ducts
The main pancreatic duct of Wirsung, located in the parenchyma close to the
posterior aspect of the pancreas, opens into the duodenum through the major
duodenal papilla, the duodenal opening of the Vaters ampulla representing
the hepatopancreatic confluence. (Figure 5)
265

Lesser duct of Santorini is an accessory duct that drains the superior portion
of the head of the pancreas and empties separately into the second portion of
the duodenum through the papilla minor. In 30 % of cases, Santorini duct is
blind, and in 10 % of cases, it bears the entire pancreatic secretion. (Figure 6)

The pressure inside the pancreatic ducts is higher than in CBD (15 - 30 mm Hg,
versus 7 - 17 in CBD), thus preventing the reflux of bile and damage of the pancreatic
ducts, that can lead to an acute pancreatitis.
The endocrine pancreas consists of 200,000-1,800,000 pancreatic islands (1-2 % of
pancreatic volume), each containing about 200 endocrine cells. Density of the
pancreatic islands is highest in the tail of the pancreas.
Islet cells are of several types:
Cells A or (20%, produce glucagon)
Cells B or (75%, produce insulin)
Cells D or (produce somatostatin)
Cells non- (PP cells, produce pancreatic polypeptid )
Cells D1 (produce vasoactive intestinal polypeptid VIP)
Cells G (produce gastrin)
Cells C (clear cells without secretory granules), etc.
Insulin secretion starts from the fifth intrauterine month.
Arterial supply of the pancreas is ensured by three main sources: (Figure 7)
1. Common hepatic artery
2. Splenic artery (from celiac trunk)
3. Superior mesenteric artery
266
There are cases with multiple hepatic arteries (two or three) when the right
hepatic artery is usually a branch of the superior mesenteric artery (SMA), having an
initially retropancreatic route. This requires making a careful retropancreatic dissection
in pancreas excision surgery to avoid extensive hepatic necrosis, leading to death by
cutting this artery. Preoperative bi-selective arteriography of celiac trunk and SMA
would be very helpful to prevent this incident.
The three biggest pancreatic arterial branches are:
1. Dorsal pancreatic artery
2. Pancreatica Magna (mid portion of body)
3. Caudal pancreatic artery (tail)

The venous drainage of the pancreas follows the arterial supply.
Anterior and posterior arcades drain the head and the body of the pancreas. The
splenic vein drains the body and the tail.
Major drainage areas are:
Suprapancreatic vein
Retropancreatic vein
Splenic vein
Infrapancreatic vein which drains into superior mesenteric vein
Ultimately, all pancreatic veins drain into the portal system.
The rich periacinar network drains
into five nodal groups:
1. Celiac
2. Hepatic pedicle
3. Retroduodenopancreatic
4. Superior mesenteric
5. Splenic
Innervation
Sympathetic fibers come from the
splanchnic nerves and parasympathetic
fibers come from the vagus nerves. Both
types of fibers give rise to intrapancreatic periacinar plexuses. Parasympathetic fibers
stimulate both exocrine and endocrine secretion whereas sympathetic fibers have a
predominantly inhibitory effect.
267
Nerves form a rich afferent sensory network around the pancreas explaining the
intense pain in expansive processes of pancreas and chronic pancreatitis.
Ganglionectomy or celiac ganglion blockade (chemoneurolysis) interrupt these somatic
fibers with benefic effect on pancreatic pain.
2. Acute Pancreatitis
Acute pancreatitis represents the anatomo-clinical expression of the acute
syndrome of pancreatic and peripancreatic autodigestion.
Acute pancreatitis is an acute pancreatic inflammation, usually with rapid onset of
pain, accompanied by vomiting and systemic inflammatory response syndrome (SIRS)
with high levels of pancreatic enzymes in blood and urine (in urine not always present).
Epidemiology
Annual incidence is ranging from 5 to 80 per 100,000 inhabitants.[1-5]
In Europe and other developed nations, more patients tend to have gallstone
pancreatitis, whereas in the United States, alcoholic pancreatitis is the most common.[1]
Mortality and morbidity
The overall mortality rate of patients with acute pancreatitis ranges between 2%
and 15% but values depend very much on author.[1-3,6] Patients with biliary pancreatitis
tend to have a higher mortality rate than those with alcoholic pancreatitis. In patients
with severe disease (organ failure), the mortality rate is approximately 30%. This rate in
mortality has not dropped in the last 10 years.[3,7]
In the first week of illness, most deaths result from multiorgan system failure
(MSOF). In the following weeks, infection plays a more significant role, but organ
failure still represents a major cause of mortality.[8]
Race. Pancreatitis incidence is three times higher in blacks than in whites. These
racial differences are more visible for males than females.[1,9]
Sex. In general, acute pancreatitis affects males more often than females. The
etiology in males is more often related to alcohol, whereas in females to biliary tract
disease.
Age. The median age at onset depends on the etiology. The following are median
ages of onset for various etiologies:[10]
Alcohol related - 39 years
Biliary tract related - 69 years
Trauma related - 66 years
Drug induced etiology - 42 years
Endoscopic retrograde cholangiopancreatography related - 58 years
AIDS related - 31 years
Vasculitis related - 36 years
Hospitalization rates increase with age. For people aged 35-75 years, the rate
doubles for males and quadruples for females.[1]
Classification
Histopathological classification:
Edematous acute pancreatitis
Necrotic acute pancreatitis
Suppurated acute pancreatitis
268
Clinical classification. The 1992 Atlanta, Ga, International Symposium on Acute
Pancreatitis has classified pancreatitis into:[11]
Mild acute pancreatitis (80 %). Systemic dysfunctions are minor and
complete reversible.[12] Histopathology reveals interstitial edema and
possible adipose necrosis zones.
Severe acute pancreatitis. Life threatening complications are present
(pancreatic effusions and systemic complications also). Mortality is high,
ranging from 14% to 25% of these patients.[2]
Etiopathogenesis
Causes of acute pancreatitis are
various, pancreatic or extrapancreatic, but
alcohol and gallstones represent the most
common (80%).[12] (Figure 9)
Alcoholic etiology (30%-40%) is
more common in young, urban population,
mostly males. It occurs when regular large
amounts of alcohol, over long periods, are
consumed.[12,13]
Lithiasic etiology (40%-50%)
appears predominantly in elderly, rural
communities and in females.[14] When
gallstones are not removed, 36-63% of
cases are complicated with recurrent acute
pancreatitis because of stones migration.
There are many factors incriminated
for inducing acute pancreatitis:[12]
ctors:

dism
sulfonamides,
acycline, thiazide diuretics, furosemide)
ic renal failure (involved dyslipidemia, hemodialysis,
)
:
pulla stenosis, ascaris
r opistorchis sinensis)
g
cute spurt)
1. Metabolic fa
Alcohol
Hyperlipoproteinaemia
Hyperparathyroi
Hypercalcemia
Drugs (immunosuppressants, corticosteroids, estrogens,
metronidazole, tetr
Genetic (familial)
Toxic (organophosphates, scorpion venom)
Final stage of chron
peritoneal dialysis
2. Mechanical factors
Cholelithiasis
Postoperative
Posttraumatic (external or surgical injury, ERCP)
Pancreatic duct obstruction (tumors, am
lumbricoides infestation o
Pancreatic duct bleedin
Pancreatic duct stones
Chronic pancreatitis (a
Duodenal obstruction
269
Penetrating peptic ulcer
cyst, duodenal duplication,
edochocele, etc)
iopulmonary bypass)
nt surgery (kidney, liver, heart)
rteritis nodosa, LES)
ire, Mycoplasma pneumoniae, Cryptococcus),
failure, associated loop obstruction after gastro-jejunal
ry moment of the pathophysiological
chain
s and hemorrhage. Al these will lead
finally
process of the peripancreatic fat, thus
spots in the peritoneal
cavity
peritoneal tissues
worsening the prognosis.
The fluid may remain stuck around the pancreas forming pseudocysts.(Figure 11)
Acute fluid collections occur early in AP in the peripancreatic areas and are not
encapsulated by a fibrous wall. Pseudocysts are well-developed collections of
Crohn's disease
Anatomical abnormalities (pancreas divisum, annular pancreas,
heterotopic pancreas, duodenal parietal
duodenal diverticula, chol
3. Vascular factors (ischemia):
After surgery (intraoperative hypotension, card
Organ transpla
Hypotension
Vasculitis (peria
Ateroembolism
4. Infectious factors:
Viruses (mumps, Coxsackie B, cytomegalovirus, Epstein-Barr.)
Bacteria (BK, Leptosp
Yeasts (Aspergillus)
5. Various causes: cystic fibrosis (mucoviscidosis), food allergy, pregnancy,
end-stage renal
anastomosis, etc.
6. Idiopathic mechanism
Acute pancreatitis was compared by Lucien Leger with "an explosion in a
weapons factory", underlying the fact that eve
causes and aggravates the next moments.
The cascade of events begins with the intraglandular activation of digestive
hydrolases (proteolytic and lipolytic enzymes) and pancreatic lysosomal hydrolases,
causing swelling, hemorrhage and tissue necrosis in the gland and its adjacent tissues.
Exposure of trypsinogen to lysosomal enzymes is the mechanism for early trypsin
activation. Digestive enzyme release is amplified as acinar cells are destroyed, leading
to a vicious cycle of inflammation and necrosis. In addition to autodigestion
(responsible for the cytosteatonecrosis), vascular lesions are an aggravating factor.
Digestion of vascular walls results in thrombosi
to acute hemorrhagic pancreatic necrosis.
Lipase is involved in saponification
consuming calcium from blood leading to
hypocalcemia and formation of
cytosteatonecrosis
. (Figure 10)
The pancreatic fluid rich in enzymes
spreads into the retroperitoneal space
(retrocolic, perirenal, the root of mesentery,
mesocolon, celiac region, even in
mediastinum) and may reach down into the
scrotum in severe cases. These effusions
consist of necrotic retro
270
pancre
te of adverse
installation MSOF.
media
es: loss of water,
and
carditis, serous or
translocation.

atic fluid encapsulated by a wall of
granulation tissue (without epithelium).
(Figure 12) Pseudocyst typically occurs at 4
to 6 weeks after an episode of AP. A
pseudocyst that has become infected,
transforms into a pancreatic abscess.
Peripancreatic fluid may leak into the
peritoneal cavity (through the Winslow
foramen) giving rise to ascites.
Systemic response
Activated pancreatic enzymes and
other mediators entering the systemic
circulation via portal and lymphatic system
will lead to installation of the so-called
enzymatic toxemia with its sui
events leading to the
The systemic inflammatory response
syndrome (SIRS) can also develop, leading
to the development of systemic shock. The
tors of inflammation can become so
overwhelming that will lead to hemodynamic
instability and death.
Physiopathological events
Electrolyte chang
hypocalcemia,
tension, associated with
serohemorrhagic pericardial exudate.
xemia with pulmonary congestion,
hypomagnesemia.
Cardiovascular failure: hypovolemia leads to hypo
toxic or septic myo
Respiratory complications: hypo
pulmonary infarction, microatelectasis and in severe cases pulmonary failure
with acute respiratory distress syndrome (ARDS) and pleural effusions.
Renal impairment (caused by hypovolemia): deposits of fibrin and fibrinogen,
toxic and/or septic glomerular nephropathy. Patients with acute renal failure
have a significantly higher rate of pancreatic infection and mortality.
Other systemic disorders
Liver function impairment, manifested by elevated serum levels of bilirubin,
alkaline phosphatase and transaminases, caused by the obstruction of common
bile duct, hepatic parenchymal necrosis, and pericholangitis.
Early intravascular thrombosis, produced by proteolytic enzymes,
documented by decreasing platelet and fibrinogen, followed by marked
thrombocytosis and hyperfibrinogenemia.
Enzymatic encephalopathy.
Severe pain caused by strong nociceptive stimulation of the aorticomesenteric,
aorticorenal and celiac plexuses.
Bacteremia, due to bacterial
271
Local complications
Paralytic ileus. The inflammatory process induces the paralysis of the first
l image of "sentinel loop" on plain
abdominal radiography.
pression caused by the enlarged head of the pancreas,
y vomiting, or bile duct compression that produces jaundice.
M
pancreatitis are caused by interstitial inflammation,
etimes accompanied by lesions of cytosteatonecrosis.
sis will separate forming sequesters.
es infected
Clinical p
c
sea
in 85-100% of cases. It has a sudden onset
(but not as in peptic ulcer perforation) preceded by a meal with fat and alcohol, reaching
the m r and lasting at least 36-48 hours. Pain
gradually intensifies until reaching a constant ache. Location of pain is usually
epigastric, radiating in transverse direction (to the left and right upper quadrant) and
often
leads to discontinuation of transit (diarrhea, as well as digestive bleeding,
are signs of severity).
jejunal loop, which determines the classica
Duodenal com
manifested b
Necrotic lesions can extend to the wall of the stomach, duodenum, transverse
colon or common bile duct resulting in ulceration, bleeding, fistulas,
peritonitis, etc.
Pseudocyst formation
Suppurative complications such as effusions and abscesses.
orphopathology
Pathological changes of acute
bleeding, infection, and necrosis. Depending on the extent and intensity of lesions there
are three forms of acute pancreatitis:
interstitial) form - the pancreas is enlarged with a gelatinous 1. Edematous (
edema, som
2. Necrotic-Hemorrhagic form (severe form) - the pancreas is increased with
hemorrhagic and necrotic areas, of soft and friable consistency. In the further
development, areas of necro
3. Suppurated form - effusions are present being considered a complication of
progressive necrotic hemorrhagic form, pancreatic necrosis becom
developing pancreatic and peripancreatic abscesses.
icture
Although initial symptoms and signs are varied and can mimic other intra- or
extra-abdominal diseases, diagnosis in most patients, can be established by a thorough
linical evaluation. Typical symptoms are:
Upper abdominal pain
Nau
Vomiting
Low fever
Pain is the cardinal symptom present
aximum intensity at several hours late
to the lower back region. Severity of pain makes the patient restless, constantly
changing position in order to reduce the pain. If the etiology is biliary, pain may be
similar to biliary colic, but the persistence of pain after treatment suggests an acute
pancreatitis.
Nausea and vomiting install early and are persistent, rarely in large quantities (the
vomit fluid is primarily gastric and duodenal content, not fecaloid). Losses by vomiting
and impossibility of ingestion are major causes of body electrolyte and volume
depletion. Ileus is initially located in the upper abdomen (stomach, duodenum, proximal
jejunum) and
272
Fever, usually around 38C, is present in most cases in the first few days,
accompanied by tachycardia. Higher values announce the presence of necrosis and
infection.
Respiratory disorders (difficult breathing, persistent hiccups) are due to limitation
of left diaphragm movements (by neighborhood irritation) and left pleural effusion. In
severe cases, signs of acute respiratory failure may appear.
m and subsequently
re often hypoactive due to gastric and transverse colonic ileus.
bile duct stones, extrinsic compression, or toxic-septic hepatitis.
ary to
Clinical examination
Clinical signs depend largely on the severity of pancreatitis. Generally, the patient
is restless because of the abdominal pain. The patients face is reddish with congested
conjunctivae.
On palpation epigastric tenderness and/or muscular defense (rarely contracture) is
found. Sometimes the abdomen is distended initially in epigastriu
diffuse. Bowel sounds a
Often, the ascitis is present found as abdominal shifting dullness on percussion.
Jaundice is rarely present and has significance for severity. Causes of jaundice
can be common
Other clinical signs of severity are: (Figure 13)
The Cullen sign manifested by discoloration around the umbilicus resulting
from hemoperitoneum.
The Grey-Turner sign is a reddish-brown discoloration along the flanks
resulting from retroperitoneal blood dissecting along tissue planes. More
commonly, patients may have a ruddy erythema in flanks second
extravasated pancreatic exudate.

Grnwald sign represented by ecchymosis, large bruise, around the umbilicus
produced by local toxic lesions of vessels.
Mayo-Robson's sign, pain while pressing at left costovertebral angle.
Pleural effusion in the left hemithorax.
Shock signs: initially the patient is confused, agitated, with polypnea,
Som
a m
tachycardia, cold sweats with vasomotor crises (red face), possible mild
transient hypertension, and thereafter becomes pale, cyanotic, hypotensive
with weak and rapid pulse, entering into collapse.
Oliguria, which rapidly evolves to oligoanuria.
Laboratory
Hyperamylasemia (normal values of amylasemia are 8-32 Wolgemuth u, or <150.
ogy u, or <300 Phadebas u) becomes positive at 2-12 hours after the onset, reaches
aximum at 24 hours and returns to normal after 4-7 days in mild pancreatitis. The
273
diagnosis of acute pancreatitis is considered when abdominal pain is associated with an
hree times higher than normal. Dosage of amylase in effusions from
insuff
compensation only in case of
ukocytosis represents inflammation or infection.
re pancreatitis even though it is not
specif
the in
14)
y may show basal pulmonary

amylasemia t
peritoneum, pleura, and pericardium has a significant diagnostic value.
NB! In alcoholic pancreatitis with fibrosis and in necrotic pancreatitis with high
volume of destroyed pancreatic tissue, paradoxically at first sight, normal or even low
serum amylase can be recorded.
Amylasuria (normal 32-64 Wu, or <300 Somogy u., or <2000 Phadebas u) is
significant at over ten times normal values. It occurs later than amylasemia and persists
for a longer time.
Lypasemia (normal <200 IU) is more frequent and longer lasting (15 days) than
hyperamylasemia. Elevated lipase levels are more specific to the pancreas than amylase
levels.[15,16]
None of these tests is specific for pancreatitis. Elevations can occur in anyone
with small intestinal obstruction, mesenteric ischemia, tubo-ovarian disease, renal
iciency. Rarely, elevations may reflect parotiditis.
NB! The level of serum amylase or lipase does not indicate whether the disease is
mild, moderate, or severe, and monitoring levels serially during the course of
hospitalization does not reflect the evolution and prognosis.
Hypocalcemia appears on day 3-5 after the onset, caused by cytosteatonecrosis
and calcium fixation during this process. It requires
tetanus.
Hyperglycemia expresses the destruction of a large proportion of the endocrine
pancreas with glycosuria, being criteria of severity.
Blood urea nitrogen (BUN) is increased resulting from intense protein catabolism,
plus the functional and then organic ARF.
Le
The C-reactive protein value higher than twice its normal value (1-3 mg/dL)
indicates an important inflammatory reaction, a seve
ic for pancreatitis.
Imaging studies
Abdominal plain radiography. In some cases,
flammatory process may damage peripancreatic
structures resulting in a colon cut-off sign, a sentinel
loop, or an ileus. (Figure
Chest X-ra
atelectasis, left hydrothorax (relatively common) and
increased bronchovascular drawing (at the onset of
shock lung).
Gastrointestinal radiography with water-
soluble contrast agent (after exclusion of a perforated
ulcer) may show antropyloric stenosis, enlarged
duodenal frame, and descent of Treitz angle.
Abdominal ultrasonography (US) is the most useful initial test in d
etiolo
etermining the
oice for detecting gallstones.
nts with acute pancreatitis.[17]
e.
gy of pancreatitis and is the technique of ch
Abnormal US findings are seen in 33%90% of patie
Ultrasonography cannot measure the severity of the diseas
274
Abdominal contrast-enhanced computed tomography (CT) scanning. (Figure 15)
This i
patien

, peripancreatic phlegmon, pseudocyst.
itoneal effusions in prerenal spaces, retrocolic, root of the mesentery,
CT Grade Points
nvestigation, generally, is not recommended for patients with mild pancreatitis
unless a pancreatic tumor is suspected
(usually in elderly patients). CT
scanning is always indicated in
ts with severe acute pancreatitis
and is the imaging study of choice for
assessing complications. It is
performed within 72 hours after the
onset and then every 7-10 days (if
necessary). It is used as a prognostic
scoring system, having an important
impact in assessing acute pancreatitis
severity, and setting correct
therapeutic attitude. Pathological
findings that can be seen on CT scan
are:[17]
Pancreas increased in volume
depending on severity (edemato
hemorrhagic lesions, pancreatic ab
irregular glandular contour and thi
Peripancreatic edema, effusions
Retroper
with more or less alteration of its structure
us poorly defined areas of low density, necrosis,
scess with air pockets and sequesters, etc),
ckened fascia of Gerota.
etc.
Intraperitoneal fluid collections
Distended intestines and thickened posterior gastric wall.
Pleural effusions, basal pulmonary atelectasis.
Based on abdominal CT findings, in 1990 EJ. Balthazar [18] developed the
Computed Tomography Severity Index (CTSI) as grading system used to determine the
severity of acute pancreatitis. (Table 1)
Table 1 - Balthazar Grade
Balthazar Grade Appearance on CT
Grade A Normal CT 0 points
Grade B Focal or diffuse enlargement of the pancreas 1 point
Grade C Pancreatic gland abnormalities and peripancreatic
inflammation
2 points
Grade D Fluid collection in a single location 3 points
Grade E Two or more bubbles in or
adjacent to p
4 points fluid collections and / or gas
ancreas
Based on this system pancreatic necrosis is:
Necro Points
the risk of
sis Percentage Points Necrosis Percentage
No nec 4 points rosis 0 points 30 to 50% necrosis
0 to 30 50% necrosis 6 points % necrosis 2 points Over
Magnetic reso s ve ful in
diagnosing the bili pancreatitis when biliary or pancreatic duct
r the ERCP is that it is safer,
nance cholangiopancreatography (MRCP) i
ary etiology of
ry help
obstruction is suspected.[1] The great advantages ove
noninvasive, and faster.
275
Endosco phy u e y nd transducer
inserte
retrograde cholangiopancreatography) has a double role in
pancre
t when pancreatitis is associated with cholangitis. It is not performed routinely
but on
pic ultrasonogra (EUS) s s a high-frequenc ultrasou
d into the stomach and duodenum tract to visualize the pancreas and the common
bile duct. It is helpful in evaluating microlithiasis, biliary sludge, and periampullary
lesions.
ERCP (Endoscopic
atitis: to diagnose the biliary etiology (common bile duct lithiasis or other
obstructive causes) and a therapeutic role by removing stones from the CBD through a
papillosphincterotomy. (Figure 16) This procedure allows drainage of CBD, very
importan
ly in case of worsening condition (jaundice) in a patient with pancreatitis and
choledocholithiasis certified by ultrasound.[19,20] In case of failure of ERCP, surgery is
the solution.

Paracentesis is valuable for determining amylase concentrations in peritoneal
fluid and physical assessment of fluid (a severe pancreatitis is characterized by the
presence of dark peritoneal fluid).

Ultrasound or computer tomography guided aspiration biopsy may be indicated
in necrotic forms to determine the nature of peripancreatic collection (infection, etc.).
Diagnostic laparoscopy is recommended by some in emergency conditions to
confirm the diagnosis and visualization of lesions, to assess disease severity, but it
cannot provide complete information (does not allow exploration of the posterior aspect
of the pancreas). Almost 5% of patients require early laparotomy to exclude or treat a
276
possib
Differ
e beginning, and requires consideration of a series of other
diagnosis, such as:
ic aneurysm

n case of subhepatic retrocecal
ocardial infarction

onary diseases located at the bottom left thorax
ens
Prog
rognosis scoring systems, which in addition to
imaging m eters and have the advantage that can be
appli
evalua patients who will develop severe disease
fe-threatening complications, for a rational treatment approach.

can be
ehydrogenase (LDH) > 600iu/L
o Aspartate transaminase (AST) > 200iu/L
le mesenteric infarction, gangrenous cholecystitis or other conditions requiring
urgent surgical treatment.
ential diagnosis
Diagnosis of acute pancreatitis is based, as any diagnosis in general, on patients
history, local examination and investigations. Usually the diagnosis is not very difficult
in the presence of a history of lithiasis or alcohol abuse in a patient with intense
epigastric pain radiating in transverse direction. However, in many cases the diagnosis
is difficult, especially at th
diseases for differential
Surgical conditions:
o Perforated ulcer
o Ulcer penetrating the pancreas
o Biliary colic
o Intestinal mechanical occlusion, acute mesenteric ischemia
o Peritonitis
o Ruptured aort
o Splenic infarction
o Acute appendicitis (especially i
appendix)
Medical conditions:
o Inferior my
o Pulmonary embolism
o Reno ureteral colic
o Saturnine colic
o Pleuropulm
o Delirium trem
nosis evaluation
Prognosis evaluation uses some p
ethods use several biological param
ed routinely allowing continuous monitoring of patients.
an unpredictable natural evolution, the prognosis Because acute pancreatitis has
tion is aimed to early identification of
with increased risk of li
Several scoring systems are used such as APACHE II (Acute Physiology and
Chronic Health Evaluation), Ranson, and Glasgow. Balthazar score based on CT
findings was mentioned above.
Apache II is available on admission but is more difficult to calculate. However,
the score can be calculated online at http://www.sfar.org/scores2/apache22.php.
Glasgow scoring is simpler. One form of the Glasgow criteria suggests that a case
considered severe if at least three of the following are true: [21,22]
Age > 55 years
Blood levels:
o P02 Oxygen < 60mmHg or 7.9kPa
o White blood cells > 15
o Calcium < 2 mmol/L
o Urea > 16 mmol/L
o Lactate d
277
o Albumin < 32g/L
Th onic PANCREAS: [20]
g or 7.9kPa
mol/L
ydrogenase (LDH) > 600iu/L Aspartate
cose > 10 mmol/L
Ra n two situations: at admission
and with score is calculated differently for those two
major ca ry pancreatitis. (Table 3) [23]
ore Ranson biliary score
o Glucose > 10 mmol/L
is can be remembered using the mnem
o P02 Oxygen < 60mmH
o Age > 55
o Neutrophilia White blood cells > 15
o Calcium < 2 mmol/L
o Renal Urea > 16 m
o Enzymes Lactate deh
transaminase (AST) > 200iu/L
o Albumin < 32g/L
o Sugar Glu
nson criteria (introduced in 1974) are calculated i
in 48 hours after admission. The
uses of pancreatitis: alcoholic and bilia
Table 3 - Ranson criteria
Criteria Ranson alcoholic sc
Age in years > 55 > 70 years
White blood cell count > 16000 cells/mm3 > 18000 cells/mm3
Blood glucose > 10 mmol/L (> 200 mg/dL) > 12.2 mmol/L (> 220
mg/dL)
Serum AST (Aspartate
transaminase)
> 250 IU/L > 250 IU/L
At
admission:
Serum LDH (Lactate
nase)
> 400 IU/L > 350 IU/L
dehydroge
Serum calcium mmol/L (< 8.0 mg/dL) /L (< 8.0 mg/dL) < 2.0 < 2.0 mmol
Hematocrit fall > 10% > 10%
Oxygen PO2 < 60 mmHg PO2 < 60 mmHg
BUN increased by 1.8 or more
r more mg/dL)
d hydration
by 1.8 or more
r more mg/dL)
d hydration
mmol/L (5 o
after IV flui
increased
mmol/L (5 o
after IV flui
Base deficit > 4 mEq/L > 5 mEq/L
Wit
hours:
hin 48
uids > 6 L > 4 L Sequestration of fl
Interpr ositive d
can vary between 0 and 11 points. A score higher than 3 already announces the
possibility of evolving to a severe p
3, severe
If the score < 3, sever ely
Or
C
creatic sequesters (sequestrum/sequestra) represent delimitated fragments of
necrotic pancreatic and peripancreatic tissue, which appear in about the second week
af of sterile necrosis will evolve to resorption.
sis of peripancreatic tissues (especially fat
ti oot of the mesentery and retroperitoneum
etation. Each p Ranson parameter is note with 1 point, so the score
ancreatitis.
pancreatitis is likely
e pancreatitis is unlik
If the score
or
Score 3 to 4 : 15% mortality
Score 0 to 2 : 2% m tality
omplications
Pan
ter the debut of pancreatitis. Almost 50%
Effusions are represented by necro
ssue) with tendency to expand into the r
278
reachi
18) e
and l

of necrotic pancreatitis. The cyst is manifested by persistent pain, fixed upper
abdom
mon bile duct.
Treat
ajor therapeutic emergencies. The treatment is
compl aptation to
the cli
y, although most do
urgery and heal with conservative treatment. However, some of these cases
will r
n or presumptive diagnosis of severe acute
pancre
ng up to the mediastinum and down to the scrotum. In advanced cases, necrotic
tissues look like mud, having the same consistency, and black gray color.
Infection of pancreatic necrosis (Figure
volves to pancreatic abscess which
usually occurs in the second week and is
manifested by fever, abdominal distension,
palpable abdominal mass, pulmonary, renal,
iver complications and positive blood
cultures (50% of cases), most often with
Gram-negative germs (E. coli, Pseudomonas,
Klebsiella, Proteus). Infection is the most
frightening complication of necrotizing
pancreatitis, and is responsible for 80% of
deaths.
Pancreatic pseudocyst is a collection of
predominantly in the body and tail, which occurs
not have well defined walls, being bounded by nea
pancreatic fluid and sometimes necrotic debris
the onset
fluid located extra- or intrapancreatic,
in 1% of cases. Initially the cyst does
rby organs and adhesions. It contains
or blood. It appears at 2-3 weeks after
inal tumor, weight loss. Ultrasound and CT scan highlight the collection. The
pseudocyst can evolve to spontaneous remission within 4-6 weeks or to complications
such as suppuration, intracystic hemorrhage, intraperitoneal or intradigestive rupture,
compression on the stomach, duodenum, and biliary tract with stenosis, or on the
splenic vein with splenomegaly.
Intraperitoneal or upper gastrointestinal bleedings caused by erosion of an
artery: pancreatic, left gastric, or lienal artery.
Erosions of hollow organs walls such as stomach, transverse colon or small
intestines, with consecutive peritonitis.
Biliary fistula caused by necrosis of gallbladder or com
Vascular thrombosis (splenic, portal, mesenteric)
ment
Acute pancreatitis is one of the m
ex and cannot be standardized, requiring strict individualization and ad
nical and physiological stage.
Most mild cases are hospitalized in the department of surger
not require s
equire surgical treatment such as cholecystectomy for biliary stones or other
surgeries for pancreatitis complications. Unfortunately, the evolution of pancreatitis is
unpredictable. Patients with know
atitis should be hospitalized in an intensive care unit for monitoring the vital
function of organs and versatile support. A multidisciplinary team consisting of
intensive care doctor, surgeon, gastroenterologist and imagistic doctor will conduct
treatment.
Goals of treatment in acute pancreatitis are:
1. Limitation of pancreatic inflammation
2. Breaking the pathophysiological chain that leads to complications
3. General support and treating complications
279
Medical treatment
Aggressive treatment for preventing and treating hypovolemia and shock
closely
mo in order to act immediately in
ca ufficiency. Arterial pO2 below 70 mmHg requires nasal
ox bation
to achieve a high protein (1.5 g/kg/day) and
o avoid the occurrence of intestinal mucosal atrophy
roton pump inhibitors) antacids (sucralfate) and
f ARF (acute renal failure) is achieved through:
20 mg/dl).
ue to its antishock, anti-inflammatory, antitoxic and
ctreotide.
ring IDC
ays of evolution when there is an increasing hypercoagulability and
surgical treatment may be classified
accord erative moment as follows:
Fighting respiratory failure: patients with acute p
ored by pulse oximetry and blood gas analysis,
ancreatitis should be
nit
se of respiratory ins
ygen, while decreasing below 60 mmHg is an indication for tracheal intu
with assisted mechanical ventilation.
Putting at rest the pancreas is achieved by absolute food abstention and establishing
a continuous nasogastric aspiration. The aim is to suppress exo- and endogenous
stimuli of pancreatic secretion.
Nutritional support: it is important
caloric (25-30 cal/kg/day) intake.[24] Classical parenteral nutrition is supplemented
with modern enteral nutrition achieved through a jejunal tube placed endoscopically
or by minilaparotomy in order t
accompanied by bacterial translocation.
Restoring electrolyte and acid-base balance by administration of sodium,
potassium, magnesium, calcium.
Analgesia is a primary objective, given the severity of pain. The continuous epidural
anesthesia has a benefic role.
Stress ulcer prophylaxis is indicated in severe forms and is performed by parenteral
administration of antisecretory (p
gastric aspiration.
Prophylaxis and treatment o
adequate volume replacement, addition of dopamine infusion (if diuresis falls below
30 ml/h and creatinine rises above 1.4 mg/dl), diuretics (dopamine is associated if
BUN increases over
Antibiotics in acute pancreatitis can be used for cure if infection is set, being
conducted based on antibiogram, or for prophylactic purpose.
Corticosteroid therapy is beneficial in the initial phase of the disease, both by
protecting cell membranes and d
anti allergic effects.
Inhibitors of exocrine pancreatic secretion: Somatostatin and O
Prostaglandins (PG E1 and PG I2) and vasopressin appear to improve pancreatic
blood flow.
Anticoagulant therapy prevents thrombosis (involved in pancreatic necrosis with
vascular injury mediated by trypsin) and severe acute pancreatitis du
(intravascular disseminated coagulation). Anticoagulant treatment is recommended
after 7-14 d
increased risk of pulmonary embolism.
Surgical treatment
Most patients with acute pancreatitis will respond favorably to conservative
treatment with complete remission.
In necrotic-hemorrhagic pancreatitis,
ing to the optimal op
280
1. Immediate emergency interventions (within 8 hours) in patients with frank and
persistent symptoms of acute abdomen, peritonitis type, occlusive or hemorrhagic, after
exclud
Duodeno-pancreatic mobilization (Kocher maneuver)
creatic tissue
by edema compression
operations have exceptional indications (severe acute
undice and angiocholitis) and may consists of:
rainage - transcystic biliary decompression or by
th Kehr tube drainage
pancrea itis who develop early form of
tomy) and extrapancreatic necrosis. (Figure 19)
ing other causes of acute surgical abdomen. Surgical gestures maximum allowed
are:
Evacuation of hematoma and other collections in pancreatic lodge
"Capsulotomy - sectioning the pancreatic capsule allows pan

distension and preventing thus ischemic lesions caused
Pancreatic drainage
Peritoneal lavage and drainage
Associated biliary
cholecystitis, with obstructive ja
o Cholecystectomy
o Cholecystostomy
o External biliary d
choledocotomy wi
2. Early interventions, performed at 8 hours - 7 days after the onset of
titis, in patients with confirmed acute pancreat
acute surgical abdomen (microbial or chemical peritonitis, mechanical bowel
obstruction, intra- and/or retroperitoneal bleeding, mesenteric infarction). In case of
persistent papillary obstruction, endoscopic approach (ERCP and sphincterotomy) is
preferred.
3. Delayed interventions, performed at 8-21 days, for removal of pancreatic
(sequestrec
4. Scheduled interventions are performed late, at 3-6 weeks, for treatment of
pancreatic pseudocysts and abscesses. (Figure 20 and 21)

281
References
1. Gardner TB. - Acute pancreatitis - Medscape reference, eMedicine http://emedicine.medscape.com/article/181364-
overview#a0156
2. Sekimoto M, Takada T, Kawarada Y, et al. - JPN Guidelines for the management of acute pancreatitis: epidemiology,
etiology, natural history, and outcome predictors in acute pancreatitis. - J. Hepatobiliary Pancreat. Surg. 2006; 13(1):10
24.
3. Spanier BW, Dijkgraaf MG, Bruno MJ. - Epidemiology, aetiology and outcome of acute and chronic pancreatitis: An
update. - Best Pract. Res. Clin. Gastroenterol. 2008; 22(1):45-63.
4. Appelros S, Borgstrm A. - Incidence, aetiology and mortality rate of acute pancreatitis over 10 years in a defined urban
population in Sweden. - Br. J. Surg. 1999; 86(4):465470.
5. Banks PA. - Epidemiology, natural history, and predictors of disease outcome in acute and chronic pancreatitis. -
Gastrointest. Endosc. 2002; 56(6 Suppl):S226230.
6. De Bolla AR, Obeid ML. - Mortality in acute pancreatitis. - Ann. R. Coll. Surg. Engl. 1984; 66(3):184186.
7. Lund H, Tnnesen H, Tnnesen MH, Olsen O. - Long-term recurrence and death rates after acute pancreatitis. - Scand. J.
Gastroenterol. 2006; 41(2):234-238.
to acute pancreatitis: a retrospective analysis of 405 autopsy
Dig. Dis. Sci. 1985; 30:1005-1018.
s PA. - A new classification system for acute pancreatitis. - Am. J. Ggastroenterol. 1994; 89:151-152.
terol. 1990;
n K, Worthley C, Reece J, Bessell J, Thomas D. - Evaluation of amylase and lipase
ent of Severity with Clinical and CT Evaluation. - Radiology 2002; 223:603-
stablishing prognosis.
V, Di Giuli M, Minervini S, Sportelli
ediction of severity in acute pancreatitis: prospective comparison of
DF, Fink SD, Eng K, Spencer FC. - Prognostic signs and the role of operative
. - Nutritional support in acute pancreatitis. - J. Dig. Dis. 2012; 13(9):445-452.
8. Renner IG, Savage WT, Pantoia JL, Renner VJ. - Death due
cases. -
9. Fagenholz PJ, Fernndez-del Castillo C, Harris NS, Pelletier AJ, Camargo CA. - National study of United States
emergency department visits for acute pancreatitis, 19932003. - BMC Emerg. Med. 2007; 7:1.
10. Chang MC, Su CH, Sun MS, Huang SC, Chiu CT, Chen MC, Lee KT, Lin CC, Lin JT. - Etiology of acute pancreatitis -a
multi-center study in Taiwan. - Hepatogastroenterology 2003; 50(53):1655-1657.
11. Bank
12. Guo-Jun Wang, Chun-Fang Gao, Dong Wei, Cun Wang, Si-Qin Ding. - Acute pancreatitis: Etiology and common
pathogenesis. - World J. Gastroenterol. 2009; 15(12):14271430.
13. Lankisch PG, Assmus C, Pflichthofer D, Struckmann K, Lehnick D. - Which etiology causes the most severe acute
pancreatitis? - Int. J. Pancreatol. 1999; 26(2):55-57.
14. Lankisch PG, Assmus C, Lehnick D, Maisonneuve P, Lowenfels AB. - Acute pancreatitis: does gender matter? - Dig.
Dis. Sci. 2001; 46(11):2470-2474.
15. Agarwal N, Pitchumoni CS, Sivaprasad AV. - Evaluating tests for acute pancreatitis. - Am. J. Gastroen
85(4):356-366.
16. Treacy J, Williams A, Bais R, Willso
in the diagnosis of acute pancreatitis. - ANZ J Surg. 2001; 71(10):577-582.
17. Balthazar EJ. - Acute Pancreatitis: Assessm
613.
18. Balthazar EJ, Robinson DL, Megibow AJ, Ranson JH. - Acute pancreatitis: value of CT in e
Radiology 1990; 174(2):331336.
19. Fiocca F, Santagati A, Ceci V, Donatelli G, Pasqualini MJ, Moretti MG, Speranza
G, Giri S. - ERCP and acute pancreatitis. - Eur. Rev. Med. Pharmaco. Sci. 2002; 6(1):13-17.
20. Fan ST, Lai EC, Mok FP, Lo CM, Zheng SS, Wong J. - Early treatment of acute biliary pancreatitis by endoscopic
papillotomy. - N. Eng. J. Med. 1993; 328(4):228-232.
21. Wikipedia - Pancreatitis - http://en.wikipedia.org/wiki/Pancreatitis
22. Corfield AP, Cooper MJ., Williamson RC, et al. - Pr
three prognostic indices. - Lancet 2, 1985; (8452):403407.
23. Ranson JH, Rifkind KM, Roses
management in acute pancreatitis. - Surgery, Gynecology & Obstetrics 1974; 139(1):6981.
24. Ong JP, Fock KM

282
3. Cancer of the Pancreas
Exocrine pancreas cancer is the fifth cause of death from cancer after lung cancer,
olorectal, breast, and prostate.[1,2] In other reports it ranges the forth.[3,4]
Malignant tumors of the exocrine pancreas are particularly aggressive, with one
f the lowest survival rates at 5 years (4-6%).[5] Pancreatic cancer has the highest
ortality rate of all the major cancers. About 75% of patients with pancreatic cancer die
ithin the first year of diagnosis.[6]
pidemiology
The incidence of pancreatic cancer in the U.S. lies about 10/100,000. Estimated
ew cases and deaths from pancreatic cancer in the United States in 2011 are 44,030
ew cases and 37,660 deaths.[6] The number of new pancreatic cancer cases and the
ber of deaths caused by this disease are increasing - not decreasing. In fact, the
expected number of new pancreatic cancer cases is estimated to increase by 55%
between the years 2010
c
o
m
w
E
n
n
num
and 2030.[3,7]
Average age of diagnosis is around 65 years. It is rare before 40 years, the
incidence increasing rapidly in the elderly.
Sex ratio shows a slight m e predominance (: = 1.5 to 2/1). al
There is a higher incidence in African-American population and in Hebrew of the
USA, and in Polynesian. The lowest incidence appears to be the Indians and people of
the Middle East.[8]
Etiology
The exact causes of cancer are not known, but statistical studies and clinical
observations incriminate some exogenous or endogenous risk factors:[9]

Exogenous factors (lifestyle factors):[10]
Smoking (mainly cigarette, but also cigar or pipe) is incriminated, inhaled
nitrosamines reaching in the pancreas through blood or bile. A recent European study
found an increasing risk by 27% for ev

ery five cigarettes smoked per day.[11]
ing coffee, tea or alcohol is under debate
(opini
medical conditions
Diet. Increased consumption of unsaturated fats and red meat are incriminated as
risk factors.[12] The possible role of drink
ons are divided).[13-15]
Professional chemical agents (naphthylamine, benzidine, oil, etc.), oncogenic
viruses, unfavorable socio-economic status, etc., represent other factors.
Predisposing
cified chronic pancreatitis - People suffering from pancreatitis have a higher
risk fo
d with diabetes
but m The risk of cancer is almost two-fold
higher in people diagnosed with type II diabetes.
and, it was found that previous tonsillectomies, as well as
Cal
r pancreatic cancer ranging from two to more than 20.[9,16]
Diabetes mellitus: About 15% of pancreatic cancers are associate
echanisms are incompletely elucidated.
[17,18] Metformin, an anti-diabetes
drug, is associated with a 62% decrease in pancreatic cancer risk, compared with
diabetic patients who do not receive this medication.[19]
Previous surgery on the digestive tract: gastric resections (decrease of gastric
acidity that cannot detoxify nitrosamines produced by bacterial metabolism and as well
digestive hormones changes)[20], cholecystectomy (elevated levels cholecystokinin)[21-
23], and others. On the other h
283
some allergic diseases, are protective factors (immunological factors involved in
pancre
isk for pancreatic [24]
and
atic carcinogenesis).[21]
Pernicious anemia is considered a condition of increased r
gastric cancer (possible caused by trophic effect of associated chronic
hypergastrinemia) although some studies did not find a causal relationship between
pernicious anemia and subsequent development of pancreatic cancer.[25]
Genetic factors
Their involvement in the etiology is supported by the existence of family clusters
of the
pancre %) in two
by direct
and via the
lymph
being the
extra-
forms and accompanied by a worse prognosis than
ductal
arc lary cys
topogra ly
f 6 cm).[29] Complete resection is accompanied by
survival at 5 years is 50% in cases plete resection and
75% i uch better than in ductal adenocarcinoma.
ms e of mucin,
es is characteristic for
enign or malignant, functional (with excessive
r non-functiona
as a large tumor with imprecise limits but with a
disease, the possible association of the disease in the context of known genetic
syndromes (familial polyposis, Gardner syndrome, Lynch syndrome, Hippel-Lindau
syndrome, etc.), as well the higher incidence in certain populations (black, Hebrew,
Polynesian, etc.).[26,27]
Morphopathology
Malignant tumors of the pancreas are mostly derived from exocrine parenchyma,
but may develop from endocrine cells also (Langerhans islets cells proliferation). (Table
1)
Ductal adenocarcinoma is
the malignant most common
atic tumor (80
thirds of cases being located in the
head, neck, or uncinate process.
When diagnosed, over 52%-
85% of cases are already extended
beyond the organ
invasion, perineural
atic system.[3,28]
Remote metastases occur
most commonly in the liver and
peritoneum, the lungs
abdominal organ most likely
to receive metastases.
Giant cell carcinoma,
squamous cell carcinoma, and
acinar cell carcinoma are rare
Table 1 Tumors of the pancreas
ORIGIN TUMOR TYPE
Ductal epithelium Adenocarcinoma (75%)
Carcinoma with giant cells
Unclassified carcinoma
Squamous carcinoma
Mucinous carcinoma
Microadenocarcinoma
Cystadenocarcinoma
Cystic papillary carcinoma
Acinary cells Carcinoma of the acinary cells
Islet cells Malignant insulinoma
VIP-oma
Glocagonoma
Gastrinoma
Nonepit

helial tissue Fibrosarcoma
Leiomyosarcoma
Hemangiopericytoma
Histiocytoma
Lymphoma

adenocarcinoma.
Cystic neoplasms (cystadenoc
types, found mostly in women, without
large dimensions (average diameter o
a very good prognosis (
inoma, papil
al
tic carcinoma, etc.) are rare
elative preferenti phy, developing to r
of incom
n cases of complete resection), m
Histologically, ductal neoplas
while the presence of zymogen granul
Endocrine tumors can be b
hormone production) o
are characterized by the presenc
acinar neoplasms.
l.
Pancreatic lymphoma appears
relatively favorable prognosis, showing a good response to chemotherapy and
radiotherapy.
284
Metastatic pancreatic tumors, although rare clinically, at necropsy are found in
number of four times higher than primitive malignant tumors of the pancreas. In
descending order of frequency, they are found in cases of breast cancer, lung cancer,
malignant melanoma, gastric cancer, and colon cancer.
Clinical picture
Evolution of pancreatic cancer is divided in four stages:
1. Asymptomatic period. There are no specific symptoms. The disease is
discovered incidentally during investigations for other complaints. The onset
of symptoms is more than 4 month earlier to the moment of diagnosis.
bophlebitis (Trousseau sign), loss of appetite, epigastric discomfort
igns depend on where the tumor
d and they are produced by invasion of the tumoral process into the
ut unfortunately, surgical
sign),
C
t of jaundice.
as, tumors can also grow to high
il in advanced stage. Tumor can produce segmental portal
ons on the spelnic vein. The incidence is 10% of
localized in
epig
pain upectoral (knee-chest) position - the
cancer is on
2. Period of onset. There are insignificant symptoms: physical and mental
fatigue, depression, tendency to weight loss, possible migratory superficial
throm
discrete dyspepsia. On clinical examination, there are no significant changes.
Diagnosis is possible only by imagistic investigations. This is the stage when,
if diagnosis is made, surgical treatment may be curable in oncological terms.
3. Symptomatic stage. Symptoms and clinical s
is locate
neighborhood organs. Diagnosis is relatively easy b
treatment, in most cases, will not have a radical or curable visa.
4. Advanced stage. Tumor may be palpable, there are liver and other systemic
metastases, ascites, supraclavicular lymphadenopathy (Virchow-Troisier
umbilical metastasis (sister Mary Joseph sign), signs of portal hypertension,
superior digestive bleeding, and other symptoms and signs of terminal state.
Only symptomatic treatment may be applied in this stage.
linical picture depending on tumor location
Cancer of the head of the pancreas (Figure 21) evolves with specific
clinical triad:
1. mild epigastric pains
2. severe weight loss
3. progressive jaundice (compression of the common bile duct)
Tumors are earlier diagnosed than in other locations due to rapid onse
The incidence is 66% of all pancreatic tumors.
Cancer located at body of pancreas evolves with unspecific clinical picture,
so tumor can grow to large dimensions compressing the retroperitoneal
structures, especially celiac nervous plexus that will induce intense permanent
dorsal pain. Tumor is diagnosed in late stages. (Figure 23)
Located at the tail of the pancre
dimensions being diagnosed in late stages. They do not produce any specific
symptoms unt
hypertension by compressi
pancreatic tumors.
Pain features. In most cases, pain is the principal symptom being
astric region, radiating to dorsal, lumbar, or interscapular region. It is a continuous
, intense in advanced cases, ameliorated by gen
"Mohammedan's prayer sign" (anterior flexed body reduces compression on the
retroperitoneal nervous plexuses). Management of pain for patients with pancreatic
e of the most important aspects of their care.
285

Jaundice features. It is present in 50% of cases at admission. Jaundice is not
accompanied by colicky pain and fever like in lithiasis. The color is verdinic (skin has
an intense yellow to green color). The jaundice is progressive and it is accompanied by
pruritus (itching). It does not react to medication (antispasmodic drugs). All these
features are important in differential diagnosis with jaundice of other causes.
Other possible symptoms:
Weight loss - by poor appetite, malabsorption, duodenal stenosis
Depressive syndrome (75% of cases)
Onset of diabetes (50% of cases)
Gastrointestinal bleeding - secondary to tumoral invasion of the stomach
or duodenum, or due to esophageal varices appeared consecutive to
splenic and portal vein compression
Migratory thrombophlebitis

are pr
b or it can
b
(
Workup
emia, increased ESR, increased values of
b inotransferase and AST - aspartate
am line phosphatase and glucose. In advanced cases, laboratory tests
l function.
1. Abdominal ultrasound is usually the first examination performed especially
when the jaundice is present to rule out its lithiasic etiology. In pancreatic head tumors,
Subcutaneous metastatic nodules
Arthritis
The intense verdinic jaundice is easily observed on inspection. On palpation the
disten the so-called Courvoisier-Trrier sign. These signs ded gall bladder can be felt,
esent when the tumor is located in the head of the pancreas but they are absent in
ody and tail location. Liver can be globally enlarged caused by biliary stasis,
e nodular when metastases are present
In advanced stage, the tumor can be palpated in the epigastric region and ascites
peritoneal carcinomatosis) can be detected on percussion (abdominal shifting dullness).
Laboratory tests could reveal an
ilirubin, transaminases (ALT - alanine am
inotransferase), alka
reveal the impaired rena
Tumoral markers such as CA 494, CA 19-9, -feto protein, CEA can be elevated
but without absolute specificity.
Imaging studies are the most important in diagnosis. A lot of investigating
procedures are available. Their application depends much on pancreatic tumor
characteristics (location, dimension, stage, etc.).
286
the ga
as is enlarged with intensely modified structure. Ultrasound can highlight the
n 2 cm. As well as ultrasound, it can guide further
diagno
ll bladder is very distended, under tension, and the CBD and intrahepatic ducts are
enlarged as the consequence of stenosis produced by tumoral compression. The head of
the pancre
liver metastases, portal hypertension, and ascites. Confusions may appear with chronic
pseudotumoral pancreatitis, which has identical aspect. Ultrasound findings are highly
related to examiners experience and ultrasound device performance.
2. Computed tomography (CT-scan), performed with oral and intravenous
contrast, has a higher sensitivity and therefore is used in all cases with suspected
pancreatic cancer. (Figure 24 and 25) The main drawback is that it might miss some
tumors with a diameter less tha
stic maneuvers such as percutaneous biopsy or biliary drainage.

3. MRI has no additional advantages over the CT scan except the fact that it does
not irradiate. Unfortunately, it cannot be applied to very obese patients, to those bearing
aneurism clips or cardiac pacemaker or to patients suffering of claustrophobia.
4. CT or MR cholangiography is a CT or MRI investigation associated with
intravenous or oral biliary contrast agent. (Figure 26) It provides noninvasive
information about the morphology of the biliary tract. The main limitation of these
contrast agents is that the rate of allergic reactions and of renal or hepatic toxicity (or
both) is relatively high.[30]
5. Endoscopic retrograde cholangiopancreatography (ERCP) can differentiate
cephalic pancreatic tumor from other periampullary tumors producing obstructive
jaundice. It offers the possibility to perform biopsies and to introduce stents into the
common bile duct in cases of inoperable patients. It is an invasive method being
associated with some risks and complications
(complications rate ranges from 0.5% to 5%).[31]
transh
can be also followed
by an external biliary drainage by introducing a
drainage tube into the intrahepatic biliary duct.
6. Percutaneous transhepatic
cholangiography (PTC) is also an invasive
method. A fine needle is introduced
percutaneously under ultrasound guidance
epatic into a biliary duct and a contrast
agent is injected. The anatomy of the biliary tract
is very well delineated. PTC is more useful in
tumors of the CBD than in those of the pancreas
where ERCP is superior. It
287
7. Endoscopic ultrasound is especially useful for evaluating tumors of the head of
the pancreas. It allows the assessment of the actual size and local invasion, and guides
the transduodenal biopsies.[32-34]
8. Vascular explorations (angiographies) are represented by splenoportography
to evaluate the segmental portal hypertension on
angiography of the celiac trunk and SMA to ev
the splenic vein, and selective
aluate the relations of the pancreatic
tumor h impo umor resectability.
sess the macroscopic aspect of the
e extension of the tumor to other
sonography of the pancreas and
g a special probe. It can also guide
fferential diagnosis (gastroscopy,
e the diagnosis with certainty is
itis mimicking pancreatic cancer and
s for histopathological examination
ing, and fever precede the jaundice. Jaundice is not as intense as in
pancre
ommon bile duct - ERCP highlights the tumor, its

ors compress the
ble to be
wit rtant vascular structures and to assess the t
8. Laparoscopic investigation can be used to as
pancreatic region, establishing the resectability and th
organs under direct visualization. Intraoperative ultra
liver may be performed through this procedure usin
the pancreatic biopsy.
9. Other investigations necessary for di
colonoscopy, etc.)
The only one investigation that can mak
histopathology. There are cases of chronic pancreat
only histopathology can make the difference. Sample
can be obtained by several methods:
For cytological examination from:
Samples obtained by fine needle aspiration (imaging guide o d)
o Cells from duodenal content
o Samples obtained by endoscopic brushing of the tumor or laparoscopic
exploration
o Peritoneal fluid
For histological examination from:
o Cylinder of tissue harvested by tru cut biopsy
o Tissue fragments removed by surgical or endoscopic biopsy
Differential diagnosis usually includes other causes of jaundice when jaundice is
present. The most common differential includes the jaundice caused by biliary stones
migrated into the CBD. In this case colicky pains in the right hypocondrium associated
with nausea, vomit
atic cancer, having fluctuant intensity. On the other hand, in pancreatic cancer,
the onset of jaundice is silent without any pain, and is constantly intensifying. Another
common differential diagnosis includes the jaundice present during decompensated liver
cirrhosis.
Other conditions that should be included in differential diagnosis are:
Periampullary tumors:
o Tumor of the Vaters ampulla - duodenoscopy and endoscopic biopsy
put the diagnosis
Tumor of th o e c
location and allows biopsies
Duodenal tumors - especially when pancreatic tum
duodenum manifested by vomiting
Chronic pancreatitis is sometimes very difficult or even impossi
differentiated from a pancreatic cancer. The clinical picture and imagistic
findings may be very similar to that of cancer. In cancer, calcifications are not
as frequent as in chronic pancreatitis.
Tumors from neighborhood organs penetrating the pancreas (stomach, colon)
288
Tuberculosis or sarcoidosis (rare cases) - biopsy can make the difference
Staging [35]
Primary Tumor (T)
TX Minimum requirements cannot be met
Nod
l node involvement (no laparotomy)
ct in
inated (Tis, N0, M0).
or is 2 cm or smaller in the pancreas. It has not spread to lymph nodes or
other the
Stage umo o lymph nodes or other
parts e body (
Stage : A tum ead to nearby arteries
, N0, M0).
Stage A tum teries or veins. It has spread to lymph
nodes or T3; N1; M0).
Stage A tumo lymph nodes but has not spread to
other the
Stage Any tu
Treat
Treatm x and multimodal (surgery, oncology,
pain therapy .).
n the head of the pancreas may have the chance to benefit from
ent. However, only 10-20% of pancreatic
lar structures (portal vein, superior mesenteric
oral inflammatory process even though on CT
are two types of surgery:
T1 Limited to pancreas, less than 2.0 cm in diameter
ncreas, 2 to 6 cm in diameter T2 Limited to pa
T3 Over 6 cm in diameter
T4 Extrapancreatic direct extension to contiguous structures
al Involvement (N)
NX Minimum requirements cannot be met
N0 No metastatic nodes
N1 One regional group involved at laparotomy
N2 Two or more regional groups involved at laparotomy
N3 Clinical evidence of regiona
N4 Involvement of juxtaregional nodes
Distant Metastasis (M)
MX Not assessed
M0 No (known) distant metastasis
M1 Distant metastasis present
Cancer stage grouping
Stage 0: Refers to cancer in situ, in which the cancer has not yet invaded outside the du
which it orig
Stage IA: The tum
parts of body (T1, N0, M0).
IB: A t r larger than 2 cm is in the pancreas. It has not spread t
of th T2, N0, M0).
IIA or extends beyond the pancreas, but the tumor has not spr
ad to any lymph nodes or other parts of the body (T3 or veins. It has not spre
IIB: or of any size has not spread to nearby ar
but not to other parts of the body (T1, T2,
III: r has spread to nearby arteries, veins, and/or
parts of body (T4, N1, M0).
IV: mor that has spread to other parts of the body (any T, any N, M1).
ment
ent of pancreatic cancer is comple
, etc
Tumors located i
radical surgery because they produce early mechanical jaundice and so the tumor can be
diagnosed in an earlier phase of developm
cancers benefit from radical surgery.[36] In most cases, tumor cannot be removed
because it penetrates the major vascu
artery) or other organs. In these cases, only palliative surgery can be performed.
Usually, there is an important peritum
scan images the tumor seems to be resectable.
Patients with unresectable cancer have a median survival of 6 months.
Satisfactory solution in these situations is the endoscopic placement of stents for biliary
obstruction (effectively equal to that of surgical bypass). Only patients with duodenal
obstruction need bypass surgery but duodenal stenting in nowadays is also possible.[37-
39]
Surgical treatment of pancreatic cancer is dependent on tumor topography and
stage. There
289
1. Operations with radical intention - they intend to remove the tumor in
oncological safety margins and the afferent lymph nodes, followed by
restoration of digestive continuity.
2. Palliative operations - do not remove the tumor but, through different
procedures, they are trying to prolong life and improve quality of life in these
patients.
Types of possible procedures are:
Radical surgery:
Cephalic duodenopancreatectomy (CDP) (pancreatoduodenectomy) - for
tumors located at the head of the pancreas
Splenopancreatectomy (corporeo-caudal pancreatectomy) - for tumors of the
tail
Subtotal pancreatectomy - for tumors of the body
Total pancreatectomy - for tumors of the body
Palliative surgery
For jaundice:
Open surgery biliary diversion
o External
o Internal
Percutaneous biliary diversion
Endoscopic stenting of the common bile duct
For duodenal compression
Gastroenteroanastomosis (GEA) operation
Duodenal stenting
For pain
Chemoneurolysis
Splanchnicectomy
1. Cephalic duodenopancreatectomy (Whipple procedure -1935) is an extensive
operation aimed to remove in oncological limits the canc
pancreas. Because the very intimate relations with m
er located at the head of the
any important anatomical
ficult one being performed by
well-trained surgeons. During this operation the following organs or parte of them
head of the pancreas (pancreatectomy), the duodenum
(d my), the gallbladder (cholecystectomy)
and the lower part of the common bile duct. After removing all this anatomical
segmen f the digestive tract must be restored. In Whipple
p -side hepaticojejunostomy, end-to-side
p (Figure 27) There are
tion regarding the type of anastomosis
b
2. Pylorus preserving cephalic duodenopancreatectomy (Traverso-Longmire
) was designed for a better physiological function of the stomach.
Preserv rapid emptying of the stomach and the consecutive
dum
structures, especially vascular, the operation is a dif
are removed: the
uodenectomy), the gastric antrum (antrecto
ts, the continuity o
rocedure, restoration is achieved by end-to
ancreatojejunostomy and end-to-side gastrojejunostomy.
some possible variations of this opera
etween the pancreas and the jejunum.
procedure -1978
ing pylorus prevents the
ping syndrome. (Figure 28)
3. Cephalic duodenopancreatectomy with pancreaticogastrostomy (Waught and
Clagett - 1944) is a variant in which the pancreas is anastomosized to the posterior
wall of the stomach. (Figure 29)
290

4. Distal pancreatectomy and splenectomy (Figure 30) is an operation that is
performed for tumors located at the tail of the pancreas. Distal pancreatectomy
means the removal of the tail and part of the pancreatic body. For oncological and
technical reasons the spleen is also removed.

291
Cancers with this location can spread to the lymph nodes in the hilum of the spleen
and can invade the splenic vessels. The distal pancreatectomy is performed in much
less time and requires a shorter recovery period, than cephalic
duodenopancreatectomy. There are no digestive anastomoses. The procedure can be
performed by laparoscopic approach. Unfortunately, almost all cases of pancreatic
body cancers are unresectable due to the high dimension of the tumor, which rapidly
invades the nearby anatomical structures. In rare cases when tumors are diagnosed
in early stages two types of operation may be performed:
5. Total pancreatectomy with regional lymphadenectomy, which means en block
excision of the entire pancreas together with duodenum, spleen, greater omentum,
6. Subtotal pancreatectomy means that the spleen and almost the whole pancreas are
excised remaining a small part of the head of the pancreas attached to the
duodenum. (Figure 31)
antrectomy and the corresponding lymphatic nodules. The operation is very similar
to CDP, but in addition, the pancreas is completely removed. (Figure 31) The
continuity of the digestive tract must be reestablished in the same way. The
procedure is followed by very serious iatrogenic diabetes, which is very difficult to
correct.

Intraoperative complications
The most common and unpleasant complications are intraoperative vascular
lesions especially of the portal vein that cause significant bleeding. They are solved
by suture or vascular reconstruction.
During the 1960s and 1970s, the morbidity and mortality for
pancreaticoduodenectomy were so high that many thought the operative procedure
ought to be abandoned.[40] During the 1980s, however, many centers reported
mortality rates around 5% and a morbidity of 25% to 60%.[41,42] In high-volume
centers, with a substantial experience, mortality decreased below 5% and morbidity
below 40%.[43]
Many early local complications (more than 45 types) may appear in the
postoperative period. Anastomotic fistulas or leaks (at site of
292
pancreaticojejunostomy, hepaticojejunostomy and gastrojejunostomy) are the most
important complications with the highest rate of mortality. The most frequent
anastomotic leak is that of the pancreas with the jejunum. When the defect is
minor, conservative treatment may lead to healing but when the dehiscence is
important, surgical repair (reanastomosis or other procedures) become necessary
due to general peritonitis. Peritonitis and intraperitoneal abscesses are
consequences of anastomotic leaks. Intraperitoneal bleeding with hemoperitoneum
is possible (about 3% of cases) [44] the more so as these patients are under
treatment with anticoagulants. Wound suppuration poses no particular problems of
treatment but it is followed in almost all cases by incisional hernia.
Palliative surgery. Unfortunately, almost 85% of pancreatic cancers are unresectable at
time of surgery. To prolong the patients life and relieve symptoms is necessary to treat
the complications of pancreatic cancer:
Common bile duct stenosis - with jaundice
Duodenal stenosis - with vomiting and inanition
Transverse colon stenosis - with intestinal occlusion
uses invasion
S
Pancreatic duct stenosis - with pain
Pain caused by nerve plex
olutions for jaundice are represented by:
xternal biliary diversions
) - is
A. E
rarely used. It is applied just incases where
B. In
patic region as
Cholecystostomy (Figure 32
endoscopic stenting or internal diversion are impossible (lack of endowment,
very ill patients who cannot undergo other type of operations).
CBD external drainage (Figure 33) - the drainage T tube (Kehr) is introduced
into the CBD during surgery when the pancreatic tumor is considered
unresectable. The procedure presumes cholecystectomy. These types of external
drainage unfortunately will lead to fluid and electrolytes (especially potassium)
loss being rarely applied.
ternal biliary diversions
Using the gallbladder: cholecysto-gastrostomy or cholecysto-jejunostomy.
Choosing one of these procedures depends on local anatomical condition and
surgeons option. (Figure 34)
Using the CBD: choledoco-duodenostomy or choledoco-jejunostomy. (Figure
35) Internal biliary diversion using the common bile duct is performed when the
gallbladder cannot be used either because of an acute cholecystitis or due to its
absence after cholecystectomy. Choledoco-duodenostomy can be performed in
many ways. The most common used is the side-to-side anastomosis between the
CBD and duodenum as in Florkens procedure. The diversion can be also
performed between the CBD and a jejunal loop ascended in subhe
an omega loop or as Roux-en-Y loop. The same jejunal loop can be used for
gastric diversion in case of duodenal compression.
293

294
C. Nonsurgical biliary diversions. There are minimal invasive procedures that can divert
the bile outside or into the duodenum. Patients in advanced stages withstand
better these procedures. The most common procedure in nowadays is the
endoscopic transduodenal stenting of the CBD. (Figure 36) This procedure can
be also the first step before the radical surgery, releasing the patient from
jaundice.

In patients who do not support any surgery or stenting cannot be performed, the
percutaneous drainage of the biliary tract may be a solution. (Figure 37) It is
performed under ultrasound and eco-doppler guidance. A catheter is placed into a
biliary duct and bile is diverted outside.
Resolving the duodenal stenosis
obstru
anasto
en-Y
(Figur
About 10-30% of patients requiring biliary bypass, develop later, duodenal
ction requiring surgical reintervention. The solution is a gastro-jejunal
mosis, which can be performed in many ways using an omega loop or a Roux-
loop. (Figure 38) A non-surgical procedure is the endoscopic duodenal stenting.
e 39)

295

Fighting pain
Chemoneurolysis represents the infiltration of the celiac plexus with 50% phenol
or alcohol during palliative surgery with net improvement in pain for several months
(Figure 40), either before or after surgery by percutaneous approach (Figure 41) under
radiological or ultrasound guidance. This nerve block may last for up to 3 or 4 months
as the nerves were "numbed" and the block tends to wear off over time.
Thoracoscopic splanchnicectomy is a minimally invasive procedure that cuts
specific nerve branches.
Another modality to assist with pain management is the external beam radiation
therapy. The radiation beam is directed at the tumor and may provide fast onset of pain
relief.

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Surgical Pathology of the Liver
SURGICAL PATHOLOGY OF THE LIVER

1. Surgical anatomy of the liver
2. Liver abscesses
3. Hydatid cyst
4. Liver tumors
1. Surgical Anatomy of the Liver
The liver is a very important intra-abdominal organ, belonging to the digestive
system and having numerous functions in the human body, especially in metabolism. It
is the largest intra-abdominal viscera.
The liver lies below the right diaphragm in the upper right quadrant of the
abdomen. It is a reddish brown organ, which has four lobes of unequal size and shape:
the right, left, caudate, and quadrate lobe. The weight of the adult liver varies from
1200 to 1800 g. Its diameter is about 28 centimeters in the transverse direction, and
about 16 centimeters in sagittal direction. (Figure 1)

Segmentation
Functionally, the right and left lobe are of about equal size and are separated by a
line extending from the inferior vena cava (posterior) to the middle of the gallbladder
fossa (anterior). This is the most important
topographic line for orientation in liver surgery.
(Figure 2)
The Couinaud classification of liver
anatomy divides the liver into eight
functionally independent segments. Each
segment has its own vascular inflow, outflow,
and biliary drainage. Segment 4 is sometimes
divided into segment 4a and 4b according to
Bismuth classification. The numbering of
segments is in a clockwise manner. (Figure 3)
299

Anatomical relationships
The liver has three surfaces: superior, inferior and posterior.
1) The superior (diaphragmatic) surface is convex and comes up against the
diaphragm and the anterior wall of the abdomen. It is completely covered by peritoneum
except along the line of attachment of the falciform ligament. The falciform ligament
defines anatomically the right and left lobes. On the right lobe, costal arch impressions
can be observed and on the left the heart impression. The diaphragm separates the liver
from the lower part of the lungs and pleura, the heart and pericardium and the right
costal arches from the seventh to the eleventh inclusive. Pathological processes located
here (especially hydatid cyst and abscesses) may affect the pleural cavity (pleurisy,
empyema) and even the lung (biliobronchial fistula).
2) The inferior surface is divided into four lobes by five fossas arranged in the
form of the letter H. (Figure 1)
The left limb of the H marks the division of the liver into right and left lobes. It
is known as the left sagital fossa, and consists of two parts: the fossa for the umbilical
vein in front and behind, the fossa for the Arantius ligament, which was a venous duct
connecting the umbilical vein to the inferior cava vein in the intrauterine life.
The right limb of the H is formed in front by the fossa for the gall bladder, and
rear by the fossa for the inferior vena cava. These two fossas are separated by a band of
liver substance, termed as caudate process. The bar connecting the two limbs of the H
is the liver hilum where the portal vein separates into two branches (transverse fissure)
entering the liver. The quadrate lobe lies in front of it and the caudate lobe behind it.
The transverse fissure contains the liver hilum formed by hepatic pedicle consisting of
hepatic artery, portal vein, hepatic duct, lymphatics and nerves. The hepatic duct lies in
front and to the right, the hepatic artery to the left, and the portal vein behind and
between the duct and artery.
The inferior surface is in relation with the stomach and duodenum, the right colic
flexure, and the right kidney and suprarenal gland. Impression of these organs can be
seen on the liver surface. The surface is almost completely covered by peritoneum.
Quadrate lobe comes against the pyloric portion of the stomach, the duodenum, and
transverse colon. Caudate lobe has relations with the diaphragm, cava vein, esophagus,
celiac trunk, the upper edge of the pancreas and the lesser curvature of the stomach.
3) The posterior surface of the liver is broad behind the right lobe, but narrows on
the left. Over a large part of its extent, it is not covered by peritoneum (pars affixa or
area nuda) and is in direct contact with the diaphragm.

300
Fastening elements of the liver
Ligaments, inferior vena cava, and hepatic pedicle represent the fastening
elements of the liver. However, the liver has a certain degree of mobility mainly related
to respiratory dynamics.
The liver is connected to the diaphragm and to the anterior abdominal wall by five
ligaments: the falciform, the coronary, the two triangular ligaments, and the round
ligament, which is a fibrous cord representing the obliterated umbilical vein. The liver is
also attached to the lesser curvature of the stomach by the hepatogastric ligament and to
the duodenum by the hepatoduodenal ligament, which together form the lesser
omentum.
Vascular supply
The common hepatic artery arises
from the celiac trunk. It branches into
hepatic artery and gastroduodenal artery.
Proper hepatic artery divides in the hilum
into the right and the left branch. It gives a
series of collateral branches: pyloric
artery, cystic artery, terminal branches.
(Figure 4)
Portal vein is the venous trunk
collector of blood from the digestive tract.
The inferior mesenteric vein joins the
splenic vein and is continued by the portal
vein. Portal vein forms behind the
pancreas, and collects the gastric vein, left
pyloric vein and right superior
pancreaticoduodenal vein. The portal vein
length is about 8-10 cm and the caliber of
10-11 mm. In the hilum, it divides into
two branches: right and left. The right
branch is larger and continues the
trajectory of the portal vein. The left
branch is longer but thinner. Segmental
branches on both sides are divided into
upper and lower branches. Last
ramifications of the portal vein are the
interlobular veins, which form the
perilobular network. (Figure 5)
There are also some small accessory
portal veins from different anatomical
regions, which enter the liver.
Oxygen is provided from both sources; approximately half of the liver's oxygen
demand is met by the portal vein, and the hepatic arteries meet the other half.
Venous drainage
Hepatic veins drain the blood from the liver into the inferior cava vein. They can
be differentiated into two groups, the upper group, and lower group. The upper group,
formed by three veins, typically arises from the posterior aspect of the liver. The left
hepatic vein engages between medial and lateral segments; the middle hepatic vein
301
crosses the main fissure between the two
lobes of the liver and the right hepatic vein
passes between anterior and posterior
segments. Almost constantly, the middle and
the left vein join into a common trunk near
the cava and the right remains independent.
(Figure 6)
The lower group arises from the right
lobe and caudate lobe; veins are variable in
number and typically smaller and shorter
than those in the upper group are.
Innervation
Nerves contain sympathetic and parasympathetic fibers originating from vagus
nerves and celiac plexus. At liver, nerves branches form the anterior and posterior
hepatic plexus.
Liver structure
The liver is covered by the Glisson capsule, which enters the liver through the
hilum following the blood vessels, and together with vascular network divides the liver
parenchyma into lobules.
The liver lobule is the anatomical and functional unit of the liver, having a
pyramidal shape with the base oriented to the liver surface and the tip inward. On cross
section, it looks like a polygon with 5-6 sides. Its dimensions are rated on average 1.5 -
2 mm long and 1 mm wide. In the center, there is a centrilobular vein. The portal spaces
of Kiernan are formed between at least three joining lobules. Kiernan spaces contain
connective tissue, a branch of the portal vein and hepatic artery, and one or two bile
canaliculi, which form the portal triad, and also lymphatics, and nerves. Blood flows
from the portal space towards the centrilobular vein and the bile in reverse, from the
center towards the periphery of the lobule.
Liver cells are contained in Remak cords arranged radially. Between cells and
capillary wall, there are the Disses spaces. Between the vascular endothelial cells,
Kupffer cells are located, phagocytes which participate to hemoglobin degradation.
Between the Remak cords, narrow spaces are formed, called the bile canaliculi.
Bile ducts represent a complex set of channels by which the external secretion of
the liver, the bile, is transported from the hepatocytes to the duodenum. Biliary ducts
inside the liver are called intrahepatic bile ducts and those outside the liver are the
extrahepatic bile ducts. In general, intrahepatic biliary ducts follow the route of portal
vein and hepatic artery branches.

302
2. Liver Abscesses
Liver abscess are represented by purulent collections developed inside the liver
parenchyma surrounded by a liver tissue transformed fibrously.
Classification. Liver abscesses can be classified according to four criteria:
According to etiology:
1. Pyogenic abscess (produced by microbial pathogens)
2. Parasitic abscess (amebic abscess)
3. Fungal abscess, most often caused by Candida species, account for less
than 10% of cases.
According to the modality of appearance:
1. Primitive, whose cause is usually unknown
2. Secondary to general, regional or hepatobiliary infection
According to location: in the right lobe, left lobe, deep, superficial, on the hepatic
dome.
According to evolution: acute and chronic abscesses.
Pyogenic Liver Abscess
Etiopathogenesis
It represents approximately 0.10% to 0.27% from the total number of liver
surgeries for various affections. It affects both sexes equally and can occur at any age,
with a maximum around the age of 50.[1,2]
In most cases, the causes of liver abscesses are unknown or obscure, yet among
them are the followings:
Biliary obstruction with cholangitis
Septic metastasis from extrahepatic intra-abdominal processes
(appendicitis, acute enterocolitis, acute pancreatitis)
Liver injury
Supramesocolic surgery
The most incriminated pathogens are: E. coli, Klebsiella pneumoniae,
streptococcus, staphylococcus, and the anaerobic Bacteroides fragilis.[3,4] These germs
can reach into the liver through varied pathways depending on the primary focus: via
the biliary tree in case of cholangitis, via the portal vein commonly in enterocolitis, via
hepatic artery in septicemia, via lymphatic vessels from neighboring septic processes or
directly as in surgery and accidents. Germs can reach into the liver as germs or septic
emboli.
Pathology
Liver abscesses are located more frequently
in the right lobe [2,5] because of the higher blood
flux through the right branch of the portal vein.
(Figure 7) The abscess wall consists of liver tissue
rich in fibrous tissue. The content is pus of various
aspects depending on the causing agent.
Clinical picture
The most frequent symptoms of hepatic
abscess include the followings:
303
Fever, which is the most constant sign associated or not with chills. In many
cases, the first diagnosis is fever of unknown origin.
Anorexia, and malaise
Right upper quadrant pain
Right diaphragmatic irritation may induce reflected pain to the right shoulder
due to the phrenic nerve irritation, cough and hiccoughs
On physical examination, fever and tender hepatomegaly are the most common
signs. Jaundice is present in some cases associated with biliary tract disease, or in case
of multiple liver abscesses.
The topography of the liver abscess influences symptoms, being almost
asymptomatic in deep and posterior location. Pleuropulmonary symptoms may be
present (pleurisy, cough, chest pain) when the abscess in located on the hepatic dome.
The left hepatic lobe abscess can be confused with left subphrenic abscess or perforated
peptic ulcer.
Untreated liver abscess evolve to serious life threatening complications. The most
common are:
Breaking into the peritoneal cavity with peritonitis
Fistulization into the bile ducts with cholangitis
Rupture into the pleural cavity with pleural effusion
Fistulization into the pericardium with pericarditis or cardiac tamponade
Fistulization into a hollow organ (digestive system), when symptoms may
improve
Laboratory tests show an increase of leukocytosis, transaminases, bilirubin, ESR and
alkaline phosphatase. Of high importance in establishing microbiologic diagnosis is the
blood culture drawn during chills.
Imaging studies
Ultrasonography (US) reveals hypoechoic masses with irregular borders and
internal inhomogeneity or cavity debris. Ultrasonography can evaluate the biliary tree
morphology and guide the aspiration of the abscess cavity. The sensitivity of the method
depends on operators experience.
Computed tomography (CT) scan with contrast has a higher sensitivity than US
and also is very helpful in detecting associated intra-abdominal pathology. CT can guide
the percutaneous aspiration and drainage. Liver abscess appears as a relatively well-
demarcated hypodense area (Figure 8) of irregular shape (not as round as a hydatid
cyst). Gas can be seen inside the abscess in 20% of cases.
Chest radiography may reveal basal atelectasis, right hemidiaphragm elevation,
and right pleural effusion when the abscess is
located on the liver dome. (Figure 9)
Positive diagnosis relies on the triad described by
Fontan:
1. Pain
2. Fever
3. Hepatomegaly, plus the rest of the
symptoms and investigations mentioned.
processes associated with fever, chills and sepsis.
Often the diagnosis is not easy, patient being
304
treated with antibiotics without knowing the exact cause.
Searching for the cause of fever, most often a modification
of the liver is found by ultrasonography and than follows
the CT scan.
Even if the abscess is found, it is sometimes difficult
to differentiate it from a tumor with central necrosis or an
infected hepatic cyst. Most time fine needle aspiration
(FNA) guided by ultrasonography is the next step in
evaluation. Hepatocellular carcinoma, biliary disease,
cholecystitis, pleuropulmonary empyema and hydatid cysts
are frequently considered for differential diagnosis.
Treatment
Once the diagnosis has been established, the aim of the treatment is abscess
evacuation and antibiotherapy based on antibiogram.
Abscess evacuation can be achieved by two methods: by percutaneous aspiration
and drainage or by laparotomy or laparoscopy.
The percutaneous procedure is performed under ultrasound or CT guidance. The
needle penetrates the liver into the abscess cavity. The pus is aspirated to be sent to
laboratory for antibiogram. Then a pigtail (or other type) catheter is inserted into the
cavity. (Figure 10) The design of this particular style of catheter (pigtail) includes small
holes that allow the drainage and a coiled
end, which acts to hold the catheter inside
the cavity.
Surgical treatment consists of
laparotomy, identifying the abscess (in deep
location intraoperative ultrasound is very
helpful), opening the abscess cavity,
debridement and pus evacuation, lavage and
drainage.
The overall risk of mortality is up to
25 % higher for multiple abscesses.[1,6,7]
Amebic liver abscess
Etiology
The protozoa Entamoeba histolytica is responsible for the amebic liver abscesses.
It exists in two forms: the cyst form (cyst stage), which is the infective form, and the
trophozoite stage, which causes the invasive disease. Cronically infected people
eliminate cysts through feces and cysts are then transmitted by food and water leading
to contamination of other people. Cysts are resistant to gastric acid, but in the small
intestine, their wall is broken down by trypsin. Once entered into the cecum the
trophozoites penetrate the mucosa layer and then enter the portal circulation traveling to
the liver where they form large abscesses.
Epidemiology
Approximately 40-50 million people are infected annually, worldwide.[8] The
annual incidence is 1.38 per million population in USA.[9] The majority of infections
occur in developing countries: Mexico, India, Central and South America, and tropical
areas of Asia and Africa.[10] Among parasitic causes of death, infection with E.
305
histolytica ranks second worldwide, following malaria. Annually, 40,000-100,000
deaths are caused by infection with E. histolytica.[11,12]
After infection with pathogenic strain, there is an annually 10% risk of
developing symptomatic invasive amebiasis.[13]
The incidence of the amebic liver abscess is 7-12 times higher in males than in
females [9,12,14] and individuals in their third, fourth, and fifth decades, are most often
affected although the disease can occur in any age group.
Morphopathology
In contrast with pyogenic abscesses, the wall of this type of abscess is much
thinner, slightly infiltrated fibrously, with eosinophilic infiltration and the content is
viscous, dark-chocolate brown.
Clinical picture is similar to that of pyogenic abscesses, but in patients history, an
episode of dysentery may be found even with 5-10 months ago. In most cases,
symptoms start at an interval of about 5 months after returning from an endemic
area.[12]
Continuous upper right quadrant pain is the most common element in patients
history. Fever is present in almost all cases. Diarrhea is present in less than one third of
patients and pulmonary symptoms such as cough and chest pain may represent a sign of
secondary pulmonary involvement by abscess rupture in the pleural cavity.
In small and uncomplicated abscesses, there are no physical signs. In large
abscesses, physical examination reveals hepatomegaly and abdominal tenderness. There
may be present signs of complications such as pleural effusions, pericardium effusions,
peritonitis, etc.
Diagnosis is based on history data (traveling in endemic zones, dysentery-like diarrhea),
symptoms (Fontan triad - fever, pain, and hepatomegaly) and investigations.
Abscess is found in most cases by ultrasound investigation and then CT scan
highlights other features. CT scan is very useful in diagnosing complications of the
abscess such as perforation in peritoneal, pleural or pericardial cavity.
The etiology of the abscess is revealed by serologic tests. The EIA (enzyme
immunoassay) test detects antibodies specific for E histolytica in approximately 95% of
patients with extraintestinal amebiasis, in 70% of patients with active intestinal
infection, and in 10% of persons who are asymptomatic cyst passers.[12] The EIA
serology findings revert to negative in 6-12 months following eradication of
infection.[12]
Serum antigen detection, the enzyme-linked immunosorbent assay (ELISA) is
positive in 96% of patients.[15]
The role of microscopic stool examination is limited. Less than 30-40% of
patients with amebic liver abscess have concomitant intestinal amebiasis, and 10% of
the population is infected with the nonpathogenic strain of E. dispar.[12]
Stool antigen detection based on enzyme immunoassay (EIA) is most common
and still quite sensitive. It facilitates early diagnosis before an antibody response occurs
(less than 7 days) and differentiates pathogenic from nonpathogenic Entamoeba
infection. Stool culture for amoeba is sensitive but has limited availability.
Multiplex real-time polymerase chain reaction (PCR) has high sensitivity for
detecting E histolytica and for distinguishing nonpathogenic amoebas.
306
Detection of E. histolytica DNA in saliva and urine could be used as a diagnostic
tool for amebic liver abscess.[16]
Detection of parasite in abscess content is rarely possible. On the other hand, the
percutaneous aspiration of the abscess may be associated with many possible
complications (infection, bleeding, and peritonitis) and therefore this maneuver is
indicated only in certain cases (see bellow).
Differential diagnosis should be made with:
Pyogenic hepatic abscesses
Hydatid cysts
Malaria
Typhoid fever
Cholecystitis
Hemangiomas
Hepatic carcinoma
Hepatic cysts
Hepatocellular adenoma
Peritonitis and abdominal sepsis
Treatment
Medication is quite more important than surgery. The aim of pharmacotherapy is
to eradicate the infection, to reduce morbidity, and to prevent complications. There are
cases of uncomplicated abscesses which can be treated successfully solely with
medication.
Once the diagnosis was established, medication should start immediately with
amebicide drugs (metronidazole, tinidazole, emetine, and chloroquine) which act on the
liver. For eradication of intestinal colonization, the treatment is continued with
antibiotics, which act on the intestinal wall (tetracycline, paromomycin, iodoquinol,
etc). Because in some cases intestinal eradication fails, a follow-up stool examination is
recommended and a second course of a luminal amebicide treatment is required.
Surgical treatment
Percutaneous aspiration and/or drainage of the abscess are indicated in the
following situations: [17,18]
1. Abscess size greater than 5 cm (high risk of abscess rupture)
2. Abscess of the left liver lobe abscess, because it is associated with a
higher mortality and frequency of peritoneal leak or rupture into the
pericardium
3. Failure to observe a clinical medical response to therapy within 5-7 days
4. Cannot differentiate from a pyogenic liver abscess
5. Multiple abscesses
If good results are obtained after medication, surgery should be avoided. Needle
aspiration is preferred instead of drainage due to lower complication rate and shorter
hospitalization.
Prevention
No prophylactic vaccine is available.
Travelers to endemic areas should eat only cooked foods or fruits peeled by
themselves and should avoid drinking local water, including ice cubes frequently used
307
for cocktails. Boiling is the only effective means of eradicating the cysts in water. Pay
attention, many types of bottled water in these countries are not properly disinfected.
To eradicate the cyst forms, vegetables must be cleaned with a strong detergent
soap and soaked in acetic acid or vinegar for
approximately 15 minutes.
Change in sexual practices to avoid fecal-oral
contamination.
Prognosis
In most cases, rapid clinical improvement is
observed in less than one week with antiamebic drug
therapy alone. The average time to radiological resolution
is approximately 12 months, with a range of 3 months to
more than 10 years.[12]
Death occurs in approximately 0.3-15% of persons
having extraintestinal infection, including liver
abscess.[9,12,19] Rupture into the peritoneal cavity and the
pericardium are responsible for most deaths. (Figure 11)
References
1. Branum GD, Tyson GS, Branum MA, Meyers WC. - Hepatic abscess. Changes in etiology, diagnosis, and management.
- Ann. Surg. 1990; 212(6):655-662.
2. Zibari GB, Maguire S, Aultman DF, McMillan RW, McDonald JC. - Pyogenic liver abscess. - Surg. Infec. (Larchmt),
2000; 1(1):15-21.
3. Chen SC, Wu WY, Yeh CH, et al. - Comparison of Escherichia coli and Klebsiella pneumoniae liver abscesses. - Am. J.
Med. Sci. 2007; 334(2):97-105.
4. Cerwenka H. - Pyogenic liver abscess: differences in etiology and treatment in Southeast Asia and Central Europe. -
World J. Gastroenterol. 2010; 16(20):2458-2462.
5. Trcoveanu E, Vlad N, Moldovanu R, Georgescu St, Bradea C, Lupau C, Crumpei F, Vasilescu A, Strat V. - Pyogenic
liver abscesses. - Chirurgia (Bucur). 2008; 103(4):417-427.
6. Alkofer B, Dufay C, Parienti JJ, Lepennec V, Dargere S, Chiche L. - Are Pyogenic Liver Abscesses Still a Surgical
Concern? A Western Experience. - HPB Surg. 2012; 2012:316013. http://www.hindawi.com/journals/hpb/2012/316013/
7. Karatassas A, Williams JA. - Review of pyogenic liver abscess at the Royal Adelaide Hospital 1980-1987. - Aust. N. Z.
J. Surg. 1990; 60(11):893-897.
8. Wuerz T, Kane JB, Boggild AK, Krajden S, Keystone JS, Fuksa M, Kain KC, Warren R, Kempston J, Anderson J. - A
review of amoebic liver abscess for clinicians in a nonendemic setting. - Can. J. Gastroenterol. 2012; 26(10):729-733.
9. Congly SE, Shaheen AA, Meddings L, Kaplan GG, Myers RP. - Amoebic liver abscess in USA: a population-based
study of incidence, temporal trends and mortality. - Liver Int. 2011; 31(8):1191-1198.
10. Salles JM, Moraes LA, Salles MC. - Hepatic amebiasis. - Braz. J. Infect. Dis. 2003; 7(2):96-110.
11. Stanley SL Jr. - Amoebiasis. - Lancet 2003; 361:10251034.
12. Brailita MD. - Amebic Hepatic Abscesses - Medscape reference, http://emedicine.medscape.com/article/183920-
overview
13. Ralston KS, Petri WA Jr. - Tissue destruction and invasion by Entamoeba histolytica. - Trends Parasitol. 2011;
27(6):254-263.
14. Acuna-Soto R, Maguire JH, Wirth DF. - Gender distribution in asymptomatic and invasive amebiasis. - Am. J.
Gastroenterol. 2000; 95(5):1277-1283.
15. Merens A, Rapp C, Fabre R, Cavallo JD. - Utility and limitations of laboratory diagnosis of amebiasis. - Med. Trop.
(Mars). 2005; 65(2):167-75.
16. Haque R, Kabir M, Noor Z, Rahman SM, Mondal D, et al. - Diagnosis of amebic liver abscess and amebic colitis by
detection of Entamoeba histolytica DNA in blood, urine, and saliva by a real-time PCR assay. - J. Clin. Microbiol. 2010
Aug; 48(8):2798-801.
17. Hoenigl M, Valentin T, Seeber K, Salzer HJ, Zollner-Schwetz I, Flick H, Raggam RB, Wagner J, Grisold AJ, Spreizer C,
Krause R. - Amoebic liver abscess in travellers: indication for image-guided puncture? - Wien Klin. Wochenschr. 2012
Nov; 124 Suppl 3:31-4.
18. Choudhuri G, Rangan M. - Amebic infection in humans. - Indian J. Gastroenterol. 2012 Jul; 31(4):153-62.
19. Cosme A, Ojeda E, Zamarreo I, Bujanda L, Garmendia G, Echeverra MJ, Benavente J. - Pyogenic versus amoebic liver
abscesses. A comparative clinical study in a series of 58 patients. - Rev. Esp. Enferm. Dig. 2010 Feb; 102(2):90-9.

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3. Hydatid Cyst of the Liver
Hepatic hydatid cyst is a parasitic disease caused by Echinococcus granulosus.
Hydatid disease involves the liver in approximately 75% of cases, the lung in 15%, and
other anatomic locations in 10%.[1]
History
Liver hydatid disease was described even by Hippocrates (460 BC), but not
knowing the parasitic etiology of the disease.
After more than 2,000 years, Pallas (1781) and Goeze (1782) made the first
scientific descriptions of hydatid disease of the liver, discovering the parasitic origin of
the disease.
Leuckart and Heupner described the great cycle of parasite development and
transmission opportunities of larval form in humans. Alexiinski, in 1897, described the
little cycle of parasite development.
Deve J. provided the most important concepts about liver hydatid cyst in the
beginning of the last century (possibility of secondary echinococcosis, allergy and
anaphylaxis, the parasiticide effect of formalin).
Tomasso Casson, Weinberg and Prvu later, established the intradermoreaction
and complement fixation reaction.
By the late nineteenth century, needle evacuations were performed exclusively.
Only in 1871, Lindemann-Landau and Rivas published the first marsupialization
technique with catastrophic results. Later authors such as Thorton, Ossadas or Llobert -
Aquarius published reduction surgical methods for solving the cyst.
Surgical techniques introduced after the development of asepsis and antisepsis
and modernization of intensive care methods, in the second half of the twentieth
century, had the first encouraging results.
Epidemiology
Echinococcosis is a very rare condition in the continental United States (< 1 case
per 100,000 inhabitants).[2] It is also unusual in northern Europe. The endemic areas are
the Mediterranean countries, the Middle East, the southern part of South America,
Iceland, Australia, New Zealand, and southern parts of Africa. In endemic areas, the
incidence ranges from 1-220 cases per 100,000 inhabitants.[2]
Regarding the profession, the disease is more common in those who work with
animals, animal products and those who have pets, especially dogs.
In Romania it is almost twice more frequent in rural than urban areas.[3] The
yearly average incidence is 3.3 cases/100,000 inhabitants.[4]
Etiopathogenesis
Hepatic hydatid cyst is caused by the development inside the liver of the larval
vesicular form of the hexacant embryo.
Taenia echinococcus granulosus is a tiny tapeworm, less than one cm long, which
anatomically is composed of a scolex or head, a thin and small neck and the strobila.
The scolex has hooks and 4 suction cups (suckers), with which it binds to the gut, and
the strobila, is composed of 3-4 proglottids, the last of which contains 400-800 eggs
(hexacant embryos).
309

Parasitic eggs are released from the last proglottid into the carnivores intestine
(especially canine animals, domestic or wild) which are the definitive host, and via the
feces into the environment. The intermediate host animal is represented usually by a
livestock animal species (e.g. sheep, cow, goat, horse, donkey, pig, and camel) or an
omnivorous or herbivorous wild animal species (e.g. marsupial, wild cattle, wild sheep,
wild goat, deer, moose, and caribou). These animals consume grass infested by parasitic
eggs. The larva hatches out of the egg and migrates through the portal vein into liver
and then to other organs where it will remain as a large larval tapeworm cyst (hydatid
cyst). Carnivores can eat infested organs with cysts and the tapeworm will become
mature producing eggs. This is the life cycle of Echinococcus. (Figure 13)

Humans are "paratenic host- essentially an accidental host - not a normal part
of the parasite's usual life cycle.
Morphopathology
The occurrence of hepatic hydatid cyst is the result of four main processes:
development of cuticle, cyst growth, germination, and
hydatid fluid secretion. The liver hydatid cyst
components are:
1. Cyst wall
2. Content
3. Pericyst
The cyst wall is composed of the outer
membrane, called the cuticle, of yellowish white,
gelatinous aspect, about 1 mm thick and the inner
membrane (germinal lining or membrane - endocyst)
310
(Figure 14) which produces the hydatid fluid, and actively buds off small mini-cysts
inside. These inner-cysts, called "brood capsules" are miniature replicas of the main
hydatid cyst. They may float about freely inside of the main cyst cavity or they may
remain attached to the inner germinal lining. The brood capsules sometimes burst,
releasing the tiny larval tapeworm protoscolices into the main cyst - this sediment out
with gravity, settling into the lower regions of the main cyst as grainy, tapeworm
sediment termed as hydatid sand.
The content of the hydatid cyst is a fluid, which
in the early stages is clear and transparent, with a pH of
7.2 to 7.4, density of 1007 to 1015 and has toxic and
anaphylactic properties. Within the cyst fluid can be
seen the so-called endogenous daughter vesicles,
which are structurally identical to the primary cyst.
(Figure 15) If the budding arise outside the primary
cyst wall, daughter vesicles are called exogenous.
The pericyst belongs to the liver being produced
as an outcome of allergic processes and mechanical compression exerted by the cyst on
the liver tissue. It consists of three layers: external, atelectatic, middle, composed of
connective tissue rich in eosinophils, and internal, fibrohyaline, in contact with the
parasite.
Once in the human liver, cysts grow to one cm during the first 6 months and 23
cm annually thereafter, depending on host tissue resistance.[1]
Daughter cysts develop from daughter vesicles, which contain the protoscolices
and are attached by a pedicle to the germinal layer of the mother cyst. At gross
examination, the vesicles resemble a bunch of grapes.
Pathophysiology
Hydatid disease is not just a local disease of the infested organ but it has a general
character with repercussions on the entire body.
Over two thirds of hydatid cysts are located in liver, because liver is the first
blood filter for the larva absorbed from the digestive tract. The second filter is the lung.
The cyst in the liver destroys the hepatic tissue with compensatory hypertrophy of
the adjacent parenchyma. Cell destruction in this case is secondary to the vascular
thrombosis, and leads ultimately to the phenomena of cellular degeneration and
sclerogenic atrophy, changes that result in two phenomena: the marginalization of the
cyst and liver cirrhosis, at first localized and then generalized.
As the cyst grows it compresses the surrounding anatomical structures (blood
vessels, bile ducts, viscera) leading to local ischemia with the consequence of fistula
formation. The most common the biliary fistulas occur. Bile enters the cyst and also
fluid and daughter vesicles from the cyst can enter into the common bile duct causing
obstructive jaundice, cholangitis and allergic symptoms. Permanent passage through the
papilla major of larvae produces local sclerotic inflammatory phenomena with the result
of sclerotic papillitis with the narrowing of the outlet. Once the biliary fistula is formed,
the bile flows into the cyst, the cyst becomes infected, and the larvae will die. The cyst
may transform into an abscess.
The occurrence of other types of fistula depends on cyst location. The other most
frequent fistula is formed between the cyst and pleural cavity or lung through the
diaphragm (especially on the right side) in posterior-superior location, leading to pleural
311
effusions, pleural hydatidosis and pleuropulmonary fistula (the content of the cyst flows
into the bronchial tree and is eliminated as hydatid vomica).
Other fistula may occur between the cyst and the stomach, colon, kidney,
pericardium, aorta, etc, depending on cyst location. Most of them are severe life
threatening complications.
If the cyst ruptures into the peritoneal cavity, the patient may die suddenly
because of the anaphylactic shock. Other possibilities are: general peritonitis (hydatid
peritonitis) or secondary peritoneal hydatidosis.
During the natural evolution toward healing, dense calcification of all
components of the cyst may occur. Complete calcification implies the death of the
parasite.
Topographic incidence
Topographic incidence is important for the evolution of the cyst, complications,
diagnosis, and surgical tactics.
Median or central liver cysts, corresponding to the segments I, IV, V and VIII
may be superior compressing the cava and hepatic veins or inferior in front or
behind the liver pedicle.
Lateral cysts or lobar cysts, corresponding to segments II, III, VI and VII, may
be marginal or inferior being available for palpation and easier to access
surgically.
Paramedian cysts on the left or right side, increase significantly in volume, and
are difficult to diagnose. Frequently intercept biliary branches being complicated
with biliary fistula and abscess formation.
Cysts on the livers dome may be anterior, of large volume being easily
diagnosed and accessible for palpation, or posterior (segments II, VII and VIII)
being more difficult to diagnose and evolving with compression on diaphragm,
cava and hepatic veins.
Multiple cysts (10-30%) can be localized in one lobe, or anywhere the liver
parenchyma.[5]
Cysts may be unilocular or multilocular.
Symptoms
For a long time cysts are asymptomatic, even in advanced stages, or with minor
nonspecific symptoms (allergic manifestations and transient dyspeptic phenomena),
diagnosis being impossible based on them. In this faze diagnosis is made accidentally
on abdominal ultrasound examination performed for other reasons.
Symptoms are caused by cyst complications, which may include:
Pain, which is continuous, intensified by breath or physical efforts, located in
the upper abdominal quadrants, due to high dimensions of the cyst.
Compression on other organs causes symptoms like intestinal obstruction
when the duodenum or the stomach is involved.
Jaundice is caused by compression on the liver pedicle or by perforation into
the biliary tree with consecutive cholangitis and obstruction with daughter
cysts.
312
Pleuropulmonary manifestations like chest pain, dyspnea, cough,
expectorations, appear when the cyst is located under the diaphragm and
induces pleural effusion or fistula formation into the bronchial tree.
Symptoms caused by perforation into the nearby hollow organs manifested by
vomiting of hydatid membranes (perforation into the stomach or duodenum)
and hydaturia (perforation into the ureter).
Fever, when the cyst is infected transformed into an abscess, or due to
cholangitis.
Signs of cirrhosis
Signs of peritonitis, when the cyst ruptures into the peritoneal cavity.
Sudden death caused by anaphylactic shock (rupture into the peritoneal or
pleural cavity) or massive internal hemorrhage due to perforation of great
vessels (cava, aorta).
Clinical examination
When the cyst is small, or with deep location, or uncomplicated, no signs can be
observed. In voluminous cysts with anterior location, the liver is enlarged
(hepatomegaly on percussion) and the cyst can be palpated.
In the presence of complications, various signs appear depending on complication
(jaundice, pleural effusion, peritonitis, etc).
Diagnosis is based on the triad:
1. History - patients from endemic zones or professions that imply animal or
animal products handling
2. Symptoms and physical signs - generally lack of information
3. Investigations - the most important
Imaging investigations
The most important are the imaging
investigations, which easily detect the cyst(s)
in the liver, and can highlight its features
(location, dimension, form, complications,
etc.).
Abdominal ultrasonography is usually
the first investigation that diagnoses the cyst
(it is the most harmless investigation).
Accuracy depends on investigators
experience. Some features suggest the
echinococcus etiology: the fine debris
(hydatid "sand), septae and daughter cysts, floating germinal membrane (the endocyst),
etc. (Figure 16)
Ultrasound classification of hydatid cysts (WHO): [6]
a. Cystic lesion - there is a simple cyst in the affected organ - not suggestive
for echinococcosis.
b. Active cysts - multiple cysts or septae are present in the parent cyst.
c. Transitional stage - daughter cysts may be present in the parent cyst with
hydatid sand or debris within the cyst.
313
d. The inactive stage - the cyst is echogenic and may be partially or
completely collapsed on itself.
Plain abdominal radiography may reveal
calcifications in the pericyst or total calcified cyst
in 20-30% of cases. [1,7]
CT scan with contrast (Figure 17) offers
the best image about the cyst and many of its
features having a high sensitivity and specificity
for hepatic hydatid disease. When ultrasound
examination encounters difficulties due to
excessive intestinal gas, obesity, abdominal wall
deformities, or previous surgery, CT scan is
indicated. Intravenous administration of contrast
material is generally not necessary unless
complications are suspected, especially infection
and communication with the biliary tree when
detachment of the germinal membrane can be
visualized as linear areas of increased attenuation
within the cyst.[1,8] Calcification of the cyst wall
or internal septae is easily detected on CT images.
MRI offers no real advantage over CT scan.
(Figure 18)
Laboratory
Routine laboratory blood tests results are
not specific for hydatid disease. Many laboratory
tests were tried over the time but most of them had positive or negative false results and
were abandoned (eg. Casonis intradermoreaction and the Weinbrg-Parvu complement-
fixation reaction).
Eosinophilia over 10% is present in only 25%-40% of all cases.[2,9]
Elevated levels of bilirubin or alkaline phosphatase reflect a mechanical
obstruction of the bile ducts by compression or by obstruction caused the daughter cysts
entered into the common bile duct.
Leukocytosis may suggest the infection of the cyst.
The most important tests for screening and follow-up are the indirect
hemagglutination test and the enzyme-linked immunosorbent assay (ELISA) that have a
sensitivity of 80% overall (90% in hepatic echinococcosis, 40% in pulmonary
echinococcosis).[2] Other tests for immunodiagnosis of hydatid disease are the
immunodiffusion and immunoelectrophoresis.
Differential diagnosis includes:
Hepatic cysts
Hepatic carcinoma
Liver abscess
Abdominal abscess
Acute liver failure
Biliary colic and biliary obstruction
Budd-Chiari syndrome
Inferior vena cava thrombosis
314
Intra-abdominal sepsis
Portal hypertension
Tuberculosis
Treatment is multimodal, medical and surgical, depending on stage of evolution and
complications, but surgery is the only hope for complete cure.
The aim of medical treatment is to destroy or inactivate the parasite and of
surgical treatment is to evacuate the cyst, treat the complications and prevent relapses or
secondary infestation.
Medication: two parasiticide benzimidazoles are used, Albendazole and
Mebendazole. The optimal period of treatment with Albendazole ranges from three to
six months. The treatment is started preoperatively and continued postoperatively. Early
pregnancy, bone marrow suppression, and chronic hepatic disease represent
contraindications, and inactive or calcified cysts do not respond to treatment.
Surgical methods can be performed by:
1. Laparotomy - most often used
2. Laparoscopy - in selected cases
3. Punction Aspiration Instilation Reaspiration and Drainage (PAIRD) -
method applied also in selected cases
Laparoscopic approach is performed in rare cases when the cyst is located in a
favorable position that afford a good access (marginal cysts on the anterior edge), of
small dimensions, calcified, inactive and not complicated with fistula. The steps are the
same as in other laparoscopic approach and treatment may be similar to classical
surgical methods. Risks are represented by an impaired access and visibility, intra-
abdominal spread of the infection, bleeding and other possible complications.
PAIRD technique is performed under ultrasound or CT guidance. The patient is
under orotracheal intubation anesthesia. A small skin incision is performed and the
needle (1822 gauge) is guided into the cysts cavity (punction). The content is aspired
and sent to cytological examination, followed by injection of a scolicidal agent (20%
hypertonic saline and 95% ethanol solution approximately equivalent to one third of the
amount aspirated) left in place for at least 15 minutes. Then the content of the cyst is
evacuated by aspiration. This is repeated until the return is clear. The cyst is then filled
with isotonic sodium chloride solution or drained with a pigtail catheter. Perioperative
treatment with Albendazole is mandatory (4 days prior to the procedure and 3-6 month
after).
Indications for PAIRD: [10]
Patients with:
Non-echoic lesion 5 cm in diameter
Cysts with daughter cysts, and/or with detachment of membranes
Multiple cysts if accessible to puncture
Infected cysts
Also
Pregnant women
Children >3 years old
Patients who fail to respond to chemotherapy alone
Patients in whom surgery is contraindicated
Patient who refuse surgery
315
Patients who relapse after surgery
Contraindications for PAIRD: [10]
Non-cooperative patients and inaccessible or risky location of the
cyst in the liver
Cyst in spine, brain and/or heart
Inactive or calcified lesion
Cysts communicating with the biliary tree
Cysts open into the abdominal cavity, bronchi and urinary tract
Benefits of PAIRD: [10]
Minimal invasiveness
Reduced risk compared with surgery
Confirmation of diagnosis
Removal of large numbers of protoscolices with the aspirated cyst
fluid
Improved efficacy of chemotherapy given before and after puncture
(probably because of an increased penetration of antihelminthic
drugs into cysts re-filling with hydatid fluid )
Reduced hospitalization time
Cost of the puncture and chemotherapy usually less than that of
surgery or chemotherapy alone
Risks of PAIRD procedure: [10]
Same risks as any puncture (hemorrhage, mechanical lesions of
other tissues, infections )
Anaphylactic shock or other allergic reactions
Secondary echinococcosis caused by spillage
Chemical (sclerosing) cholangitis if cysts communicate with the
biliary tree
Sudden intracystic decompression, thus leading to biliary fistulas
Persistence of satellite daughter cysts
Systemic toxicity of alcohol or hypertonic saline in case of large
cysts (total volume injected must be carefully calculated)
In open surgery the most frequent approach used is a subcostal or median
laparotomy (a good access to the cyst and liver pedicle is mandatory).
Over the time, many surgical techniques have been developed and surgeons have
to choose which the best for the specific case is.
Principles of the surgical therapy are:
1. Cyst emptying
2. Cyst inactivation
3. Treating the residual cavity
Some precautions must be taken during operation such as a good isolation of the
peritoneal cavity around the cyst, with gauzes impregnated in alcohol, to prevent the
spread of cyst content and avoiding electrocautery in cases when alcohol is used for
inactivation.
Cyst inactivation and emptying. Finding the cyst is generally easy. Difficulties
may be encountered in small cyst and deep location. Intraoperative ultrasound is very
helpful in these cases.
316
After peritoneal cavity isolation, the cyst is punctured and a quantity of fluid is
extracted and examined. If the liquid is transparent, clear, probably the cyst is active. If
the liquid is yellowish, probably there is a biliary fistula and the cyst may be inactive. If
the liquid is transformed in pus, that means the infection of the cyst, and there is a high
chance that the cyst is being inactive.
On the same needle, alcohol is introduced into the cyst cavity in order to
inactivate the parasite. Other scolicidal agents include formalin, hydrogen peroxide,
hypertonic saline, chlorhexidine, and cetrimide.
The cyst is then opened and the content is aspirated. All daughter cysts are
removed and also the germinal membrane. The cavity is copiously washed with alcohol
or hydrogen peroxide and thoroughly inspected for remnant cysts and biliary fistulas.
Treating the residual cavity. This step is a challenge for the surgeon, depending
on where the cyst is located. There have been imagined many techniques in order to
avoid collection into the residual cavity and abscess formation. Types of procedures can
be classified as:[11]
1) Conservative procedures that do not resect the pericyst
a) External drainage (Figure 19)
i) Wide marsupialization - Lindemann Landau (1871)
ii) Narrow marsupialization - Rivas
b) Internal drainage (bypass between the cyst and the stomach, duodenum or
jejunum) - Goinard, Pelissier (1959) (Figure 20)
c) Filling and sealing the cavity with epiploon - Mauclaire (1900) (Figure 21)
2) Procedures that remove totally or partially the pericyst
a) Partial pericystectomy - Mabitt Lagrot (1896) (Figure 22)
b) Total pericystectomy with opened cyst - Constantini
c) Total pericystectomy with closed cyst - Pozzi (1887), Napalkoff (1904)
(Figure 23)
3) Radical procedures that remove the whole cyst and a segment of the liver
a) Atypical liver resection - Imperatti (Figure 24)
b) Major anatomical liver resection

317

If biliary fistula is observed in residual
cavity, it should be closed by sutures. CBD should
be inspected. Enlarged CBD and jaundice in
history could represent the presence of daughter
cysts in CBD, in which case the CBD should be opened, explored and cysts removed. A
T-tube drainage (Kehr) is placed into the CBD. It will also help to find the biliary
fistulas in the residual cavity and by decreasing the pressure in the CBD will prevent
residual chronic biliary fistula formation. (Figure 25)
Intraoperative complications
Difficulties in accessing the cyst located on the posterior surface of the right
lobe, or when the cyst is small and deep located
Anaphylactic shock during puncture of the cyst
Rupture of the cyst during manipulation and spread of the content into the
peritoneal cavity - risk of secondary peritoneal hydatidosis
Diaphragmatic rupture and opening the pleural cavity when cysts are located on
the posterior-superior surface
Bleeding from liver parenchyma
Lesions of surrounding organs (gallbladder, duodenum, colon, stomach, etc)
Other incidents such as intra-abdominal blast or fire when electrocautery is used
in the presence of alcohol used for cyst inactivation
Postoperative care and complications
Usually the postoperative evolution is long, with possible complications
depending on many factors such as: cyst location, dimensions, number, presence of
biliary fistula, etc. Drainage tubes are removed depending on quantity and quality of the
drained fluid. The patient is advised to take parasiticide drugs (Albendazole).
The most frequent postoperative complications are:
Wound infection
Bleeding from residual cavity
Relapse of the hydatid cyst
External biliary fistula
Residual cavity abscess
318
References
1. Pedrosa I, Saz A, Arrazola J, Ferreirs J, Pedrosa CS. - Hydatid Disease: Radiologic and Pathologic Features and
Complications. - RadioGraphics 2000; 20:795-817.
2. Dandan IS. - Hydatid Cysts. - Emedicine, MedScape Reference, http://emedicine.medscape.com/article/178648-
overview#a0199
3. Vlad DC, Neghina AM, Dumitrascu V, Marincu I, Neghina R, Calma CL. - Cystic Echinococcosis in Children and
Adults: A Seven-Year Comparative Study in Western Romania. - Foodborne Pathog. Dis. 2013 Jan 21.
4. Moldovan R, Neghina AM, Calma CL, Marincu I, Neghina R. - Human cystic echinococcosis in two south-western and
central-western Romanian counties: a 7-year epidemiological and clinical overview. - Acta Trop. 2012; 121(1):26-29.
5. Megherbi MT, Oulmane D, Hireche L, Abid L, Benabadji R. - Multiple hydatid cyst of the liver: indication for a
conservative treatment in uncomplicated univesicular localizations. Apropos of 19 cases. - J. Chir. (Paris) 1988;
125(5):358-363.
6. WHO Informal Working Group. - International classification of ultrasound images in cystic echinococcosis for
application in clinical and field epidemiological settings. - Acta Trop. 2003; 85(2):253-261.
7. Beggs I. - The radiology of hydatid disease. - Am. J. Roentgenol. 1985; 145:639-648.
8. de Diego J, Lecumberri FJ, Franquet T, Ostiz S. - Computed tomography in hepatic echinococcosis. - Am. J. Roentgenol.
1982; 139:699-702.
9. Holman Jackson HH. - Hepatic Cysts Workup. - Medscape Reference, Emedicine
http://emedicine.medscape.com/article/190818-workup
10. PAIR: Puncture, Aspiration, Injection, Re-Aspiration An option for the treatment of Cystic Echinococcosis -
WHO/CDS/CSR/APH/2001.6 http://whqlibdoc.who.int/hq/2001/WHO_CDS_CSR_APH_2001.6.pdf
11. Burlui D, Roca Monica - Chirurgia chistului hidatic hepatic - Editura Medical Bucureti, 1977.

319
4. Liver Tumors
A. BENIGN TUMORS
Benign tumors can develop from liver parenchyma cells (adenoma, cholangioma)
or from the mesenchymal tissue (hemangioma, lymphangioama, fibroma).
The most common type of benign liver tumor is mesenchymal hamartoma.
Tumors may be unique or multiple, in one or both lobes, cystic or solid. Some of
them can turn malignant. They do not have specific symptoms. Clinical picture is poor
represented by mild pain in the upper right abdominal quadrant especially when tumors
are large.
Surgical treatment is not needed in most cases. Indications for surgical removal of
benign tumors are:
High volume tumors with persistent symptoms
If the tumor compresses surrounding anatomical structures (vessels, bile
ducts, etc)
If there is no possibility to differentiate from a malignant tumor
Based on histological criteria, the classification of benign liver tumors is:
Epithelial tumors: adenoma, cystadenoma, papilloma
Mesenchymal tumors: cavernous hemangioma, capillary angioma
Mixed tumors: teratoama
Tumor-like lesions: focal nodular hyperplasia, anoxic necrosis,
hamartoma
Glissonian tumors and of the liver ligaments
Liver adenoma
It occurs more frequently in women and appears to have hormonal causes
(frequently found in women who consumed oral contraceptives) but may occur in men
who are treated with anabolic steroids.[1-5]
Tumors are well defined, with or without a capsule. They are composed of
hepatocytes arranged in cords, without biliary structures and normal liver tissue.
Adenomas blood supply is exclusively arterial. Rarely turn malignant.
There are several types of adenomas:
Solitary adenoma is the most common. It may be of variable size and has a
tendency to marginalization. The tumor adheres or not to parenchyma according to the
presence or absence of the fibrous capsule. Only tumors over 10 cm in diameter are of
therapeutic interest due to possible compression on liver pedicle.
Lecene solitary adenoma (liver simple dysembryonic tumor). The tumoral
epithelial cells are similar to hepatocytes and grouped in the form of lobules. They are
well encapsulated that allows an easy surgical removal.
Trabecular adenoma. Tumoral cells are arranged in trabeculae, without any
lobular organization and without any capsule; out of all adenomas they have the greatest
tendency to turn malignant.
Forms less frequent are cholangiomas - benign tumors, usually of 1-2 cm
diameter, multiple, grouped or disseminated in one or both lobes, discovered
incidentally, difficult to differentiate from secondary liver metastases.
320
Clinical picture is poor. The diagnostic is usually based on investigations such as
ultrasound, CT, MRI. Surgical excision is indicated in large or small and spread tumors
(liver adenomatosis) with clinical symptoms, and when there is no certainty of
benignity.
Hemangioma
It is the most common liver tumor after liver metastases. It can be seen in both
adults and children, which is of particular importance due to arteriovenous shunts that
may develop. There are two forms:
1. Diffuse form, mostly involving the whole liver, looking like disseminated
teleangiectasia.
2. Solitary form may be present in several pathological types:
cavernous hemangioma with large vascular spaces
capillary hemangioma, with more abundant stroma
schirous hemangioma, vascular spaces collapsed and abundant
stroma,
hemangioendothelioma
Symptoms are very poor. However, the following signs and symptoms may be
attributed to hepatic hemangioma: right upper quadrant pain with palpable tumor, fever,
anemia and biological inflammatory signs (leukocytosis, elevated ESR, etc.).
Diagnosis of hemangiomas is most often incidental at ultrasound examination or
CT scan where hypodense areas filling from the periphery to the center after the
administrations of contrast agent may be observed.
The small hemangiomas or located near the bilio-vascular tree may be diagnosed
by scintigraphy with marked red blood cells and nuclear magnetic resonance.
The indication of surgical treatment is still under debate. Widely accepted
indications are: [6-8]
intense symptoms,
changes in shape and volume of the tumor,
repeated intratumoral hemorrhage or subcapsular rupture,
central necrosis,
benign-malignant uncertainty
Focal nodular hyperplasia (FNH)
Also known as solitary hyperplastic nodule, focal cirrhosis, pseudocirrhosis, or
mixed adenoma, FNH occurs in two forms: solid form as a stellar scar consisting of
convergence liver nodules and the teleangiectatic form with dilated spaces filled with
blood between nodules.
It is usually asymptomatic. In rare cases, growing in volume, rupture or cirrhosis
with portal hypertension can be found. Malignant transformation was not recorded.
As in most liver tumors, diagnosis is made by means of imaging: ultrasound, CT,
MRI.
Diagnostic uncertainty dictates surgery and the most commonly performed
surgery is limited to resection, with good results.
Hepatic cystadenoma (liver cysts)
The tumor has two morphological aspects: solitary or multiple. The right lobe is
the most frequent location. Tumor size is variable containing liquid of different aspects:
321
serous or serohematic. Cystadenomas wall is thin, transparent, consisting of two layers:
epithelial, the inner, and the outer formed by fibrous connective tissue, always with a
cleavage space between the cyst and the liver parenchyma.
Symptoms are poor but some may be associated with digestive disorders: right
upper quadrant pain, nausea, vomiting, and flatulence.
Cysts can become complicated by rupture and hemorrhage or superinfection.
Surgery is required in cases with obvious symptoms and consists of enucleation,
liver resection and rarely marsupialization.
B. MALIGNANT TUMORS
Like benign tumors, malignant tumors may have:
Mesenchymal origin (sarcomas), or
Parenchymal origin (the most frequent) represented by hepatocarcinoma
and cholangiocarcinoma.
Liver malignant tumors may be primary or secondary (metastases), particularly
from gastrointestinal carcinomas.
Hepatocellular carcinoma (HCC)
HCC is the most frequent primary liver cancer. It can develop on a normal liver
but in most cases on a liver affected by cirrhosis caused to hepatitis B or C or
alcoholism.
Incidence
It is estimated that 28,720 men and women (21,370 men and 7,350 women) will
be diagnosed with, and 20,550 men and women will die of cancer of the liver and
intrahepatic bile duct in 2012.[9]
The tumor occurs with predilection in males in a ratio of 5:1, with a maximum
incidence and endemic in Asia, Africa, and South America, and with low incidence in
North America and Europe.[10]
Risk factors are represented mainly by:
Alcoholism
Hepatitis B and C
Aflatoxin
Cirrhosis of the liver
Hemochromatosis
Wilson's disease
Type 2 Diabetes (probably aided by obesity)
Toxic substances (vinyl chloride)
About 60%-90% of patients with liver cancer are positive for antigen HBs and
HBc and patients with hepatitis C with virus antibodies antiHBc positive have a risk of
cancer 4 times higher. Hepatitis B virus is incriminated in the etiology of HCC by itself,
not by induced cirrhosis. The proof is the presence of HB antigen in individuals with
HCC who did not develop cirrhosis. The risk of patients infected with hepatitis B and C
viruses to develop HCC is 7 times higher.[11-13]
A particular pathogenetic importance is given to aflatoxin B1 produced by
Aspergillus flavus, whose presence may cause an eight times higher incidence of the
HCC.
322
Signs and symptoms
HCC may manifest with jaundice, loss of appetite, emesis, nausea and
unintentional weight loss, abdominal pain especially in the upper-right part, bloating
from ascites, fatigue and easy bruising from blood clotting abnormalities.
Morphopathology
HCC is a tumor of soft consistency of gray-brown aspect that distinguishes it
from cholangiocarcinoma and liver metastases. It appears either as a single massive
tumor or as disseminated tumor.
Microscopically tumoral cells are like hepatocytes but multinucleated and giant.
Overall tumor is well vascularized mainly arterially.
Tumoral extension uses the following routes:
Nearby extension
Through sinusoidal spaces
Anterograde extension through hepatic veins and retrograde through
portal veins
Lymphatic extension
Transdiaphragmatic
Staging
Staging is important for defining prognosis and optimal therapeutic methods
applied according to the prognosis. A variety of staging systems have been used for
liver cancer. The American Joint Committee on Cancer (AJCC) TNM system is based
on the results of the physical exam, imaging and other tests:[14,15]
T groups
TX: Primary tumor cannot be assessed
T1: A single tumor (any size) that hasn't grown into blood vessels
T2: Either a single tumor (any size) that has grown into blood vessels, OR more than one
tumor where no tumor is larger than 5 cm (about 2 inches) across
T3a: More than one tumor, with at least one tumor larger than 5 cm across
T3b: At least one tumor (any size) that has grown into a major branch of a large vein of the
liver (the portal or hepatic vein)
T4: The tumor (any size) has grown into a nearby organ (other than the gallbladder), OR the
tumor is growing into the thin layer of tissue covering the liver (called the visceral
peritoneum)
N groups
NX: Regional (nearby) lymph nodes cannot be assessed.
N0: The cancer has not spread to the regional lymph nodes.
N1: The cancer has spread to the regional lymph nodes.
M groups
M0: The cancer has not spread to distant lymph nodes or other organs.
M1: The cancer has spread to distant lymph nodes or other organs.
Stage grouping
Stage I: T1, N0, M0: There is a single tumor (any size) that has not grown into any blood
vessels. The cancer has not spread to nearby lymph nodes or distant sites.
Stage II: T2, N0, M0: Either there is a single tumor (any size) that has grown into blood
vessels, OR there are several tumors, and all are 5 cm (2 inches) or less across. The cancer
has not spread to nearby lymph nodes or distant sites.
Stage IIIA: T3a, N0, M0: There is more than one tumor, and at least one is larger than 5 cm
(2 inches) across. The cancer has not spread to nearby lymph nodes or distant sites.
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Stage IIIB: T3b, N0, M0: At least one tumor is growing into a branch of a major vein of the
liver (portal vein or hepatic vein). The cancer has not spread to nearby lymph nodes or
distant sites.
Stage IIIC: T4, N0, M0: A tumor is growing into a nearby organ (other than the
gallbladder), OR a tumor has grown into the outer covering of the liver. The cancer has not
spread to nearby lymph nodes or distant sites.
Stage IVA: Any T, N1, M0: Tumors in the liver can be any size or number and they may
have grown into blood vessels or nearby organs. The cancer has spread to nearby lymph
nodes. The cancer has not spread to distant sites.
Stage IVB: Any T, Any N, M1: The cancer has spread to other parts of the body. (Tumors
can be any size or number, and nearby lymph nodes may or may not be involved.)
Although the TNM system defines the extent of liver cancer in some detail, it
does not consider the liver function. Several other staging systems have been developed
that include both of these factors and others - the Okuda classification, French
classification, the Cancer of the Liver Italian Program (CLIP) score, The Chinese
University Prognostic Index (CUPI), The Japan Integrated Staging (JIS).[16] The
Barcelona Clinic Liver Cancer staging system (BCLC) is the most common used and it
includes four stages:[17,18]
A. Includes patients with asymptomatic early tumors
B. Patients with asymptomatic multinodular HCC
C. Patients with symptomatic tumors and/or invasive tumor pattern
D. End stage disease
Patients at stage A are candidates for radical therapies (resection, liver
transplantation or percutaneous treatments). Those at stage B with intermediate HCC
may benefit from chemoembolization. Patients at stage C with advanced HCC may
receive new agents in the setting of randomized controlled trials, and patients at stage D,
with end-stage, disease will receive only symptomatic treatment.[16,19]
Diagnosis
The main biological data on a possible HCC are: accelerated ESR, increased
serum bilirubin, increased LDH, decreased albumin (announce a poor prognosis). In
addition, alpha-fetoprotein and GTP may be increased. The screening for HCC includes
the alpha-fetoprotein level and ultrasonography.[20]
Imaging studies are of particular importance and are represented by ultrasound
CT, MRI, scintigraphy (with technetium, colloidal gold or Gallium for differential
diagnosis from liver metastases) and selective angiography. The sensitivity of
ultrasound for HCC detection is low since small nodules can be missed in a cirrhotic
liver.[20]
On CT imaging, the HCC has three distinct patterns of growth: (Figure 26)
1. A single large tumor
2. Multiple tumors
3. Poorly defined tumor
with an infiltrative
growth pattern
The key characteristic on CT is
the hypervascularity in the arterial
phase scans, with an early arterial
uptake followed by washout in the
portovenous or late, equilibrium phase.
324
Other features are a pseudocapsule and a mosaic pattern. Both, intratumoral
calcifications and fat may be present. The use of tissue-specific contrast agents,
including superparamagnetic iron oxides (iron oxide nano-particles) and hepatobiliary
contrast agents, improves lesion detection.[20,21]
HCC diagnosis is no longer based on biopsy if certain imaging criteria are met,
but biopsy is the single method of certain histological diagnosis. According to the
European Association for the Study of the Liver (EASL) and the American Association
for the Study of Liver Diseases (AASLD) [5] a nodule larger than 2 cm that displays a
typical vascular pattern on contrast-enhanced CT or contrast-enhanced MRI can be
considered HCC without biopsy. For nodules measuring between 1 and 2 cm, the
diagnosis of HCC without biopsy requires a confirmation of the typical vascular pattern
on both imaging modalities. In comparison with EASL and AASLD criteria, the
consensus statement from the Asian Oncology Summit from 2009 [6] recommends that
for any nodule, regardless of size, the characteristic features on contrast-enhanced CT or
contrast-enhanced MRI will suffice for a diagnosis of HCC, and obviates the need for
biopsy.[20]
Pre-operative staging in surgical candidates should include CT of thorax,
abdomen and pelvis and bone scintigraphy.[22]
Cholangiocarcinoma (CCA)
The tumor originates from the epithelium of intrahepatic bile ducts. In terms of
etiology, in addition to the factors referred to CHC, sclerogenic cholangitis lesions have
an important role.
In terms of morphological characteristics, cholangiocarcinoma usually appears as
a single large tumor with relatively uniform in structure of yellow-gray color, and cells
are small, with voluminous nucleus with secretion of mucus.
Due to the above characters, cholangiocarcinoma is sometimes difficult to
distinguish from HCC, this being based on immunohistochemical profile, by
highlighting specific epithelial membrane antigen, cytokeratin and impregnation of
biliary canaliculi with polyclonal antibody for carcinoembryonic antigen. Frequently
used tumor markers are the Carbohydrate antigen 19-9 (CA 19-9), Carcinogenic
Embryonic antigen (CEA), and Cancer Antigen 125, which in combination have shown
sufficient sensitivity and specificity to detect and monitor CCA.[23]
The differential diagnosis with liver metastases from gastrointestinal carcinomas
is sometimes impossible, even after immunohistochemistry tests and laparotomy; in this
case, the differential diagnosis relies on exclusion of primary tumors.
Symptoms have nothing particularly being almost the same as in CHC,
hepatomegaly and jaundice being frequently met.
Liver sarcomas
The main types of liver sarcomas are angiosarcoma, mesenchymoma,
hemangioendothelioma. These tumors are very rare, with non-specific symptoms and
have a very rapid evolution with unfavorable prognosis. Surgical treatment is indicated
only when these tumors can be found in resectable stages.

325
Liver metastases
By far the most common malignant liver tumors are metastases. The liver is an
important blood filter especially for gastrointestinal tract. Three major types of liver
metastases are more often met:
colorectal cancer metastases
liver metastases from endocrine cancers, and
metastases from other cancers
Liver metastases of colorectal cancers occur via the portal system. Usually the
evolution of these metastases is slow depending on primary tumor characteristics, and
they can be treated. Colorectal cancer metastases are of two kinds:
synchronous, when they occur
simultaneously with the primary tumor
or shortly after primary tumor removal,
and
metachronous, they occur at a distance
greater than five years after primary
tumor removal
In synchronous metastases, symptoms overlap
the primary tumor and in the metachronous,
symptoms are similar to any other forms of malignant
liver tumors.
Diagnosis relies on history, imaging
(ultrasound, CT) (Figure 27), biopsy and elevated levels of CEA (carcinoembryonic
antigen).
Principles of treatment of hepatic malignancies
Important tumoral features that guide treatment include:
size
spread
involvement of liver vessels
presence of a tumoral capsule
presence of extrahepatic metastases
presence of daughter nodules
vascularity of the tumor
When liver cancer is diagnosed in early stages, the liver transplantation is the first
line of treatment.
The Milan criteria (based on Mazzaferro et al. studies in 1996) state that a patient
is selected for transplantation when:[24]
1. Only one lesion smaller than 5 cm in present
2. Up to 3 lesions smaller than 3 cm
3. No extrahepatic manifestations
4. No vascular invasion
Because the Milan criteria were considered too restrictive and acceptable
outcomes can still be achieved using more liberal tumor criteria [25-27], researchers at
the University of California at San Francisco (UCSF) proposed the following criteria for
tumor size:

326
1. Single lesion 6.5 cm
2. Multiple lesions 3 cm
3. Largest tumor diameter if multiple 4.5 cm
4. Total tumor diameter if multiple 8 cm
The second line of treatment is the surgical removal (resection) of the affected
part of the liver. However, in advanced cases, there may not be enough normal liver
tissue left to maintain liver function after a resection. The decision and the extent of
surgical resection are based upon the patient's condition, extent of the disease, and liver
function. Most patients with cirrhosis have abnormal liver function and are not
candidates for resection. Tumor size is not relevant to resectability; patients who have
circumscribed single tumors are potentially resectable regardless of tumor size.[28]
In CCA, surgery offers the most beneficial curative option and outcome,
emphasizing the importance of resectability as a major prognostic factor. Chemotherapy
is rarely useful.[29] In advanced cases only, locoregional therapy is an interesting
therapeutic strategy.[30]
In case of metastases from colorectal cancer, only 1025% of patients are
candidates for surgery.[31,32] The oncosurgical modalities that are available include
liver resection following portal vein ligation/embolization, "two-stage" liver resection,
one-stage ultrasonically guided liver resection, hepatectomy following conversion
chemotherapy, and liver resection combined with thermal ablation.[33]
The hepatic functional reserve should be sufficient to allow adequate
postoperative liver function. If remnant liver parenchyma is healthy, up to 6 of the 8
anatomical segments (75% of the volume of the liver) can be resected without inducing
postoperative liver failure. Such major resections cannot be performed safely if remnant
liver parenchyma is abnormal.[31] Therefore, before resection for HCC, the non-tumoral
portion of the liver should be biopsied to determine whether there is associated
cirrhosis.
When a portion of a normal liver is removed, the remaining liver can regenerate
to the original size within 6-8 weeks. A cirrhotic liver, however, cannot grow back. The
liver failure can occur if the remaining portion of the liver is inadequate (for example,
because of associated cirrhosis) to provide the necessary support for life. About 3.6-
5.6% of patients are expected to die shortly after surgery, usually as a result of liver
failure and sepsis. [34,35]
Liver resection may be performed laparoscopically (for small superficial tumors)
or using open surgical methods.
Liver resections can be divided into two groups: [32]
1. anatomical resections are those procedures that remove one or more liver
segments described by Couinad.
2. atypical or wedge resections
are non-anatomical, removal
of a portion of liver
parenchyma surrounding the
hepatic lesion without
respecting the liver
segmentation and without
following any of the
anatomical planes. (Figure 28)

327
Depending on the moment of the ligation of the afferent liver pedicle, Bismuth
classifies lever resections in:[32,36]
1. Controlled - when the ligation of pedicle precedes the liver resection, and
the resection follows the line of demarcation of the ischemic territory.
2. Uncontrolled - when the operation starts with liver resection, the bilio-
vascular structures being resected and ligated along the resection plane.
Depending on how much liver tissue is removed, resections can be major or
minor. Resections removing at least two continuous segments are defined as major
hepatic resections.[37] Other authors consider that removing at least four segments
represents a major hepatectomy.[38]
Four types of major liver resections are commonly performed [31]: (Figure 29)
1. left lateral lobectomy (segments II, III)
2. right hepatectomy (segments V, VI, VII, VIII)
3. left hepatectomy (segments II, III, IV) and
4. extended right hepatectomy also called right lobectomy (segments IV, V,
VI, VII, VIII).
Other types of anatomical resections that can be performed are:
1. extended left hepatectomy (left trisegmentectomy)
2. extending a left hepatectomy to segments V and VIII,
3. central hepatectomy (segments IV, V, VIII)
4. bi-segmentectomies (VVI, VIIVIII, VIVII

Postoperative possible complications
Although important advances in liver surgery reduced blood loss and infections,
this type of surgery remains burdened by a high rate of complications, especially in
patients with liver cirrhosis (morbidity 1040% and mortality of 020%) depending on
the extent of liver resection, status of liver disease, and experience of the center.[39-41]
Local complications
Perihepatic fluid collection or abscesses
Bile leak
Liver failure
Bleedings
328
Wound infection
Intra-abdominal sepsis
General complications
Pleural effusion
Pneumonia
Deep vein thrombosis/pulmonary embolism
Cardiac failure, myocardial infarction
Prognosis
Liver resection of colorectal metastases is associated with 3 and 5-year survival
rates close to 40% and 30%, respectively. After resection, recurrences are observed in
two-thirds of the patients.[31,42]
Postoperative survival rates in HCC are in the range of 80-92% at 1 year, 61-86%
at 3 years, and 41-74% at 5 years. During the first 2 years after resection, the
predominant issue is the appearance of intrahepatic metastases from the resected
primary site.[43,44]
In cholangiocarcinoma, unfortunately, curative resection is possible in only about
30% of patients due to locally advanced disease, distant metastases or co-morbidities in
elderly patients. Even after resection, the recurrence rate is approximately 60%,
resulting in a low 5-year overall survival.[30]
When transplant or resection is not possible, chemoembolization and radio-
frequency ablation are the treatments of choice. These therapies can be applied alone or
in association.
Chemoembolization is based on the rich arterial supply of tumors. It combats
cancer in two ways. First, it gives a high dose of chemotherapy directly to the tumor
(embolizing agent is mixed with anticancer drugs such as doxorubicin, mitomycin or
cisplatin). Second, it cuts off the tumors blood supply, a process known as
embolization. The physician (interventional radiologist) inserts a catheter into femoral
artery and using X- ray imaging, guides the catheter into the hepatic artery, which
supplies the tumor with blood. (Figure 30) Because these agents are applied only at the
tumor site, and because non-cancerous liver tissue
does not rely on the hepatic artery as its main
source of oxygenated blood, healthy tissue
remains unaffected by this treatment. Transarterial
chemoembolization is the treatment of choice
when the tumor is greater than 4 cm in diameter,
or when there are multiple lesions within the
liver.[45] Side effects are represented by fever,
abdominal pain and elevated transaminases.
Patients should be carefully chosen as the
procedure may produce liver failure and death in
those with cirrhosis. Chemoembolization may
also be used to shrink liver tumors while the
patient awaits a donor organ.
An alternative to embolization is intraoperative ligation of the hepatic artery or its
branches.
Many local ablative therapies have been developed for unresectable liver cancer
including percutaneous ethanol injection (PEI), percutaneous acetic acid injection,
329
radiofrequency ablation (RFA), cryoablation, microwave ablation, laser-induced
thermotherapy, and high-intensity focused ultrasound.[33]
Radio-frequency ablation (RFA) is a minimally invasive treatment for focal
cancers. During the procedure, the tumor is destroyed with localized heat from electrical
energy. A special needle is inserted into the liver tumor through the skin (or directly in
open surgery) by ultrasound guidance and at its tip intense heat is generated. Because
the heat is generated within the tumor, surrounding healthy tissue is mostly spared. This
procedure can be performed under local anesthesia but is usually performed under
general anesthesia. Patients generally stay in the hospital overnight, but many go home
the same day. Radiofrequency ablation can be performed either during open surgery,
laparoscopy, or percutaneously. The technique has outcomes similar to those of surgical
resection for appropriately selected tumors.
Radiofrequency ablation can be used alone or in conjunction with liver resection.
Sometimes when patients have multiple tumors, some of them may be surgically
removed while the remaining disease is treated with radiofrequency.
Percutaneous ethanol injection (PEI) is very effective in destroying small HCC
tumors. Performed under percutaneous ultrasound guidance, a needle is placed into the
tumor and absolute alcohol is injected. For HCC tumors that are 2 cm in diameter, PEI
seems to have efficacy similar to that of RFA. Percutaneous ethanol injection and
radiofrequency ablation reportedly yield survival rates similar to resection.[46,47] The
therapeutic effect of RFA on small HCC is better than that of PEI. Small HCC is the
optimal indication of RFA. For recurrent HCC (diameter > 3 cm), the combined
treatment of RFA and PEI should be used.[48]
Systemic chemotherapy is based on tumor response to chemotherapy.
Unfortunately, it does not exceed an average of 20% of cases. Survival is short and is
measured in months. For monochemotherapy are used the followings: adriamycin, 5
fluorouracil, nitomicina C. Polichemotherapy does not provide additional benefits.
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Surgical Pathology of the Extrahepatic Biliary Tree
SURGICAL PATHOLOGY OF THE EXTRAHEPATIC
BILIARY TREE

1. Surgical anatomy
2. Pathology of the gallbladder - lithiasis
3. Common bile duct lithiasis
4. Non tumoral stenosis of the biliary ducts
5. Tumors of the biliary ducts

1. Surgical Anatomy
Bile ducts represent a system of ducts that drain the bile from the liver secreting
cells toward the duodenum. Biliary tree is divided into two regions: (Figure 1)
1. Intrahepatic bile ducts - inside the liver, and
2. Extrahepatic bile ducts - outside the liver
Extrahepatic bile ducts are formed by:
Right and left hepatic ducts
Common hepatic duct - ductus hepaticus
Gallbladder
Cystic duct
Common bile duct or choledochus (CBD)
which in the duodenal wall joins the main
pancreatic duct of Wirsung forming thus the
ampulla of Vater, which opens into the
duodenum through the papilla major.
The anatomically normal length of the CBD
is 10-12 cm and the thickness of 6 mm.
The common hepatic duct has posterior
relations with the portal vein and the right branch
of the hepatic artery. Hepatic artery is located on
the left side of the duct. (Figure 2)
There are many anatomical variations of the
liver pedicle. These variations refer especially to
the hepatic artery but also to the biliary tree.
Ignoring these variations can be very dangerous,
sometimes life threatening for the patient.
The common bile duct has four portions:
(Figure 3)
1. Supraduodenal portion which, is
bordered rear by the portal vein and on
the left side by the proper hepatic
artery.

332
2. Retroduodenal portion, which has
relations in front with the duodenum. It
is crossed by the gastroduodenal
artery. Posterior is bordered by the
portal vein and on the left side by the
proper hepatic artery.
3. Retropancreatic portion has in front
the pancreas and posterior the vena
cava and the right renal vein.
4. Intraparietal (transduodenal) portion
crosses the duodenal wall thickness
together with the pancreatic duct
forming the Vaters ampulla.
The gallbladder is a pear-shaped reservoir
of 5-12 cm length located on the visceral surface of the liver, in the boundary between
the two lobes. Gallbladder has three anatomical segments: (Figure 4)
1. Fundus, usually reaches the free edge of the liver being in contact with the
abdominal wall.
2. Body, attached to the liver, it
comes in contact inferiorly with
the transverse colon and D2
duodenum.
3. Cervix (neck) which is the
terminal portion continuing with
the cystic duct. Here is located the
Mascagnis lymph node which
may become hypertrophic in
acute inflammation of the
gallbladder or in metastasis. A
voluminous biliary stone may
dilate the terminal portion of the
gallbladder forming the
Hartmanns pouch.
Cystic duct connects the gallbladder to the common bile duct and has a variable
length. The coiled fibers of the cystic duct wall form the Lutkens sphincter and the
inside lining mucosa disposition forms the Heister's spiral valves. The cystic duct
participates in delineation of an important surgical area of subhepatic region, the so-
called bilio-vascular triangle of Calot. (Figure 5) The triangle is bounded by the cystic
ducts laterally, the common hepatic duct medially, and the inferior edge of the liver
superiorly. The cystic artery can be found in Calot's
triangle in about 80% of patients and a careful dissection
of Calot's triangle is important for a safe
cholecystectomy.
Vascularization
Intrahepatic bile ducts are perfused by adjacent
vessels. Common bile duct is irrigated by
gastroduodenal, hepatic and cystic arteries.
Gallbladders blood supply comes from the cystic artery
333
and small vessels from liver. The cystic artery derives from proper hepatic artery and
usually is located above the cystic duct. There are many anatomical variations, and the
surgeon must be aware of this and be very careful not to confuse hepatic artery with the
cystic artery. The cystic artery is solitary in approximately 75% of cases.
Lymphatic drainage of the gallbladder is carried to the liver, the most important
anatomical structure being the lymph node of Mascagni.
2. Gallbladder Lithiasis (Cholecystolithiasis)
Gallbladder lithiasis is a widespread disease, with a relatively easy therapeutic
solution in uncomplicated forms. Complications instead, may have an unfavorable
evolution endangering the patients life. Laparoscopic cholecystectomy is the most
frequent laparoscopic operation.
Incidence
The incidence of gallstones increased over decades. The actual average incidence
is about 10-15% of the adult population in developed societies (20 to 30 million
Americans).[1-4] Gallstones reach epidemic proportions in the North and South
American Indian populations, accompanied by an increased risk for gallbladder
cancer.[5] In contrast, the rate in sub-Saharan Africa and Asia is quite low.[2]
An estimated 20% of adults over 40 years of age and 30% of those over age 70
have biliary calculi.[6] The female-to-male ratio is about 4:1, with the sex discrepancy
narrowing in the older population to near equality.[6]
Etiopathogenesis
Even though, from clinical point of view, the composition of stones is not very
important because regardless of their composition biliary stones can be removed by
cholecystectomy and ERCP, however composition analysis is important in order to
understand the pathogenesis of the disease and its prevention. Gallstones
etiopathogenesis is reflected in stones composition. Depending on composition, there
are three types of stones: (Figure 6)
1. Cholesterol stones vary in color
from light yellow to dark-green or
brown, with oval shape, 2 to 3 cm
in length, often having a tiny dark
central spot. To be classified as
cholesterol stones, they must have
at least 70% cholesterol in
composition.[7]
2. Pigment stones are small, dark
stones composed of bilirubin and
calcium salts (calcium
bilirubinate). They contain less
than 20% of cholesterol.
3. Mixed stones contain other common constituents such as calcium carbonate,
palmitate, phosphate, bilirubin, and other bile pigments. Because of their
calcium content, they are often radiographically visible.
Cholesterol gallstone formation has three causes: metabolic, mechanical and
mixed causes.
334
According to metabolic theory, stone formation occurs mainly by breaking the
balance between lecithin and bile acids on one hand (to keep cholesterol in soluble
form) and cholesterol on the other. The cholesterol-saturated bile is not sufficient to
explain the occurrence of stones; it requires the presence of a nucleus of precipitation
called nidus, represented by muco-glycoproteins in the gallbladder wall or crystals of
bilirubin.
Mechanical causes are represented by the failure of the gallbladder to evacuate
adequately its content. Obstacles can be represented by:
Sclero-inflammatory stenosis of the cystic duct
Dystrophic modifications (cholesterolosis)
Biliary dyskinesia caused by a lack of synergy between gallbladder
contraction and cystic sphincter relaxation
Congenital malformation of the gallbladder (Heister's valves hyperplasia)
Brown (bilirubin) pigment stones are formed within the intrahepatic and
extrahepatic bile ducts as well as in gall bladder. They form as a result of stasis and
infection, usually in the presence of Escherichia coli and Klebsiella spp.
Pigmented stones occur more frequently in patients with hemolytic diseases
(sickle cell anemia, hereditary spherocytosis, and thalassemia) and cirrhosis. Bile in
these patients contains a high concentration of unconjugated bilirubin and presents an
increased activity of beta glucuronidase which is an enzyme produced by E. Colli.
Perhaps the enzyme converts the conjugated bilirubin to unconjugated form, which then
precipitates to form stones.
Risk factors for gallstones. There are many risk factors incriminated but four of
them have a greater significance: the 4F
1. Female
2. Forty (age)
3. Fatty
4. Fertile
Sex. Women are twice as likely to develop gallstones as men are. Causes may be
represented by the excess of estrogen from multiple pregnancies [8,9], hormone
replacement therapy [10,11] or birth control pills.[11,12] Estrogens appear to increase
cholesterol levels in bile and decrease gallbladder movements, which can lead to
gallstones.
Age. Patients over age of 60 are more likely to develop gallstones. It seems that
aging leads to an increase amount of cholesterol in bile.
Weight. Obesity is a major risk factor for gallstones, especially in women.[13] The
most likely reason is the effect on serum lipids with higher levels of cholesterol than
normal and decreased amount of bile salts in bile, resulting in more insoluble
cholesterol. Additionally, in the obese, gallbladder may not empty normally or
completely.
Rapid weight loss. As the body metabolizes fat during prolonged fasting and rapid
weight loss (such as after bariatric surgery) the liver secretes extra cholesterol into
bile, which can cause gallstones. Weight loss further increases the risk of gallstones:
the prevalence of new gallstones reaches 10-12% after 8-16 weeks of low-calorie
diet and more than 30% within 12-18 months after gastric by-pass surgery.[14]
Diet. Diets high in fat and cholesterol and low in fiber increase the risk of gallstones
due to increased cholesterol in the bile and reduced gallbladder emptying. High
335
carbohydrate intake has been linked to insulin resistance, obesity, and abnormal
serum lipid profiles, conditions, which favor gallstone formation.[15]
Family history. Gallstones are common in relatives, suggesting that genetic factors
are responsible for at least 30% of symptomatic gallstone disease.[4]
Cholesterol-lowering drugs. Drugs that lower cholesterol levels in the blood
actually increase the amount of cholesterol secreted into bile. In turn, the risk of
gallstones increases.
Diabetes. People with diabetes generally have a higher risk to develop gallstones,
acalculous cholecystitis and biliary complications probably due to dysmetabolic
syndrome with high levels of fatty acids.
Symptoms
The disease may progress through three phases of evolution but not necessarily in
that order of succession. The patient may remain asymptomatic until complications
phase:
1. Dyspeptic phase manifested mainly by digestive discomfort, bloating,
nausea, abnormal intestinal transit and migraines.
2. Phase of pain with biliary colic manifested as violent colicky pain usually
located in the right upper quadrant or epigastrium, occurring after
cholecystokinetic foods, radiating toward the right interscapulo-vertebral
space or shoulder. In most cases, the pain diminishes under treatment with
antispastic medication. When pain does not react to medication, it means that
there are some complications such as infection or migration. Pain is
frequently associated with nausea and vomiting.
3. Stage of complications with different kind of symptoms depending on the
type of complication, but generally require emergency treatment even
surgery.
In uncomplicated forms, signs are poor. Murphy maneuver may be positive:
palpating the right upper quadrant induces tenderness of the right upper quadrant when
patient inspires deeply. In phase of complications, other signs appear such as: palpable
gallbladder in hydrops, intense tenderness with muscular defense in the right upper
quadrant in acute cholecystitis, generalized abdominal pain in gallbladder perforation
with generalized peritonitis, jaundice in migrated CBD stones, and signs of associated
pancreatitis (see acute pancreatitis).
Clinical forms
Several clinical forms can be observed depending on the main symptoms:
Asymptomatic form, when the disease is discovered during an ultrasound or
CT examination performed for another illness
Painful form manifested by frequent biliary colicky pains
Dyspeptic form manifested mainly by nausea, vomiting and bloating
Tumoral like form when pain is associated with intestinal transit disorders,
and tumoral mass palpable in the right hypochondrium
Positive diagnosis
Diagnosis is based on history and imaging explorations, less on physical
examination. The most common and useful is the ultrasound examination which can
336
reveal the gallstones, their dimension, position and also gives many important
information about the gall bladder, biliary tree and the surrounding organs. (Figure 7)
ERCP and MRCP (magnetic resonance
cholangiopancreatography) are used only in special
cases when common bile duct lithiasis is suspected.
diseases manifested by pain in the upper abdomen:
Renal colic - the pain usually is located
in the lumbar region radiating toward
the inguinal region and external
genitalia. Giordano sign is positive.
Urinalysis is modified. Ultrasound will
reveal the urinary stones or dilated
urinary tract.
Peptic ulcer pain - is locate in the epigastric region, is not colicky, has the
lesser periodicity (pain - food ingestion - relief - pain), is not calmed by
antispastics and gastro-duodenoscopy shows the ulcer.
Acute pancreatitis - the pain is in the epigastrium with transverse radiation,
associated with vomiting and altered general condition. There are high levels
of amylases. CT investigation is very useful in diagnosis.
Transverse colon tumor - the pain is associated with digestive transit
modification, anemia, and palpable tumor. Colonoscopy will highlight the
tumor.
Complications
A. Mechanical complications: (Figure 8)
Hydrops of the gallbladder
Passage of stones into the common bile duct with jaundice and possible
pancreatitis
Biliary-digestive fistulas (cholecysto-duodenal, cholecysto-gastric,
cholecysto-colic)
Biliary ileus
Bilio-biliary fistulas (cholecysto-choledocian)
B. Inflammatory complications:
Acute cholecystitis (edematous, phlegmonous, gangrenous)
Perforation
o Of the posterior wall with intrahepatic abscess
o Pericholecystic abscess
o Biliary generalized peritonitis
Chronic cholecystitis
Acute pancreatitis
Cholangitis
C. Degenerative complications:
Cancer of the gallbladder
Cholesterolosis
Calcium impregnation of the gallbladder

337
Mechanical complications
Gallbladder hydrops, happens when the biliary stones clog the cystic duct. Intense
prolonged colicky pains manifest it initially and the gallbladder may be palpable. It
produces bile stasis with superinfection and evolution is toward a possible acute
cholecystitis with perforation. The bile in the gallbladder is discolored (transparent).
Migration into the common bile duct. Small stones are more dangerous as they could
migrate through the cystic duct into the common duct. If stones are less than 3 mm
in diameter, they can pass through the papilla major into the duodenum. Symptoms
are represented by intense and prolonged colicky pain associated with transient
jaundice. If stones are larger, they will remain stocked into the common bile duct
and will produce periodically jaundice associated with colicky pains and fever
(acute cholangitis). In most cases, CBD stones are the cause of an associated acute
pancreatitis as a result of bile reflux into the Wirsung duct.
Cholecysto-choledochus fistula usually appears when a large biliary stone develops
inside the Hartmanns gallbladder pouch. Compression on gallbladder and common
bile duct walls and associated inflammatory processes will erode the walls
developing a communication between the gallbladder and CBD. Due to
compression, the most frequent clinical manifestation is the jaundice. Smaller stones
can also migrate through the fistula into the common bile duct.
Cholecysto-digestive fistulas are represented in most cases by cholecysto-duodenal
fistula and in rare cases, the stomach or even the colon may be involved. The
pathogenesis is the same as in the above type of fistula. There are unspecific
symptoms. In some cases, when the migrated stone is large enough, it can produce
intestinal obstruction, the so-called biliary ileus. On abdominal radiography, air can
be observed in the gallbladder (pneumobilia) and on barium swallow, the contrast
passes into the gallbladder.

Inflammatory complications
Acute cholecystitis is the result of stasis and infection of the bile inside the
gallbladder most often caused by obstruction of the cystic duct. There are also cases
of acute cholecystitis without biliary stones. Symptoms debut with a biliary colic.
The pain becomes permanent located in the right hypochondrium and frequently
associated with fever, nausea and vomiting. Fever and chills are signs of cholangitis
or abscess formation. On palpation, an intense pain is found in the right
338
hypochondrium (painful Murphy maneuver), with muscular guarding, even
contraction and sometimes the gallbladder can be palpated. In a next stage, when
surrounding organs come to isolate the inflammatory process, a subhepatic block
will develop containing in the middle the gallbladder that may perforate and produce
a pericholecystic abscess. In advanced stages, general signs of infection are present.
If the gallbladder perforates into the peritoneal cavity, the signs of generalized
peritonitis will appear.
Morphological types of the acute cholecystitis are: edematous, phlegmonous and
gangrenous. Emphysematous acute cholecystitis is a rare but serious condition
caused by anaerobes, which is highlighted at ultrasound and radiological
examination by the presence of air in the gallbladder wall.
Germs most often involved are: E. Colli, Klebsiella, enterococcus, staphylococcus
and anaerobes. Laboratory will show leukocytosis, high ESR and sometimes, higher
values of bilirubin. Ultrasound examination is the most useful; it will show the
presence of stones and the enlarged gallbladder with thickened (>3 mm) walls. CT
and MRI scans are indicated in difficult diagnosis cases.
Chronic cholecystitis is a condition caused by repeated inflammatory reactions of
the gallbladder with a good passage of the bile through the cystic duct. It appears in
two morphological types:
hypertrophic and atrophic. In
hypertrophic type, the gallbladder is
enlarged with thick and hard walls
of whitish color (sclerosis). (Figure
9) Calcium deposits in the wall may
accompany chronic inflammation.
When the whole gallbladder is
calcified it is termed as porcelain
gallbladder which can be easily
observed on plain abdominal
radiograph. (Figure 10) In atrophic
type, the gallbladder is small
molded on biliary stones.
Acute cholangitis can be a life-threatening complication. Characteristic symptoms
include jaundice, fever, chills, abdominal pain, and in severe cases, low blood
pressure, oligo-anuria and confusion.
Degenerative complications
Gallbladder cancer - even if it is not demonstrated a direct
correlation between cancer and biliary stones, the
gallbladder cancer is more often seen in patients with
gallbladder stones.
Cholesterolosis represents a change in the gallbladder wall
caused by the deposits of cholesterol. The name of
strawberry gallbladder comes from the typically
stippled appearance of the mucosal surface on gross
examination, which resembles the appearance of a
strawberry. (Figure 11)

339
Treatment
The main treatment is surgical represented by cholecystectomy (removal of
gallbladder). Even though surgery is the principal treatment, patients will require in
many cases, especially in complication, a multimodal treatment. Colicky pains will be
treated with antispastic drugs and antibiotics will be given in pre- and postoperative
period for acute cholecystitis. Bile can be harvested during operation and sent to
laboratory for bacteriological examination and then medication is adapted to the result
of the antibiogram.
Surgical treatment
Indications for surgery are divided in
absolute, definite and relative. Absolute
indications are represented by all acute
complications of gallstones (perforation
with peritonitis, biliary ileus, abscesses,
etc.). In most cases, surgery will be
performed in emergency condition. Definite
indications are represented by frequent
colicky pains, other complications such as
biliary fistulas, acute pancreatitis, jaundice,
cancer, etc. Asymptomatic gallbladder
stones represent relative indications.
Cholecystectomy can be performed
by laparoscopic or classical open
(laparotomy) approach. (Figure 12) In
severe complications such as perforated
cholecystitis and biliary fistula, open access
is preferred.
Classical cholecystectomy. There
are many types of incisions but the most
frequently used is the right subcostal
Kochers incision or median laparotomy.
After sectioning the abdominal wall, the
peritoneal cavity is exposed and isolated. A
general, regional and local inspection is
performed. Cholecystectomy can start from
the fundus, the so-called anterograde
cholecystectomy, from the cystic duct, the
retrograde cholecystectomy or from both
sides, the bipolar cholecystectomy. (Figure
13) Generally, the retrograde procedure is
preferred because ligation and resection of
the cystic duct as a first step prevents
further migration of stones into the common
bile duct during gallbladder manipulation
and ligation of cystic artery prevents further
bleeding during cholecystectomy. The
common bile duct is inspected and its
diameter is assessed. Palpation between two fingers (to assess the presence of stones) is
possible through the Winslow foramen. The cystic artery is found in the Callots
340
triangle, sectioned and ligated. The cystic duct is then ligated and resected. From this
point, the operation may progress in retrograde or bipolar way. The gallbladder is
divided from its hepatic bed and careful hemostasis is performed. Lavage and drainage
of the subhepatic space and then suturing the abdominal wall finishes the operation.
Cholecystendesis (cholecystolithotomy) represents the removal of gallstones
without cholecystectomy (the gallbladder is left intact). The procedure was abandoned
because of short-term recurrence of lithiasis after operation.
Mucoclasis represents the surgical removal or destruction of the inner lining (the
mucosa) of a hollow organ, in this case the gallbladder's mucosa. This procedure is
applied in advanced cases of acute cholecystitis (gangrenous) when the posterior wall of
the gallbladder cannot be detached from the hepatic bed and instead it is destroyed using
electrocautery.
Laparoscopic cholecystectomy is the most used procedure in nowadays. It is a
minimal invasive procedure, which allows rapid healing and recovery of the patient. A
laparoscopic unit (CO2 insufflator, video camera, light source, electrocautery and
monitor) and special instruments are necessary.
Absolute contraindications for laparoscopic approach are represented by major
coagulation disorders (hemostasis in laparoscopic approach is more difficult) and
contraindications for general anesthesia with tracheal intubation. Previous
supramesocolic surgery (due to adhesions) and some patients with pacemaker represent
relative contraindications. In case of previous abdominal surgery the Hassons
technique is used for introduction of the first trocar to prevent damages to the intra-
abdominal organs especially small and large intestines (a small incision at umbilicus is
performed and under direct visualization and finger control the trocar is inserted into the
peritoneal cavity avoiding intestinal lesions). Associated CBD stones represent a
contraindication just for those centers, which are not endowed with special instruments
(choledochoscope, ERCP), and for inexperienced surgeons.
The patient is positioned in supine, left rotated and in anti-Trendelemburg
position (for a better access to the gallbladder). The CO2 is insufflated through a special
needle (Veres needle) into the peritoneal cavity. The intra-abdominal pressure should
reach 12 mm Hg.
Four ports are used as follows:
1. the first trocar of 10 mm diameter inserted at umbilicus for the camera,
2. the second trocar (10 mm) in the epigastrium for working instruments (hook,
scissors, suction, etc.),
3. the third trocar (5 mm) under the right costal margins for handling the
gallbladder or elevate the liver and
4. the fourth trocar (5 mm) in the lower part of the right hypochondrium for
gallbladder manipulation and drainage.
Other position of trocars is possible but very important is the triangulation for a
good access to the gallbladder. (Figure 14)
341

In laparoscopy, tension and contra-tension is very important for exposing tissues,
to be divided. Principles and technique are almost the same as in classical
cholecystectomy, but using other kind of instruments. The cystic duct and artery are
sealed by titanium clips applied with a clip applicator. The gallbladder is detached from
the hepatic bed and then extracted through
one of the 10 mm trocar. Laparoscopic
approach can be converted to open
approach at any time when needed
(intraoperative difficulties).
If there is a suspicion of migrated
calculi into the common bile duct
(enlarged CBD, associated jaundice), an
intraoperative cholangiography through
the cystic duct or choledochoscopy can be
performed. (Figure 15) If migration is
confirmed, CBD stones can be removed in
the same operative episode, also by
minimal invasive access. Small stones can be removed
through the dilated cystic duct using the Dormia basket
through the choledochoscope. (Figure 16) In open access the
CBD is opened, stones removed and a T-tube (Kehr
drainage) is inserted into the CBD. In laparoscopic approach,
more frequently stones are removed through the papilla
major by ERCP procedure performed in the same operative
session or later. (Figure 17) If the procedure is performed in a
later session, transcystic drainage will be installed for CBD
decompression.
After removing the gallbladder its content and mucosa
are inspected. If only one faceted stone is found inside the
gallbladder it means that other faceted stones have been
migrated into the CBD (faceted gallstones appear only when
multiple).
In case of biliodigestive fistula, the gallbladder is
removed and the opening of the involved digestive segment
(duodenum, stomach or colon) is closed using sutures.
In patients with severe comorbidities that
contraindicate surgery and the gallbladder is under tension
(hydrops) or with empyema (pus) the simplest procedure is the ultrasound guided
342
percutaneous drainage of the gallbladder that allows gallbladder decompression and
prevents evolution to perforation. Cholecystostomy (external diversion of gallbladder by
minimal classical approach) is a temporary solution applied in rare cases of cholecystitis
when the patient is very ill, and there is a need to delay or defer cholecystectomy.
Intraoperative incidents could be represented by:
Perforation of the gallbladder is not a serious incident. Leaked bile is aspirated and
stones are collected from peritoneal cavity (more difficult task in laparoscopic
approach when stones are multiple and small).
Hemorrhage from cystic artery - the artery is ligated or clipped.
Lesion of the right hepatic duct or
common hepatic duct must be
recognized and repaired during the
operation. Otherwise, in the
postoperative period, biliary fistula
(exteriorized through the drainage tube)
and peritonitis will appear. When the
common bile duct was ligated, as
confusion with cystic duct, jaundice will
appear in the following days.
Reintervention and bile duct repair is
mandatory. There are several options to
repair the CBD depending on lesion
type. (Figure 18)
Lesion of the hepatic artery is a severe
complication possible evolving to liver
necrosis. Sutures or arterial
reconstruction should repair the arterial
damage.
Lesions of the liver. Bleeding will be
stopped by electrocautery or sutures.
Lesions of the duodenum or colon
should be recognized and solved by
suture during operation. Otherwise,
severe peritonitis will develop
threatening the patients life.
Generally, patients operated laparoscopically will be discharged in the next 2-3
days after operation or even earlier if there are no complications and comorbidities.
Immediate local complications:
o Intraperitoneal bleeding exteriorized through the drain tubes, in most cases
requires re-laparoscopy or laparotomy and hemostasis. Usually, bleeding
sources are represented by short vessels in the hepatic bed of the gallbladder
or the cystic artery.
o Bile leakage can be produced by aberrant bile ducts (Luska ducts), cystic duct
clip slippage, lesions of the common or right bile duct, etc. In almost all cases,
reintervention is necessary to stop the bile leak.
343
o Generalized peritonitis produced by lesions of the duodenum or colon is a
very serious complication, which need urgent re-laparotomy and suture of the
injured hollow organ.
o Acute pancreatitis occurs more frequently after instrumental manipulation
inside the CBD (ERCP).
o J aundice can be produced by remnant gallstones into the common bile duct or
lesions of the common bile duct.
o Wound suppuration especially in classic approach in acute cholecystitis.
Late postoperative complications
o Incisional hernia occurs especially after the open approach but it is also
possible at port sites in laparoscopic approach.
o Remnant gallstones in the common bile duct can be removed by ERCP.
o Stenosis of the common bile duct especially after repaired lesions of the duct.
o Postcholecystectomy syndrome is represented by a wide range of dyspeptic
and other symptoms that occur after cholecystectomy.
3. Choledocholithiasis (CBD Lithiasis)
Choledocholithiasis is defined as the presence of biliary stones inside the CBD.
The condition is present in 8% to 20% of patients undergoing cholecystectomy and in
2% to 4% of patients after cholecystectomy.[16-18]
CBD lithiasis may be primary or secondary.
Primary choledocholithiasis occurs rarely. Stones are formed within the
choledochus having a soft consistency, light brown color and an elongated shape. They
are composed of calcium bilirubinate. Favorable conditions for their appearance are
biliary stasis, infection and foreign bodies inside the common bile duct. In Asia, stones
are frequently associated with parasitic infections. Gallstones may come from
intrahepatic ducts where are formed as a result of congenital or acquired stenosis of the
main biliary pathway.
Secondary choledocholithiasis is the most common, being caused by migration of
gallstones from gallbladder. Migration usually requires small stones, less than 0.5 mm
or a large cystic duct. Rarely migration can be produced through a cholecysto-
choledochus fistula. Gallstones are usually of cholesterol with structure and
macroscopic properties like gallbladder stones.
Morphopathology
Number, location and size of stones are important factors in evolution and
treatment. There are cases with multiple stones that clog the CBD, the so-called
steinstrasse or "stone-street". Depending on position, stones can be located in the
retroduodenal (the most common) part of the choledochus, in the terminal part or
impacted into the Vater's ampulla. In rare cases, stones can be located in the intrahepatic
biliary tree or in diverticula of the CBD. Stones smaller then 3 mm usually pass through
the papilla major and enter into the duodenum. In most cases, intense colicky pains and
transient jaundice accompany this passage. Stones with diameter between 3 and 5 mm
are usually floating inside the CBD but they can block the papilla producing intense
pain, jaundice and pancreatic reaction. Stones larger than 5 mm may become impacted
into Vater's ampulla.
344
The presence of stones inside the CBD modifies the external aspect of the
choledochus. An enlarged choledochus with thin walls of venous aspect is the
consequence of recent passage of stones inside the CBD and through the papilla. An
arterial aspect of choledochus walls (thick whitish walls) means a chronic inflammatory
process.
Clinical picture
CBD stones can remain asymptomatic for a long period, but also can produce
serious complications such as obstructive jaundice, cholangitis and acute pancreatitis.
The main symptom is the jaundice that should be differentiated from other types
of jaundices. J aundice is frequently associated with other symptoms such as pain and
fever. The Charcot-Villard classic triad is represented by: pain, fever and jaundice, and
is characteristic for forms associated with angiocholitis.
Pain is intense, continuous, located in the right hypochondrium radiating
towards epigastric region and right scapula. It appears suddenly after meal and
may last several hours. Characteristic is the poor response to treatment with
antispastic drugs, which distinguishes it from a simple biliary colic.
Fever is of septic type being accompanied by chills and has sudden
exacerbations and higher values (39-40
0
C) then in acute cholecystitis.
Jaundice appears at 24 hours after the onset of pain. It is a mechanical jaundice
being associated with acholic stools (discolored stools), dark urine and pruritus
(because of the skin deposition of bile salts). In forms with mobile (floating)
stones the jaundice has a wavy character (appears and disappears as pain comes
and goes) and it is due to transient cholangitis. Impacted stones cause a
progressive jaundice, but not a melas (verdinic) jaundice (grayish-green to
greenish-black) which is characteristic for malignant stenosis (cancer of the head
of the pancreas or of the biliary ducts).
The most serious complications of choledocholithiasis are the acute cholangitis
and acute pancreatitis.
Acute cholangitis. There are two forms of cholangitis depending on severity:
Catarrhal cholangitis, characterized by the classical Charcot-Villard triad
associated with hemodynamic instability (hypotension leading to shock), and
impaired consciousness from dizziness to coma. This association is called
Reynolds pentad.
Suppurated acute cholangitis, which is extremely serious requiring
antibiotics and emergency biliary drainage.
Biliary obstruction is associated with systemic infection when pressure in the
biliary tree exceeds a critical point and bilio-venous reflux becomes possible with
the consequence of bacteremia and septicemia. The most common local septic
complications are liver abscesses, commonly multiple. Metastatic abscesses
(brain, lungs, etc.) and endocarditis can also develop.
Kidneys are affected by a complex mechanism: by hypotension (prerenal factor)
and by renal impairment produced by endotoxins causing tubular necrosis.
Clinically is manifested by renal failure with oligo-anuria. This is the hepato-renal
syndrome or so called cholangitis icterus uremigene of Caroli.
E. Coli, Klebsiella, Enterobacter and Enterococci are the most frequent involved
bacteria.
345
Acute pancreatitis is caused by the obstruction of Wirsung duct and bilio-
pancreatic reflux in cases when stones are impacted or passing the papilla.
Diagnosis relies on clinical picture and investigations.
Laboratory findings emphasize the cholestasis syndrome represented especially
by increased levels of total bilirubin above 1 mg%, with predominance of direct fraction
over 0.2 mg% (jaundice becomes clinically evident when bilirubin level exceeds 3
mg%) and alkaline phosphatase (AP) levels over 80 ui. Amylasemia is increased in case
of associated acute pancreatitis. Leukocytosis, especially over 15000/cm3.
Aminotransferases may increase but not at such levels as in acute viral hepatitis.
Abdominal ultrasound (US) is usually the first exploration. It can highlight the
biliary obstruction in up to 90% of cases (CBD dilated over 9 mm). Stones are identified
as echogenic foci with a distal acoustic shadow. Stones can be visualized in 30% - 90%
of cases, depending on the experience of the examiner and ultrasound device
performance.[16-22] 20%-30% of gallstones do not cause CBD dilatation [17] being more
difficult to be found on US investigation. Doppler may be extremely valuable by
demonstrating that dilated tubular structures in the liver are indeed ducts and not
vascular structures.
Endoscopic ultrasound examination (EUS) with transducer of high resolution
(12-15 MHz), which is inserted in the duodenum, allows the exploration of the CBD
with an index of diagnosis approximately equal or even better (99%) then ERCP [23] but
without its possible complications. EUS could replace diagnostic ERCP selecting
patients with choledocholithiasis for therapeutic ERCP.[24-26]
Computed tomography (CT) has a slightly higher index of diagnostic than
abdominal ultrasound. It is indicated especially in obese patients and for differential
diagnosis of mechanical jaundice (tumors of the head of the pancreas or other expansive
processes).
Magnetic resonance cholangiography (MRCP) has the possibility to highlight
slow flows in the abdomen such as bile and pancreatic juice. It may reveal stones in up
to 95% of cases being indicated especially in cases when choledocholithiasis is not
confirmed by ultrasonography.[27] Unlike CT exploration, MRCP has the advantage of
having less allergic reactions (the contrast material used for an MRI exam is based on
gadolinium and does not contain iodine being less likely to cause allergic reactions
compared to the iodinated contrast agents used in CT scanning) and does not use
ionizing radiation.
Endoscopic retrograde cholangiopancreatography (ERCP) is very accurate in
detecting choledocholithiasis. It is an invasive exploration, most often used for
diagnosis of CBD lithiasis but also for removing stones and for stenting the CBD in
neoplastic mechanical jaundice. ERCP is performed through an endoscope with side
view introduced into the duodenum. The papilla is found and then a catheter is inserted
into the CBD and the radiopaque substance will fill the biliary tree highlighting the
stones. It has also the possibility to perform papilla sphincterotomy and extract stones
from CBD and pancreatic duct. ERCP is encumbered by some complications of which
the most important are acute pancreatitis and reflux cholangitis with liver abscesses.
Differential diagnosis should be performed with other causes of jaundice:
Non-obstructive jaundice
Prehepatic jaundice is caused by an increased rate of hemolysis (malaria, sickle
cell anemia, spherocytosis, thalassemia and glucose 6-phosphate dehydrogenase
346
deficiency). The increased production of bilirubin, leads to the increased production of
urobilinogen. Bilirubin is not usually found in the urine because unconjugated bilirubin
is not water-soluble, so the combination of increased urine-urobilinogen with no
bilirubin in urine is suggestive for hemolytic jaundice. Laboratory findings include: no
bilirubin present in urine, urobilinogen >2 units in urine, increased unconjugated serum
bilirubin. Differential diagnosis is not difficult considering the clinical picture (without
colicky pain or fever) laboratory tests (increased indirect fraction of bilirubin) and other
investigations. On the other hand the increased rate of hemolysis predispose to
development of black pigmented gallstones.
Genetic disorders (present at birth) such as Crigler-Najjar syndrome caused by a
defect in the conjugation of bilirubin, Dubin-J ohnson and Rotor's syndromes caused by
abnormal secretion of bilirubin into bile, Gilbert's syndrome caused by a mild reduction
in the activity of the enzyme responsible for conjugating the glucuronic acid to
bilirubin, are other causes of jaundice, but not associated with other symptoms of
Charcot triad (pain and fever).
Hepatocellular jaundice in most cases is produced by the acute (viral hepatitis,
toxic hepatitis, leptospirosis, etc.) or chronic (cirrhosis - viral, alcoholic, autoimmune,
etc.) inflammation of the liver. Other causes are infiltrative diseases of the liver (liver
cancer or metastases, Wilson's disease, etc,), inflammation of the bile ducts (primary
biliary cirrhosis or sclerosing cholangitis) and drug induced jaundice (many drugs can
cause jaundice and/or cholestasis). In this case, laboratory test show elevated levels of
conjugated bilirubin, urobilirubin >2 units, and high levels of aminotransferases.
Obstructive (post-hepatic) jaundice is a mechanical jaundice and can be
produced by many causes such as:
Tumors of the head of the pancreas
Ductal carcinoma
Vaters ampulloma
Biliary ducts stenosis
Pancreatitis and pancreatic pseudocysts
Parasites
The most common differential diagnosis of choledocholithiasis is the jaundice
produced by cancer of the head of the pancreas. In the absence of investigations, history
and clinical picture are relevant enough to make the differentiation between those two
conditions. (Table 1)
Table 1 - Differential diagnosis between choledocholithiasis and tumor of the head of the
pancreas
Symptom/sign Choledocholithiasis Tumor of the head of the pancreas
Debut Acute Insidious
Pain From the beginning, colicky
pain precedes the jaundice
In initial phases, there is no pain and symptoms
begin with jaundice and/or pruritus. In advanced
stages pain is continuous but not colicky
J aundice Appears after the pain,
variable in intensity, and may
disappear after a while
Is the first sign and continuously intensifying. The
verdinic jaundice
Pruritus Rarely present Frequently present sometimes even before
jaundice
Fever Frequently present Rare
On palpation Gallbladder rarely is palpable Gallbladder is palpable - the Curvoisier -Terrier
sign and also there is a liver enlargement
Urine Darker Very dark
Stools Variable color Pale stools, even acholic
347
In complete obstruction of the bile duct, no urobilinogen is found in the urine,
since bilirubin has no access to the intestine. In this case, presence of bilirubin
(conjugated) in the urine without urine-urobilinogen suggests obstructive jaundice.
Treatment
The aim of treatment is to evacuate stones from the common bile duct and to
ensure a good emptying of bile into the digestive system. Surgery, ERCP, or both
represent the main treatment. In addition, the patient will receive antibiotherapy and
other necessary medication.
Surgery is recommended when gallbladder was not yet removed. Removing
gallbladder can be achieved by laparoscopic or open approach and the aim is to remove
the source of stones in secondary cholelithiasis.
If open surgery was chosen for cholecystectomy the next step of operation is
choledocholithotomy, (Figure 19) which means to open the CBD and remove stones.
After choledochotomy (longitudinal incision of the anterior wall of the choledochus),
the CBD is thoroughly explored by visual inspection, palpation, instrumental,
endoscopic and radiologic exploration. Biliary stones are removed using the Dejardin
forceps, Dormia basket or Fogarty probe. The main dilemma of the surgeon is whether
all the stones have been removed. The best ways to find out is to perform a
choledochoscopy or an intraoperative cholangiography. After removing all stones, the
attitude toward the choledochus may be different depending on its diameter, the
etiology of choledocholithiasis, the permeability of the papilla and the age of the
patient.

Possibilities:[28]
Primary closure of the choledocus after choledochotomy (choledochorrhaphy),
without a biliary drainage procedure. It is rarely performed because of the risk of
biliary leak (during interdigestive periods the Oddi sphincter contracts and the
pressure inside the biliary tree will rise forcing the suture).
External drainage of the CBD using a T-tube (Kehr drainage) is the most frequent
solution for choledochus less than 1.5 cm in diameter. The drainage will prevent
the biliary leak but also will allow the radiological exploration of biliary tree in
the postoperative period. If the cholangiography shows no remnant stones and a
good passage of the contrast into the duodenum, the tube can be removed after
few weeks. If remnant stones are found, ERCP is the solution for removing them.
348
Choledochoduodenostomy represents an internal biliary diversion. (Figure 20)
The opening of the choledochotomy is anastomosized to an opening of the
duodenum (duodenotomy). There are many technical variants but the most often
used is the Florken procedure. This solution is preferred in larger choledochus
(1.5-2 cm) and elderly patients. It will allow a good passage of bile into the
digestive system and of possible remnant or newly formed stones, but
unfortunately may be a cause of intermittent angiocholitis caused by alimentary
reflux into the biliary tree.
Choledochojejunostomy represents also an internal biliary diversion but the
choledochus is anastomosized to the jejunum. (Figure 21) It is chosen especially
when CBD is much dilated (over
2.5 cm) associated with chronic
inflammatory stenosis of the
papilla and a high probability of
recurrent stone formation
(multiple primary stones -
steinstrasse and intrahepatic
lithiasis). The anastomosis can be
performed with an omega jejunal
loop with Braun fistula or better
with a Roux-en-Y jejunal loop of which
length should be about 70 cm to avoid reflux
angiocholitis.
During laparoscopic approach for gallstones
if choledocholithiasis is suspected the best attitude
is to explore the CBD through the cystic duct via
choledochoscopy or cholangiography. If stones are
found, transcystic drainage and then ERCP or
ERCP in the same operative session is the best
solution. Choledochotomy and removal of stones
followed by T tube drainage is possible by
laparoscopic approach but more difficult than in
open surgery.
If the patient underwent previously
cholecystectomy, the first choice for removing stones (primary or secondary) from the
CBD is ERCP. In case of impacted stones into the papilla, and if ERCP is impossible to
be performed, the open surgery, as described above, is the solution.
4. Benign Stenosis of the Biliary Ducts
Benign stenosis of the biliary ducts is a rare pathological condition most
commonly iatrogenic, caused by surgical trauma of the biliary ducts, which may remain
asymptomatic for a long time but can cause very serious complications such as: acute
suppurated cholangitis, liver abscesses or secondary biliary cirrhosis.
Etiopathogenesis
Possible causes of benign stenosis are:
Lesions of the biliary ducts by pinching, clipping suturing or
electrocoagulation during classical or laparoscopic surgery. Extrahepatic ducts
349
could be affected in any segment but most often, the choledochus and right
hepatic duct are injured. The main causes of injuries are inability to recognize
local anatomy and inexperienced surgical team.
Abdominal trauma
Stones with repeated episodes of cholangitis and Mirizzi syndrome
(obstructive jaundice due to a bulky stone in the Hartmanns pouch which
compresses the CBD)
Repeated episodes of acute pancreatitis
Chronic pancreatitis
Sclerosing cholangitis (stenosis of intra and extrahepatic bile ducts)
Chgolangiopathy during HIV infection [29,30]
Clinical picture
Lesions usually remain undetectable until the
stenosis is sufficient to produce mechanical jaundice.
Rarely other symptoms such as right upper quadrant
pain occur before jaundice, or the illness begins
directly with signs of suppurated acute cholangitis.
The onset may be sudden or insidious. In
insidious forms of chronic cholestasis, xantelasma
appear around the eyes and dorsal thoracic region.
Weight loss raises problems of differential diagnosis
with malignant stenosis. Patients history is very
important and can give details about the etiology of
stenosis: biliary or endoscopic interventions in the past,
recurrent pancreatitis or cholangitis, etc.
Investigations are the same as for CBD lithiasis.
(Figure 22)
Classification
The Bismuth's classification (1982) [31] is based on the lowest level at which
healthy biliary mucosa is available for anastomosis and is intended to help the surgeon
to choose the appropriate technique for repair. (Figure 23)
Type 1 - Low CHD stricture,
with a length of the common
hepatic duct stump of >2 cm
Type 2 - Proximal CHD
stricture-hepatic duct stump <2
cm
Type 3 - Hilar stricture, no
residual CHD, but the hepatic
ductal confluence is preserved
Type 4 - Hilar stricture, with
involvement of confluence and
loss of communication between
right and left hepatic duct
Type 5 - Involvement of
aberrant right sectorial hepatic duct alone or with concomitant stricture of the CHD
350
Treatment
Whatever the cause of stricture, the main goal of treatment is to ensure a proper
biliary flow from liver into the digestive tract.
Medication is often associated to fight cholangitis and to repair imbalances
caused by prolonged cholestasis. Antibiotics are mandatory and should include
antibiotics with biliary excretion and those that cover the broad spectrum of gram
negative, gram positive and anaerobic germs. K vitamin is administered for coagulation
disorders.
Patients not responsive to conservative treatment require emergency biliary
decompression. Decompression can be achieved surgically or by minimal invasive
approach (percutaneous transhepatic or endoscopically). Minimal invasive approach is
preferable because is accompanied by morbidity and mortality rates lower than surgery.
Endoscopic treatment performs a papilla sphincterotomy and then inserts a
prosthesis (stent) into the bile duct. However, plastic stents should be replaced every 4-6
months to prevent cholangitis by their clogging. The endoscopic treatment of sclerosing
cholangitis is used to prepare the patient for liver transplantation. (Figure 24)
Percutaneous cholangiostomy is more useful for proximal malignant stenosis or
when endoscopic procedure fails. (Figure 25)
Surgery is used when endoscopic treatment fails and in young patients in good
biological condition with long life expectancy to avoid the inconveniences of
endoscopic replacement of the stent. Surgical treatment varies depending on the site of
stenosis but in principle is represented by a bilio-digestive anastomosis between the
suprastenotic segment of bile duct(s) and usually the jejunum (excluded from digestive
circuit in a Roux-en-Y variant). Surgical treatment has good results but is encumbered
by a higher rate of perioperative mortality and morbidity.

Prognosis
Treated before the onset of chronic complications (biliary cirrhosis) the disease
has a favorable prognosis, but depending on the underlying cause. Patients with stenosis
or sclerosing cholangitis occurring in HIV infection, have a poor outcome.

351
5. Tumors of the Extrahepatic Biliary Ducts
Tumors of the gallbladder
Benign tumors are rare, more frequently represented by polyps. (Figure 26) From
histological point of view, polyps may be:
cholesterol polyps (most frequent),
adenomyomatosis or adenomas.
Cholesterol polyps (cholesterolosis) are
caused by the excessive accumulation of
cholesterol within macrophages of the
gallbladder mucosa.
In adenomyomatosis the gallbladder wall is
excessively thick caused by the extensions
of Rokitansky-Aschoff sinuses through the
muscular wall.[32]
Adenomatous polyps are benign epithelial neoplasms with a potential risk of
malignant transformation, the risk being size related. They can be papillary
adenomas (pedunculated) or tubular adenomas (flat, sessile).
Most tumors are clinically asymptomatic, being discovered incidentally at
ultrasound examination for other pathology. Most small gallbladder polyps remain static
for years. Three- to six-monthly ultrasonography examination is warranted in the initial
follow-up period but it is probably unnecessary after 1 or 2 years.[33] Tumors exceeding
1 cm, or associated with gallstones, or in patients over 50 years, require
cholecystectomy.[33]
Malignant tumors ranks the 5
th
among digestive tract malignancies, and are found
in 1% of cholecystectomies.[34,35] In 2013 there are estimated 10,310 new cases of
gallbladder and bile ducts cancer with 3,230 death in US.[36] Gallbladder cancer is one
of the most lethal carcinomas.
Etiopathogenesis. Risk factors.[37]
Gallstones: 75-90% of malignancies are associated with gallbladder lithiasis.
Stones-cancer relationship is related to the evolution of the disease (over 15-20
years), and the size of stones (over 3 cm the risk increases 10 times).[34,38]
Porcelain gallbladder have been reported to be associated in 21-25% of cases
with cancer [39,40] but recently other authors advocate that porcelain gallbladder
is only weakly or not at all associated with gallbladder cancer.[38,41]
Female gender
Obesity
Older age
Ethnicity and geography - worldwide, gallbladder cancer is much more common
in Asian, Eastern European, and South American countries than it is in the
United States
Choledochal cysts
Benign tumors: adenomas mostly over one cm in diameter appear to be a risk for
malignant degeneration.
Chronic typhoid (Salmonella typhi) carriage (persistence) leading to chronic
inflammation and releasing oncogenic factors.[42,43]
352
Genetic abnormalities: mutations in the p53 suppressor gene on chromosome 17
are associated more frequently with gallbladder cancer.
Malformations of bilio-pancreatic tree when pancreatic juice can freely flow
back into the gallbladder causing bile stasis, leading to precancerous changes in
the gallbladder mucosa.[42]
Carcinogenic agents: nitrosamines, methylcolantren, etc.
Morphopathology
Even though there are many histopathological forms, gallbladder cancer is
represented in 80% -95% of cases of by adenocarcinoma [42] and less frequently by
other types (undifferentiated carcinomas - 7%, squamous carcinoma - 3%, mixed
carcinoma - 1%).
WHO classification is most comprehensive including the following types of
tumors:[44]
Epithelial tumors
Nonepithelial tumors
Rhabdomyosarcoma
Kaposi sarcoma
Leiomyosarcoma
Malignant fibrous histiocytoma
Angiosarcoma
Miscellaneous tumors
Carcinosarcoma
Malignant melanoma
Malignant lymphomas
Unclassified tumors
Secondary tumors
Tumor-like lesions
Carcinoma in situ
Adenocarcinoma
Papillary adenocarcinoma
Adenocarcinoma, intestinal type
Clear cell adenocarcinoma
Signet ring cell carcinoma
Squamous cell carcinoma
Small cell carcinoma (oat cell carcinoma)
Undifferentiated carcinoma
Endocrine tumors
Carcinoid tumor
Paraganglioma
Because gallbladder cancers are mostly infiltrative with high aggressiveness and
frequent metastases, there are many inoperable forms.
Gallbladder carcinoma usually produces asymmetric thickening of the gallbladder
wall with infiltration of surrounding structures. Most cancers originate in the gallbladder
fundus. As the tumor progresses, the gallbladder may fill with tumor or may contain
pus, mucus, or stones.[42]
The first organ usually invaded is the liver but spread can be over the bile ducts.
Cancer can extend through hepato-duodenal ligament to the duodenum, colon, stomach,
and pancreas. Tumoral cells spread via the lymphatic system to the Mirizzi lymph node,
around the choledochus, pancreatico-duodenal lymph nodes and so on. Spread over the
venous system involves the liver and other organs. Peritoneal spread will lead to
peritoneal carcinomatosis.
AJ CC classification (the sixth edition) [45,46]
Primary tumor (T)
T0 No evidence of primary tumor
Tis Carcinoma in situ
T1 Tumor invades lamina propria (T1a) or muscle layer (T1b)
T2 Tumor invades perimuscular connective tissue; no extension beyond serosa or into liver
T3 Tumor perforates the serosa (visceral peritoneum) and/or directly invades the liver
and/or 1 other adjacent organ or structure, such as the stomach, duodenum, colon, pancreas,
omentum, or extrahepatic bile ducts.
353
T4 Tumor invades main portal vein or hepatic artery or invades multiple extrahepatic
organs or structures.
NX Regional lymph nodes cannot be assessed
N0 No regional lymph node metastasis
N1 Regional lymph node metastasis
MX Distant metastasis cannot be assessed
M0 No distant metastasis
M1 Distant metastasis

TNM groupings by stage
Stage 0 Tis N0 M0
Stage IA T1 N0 M0
Stage IB T2 N0 M0
Stage IIA T3 N0 M0
Stage IIB T1 N1 M0
T2 N1 M0
T3 N1 M0
Stage III T4 Any N M0
Stage IV Any T Any N M1
An alternative and commonly used
system is the Nevin classification
which includes five stages:[47]
1. In situ carcinoma
2. Mucosal or muscular invasion
3. Transmural direct liver invasion
4. Cystic lymph node metastasis
5. Contiguous or/and distant liver
metastasis or metastasis to any
other organs
Clinical picture
Symptoms of gallbladder cancer overlap with symptoms of gallstones and biliary
colic. Abdominal pain may be of a more diffuse and persistent nature than the classical
right upper quadrant pain of gallstone disease. J aundice, anorexia, and weight loss often
indicate disease that is more advanced.
Physical signs in gallbladder cancer are present only in advanced cases and may
be represented by:
J aundice
Palpable mass in the right upper quadrant (Courvoisier sign, if this is due to a
palpable gallbladder)
Periumbilical lymphadenopathy (Sister Mary J oseph nodes)
Left supraclavicular adenopathy (Virchows node)
Pelvic seeding: masses palpated on digital rectal examination (Blumers shelf).
Laboratory
Tumor markers - CA 19-9 may be significantly elevated in both
cholangiocarcinoma and gallbladder cancer.
Liver function tests: elevated alkaline phosphatase and bilirubin levels are often
found with more advanced disease.
Urea, creatinine and urinalysis assess renal function prior to enhanced CT scan
examination.
CBC (Complete Blood Count): anemia may be an indicator of more advanced
disease.
Imaging Studies
Ultrasonography represents usually the first imaging investigation, which
highlights abnormalities of the gallbladder wall raising the suspicion of cancer.
A mass can be identified in 50-75% of patients with gallbladder cancer.[40]
Liver metastasis can be also seen on ultrasound.
354
Computed tomography is more accurate and demonstrates tumor invasion into
the liver and nearby organs. It also highlights other metastases and other
possible comorbidities.
Magnetic resonance imaging (MRI) offers information about cancer extension
and about vascular and biliary tree via magnetic resonance angiography (MRA)
and magnetic resonance cholangiography (MRCP).
Percutaneous cholangiography is used in advanced cases when gallbladder
cancer has extended and blocked hepatic ducts producing jaundice. It is
performed under ultrasound guidance and can be followed by percutaneous
cholangiostomy in unresectable cases.
ERCP is useful for differential diagnosis and biliary stenting.
Endoscopic ultrasonography can be used to assess regional lymph nodes
metastases.
Angiography is used to assess the involvement of important vessels (portal vein,
hepatic artery, cava vein) by the tumoral process and for planning the operative
strategy.
Chest radiography highlights lung metastases and other pleuropulmonary
comorbidities.
Diagnosis of gallbladder cancer, in most cases, is suspected by the ultrasound
image aspect, but the final diagnosis is established only by histopathologic examination
of the gallbladder or of the biopsy specimen. Incidental gallbladder carcinoma
diagnosed during or after a laparoscopic cholecystectomy is reported to be around 0.19-
3.3% in the literature.[48]
Differential diagnosis based on anamnesis clinical picture and investigations should
include:
Choledocholithiasis
Cholelithiasis
Clostridial Cholecystitis
Gallbladder Mucocele
Gallbladder Volvulus
Hepatic Carcinoma, Primary
Liver Abscess
Neoplasms of the Endocrine
Pancreas
Pancreatic Cancer
Pericholangitis
Primary Biliary Cirrhosis
Primary Sclerosing Cholangitis
Acalculous Cholecystitis
Acalculous Cholecystopathy
Ampullary Carcinoma
Bile Duct Strictures
Bile Duct Tumors
Biliary Colic
Biliary Disease
Biliary Obstruction
Carcinoma of the Ampulla of
Vater
Cholangiocarcinoma
Cholangitis
Cholecystitis
Choledochal Cysts
Treatment
Unfortunately, gallbladder cancer has a very poor prognosis because the tumor
rapidly grows and invades the neighboring organs, so few cases are suitable for surgical
resection with radical intent. Alfred Blalock wrote in 1924 that: in malignancy of the
gallbladder when a diagnosis can be made without exploration, no operation should be
performed, inasmuch as it only shortens the patients life.[49,50] In nowadays things
have changed and the only therapy with a chance of cure is considered the en bloc
355
resection of gallbladder and surrounding liver tissue, the so-called Mirizzis operation,
and regional lymphadenectomy of the hepatoduodenal ligament, performed by classical
open approach.
A significant number of gallbladder cancers will present for the second surgery,
the radical resection, after prior cholecystectomy. This is particularly common for early-
stage tumors, where most cancers are diagnosed upon pathologic analysis after simple
cholecystectomy.
Adequate surgical margins may be difficult to achieve in advanced stages. George
Pack was the first to advocate radical liver resection as treatment for gallbladder cancer,
reporting in 1955 the first three gallbladder cancers treated by right hepatic lobectomy
and portal lymph node dissection.[50,51]
The surgery role in treatment of unresectable disease is usually limited to biopsy
of the tumor for diagnosis and possible biliary decompression procedures such as
percutaneous cholangiostomy and ERCP stenting.
Adjuvant therapy consists of chemotherapy and radiotherapy.
Prognosis
Survival is correlated with the stage of disease at presentation. The best 5-year
survival rates (approximately 40%) [40] are for localized tumors whereas survival in
advanced cases is very short (few months).
Tumors of the extrahepatic bile ducts
Benign tumors of the extrahepatic biliary tree are very rare (papilloma, adenoma
or myoblastoama). Malignant tumors are also rare compared to gallbladder, in
proportion of 1/3. The common characteristic of all these tumors is that they produce
mechanical jaundice.
Etiopathogenesis and risk factors for malignant tumors
Sclerosing cholangitis increases very much the risk of
cholangiocarcinoma.[52,53]
Parasitic diseases: Clonorchis sinensis, a parasite encountered especially in
Asia, which lives in humans liver being found mainly in the common bile duct
and gall bladder, feeding on bile.[54] Ascaris lumbricoides and Pishorchis
viverrini are also incriminated in the etiology of biliary neoplasia.
Congenital anomalies of the biliary tract, and congenital cysts of choledochus
Benign tumors of the biliary tract
Ulcerative colitis is an important risk factor, cancer appearing 10 times more
frequently in these patients than the general population.[55]
Other factors such as Caroli's disease, familial adenomatous polyposis,
congenital hepatic fibrosis, hepatolithiasis, prior biliary-enteric anastomosis,
asbestosis, and exposure to dioxin, methyldopa and isoniazid.
Epidemiology
Intrahepatic cholangiocarcinoma has extremely high incidence in regions where
liver flukes (trematode) are endemic for example, in the Khon Kaen region in Thailand
(>85% of all cancers).[56] Extrahepatic cholangiocarcinoma incidence is variable, but is
twice as high in Manitoba, Canada compared to the United Kingdom. The incidence and
mortality rates of extrahepatic cholangiocarcinoma have been decreasing (by 14% in
356
USA) in the last decades.[57] Cholangiocarcinomas arise slightly more often in men,
with a male/female ratio of 1.3:1, with an average age between 50 and 70 years.[58]
Morphopathology
In 90% of cases, tumors are represented by cholangiocarcinoma Rare tumors are
squamous carcinoma, mucoepidermoid or sarcomas.
The macroscopic appearance may be:
Infiltrative, which is the most frequent form (differential to sclerosing
cholangitis very difficult)
Nodular intramural
Polypoid intraductal growing (the rarest)
Location of extrahepatic bile duct tumors can be classified as: proximal, located
at convergence of bile ducts, (the hilar type or Klatskin tumor), medium, of the liver
pedicle, and distal located on retroduodeno-pancreatic choledochus.
Clinical picture
There are 2 phases of evolution: the pre-jaundice phase, when diagnosis is
difficult with symptoms of dyspepsia, right upper quadrant pain, weight loss and the
jaundice manifested phase with
obstructive jaundice, acholic
stools, dark urine, itching, continue
pain (typically for cancer), rarely
phenomena of cholangitis.
On clinical examination
often hepatomegaly can be
detected, which may be associated
with palpable gallbladder
(Courvoisier Terrier sign) when the
tumor is located below the junction
between cystic duct and
choledochus. (Figure 27)
Investigations
The main investigations useful for diagnosis are those imagistic.
Abdominal ultrasound usually is performed first and results depend on tumor
location. In Klatskin tumor (hilar location), the gallbladder and CBD have normal
dimensions but intrahepatic ducts are dilated. When the tumor is located at the
insertion of the cystic duct into the CBD the gallbladder may be normal or distended
(hydrops) and the bile ducts upstream the tumor dilated. Retro-duodenal region
tumors are more difficult to reveal because of gases in the duodenum, but the
suspicion of tumor relies on the dilated CBD and distended gallbladder in the
absence of a pancreatic tumor.
CT scan offers more information regarding the morphology of the biliary tree and
local invasion and distant metastases. It is valuable in differential diagnosis with
tumors of the head of the pancreas.
MRI and MRCP have a higher resolution and can detect small formations with
incomplete obstruction.
ERCP highlights precisely the location of the tumor; it can perform biopsies, and
allows a temporary or palliative biliary stenting.
357
Percutaneous transhepatic cholangiography is especially useful in proximal tumors
(Klatskin) and allows for temporary or permanent biliary decompression
(cholangiostomy).
Serology tests are not very useful because there are no specific tests for biliary
cancer, but the serum CA19-9 marker is elevated in most patients with bile duct cancer.
Because there are cases in which differential diagnosis with sclerosing cholangitis
is difficult based only on macroscopic aspect and imaging tests, the histopathology test
is necessary to establish the diagnosis with certainty. There are several methods used to
obtain biologic material for histopathology. The fine needle aspiration (FNA) is
performed using a fine needle guided by ultrasound into the lesion and then gently
sucking out cells for microscopic examination. The procedure has the advantage of not
requiring an operation or general anesthesia. Bile duct brushings can be performed
through an endoscope to detect malignant cells. Biopsy of the biliary tract can be
performed by ERCP or through an exploratory laparotomy or laparoscopy.
Differential diagnosis is that of mechanical jaundice. Lithiasic etiology is easily
excluded (colicky pains are missing, jaundice has other features and ultrasonography
does not find any stone into the CBD), but the surgeon should be aware that
cholangiocarcinoma is frequently associated with lithiasis. Tumors of the head of the
pancreas are the most frequent considered for differential diagnosis but imagistic
investigations are very helpful in this regard. The most difficult is to make the
difference between an invasive (along the biliary tract) tumor and sclerosing cholangitis,
and histopathology is the only helpful examination.
Treatment
The treatment is multimodal including more or less invasive methods and
chemotherapy depending on the evolution stage of the disease. The aim of radical
surgery is to remove the tumor in oncological limits and to reestablish the bile flow into
the digestive system. Palliative methods are aimed to remove the jaundice by biliary
tract decompression. In most cases, the type of surgery (curative or palliative) to be
applied can be established only during operation. Decision-making is facilitated by
intraoperative ultrasound and cholangiography exploration.
Curative resection depends much on tumor location.
Tumors of the distal third benefit from cephalic duodenopancreatectomy (the
same operation performed for cancers of the head of the pancreas). (Figure
28)

Tumors of the middle third require partial resection followed by anastomosis
of the upper bile duct stump to a jejunal loop (Roux-en-Y procedure most
frequent used). (Figure 29)
358

Proximal tumors (Klatskin) raise special problems requiring resection of the
junction and anastomosis of the right and left bile ducts to an intestinal loop.
Klatskin tumors with invasion of the liver require combining different types
of hepatic resections. (Figures 30,31)

If the tumor cannot be removed, bypass procedures may be performed to relieve
the patient's symptoms (jaundice, pain, pruritus, etc.) and to prevent further obstruction
of the gastrointestinal tract. Tumors that have a free supratumoral segment can benefit
from different types of bilio-digestive diversions:
Cholecysto-gastrostomy, or -duodenostomy, or jejunostomy
Choledocho-duodenostomy or choledocho-jejunostomy
Hepatico-jejunostomy
In Klatskins tumors, the possibilities are represented by:
Transtumoral intubation (forage) and drainage (Figure 32)
Transparietohepatic (percutaneous) biliary drainage
Intrahepatic cholangiojejunostomy
Hapato-gastrostomy (Figure 33A)
Hepato-jejunostomy (Figure 33B)
359

In patients in whom the inoperability of the tumor is well established and in those
with associated comorbidities where surgery is contraindicated minimal invasive
procedures are the best solution. These procedures are represented by biliary stenting
via ERCP or biliary decompression via the percutaneous cholangiostomy.
Prognosis is poor with an overall survival rate at 5 years of less then 5% of cases. A
better survival rate was reported by J apanese authors (10% to 44% depending on tumor
stage) in distal resectable tumors.[59,60]
Vater's Ampulloma (Tumors of the Ampulla of Vater)
Carcinoma of the ampulla of Vater develops in the last portion of the biliary tree
within the ampulla of Vater and papilla major. (Figure 34) It is a rare tumor accounting
for approximately 0.5% of all gastrointestinal tract
malignancies.[61,62]
From histopathological point of view, 10% are
benign and 90% malignant.
Clinical picture is not specific being represented
often by digestive complains such as anorexia, nausea,
vomiting. In advanced cases jaundice, pruritus and
weight loss appear. J aundice is intermittent due to
partial necrosis of the tumor, which causes a partial
desobstruction. Tumor necrosis may be associated with
an episode of upper gastrointestinal bleeding with
melena (black, tarry, and foul-smelling stools).
Differential diagnosis should
consider all other causes of mechanical
jaundice.
The diagnosis relies on
endoscopy (duodenoscopy) which
allows direct visualization of the tumor
and biopsy harvesting. Endoscopic
ultrasonography is useful in assessing
the tumoral extension and lymph nodes
metastases. CT scan is performed to
assess tumoral invasion. (Figure 35)

360
The evolution and the local extension of the tumor are slower than that of
cholangiocarcinoma.
Radical treatment is possible in as many as 80% of cases and is represented by
cephalic duodenopancreatectomy and postoperative results are more favorable than after
resection for pancreatic or distal CBD cancer. Transduodenal ampullectomy is an
alternative reserved to patients who do not support the extent of PCD intervention.
(Figure 36)

Endoscopic stenting of ampulla may be a temporary solution for preoperative
CBD decompression, or a definitive one in inoperable patients.
Other palliative solutions for unresectable cases are represented by bilio-digestive
bypasses.
Prognosis in resectable cancers is relatively good with a survival rate around 65%
at five years.[63,64]
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cholangitis. - J . Hepatol. 2002; 36(3):321-327.
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Clonorchiasis in Korea. - J . Korean Med. Sci. 2010; 25(7):10111016.
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13(5):495-505.
56. Braconi C, Patel T.- Cholangiocarcinoma: New insights into disease pathogenesis and biology. - Infect. Di.s Clin. North
Am. 2010; 24(4):871884.
57. Yasser Shaib, HashemB. El-Serag - The Epidemiology of Cholangiocarcinoma. - Semin. Liver Dis. 2004; 24(2):115-
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24(2):131-136.
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Peritonitis
PERITONITIS

1. Anatomical aspects
2. Peritonitis, generalities
3. Etiologic forms of primary peritonitis
a. Streptococcus peritonitis
b. Pneumococcal peritonitis
c. Gonococcal peritonitis
d. Chlamydia peritonitis
e. Tuberculous peritonitis
4. Acute localized peritonitis
1. Anatomical Aspects
The peritoneum is the serosa membrane that forms the lining of the abdominal
cavity and covers the most of the intra-abdominal organs. It is the largest serosa in the
body (1.7 to 2 square meters).
The intraperitoneal cavity is the space located within the abdomen and wrapped
by peritoneum. It is divided arbitrary by anatomical structures in some compartments
and recesses creating an interconnecting network that allows the spread and
sequestration of intraperitoneal effusions.
The transverse mesocolon divides the
peritoneal cavity in two spaces: (Figure 1)
1. Supramesocolic space, and
2. Inframesocolic space
The small bowel mesentery divides the
inframesocolic space into two compartments:
1. The right infracolic, and
2. The left infracolic
The right and left paracolic gutters are formed
between the lateral aspect of the ascending and
descending colon and the peritoneal reflection on the
abdominal wall. These gutters represent
communications between the supramesocolic and
pelvic area.
The falciform ligament separates the right from the left subphrenic space.
Normally, the amount of intraperitoneal fluid is less than 50-100 mL.

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Peritonitis
2. Peritonitis - Generalities
Peritonitis represents an inflammation of peritoneal serosa, generalized or
localized, of bacterial or chemical etiology. Peritonitis is a serious life threatening
condition, a surgical emergency in most cases.
Classification
Classification by origin of germs:
1. Primary, when the source of contamination is extraperitoneal, the
infection being produced via the blood or lymphatic circulatory system.
This type is very rare (5%) and is monobacterial (a single germ is
responsible for peritonitis).
2. Secondary peritonitis are the most frequent (95% of cases) having a
plurimicrobial etiology. There is an intraperitoneal source of
contamination, such as: perforations of intra-abdominal hallow organs,
inflammation of abdominal organs or postoperative.
3. Tertiary peritonitis is represented by persistent or recurrent infection
after adequate initial therapy.
Classification by evolution:
1. Acute peritonitis, which are the most frequent.
2. Chronic peritonitis (rare) caused in most cases by recurrent inflammatory
disease of the intraperitoneal organs (pelvic), intraperitoneal presence of
foreign substances (talcum, barium) or tuberculosis.
Classification by extension:
1. Generalized (diffuse) - extended to the entire peritoneal cavity.
2. Localized - in some peritoneal regions or compartments.
Classification by the presence of germs:
1. Bacterial peritonitis (septic) - the vast majority.
2. Chemical peritonitis - produced by gastric juice from a perforated peptic
ulcer, bile, pancreatic enzymes (pancreatitis), etc.
The most common bacteria identified in peritonitis are gram-positive germs (E.
coli, Enterobacter, Klebsiela), gram-negative germs (Enterococci), anaerobic
(Bacteroides, Clostridium) and fungi (Candida).
Pathophysiology
In secondary septic peritonitis, which is the most common type, intra-abdominal
sepsis results from a perforated viscus with direct spillage of luminal content into the
peritoneal cavity (eg. perforated peptic ulcer, diverticulitis, appendicitis, iatrogenic
perforation). With the spillage of the content, gram-negative and anaerobic bacteria,
including common gut flora, such as Escherichia coli and Klebsiella pneumoniae,
contaminate the peritoneal cavity. Germs multiply producing different kind of toxins
leading to local inflammatory reaction and tissue damages. Toxins will enter the blood
stream inducing a general inflammatory response also affecting the functionality and
structure of important organs such as cardio-vascular system, kidneys, lungs, and so on.
Not just toxins but germs themselves spread over the vascular system resulting in
metastatic abscesses. Defense mechanisms of the body try to limit the effects of
infection but in most cases, they are outweighed by the massive infection.
The most dangerous toxins are endotoxins produced by gram-negative bacteria,
which lead to the release of cytokines that induce cellular and humoral cascades,
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Peritonitis
resulting in cellular damage, septic shock, and multiple organ dysfunction syndrome
(MODS). Massive quantities of toxins are released also by bacterial destruction
produced by antibiotics, so antibiotherapy, which otherwise is very important in limiting
bacterial multiplication, cannot be considered the single therapy in these cases. The
most efficient therapy is represented by surgical removal of septic peritoneal content
(abundant lavage) and resolving the cause of contamination. Both methods surgery and
antibiotherapy (and other measures too) should be applied together for the best
outcome.
The gravity and evolution of peritonitis depends on two factors: 1. the number
and virulence of germs, and 2. the particular reactivity of the patient.
Chemical (sterile) peritonitis is caused by irritants such as gastric juice, bile,
blood, or other substances without bacterial inoculation of the peritoneal cavity. The
most intense abdominal pain is caused by gastro-duodenal juice. Although initially there
was no bacterial contamination, in evolution, the chemical peritonitis will turn into a
septic peritonitis.
The primary defense reaction of the peritoneum to the contamination is to isolate,
by different kinds of mechanisms, the infection. Chemotactic and plastic proprieties of
the greater omentum and other mobile intra-abdominal organs, especially the small
intestine, contribute to isolation of the infection by compartmentalization, but in most
cases fail to completely eliminate the infection resulting in intraperitoneal abscess
formation. The production of fibrin exudates is an important part of the host defense,
but large numbers of bacteria may be sequestered within the fibrin matrix. It is also an
important factor in development of residual infection and abscess formation.
In primary peritonitis, contamination of the peritoneal cavity may be caused by
translocation of bacteria across the gut wall or mesenteric lymphatics and, less
frequently, via hematogenous seeding in the presence of bacteremia. More than 90% of
cases are caused by a monomicrobial infection (gram-negative or gram-positive). Most
cases occur in cirrhotic patients with ascites. In adults, primary peritonitis develops in
up to 25% of patients with alcoholic cirrhosis.[1]
The most common cause of postoperative peritonitis is the anastomotic leak, with
symptoms generally appearing around postoperative days 5-7. Peritonitis leads to
increased hospitalization and mortality rates.
Symptoms are represented by abdominal pain, vomiting, stop of bowel movements,
hiccups, fever, impaired general condition and others.
The onset of pain may be sudden, violent like a knife stab, when a hollow organ
is perforated (such as the stomach, duodenum, or colon) or it may be gradual when
infection spreads from an inflamed organ (appendicitis, pancreatitis, diverticulitis, etc).
The initial location of the pain (localized peritonitis) depends on the main organ
involved, but in few hours, it becomes generalized. Initial location of pain depending on
the affected organ:
Stomach and duodenum - in the epigastrium
Appendicitis - in the right iliac fossa
Colic diverticulitis - in the left iliac fossa
Genitalia inflammation - in the hypogastrium
The pain is very intense, especially in perforations, with maximum of intensity in
the causing organ region and the patient takes an antalgic position, any movement
exacerbating the pain.
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Peritonitis
Vomiting is initially reflex (sometimes may be absent as in perforated peptic
ulcer) but as peritonitis progresses to paralytic ileus it becomes more frequent with
fecaloid content. According to Stokes law, the smooth muscles paresis produced by
peritoneal serosa irritation will lead to paralytic ileus and the abdomen becomes
distended.
Hiccups are caused by the diaphragmatic irritation.
General and local signs
Patients position is generally on lateral decubitus with legs folded as an antalgic
position.
The facial aspect of the patient has some special features, the so-called peritonitic
or Hippocratic face described by Hippocrates as follows: the nose sharp, the eyes
sunken, the temples fallen in, the ears cold and drawn in and their lobes distorted, the
skin of the face hard, stretched and dry, and the color of the face pale or dusky. and if
there is no improvement within [a prescribed period of time], it must be realized that
this sign portends death.[2]
Fever (38-39
0
C) is present in most case but may be absent at the debut, in
elderly, children and immunosuppressed patients. In postoperative peritonitis, fever may
represent the first alarming sign drawing the surgeons attention to an imminent intra-
abdominal complication.
The pulse is accelerated in concordance with fever. Blood pressure at the
beginning is normal but as the process evolves toward hypovolemic shock, it lowers.
Dyspnea is due to abdominal wall contraction, diaphragmatic irritation and
abdominal distension. Sudden postoperative dyspnea is a very important sign in surgery
for patients operated on the abdomen because it may represent the first symptom of an
intra-abdominal complication with peritonitis, especially in unreactive elderly patients,
where pain is also faded by analgesic medication.
Signs of hypovolemic and/or septic shock are represented by pale skin, cold and
sweaty, hypotension, tachycardia, and oligo-anuria.
Local signs
On inspection, the abdominal wall is immobile, it does not participate to the
respiratory movements (muscular contracture) and in non-obese patients, even the relief
of abdominal muscles is visible. This aspect is seen in early phases of generalized
peritonitis, especially in chemical peritonitis caused by perforations.
Pain is the cardinal sign of peritonitis. It can be observed on superficial and deep
palpation and on percussion.
Palpation is the most important physical examination. Depending on type and
etiology of peritonitis there are many signs and maneuvers very helpful in diagnosis. On
superficial palpation, the cutaneous hyperesthesia can be noticed, representing the
increased sensitivity to sensory stimuli, such as pinch or touch (Voskresenski maneuver
in appendicitis, Dieulafoy sign). Abolition of cutaneous reflexes (abdominal muscles
will not contract on skin stimuli) can be also observed. Induced pain on profound
palpation has the maximum intensity in the region of the causing organ.
Muscular guarding represents the reflex contraction of the abdominal wall
muscles on palpation that resumes after palpation is finished, but reappears in a new
attempt. It is present in the early phases of the peritonitis.
Muscular contracture is not induced by palpation, the abdominal wall muscles
being permanently contracted. It is a painful contraction, initially localized and then
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Peritonitis
generalized. The aspect of abdomen of board is most often seen in chemical
peritoneal irritation during peptic ulcer perforation. Abdominal contracture disappears
in advanced stage of peritonitis being replaced by abdominal distension.
Even gentle abdominal percussion can induce abdominal pain in acute peritonitis,
the so-called Bell sign. In case of perforated peptic ulcer, the prehepatic dullness
disappears on percussion due to the intraperitoneal gas (from stomach). Shifting
dullness is present in intraperitoneal effusions.
On auscultation, in initial phases, intestinal movements can be heard but later
these disappear (the abdominal silentium) as intestines enter in paresis.
On rectal and/or vaginal digital examination the pain is induced by finger
palpation of the Douglass pouch and the patient screams of pain, the so-called
Douglass pouch screaming sign.
Diagnosis
In most cases, the diagnosis relies on anamnesis and physical examination. Lab
and other investigations serve to determine the cause of the peritonitis and the patients
status.
Lab tests will show hyperleukocytosis and other modifications depending on
status of the patient.
Plain abdominal radiography may show pneumoperitoneum in case of hollow
organs (stomach, duodenum, colon) perforation, and fluid-air levels when paralytic ileus
is installed.
Abdominal ultrasound examination can reveal intraperitoneal collections
(effusions) and other pathological changes suggestive for the underlying disease.
Paracentesis is sometime performed to extract peritoneal fluid for assessing its
macroscopic aspect and for bacteriological examination and antibiogram for the
sensitivity of an isolated bacterial strain to different antibiotics. Diagnostic peritoneal
lavage may be helpful in patients who do not have conclusive signs on physical
examination or who cannot provide an adequate history.
Laparoscopy can be performed in uncertain cases, and may represent a way of
approach for surgical treatment.
CT scan is performed in uncertain cases or uncertain underlying disease. It is very
useful to determine the presence of isolated intraperitoneal effusions (intraperitoneal
abscesses).
Differential diagnosis will include other conditions manifested by intense abdominal
pain, such as:
Acute medical abdomen:
o Biliary and renal colic - the pain is colicky, not continuous, being
calmed by antispastic drugs
o Saturnine colic is produced by lead poisoning
o Tabes pain, "tabes dorsalgia", is a related back pain produced by
advanced syphilis
o Intense abdominal pains can manifest acute porphyria, a rare autosomal
dominant metabolic disorder affecting the production of heme, the
oxygen-binding prosthetic group of hemoglobin

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Peritonitis
False acute surgical abdomen:
o Myocardial infarction, especially in posterior-inferior location, could
be easily confused with a perforated peptic ulcer
o Basal pneumonia or pleural effusions
o Zona zoster
o Mesenteric lymphadenitis is the most frequent misleading condition
in differential diagnosis of acute appendicitis
Acute surgical abdomen:
o Acute mesenteric ischemia
o Intestinal obstruction
o Acute pancreatitis
o Hemoperitoneum
o Intra-abdominal organs torsion (volvulus)
In all these cases, besides anamnesis and physical examination, other laboratory
tests and investigations should be carried out for a correct diagnosis because sometimes
an unnecessary surgical exploration of the abdominal cavity, the so-called white
laparotomy may endanger the life of patient suffering of other comorbidities.
Treatment - general principles
Peritonitis prognosis depends on etiology, time elapsed between onset and
treatment and patients status and comorbidities. Generally, the mortality rate is around
5% but it may increase up to 80% for neglected peritonitis.[3]
There are three main objectives of treatment: resuscitation, antibiotics and
surgery.
1. Resuscitation must be rapid and sustained and will include:
1. Gastric decompression (nasogastric tube, prohibition of oral nutrition)
2. Fluid, electrolyte and energetic (glucose, saline, ringer, blood plasma
amino acid solutions, etc.) rebalancing
3. Prevention and control of acute respiratory failure (oxygen, naso-tracheal
suctioning of bronchial secretions, tracheostomy if necessary)
2. The patient will receive broad-spectrum antibiotics, both for aerobic and
anaerobic bacteria, and fungi as well.
3. Surgical treatment is the most important in secondary peritonitis. Patients will
be operated as soon as possible, but their overall condition and associated illnesses will
be considered (preoperative preparations and vigorous resuscitation are important even
if the operation will be delayed for several hours). Choosing the type of operation
depends on many aspects. If the diagnosis is known, the incision will be targeted
directly to the septic focus. If the diagnosis is not known, a median laparotomy
(extended if necessary) will be performed, or better a laparoscopic exploration when
possible and if not contraindicated. Laparoscopy is useful for diagnosis, to rule out other
causes of abdominal pain in uncertain cases, but laparotomy offers the best approach for
a good exploration and lavage of the peritoneal cavity.
There are three possible ways to treat the perforated organ:
Direct closure of perforation or excision and suture
External drainage of the perforated organ
Partial resection or removal of the perforated organ
The peritoneal cavity will be washed thoroughly with warm saline and drained to
prevent intra-abdominal fluid sequestration and abscesses formation. Abdominal
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Peritonitis
drainage will also provide clues on the integrity of anastomoses (drains shall be placed
in the vicinity of lesion and in declivities such as Douglas pouch, paracolic and
subphrenic spaces).
Postoperative evolution may be shadowed by some complications such as:
Death in the first 2-5 days caused by toxic-septic shock
Intraperitoneal abscesses formation
Intestinal occlusion due to intraperitoneal adhesions
Wound infection and evisceration or later incisional hernias
Prognosis
In case of uncomplicated peritonitis, the mortality rate is less than 5%, but this
rate may increase in severe infections. Factors that independently predict worse
outcomes include:[3]
advanced age
malnutrition
presence of cancer
preoperative organ dysfunction
The development SIRS (systemic inflammatory response syndrome) and MSOF
(multiple system organ failure) can increase the mortality rate to greater than 70%-
80%.[3]
3. Etiologic Forms of Primary Peritonitis
a. Streptococcus peritonitis
This type of peritonitis is produced by beta hemolytic streptococcus in patients
bearing infectious foci such as angina, erysipelas, otitis, puerperal infections, and scarlet
fever. Intraperitoneal there is a large quantity of pus of matted aspect, of yellow gray
color, without false membranes, without tendency to seclusion. Peritoneum is intensely
congested, and bowels are distended.
b. Pneumococcal peritonitis
It was the main cause of peritonitis in preantibiotic era. The frequency in
nowadays is low (occurs in children between 5 and 10 years old). It is caused by
encapsulated pneumococcus usually originating from lungs and oropharyngeal
infections in patients with impaired immunity. Intraoperative aspect is of greenish-
white, creamy, odorless pus, with false membranes, mesenteric lymph nodes swelling
and congested intestines.
c. Gonococcal peritonitis
It occurs in girls and young women with poor hygiene, the infection propagating
from the external genitalia. It can occur in men exceptionally (from epididymitis
gonorrhea), in which case, the route of inoculation is hematogenous. After a sudden
onset with violent pain, symptoms vanish in 4-5 days, which is why the disease was
characterized by Mondor as a lightning. The peritoneal fluid is gelatinous, greenish,
and rich in fibrin.
d. Chlamydia peritonitis
A particular form of peritonitis is that represented by the Curtis-Fitz-Hugh
syndrome, caused by Chlamydia Trachomatis infection. From the salpinx, the infection
spreads to the perihepatic area, mimicking an acute cholecystitis. The infection will
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Peritonitis
develop perihepatic adhesions. The ultrasound examination is normal and Pap smears
confirm infection with Chlamydia.
e. Tuberculous peritonitis
Peritoneal tuberculosis is an uncommon site of extrapulmonary infection caused
by Mycobacterium tuberculosis. It appears more frequently in young people up to 40
years old.
The infection spreads via hematogenous route from active pulmonary TB or less
frequently, directly from an infected small bowel or salpinx.[4] In most cases, these
patients have an impaired immunity because of associated comorbidities such as cancer,
cirrhosis, diabetes, AIDS or are treated with corticosteroids.
The onset is insidious with unspecific misleading abdominal symptoms, the
diagnosis being delayed with several months, in part because it is not suspected.
There are three possible intraoperative aspects:
1. Wet peritonitis, or the ascitic form, characterized by transparent, yellowish
peritoneal fluid and tendency to coagulation. On the peritoneal surface,
there are numerous miliary granules, whitish nodules, small as needle pins
with discontinuous distribution. In this case, surgical treatment is
represented by fluid evacuation abundant lavage without drainage.
2. Dry peritonitis, the fibrocaseous, adhesives or plastic form, characterized
by reduced volume of ascites, but plurivisceral conglomerates with
median location. Frequently patients are operated for an intestinal
occlusion or tumoral mass of obscure origin. The solution is represented
by adhesiolysis (bands of adhesions are divided) freeing up all intestines
followed or not by enteroplication. Enteroplication represents a surgical
technique by which adjacent loops of intestine are sutured to each other to
prevent further intestinal occlusion. If necessary, segments of affected
intestines can be resected followed by intestinal tract reconstruction.
3. Purulent form, characterized by a cold abscess filled with by tuberculous
pus surrounded by adherent intestine and omentum. External fistulization
may appear to the skin, or internal into the bowel. Surgical solution is
represented by adhesiolysis, evacuation of the abscess, or if necessary,
segmental resection of the affected intestines.
4. Acute Localized Peritonitis
Localized peritonitis is represented by intraperitoneal abscesses, which are
purulent collections in a limited area of the peritoneal cavity. (Figure 2) The main cause
is the perforation of a hollow organ (stomach, duodenum, appendix, colon, gallbladder,
etc) followed by rapid isolation of the pathologic process by nearby organs and greater
omentum. This is the mechanism generally encountered in acute appendicitis with
periappendicular abscess, peritumoral abscess after colon tumor perforation or
subhepatic abscess after gallbladder or peptic ulcer perforation. Another mechanism is
the following: the patient was initially operated for a generalized peritonitis but because
of an insufficient cleaning and drainage of the peritoneal cavity, some fluid collections
remain stocked in different abdominal compartments or between intestinal loops and
transform in abscesses.
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Peritonitis

Abscesses occur thanks to the defense role of the peritoneum, which seeks to limit
the infection, but cannot totally defeat it. They are less serious than acute diffuse
peritonitis, but may cause severe complications such as rupture into the large peritoneal
cavity, and sepsis.
Topographically, purulent collections may be located anywhere but the most
common are those located under the diaphragm and in the Douglas pouch.
Initially, collections walls are formed by surrounding anatomic structures but
thereafter they become fibrous, hard and thick.
Clinical picture
Symptoms vary by location of collection, but general signs, especially septic
fever are those that attract attention to the existence of a septic process.
1. Interhepatophrenic abscess manifests with pain in the right upper quadrant
and hemithorax. Tachypnea is associated with polypnea. Hiccups and pain on the
phrenic nerve route (shoulder) represent the phrenic irritation syndrome.
2. Left subphrenic abscess (collection of pus located between the left diaphragm
and sustentaculum lienalis) is manifested by spontaneous pain in the left upper
quadrant, hemithorax and shoulder, pain on palpation of intercostal space and parietal
edema.
3. Subhepatic abscess manifests with diffuse abdominal pain or fixed (Carnot),
painful muscular guarding (not a true contracture) and painful dullness in the right
upper quadrant.
4. Abscess in the bursa omentalis manifests with nonspecific, misleading
symptoms (epigastric pain, fever, digestive disorders, nausea and vomiting).
4. Inframesocolic abscesses manifest with local pain, abdominal guarding, bowel
disorder (especially constipation) offering a clinical picture of an intestinal febrile
occlusion.
5. Pelvic abscess is manifested by a deep suppuration syndrome with signs of
pelvic irritation: rectal and bladder tenesmus, polyuria, dysuria, diarrhea. Digital rectal
or vaginal examination is relevant finding the Douglas pouch bulging and very painful
(the Douglas scream").
Investigations
Establishing the diagnosis is not always an easy task because small abscesses are
difficult to be visualized by various imaging procedures the more if they are located
between intestinal loops.
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Peritonitis
373
Standard abdominal X-ray offers limited information. The only positive sign is
the presence of fluid-air levels, which can be confused with or even may represent an
intestinal occlusion.
Ultrasound examination of the abdomen is useful in most cases because it can
highlight the collection, describe the wall and the contents of collection, allowing
guided puncture.
Computer tomography with contrast is the preferred examination that confirms
the diagnosis in almost all cases and also can guide the puncture.
Laboratory investigations are not useful for diagnosis except the microbiologic
examination of the extracted fluid, which is important for conducting an etiologic
treatment based on antibiogram.
Treatment
Percutaneous drainage, guided by ultrasound or tomography is the treatment of
choice in many cases. Effectiveness of the method is 85% but there is a rate of 0-15%
possible complications represented especially by gastrointestinal, pleural or vascular
perforation.
Surgical approach is performed in case of percutaneous drainage failure, or when
additional surgical gestures are required. Abscesses between intestinal loops or deep and
multiple collections require laparotomy. The parietal approach should be as direct as
possible to avoid contamination of the pleura or peritoneum. Douglas pouch abscesses
can be evacuated and drained through the vagina in women and through the rectum in
men.
References
1. Radojkovic M, Stojanovic M, Zlatic A, Ljiljana J eremic-Savic, Danijela Radojkovic, Mirjana Radisavljevic - Primary
peritonitis. - Acta Fac. Med. Naiss 2008; 25:133-138.
2. J ane M. Orient - Sapira's Art and Science of Bedside Diagnosis, chapter 9, pp 161 - Lippincott Williams & Wilkins, Mar
28, 2012.
3. Daley BJ . - Peritonitis and Abdominal Sepsis - Medscape reference, Emedicine -
http://emedicine.medscape.com/article/180234-overview#aw2aab6b2b6aa
4. Valerie Byrnes, Sanjiv Chopra - Tuberculous peritonitis - UpToDate, Inc. updated: Oct 18, 2012,
http://www.uptodate.com/contents/tuberculous-peritonitis
Intestinal Occlusion
INTESTINAL OCCLUSION

Intestinal occlusion represents the stop of the intestinal transit produced by
various causes, being one of the most common abdominal surgical emergencies.
Classification
There are two main criteria of classification, which are used with immediate
practical implication, such as:
1. Etiopathogenic criteria
a. Mechanical occlusions - by obstruction or by strangulation
b. Dynamic occlusions - paralytic or spastic
c. Vascular causes of occlusions - embolic or thrombotic
2. Topographic criteria
a. High-level occlusions - pylorus, duodenum, jejunum
b. Intermediate occlusions - ileum, transverse and right colon
c. Low-level occlusions - left colon, sigmoid, and rectum
Dynamic (functional) Occlusions
The dynamic intestinal occlusion represents the stop of the intestinal transit due to
functional (not mechanical) causes (there is no obstacle along the digestive tract, the
lumen being free). Pathological processes that disturb the function of the autonomic
nervous system of the intestines may be:
Abdominal infections (peritonitis)
Injuries (craniocerebral or abdominal trauma)
Lung infection (pneumonia)
Systemic infections (septicemia)
Vascular (portal vein thrombosis, myocardial infarction, mesenteric
thrombosis)
Abdominal colic (kidney, biliary)
Metabolic changes in (hypokalemia, hyponatremia, uremia)
Dynamic occlusions have the following characteristics:
1. General condition declines slowly
2. Vomiting are abundant
3. Abdominal distension is intense
4. Intestinal transit stops completely
5. Untreated, functional occlusions can turn into mechanical occlusions
Pathologically there are two types of dynamic occlusions with obvious
differences between them:
1. Paralytic occlusions, which occur because of the inhibition of intestinal
smooth muscles contraction. Intestines are distended containing gas and
fluids. Intestinal wall is very thin with well visible vascular drawing.
2. Spastic occlusion characterized by segments of intestines with spastic
contraction alternating with segments of dilated intestines. Intestinal loops
are contracted without evident lumen, with pale serosa and unapparent
vascular drawing.
374
Functional occlusions do not require surgical therapy except peritonitis.
Treatment is represented by correction of metabolic imbalances and other measures
such as nasogastric aspiration, enemas, drugs that stimulate intestinal peristalsis.
Mechanical (Organic) Occlusions
Considering the evolution and physiological implications, there are essential
differences between the two types of mechanical occlusions: by obstruction and by
strangulation. (Figure 1)

1. Occlusions by obstruction, produced by endogenous (located inside the bowel
lumen) or exogenous (located outside the intestinal wall) factors such as inflammatory
stenosis, tumors, foreign body, external compression, adhesions, etc., are considered
occlusions with "open loop" which means that the obstructed loop may evacuate
through its proximal opening. The intestinal loop vascularization remains functional and
necrosis and perforation occur later.
In occlusion by obstruction, chronic colicky pains frequently precede the
occlusion. At onset, the pain intensity is low, and then gradually increases. There is a
slow progression of symptoms with relatively good general condition. Vomiting and
abdominal distension appear later. The abdomen is usually painless and there is no
abdominal guarding.
Occlusion caused by colon cancer is more common when tumor is located on the
left colon than on the right one because the lumen is narrower. Tumors located on the
right colon may increase more in volume until they produce obstruction because of the
larger diameter of the colon, so that tumors may become palpable.
Causes of occlusion by obstruction can be classified as:
Defects of the intestinal wall (within the wall)
Congenital or acquired defects such as stenosis and Meckel
diverticulum
Inflammatory processes: tuberculosis, Crohn's disease, ulcerative
colitis
Tumors: benign or malignant
Trauma: intraparietal hematoma
Irradiation: thickening of the intestinal wall
Intraluminal obstruction (inside the bowel)
Gallstones: the biliary ileus
Parasites
Phytobezoar: a concretion formed in the stomach or intestine and
composed chiefly of undigested compacted vegetable fiber
Foreign bodies
Fecaloma
375
Extrinsic compression (from outside the bowel)
Adhesions
Abdominal tumors
Retroperitoneal tumors
Surgery (foreign bodies forgot in the abdomen)
2. Occlusions by strangulation, produced by volvulus, intussusception, or
incarceration are considered occlusions with "closed loop" because the both ends of the
loop are closed and the occluded loop cannot evacuated. The vascularization of the
intestine suffers from the beginning leading to early necrosis and perforation.
The onset is sudden with violent pain, vomiting is early and general condition
rapidly declines, the patient becoming shocked. Abdominal distension is achieved from
the beginning and is sensitive to touch, presenting abdominal guarding, even located
muscular contracture. Bowel movements are interrupted from early moments.
Mechanisms of this type of occlusion are:
Volvulus
Torsion of the bowel around its axis
Intussusception (the telescoping of a bowel segment into another)
Clamping by peritoneal straps (adhesions)
Strangulated internal hernias (produced by the intrusion of an intestinal
loop into an opening of the peritoneal cavity or a breach of the mesentery
or mesocolon).
Strangulated external hernias
Topographic classification of occlusions
1. High-level occlusions
The obstacle is located up to the angle of Treitz or up to the first jejunal loop.
Clinical picture is represented by sudden onset of symptoms with rapid
development. Vomiting is early and reduced in quantity leading to fast general status
impairment. Abdominal distension is absent and the intestinal transit can be maintained
in segments below the obstructed area. There is discrepancy between the serious general
status and the poor abdominal signs. These types of occlusions are more frequently
produced by strangulation (volvulus).
2. Low-level occlusions
The obstacle is located mainly on the large intestine.
The onset of symptoms is insidious, with mild pain, vomiting is missing in the
early phases but appears later becoming abundant and fecaloid. There is an intense
abdominal distension. General condition remains good for a long period. These types of
occlusions are commonly produced by obstruction (tumors).
Classification by evolution
1. Acute. The onset is sudden and generally belongs to high occlusion by
strangulation. Acute occlusions evolve quickly to hydromineral imbalances
(through early and massive vomiting). There are early lesions on bowel loops.
2. Subacute (subocclusion or incomplete occlusion). The onset is less rapid and
evolution is slower. Clinical picture is characterized by mild colicky pains, rare
vomiting, abdominal distension and incomplete abolition of bowel movements.
This type is frequently encountered in adhesions.

376
Pathophysiology
Intestinal obstruction produces superjacent digestive segment distension by
accumulation of air from swallowed air, bacterial fermentation processes, diffusion
from blood and accumulation of fluid from digestive secretions and food intake.
Obstruction will induce increased peristaltic contractions that contribute to
increased intraluminal pressure and small-bowel distention causing compression on
parietal lymphatic vessels, leading to bowel wall lymphedema with wall thickening.
Compression on submucous venous network accelerates the edema. Finally, the
continuous increase in bowel wall pressure blocks the arterial vessels, leading to
ischemic necrosis and perforation. This sequence may occur more rapidly in a closed-
loop obstruction with no proximal escape for bowel contents. (Figure 2)

Due to increased intraluminal pressures, intestinal absorption and lymphatic
drainage decrease leading to accumulation of intraluminal fluid. Increased hydrostatic
pressure in the capillary beds results in massive third spacing of fluid and loose of
electrolytes and proteins into the intestinal lumen. Massive third spacing of fluids (or
ghost Randal space) rapidly leads to hypovolemia in addition to the extravascular space
dehydration (interstitial or even intracellular) and consecutively to tissue hypoxia,
metabolic acidosis and functional renal insufficiency. Electrolyte disturbances,
especially hypokalemia will affect the heart leading to rhythm disorders. The shock type
in intestinal occlusion is mainly a hypovolemic one.
The bowel becomes ischemic when capillary blood flow stops, allowing bacteria
to pass into the peritoneum, process known as bacterial translocation. Intestinal stasis
favors proliferation of bacterial flora followed by resorption of endotoxins and
microbial toxic-septic shock.
Ascites is also present in intestinal occlusion contributing to abdominal distension
and conveying of translocated bacteria into the bloodstream, leading to septicemia.
Abdominal distension produced by distended bowels and ascites produces
compression on the inferior vena cava with the consequence of decrease of venous
return to heart and hypovolemia. Compression on the diaphragm reduces expansion of
chest in inspiration, leading to hypoxia and respiratory acidosis.
Hemodynamic, metabolic, respiratory, and renal disorders produced by intestinal
occlusion will lead to hypovolemic shock, which in the absence of any treatment is
lethal.
Symptoms
The main constant symptom is pain, which is violent with sudden onset,
accompanied by pallor and sweating, immobilizing the patient to bed, in occlusions by
strangulation. Pain is less violent, with intermittent exacerbations followed by quiet
periods, in occlusions by obstruction. The free of pain interval for jejunum is 2-5
minutes, 5-20 minutes for ileum and over 20 minutes for colon (the period of peristaltic
waves). Diffuse, continue, ill-defined pain, accompanied by an important abdominal
377
distension is present in paralytic occlusions. The original location of spontaneous pain
can indicate where the obstacle is: where peristalsis dies and starts the pain, there is
the location of obstruction.
Vomiting is less constant than pain. It is important in terms of frequency, quantity
and content. A characteristic of patients suffering from intestinal obstruction is the
intolerance to any food or fluid intake. Nausea, hiccups and eructation (gastric stasis
signs) always accompany vomiting. In high occlusions, vomiting is early, less abundant
but common, and the content is food or bile. It is produced mainly by reflex mechanism.
In low occlusions, vomiting is abundant and repeated at some intervals. It is produced
by stasis and retrograde peristaltic waves. Fecaloid vomiting has a serious prognosis.
Bloody vomiting is a sign of extreme gravity (parietal necrosis).
The stop of intestinal transit for gases and feces defines the occlusion, but there
are situations in which transit is not completely abolished (high-level occlusions) and
sometimes even false diarrhea may appear.
Signs
On inspection, the abdominal distension can be observed in most cases but in
high occlusion, it may be absent leading to errors of diagnosis. In small bowel
occlusions, distension is located particularly around the umbilicus, whereas in large
bowel occlusions, abdominal distension is located predominantly in epigastric region
and flanks (the colic frame).
In strangulation, distension occurs suddenly, is asymmetrical, immobile, elastic
on touch and tympanic on percussion, these features representing the Von Wahl sign. In
sigmoid volvulus, bloating is ovoid, oriented from left iliac fossa toward the right upper
quadrant - the Bayer sign.
In thin patients, the peristaltic waves can be seen through the abdominal wall
progressing toward a fixed point (where the obstacle is located) - the Knig sign.
On palpation, the abdomen is usually supple, elastic, without contracture or signs
of peritonitis. The occurrence of contracture, announces the intestinal loop necrosis with
consecutive peritoneal reaction. A thorough palpation, can detect the tumor, the
"sausage of intussusception (the Boudin sign), successive contraction and relaxation of
the underlying loop (the Besges sign) or points of painful hernia (umbilical, inguinal,
femoral). Unfortunately, hernial regions are often overlooked on physical examination.
Sensitivity located at 2 cm above the umbilicus in the small bowel occlusions represents
the Thevenard sign.
On percussion, excessive sonority (because of intestinal gas distension) and
shifting dullness (the Gangolphe sign of ascites) can be found.
On auscultation, the so-called steam spout sound can be heard being caused by
gases crossing the intestinal narrowed zone - the Knig syndrome. Rapid succession of
Knig syndrome is characteristic for multilevel stenosis - the Keberle syndrome. Due to
hyperperistalsis, bowel sounds are strong, metallic, interrupted by crackling that mimics
the release of an intestinal loop from a stenosis - the Schlange sign. In most cases,
auscultation reveals total silence - "silent abdomen" described by Mondor.
Digital rectal examination may highlight the stenosing rectal tumor or a distended
intestinal loop in the Douglas pouch - the Gold sign.
General signs. In high-level occlusion, the general condition declines faster and
more intensively. In severe forms, skin and mucous membranes are dry, the eyes are
sunken, and the general condition is gradually impaired evolving to death.
378
Investigations
Abdominal plain radiography, for best result, is usually performed in supine
position. The characteristic picture is that of air-fluid level (horizontal fluid level and air
bubble above). Gaseous distension of an intestinal loop appears in the first 3-6 hours
after the clinical onset. If there are no air-fluid levels at 24 h after the onset of clinical
symptoms, the occlusion is usually ruled out, but distended loops and air-fluid levels
may be absent with an obstruction of the upper jejunum or with closed-loop
strangulating obstructions.
In case of small bowel occlusion, air-fluid images are numerous, relatively
small, arranged centrally in the vertical axis and appear as "organ pipes",
"swallow nests" or "stairs. (Figure 3)

In case of large bowel occlusion,
air-fluid images are less
numerous, large in the transverse
axis, arranged peripherally on the
colic frame.
In volvulus of sigmoid colon, the
appearance is typical of "sand
clock" or "coffee bean". (Figure
4)

Abdominal ultrasound is less useful because it cannot
specify the diagnosis with certainty.
Abdominal CT with contrast is useful in some cases when
it can specify the location and cause of intestinal obstructions
especially of tumoral etiology. (Figure 5)
Colonoscopy is useful in diagnosis of obstructing colon
tumors.
Barium enema may be also helpful in some cases of
intussusception or volvulus.
Enteroclysis (Figure 6) is a fluoroscopic X-ray
examination of the small intestine using a radiopaque substance
introduced directly into the duodenum via a catheter. Barium
offers a very good contrast but it cannot be used when there is a suspicion of ischemic
lesion or perforation. Enteroclysis distinguishes adhesions from metastases, tumor
recurrence, and radiation damage.[1]
379

Laparoscopy and laparotomy are methods of diagnosis
as in majority of cases the main treatment is surgical.
Laboratory investigations are useful for evaluating the
general condition of the patient, the metabolic changes,
establishing the therapeutic guidelines and have some
prognostic significance. Usually performed tests are: CBC,
proteinemia, electrolytes, serum urea and creatinine, blood pH
and acid-base balance parameters, alkaline reserve. Keep in
mind that hemoconcentration may mask some electrolyte
disorders.
Positive diagnosis of intestinal occlusion is based on:
intestinal transit stop
abdominal pain
vomiting
abdominal distension
fluid-air levels on abdominal x-ray
Preoperative diagnosis should also specify:
the location of the obstacle (high or low occlusion)
the presence or absence of strangulation (vascular impairment)
the etiology of occlusion (not always possible)
metabolic complications
In many cases of bowel obstruction, the etiologic diagnosis is only suspected in
preoperative period, without being able to specify the certain etiology. Anyway, the
complete acute intestinal occlusion represents a surgical emergency and the patient must
be operated even in the absence of the etiologic diagnosis (the etiology will be clarified
during the operation). In other cases, especially in low-level or incomplete occlusions
there is enough time to perform investigations to find out the cause of occlusion.
First, the differential diagnosis should be performed between different forms of
intestinal obstruction, based on several criteria. (Tables 1 and 2)
Table 1 - Differences between high-level and low-level occlusions
Criteria High-level occlusion Low-level occlusion
Vomiting Occur rapidly after pain Occur late after pain or even are
absent
Intestinal transit
stop
Occurs late (gas and stool emission is still
possible from lower intestinal segments)
Rapid onset (rectal ampulla is
empty)
Dehydration Rapid Late
General condition Rapid altered Remains relatively good for a
long time (1-2 days)
Abdominal
distension
Absent or not very evident and with
central location
Important
Colicky abdominal
pains features
Intermittent at 3-5 minutes interval Intermittent at 5-10 minutes
interval
Rx images aspect Small, numerous, centro-abdominal fluid-
air levels
Large, rare and with peripheral
location fluid-air levels
Age Frequent in young patients Frequent in elderly due to tumors
of the colon
History Crohn disease, TB, laparotomies Colon cancer

380
Table 2 - Differences between occlusion by strangulation and by obstruction
Criteria By strangulation By obstruction
Pain Sudden onset Continuous Insidious onset - colicky
General condition Rapidly altered Slow alteration
Peritonitis Relatively frequent and rapid due
to intestinal necrosis and
perforation
Is possible but in advanced and
complicated cases especially due
to diastatic perforations
Pseudo-occlusive appendicitis in elderly. Signs of paralytic ileus are predominant
and there is a right iliac fossa pain with abdominal guarding and hyperleukocytosis.
Acute cholecystitis (with or without perforation). Patient usually has a history of
previous colicky pains induced by cholecystokinetic foods, with right upper quadrant
pain on palpation and muscular guarding in gallbladder perforation. Biliary stones are
revealed by ultrasound examination.
Acute pancreatitis. Usually there is an intense epigastric pain radiating in
transverse direction (to the left and right upper quadrants) and often to the lower back
region. It is associated with biliary colic or alcohol abuse in most cases. Increased
amylasemia and amylasuria are characteristic and CT examination reveals the pancreas
alterations.
Mesenteric ischemia appears in elderly patients with cardiac rhythm disorders
and manifested by sudden onset, deep presacral pain, pseudo-occlusive syndrome and
false syndrome of internal bleeding.
Perforated peptic ulcer is associated with a previous history of peptic ulcer
disease. The pain is intense as a stab in the epigastrium and on plain abdominal
radiograph, pneumoperitoneum is the pathognomonic sign.
Renal colic, when intense, is associated with reflex ileus, nausea and vomiting.
Lumbar pain radiating to the external genitalia, accompanying urinary signs (hematuria,
dysuria), suggests the diagnosis. Abdominal radiography, urography and ultrasound
specify the diagnosis.
Acute retention of urine manifests with hypogastric pain, no urination and
palpable elastic tumor above the pubis. Ultrasound examination and urinary bladder
catheterization solve the problem.
Acute stomach dilatation is a severe condition manifested by abdominal
epigastric distension, continuous pain, hiccups, vomiting and impaired general status.
On palpation, splashing sound of the stomach (clapotage) can be heard. On abdominal
X-ray, a unique bulky fluid-air level in the left upper quadrant is observed.
Ascites appear most frequently in hepatic decompensation and acute renal failure.
It manifests with diffuse abdominal pain, abdominal distension, vomiting and dyspnea.
History and ultrasound help the diagnosis.
Hysterical and psychogenic syndromes may be accompanied by abdominal
distension. Symptoms disappear when patients attention is distracted during
examination.
Treatment
Intestinal occlusion represents a surgical emergency and all patients should be
admitted to the surgical service, preferably in the intensive care unit. The first measures
are to ensure a good venous access, to relief intestinal distension and to monitor the
patient. Three catheters are applied:
1. Central venous catheter, necessary for fluids and drugs administration and
for central venous pressure measuring
381
2. Nasogastric tube, for emptying the stomach and thus preventing aspiration
and for monitoring the quantity and quality of lost digestive fluids
3. Urinary catheter, for monitoring the urine output
Even though the most efficient method of treatment is the surgical solution,
before any surgical intervention, the patient should be reanimated. Duration of
reanimation depends on hypovolemic shock severity and associated illness, but it cannot
last long especially in upper digestive occlusions and occlusions with "closed intestinal
loop" (strangulation). The reanimation should be short and effective. In low intestinal
occlusion such as those caused by tumors of the colon (without peritonitis) emergency
surgery can be postponed and preoperative investigations and preparation carried out for
a longer period.
For volume rebalancing, which is the most important because in occlusion there is
a hypovolemic shock, the loss of fluids (gastric aspirate, diuresis, vomiting) must be
correctly assessed. Fluids such as 5% dextrose and saline will be administered until the
resumption of normal diuresis (1 ml/minute). If the patient is shocked, administration of
blood or plasma might be necessary.[2] High quantities of fluids will be administered
with caution in elderly because of the risk of cardiac insufficiency and acute pulmonary
edema.
Electrolyte rebalancing is based on administration of electrolyte solutions (NaCl,
KCl, Ringer, saline, etc.). In case of acidosis, serum bicarbonate will be administered.
For alkalosis with hypochloremia caused by abundant vomiting (loss of HCl), 5%
arginine hydrochloride will be used.
Because the patient cannot be fed orally, nutritional rebalancing will be carried
out by parenteral administration of 10% glucose (dextrose) solution buffered with
insulin (one unit/2 g of glucose), amino acid solutions, and solutions of soluble lipids or
combined solutions.
Depending on clinical picture severity, oxygen and antibiotics will be
administered. Associated diseases (heart, lung, liver) will be treated.
Rebalancing will continue until the return of pulse rate and blood pressure as
close to normal values, the resumption of diuresis and improvement of biological
constants. It is required and will be continued postoperatively too.
Surgery is aimed to remove the intestinal obstacle, intestinal content and
peritoneal effusions and to prevent relapses. Specific surgical measures depend on the
etiology of the occlusion, the status of the affected intestines and the general status of
the patient. Generally, surgical measures can be classified as:
1. For the small bowel occlusions
a. Adhesiolysis in case of adhesions
b. Devolvulation in case of volvulus
c. Reduction of internal strangulated hernias
d. Removal by enterotomy of biliary stone or other foreign body
e. Bowel resection (when the loop is not viable)
f. Ileostomy or intestinal bypass (when lesions cannot be removed)
g. Enteroplication (in patients with intense adhesive syndrome or
recurrent occlusions) to prevent further relapses of occlusion
2. For large bowel occlusions
a. In case of volvulus of the sigmoid colon (which is the most common),
the procedure is devolvulation and sigmoidopexy (suturing the loop to
the abdominal wall to prevent recurrences) when the colon is viable. If
382
there are ischemic lesions, segmental resection of the sigmoid colon is
the solution followed by end-to-end anastomosis or by Hatmann's
operation (the distal end is closed and the proximal end is exteriorized
in terminal colostomy). The second stage of the Hartmann's operation
(re-intervention and end-to-end anastomosis) can be performed after a
few months.
b. When occlusion is caused by stenosing colon tumor, there are many
possibilities depending on which side of the colon the tumor is, the
extension of the tumor and the general status of the patient. These
procedures were mentioned in colon pathology chapter. Mainly there
are two types of procedures: those that remove the tumor (with or
without radical intention) and those that do not remove the tumor but
perform an external or internal diversion (palliative surgery).
If patient's general condition is very impaired, seriated operation can be
performed. The first operation is a minimal invasive one being represented in most
cases by external diversion (colostomy or ileostomy), which resolves the occlusion. The
second operation, after patients recovery, is represented by the radical surgery, which is
more extensive than the first one, but the general condition of the patient is significantly
improved.
Prognosis
Intestinal occlusion is a surgical emergency, potentially lethal. Prognosis depends
very much on patient's age, comorbidities and degree of shock. Negative prognostic
factors in the evolution of intestinal occlusion are:
The high-level of obstruction
Occlusion by strangulation
Associated intestinal perforation
Patients history (associated illnesses)
Elapsed time between onset of occlusion and surgery
If untreated, strangulated obstructions cause death in 100% of patients. If surgery
is performed within 36 hours, the mortality rate decreases to 8%. The mortality rate is
25% if the surgery is postponed beyond 36 hours in these patients.[1]
PARTICULAR FORMS OF INTESTINAL OCCLUSION
Intestinal intussusception and small intestine volvulus are described in chapter about
the small intestine pathology.
Colonic volvulus represents approximately 5% of all cases of intestinal obstruction and
10-15% of all large bowel obstructions. The most common site of large bowel torsion is
the sigmoid colon (60-80%), followed by the cecum (15%), transverse colon (3%), and
splenic flexure (2%).[3,4]
Volvulus of the Sigmoid Colon
The mechanism of volvulus is the twisting of colon around its axis. (Figure 7)
Patients with a long and mobile sigmoidian loop are prone to volvulus. The cause of
torsion may be a congenital or acquired postoperative adhesion band (strap) or the
Meckels diverticulum. The presence of a pelvic mass also increases the risk of
developing sigmoid volvulus. The mass displaces the sigmoid colon sufficiently to
383
result in torsion of the mesosigmoid and a resultant
volvulus. Pregnancy and large ovarian tumors may
induce sigmoid volvulus.[5]
The condition is more common in men aged over
50 and a higher incidence is observed in patients with
Parkinson disease, multiple sclerosis, or spinal cord
injury.[3,6-8]
Torsion can be partial or complete (360). The
complete volvulus leads to the development of a closed
loop obstruction. Torsion of the mesosigmoid quickly
leads to ischemic changes of the bowel wall, followed by
rapid necrosis and perforation.
The onset is sudden with violent pain located
mainly in left iliac fossa. On inspection, asymmetric
abdominal distension can be observed. The Von Wahl triad is represented by:
1. Tumor that mimics a balloon
2. Hyper-sonority on percussion
3. Elastic resistance on palpation
Depending on the extent of bowel ischemia or fecal peritonitis, signs of systemic
toxicity may be apparent. Because of the massive abdominal distension, the patient may
have respiratory and cardiovascular function compromised.
On plain abdominal X-ray images, the twisted loop forms two large
compartments with a central double wall ending at the point of the twist, the so-called
"coffee bean" sign. (Figure 4) CT scanning is not often needed, since the plain
radiographic findings are typical for sigmoid volvulus.
Urgent endoscopic detorsion should be considered as the first choice of treatment
with a success rate up to 90% of patients.[9-11] The endoscope is advanced into the
rectum under direct visualization, the rectum is insufflated and occasionally, the
pressure of the air causes detorsion, reducing the volvulus. If detorsion does not occur,
the spiraling rectal mucosa is followed upward to the apex, and a soft rectal tube is
passed up under direct visualization. The tip of the endoscope can also be used to apply
constant pressure at the apex, which can lead to detorsion and decompression.[3]
Unfortunately endoscopic detorsion has a high recurrence rate (>60%)[11] and it should
be followed in most cases by elective definitive surgery. Resection of the redundant
sigmoid colon is the gold standard operation.[12]
Surgical treatment is indicated in the following situations:
In emergency when there are signs and symptoms of intestinal loop
necrosis and / or peritonitis
When detorsion failed at endoscopic approach
When ischemic lesions are found at endoscopy
Procedures are represented by:
Detorsion and colopexy when the sigmoid loop is viable
Hartmanns procedure when there are ischemic lesions and anastomosis is
dangerous due to peritonitis (Figure 8)
Resection followed by colo-rectal anastomosis (hand sewn or stapled)
The overall mortality in recent studies is <5%.[12]
384

Volvulus of the Cecum
Cecal volvulus is much less common than sigmoid volvulus, predominately
affecting women in the sixth decade of life. The facilitating factor is the congenital
incomplete posterior fixation of cecum or ascending colon associated with an abnormal
mobility. Adhesions and appendicitis are favoring factors. Gaseous dilation of sigmoid
colon and cecum following colonoscopy has also been described as a cause of
volvulus.[13]
Cecal volvulus may be organoaxial (cecal or cecocolic volvulus) or
mesentericoaxial (cecal bascule). In organoaxial volvulus, vascular integrity is
commonly affected because of mesenteric torsion, which can lead to gangrene and
perforation. In contrast, in cecal bascule, which occurs when the malfixed cecum folds
anteriorly over the ascending colon, the vascularization is rarely affected, only when the
cecum is very much distended. (Figure 9)

The patient may describe previous episodes of
abdominal pain, distension, and constipation, suggestive of
repeated subclinical episodes of volvulus. After that, a
violent pain installs brutally, located on the right side of the
abdomen. Intestinal transit stops and asymmetrical abdominal
distension appears (Von Wahl triad). The right iliac fossa is
depressed due to the absence of the cecum.
Cecal volvulus produces large and small bowel
obstruction. Radiographic findings reveal a markedly
distended bowel loop extending from the right lower
quadrant upward to the left upper quadrant. (Figure 10) The
385
small bowel is distended, whereas the distal colon is decompressed. A fold like
termination may be observed at the point of obstruction in the ascending colon at
barium enema examination.[14]
In most cases, plain radiograph is insufficient to confirm cecal volvulus.[15]
Endoscopic decompression is successful in rare cases, the most frequent
performed procedure being the right hemicolectomy. Other possible procedure is
devolvulation followed by cecopexy when the cecum wall is viable.
Biliary Ileus
It is produced by the passage of a big biliary stone into the digestive tract through
a biliodigestive fistula (cholecysto-duodenal in most cases). The biliary stone stops
more frequently in the duodenum (Bouveret syndrome), in the Treitz angle, the terminal
ileum or more rarely in the jejunum, producing obstructive occlusion. (Figure 11)
Biliary ileus is a rare condition, being reported in 0.06% of patients with
gallstones.[16]
Symptoms have three distinct phases:
1. Biliary history with colicky pains, fever and jaundice, characteristic for
biliodigestive fistula
2. Free interval
3. Occurrence of occlusive syndrome
with violent abdominal colicky pains
Abdominal X-ray image highlights the fluid-
air levels (sign of occlusion) and sometimes
pneumobilia (the presence of air into the biliary
tree). In most cases, biliary ileus is an
intraoperative surprise.
Treatment is represented by enterotomy,
removal of the stone and enterorraphy (closure of
the intestine by sutures), or segmental bowel
resection. Hepatobiliary region must be explored
and lesions (fistula) treated as necessary
(cholecystectomy and duodenorraphy).[17]
Postoperative Occlusions
One of the most frequent postoperative complications, especially after open
abdominal surgery, is the intestinal occlusion. Depending on when it appears after the
surgery, occlusion can be classified as:
1. Immediate occlusions. Almost all of these occlusions are exclusively
mechanical, produced by breaches in the transverse mesocolon, mesentery, or
omentum. Intestinal loops intrude through these breaches producing an
incarcerated internal hernia. It is very difficult to establish the etiology of these
occlusions before reintervention. In most cases, the confusion is made with acute
gastric dilatation.
2. Early occlusions occur within the first 4-7 days after surgery being caused by
adhesions or internal hernias. Unfortunately, they are burdened by a high rate of
mortality. Symptoms are diminished especially by pain medication, and general
condition of patient alters rapidly.
386
387
3. Late occlusions are always mechanical being produced by adhesions, tumor
recurrences, incisional hernias or morphological bowel changes after
radiotherapy (enteritis radica).
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14. Khan AN. - Cecal Volvulus Imaging. - Medscape Reference, Emedicne http://emedicine.medscape.com/article/364967-
overview
15. Swenson BR, Kwaan MR, Burkart NE, Wang Y, Madoff RD, Rothenberger DA, Melton GB. - Colonic volvulus:
presentation and management in metropolitan Minnesota, United States. - Dis. Colon Rectum. 2012; 55(4):444-449.
16. Rojas-Rojas DJ , Martnez-Ordaz J L, Romero-Hernndez T. - Biliary ileus: 10-year experience. Case series. - Cir. Cir.
2012; 80(3):228-322.
17. Martnez Ramos D, Daroca J os J M, Escrig Sos J , Paiva Coronel G, Alcalde Snchez M, Salvador Sanchs J L. -
Gallstone ileus: management options and results on a series of 40 patients. - Rev. Esp. Enferm. Dig. 2009; 101(2):117-
120, 121-124.

Acute Mesenteric Ischemia
ACUTE MESENTERIC ISCHEMIA

Every organ needs a good arterial supply and venous drainage. If one of this
component is affected the life of that organ, and in most cases of the whole body, will
be in great danger. There are differences between different kinds of organs or tissues
regarding their resistance to ischemia, but the complete stop of blood flow will finally
lead to necrosis.
Arterial supply of intra-abdominal digestive tract is coming from the abdominal
aorta through the celiac trunk, and the superior and inferior mesenteric arteries. The
stomach, the duodenum and the first loop of the jejunum have a very good arterial
supply from multiple sources (celiac trunk and superior mesenteric artery) and that is
the reason why they are rarely affected by ischemic lesions. On the other hand, the
small intestine and the right colon have a single source of arterial supply represented by
the superior mesenteric artery, which may become occluded by various causes leading
to intestinal ischemia and necrosis. The left colon is vascularized mainly by the inferior
mesenteric artery but it also receives arterial blood from rectal arteries. Anastomotic
vascular arcades between different arterial sources are very important in preventing
ischemic lesions. Intestinal lesions can occur as well after venous blood drainage
impairment.
Mesenteric vascular disease includes:
1. Mesenteric arteries stenosis or complete obliteration by emboli, thrombi
or other causes
2. Mesenteric veins thrombosis
3. Extraluminal mesenteric artery obstruction due to aortic aneurysm,
dissecting aneurysm, tumors or compressive adhesion
4. Splanchnic artery aneurysms
5. Mesenteric vascular trauma
Mesenteric arterial obliteration may be:
1. Acute or complete produced by thrombi and emboli, or
2. Gradual or partial produced by atheromatous plaques (obliterative
arterial disease)
Collateral arteries permit a well-tolerated asymptomatic gradual obliteration of
celiac trunk or superior mesenteric artery and acute inferior mesenteric artery
obliteration, while an untreated acute superior mesenteric artery obliteration, leads to
intestinal infarction and death.
Obliteration of venous circulation is usually sudden and complete, and invariably
the result is thrombosis. Partial obliteration of mesenteric venous circulation is usually
the result of extrinsic compression and is asymptomatic.
Obliteration of one of the circulatory system (arterial or venous) induces the
formation of clots (thrombus) in the other.
Clinical differentiation between arterial and venous thrombosis is very difficult.
It is estimated that 15-20% of all mesenteric vascular accidents are caused by
mesenteric venous thrombosis and about 50% by arterial thrombosis. In the remaining
30-35% of cases, intestinal infarction occurs in the absence of clear evidences of arterial
or venous thrombosis.

388
The four major causes of acute mesenteric ischemia and their incidence are:[1]
1. Superior mesenteric artery embolism (40-50%)
2. Thrombosis of superior mesenteric artery (25-30%)
3. Mesenteric venous thrombosis (5-10%)
4. Ischemia without vascular occlusion (15-20%)
Mesenteric arterial embolism is the most common cause of acute mesenteric
ischemia and the superior mesenteric artery is the most frequently affected because of
its acute angle of origin from the abdominal aorta. Generally, the emboli originate from
the heart in persons with atrial fibrillation but other sources are also possible. A big
embolus may stop at the origin of the artery leading to total intestinal infarction
including the right colon. More frequently, a smaller embolus stops in one branch of the
artery distal to the middle colic artery producing segmental intestinal infarction or
extended small intestine and ascending colon infarction. In this case, usually the
transverse colon and the first jejunal loop (10-12 cm) will be unaffected. The transverse
colon receives arterial blood from the inferior mesenteric artery through the left colic
artery whereas the first left jejunal loop receives blood from superior and inferior
pancreaticoduodenal arteries (celiac trunk).
Thrombosis of superior mesenteric artery appears on an atherosclerotic
background. Thrombosis typically occurs at the origin of the artery as a consequence of
the atheromatous plaques which progress to complete obliteration. Localized
atherosclerotic lesions on smaller branches are also possible. Thrombosis is worse than
embolism resulting in infarction of the entire midgut.
Ischemia without vascular occlusion is caused by intense vasospasm of the
superior mesenteric arterial branches as a consequence of hypoperfusion encountered in
shock (cardiogenic, hypovolemic or septic). It is the most deadly type of intestinal
ischemia, because the spasm persists even after correcting the initial cause, probably
mediated by neurohormonal substances (vasopresin, angiotensin).[2,3]
Mesenteric venous thrombosis affects the superior mesenteric vein. Except the
cases caused by external mechanical compression, causes of this condition remain
unknown.
Specific risk factors in producing an acute superior mesenteric artery ischemia
are:
Old age
Atherosclerosis
Pathologic conditions with low cardiac output
Cardiac arrhythmias
Severe cardiac valvulopathy
Recent myocardial infarction
Abdominal malignancies
Pathophysiology
The first consequence of complete or almost complete obstruction of the superior
mesenteric artery is a spasm in the entire mesenteric vascular system, which will worsen
the ischemia.
The mucosa is the most sensitive layer if the digestive tract to ischemia.
Ulcerations and erosions often manifest clinically by gastrointestinal bleeding. Mucosa
lesions can be identified endoscopically. Peristalsis may remain unaltered hours after
the onset of ischemia.
389
As the process progresses, about 6 hours after the
onset, intestinal wall darkens, becomes cyanotic and
then ultimately gangrenous with multiple perforations.
(Figure 1)
Bowel ischemia and infarction by mesenteric
venous obstruction can be easily distinguished, during
operation, from arterial ischemia as the bowel wall is
extensively hemorrhagic and more edematous and the
mesentery is more thickened. (Figure 2) The mesenteric
venous thrombosis is more virulent in evolution and
more difficult to treat than arterial thrombosis.
Septic shock and multiple organ failure is the last
stage, which will lead to death. It occurs because of
bacterial translocation, autoimmune aggression and
generalized peritonitis.
Clinical manifestations
Regardless of etiology, acute mesenteric ischemia
is characterized by constant abdominal pain in discordance with other local signs. The
pain starts suddenly, is intense and diffuse, may be accompanied by vomiting and does
not reduce after pain medication. The presence of occult bleeding in the stool or bloody
diarrhea is a late manifestation indicating the presence of mucosal necrosis, and signs of
peritoneal irritation mark the presence of complete infarction of the bowel wall.
Patients history (atrial fibrillation, atherosclerosis, heart infarction, strokes etc.)
is very important in presuming the diagnosis. Premonitory sign of an acute mesenteric
ischemia is the abdominal (intestinal) angina, which is a postprandial abdominal pain
that occurs at a fixed time after eating caused by an inadequate mesenteric blood supply.
It is similar to angina pectoris due to obstruction of the coronary artery.
Investigations
There are no specific laboratory tests for intestinal ischemia, so the diagnosis is
more one of presumption based on clinical criteria. Usually findings are: leukocytosis,
hemoconcentration, metabolic acidosis with increased serum lactate. Increase in serum
amylase and creatine kinase shows wall necrosis and is usually a late sign. Alpha
glutathione s-transferase (alpha-GST) and intestinal fatty acid-binding protein (I-FABP)
levels are elevated in most cases.
Plain abdominal radiography usually is not very helpful in diagnosis but is
performed always as a standard examination in acute abdomen. Suggestive signs could
be: distended intestinal loops, absence of colon haustres due to submucous edema,
bowel wall thickening (in venous thrombosis) and/or the presence of air in the intestines
(in advanced stages of disease). Making a barium-passage is contraindicated because it
provides little information and interferes with the angiographic images.[4]
Selective arteriography of superior mesenteric artery remains the only conclusive
exploration in patients suspected of mesenteric ischemia. (Figure 3) Emergency
selective mesenteric arteriography and medication by direct infusion therapy is the basis
for aggressive acute mesenteric ischemia diagnosis and treatment, introduced by Boley
and colleagues since the early '80s.[5] Arteriography almost always is able to distinguish
between embolism or thrombosis, which is of paramount importance in choosing the
type of surgery. It can also diagnose mesenteric ischemia without vascular occlusion,
which is suitable for medical treatment.
390
CT with three-dimensional
reconstruction offers information about
mesenteric vessels and bowel
morphology being a commonly
requested investigation in patients with
acute abdominal pain. It is the
investigation of choice in suspected
mesenteric venous thrombosis.[6]
Acute pancreatitis and intestinal
obstruction by strangulation can pose problems of differential diagnosis with acute
mesenteric ischemia. Increased levels of amylasemia associated with increased
pancreatic volume and modified structure visible on CT images help the diagnosis of
acute pancreatitis. Differential diagnosis with occlusion by strangulation is less
important, because both conditions require surgery. In most cases, even experienced
surgeons cannot make an early diagnosis of intestinal ischemia based only on clinical
picture.
Treatment
The main goal of acute mesenteric ischemia treatment is to restore the
discontinued blood flow as quickly as possible.
The first therapeutic measures are aggressive, represented by hemodynamic
monitoring, correction of acidosis, broad-spectrum antibiotics and bowel decompression
by gastric aspiration.
Anticoagulants should be given to prevent the formation or extension of
thrombosis in all patients without active bleeding and postoperative.
Emergency surgery is indicated in patients with suspected intestinal perforation or
gangrene. If at intra-operative exploration, intestinal necrosis is generalized,
unfortunately nothing can be done. In limited bowel necrosis, intestinal resections are
more or less extensive (segmental enterectomy associated with right hemicolectomy - if
the right colon is also affected followed by jejuno-transversostomy). To allow survival,
at least 50 cm of viable intestine (preferably 100) are needed. Assessing the intestinal
viability is made by palpation of mesenteric pulse, and in case of its absence, mesenteric
revascularization will be tried before resection.
In patients whose diagnosis is unclear, making angiography is imperative to
specify the diagnosis and for possible therapeutic intervention.
Angiography allows the initiation of treatment by direct intra-arterial infusion of
vasodilators and thrombolytic agents, but also performing endoscopic maneuvers such
as angioplasty, stenting or embolectomy.
As a rule, intra-arterial papaverine administration is indicated in all forms of
mesenteric ischemia because vasoconstriction accompanies all forms of ischemia and
persists several hours after resumption of blood flow. Papaverine may be administered
intra-arterial 5 days.
Treatment of mesenteric arterial embolism
The classical methods of treatment in case of superior mesenteric artery embolism
are laparotomy and embolectomy. Making an embolectomy involves performing a distal
arteriotomy, retrograde introduction of the catheter (Fogarty catheter) above the
embolus, inflation of the balloon and carefully extracting the emboli by catheter
391
retraction. (Figure 4) Then intestines are examined to assess viability and areas of
necrosis are removed. Intraoperative Doppler ultrasound can be performed to identify
persistent ischemia.
Postoperative administration of papaverine can reduce vascular spasm.
Sometimes reintervention is required after 24-48 hours for resection of ischemic
segments of the bowel.
Prevention of new embolic episode is performed by early mobilization, treating
the condition generating the emboli and administration of anticoagulants.
Treatment of mesenteric arterial thrombosis
Treatment is mainly surgical. Thrombectomy (removing the thrombus) alone is
not effective for long term because of atherosclerosis process responsible for producing
thrombosis, thus thrombectomy is accompanied by mesenteric revascularization
procedures (stenting, bypass grafting,
superior mesenteric artery reimplantation
into aorta, etc.) and resection of necrotic
bowel segments. (Figure 4)
Superior mesenteric artery
reconstruction is a procedure with success
rates of 77-79% for long term, despite the
immediate postoperative mortality of 50-
60%.[7-9]
Conservative treatment in such cases
is to keep the patient under observation and
under the protection of anticoagulants.
Treatment of mesenteric venous thrombosis
Standard treatment in these cases is the administration of heparin and resection of
necrotic bowel segments.
Heparin is administered even in patients with gastrointestinal bleeding if intestinal
infarction risk is higher than that of bleeding. In patients with angiographically proven
sufficient collateral mesenteric flow, treatment may be conservative (heparinization and
monitoring).
Administration of papaverine is indicated in cases of subsequent venous
thrombosis due to coexisting arterial spasm.
Laparotomy for bowel viability assessment is also recommended.
Venous thrombosis prophylaxis with anticoagulants (wafarin) is indicated for at
least 6 months.
Thrombolysis with streptokinase or urokinase administration through the catheter
was successfully applied in a small number of cases.[10,11]
Treatment of mesenteric ischemia without vascular obstruction
Initial treatment consists in administration of papaverine through the angiography
catheter left in place and treatment of causes that produced vasoconstriction.
In patients without peritoneal signs, angiography is repeated after 24 hours to
assess vasoconstriction. Administration of heparin is indicated to prevent catheter
endovascular thrombosis.
Laparotomy is performed only in patients with peritoneal signs, but papaverine is
continued postoperatively.
392
393
Prophylaxis in such cases is aspirin.
Prognosis
Mesenteric vascular obstruction is often fatal because of delays in diagnosis and
treatment, rapidly extension of infarction to large territories and difficulty of intestinal
revascularization.
The prognosis after acute mesenteric venous thrombosis is better than that
following acute arterial mesenteric ischemia; the prognosis after mesenteric arterial
embolism is better than that after arterial thrombosis or non-occlusive ischemia.[12]
The overall death rate is about 60%.[13] For infarction affecting over half of the
length of the intestine and the colon, it increases to 85%.[14] The mortality rate
following surgical treatment of arterial embolism and venous thrombosis (54.1% and
32.1% respectively) is less than that after surgery for arterial thrombosis and non-
occlusive ischemia (77.4% and 72.7 % respectively).[12]
Arterial reconstruction is often technically impossible and when is performed, is
not feasible for long term. Timely revascularization in patients who are symptomatic
with chronic mesenteric ischemia should be considered to decrease the high mortality
rate.[15]
Mesenteric venous thrombosis has a mortality rate of about 30% and in the
absence of long-term therapy with anticoagulants, about 25% of those who survive
develop a recurrent thrombotic episode and could lead to portal hypertension.[16]
References
1. Assar AN, Zarins CK. - Acute mesenteric ischaemia: facts and perspectives. - British J ournal of Hospital Medicine,
2008; 69(12):634-640.
2. Bassiouny HS, Desai TR. - Diagnosis and treatment of nonocclusive mesenteric ischemia. - In: Rutherford RB, ed.
Vascular Surgery. 6th edn. 2005, Elsevier, Philadelphia: 172831.
3. Yasuhara H. - Acute mesenteric ischemia: the challenge of gastroenterology. - Surg. Today 2005; 35(3):185-195.
4. Oldenburg WA, Lau LL, Rodenberg TJ , Edmonds HJ , Burger CD. - Acute mesenteric ischemia: a clinical review. -
Arch. Intern. Med. 2004; 164(10):10541062.
5. Boley SJ , Sprayregan S, Siegelman SS, et al. - Initial results froman aggressive roentgenological and surgical approach
to acute mesenteric ischaemia. - Surgery 1977; 82:848-855.
6. Kumar S, Sarr MG, Kamath PS. - Mesenteric venous thrombosis. - N. Engl. J . Med. 2001; 345(23):16831688.
7. Kieny R, Batellier J , Kretz J G. - Aortic reimplantation of the superior mesenteric artery for atherosclerotic lesions of the
visceral arteries: sixty cases. - Ann. Vasc. Surg. 1990; 4(2):122-125.
8. Cormier J M, Fichelle J M, Vennin J , Laurian C, Gigou F. - Atherosclerotic occlusive disease of the superior mesenteric
artery: late results of reconstructive surgery. - Ann. Vasc. Surg. 1991; 5(6):510-518.
9. Aouini F, Bouhaffa A, Baazaoui J , Khelifi S, Ben Maamer A, Houas N, Cherif A.- Acute mesenteric ischemia: study of
predictive factors of mortality. - Tunis Med. 2012; 90(7):533-536.
10. Shanley CJ , Weinberger J B. - Acute abdominal vascular emergencies. - Med. Clin. North Am. 2008; 92(3):627647.
11. Schoots IG, Levi MM, Reekers J A, Lameris J S, van Gulik TM. - Thrombolytic therapy for acute superior mesenteric
artery occlusion. - J . Vasc. Interv. Radiol. 2005; 16(3):317-329.
12. Schoots IG, Koffeman GI, Legemate DA, Levi M, Van Gulik TM. - Systematic review of survival after acute mesenteric
ischaemia according to disease aetiology. - Br. J . Surg. 2004; 91(1):1727.
13. Kassahun WT, Schulz T, Richter O, Hauss J . - Unchanged high mortality rates fromacute occlusive intestinal ischemia:
six years review. - Langenbecks Arch Surg. 2008; 393(2):163-171.
14. Aliosmanoglu I, Gul M, Kapan M, Arikanoglu Z, Taskesen F, Basol O, Aldemir M. - Risk factors effecting mortality in
acute mesenteric ischemia and mortality rates: a single center experience. - Int. Surg. 2013; 98(1):76-81.
15. Park WM, Gloviczki P, Cherry KJ J r, et al. - Contemporary management of acute mesenteric ischemia: Factors
associated with survival. - J . Vasc. Surg. 2002; 35(3):445-452.
16. Harnik IG, Brandt LJ . - Mesenteric venous thrombosis. - Vasc. Med. 2010; 15(5):407-418.

Upper Gastrointestinal Bleeding
UPPER GASTROINTESTINAL BLEEDING

Acute gastrointestinal bleeding represents a major emergency associated with
substantial mortality.
Definitions
Upper gastrointestinal bleeding (UGIB) represents the bleeding from a source
proximal to the ligament of Treitz.
Hematemesis represents the bleeding exteriorized through vomiting. The blood is
fresh of arterial or venous source.
Melanemesis represents vomiting of black blood with aspect of used coffee
grounds. It appears when the blood spent more time in the stomach (hemorrhage
has reduced or stopped) and the hemoglobin was converted to hematin (of brown
color) under the action of gastric acid.
Hematochezia represents fresh blood passage through the rectum. Usually indicates
a bleeding from the lower part of the digestive tract, but may be as well the result of
increased amounts of upper gastrointestinal bleeding associated with a rapid
intestinal passage of blood (less than 14 hours).
Melena represents eliminating black stools, typically indicating a higher
gastrointestinal source of bleeding, but bleeding source can be as well the small
intestine or the colon. To produce melena, 150-200 milliliters of blood are required,
which is transformed under the action of digestive juices and intestinal bacteria.
Melena may continue several days after bleeding has stopped. Note, that black stool
with negative occult bleeding tests, may be due to ingestion of iron, bismuth and a
variety of foods.
Massive gastrointestinal bleeding is considered when bleeding is accompanied by
shock, or orthostatic hypotension; the decrease in hematocrit is of 6-8%, or
minimum two units of blood transfusion are required. Alternative definitions that
may be more helpful in acute situations include:[1-3]
o An ongoing transfusion requirement in an adult of more than 150 ml per
minute
o Replacement of more than 50% of blood volume in 3 hours or less
o Replacement of one blood volume, or transfusion of 10 units or more of red
cells in a 24 hour period
Occult bleeding represents the loss of small quantities of blood (less than 50 ml) by
stool, as evidenced by fecal occult blood tests (Hemocult, Hemoquant).
Incidence and mortality
For the U.S. it is estimated an incidence of 165 per 100,000 inhabitants per
year.[4] Peptic ulcer disease is responsible for more then half of all bleeding episodes.[5]
For Europe, the incidence is 48-145 per 100,000 inhabitants per year.[6-8]
Mortality after digestive bleeding remained almost unchanged over the past 25
years, being 8-14%, but in severe bleedings it may raise up to 25-50%.[8-10]
Almost two thirds of bleedings are due to acid-peptic disease (peptic ulcer,
gastritis, esophagitis), 10-15% secondary to portal hypertension (esophageal and gastric
394
395
varices) and the remaining have mixed causes (neoplasms, Mallory-Weiss syndrome,
etc).[7]
Etiology of upper gastrointestinal bleeding
Peptic ulcer
Idiopathic
Induced
Aspirin
NSAIDS
Infectious
Helicobacter pylori
Cytomegalovirus b.
Herpes simplex virus
Stress ulcer
Zollinger Ellison syndrome
Esophagitis
Peptic
Infectious
o Candida albicans
o Herpes simplex virus
o Cytomegalovirus
o Other
Induced
o Alendronate
o Tetracycline
o Qinidin
o Potassium chloride
o Aspirin
o NSAIDS
Portal hypertension
Esophageal varices
Gastric varices
Duodenal varices
Portal hypertension gastropathy
Arterial or venous malformations
Idiopathic angioma
Rendu-Osler-Weber syndrome
Dieulafoy lesions
Antral vascular ectasia (watermelon
stomach)
Radiation induced teleangiectasia
"Blue rubber bleb nevus syndrome
(association between cavernous
hemangiomas of the skin and similar
lesions in the GI tract.)
Traumatic or postoperative
Mallory-Weiss syndrome
Ingested foreign bodies
Surgical anastomosis
Enteric arterial fistula
Tumors
Benign
o Leiomyomas
o Lipomas
o Polyps (hyperplasic,
adenomatous, hamartomatos)
Malignant
o Adenocarcinoma
o Leiomyosarcoma
o Lymphoma
o Kaposi's sarcoma
o Carcinoid
o Melanoma
o Metastases
Others
Hemobilia
Haemosuccus pancreaticus
Highlighting bleeding
Medical history, color and amount of gastric aspiration or vomit, or the aspect of
stools are suggestive and provide important data. A positive gastric aspirate confirms
the upper gastrointestinal bleeding, but a negative aspirate does not exclude a source of
bleeding.
Quantitative assessment of bleeding
Assessment of bleeding and replacing lost blood is the most important aspects of
the therapy of patients with gastrointestinal bleeding. Usually, blood loss is
underestimated.[2] Assessment of blood loss requires an accurate assessment of vital
signs, central venous pressure, hemoglobin and hematocrit and a degree of clinical
experience.
Indicators of a massive hemorrhage are:
Resting tachycardia (100 beats /min)
In standing position heart rate increases by more than 20 beats/min, the
systolic pressure decreases by more than 20 mmHg, and diastolic pressure
decreases by more than 10 mmHg.
Acidosis
Azotemia (blood urea increased by over 40 mg/100 ml without pre-
existing renal disease)
Transfusion requirements of more than one unit at 8 hours or six units in
total (10 units or more of red cells in a 24 hour period)
Hematochezia from upper gastrointestinal source
Unable to clarify the fresh red blood in gastric lavage
Continued bleeding or rebleeding during endoscopy
An increased risk is when there are met more criteria:[11,12]
Age over 60 years
Significant comorbidities
Coagulopathy
Hemodynamic instability
Signs of active bleeding
These patients will be admitted in ICU and subjected to emergency endoscopic
diagnostic and therapeutic maneuvers. Surgical and interventional radiology services
will be alerted for possible evaluation. About 25% of patients hospitalized in ICU are
low risk patients and can be treated in other hospital departments (gastroenterology,
surgery).
In order to standardize and improve care, various scoring systems (Rockall,
Blatchford and Baylor) have been developed to identify those individuals at high risk
requiring treatment (transfusion, endoscopic or surgical intervention) or at risk of
rebleeding and death.[10]
Rockall score, [13] (Table 1) based on clinical and endoscopic criteria in patients
with a history of UGIB, provides predictability of recurrence and severity of bleeding.
Table 1 - Rockall Severity Score
Score
The variable
0 1 2 3
Age <60 60-79 >80
P Without shock
BP > 100 mmHg
Pulse 100
BP 100 mmHg
Pulse 100
BP 100 mmHg
Co-morbidity Without major
comorbidities
Renal impairment
Ischemic cardiopathy
Digestive cancer
Any other major co-
morbidity
Renal
failure
Liver
failure
Metastases
Diagnosis Mallory-Weiss
syndrome
Without
highlighted lesions
and without
massive bleeding
Any other
diagnostics

Major signs of
recent
hemorrhage
at endoscopy
Without active
bleeding or just a
dark spot
Blood into the
digestive tract,
adherent thrombus,
visible bleeding vessel

Patients with scores of up to 2 have a relapse rate of 5.3% and mortality of 0.2%.
They will be monitored for a short time and will be discharged early after endoscopy
with the recommendation to continue treatment at home.[14]
396
Patients with Rockall score 3 are likely to evolve unfavorably (rebleeding in 21%,
surgery in 5% and death in 10% of cases) and they require bleeding relapse
prevention.[14] Hemorrhagic risk predictability allows the selection of cases that are
suitable for interventional endoscopic treatment and those requiring continuous
surveillance in ICU.
Another system of classification is the endoscopic Forrest classification, useful in
selecting patients for endoscopic treatment or surgery. (Table 2) [15,16]
Table 2 - Forrest classification system with respective prognosis
Forrest Classification Rebleeding
incidence
Surgical
requirement
Incidence
of
death
Type I: Active bleed
Ia: Spurting hemorrhage
Ib: Oozing hemorrhage
55-100% 35% 11%
40-50% 34% 11% Type II: Signs of recent hemorrhage
Ila: Non-bleeding visible vessel
Ilb: Adherent clot
IIc: Hematin on ulcer base
20-30% 10% 7%
10% 6% 3% Type III: Lesion without signs of recent bleeding
Flat spot
Clean base
5% 0.5% 2%
Ulcers located on the lesser curvature of the stomach and duodenum and those
located on the posterior wall are more likely to produce an UGIB due to the rich
vascularization of those zones.
Resuscitation protocol for UGIB
Adequate venous access
o Two large-bore intravenous catheter
o Central vein catheterization if necessary
o In case of myocardial infarction or severe ischemia, pulmonary artery
catheter
Bladder catheter
Hydroelectrolytic rebalancing
Oxygen
Monitoring:
o Blood pressure and its changes in standing position (invasive monitoring)
o Pulse
o Hematocrit
o Flow of urine
o Central venous pressure or cardiac output
Transfusion of blood and derivatives
Gastroenterology consult
Radiological or other investigations
Establishing the source of bleeding
The presence of blood in the gastric aspirate after lavage, indicates an UGIB. If
gastric aspirate is negative, an upper gastrointestinal endoscopic exploration could rule
out the superior digestive source of bleeding in most cases. Anyway, endoscopic
exploration should be performed in cases with positive gastric lavage.
Intense bowel sounds indicate a superior source of bleeding. The pain usually
indicates a peptic ulcer and vomiting before bleeding suggests Mallory-Weiss
syndrome.
397
If the patient has experienced a hemorrhage from a known source, there is a high
chance to rebleed from the same source.
Particular attention is given to self-administration of drugs, because there is a
significant relationship between severe bleeding and use of aspirin or other drugs.
Physical examination may reveal an injury, bleeding diathesis or stigmata of
chronic liver disease.
Intensive care of patient with severe gastrointestinal bleeding
The main therapeutic goals of intensive care are the maintenance of tissue
perfusion and oxygenation by restoration of blood volume and arrest of bleeding.[3]
Management of severe gastrointestinal bleeding:[2]
Assessment of onset and severity of bleeding
Resuscitation and stabilization
o Provide adequate ventilation and oxygenation
o Restore the circulating volume. A rough guideline for the total amount of
crystalloid fluid volume needed to correct the hypovolemia is the 3-for-1
rule. Replace each milliliter of blood loss with 3 milliliter of crystalloid
fluid.[17]
o Start blood component therapy:
Early transfusion of red cells will be required
Correction of coagulopathy by appropriate blood component
therapy
o Maintain or restore normothermia
o Evaluate the therapeutic response
Diagnostic endoscopy
Therapeutic endoscopy - aims:
o control the source of active bleeding or high-risk lesions
o minimize complications related to endoscopic treatment
o treatment of persistent or recurrent bleeding
Physical examination, history and gastric lavage will be performed
simultaneously with the initiation of resuscitation.
One or two intravenous catheters with wide lumen (14-16 G) will be installed and
blood samples will be harvested for laboratory tests (Htc, Hgb, prothrombin time,
partial thromboplastin time, chemistry analysis, blood group and Rh).
Saline will be rapidly infused to maintain systolic pressure, above 100 mmHg and
pulse under 100/min.
Patients will be transfused with packed red blood cells, platelets and plasma
cryoprecipitate necessary to maintain Htc over 24%, platelets over 50,000 and
prothrombin time less than 15 ".
The surgeon and the gastroenterologist will examine the patient as soon as
possible to establish the diagnosis and to indicate the appropriate therapy.
Criteria for a favorable prognosis: [18]
Age under 75 years
Absence of concomitant unstable diseases
Absence of ascites
Normal prothrombin time
Systolic pressure over 100 mmHg at one hour after admission
Gastric lavage without fresh blood at one hour after admission
398
Gastric lavage is used for three proposes: diagnosis, preparation for endoscopy
and hemostasis.
The introduction of nasogastric tube should be gentle to prevent the damage of
nasal or digestive mucosa.
Some authors contraindicate the introduction nasogastric tube in cases of
suspected esophageal varices due to the risk of damage them and therefore bleeding.
However, esophageal varices are only a relative contraindication because the atraumatic
probe can be inserted gently in these cases also, without causing damage to the
esophageal varices.[19]
Absolute contraindications for nasogastric intubation are:[19]
o Severe midface trauma because there is high risk of malpositioning and
false passage
o Recent nasal surgery
Relative contraindications are: [19,20]
o Coagulation abnormality
o Esophageal varices or stricture
o Recent banding of esophageal varices
o Fractured base of skull
o Alkaline ingestion
The presence of blood in the gastric lavage fluid demonstrates the UGIB. Blood
characteristics can give us information about the source of bleeding. If blood is bright
red, then very probable, the source is a peptic ulcer or gastritis. The darker, venous,
aspect of the blood indicates a source of bleeding from esophageal or gastric varices.
Nasogastric tube allows also the evaluation of the amount of blood that is lost.
The main purposes of gastric lavage are gastric emptying and stop of bleeding.
The first measure in stopping the bleeding from a peptic ulcer is the evacuation of blood
from the stomach. A stomach full of blood will still bleed. This maneuver also prevents
the passage of the blood into the intestines and thus prevents the increase of azotemia
due to blood digestion. In reducing azotemia, enema is frequently used to evacuate the
bowel blood content.
Gastric lavage may be performed with tap water (in most cases) or saline.[21] Iced
cold water is used for its vasoconstrictor effect and because cold may reduce peptic acid
secretion [22,23], although there are studies that deny the hemostatic effect of cold water
[24-26] or saline. In addition, the lavage fluid may contain vasoconstrictor
(Adrenostazin), astringent and alkalizing agents. Lavage is repeated until the returning
fluid shows no further gastric content. In most cases, bleeding caused by peptic ulcers or
anti-inflammatory drugs can be stopped by this procedure after using about 2 liters of
lavage fluid. Nasogastric tube will remain in place because rebleeding is always
possible. If after gastric lavage bleeding does not stop, endoscopic hemostasis or other
surgical methods are required.

399
Bleeding from gastroduodenal ulcers
Gastroduodenal ulcers are the leading cause of upper gastrointestinal bleedings
(50-75% of all cases).[27-30] Bleeding may come either from small vessels of
inflammatory granulation tissue of the ulcer or from a larger vessel, in the bottom of
ulcerous crater, as a result of its wall erosion. Usually the posterior duodenal ulcers,
penetrating the gastroduodenal (pancreaticoduodenal) artery are the cause of massive
bleeding.
Based on endoscopic aspect (Table 2 - Forrest classification) ulcers are more or
less likely to rebleed. After recent arrested hemorrhage, rebleeding has the highest
incidence (40-50%), and the highest mortality, when a vessel is visible on the bottom of
the ulcer.
Patients at low risk for recurrent UGIB (no clinical evidence of severe
hemorrhage and a clean ulcer base documented by endoscopy) may be safely discharged
from hospital in the same day as endoscopy and can be fed within 24 hours.[31] Early
discharge and return to work can significantly reduce both direct costs (hospitalization
cost) and indirect costs (days lost from work).[29,32,33]
The following factors are known as a poor prognosis of bleeding from gastro-
duodenal ulcers associated with a high morbidity and mortality:[34-36]
Age over 60 years
Coexisting medical illness
Shock or orthostatic hypotension
Coagulopathy
Onset of the hemorrhage in the hospital
Need for blood transfusion
Evidence of fresh blood in the gastric aspirate
High location of the gastric ulcer on the small curvature (adjacent to the left
gastric artery)
Posterior duodenal ulcer (adjacent to the gastro-duodenal artery)
Endoscopic highlighted arterial type of bleeding or visible vessel on the
ulcers base
In cases with acute bleeding, a proton pump inhibitor (PPI) is administered
immediately after endoscopic treatment. For example, Pantoprazole 80 mg in iv bolus
followed by an infusion of 8 mg per hour. If rebleeding does not occur within 24 hours,
the patient will receive Pantoprazole 40 mg/day or Omeprazole 20 mg/day.[37-39] H2-
receptor antagonists are not recommended in the management of patients with acute
upper GI bleeding.[40]
Somatostatin and its retard analogue Octreotide have the advantage of reducing
the splanchnic blood flow, inhibit gastric acid secretion and have cytoprotective effects.
Somatostatin can be used as adjuvant therapy before endoscopy in doses of 250
micrograms in bolus, followed by hourly administration of the same dose for 3-7 days.
Octreotide dose is 50-100 micrograms in bolus followed by 25 micrograms per hour for
3 days.[41-44] Somatostatin and Octreotide are not routinely recommended for patients
with acute ulcer bleeding.[40]
Endoscopic therapy
Endoscopic hemostasis is the most effective non-surgical treatment of bleeding
ulcers. Patients receiving endoscopic hemostasis are those who are at high risk of
400
rebleeding and those where endoscopy shows active arterial bleeding, a visible vessel or
adherent clot.
Endoscopic hemostasis aim is to coagulate the eroded vessels. This can be
achieved by various methods:
Using thermal devices such as contact probe (monopolar, bipolar or multipolar
electrocoagulation and thermocoagulation) or laser photocoagulation devices
(yttrium-aluminum-granat laser-NEODYN, Argon laser). These methods are
effective in sealing arteries up to 2 mm diameter, usually found in bleeding
ulcers. Rebleeding occurs in 2-10% of cases.[45,46]
Chemical hemostasis is achieved by injection in and around the bleeding spot of
chemicals such as epinephrine (0.5 to 1 ml in concentration of 1:10.000) that
produces vasoconstriction, Polidocanol (produces sclerosis), or alcohol (98%
concentration, produces coagulation). It is an effective and inexpensive method
but failure is possible in about 20% of cases, especially related to shock on
admission, spurt bleeding, posterior located duodenal ulcer and anastomotic
ulcer.[47]
Mechanical hemostasis by application of hemoclips (first introduced by Hayashi
in Japan) on bleeding vessel has become popular in recent years. The advantage
of the method is that it does not produce tissue damage and the risk of
perforation is reduced. Rebleeding risk is 2-20%.[48,49]
A relatively new method, with very good results is the endoscopic application of
fibrin glue.[50,51]
A second-look endoscopy is recommended only selectively (not by routine) after
endoscopic hemostasis in cases where visualization during the initial endoscopy was
obscured by blood or debris, or if there is concern on the part of the endoscopist that the
ulcer is likely to rebleed.[32,52,53] In cases of rebleeding, a second attempt at endoscopic
therapy is generally recommended.[40]
Surgical therapy
Absolute indications of surgical therapy are:[32]
Failure of endoscopic therapy
Sustained hemodynamic instability despite resuscitation (more than three
units of blood transfused)
Recurrent bleeding after initial stabilization (up to two attempts of
endoscopic hemostasis)
Shock associated with recurrent bleeding
Reduced bleeding but persistent, requiring more than three units of blood
per day
Relative indications of surgical treatment are:
A rare blood group
Refusal of transfusion
Emergency patient presenting shock
Advanced age
Severe associated disease
Chronic peptic ulcer known as the origin of bleeding
These criteria also apply to elderly patients who do not bear prolonged
resuscitation, large amounts of transfusions and prolonged hypotension.
401
Surgical procedures in emergencies should be limited to safe hemostasis. The
addition of acid-reduction surgery may be unnecessary as a result of the increasing
utilization of proton pump inhibitors.[54]
Emergency surgical options in emergency are:
Gastrotomy + suturing the bleeding vessel (in most cases located on the
posterior duodenal wall) + gastrorraphy. It is the easiest way to stop the
bleeding, but since the ulcer remains in place, bleeding can recur anytime.
Excision of bleeding gastric ulcer + suture of the stomach (gastrorraphy). It can
be an easy procedure when ulcer is easily accessible, but it can be difficult or
even impossible when the ulcer is located on the posterior wall or under the
cardia.
Excision of the anterior duodenal ulcer (pylorectomy) + pyloroplasty (a gastric
drainage procedure) + truncal vagotomy (decreases the acid secretion).
Bulbantrectomy (removal of the antrum and duodenum bulb with the ulcer) +
truncal vagotomy + gastro-duodenostomy (Pean procedure) - it seems to be the
best choice in duodenal ulcers and Johnsons type III of gastric ulcer.[55]
Vagotomy reduces the neurogenic stimulated acid secretion and antrectomy
reduces the secretion of gastrin.
In case of bleeding gastric ulcers Johnson's type II the procedure of choice is
gastric 2/3 resection, including the ulcer + restoration of digestive continuity by
gastro-duodenostomy (Pean) or gastro-jejunostomy (Bilroth II procedures).
In case of gastric ulcer Johnson's type I (under the cardia), the easiest and fastest
solution is gastrotomy, visualization of bleeding source, hemostasis by suture
and gastrorraphy. Another possibility is the superior gastric resection with eso-
gastrostomy and pylorotomy or pyloroplasty.
In bleeding gastric ulcer, the main problem is the differential diagnosis between
the benign and malignant ulcer. Whenever possible, a biopsy specimen should be
harvested before operation. If surgery has to be performed in emergency condition, two
solutions are available depending on patient's condition:
1. In unstable patients the procedure steps are: anterior gastrotomy + hemostatic
suture + biopsy + gastrorraphy. Then, if the histopathologic diagnosis is cancer,
the patient should be reoperated (respecting the oncological principles) when its
general condition permits.
2. In stable patients, who can bear a more complex operation, the ulcer should be
considered as malignant and resection whenever possible should be performed
with curative intention. Another possibility is to send a specimen of ulcer tissue
to frozen sections examination and wait for the result, and then continuing the
operation accordingly to histopathological findings (simple resection or
oncological resection).
All patients with upper GI bleeding should be tested for Helicobacter pylori and
receive eradication therapy if infection is present.[40]

402
Bleeding from esophageal varices
Bleeding from esophageal varices is one of the most serious, having a mortality
rate of at least 30% during the first hospitalization, and approximately 60% after one
year.[56] The risk of rebleeding is 70% and every rebleeding worsens the prognosis.[57-
59]
Four major issues are important in the prevention of morbidity and mortality and
for treatment in case of esophageal varices:
1. Prediction of patients at risk
2. Primary prophylaxis against bleeding from varices in patients with cirrhosis
3. Treatment of active bleeding
4. Prevent rebleeding
Current definitions:
Time zero is the time of admission to a medical facility.
Clinically significant bleeding is defined as a necessary of blood transfusion
of two or more units within 24 hours from time zero, together with a systolic
blood pressure below 100 mmHg.
Acute bleeding episode is the event occurring within 48 hours from time
zero. Bleeding in this time interval is considered a treatment failure.
Portal hypertension is defined as portal pressure gradient exceeding 5 mm Hg.[59]
Esophageal varices develop when the gradient pressure between the portal and hepatic
vein is greater than 10 mmHg and bleed only when the gradient exceeds 12 mm
Hg.[60,61]
Many clinical and physiological factors are useful in predicting bleeding from
varices in cirrhotic patients, such as:[59]
Location of varicose veins: distal esophagus, stomach
The size of varicose veins: varices occupying more than 1/3 of the
esophageal lumen
The endoscopic appearance of varicose veins: "red marks" (red streaking or
spotting)
The degree of hepatic dysfunction according to Child-Pugh classification
Bleeding in patients history
The presence of ascites
The pressure in varices. The incidence of bleeding according to the pressure
is:[62]
13 mmHg - 0%;
14 mmHg - 9%;
15 mmHg - 17%;
16 mmHg - 50%;
16 mmHg - 72%.
Therapeutic options in bleeding varices (see also the esophageal varices in chapter:
surgical pathology of the esophagus)
Therapy begins with stabilizing the patient and confirming the diagnosis. The
patient will be monitored in an intensive care unit. Resuscitation is initiated by volume
rebalancing with blood and blood substitutes. Irrational use of saline solutions should be
avoided as worsening ascites and portal hypertension due to secondary
hyperaldosteronism present in cirrhosis.
403
Nasogastric lavage is beneficial in removing blood and for stomach
decompression, decreasing the risk of aspiration and facilitating the endoscopic
exploration.
A. Medical treatment
Use of isosorbide 5-mono-nitrate (2x20 mg/day) is safe and effective in
preventing first bleeding, administered for at least 2 years.[63] A combination
of nonselective beta-blockers in association with isosorbide-5-mononitrate
may be the best alternative.[64]
Vasopressin reduces portal pressure by constricting mesenteric arterioles.
The first dose is of 0.4 U in bolus and then continued with 0.4 to 1 U/min
infusion. Vasopressin has a high incidence of cardiac complications due to
nonspecific vasoconstriction.[65-67]
Terlipresin is a long acting congener of Lizin-vasopressin, which has fewer
side effects.
A somatostatin infusion result in a selective splanchnic vasoconstriction
without cardiac complications, and it is considered more effective than
vasopressin. The dose of Octreotide (Sandostatin) is 25-250
micrograms/hour. Somatostatin is considered as initial therapy until the
elective treatment.[68] The effects are antisecretory (inhibiting gastric acid,
pepsin and gastrin secretion), increases gastric mucus production, and
decreased blood flow in the azygos vein and esophageal varices in portal
hypertension. Somatostatin inhibits the release of vasodilator hormones such
as glucagon, producing moderate vasoconstriction and thereby decreasing
splanchnic portal flow.[69,70]
If coagulopathy cannot be adequately corrected with FFP (fresh frozen
plasma) then recombinant human factor VIIa (NovoSeven) will be given in a
dose of 80 micrograms/kg in 30 minutes. However, decision regarding the
use of this hemostatic agent in acute variceal bleeding should be carefully
considered, because studies results do not support the routine use of
rFVIIa.[71]
B. Balloon tamponade for esophageal varices
There are three types of probes with balloons: Sengstaken-Blakemore, Linton-
Nicholas and Minnesota, each with two balloons, gastric and esophageal of different
forms. It is considered that balloon tamponade has an 85-98% initial success, but after
balloon deflation, rebleeding rate is of 21-60%.[72-74] This method has an incidence of
30% severe complications such as aspiration pneumonia, esophageal rupture [75] and
airway obstruction. There are no significant differences in efficiency between balloon
tamponade and vasopressin. In recent years, the self-expanding esophageal stents are
used as an alternative to balloon tamponade.[76,77]
C. Endoscopic procedures
Sclerotherapy of esophageal varicose veins. The aim of sclerotherapy is the
initial hemostasis followed by weekly sessions of sclerotherapy until all
varices are obliterated. Esophageal varices are more suitable for endoscopic
therapy than those in the stomach are. Complications of endoscopic
sclerotherapy are: esophageal ulcers that can bleed or perforate, esophageal
strictures, mediastinitis, pleural effusion, aspiration pneumonia, ARDS,
chest pain and superinfection.
404
Rubber band ligation of esophageal varices. Varices are suctioned into a
cylinder and then, a tensioned rubber band is downloaded at its pedicle. The
rubber band will strangulate the varices producing hemostasis but also after a
few days, the varices will be eliminated because the rubber band will slowly
cut the pedicle. It has been shown that the method is equally effective in
achieving hemostasis and preventing rebleeding as sclerotherapy, but with
fewer local complications mainly stricture.[78,79]
D. Surgical therapy of bleeding varices (see also the chapter of esophageal
pathology at esophageal varices)
Nowadays, when endoscopic procedures are widely developed, accessible and
highly effective, when percutaneous intrahepatic shunts are possible and liver
transplantation is more accessible, surgical procedures are becoming less used.
Surgical procedures are applied in two circumstances: in emergency for bleeding
broken esophageal varices and in elective cases (non-bleeding varices). In emergency,
the first aim of surgery is to arrest bleeding and in elective surgery to reduce the
pressure inside the portal system thus preventing bleeding. Generally, surgical
procedures differ in those two circumstances because of the impaired general status of
the patient in emergency that does not allow extensive and long lasting operations.
In emergency, the operation is recommended only in case of failure of other more
conservative methods (medication, endoscopic procedures, and balloon tamponade).
The minimal intervention in emergency is represented by longitudinal gastrotomy,
followed by inspection of the gastric mucosa and hemostasis by suturing the bleeding
varices. The suture of the esophageal varices is more difficult or even impossible by this
approach, but gastrotomy facilitates a correct positioning and inflation of the
Blackemore probe balloons. This approach is also useful in bleeding from associated
gastric ulcer.
A more radical operation in emergency but with better result for longer period is a
procedure based on esophageal transection and esogastric devascularization. There are
more variants but the principle is the same: transecting the esophagus (and
reestablishing it continuity) will produce a discontinuation of venous blood flow
through the esophageal venous plexuses from the portal vein towards the azygos veins,
and so the pressure inside the varices will decrease (azygo-portal disconnection
procedure). The most known procedure in this category is the Sugiura-Futagawa which
consists in: splenectomy + devascularization of 8 to 10 cm of the esophagus +
transection and end-to-end anastomosis of the lower esophagus 4 to 5 cm above the
gastroesophageal junction (EEA-stapler, introduced through anterior gastrotomy) +
devascularization of the lesser and greater curvatures of the stomach + pyloroplasty +
feeding jejunostomy. The left gastric vein is carefully preserved to allow blood drainage
into the azygos system.
In elective conditions (secondary prophylaxis), the aim of surgical procedures is
to decrease the pressure in the portal system by diverting the blood flow into the inferior
cava vein. There are two types of shunts:
1. Troncular portosystemic shunts in which the shunt is performed between
venous trunks such as the inferior cava vein and portal vein or superior
mesenteric vein. These types of shunts ensure an important and very fast
decrease of portal pressure but also divert a great amount of mesenteric
blood into the systemic circulation bypassing the liver, blood that will not
405
be processed and detoxified by the liver. This will lead to portal
encephalopathy.
2. Radicular or selective shunts are those in which the roots of portal vein,
(especially the splenic vein) are connected to another vein that flows into
the inferior cava vein, for example the left renal vein. The main advantage
is that the portal blood flow is preserved preventing portal encephalopathy
but in addition, the pressure into the varices is lowered preventing
bleeding.
Troncular portosystemic shunts were the most commonly used therapy in the '70s,
but also encumbered by a high mortality rate. Many types of portosystemic shunts have
been developed and applied to reduce portal hypertension, but in nowadays the distal
splenorenal shunt (Warren-Zeppa procedure) is the most used. It has the advantage that
prevents certain complications of the troncular shunts, particularly the portal
encephalopathy. This technique is more difficult, produces a smaller decrease in portal
tension, but does not interfere with subsequent liver transplantation.
Compared with sclerotherapy, surgical shunts significantly reduce bleeding
recurrence rate, but does not alter survival. Combining shunts with sclerotherapy
improves survival.
E. Transjugular intrahepatic portosystemic shunt (TIPS)
It is an interventional radiology procedure in which a metallic stent, of 8-10 cm
length and up to 8-12 mm diameter is placed percutaneously inside the liver to perform
a portosystemic shunt between a portal vein branch and a hepatic vein, and thus the
gradient pressure between those two veins is significantly decreased. The method is
expensive.
For a short time control of bleeding from varices, the procedure is very effective
(95%) but stent obstruction, followed by recurrence of bleeding, occurs in 15-70% of
cases after two years depending much on type of the stent.[80,81] In addition, it induces
portal encephalopathy in 10-20% of cases.[82] Recently, stents with internal
antithrombotic coverage have been developed, that would prevent obstruction, but these
are reserved for cases in waiting list for liver transplantation.
This method does not interfere with subsequent liver transplantation and is mostly
used before a scheduled transplantation.
Contraindications to TIPS include severe congestive heart failure, severe
pulmonary hypertension, severe hepatic failure, portal vein thrombosis with
cavernomatous transformation, and polycystic liver disease.[59]
F. Liver transplantation is the radical and most effective procedure in the
management of portal hypertension and its consequences caused by liver cirrhosis.
Always consider liver transplantation if the patient is Child-Pugh B or C.
A rare cause of portal hypertension manifested by upper gastrointestinal bleeding
is the hepatic arterioportal fistula that may occur after a trauma or rupture of the hepatic
artery aneurysm.[83] Angiography is the diagnostic procedure of choice but Doppler
ultrasound is also useful. Treatment is indicated even in asymptomatic cases to prevent
portal hypertension.[83] Embolization is performed for intrahepatic fistulas and surgery
for extrahepatic fistulas.[83]

406
Stress ulcers
Definition and clinical features
Stress ulcer is the most common cause of gastrointestinal bleeding in intensive
care unit and it increases mortality in those patients for up to five times. The risk of
stress ulcers complicated by significant bleeding is estimated at 1.5% to 5% in patients
from intensive care unit.[84]
Stress ulcers are erosions of the digestive mucosa that generally appear in the
gastric fundus but sometimes also in the antrum, duodenum and distal esophagus.
Generally, erosions are superficial, the source of bleeding being capillary vessels, but
deeper lesions can erode the submucosa causing massive bleeding and /or perforation.
There are two major risk factors for bleeding:[85]
1. Mechanical ventilation for more than 48 hours, and
2. Coagulopathy
Other risk factors are: shock, sepsis, liver failure, renal failure, multiple trauma,
burns over 35% of body surface, post-transplant, head and column trauma, history of
peptic ulcer or upper digestive bleedings.
Pathogenesis
Mucosal lesions occur when the effects of gastric acid and pepsin exceed the
gastric mucosal defense mechanisms:
by excessive production of acid and pepsin (aggressive factors), and/or
by overcoming the defensive factors of gastric mucosa
Physiological mechanisms of gastric mucosa defense are represented by:[86-88]
secretion of mucus and bicarbonate, that form a protective layer on the
cell surface
rapid regeneration of damaged cells
endogenous prostaglandin production
maintenance of an adequate gastric mucosal blood flow
Breaking one of the mechanisms of mucosal defense will result in the inability to
maintain a pH gradient and allows both acid and pepsin to produce mucosal lesions.
The gastric mucus has three main functions:[89,90]
1. Mechanical protection of the epithelial surface
2. Prevents the retrodiffusion of hydrogen ions
3. Focuses the bicarbonate secreted by gastric mucosa on the epithelial cell
surface.
Neither mucus, nor bicarbonate alone can prevent the diffusion of hydrogen ions
to the surface of the mucosa. The combination of the two mechanisms maintains a pH of
7 to the lining surface when the intraluminal pH is 2.
Cell membranes are also part of the barrier to hydrogen ions, being composed of
phospholipids. These phospholipids allow diffusion of soluble molecules but prevent
diffusion of hydrogen ions. Bile salts, which are natural detergents, harm the cell
membrane by dissolving the lipid components.
Gastric epithelial cells have the capacity of rapid regeneration. Under normal
conditions, epithelial cell surface is completely replaced by the progenitor cells of the
basal lamina every 3 days. In acute injuries, the epithelium may be replaced in a few
hours. Erosions destroy the progenitor cells and stop reepithelialization.[91]
407
Endogenous prostaglandins do not have a direct protective effect on gastric
mucosa but they help through other mechanisms: stimulating mucus and bicarbonate
production, increase the concentration of phospholipids in cell membranes, decrease
acid secretion, stimulate cell regeneration and increase blood supply of the mucosa.
Inadequate gastric mucosal blood flow is one of the primary factors contributing
to the "stress gastritis." Decreased blood flow results in decreased oxygen and nutrients
to the gastric mucosa. As long as mucosal blood flow is adequate, the bicarbonate is
available to neutralize the retrodiffused hydrogen ions into the cells.
Shock and hypoperfusion not directly cause erosion of the gastric mucosa.
Mucosal damage occurs as a result of reperfusion. Reperfusion of ischemic cells after
shock potentiates mucosal lesions by releasing oxygen free radicals. These radicals
attack the lipids increasing the membrane permeability for acid.
Neutralization of acid by the gastric mucosal cell depends not only on the gastric
mucosal blood flow, but also on systemic acid-base balance. Systemic acidosis in shock
exacerbates the gastric mucosal lesions.
There have been described several "barrier destroyers such as: aspirin,
nonsteroidal anti-inflammatory drugs (NSAIDs) and alcohol. NSAIDs block the
formation of prostaglandins leading to changes in the layer of mucus and
polysaccharides. Alcohol lowers glutathione, which is a "scrubber of free radicals.
Urea, in chronic renal failure, also compromises the barrier. Steroids decrease mucus
production and lead to changes in the composition of polysaccharides.
However, gastric lesion does not occur in the absence of intraluminal acid and
pepsin.
Clinical picture
Acute gastric mucosal erosions are found in 75% to100% of critical patients at
endoscopy.[84,92] In most cases, they heal spontaneously in 10-14 days in parallel with
the improvement of the primary disease.
Patients in intensive care unit, with stress ulcers, do not have pain or other
gastrointestinal symptoms compared to other patients with peptic ulcer. In addition,
many patients are unable to report symptoms because they are mechanically ventilated
or have mental status changes.
Stress lesions become clinically apparent only when they are complicated with
hemorrhage or perforation.
Generally, bleeding is manifested by a gradually declining of the hematocrit or
occult blood loss, highlighted in gastric aspirate or by positive Hemocult test from stool.
An active hemorrhage manifested as a frank hematemesis or melena, with tachycardia
and hypotension, is a clear indication for emergency endoscopy.
Endoscopy is the "gold standard" for diagnosis of stress ulcers. In addition,
endoscopic treatment can be applied to stop an active bleeding or prevent a bleeding
recurrence.
Barium studies have no role in the diagnosis of stress bleeding in intensive care
patients.
If the endoscopy is negative, angiography may occasionally detect a lesion with
active bleeding or a little bleeding ulcer.

408
Prognosis
Bleeding stops with medical treatment in most cases, but 2-6% of patients, who
develop severe bleeding, require surgery. The incidence of clinically significant
bleeding appears in 1.5% to 30% of critically ill patients.[93-95]
Mortality in patients with gastric stress ulcers is usually due to the underlying
disease and it ranges between 50% and 90%.[96] Only one third of cases are assigned to
hemorrhage, the remaining is based on disease specific mortality.[96,97]
Stress gastritis associated to multiple organ failure syndrome is considered a poor
prognostic sign.
Prophylactic treatment of bleeding from stress ulcers
Different agents have been studied in an attempt to prevent acute mucosal lesions
in patients with critical stress:
Antacids
Histamine receptor antagonists
Proton pump inhibitors
Prostaglandin analogues
Sucralfate (cytoprotective agent)
Enteral nutrition
Antacids neutralize the stomach acid; stimulate prostaglandin synthesis in gastric
mucosa and thus improve its irrigation. Disadvantages are: short-term
effectiveness, osmotic diarrhea, acid secretion rebound by hypergastrinemia,
mucosal micro trauma by microcrystals of aluminum, hypophosphatemia and
hypomagnesemia.
H2 receptors blockers increase the gastric pH and decrease the stimulatory effects of
histamine on parietal cell acid secretion. H2 blockers administration in
continuous infusion allows a better control of gastric pH but with notable
complications such as interstitial nephritis, confusion, and thrombocytopenia.
Ranitidine and Famotidine give less mental confusion and antiandrogenic
effects. It was reported an increased incidence of nosocomial pneumonia in
patients under therapy with antacids: bacterial colonization of the stomach was
demonstrated in 100% of cases when gastric pH was continuously maintained
above 4.
Anticholinergic drugs (pirenzepine) decrease the gastric acid production by producing a
vagal blockade, however, achieving only a small increase in gastric pH. Side
effects are tachycardia and central nervous system effects (agitation, dizziness).
Proton pump inhibitors (PPI) (omeprazole, esomeprazole, lansoprazole, pantoprazole,
rabeprazol) are more effective in stopping and preventing active bleeding than
H2 blockers because gastric pH control is more accurate, and platelet
aggregation and blood clot formation is enhanced at pH values above 7.
Inhibition of acid secretion is rapidly achieved by iv administration
(Esomeprazole 80 mg in bolus over 30 minutes, followed by a continuous
infusion of 8 mg/hr administered for 3 days).
Prostaglandins: administration of endogenous prostaglandin increases the irrigation of
mucosa, the mucus and bicarbonate secretion, stimulates epithelial cell
regeneration and inhibits gastric acid secretion. Analogues of prostaglandin E1
and E2 were used in the prophylaxis of stress ulcers. The Misoprostol, a
409
prostaglandin E1 analogue, has efficiency similar to antacids, but causes
diarrhea in 25% of cases and is more expensive.
Sucralfate is a cell protector agent. It is an aluminum salt of sucrose composed of eight
sulphate groups. At a gastric pH below 3, dissociation of aluminum hydroxide
ions occurs forming polymerized viscous mass covering gastro-duodenal
mucosa. Thus, it protects the lining against the action of hydrochloric acid,
pepsin and bile acids, and it improves the mucosal irrigation stimulating
prostaglandin release.
It is not absorbable, does not modify the gastric pH and it has a bactericidal
effect. It is a drug of choice, given the low incidence of nosocomial pneumonia
compared with other drugs.[98,99]
Sucralfate is also the drug of choice for prophylaxis of bleeding and stress
ulcers, in a dose of 3x2 g/day as a suspension, in acute coronary syndromes,
acute myocardial infarction with thrombolytic therapy or unstable angina.
Early enteral nutrition is recommended by many authors in the prevention of stress
ulcers. Small amounts of foods have a protective effect even in cases of ileus, or
when other medical conditions do not permit normal enteral nutrition. Enteral
nutrition is not important for increasing gastric pH, but it can prevent depletion
of energy needs in gastric epithelium, thus avoiding necrosis and ulcer
formation.
Currently available data show that prophylactic medical therapy reduces bleeding
in patients with stress gastritis from 15% to 3%. There are studies that conclude that
routine prophylaxis for stress-related bleeding even in high-risk patients seems not to be
justified, suggesting that gastric pH increasing medication could increase the risk for
nosocomial pneumonia.[100-102]
Treatment of bleeding from stress ulcers
In most cases, bleeding will stop under medical treatment. The goals of treatment
are directed mainly to the underlying disease and to eliminate additional stress. If the
stress is removed, the digestive mucosa heals spontaneously. Treatment aims primarily
rapid resuscitation of shock, rapid restoration of intravascular blood volume, sepsis
treatment, acid-base rebalancing, correcting the coagulation defects, ventilator and
nutritional support.
Gastric lavage is performed to empty the stomach and prepare the patient for
endoscopy.
Early endoscopy is performed to determine the source of bleeding. Endoscopic
control of bleeding is rarely possible due to the diffuse nature of bleeding from stress
gastritis.
Angiographic embolization of bleeding sites is possible but diffuse nature makes
it difficult to control bleeding permanently.
Surgery is the last alternative therapy being less practiced in recent years. Most
reports of surgical therapy for stress gastritis dates from the '70s with an overall
mortality of 40-65%.[103,104] Suggested surgical operations are: vagotomy and
pyloroplasty with or without hemostatic suture, vagotomy and antrectomy, subtotal or
total gastrectomy.

410
Esophagitis
Acute hemorrhage from esophagitis is treated by H2 receptor blockers or PPI
medication in cases when the patient has resumed eating. Endoscopy or surgery is not a
choice for the treatment of bleeding esophagitis.
Mallory-Weiss syndrome
There are longitudinal lacerations of the mucosa at the gastroesophageal junction.
The pathogenesis is not yet well understood but it seems that lesions are produced by
increased transmural pressure. The syndrome is linked to the effort of vomiting,
coughing, defecation, hiccups and seizures.
Bleeding usually stops without therapy but in cases of active bleeding endoscopic
therapy (local injection, hemoclipping, or multipolar electrocoagulation) can be applied
successfully. Relapse is rare after endoscopic therapy and occurs more often in patients
with portal hypertension.
Dieulafoy lesion
A submucosal, large, aberrant, artery causes bleeding into the gastric lumen. The
lesion is usually located at 6 cm below the gastro-esophageal junction but other
locations are possible (body or fundus of stomach, cardia, esophagus, duodenum,
etc).[105] It is characterized by recurrent gastric bleeding without an obvious source,
unless the vessel is visible. There are no ulcerative changes. Angiography helps to find
the source of bleeding.
Advances in endoscopy and angiography have increased the detection of
Dieulafoy lesions and decreased the mortality from 80% to 8.6%.[106] Endoscopic
treatment combining different kinds of methods are becoming more and more effective
but rebleeding is possible.[107] Bleeding arrest is also possible by embolization.[108,109]
Definitive treatment is represented by surgery (gastric resection) by open or
Upper gastrointestinal angiodysplasia
Angiodysplasia is represented by enlarged submucosal vessels that appear like a
spider-shaped lesion of red color at endoscopy. The cause is unknown. Some patients
have Rendu-Osler-Weber disease, chronic renal failure, cirrhosis and aortic stenosis.
In nowadays the diagnosis is facilitated thanks to the development of push-
endoscopy and video capsule endoscopy.[110]
In one study [111], angioectasias were most commonly found in the jejunum
(80%) followed by the duodenum (51%), stomach (22.8%), right colon (11.4%) and
ileum (5.7%). 60% of patients had angioectasias in more than one location.
Bleeding is usually self-limited or occult but may be as well massive.
Endoscopic therapy is efficient in arresting bleeding thereby decreasing the need
for blood transfusions.

411
Upper gastrointestinal tumors
Tumors represent 1% of severe upper gastrointestinal bleedings. The endoscopic
control of active bleeding, when possible, allows the patient hemodynamic stabilization
before surgical resection.
Aortoenteric fistula
Most fistulas are associated with the placement of intraaortic prosthesis
(secondary fistula) or abdominal aortic aneurysm (primary fistula). Common location is
in the third portion of the duodenum while other segments of the small intestine or
colon may be affected as a consequence of erosion of the intestinal wall. Typically, the
patients with aortoenteric fistula have a small initial hemorrhage, known as heraldic
bleeding that occurs before a massive hemorrhage.
Surgical solution is to by-pass the affected artery and repair of intestinal damage
(duodenorrhaphy) or removal of the prosthesis and fistula, and performing an axilo-
femoral bypass.
Watermelon stomach
The pathognomonic endoscopic pattern of gastric mucosa is of red stripes
arranged radially, departing from pylorus resembling the aspect of watermelon. When
red stripes are arranged in a diffused-way, is the so called honeycomb stomach.[112]
Autoimmune disorders, mainly represented by Reynauds phenomenon and
sclerodactyly, are co-present in about 60% of patients.[112] The anomaly is more
common in women and is related to severe liver disease and renal failure. Laser
photocoagulation was successful in many cases [113,114] but surgical resection
(antrectomy) is the treatment of choice.[115]
Hemobilia and hemosuccus pancreaticus
Hemobilia represents the bleeding into the biliary tract. Clinical picture is
represented by the classical triad of upper gastrointestinal bleeding, jaundice and
abdominal pain. The diagnosis of hemobilia needs to be considered in patients with
recent or previous percutaneous liver intervention, liver transplantation or abdominal
trauma.[116] Diagnosis is confirmed by ultrasound, computed tomography and
angiographic examinations. Angiographic
embolization is indicated, but when it is not
technically possible, surgical treatment
should be applied.[117]
Hemosuccus pancreaticus (Figure 1)
represents the bleeding into the Wirsung duct
exteriorized through the ampulla Vater as an
upper digestive bleeding. The source of
bleeding in most cases is the splenic artery.
A variety of etiological factors, most
commonly chronic pancreatitis but also
tumors and vascular diseases, can lead to this
condition.[118] Most frequently, it occurs in
412
connection with the formation of pseudoaneurysm of splenic or hepatic artery and
pancreatitis as a communication between the aneurysm and the pancreatic duct.
Although ultrasound, magnetic resonance and CT scan reveal a pancreatic pseudocyst,
angiography is necessary to highlight the blood flow and thus clarify the diagnosis.
Therapeutic options are the angiographic embolization and surgery (distal
pancreatectomy, pancreatoduodenectomy, or total pancreatectomy). Angiographic
embolization is the treatment of choice for stable patients.[118,119]
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Abdominal Trauma
ABDOMINAL TRAUMA

Background
Injuries resulting from traffic collisions, drowning, poisoning, falls or burns and
violence from assault, self-inflicted violence or acts of war, kill more than five million
people worldwide annually accounting for 9% of global mortality.[1]
In 2004, cumulative mortality through intentional and unintentional injuries
ranked sixth after cardiovascular diseases, infectious and parasitic diseases, cancers and
respiratory diseases.[2]
Estimations indicate that by 2020, 8.4 million people will die yearly from injuries,
and injuries from traffic collisions will be the third most common cause of disability
worldwide and the second most common cause in the developing world.[3]
On the other hand, injuries represent a major public health and economic problem
due to the high costs of treatment and to the temporary or permanent disabilities.
Injuries affect mostly young people, the mortality produced by injuries
representing 22%-29% of all death at ages 1559, except in Africa, where it is 13%.[2]
In US mortality produced by unintentional injuries in 2010, ranked first in the group age
of 1-44 years.[4]
Data from the World Health Organization (WHO) indicate that falls from heights
of less than 5 meters are the leading cause of injury, and car crashes are the next most
frequent cause.[3]
In Romania, injuries are the fourth leading cause of death. The rates for
unintentional injuries and for all the intentional injuries are lower than the regional
averages but higher than the European Union (EU) values. The leading causes of
unintentional injury-related death are road traffic injuries, followed by falls, poisoning,
drowning and fires. The leading causes of intentional injury-related death are suicide
followed by homicide.[5]
The abdomen is one of the most involved parts of the body in trauma, requiring
surgical exploration in up to 20% of cases.
Abdominal cavity can be divided into four areas where organs are more or less
protected by anatomical structures from external injuries.
1. The upper abdomen beneath and protected by ribs cage containing the liver,
gallbladder esophagus, stomach, duodenum and spleen
2. The lower or pelvic abdomen, protected by pelvic bones containing the:
bladder, uterus with annexes, rectum and some small intestines
3. The retroperitoneal area, containing the kidneys, ureters and suprarenal
glands, pancreas, duodenum, aorta and cava vein
4. Anterior middle part of the abdomen, the most exposed to trauma containing
small and large intestine, omentum and part of the stomach
Classification
Abdominal trauma may be classified as blunt (contusions) and penetrating
(wounds).
Blunt trauma (contusions):
o Traffic accidents (most common 50-75%)
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Abdominal Trauma
o Falls
o Aggressions
o Accidents
o Sport (recreational) and domestic accidents
o Iatrogenic (resuscitation, Heimlich maneuver)
Penetrating trauma (Wounds):
o Stab wounds
o Gunshot wounds
A. Blunt Abdominal Trauma
Blunt abdominal injuries are the most frequent being more common in men at a
rate of 6:4.[3]
Pathophysiology
Blunt trauma is produced by collision between the body and the external agent.
There are three mechanisms acting during collision:
1. Deceleration - induces high tensions in the fixing points of intra-abdominal
organs resulting in breaking of fixing anatomical structures or of the organ
itself. Classic deceleration injuries include hepatic tear along the coronary
ligament, injuries to the renal pedicles, and mesenteric tears with resultant of
splanchnic vessels injuries.
2. Crushing - because of deceleration, intra-abdominal organs are crushed
against the vertebral column or posterior thoracic cage. This affects
especially solid viscera such as the spleen, pancreas, liver, kidneys.
3. External compression - directly produces injuries to the abdominal wall and
solid intra-abdominal organs or indirectly by increasing the pressure inside
the hollow viscus producing their explosion.
The liver and the spleen and then the small and large intestines are the most
frequently injured organs.
I. Contusions Limited to the Abdominal Wall
Many abdominal traumas are limited only to the abdominal wall and may
manifest as:
Serohematic Morel-Lavallee effusion - is a serohematic fluid accumulation
between the skin and the fascial plane resulting after a tangential abdominal wall
trauma. (Figure 1) It appears as a local swelling, bruising, associated with fluctuance.
Treatment includes percutaneous drainage, or in case of massive accumulation, incision
and drainage under antibiotic protection.
Subaponeurotic hematoma - is caused by the rupture of the abdominal muscles
fibers, frequently located in the sheath of the rectus abdominis. (Figure 2) Symptoms
consist of pain exacerbated by physical activity. Local signs are swelling, bruising and
sometimes in the extensive forms intestinal transit disorders and pseudo-occlusive
syndrome. Diagnosis is confirmed by ultrasound or CT examination. (Figure 3)
Treatment can be conservative (ultrasound guided puncture) when intra-abdominal
organs injuries are excluded, or incision and drainage in case of massive accumulation.
When the hematoma is associated with intra-abdominal injuries laparotomy is required.

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Abdominal Trauma

Properitoneal hematoma - represents the accumulation of blood into the
properitoneal space after a serious contusion being associated with abdominal muscles
rupture, subaponeurotic hematoma and frequently intra-abdominal organs injuries. The
hematoma produces a peritoneal irritation syndrome and paralytic ileus. Usually,
treatment is represented by laparotomy and thorough exploration of intra-abdominal
organs.
Posttraumatic hernias - appear after abdominal trauma associated with rupture of
the muscular layer. The clinical aspect is of a reducible swelling usually associated with
local bruising. The diagnosis is facilitated by ultrasound examination, which reveals the
parietal defect. The treatment is strictly surgical as in incisional hernias.
Posttraumatic eviscerations - appear after a complete solution of continuity of
the abdominal wall including all layers inclusive the peritoneum. It is an emergency
because is always associated with general peritonitis as intra-abdominal organs are
exposed to air. The treatment consists of wall reconstruction but also thorough
inspection of the abdominal cavity and abdominal organs.
II. Contusions with Visceral Lesions
Usually, lesions of the internal organs are produced by complex mechanisms
(presented above: deceleration, external compression, crushing). Lesions will be
presented later, separately for each organ.
From pathophysiological point of view, three types of abdominal syndromes may
occur:
1. Intraperitoneal hemorrhagic syndrome. General signs are tachycardia,
hypotension, dizziness followed by tendency to collapse, pale skin, and oliguria.
Local signs are distended abdomen, mild diffuse pain, mild muscular guarding,
abolition of bowel movements.
2. Peritoneal irritation syndrome (post-traumatic peritonitis). The classic signs
are those of a generalized peritonitis.
These two syndromes may develop simultaneously while the predominant usually
is the hemorrhagic syndrome.
3. Late clinical syndromes - in which lesions are clinically manifested after an
asymptomatic period.
a. The two-stage hemoperitoneum. The subcapsular rupture of a
parenchymatous organ resulting in subcapsular hematoma represents the
first stage. After a while, spontaneously of after a trauma, the capsule
ruptures leading to intraperitoneal bleeding. It is the second stage.
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Abdominal Trauma
b. The two-stage peritonitis. The first stage is represented by the organ
trauma and then, the peritonitis as the second stage that may occur as a
result of:
Eschar of a hollow organ wall (decubitus sores) due to necrosis or
compressive hematoma.
Bacterial translocation: appears in traumatic shock when oral feeding
is resumed after a long time.
Necrosis of the intestine due to ischemia as a result of vascular
injuries (ruptures, compressive hematoma of mesenteric desinsertion
with a segmental bowel ischemia).
c. Localized peritonitis represented by intraperitoneal abscess.
d. Intestinal obstruction produced by internal hernia (for example a breach
in the mesentery as a result of the abdominal trauma - see at hernia
chapter).
B. Penetrating Abdominal Trauma - Abdominal Wounds
Penetrating abdominal trauma involves the penetration of the entire thickness of
the abdominal wall and violation of the peritoneal cavity by a gunshot or stab wound.
The management of penetrating abdominal trauma has evolved greatly over the
last century. Laparotomy has become the treatment of choice during World War I, but
mortality remained high. By World War II, early laparotomy resulted in a survival rate
close to 50%.[6]
In recent decades, many progresses have been made in multiple areas, progresses
that increased the rates of survival after penetrating abdominal trauma: development of
antibiotics, anesthesia, and methods of investigation, resuscitation, transportation,
surgical treatment and so on.
Epidemiology
The incidence of penetrating abdominal trauma differs widely from one
geographical region to another, from one country to another, depending on the degree of
industrialization, culture and military conflicts. Incidence or murders with firearms vary
from 0% in Iceland and Luxemburg, to 9.3% in United States, 19.9% in Thailand,
21.7% in Colombia and 31.7%in South Africa.[7]
Males constitute the great majority of patients with penetrating trauma.
Classification
There are two types of abdominal wounds: penetrating abdominal wounds, when
the entire abdominal wall thickness is penetrated and there is a communication between
the peritoneal cavity and the atmosphere, and non-penetrating wounds when only some
layers of the abdominal wall are injured except the peritoneum.
Non-penetrating wounds may be:
Simple without association with contusion of the intra-abdominal organs
Associated with visceral lesions
Penetrating wounds may be:
Simple without visceral injuries
With a visceral involvement
Complex within a polytrauma
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Abdominal Trauma
Etiopathogenesis:
Wounds caused by firearms (gun shot wounds) are characterized by an important
transfer of energy and the impossibility to appreciate the exact extent of intra-abdominal
lesions. The amount of energy transferred depends on:
missile speed
missile shape (irregularly shaped splinters cause more serious injuries)
occurrence of secondary missiles (the missile fragments or fragments of
tissue displaced by it)
shooting distance
In penetrating abdominal trauma due to gunshot, the most commonly injured
organs are as follows: small bowel (50%), colon (40%), liver (30%) and abdominal
vascular structures (25%).[6]
Wounds caused by weapons (stab wounds) are more common, and generally,
intra-abdominal organ injuries are more predictable. Usually a single organ is affected
but occult injuries can be overlooked, resulting in devastating complications.
In penetrating abdominal trauma due to stab wounds, the most commonly injured
organs are as follows: liver (40%), small bowel (30%), diaphragm (20%) and colon
(15%).[6]
In abdominal trauma, the prognosis is correlated with the number of injured
organs, the speed of diagnosis and of establishing the therapeutic measures.
Diagnosis of abdominal trauma is associated with first aid measures, measures to
maintain vital functions and to combat the shock. Priorities in diagnosis and treatment
can be grouped in this order:
1. Recognition of the presence of shock or intra-peritoneal hemorrhage
2. Starting resuscitation measures
3. Assess the cause of shock or hemorrhage (abdominal / extra-abdominal)
4. Asses the need for emergency laparotomy
5. Completion of secondary examination (laboratory tests) to detect occult
lesions
6. Frequent reassessment to follow the evolution of the patient considering
the possibility of undetected lesions or to detect late lesions (2 or 3 stages
lesions).
History is sometimes impossible or difficult in dizziness or unconscious patients.
Important information refers to:
The nature and circumstances of injury
The time when it occurred
The status of the patient at the time of trauma:
o physiological (food, stool, urination, pregnancy)
o pathological (associated diseases, allergies, previous interventions,
ingestion of medicines or drugs)
Pain: timing, location and radiation, the evolution in time.
Other symptoms or signs: hematemesis, melena, rectal bleeding,
hematuria.
A. Primary survey
Any penetrating abdominal trauma represents a surgical emergency because is
burdened by certain risk of death. Therefore, these patients will be evaluated and will be
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Abdominal Trauma
given first aid in the emergency service. Patient health status brought to the emergency
room can vary greatly depending on the severity of injuries. Some patients are in good
condition, hemodynamically stable, but others are unstable and comatose. Polytrauma
patients are a challenge for doctors in evaluation and stabilization of their vital
functions. On the other hand, a penetrating abdominal wound may have a lower risk of
death from abdominal vascular injuries.
Evaluation and stabilization measures must be made quickly and simultaneously.
The primary survey will not take more than 20-30 seconds. Vital functions should be
evaluated first according to the mnemonic ABCDE: Airway, Breathing, Circulation,
Disability, and Exposure/Environment.
A. First airway patency should be checked. If foreign bodies or secretions
are found, they will be removed.
B. Breathing is assessed and if necessary, orotracheal intubation will be
performed.
C. Assessment of circulation begins by assessing the patient's mental status,
skin color and temperature. Pulse and blood pressure are not very
characteristic of hemorrhagic shock and will be evaluated in secondary
survey if there is cardiac activity.
D. Disability is assessed for neurologic deficits before giving sedation or
paralytics. The Glasgow Coma Score and the gross motor and sensory
status of all four extremities should be determined and recorded.
E. Exposure is very important especially in the patient with polytrauma.
Complete exposure and head-to-toe visualization is mandatory to
discover other potentially life-threatening injuries.
B. Secondary survey and injury assessment
After primary evaluation, first measures, including surgery if necessary, are
aimed to save the patient's life. Blood samples will be collected for laboratory tests and
including blood group. If necessary, the patient will be intubated, and an intravenous
access will be established. In pure abdominal trauma, the most dangerous is the
hemorrhagic shock caused by intraperitoneal bleeding and surgical measures are
addressed to stop bleeding.
Secondary survey presumes a complete head-to-toe physical examination that can
be delayed until the patient is stabilized but performed as soon as possible.
On inspection, skin lesions may guide the prediction of injured intra-abdominal
organs. In gunshot wounds, skin lesions may represent either entrance or exit wounds.
In transfixiante wounds, the exit lesion is 2-3 times larger than the entrance lesion, and
frequently does not overlap the entrance wound because the bullet was deflected by the
skeletal structures. In the so-called blind wounds, represented only by the entrance
lesion, multiple missiles or foreign objects are retained within the body. Pathological
fluids (blood, intestinal content, urine) may be observed leaking through wounds.
Abdominal distention may mean a bowel obstruction or hemoperitoneum. A
visible mass may represent a massive abdominal hematoma, a traumatic hernia or intra-
parietal collection.
On palpation, a reducible swelling confirms the traumatic hernia, the presence of
fluctuance confirms a parietal collection. Pain is the most important sign. Abdominal
guarding or contracture reveals the peritoneal irritation. There is a lack of signs of
peritoneal irritation in patients with severe shock, coma, poisoning by drugs or alcohol.
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Abdominal Trauma
In other circumstances, signs of peritoneal irritation may be misleading such as in case
of false acute abdomen produced by injuries of the spinal cord, of the base of thorax or
properitoneal hematoma.
On percussion, the shifting dullness in lateral quadrants is a sign of
intraperitoneal fluid collection, most often blood. Tympanic sound means possible
bowel obstruction. Induced pain on percussion (Mandel positive maneuver), means
peritoneal irritation.
On auscultation, the absence of bowel sounds is observed in peritonitis and
exacerbation in mechanical bowel obstruction. Bowel sounds heard over the thorax are
present in diaphragmatic rupture. Arterial murmurs can be heard in large vessel lesions.
Rectal and vaginal finger examination is mandatory and can detect pain induced
by palpation of the Douglas bag that confirms pathological peritoneal fluid collection.
Local deformations produced by fractures of the pelvic bones can be detected.
Wounds located on the anterior abdomen can be explored locally (using sterile
instruments) to determine whether they penetrate the peritoneum. On the flank and back
areas, exploration is more difficult and less reliable. Imagistic explorations (ultrasound,
CT) are indicated in these cases.[8]
Repeated physical examination is the most important element of observation.[9,10]
Investigations
Three tests are the most important in diagnosis (of course in adjunction to history
and physical examination): CT scan, ultrasound examination and the diagnostic
peritoneal lavage. In Europe in the first preferred examination is the abdominal
ultrasound.
Laboratory tests. Blood count and blood group are required even if in the first
stage there is no obvious important bleeding. Leukocytosis, even in the presence of a
normal hemoglobin and hematocrit may reveal hemorrhage. Coagulation tests and
platelets count are important for further therapeutic decisions. Amylasemia may be
increased in posttraumatic pancreatitis without a direct correlation with the severity of
injuries. Transaminases, LDH, gamma GT, can increase in liver damage. Urinalysis, to
detect hematuria. Blood glucose, urea and creatinine, electrolytes and routine tests are to
be interpreted in evolution. Sometimes, detection of medicines and drugs levels in
biological fluids is necessary.
Abdominal ultrasound (US) is important because it can be done at bedside in
unstable patients.[11] Abdominal US is a useful and valuable diagnostic tool after
clinical evaluation in patients with blunt abdominal trauma.[12,13] The main
disadvantage is the lack of sensitivity especially in penetrating trauma.[11] Some deep
lesions could go unnoticed especially in uncooperative patients with significant gaseous
abdominal distension.
FAST (Focused Assessment with Sonography for Trauma) uses 4 views of the
chest and abdomen (pericardial, right upper quadrant, left upper quadrant, pelvis).
Ultrasound signs of lesions are:
1. The presence of free liquid in the Douglas pouch or Morrison (under the
liver) space
2. The presence of fluid in the pleural sinuses or pericardium
3. Parenchymal lesions such as spleen or liver rupture with hematoma
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Abdominal Trauma
Radiological explorations. Even in cases of pure abdominal wounds, a chest
radiograph should be performed (except when a CT scan was performed initially) to
rule out the penetration of the thoracic cavity.
Plain abdominal X-ray highlights the pneumoperitoneum in hallow organ
perforation, fluid-air levels in occlusions, foreign bodies (bullets) or disappearance of
psoas muscles shadow in retroperitoneal effusions. X-ray of pelvis and spine is used to
highlight fractures but CT scan and MRI are more reliable.
In patients with a good status and clinical course, investigations that are more
complex can be performed: examination of the digestive tract with hydrophilic contrast,
urography, and angiography.
CT scan can provide important data regarding the presence of intra-peritoneal
fluid and about its source but the diagnosis of significant penetrating injury should not
be delayed by routine CT. It is the standard examination for the detection of
parenchymatous organ damage. The indications for CT scan are:[14]
Patients hemodynamically stable, but with equivocal clinical examination
Spinal cord injuries (back and flank trauma)
Hematuria in stable patients
Head trauma
Pelvic fractures
CT requires a hemodynamically stable patient. In modern services spiral CT is
performed in all cases of severe injuries.
Diagnostic peritoneal lavage (DPL) shall be performed only after inserting a
nasogastric tube and a bladder catheter for decompression. It is indicated in:
Blunt abdominal trauma :
o Unstable patient
o Stable patients with inconclusive clinical examination
Penetrating abdominal trauma:
o Stabbed abdominal wound with no signs of peritoneal irritation
o Chest wound under the nipples level (possible diaphragmatic
injury)
o Stabbed wound in the flanks.
Contraindications are:
In penetrating stab wounds to the abdomen or flank, if the patient is
hemodynamically instable or has signs of peritonitis, diagnostic testing
should not delay laparotomy.[15]
Open abdominal surgeries in patients history (risk to damage the bowel
loops adherent to the abdominal wall)
Advanced pregnancy
DPL can be performed in two ways: 1. via the
open method, when the catheter is inserted into the
peritoneal cavity under direct visualization through a
small infraumbilical incision, or 2. via the closed
method when the catheter is inserted blindly over a
guide wire through a trocar.
Aspiration of gross blood (Figure 4) or food
particles is positive for peritoneal penetration and organ
injury. If aspiration is negative, one liter of warm
normal saline is infused rapidly and allowed to return by
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Abdominal Trauma
placing the bag on the floor. The fluid is then sent for analysis.
Indications for laparotomy are:[15,16]
1. 10 ml gross blood on initial aspiration
2. More than 100,000 red blood cells/ml, (30 ml of blood in the peritoneum
is enough to return a positive lavage)
3. More than 500 leukocytes/ml
4. The presence of bile, amylase, intestinal or fecal content, bacteria, fiber, or
urine
5. Failure to recover the fluid is considered positive point
Treatment principles
The management of abdominal trauma varies according to the following factors:
Mechanism and location of injury
Hemodynamic and neurologic status of the patient
Associated injuries
Institutional resources
Abdominal trauma, either penetrating or blunt, is considered an emergency, and
therefore the patient will be monitored and resuscitated (if necessary) in the emergency
room. After the primary evaluation, priority will be given to support vital functions.
Airways will be released and if necessary, the patient will be intubated to ensure
appropriate oxygenation or ventilation. If there are intrathoracic lesions (ie
pneumothorax, hemothorax) these will be treated with priority to ensure respiratory
function. Airway protection and ventilatory support are followed by circulatory
resuscitation with fluid infusion.
Immediate laparotomy is indicated when the patient is instable hemodynamically,
in cases of evisceration, peritonitis or gunshot. However, in less serious cases, selected
patients may be managed expectantly [8] or an exploratory laparoscopy can be
performed.
Surgical treatment of abdominal trauma in hospital setting is adapted to specific
lesions but there are some general principles:
Diagnostic maneuvers and then surgery is associated with concomitant re-
balancing maneuvers.
The patient should be placed on a cardiac monitor, pulse oximeter, and 100%
nonrebreather oxygen mask.
Antibiotic therapy is mandatory in any penetrating trauma and in any possible
damage to the digestive tube, covering the whole spectrum (aerobic and
anaerobic germs).
Tetanus prophylaxis (Tetanus Toxoid), if five years have passed since the last
immunization.
Major painkillers to combat pain are used only in stable patients with
complete diagnostic (to avoid masking symptoms of an acute abdomen).
Abdominal trauma often is a part of polytrauma. For a good monitoring and
resuscitation, in most cases it is necessary to apply the rule of the four catheters: central
venous catheter, nasogastric tube, bladder catheter and tracheal intubation (if
necessary).
Nasogastric aspiration is useful because:
Ensures the stomach decompression
425
Abdominal Trauma
Reduces the risk for aspiration into the lungs
Removes toxins and waste from the stomach
Highlights the occurrence of upper gastrointestinal bleeding
It is mandatory prior to DPL
Bladder catheterization is useful because:
Establishes the permeability of urethra
Solves the acute urinary retention
Allows tracking the diuresis for assessment of necessary volume of
rebalancing fluids
Central venous catheter is useful for:
Administration of medication or fluids
Measurement of CVP (central venous pressure) to assess the volemic load of
the patient
Collection of blood samples for analysis
Stomach Trauma
The stomach is injured in most cases during anterior abdominal penetrating
trauma and its anterior wall is the most affected. In blunt trauma, a full stomach may
explode due to sudden increase of pressure.
Extravasation of gastric content is followed by generalized peritonitis, with
specific symptoms requiring laparotomy. Rarely, small lesions may be followed by
covered perforation and peritoneal abscess.
For a positive diagnosis, plain abdominal radiography is helpful in highlighting
the pneumoperitoneum.
Contusions are usually followed by a gastric wall hematoma, clinically
manifested by abdominal pain associated with superior digestive hemorrhage, or if there
is a rupture of the gastric wall, with signs of peritonitis.
Gastric injuries are classified by AAST (American Society of Trauma Surgery)
in:[17]
Grade Lesion
I Contusion/hematoma
Partial thickness laceration
II Laceration
- <2cm in GE junction or pylorus
- <5cm in proximal 1/3 stomach
- <10cm in distal 2/3 stomach
III Laceration
- >2cm in GE junction or pylorus
- >5cm in proximal 1/3 stomach
- >10cm in distal 2/3 stomach
IV Tissue loss or devascularization <2/3 stomach
V Tissue loss or devascularization >2/3 stomach
Contusions without rupture of the gastric wall can be treated conservatively with
nasogastric aspiration, symptomatic treatment and frequent reassessment. If laparotomy
is necessary, surgery can be grouped as follows:
For grade I and II: - Hematoma evacuation, hemostasis and suture.
For grade III: - Lesion excision and suture, or lesion excision and
gastroenteroanastomosis. (GEA)
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Abdominal Trauma
For grade IV: - Proximal or distal gastrectomy adapted to concomitant injuries
of other organs.
For grade V: - Total gastrectomy with esojejunal anastomosis with Roux-en-
Y loop or omega loop.
Duodenal Trauma
Duodenal trauma is a very serious, life threatening condition and represents 1-2%
of all abdominal traumas. It is usually discovered during laparotomy for hypotension or
peritoneal irritation. The mechanism depends on the anatomical region involved. For the
D3 portion, crushing against the spine is main mechanism. Traction can cause the
rupture in duodenal extremities. The explosion of a full duodenum by compression is
another possibility.
Pathological classification of duodenal lesions according to AAST:[17]
Grade Lesion
I Hematoma - Involving single portion of duodenum
Laceration - Partial thickness, no perforation
II Hematoma - Involving more than one portion
Laceration - Disruption <50% of circumference
III Laceration
- Disruption 50%-75% of circumference of D2
- Disruption 50%-100% of circumference of D1,D3,D4
IV Laceration
- Disruption >75% of circumference of D2
- Involving ampulla or distal common bile duct
V Laceration - Massive disruption of duodenopancreatic complex
Vascular - Devascularization of duodenum
Clinical picture is nonspecific, usually manifested by peritoneal irritation
(intraperitoneal rupture) syndrome or internal bleeding. Postponing the diagnosis and
the surgical treatment over 24 hours, leads to a very high mortality. The severity is
given by the autodigestion produced by the extravasated pancreatic juice similar to an
acute pancreatitis worsened by bile leakage and bleeding. Retroperitoneal rupture has a
more subdued clinical picture of reflex arrest of bowel movements, restlessness or
dizziness associated with a febrile syndrome (retroperitoneal effusion).
Laboratory tests will show hyperamylasemia. On plain abdominal radiograph,
pneumoperitoneum may be present in case of intraperitoneal rupture. Retroperitoneal
and/or intraperitoneal extravasation of hydrophilic contrast agent is seen on abdominal
X-ray or CT. DPL may indicate the laparotomy but does not specify the injured organ.
Intraoperative, a retroperitoneal rupture of the duodenum is suggested by the
Winiwarter triad: the presence of blood +bile +gas in the retroperitoneum.
The treatment of duodenal lesions varies depending on location and severity of
injuries:
For grade I: - Hematoma usually is treated conservatively. Laceration is
treated by simple suture.
For grade II: - Hematoma requires evacuation and multiple sutures.
Laceration of less than 50% of circumference is sutured in two layers. An
intestinal loop can be used to patch the parietal defect.
For grade III: - In total duodenal transection, distal duodenal segment can be
closed and the proximal segment is anastomosed to a jejunal loop. In large
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Abdominal Trauma
defects, after duodenal suture, an intestinal loop can be used to patch the
parietal defect, or for anastomosis.
For grade IV: - When lesions are not involving the bile duct or Vaters
ampulla, a duodeno-jejunal anastomosis is preferred. For lesions of the CBD
without pancreatic lesions, reimplantation of CBD (in duodenum or jejunum)
is performed. When pancreatic lesions are associated, cephalic duodeno-
pancreatectomy is the solution.
For grade V: - Major duodeno-pancreatic injury requires cephalic duodeno-
pancreatectomy unfortunately burdened by a high mortality rate.
Traumatic duodenal lesions are frequently associated with other intra-abdominal
injuries particularly of the pancreas; they are difficult to diagnose and pose special
problems concerning surgical technique and therefore they have a high postoperative
morbidity and mortality.
Small Intestines and Mesentery Trauma
Small intestine and mesentery are the most frequently damaged organs during
abdominal trauma. Small intestines lesions occur in up to 50% of penetrating abdominal
wounds and in association with mesenteric lesions in up to 70% of abdominal
contusions.
Mechanisms of lesion include crushing, sudden explosion by increasing
intraluminal pressure, rupture at site of the junction between fixed and mobile segments,
mesentery lesions with vascular impairment and intestinal necrosis.
Posttraumatic mesenteric thrombosis is rare but a serious cause of intestinal
venous infarction.
AAST classification of traumatic lesions of the small intestine:[17]
Grade Lesion
I Hematoma - Contusion or hematoma without devascularization
II Laceration <50% of circumference
III Laceration >50% of circumference without transection
IV Laceration - Transection of the small bowel
V Laceration - Transection of the small bowel with segmental tissue loss
Vascular - Devascularized segment
Three clinical syndromes are associated to bowel injuries:
1. Traumatic shock caused by irritation of vegetative plexuses with no
visceral lesions (respond quickly to treatment)
2. Internal bleeding syndrome
3. Perforation syndrome (symptoms of peritonitis)
Frequently there are mixed forms, usually with the preponderance of the internal
bleeding syndrome.
Late posttraumatic manifestations are peritonitis due to late perforations as a
result of parietal necrosis and intestinal obstruction caused by incarcerated internal
hernia or compressive hematoma.
Surgical treatment depends on the grade of lesion.
For grade I: - Seromuscular suture of the parietal defect and hematoma
evacuation.
For grade II: - Suture in two layers or resection.
428
Abdominal Trauma
For grade III: - Transverse suture to prevent stenosis, if intestinal vascular
supply is preserved. If not, resection is necessary.
For grade IV and V: - Resection with primary anastomosis.
Breaches of the mesentery should be sutured to prevent internal hernias. If
intestinal vascular supply is compromised, resection is the solution with
end-to-end or side-to-side anastomosis.
Colon Trauma
Colon injuries have a lower frequency than those of the small bowel, up to 5%,
but are of extreme gravity with a mortality of about 5% due to the septic content of the
colon.[18,19]
The most frequent causes are the abdominal stab wounds, gunshot wounds and
colonoscopies (1 in 1400 for overall colonoscopies and 1 in 1000 for therapeutic
colonoscopies).[20,21]
AAST classification of traumatic lesions of the colon:[17]
Grade Lesion
III Laceration >50% of circumference without transection
IV Transection of the colon
V Transection of the colon with segmental tissue loss
Devascularized segment
Clinical picture depends on the degree of laceration and whether it is located
intra- or retroperitoneal. Intraperitoneal rupture causes stercoral peritonitis with toxic-
septic shock. Pneumoperitoneum is present in up to 70% of cases. Retroperitoneal
rupture causes a retroperitoneal abscess or phlegmon with nonspecific symptoms: fever,
ileus, local Celsian signs. Ultrasound and CT scan facilitate the diagnosis and can guide
the puncture of the abscess.
The treatment of traumatic lesions of the colon is different depending on location
and the severity of injuries, but requires mandatory association with broad-spectrum
antibiotics.
Possible types of operations are:
Suture of a simple lesion protected or not by a superjacent colostomy or
ileostomy.
Exteriorization of the injured segment as a loop colostomy if a mobile
segment of the colon is affected.
Segmental resection, when lesions are more serious, followed by:
o Colocolic anastomosis - in mobile segments (transverse and
sigmoid colon) - is not recommended in stercoral peritonitis due to
the high incidence of fistula. The anastomosis can be protected by
a colostomy or ileostomy.
o Hartmanns I procedure when the distal end is closed and the
proximal one is exteriorized in terminal colostomy.
o Exteriorization of the both ends of the colon in colostomy.
Right hemicolectomy, when the right colon is affected - may be followed
by ileo-colic anastomosis or just ileostomy and closure of the colic stump.
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Abdominal Trauma
Left hemicolectomy, usually followed by colostomy of the proximal end
and closure of the distal one, in left colon injuries.
Subtotal colectomy with ileostomy and closure of the distal end or
ileosigmoidostomy.
Performing a colostomy is recommended in:[22-24]
Massive intraperitoneal contamination
Shocked patient with hypotension
Patients requiring multiple blood transfusions
Multiple colic lesions or association with a rectal injury
Ischemic necrosis of the colon
Interventions performed at more than 6 hours after injury (major septic
risk)
Association with a retroperitoneal hematoma from a fractured pelvis
Association with a posttraumatic parietal defect
Rectal Trauma
The mechanisms of rectal lesions are mainly endoluminal during endoscopy or
produced by foreign bodies. Another possible mechanism is the rectal lesion by bone
fragments during pelvic fractures.
Rectum may be injured in its intraperitoneal segment resulting in peritonitis or
subperitoneal segment resulting in abscess formation with a mortality rate up to 20%.
AAST classification of traumatic lesions of the rectum:[17]
Grade Lesion
Laceration - Partial-thickness laceration
III Laceration >50% of circumference
IV Full-thickness laceration with extension into the perineum
V Devascularized segment
Clinical picture is variable depending on the involved rectal segment, but rectal
bleeding is the common element (but not constant).
Intraperitoneal segment lesions are followed by rapidly evolving stercoral
peritonitis.
Retroperitoneal lesions are followed by cellulites or pelvic abscess manifested by
fever, pain, local Celsian signs.
The association with urethral and bladder lesions is followed by leakage of urine
through the rectum and pneumaturia (presence of air in the bladder), fecaluria (mixture
of feces and urine that is passed through the urethra) and severe ascending urinary
infections.
Digital rectal examination is mandatory in these patients and can highlight: rectal
solutions of continuity, fluctuance of perirectal tissues, intrarectal or perirectal foreign
bodies (bone fragments) and bleeding.
Diagnosis is confirmed by rectoscopy. Endorectal ultrasound can detect
incomplete lesions of the rectal wall and the presence of perirectal collections.
Surgical treatment varies according to severity of lesions.
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Abdominal Trauma
The grade I and II of injuries can be primary sutured. In more serious injuries,
when rectal sutures are possible, an upstream protective colostomy should be
associated. Injuries that cannot be sutured require Hartmanns I type procedure.
Rectal injuries associated with pelvic collections require drainage. In most cases,
the appropriate approach is through an incision between the anus and coccyx.
Very rare, perianal gunshot produces massive lesions with devascularization of
the rectum, requiring resection with colostomy.
Intrarectal foreign bodies are extracted through the anus in spinal or general
anesthesia. After extraction, rectosigmoidoscopy is required to assess any damage to the
rectum. When the foreign body cannot be extracted through the anus, it will be extracted
by laparotomy through a colotomy.
Urethral and bladder injuries are treated by suture associated or not with
cystostomy.
The mortality rate of rectal injuries caused by blunt trauma is more than 50%,
which is higher than the mortality rate of colon injuries caused by blunt trauma
(17%).[23]
Pancreatic Trauma
Although pancreatic traumas are rare (<10%) they are very serious due to
frequent association with other abdominal lesions, especially of the large vessels that
cause a significant morbidity (3660%) and mortality (1823%).[25-28] Early mortality
usually results from uncontrolled or massive bleeding due to associated vascular and
adjacent organ injuries. Late mortality is mainly due to infection complicating acute
post-traumatic pancreatitis.
The most frequent causes are traffic accidents (in frontal collisions when the
abdomen is crushed against the steering wheel), followed by gunshots. Iatrogenic
pancreatic lesions may appear during abdominal surgery and endoscopic maneuvers
(ERCP).
AAST classification of pancreatic trauma:[17]
Grade Lesion
I Hematoma - Minor contusion without duct injury
Laceration - Superficial laceration without duct injury
II Hematoma - Major contusion without duct injury or tissue loss
Laceration - Major laceration without duct injury or tissue loss
III Laceration - Distal transection or parenchymal injury with duct injury
IV Laceration - Proximal transection or parenchymal injury involving
ampulla
V Laceration - Massive disruption of pancreatic head
Clinical picture. Isolated pancreatic injuries are difficult to diagnose, and may
evolve as a post-traumatic acute pancreatitis with its early and late complications. In
blunt abdominal trauma, pancreatic lesions are frequently associated with other organ or
vessels lesions and the clinical picture is that of a traumatic shock with a mixed
syndrome of internal bleeding and pancreatitis. Increased amylasemia and visible
lesions on CT scan facilitate diagnosis. In blunt trauma by frontal collision, a high index
of suspicion of pancreatic lesion should be present.
Surgery is adapted to lesion types.
431
Abdominal Trauma
Abdominal wounds, especially the gunshots, where pancreas lesions are
suspected, are considered a major emergency and are explored through laparotomy
without any other investigations.
Pancreatic injuries should be treated by debridement and simple drainage unless
there is clinically obvious duct involvement.[25]
In grade I and II lesions the surgical measures are represented by hematoma
evacuation and hemostasis, infiltration with lidocaine, and neighborhood drainage to
avoid a pancreatic pseudocyst in case of unrecognized minor injuries of the pancreatic
duct.
Grade III of severity with lesions located to the left mesenteric pedicle requires
caudal pancreatectomy with or without splenectomy.
Grade IV lesions located to the right of superior mesenteric vein may be treated
by subtotal pancreatectomy or pancreatico-jejunal anastomosis with closure of the
cephalic stump duct.
In grade IV with ampulla lesions and massive disruption of the pancreatic head,
cephalic duodenopancreatectomy can be a solution but it is encumbered by a high
mortality rate considering the general status of the patient with multiple lesions and
hemorrhagic shock. The first concern is to stop bleeding and then, if the patient is
stable, cephalic duodenopancreatectomy is performed. If the patient is unstable, only
multiple drainages are performed after hemostasis and the resulting fistula dealt with at
a later operation, if necessary.[25,29]
The most common postoperative complications are fistulas and pancreatic
pseudocysts and the overall mortality is around 15%.
Splenic Trauma
The spleen is the most commonly injured parenchymatous organ in blunt
abdominal trauma, especially in traffic accidents associated with left ribs fractures. It
may be also injured during penetrating trauma and surgery. The consequence is the
intraperitoneal bleeding with hemoperitoneum and hemorrhagic shock.
Lesions on a pathological spleen in splenomegaly of any etiology may occur after
apparently minor trauma.
AAST classification of traumatic lesions of the spleen:[17]
Grade Lesion
I Hematoma - Subcapsular <10% surface area
Laceration - Capsular tear <1cm parenchymal depth
II Hematoma - Subcapsular, 10%-50% surface area; intraparenchymal, <5 cm in
diameter
Laceration - Capsular tear, 1-3cm parenchymal depth that does not
involve a trabecular vessel
III Hematoma - Subcapsular, >50% surface area or expanding; ruptured
subcapsular or parecymal hematoma; intraparenchymal hematoma >5 cm or
expanding
Laceration - >3 cm parenchymal depth or involving trabecular vessels
IV Laceration - Laceration involving segmental or hilar vessels producing
major devascularization (>25% of spleen)
V Laceration - Completely shattered spleen
Vascular - Hilar vascular injury with devascularized spleen
432
Abdominal Trauma
Clinical picture. Splenic trauma symptoms vary depending on the severity of
injury but the forefront syndrome is that of intraperitoneal hemorrhage and hemorrhagic
shock (tachycardia, tachypnea, restlessness, anxiety).
In severe forms, associated with liver damage or large vessels injuries, the patient
can die in few minutes even before applying of the first therapeutic measures.
In addition to general signs of acute anemia and hemorrhagic shock, there are
local signs of pain or left upper quadrant guarding and referred pain to the left shoulder
(the Kehr sign). Saegessers splenic point of tenderness is an area on left side between
scalenus medius and sternocleidomastoid muscle.
There are cases when the spleen ruptures under the intact capsule developing a
subcapsular hematoma. As the hematoma develops in the following days, the capsule
becomes more and more tensioned and suddenly it ruptures (spontaneously or after
minor trauma) leading to hemoperitoneum. This is the so-called two-stage splenic
rupture. It may occur after several days or even weeks.
In subcapsular hematoma and coagulated blood around the spleen, fixed dullness
on the left flank on percussion can be noticed representing the Balances sign.
Obliteration of the resonant note in the Traube's space can be also observed on
percussion.
In lower left hemithorax and left flank contusions, the surgeon should have a high
degree of suspicion regarding spleen rupture even if the patient is in a good condition
without signs of internal bleeding and should proceed to investigations such as
ultrasound and chest X-ray. Although imaging explorations have a good diagnostic
index, some minor injuries may go unrecognized at initial evaluation. Therefore, all
patients with trauma that may be associated with splenic lesions require periodic
reassessment.
The most used investigation for detecting spleen rupture and hemoperitoneum is
the ultrasound examination, which is fast, noninvasive, cheap, and can be performed at
bedside. Focused assessment with sonography for trauma (FAST) is a rapid bedside
ultrasound examination performed as a screening test for blood around the liver, in the
pericardium, around the spleen and in the pelvis. Ultrasonography reveals the
subcapsular or perisplenic hematoma, parenchymal rupture and the presence of fluid in
the peritoneal cavity.
Plain radiography of the abdomen and chest can show the left ribs fracture,
elevation of the left hemidiaphragm and the displacement of gastric fluid-air level.
In uncertain cases, the diagnostic peritoneal lavage (DPL) can be performed but it
is less used in nowadays when ultrasound and CT scan are available in most medical
centers.
CT with contrast highlights with high accuracy the splenic lesions.
Angiography is performed after CT scan, more frequently for primary therapeutic
management by angioembolization (gelfoam).
Treatment of splenic injuries, depending on severity, can be managed surgically
or nonsurgical.
The surgical treatment of choice is spleen conservation (hemostasis by electro-
coagulation, plasma-coagulation, local hemostatics, suturing, partial resection)
whenever possible to avoid post-splenectomy infections especially in children. Spleen
has an important role in immunity.[30,31] Splenectomy is the choice in severe and
multiple lesions of the spleen with hemodynamic instability.
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Abdominal Trauma
Indications for splenectomy are:[32]
Parenchyma explosion
Vascular lesions in the hilum
Intra-parenchymal massive hematoma
Patients in critical condition or other severe intra-abdominal injuries
Failure of conservative surgical techniques
Criteria for nonoperative management are:[33-35]
Patient in an intensive care unit with continuous monitoring of vital
functions. Daily or even several times a day ultrasound assess of lesion
development (expanding hematoma or hemoperitoneum requiring
surgery)
Surgical team always available for secondary bleeding
Hemodynamic stable patient
Negative abdominal scan
Absence of contrast extravasations on CT
Absence of other indication for laparotomy
Absence of other condition associated with high risk of bleeding
(coagulopathy, use of anticoagulants, etc)
Contraindications to nonoperative management are:[36]
Patients with hemodynamic instability
Generalized peritonitis
Other intra-abdominal injuries requiring surgical exploration
Portal hypertension
Higher-grade splenic injury with large volume hemoperitoneum
Refusal of blood transfusion
Altered neurologic status
Angiographic splenic embolization was first applied in 1981. It requires
specialized imaging facilities and an experienced vascular interventionalist. It is most
useful when employed selectively in hemodynamically stable patients who have CT
findings that include active contrast extravasation, splenic pseudoaneurysm, or large
volume hemoperitoneum.[36-39] The success rate is 57 to 93 percent.[36]
Liver and Biliary Tract Injuries
The liver is interested in 3545% of abdominal trauma.[40] Its lesions are
associated with a rapid loss of important quantities of blood and a high mortality rate
due to its rich vascularization, its low resistance to anoxia and difficult surgical
approach. Mortality varies depending on the cause of hepatic trauma being of 1015 %
in most cases but exceeding 25% in blunt trauma.[40]
AAST classification of liver trauma:[17]
Grade Lesion
I Hematoma - Subcapsular, <10% surface area
Laceration - Capsular tear, <1cm parenchymal depth
II Hematoma - Subcapsular, 10% to 50% surface area: intraparenchymal <10 cm in diameter
Laceration - Capsular tear 1-3 parenchymal depth, <10 cm in length
III Hematoma - Subcapsular, >50% surface area of ruptured subcapsular or parenchymal
hematoma; intraparenchymal hematoma >10 cm or
Laceration - expanding >3 cm parenchymal depth
434
Abdominal Trauma
IV Laceration - Parenchymal disruption involving 25% to 75% hepatic lobe or 1-3 Couinauds
segments
V Laceration - Parenchymal disruption involving >75% of hepatic lobe or >3 Couinauds
segments within a single lobe
VI Vascular - Juxtahepatic venous injuries; ie, retrohepatic vena cava/central major hepatic veins
Vascular - Hepatic avulsion
Clinical picture of liver trauma depends very much on the severity of injury.
Major liver trauma with vascular injuries or liver explosion is above the therapeutic
resources, patients dying at the scene, even before the first aid measures can be applied.
The main syndrome is that of intraperitoneal hemorrhage associated with intense
acute anemia. Finsterer's triad in liver trauma is represented by:
1. The paradoxical bradycardia in a patient with hypotension
2. Shock
3. J aundice
On local examination, abdominal contracture may occur due to extravasation of
bile into the peritoneal cavity.
Liver lesions can be manifested as a two-stage clinical picture: the initial liver
trauma with subcapsular hematoma and then the rupture of the capsule with
intraperitoneal hemorrhage.
Liver trauma can be associated with immediate or late complications:
Hemobilia, the penetration of blood into the biliary tree manifested by
Owen triad: colicky pain, upper gastrointestinal bleeding and mechanical
jaundice
Bile leakages due to biliary tree injuries
Hepatic necrosis due to vascular disruption leading to liver abscess with
abdominal pain, fever, jaundice and progression to severe sepsis and
hepato-renal failure
The diagnosis is based especially on imaging explorations.
Abdominal ultrasound may reveal the liver rupture, the subcapsular hematoma
and the presence of fluid in the peritoneal cavity, but lesions severity are difficult to be
assessed. Recent advancement of contrast-enhanced sonography improved the
diagnostic accuracy.[41] Furthermore, contrast-enhanced ultrasound can be used in the
follow-up of patients who are managed with nonoperative treatment, avoiding radiation
and iodinated contrast medium exposure.[42] The hemodynamically unstable patient
with a positive ultrasound is moved directly to the operating room without further
imaging.[40]
CT scan is the standard imaging study for stable patients assessing more
accurately the severity of liver injuries. It plays an important role in the nonoperative
management of liver injuries and it can guide the percutaneous drainage of biloma (a
collection of bile inside the abdomen that has become encapsulated) and intra-
abdominal collections.
Angiography is performed after CT scan if this highlights extravasation of
contrast in stable patients. Angioembolization is an important method of conservative
treatment in hemodynamically stable patients. The success rate is about 6893%.[43-45]
Positive DPL impose the surgical exploration.
The treatment of patients with liver trauma depends much on the severity of
lesions, hemodynamic status and associated intra-abdominal lesions.
435
Abdominal Trauma
If the patient, with hepatic laceration, intrahepatic or subcapsular hematoma, is
hemodynamically stable continuously from the trauma through evaluation, the choice
will be the conservative management with frequent imaging and laboratory
reassessment. Conservative management is not contraindicated even if the patient is
unconscious after an associated head injury if he is hemodynamically stable.[40] If the
CT scan shows extravasation of contrast, angiographic embolization is recommended if
it is available.
Contraindications to nonoperative management include the following:
1. Hemodynamically unstable patient
2. Associated intra-abdominal injuries that require laparotomy
3. Extravasation of intravenous contrast when angiographic embolization is
not available or unsuccessful
4. AAST grade III, IV, or V hepatic laceration and large hemoperitoneum
In liver trauma, the first aim of surgical treatment is to stop the bleeding and then
to repair the damages.
The most preferred approach is the median laparotomy that can be extended in
any direction as needed for a better access. It is the fastest way to reach into the
peritoneal cavity and offers the best access over the whole cavity. In delayed emergency
or scheduled operations the right subcostal incision is preferred (extended to the left if
necessary or the so-called "Mercedes" incision) because it confers the best access to the
liver.
Surgical procedures depend very much on lesions type, but generally, hemostasis
is the first concern. One of the fastest ways to reduce liver bleeding is the compression
of the affected lobe between two hands. This maneuver will confer the time necessary
for intraoperative volume rebalancing and temporary hemodynamic stabilization and
provides conditions for achieving final hemostasis. However, it does not help when
large vessels such as hepatic, portal or cava veins are damaged. Another method of
temporary hemostasis is the compressive wound packing.
Clamping the hepatic pedicle (stops the hepatic inflow), also known as the Pringle
maneuver (in1908 J . Hogarth Pringle described first the maneuver), allows surgeons to
evaluate traumatic liver injury. Pringle maneuver can be performed with atraumatic
vascular clamp or with fingers. (Figure 5) It has
also a diagnostic value: if the bleeding comes from
branches of the portal vein or hepatic artery, after
this maneuver, it stops or significantly reduces in
quantity; if bleeding comes from suprahepatic
veins or cava vein, bleeding will not stop.
Continuous Pringle maneuver can be maintained up
to one hour in normothermia without major effects
on a normal liver.[46,47] Intermittent clamping
(clamping periods of 10 to 15 minutes are
separated by 5-minute periods of declamping) is a
useful maneuver for cumulative periods exceeding
120 minutes without major intraoperative blood
loss or complications.[48,49]
Total vascular exclusion of the liver (TVE) (Figure 6) means: clamping of the
inferior vena cava (IVC) above and below the liver always preceded by inflow
occlusion (the Pringle maneuver). The right adrenal vein must be ligated and divided.
The phrenic veins (usually three) draining into the IVC should be also clamped or
436
Abdominal Trauma
ligated. This will completely exclude the liver from
the splanchnic and systemic circulations. However,
this maneuver is associated with significant
decrease of volume return to the heart, which can
severely complicate the post-operative course.
A less drastic clamping procedure is
represented by the selective clamping of the hepatic
veins themselves, leaving the caval flow
undisturbed.
Lesions of the suprahepatic veins or vena
cava are usually lethal. In suprahepatic veins lesions
the inflow clamping is not sufficient because the
hepatic veins remain patent and bleeding may
continue. Air embolism may be significant with a
low central venous pressure. Making temporary
hemostasis in these cases is very difficult but can be
performed in several ways. The total vascular
exclusion of the liver (TVE) is one of the choices.
To maintain a sufficient venous flow to the heart an
atrio-caval shunt can be performed by introducing a
catheter with balloon through the atrium into the
vena cava in suprarenal position. (Figure 7) Another
solution is the use of the Moore-Plicher balloon. A
catheter is inserted through the femoral vein into the
vena cava and the balloon is inflated in the place of
venous lesion (suprahepatic veins or cava vein) to
achieve hemostasis.[50]
Whichever method is chosen mortality in these cases exceeds 50%.
Definitive treatment methods of liver injuries are ranging from haemostatic
packing of liver wounds to liver transplantation.
The grade I and II represented by superficial lesions of the liver can be managed
by manual compression followed by hemostatic procedures such as electro-coagulation,
argon plasma coagulation or application of haemostatic sponges or sutures.
The grade III and IV of lesions may benefit from superficial hemostasis but most
often, other methods are needed such as:
Haemostatic suture of liver parenchyma for superficial wounds less than 3
cm deep
Hepatotomy with elective ligation of injured pedicles
Atypical or anatomical (controlled) liver resections
Haemostatic packing is used for serious grade III-V injuries especially in shocked
patients to prevent further blood loss. It can be performed in two ways: packing the tears
with gauze or introducing a high quantity of gauze into the hepatophrenic space to
achieve compression and hemostasis. The main risk is that of severe sepsis that may
follow. If hemostasis was achieved, after 24-48 hours gauzes have to be extracted. If
bleeding restarts after removing the gauze, the angioembolization could be a less
invasive method of hemostasis.
Biliary tract injuries are recognized by the presence of bile in the peritoneal
cavity.
437
Abdominal Trauma
Gallbladder lesions are solved by cholecystectomy unless gallbladder will be used
for a bilio-digestive bypass.
Injuries of the common bile duct are more serious because their repair may be
complicated by late stenosis. In case of linear lesions, without loss of substance,
suturing can be performed associated with Kehr type (T-tube) drainage. Major injuries
or complete section can be solved only by choledochojejunal anastomosis with a Roux-
en-Y loop.
External biliary drainage is recommended in any important hepatic laceration
even without obvious biliary injury to prevent postoperative biliary leakage.
Overall mortality of patients with liver injury is around 10%-20%.[51-55] The
main cause of death is exsanguination. Frequent postoperative complications in liver
trauma surgery are postoperative bleeding (unrecognized injury or transfusion
coagulopathy), infection (most commonly in ischemic territories due to ligation, en
block suture or embolization [53]) and biliary leakage.
Major Abdominal Vascular Injuries
Traumatic lesions of the large abdominal vessels are encumbered by a very high
mortality rate (30-60%), despite of all improvements in transportation and resuscitation
of patients.[56-59] Penetrating abdominal wounds are the main cause of vascular injuries
and the leading cause of death is exsanguination.
Vascular lesions are usually associated with other intra-abdominal injuries and in
up to 40% of cases, the damage involves at least two major vessels.
Injury severity is directly influenced by three factors:
1. The number of injured vessels
2. Location
3. Mechanism
The most serious are those that involve the aorta, and those produced by firearms.
The extent and morphology of aortic injuries vary widely, ranging from intimal
hemorrhage to complete transection. Aortic injury most commonly results from
transverse tears and can be segmental (55%) or circumferential (45%) and may be
partial (65%) or transmural (35%).[60]
From clinical point of view, two situations may be encountered: patients with
active bleeding or those with retroperitoneal hematoma. Patients with retroperitoneal
hematoma present a transitional shock with hypotension that responds to volemic
rebalancing, but hypotension reappears when the hematoma is surgical explored or
when the hematoma breaks into the peritoneal cavity. The bleeding usually comes from
an injured vein.
Patients with active bleeding (frequently arterial) present shock and hypotension
refractory to resuscitation associated with abdominal distension. A specific sign is the
absence of unilateral femoral pulse, suggestive for a lesion of the common or external
iliac artery.
Injuries to major vessels require emergency surgery. The first goal is to stop the
bleeding by various maneuvers of temporary hemostasis (compression, clamping, and
endovascular balloon) followed by final hemostasis either by suture, reconstruction or
sometimes vessel ligation. Endovascular stent-grafting repair is a valuable alternative to
open aortic repair [61,62] but rarely applied in abdominal trauma (more frequently in
438
Abdominal Trauma
thoracic aorta lesions) because vascular lesions are frequently associated with other
internal organs lesions, which require laparotomy. Choosing an open surgical repair
versus an endovascular stent graft depends upon physician expertise, clinical status of
the patient and clinical setting.
Mortality in traumatic abdominal aorta injury ranges from 70 to 90%.[63-66]
Retroperitoneal Hematoma
Retroperitoneal hematoma represents the accumulation of blood into the
retroperitoneal space. Sources of bleeding are various, but in order of frequency, they
are:
Lesions of the retroperitoneal organs (kidney, pancreas)
Fractures of the pelvis or lumbar spine
Lesions of retroperitoneal vessels
Retroperitoneal hematoma due to renal trauma has a high lateral topography but it
can descend to the pelvic-subperitoneal space.
Bleeding from large vessels (aorta or cava) is associated with a very high
mortality rate.
In terms of size, retroperitoneal hematoma is considered:
Very large, when it extends from the upper pole of the kidney to the
Douglas
Large, when it does not exceed the lower renal pole
Medium and small, when is located only in the pelvis or around the
various organs (kidney, duodenum, pancreas, etc.)
By its presence, the hematoma is a permanent cause of irritation of the
retroperitoneal nerve plexuses and may result in ischemic necrosis of the intestine by
compression on mesenteric vessels. Paralytic ileus causes serious fluid and electrolyte
disorders, leading to complex shock. The toxic component of the shock results from the
resorption of blood.
Diagnosis relies especially on imagistic findings but a careful clinical
examination may reveal some clinical signs suggestive for retroperitoneal hematoma
such as palpation of the hematoma mass, abdominal distension and muscular guarding
in lateral quadrants and dullness in the lower parts of the abdomen on percussion.
Imagistic investigations, which help the diagnosis, are:
Plain abdominal radiography which, may reveals bone lesions, signs of
paralytic ileus, and decrease of psoas shadow
Abdominal ultrasound, which reveals fluid collections and kidney lesions
CT scan with contrast is the most important investigation
Other investigations as needed: cystography, selective arteriography, etc.
A central venous catheter placed into the femoral vein is useful in
performing the cavagraphy but also for urography, harvesting blood for
laboratory investigations and for fluid rebalancing.
Evolution of post-traumatic retroperitoneal hematoma may be complicated in
many cases. Complications could be represented by:
The resorption syndrome is dominated by the development of variable
intensity jaundice resulting from blood resorption. The most important
439
Abdominal Trauma
aspect is however, the increasing of K+ion levels very dangerous with
concomitant development of the acute renal insufficiency (source of
hyper-potassium levels).
Suppuration can progress to diffuse cellulites, with fatal outcome.
Favoring circumstances are the coexisting organ damages (rectum, colon,
etc.).
Lymphorrhagia and pancreatic fistula are other possible complications.
Retroperitoneal seroma develops due to incomplete resorption. It evolves
like a retroperitoneal compressive tumor that may rupture into the
peritoneal cavity.
Early rupture of hematoma into the peritoneal cavity explains the
presence of the concomitant hemoperitoneum without any lesions of
intraperitoneal organs.
Late rupture into the peritoneal cavity is also possible.
Retroperitoneal liposclerosis is represented by intense adhesions around
retroperitoneal anatomical structures.
Treatment may be surgical or conservative (observational or angioembolization).
In 1984, Sheldon [67] introduced a treatment principle founded on a location-
based classification of traumatic retroperitoneal hematoma as central-medial (zone I)
flank or perirenal (zone II) and pelvic (zone III). (Figure 8)
The surgical indication will be established
after immediate resuscitation, aimed to control
the traumatic shock and hemorrhage and
maintenance of the important functions. Absolute
surgical indication of surgery in emergency is the
retroperitoneal hematoma of vascular origin.
Relative indication for surgery is the hematoma
due to bone fractures of the pelvis with no
vascular or visceral lesions. Conservative
treatment will be applied to small hematomas, or
hematomas without major vascular lesions or
visceral lesions.
The judgment of whether and when to
explore the retroperitoneal hematoma is guided
by the mechanism of injury (blunt or penetrating)
and the location of the hematoma. Usually
hematoma localized to the upper central area after penetrating trauma implies injury to
the great vessels and always requires urgent surgical exploration.[68-70] Exceptions
include isolated lateral perirenal hematomas that have been carefully staged by CT and
some lateral pericolonic hematomas.[68] Retrohepatic hematomas without obvious
active hemorrhage are not opened. For zone III of retroperitoneal hematoma, associated
with pelvic fracture the attitude is no exploration in blunt pelvic trauma and surgery for
penetrating trauma.
Some ongoing hemorrhage may respond to therapeutic embolization.[71]

440
Abdominal Trauma
Prognosis depends on the period of evolution as follows:
1. During the first 24-48 hours, the prognosis depends mainly on intensity of
the shock (traumatic, hemorrhagic) and the involvement of the celiac plexus
(vagal-sympathetic irritation).
2. During the first 3-5 days, the prognosis may be worsened or remains
reserved because of persistence or recurrence of shock, occurrence of acute
renal failure, intestinal paresis.
3. The local complications period affects less the vital prognosis.
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444

Abdominal Wall Pathology
ABDOMINAL WALL PATHOLOGY

1. General aspects of anatomy of the abdominal wall
2. Hernias generalities
3. Specific types of hernias
4. Incisional hernias
5. Eviscerations
6. Diaphragmatic hernias
7. Diaphragmatic ruptures
8. Diaphragmatic relaxations

Definitions
Hernia - represents the protrusion of an organ or a part of it, outward the
abdominal cavity (under the skin or into the thoracic cavity) through a congenital or
acquired route, located in a naturally weaker zone of the abdominal wall (hernial
region), so that the structural integrity of the abdominal wall is not destroyed.
Incisional hernia - represents also the protrusion of an organ or a part of it, under
the skin, but usually through a parietal defect located in any region of the abdominal
wall, most often as a consequence of the destruction its integrity such as after abdominal
surgery.
Evisceration - represents the extrusion of an organ or part of it outside the
abdominal cavity in direct contact with the atmosphere as a consequence of a breach
through the entire abdominal wall thickness.
1. General Aspects of Surgical Anatomy of the
Abdominal Wall
Along the thorax and pelvis, the abdomen is a component of the human body
trunk. The abdominal cavity houses most of the organs belonging to the digestive
system, and part of organs of the urinary and reproductive system. The trunk walls are
formed by somatic elements: bones, joints, muscles, fascia, vessels, and nerves.
Posterior wall of the thorax and abdomen form the
back.
The abdominal cavity has an egg-shape
form with the base oriented upwards, but for
descriptive reasons it is compared to a
parallelepiped with six walls, which are: (Figure
1)
Anterior or ventral wall
Posterior wall
Two lateral walls
Superior wall represented by the
diaphragm
Inferior wall represented by the pelvic
floor
445
Roles of the abdominal wall are:
Contention - keeps the viscera in a closed cavity.
Protection - provides protection from outside aggressions, maintaining local
constant conditions.
Participation in physiological processes by increasing the intra-abdominal
pressure (coughing, phonation, defecation, urination, etc.) and to the kinetic of
the trunk and of the whole body in general.
Features of the abdominal wall are:
It is an active structure, mobile (not a rigid tube) able to change its shape and
size, thus changing the intra-abdominal pressure.
It is a musculo-fibrous structure (muscles, fascia, and ligaments) and bones
(column, ribs, and pelvis) on which the soft parts are inserted.
Its structure is diverse being different from one region to another depending on
the function played by that region.
Normal abdominal wall structure is symmetrical.
Consisting of various anatomical elements, the abdominal wall has naturally some
areas of low resistance or weakness.
The posterior wall is the strongest being composed of spine column, the last ribs,
muscles (latissimus dorsi, serratus posterior, erector spinae, paravertebral group of
muscles quadratus lumborum, psoas, etc.), aponeuroses and fascias (toracolumbar
fascia). Abdominal cavity is separated from the posterior musculoskeletal plane by the
retroperitoneal compartment, which contains fatty tissue, important blood and lymphatic
vessels, nerves and organs such as kidneys with ureters, adrenals, duodenum, and
pancreas. Areas of low resistance of this wall are represented by the Petit triangle and
Grynfeltt quadrangle, rarely manifested by lumbar hernias.
The Jean Louis Petit lumbar triangle (Figure 2) is delimited between the iliac
crest (inferior), the rear edge of the external oblique muscle (superior) and latissimus
dorsi muscle edge (posterior). Through this area are passing the subcostal nerve, the
iliohypogastric nerve, the ilioinguinal nerve and the last two lumbar veins. Hernia in
this region is relatively easily visible.
The Grynfeltt quadrangle (Figure 3) is delimited between the posterior-inferior
serratus muscle, the last rib, the internal oblique muscle and the paravertebral muscles.

The resistance structure of lateral walls is provided by the following abdominal
muscles: external oblique, internal oblique and transverse abdominis. Because of the
flattened shape of the abdomen, often the lateral walls are divided between the anterior
and posterior walls and referred as anterolateral and posterolateral wall. In this region,
446
the weak zone is represented by the semilunar line o
and right Spigelian points (the intersection of
semilunar line with the Douglas arch). (Figure 4)
This is the region of connection between the lateral
muscles of the abdomen and the rectus abdominis
muscles.
f Spiegel and especially by the left
The anterior wall of the abdomen is the
largest area and has the lowest naturally resistance.
Layers of the anterolateral abdominal wall are:
Skin (epidermis, dermis) with appendages
Fatty tissue with fibrous condensations
(fascia Scarpa, fascia Camper, fascia
cribriformis, etc.)
Muscles and aponeuroses
Fascia transversalis
Preperitoneal space of Bogros
Peritoneum
Muscles are represented by a paired rectus abdominis muscles, running vertically
on each side of the anterior wall separated by a midline band of connective tissue called
the linea alba. Rectus abdominis extends from the xiphoid process and costal cartilages
of ribs V to VII to the pubic bones and are contained in the rectus sheaths, which
continue the aponeuroses of the lateral abdominal muscles. The linea alba is a structure
that is made of intersected fibers connecting the rectus sheaths. Weak zones are
represented by linea alba itself and by umbilicus. In the lower part of the abdominal
wall, there are also natural zones of weakness represented by inguinal and femoral
canal, which will be detailed at section of inguinal and femoral hernias.
The diaphragm represents the superior wall of the abdominal cavity separating it
from the thoracic cavity. Natural zones of weakness of this wall are the passages zones
of the anatomical elements through the diaphragm (esophagus, aorta, cava vein, nerves,
etc.).
The inferior wall is represented by the pelvic floor (pelvic bones, the urogenital
diaphragm and the pelvic diaphragm essentially formed by the levator ani muscle)
which has also naturally weak zones (obturator canal, sciatic foramen, and others).
Hernias in this region are very rare.
The structural integrity of the abdominal wall can be however altered by
pathological processes that will decrease its resistance leading to hernia formation.
For a better orientation and description of the pathological processes, the
abdominal cavity has been divided into
quadrants and regions with projection on the
anterior abdominal wall. (Figure 5) The scheme
with nine quadrants includes: epigastrium,
umbilical, hypogastrium, right and left
hypochondrium, right and left flanks and right
and left iliac regions. The four quadrants scheme
includes the upper right, upper left, lower right
and lower left quadrants.
447
2. Hernias - Generalities
Internal hernia represents an exception from the definition of "hernia represents
the protrusion . outward the abdominal cavity", because it represents the penetration
(or intrusion) of the intestines through preformed or acquired holes or cavities, inside
the peritoneal cavity.[1,2] (Figure 6)
Examples of intra-abdominal preformed
holes or cavities that may cause internal hernias
are:
Through the foramen of Winslow - the
TREITZ hernia
Inside the small pouches formed by the
peritoneal folds (periduodenal,
periappendicular, retrocecal or
intersigmoidian) - the RIEUX hernia
Acquired internal hernias appear when
intestines engage through the unclosed holes of
the mesocolon or mesentery intentionally
performed during different kind of operations. For
example, the PETERSEN internal hernia appears
when intestine passes through the mesocolon
defect. That loop is at risk for incarceration and
strangulation with intestinal occlusion.
Etiopathogenesis.
Abdominal wall hernias occur due to an imbalance between the intra-abdominal
pressure (Figure 7) and the abdominal wall strength. From this point of view, hernias
can be classified as hernias of force (caused by
excessive intra-abdominal pressure) and hernias
of weakness (due to the impaired strength of the
abdominal wall). Factors that lead to high intra-
abdominal pressure acting against the abdominal
wall are:
Voluntary factors - physical effort
Involuntarily factors - obesity
Physiological factors - pregnancy
Pathological factors
o Intra-abdominal - ascites, tumors, etc.
o Extra-abdominal - trauma
Abdominal wall strength and structural
integrity depend on factors such as:
Constitutional (genetic) - congenital hernias
Nutritional - obesity, vitamin deficiencies, etc.
Educational - sports
Pathological - trauma, subnutrition, paralysis, etc.

448
Classification
Depending on the way of development and the structure of the abdominal wall,
hernias may be classified as:
Congenital
o Embryonic - the parietal defect appears before the fourth intrauterine months
when the peritoneum is not yet developed. There is no hernial sac.
o Fetal - the parietal defect appears after the fourth intrauterine months when
the peritoneum is already developed and the hernial sac is present.
Of the small child
Of the adult
o Acquired
Of weakness - usually there are multiple
or bilateral hernias such as the bilateral
direct inguinal hernias
Of force - caused by the increased intra
abdominal pressure
Depending on the anatomical region hernias are:
Ventral hernias (Figure 8)
o Inguinal - developed in the inguinal region
o Femoral - through the femoral canal
o Umbilical - thorough the umbilicus
o Periumbilical - around the umbilicus
o Epigastric - of the linea alba located in the
epigastric region
By frequency: [3]
o Inguinal hernia 71-75%
o Umbilical hernia 1-10%
o Epigastric hernia 5 %
o Femoral hernia 3.7-5 %
o Others
o Spigelian hernia - lateral hernia at level of
Spiegel's line
Posterior hernias
o Lumbar - which develop through the Petit
and Grynfeltt spaces
Of the pelvic floor
o Sciatic hernia - through the sciatic foramen, a passage from the pelvis to the
gluteal and perineal regions formed by the hipbone, the sacrospinous
ligament, and the sacrotuberous ligament. (Figure 9)
o Obturator hernia - through the obturator canal, a passageway for the
obturator artery, obturator vein, and obturator nerve. (Figure 10)
o Perineal hernia - through the urogenital diaphragm

449

Lateral hernias - between levator ani
and sacrococgigian muscle
Medial anterior - Elytrocele [Gr. elytron
= sheath, kele = hernia]. The sac is
represented by an extension of the
peritoneal cavity (pouch of Douglas)
between the rectum and the posterior
wall of the vagina (bulging into the
vagina). (Figure 11)
Medial posterior - Hedrocele [Gr. hedra
= anus] is a rectal hernia. The extension
of Douglas pouch protrudes through the
posterior wall of the rectum. (Figure 12)
Of the superior abdominal wall -
diaphragmatic hernias
Morphopathology. Anatomical components of the
hernia are:
1. The hernial canal
2. The hernial sac
3. The content of the sac
In most cases, the canal is a real canal, which
has two openings: internal and external, but there are
cases when the canal is absent being represented
only by a hole in the abdominal wall (eg. umbilical
hernia). The trajectory of the canal may be
perpendicular to the abdominal wall and that
determines the direct hernias, or oblique that
determines the indirect or oblique hernias. (Figure
13)
The sac is an extension of the parietal
peritoneum communicating with the peritoneal
cavity. The sac has three portions: (Figure 14)
1. The neck
2. The body
3. The fundus
Depending on its shape, the sac can be:
globular, pear-shaped, conical, cylindrical, spherical.
The sac can be free or adherent to the surrounding
tissues. Hernias may have a single sac or multiple
sacs.
The sac may be partially or totally absent. The sac is absent in embryonic hernias
when the peritoneum is not yet developed. Partially absent sac is found in sliding
hernias, when the sliding organ (usually the colon) drags along a part of the peritoneum,
or in other words, the organ itself represents a part of the hernial sac. (Figure 15) The
colon and the urinary bladder are the most often involved in such hernias.
450

Depending on the position of the sac inside the hernial canal (the degree of
protrusion) hernias are: (Figure 16)
Hernial point - the sac protrudes
into the canal being located at the
internal ring
Interstitial hernia - the sac is
contained inside the canal
between the internal and external
openings
Complete hernia - the sac
protrudes under the skin. It has
exceeded the external ring
The content of the sac could be any intra-
abdominal organ except the pancreas,
which is a retroperitoneal organ well, anchored to the posterior wall. Depending on
which organ is contained inside the sac, hernia may be:
Enterocele - the sac contains intestines
Epiplocele - the sac contains greater omentum
Littre hernia - the sac contains the Meckel's diverticulum
Garengoff hernia - the sac contains the vermiform appendix
Littre hernia - the sac contains Meckel's diverticulum
Depending on evolution, hernias can be classified as:
Uncomplicated hernias (simple, reducible hernia)
Complicated hernias (irreducible, incarcerated, strangulated, etc.)
A. Uncomplicated Hernia (Simple, Reducible Hernia)
Clinical picture
The onset of symptoms is insidious with mild pain in a hernial region during
efforts but the pain gradually disappears. The patient observes the appearance of a bulge
that gradually increases in volume, especially in standing position and on physical
efforts, which is spontaneously reducible in supine position or after manual maneuvers.
Other symptoms may be present, depending on which organ is herniated.
Physical examination. Physical signs are dominant.
On inspection in standing position, a tumor in a hernial zone, which is bulging
under the unmodified skin of the region, can be observed. (Figure 17) During coughing,
the tumor increases its volume. In supine position, the bulge reduces its volume or
disappears.
451

On palpation, the bulge is of soft elastic consistency, painless, and can be reduced
by taxis into the abdominal cavity on a trajectory through a hole of the abdominal wall.
Asking the patient to cough, the examiner feels that the hernial sac pushes the fingers
that are still in the hernial canal. This is the impulsion sign of the hernia. After removing
the fingers, the hernial sac bulges again under the skin spontaneously or after coughing.
This is the expansion sign of the uncomplicated hernia.
On auscultation of the hernial zone, nothing or intestinal sounds can be heard.
Symptomatic hernias are hernias that represent a symptom of another disease,
usually more serious, which produces an increase of intra-abdominal pressure (cirrhosis
with ascites, intra-abdominal mass, tumors of the colon, prostate adenoma, chronic
cough, etc.) (Figure 18)

Coercible hernia is the hernia whose content remains inside the abdominal cavity
in standing position after reduction maneuvers.
Incoercible hernia is the hernia that immediately restores after taxis (usually
hernias with wide opening).
The positive diagnosis of an uncomplicated hernia is easy, based on history and
physical examination. Usually there is no need for other investigations. Clues for
diagnosis are:
A recurrent bulging tumor under the skin, which usually appears after physical
effort, and gradually increases in volume (especially during physical effort), but
reduces itself or disappears in supine position or by taxis,
The presence of a tumor in a region of low resistance of the abdominal wall,
which after digital reduction presents the signs of impulsion and expansion
during coughing effort
Differential diagnosis could be difficult sometimes, especially in complicated femoral,
perineal, obturator hernias and posterior hernias. Differential diagnosis should include
other tumors of the respective anatomical region. In difficult cases, complementary
452
investigations are necessary represented by: ultrasound, CT-scan, barium swallow or
enema, cystoscopy, phlebography, and others.[4-6]
Treatment. The surgery is the rule in hernia treatment and the contraindication is the
exception. Contraindications are represented by:
Altered general condition of the patient who cannot
withstand the operation or the anesthesia. In this case,
orthopedic methods with content devices (harness, belts)
can be recommended. (Figure 19)
Relative contraindications are represented by
neighborhood suppurations (should be treated before
surgery), coagulation disorders and symptomatic hernias,
in which case the causing disease must be treated at first.
The aims of surgical treatment are to repair the parietal
defect and reinforce the abdominal wall to prevent the relapse. There are three
operative steps in classical open surgery:
1. Finding and dissecting the sac
2. Treating the content and resection of the sac (in most cases, but not
always, depending on hernia type)
3. Closing the parietal defect and/or reinforce the abdominal wall
The open hernia repair (of an uncomplicated hernia) is considered an aseptic
operation, so that wound suppuration is an indicator of compliance with aseptic intra
and postoperative maneuvers.
The prognosis is favorable, hernia being compatible with life as long as incarceration
or strangulation does not occur. Postoperative prognosis is also good, with a very low
relapse rate according to compliance with postoperative physical efforts avoidance, the
state of the abdominal wall and surgical technique used.
B. Complicated Hernias
The most frequent complications are represented by:
Incarceration - appears when intestinal loops (or other organs) are unable to
return into the abdominal cavity (are trapped inside the sac) due to a
compression at level of internal or external ring, but the blood supply of the
organs inside the sac is not particularly affected. However, a bowel obstruction
will develop (when intestines are interested) and eventually will lead, through
distension, to ischemic lesions (similarity to a jailed prisoner who has not yet
been sentenced to death by hanging).
Strangulation - appears when, besides the fact that the bowel (or other organ)
can not return into the abdominal cavity, the compression at level of
strangulation ring is so intense that it affects the vascularization and the
intestinal loop "dies" (similarity to a jailed one convicted to death by
strangulation - hanging).
Other complications are:
Hernias with adhesions
Voluminous hernias with loss of domain ("lost their right of domicile)
Tumors in the hernial sac
Peritonitis in the hernial sac
453
Frequency of incarceration is about 3-15%.[7-9] Incarceration appears usually after an
intense physical effort or after a rich meal. Hernias with narrow ring are more prone to
incarceration and strangulation. Femoral hernias incarcerate more frequently due to the
inextensible (rigid) hernial canal. The inguinal and femoral hernias incarcerate more
frequently on the right side because of the longer mesentery on this side.[3,5]
Morphopathology. There are three stages of evolution:
1. Venous stasis stage - the intestinal loop is distended
with edematous and thickened walls; the color is
violet, cyanotic; (Figure 20) there is a lymphatic and
venous stasis, but peristaltic movements are present.
On the peritoneal surface, petechiae are present. In
the sac, there is serocitrin fluid and then
hemorrhagic exudate. The mesentery is edematous
with blood suffusions. Lesions in this stage are
reversible.
2. Stage of ischemia and thrombosis - the intestinal
loop becomes darker without peristaltic movements; the fluid in the sac is
bloody, and there is a mesenteric edema with blood suffusions. The content of
the intestine is bloody due to ischemic lesions of the mucosa. In most cases,
lesions are irreversible requiring resection of the affected intestinal loop.
3. Stage of necrosis (gangrene and perforation) - intestinal loop is soft, greenish-
black with necrotic spots, progressing toward perforation and the fluid in the sac
is fecaloid. This is an irreversible stage. Bowel lesions are the most serious at the
level of strangulation ring. Lesions progress to a pyo-stercoral phlegmon with
acute inflammatory signs and then fistulization to the skin. (Figure 21) The
length of strangled loop can be variable (usually 10-20 cm) reaching even to 1 m
in large umbilical hernias.

Particular forms of incarcerated hernias:
Richter hernia is represented by a lateral pinch of the intestine. (Figure 22)
Maydl hernia is a retrograde strangulation of the intestine, in "W-shape.
Usually the intra-abdominal intestinal loop is much more affected by ischemic
lesions than those in the sac are, and that is the reason why intestines must be
carefully inspected during operation. (Figure 23)
454

Clinical picture of incarcerated hernia
Symptoms are represented by sudden onset of intense pain in a hernial region,
usually after physical efforts or a rich meal. The hernial bulge grows in volume,
becomes increasingly painful and is not reducible. Intestinal occlusion phenomena
occur when intestinal loops are in the sac (the transit for gases and faces stops, bloating
and vomiting appear). When only the omentum is present in the sac, the occlusion
symptoms are usually absent.
In Richter hernia (lateral pinch), symptoms may be confusing because there is no
intestinal occlusion and even diarrhea may be present yet the intestinal wall necrosis is
progressing.
On inspection, a bulge can be observed in a hernial region, which does not reduce
in supine, nor increases in volume during the effort of cough. On palpation the bulge is
very painful, of hard consistency, cannot be reduced by taxis and the sign of expansion
is absent. On percussion, there is a painful dull sound.
Later in evolution, the signs of intestinal obstruction with distended meteoritic
abdomen appear. In advanced stages, signs of pyo-stercoral phlegmon are present and
the evolution is to death if the patient is not operated.
The diagnosis is usually easy when a history of hernia is present, but sometimes it is
very difficult (obturator hernia, sciatic hernia, some femoral hernias, etc.) In most cases
of difficult diagnosis, the patient is operated for intestinal obstruction and the hernia is
an intraoperative surprise (most often in case of obturator hernia and internal hernias).
Differential diagnosis is made with other painful tumors in that zone.
Treatment. Incarceration is an absolute indication for surgical treatment. Taxis, the
manual reduction of hernia by manipulation, generally should be avoided. Exceptions to
this rule are hernias in newborns and small children where the immersion in warm bath
and administration of sedatives and muscle-relaxant drugs may lead to resolution of
incarceration. Taxis may lead to severe complications that endanger the patients life.
Complications of taxis are: (Figure 24)
The false reduction of the sac content.
The intestinal loop remains incarcerated
inside the abdominal cavity.
Intra-abdominal reduction of an unviable
intestine will lead to peritonitis.
Perforation of the intestinal loop.
Surgical treatment has four stages:
1. Discovering the sac and the strangulation
site
2. Sectioning the ring of strangulation
(Kelotomy - introduced by Ambroise
455
Pare)
3. Treatment of intestinal lesions
4. Reinforcement of the abdominal wall
Options for treatment of intestinal or greater omentum lesions are:
Lidocaine infiltration of the mesentery to relieve angiospasm in cases when the
intestine is at limit of viability
Circular invagination by sutures (clogging) of the intestinal ring of strangulation.
Remember that the most intense lesions are at this site. (Figure 25)
Segmental intestinal resection with end-to-end or side-to-side anastomosis
(Figure 26)
Resection of the omentum when the omentum is the affected organ

Voluminous hernias with loss of domain
In large parietal defects, most of the greater omentum and intestines are located
outside the abdominal cavity so that it shrinks and the content of the sac cannot be
replaced into the abdominal cavity. (Figure 27) It is not
advisable to operate such a hernia before a thorough
assessment of the patient. Sometimes, the greater omentum
resection or/and a portion of the intestine is required in
order to reduce the organs into the abdominal cavity, but
surgeons generally avoid such situations. A forced
reduction will produce a high intra-abdominal pressure that
will lift the diaphragm and limit its mobility leading to
serious cardiorespiratory disorders and possible death of the
patient.
Goni Moreno, distinguished surgeon from Argentina,
50 years ago, developed a method in which a progressive
amount of room air was injected preoperatively into the
peritoneal cavity over a period of weeks. Progressive preoperative pneumoperitoneum
prepares the hernia with loss of domain for operation. The patient becomes adjusted to
an increased intra-abdominal pressure and tolerates the sudden reduction of the viscera
during the repair, free of respiratory distress as it occurs with the standard technique. In
nowadays, large parietal defects can be repaired by a tension free procedure using
special meshes that does not adhere to the intestines.
456
3. Specific Types of Hernias
A. Inguinal Hernia
Anatomical aspects. The inguinal region is a symmetrical triangular shape region
delimited by the lateral margin of the rectus abdominis muscle, the Malgaigne line (the
skin reflection of the inguinal ligament of Poupart) and the interspinous line (a line
drawn between the both anterior-superior iliac spines). (Figure 28)
Layers of this region are represented by:
Skin
Campers fascia (fatty condensation)
Scarpas fascia (membranous)
Deep fascia
External oblique muscle with its aponeurosis
Internal oblique and transverse abdominis
muscles, which form the conjoint tendon
Transversalis fascia
Preperitoneal space (filled with fat) of Bogros
Parietal peritoneum
The region is crossed by anatomical elements such as:
The spermatic cord in men contained into the inguinal canal
The round ligament in women, also into the inguinal canal
Inferior epigastric vessels, and
Ilioinguinal, iliohypogastric and genitofemoral nerves
The inguinal canal is 4 cm long, being located just above the inguinal ligament. (Figure
29) The openings of the canal are the deep inguinal ring (the entrance) and the
superficial inguinal ring (the outlet). It carries the spermatic cord in males or the round
ligament of the uterus in females, and also the ilioinguinal and genitofemoral nerves.
The walls of the canal are represented by:
The anterior wall, by the aponeurosis of the external oblique muscle
The posterior wall, by transversalis fascia and the conjoint tendon
The roof, by the internal oblique and transverse muscles
The floor, by inguinal ligament of Poupart and lacunar ligament of Gimbernat

The Hesselbachs triangle is an important area of weakness as it is the site for direct
hernias. The triangle has the following borders: (Figure 30)
Medial - the border of rectus abdominis muscle
457
Inferior - the inguinal ligament
Lateral - the inferior epigastric vessels

The spermatic cord begins at the deep inguinal ring located lateral to the
epigastric vessels, then passes into the inguinal canal and exits at the superficial inguinal
ring and finally ends in the scrotum at testis. The spermatic cord is covered by:
Internal spermatic fascia (derived from transversalis fascia)
Cremasteric fascia (derived from internal oblique)
External spermatic fascia (derived from external oblique)
The spermatic cord contains: (Figure 31)
Ductus deferens (and its artery)
Testicular artery
Cremasteric artery
Panpiniform plexus
Genital branch of the genitofemoral nerve
Sympathetic nerve fibers
Lymphatic vessels
The fibrous vestige of the processus vaginalis

Classification
Depending on the degree of progression of the sac through the inguinal canal,
inguinal hernias can be classified as: (Figure 32)
1. Hernial point
458
2. Interstitial hernia
3. Inguino-pubian (bubonocele)
4. Inguino-funicular
5. Inguino-scrotal (labial)

Looking from inside the abdominal cavity to the hypogastric region, three folds of
peritoneum can be noticed: one median determined by the urachus, two medial
produced by umbilical arteries, and two laterals produced by inferior epigastric vessels.
These three folds delimit between them three fossae: lateral, medial, and supravesical,
which are sites of weakness favorable for development of three types of inguinal
hernias, respectively external oblique, direct and internal oblique hernias. (Figure 33)

Indirect (congenital) hernia is the most common type of inguinal hernias. The hernial
sac is outpunching lateral to the inferior epigastric vessels. Abdominal content enters
the deep inguinal ring via the hernial sac, a congenital abnormality, represented by the
persistent processus vaginalis. The sac is elongated, pear shaped. The hernia then passes
459
the full length of the inguinal canal to exit through the superficial ring and enters the
scrotum.
Direct (acquired) hernia develops medial to the epigastric vessels and the sac is
protruding through an area of weakness in the posterior wall of the inguinal canal
represented by the Hesselbachs triangle. The sac is globular with a large orifice, so it
rarely incarcerates. The sac rarely descends into the scrotum. It is considered a hernia of
weakness and usually appears on the both sides in elderly. In the table below, there is a
comparison between those three types of hernia. (Table 1)
Table 1 - Comparison between features of inguinal hernias.
Oblique external hernia Direct hernia Oblique internal hernia
Frequent
Congenital
Hernia of force
Through the internal inguinal
ring
The sac has a long neck, pear
shaped
It can be reduced by an upward
trajectory and from medial to
lateral
The content of sac in most
cases is represented by
intestines or greater omentum
Frequent
Acquired
Hernia of weakness
Through the Hesselbachs
triangle
Globular shape sac
The sac is reducible by a
trajectory perpendicular on the
abdominal wall
Frequently bilateral in elderly
The content of the sac may be
intestines, omentum or bladder

Rare
Acquired
Hernia of weakness
Frequently the sac
contains the bladder
Differences between congenital and acquired hernia: (Figure 34)
Congenital hernia occurs through the persistent peritoneo-vaginalis canal (patent
processus vaginalis) in men and the Nuck canal in women. The sac is located
inside the spermatic cord and sometimes intestines are in direct contact with
testis (tunica albuginea) in men.
In acquired hernia, the sac is located outside the processus vaginalis or Nuck
canal, which is closed, and the organs in the sac never come in direct contact
with testicles, which are wrapped by tunica vaginalis.

There are four types of congenital inguinal hernia: (Figure 35)
1. Inguino-testicular
2. Funicular
3. Funicular with cyst of the spermatic cord
4. Associated with hydrocele
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Congenital inguinal hernia, especially in children, is frequently associated with
ectopic testicle. In this regard, there are three types of congenital hernia depending on
the testicle position: (Figure 36)
1. Inguino-preperitoneal, when the testicle is located at the internal ring and the
sac is under the peritoneum
2. Inguino-intestitial, when the testicle is located inside the inguinal canal and the
sac lies between muscular layers
3. Inguino-superficial, when the testicle is located at the external ring and the sac is
under the skin

Other classifications [10]
Many classification systems have been proposed but the most commonly used by
members of the American Hernia Society are the classical Indirect/Direct designation,
that of Nyhus, and that of Gilbert/Rutkow and Robbins.
Nyhus developed a classification designed for the posterior approach based on the size of the
internal ring and the integrity of the posterior wall. According to this scheme:
Type 1 is an indirect hernia with a normal internal ring
Type 2 is an indirect hernia with an enlarged internal ring
Type 3a is a direct inguinal hernia
Type 3b is an indirect hernia causing posterior wall weakness
Type 3c is a femoral hernia
Type 4 represents all recurrent hernias
In Gilbert classification there are five types of primary and recurrent inguinal hernias. Types 1,
2 and 3 are indirect hernias; types 4 and 5 are direct.
Type 1 hernias have a peritoneal sac passing through an intact internal ring that will not admit
1 fingerbreadth (ie,<1 cm.); the posterior wall is intact.
461
Type 2 hernias (the most common indirect hernia) have a peritoneal sac coming through a 1-
fingerbreadth internal ring (ie, 2 cm.); the posterior wall is intact.
Type 3 hernias have a peritoneal sac coming through a 2-fingerbreadth or wider internal ring
(ie, >2 cm.).
Type 3 hernias frequently are complete and often have a sliding component. They begin to break
down a portion of the posterior wall just medial to the internal ring.
Type 4 hernias have a full floor posterior wall breakdown or multiple defects in the posterior
wall. The internal ring is intact, and there is no peritoneal sac.
Type 5 hernias are pubic tubercle recurrence or primary diverticular hernias. There is no
peritoneal sac and the internal ring remains intact. In cases where double hernias exist, both
types are designated (eg, Types 2/4).
In 1993, Rutkow and Robbins added a type 6 to the Gilbert classification to designate double
inguinal hernias and a type 7 to designate a femoral hernia.
Laparoscopic classification. Closely related to Hyhuss based on transabdominal aspect,
classified hernias as:
Type 1 - Congenital with narrow internal ring
Type 2 - External with dilated internal ring
Type 3 - Posterior wall with defect
Type 3 A - Direct hernia
Type 3 B - Oblique hernia with a large internal ring - the inguinal canal is shortened
Type 3 C - Femoral hernia
Type 4 - Recurrent hernia
Unfortunately, all these classifications are imperfect making confusion between
inguinal and femoral hernia, which are two distinct anatomical types of hernia, so I
recommend the simplest classification that of Direct/Indirect for inguinal hernia.
Epidemiology
The incidence of inguinal hernias ranges between 15% and 47%, higher with age
and in men (approximately 25% in males and 2% in females).[11,12] Indirect inguinal
hernias are more common then direct hernias representing two thirds, [7] and a right-
sided predominance exists.
Clinical picture
The patient complains about the occurrence of a bulge in the inguinal region,
which progressively enlarges especially during physical efforts, but it reduces its
volume in supine, or taxis can reduce it. The main complaints are the unaesthetic aspect
of the region and the mild local pain.
On inspection, in standing position, a bulge can be observed in the groin
deforming the region. The bulge may be small, limited only to the inguinal region
(inguino-interstitial and inguino-pubian) or it may distend the scrotum in men or labia in
women (inguino-funicular or inguino-scrotal or labial). During the effort of cough, the
bulge enlarges. In supine position, the bulge reduces its volume or completely
disappears.
On palpation, the bulge has a soft consistency and it can be reduced into the
abdominal cavity over a trajectory, which depends on the hernia type. In oblique-
external hernias, the trajectory is upwards and from medial to lateral through the
external inguinal ring, above the inguinal ligament. In direct hernias, the trajectory is
perpendicular to the abdominal wall above the inguinal ligament.
On auscultation, intestinal sounds can be heard when there are intestines are
present inside the sac.
Both inguinal regions should be inspected and as well, both testicles and inguinal
lymph nodes should be palpated.
462
Diagnosis is simple based on clinical aspects. In rare cases, ultrasound examination is
necessary. Digestive system examination with contrast (barium swallow or enema) may
highlight the presence of intestines inside the hernial sac, but this examination is rarely
necessary. Differential diagnosis includes:
Femoral hernia - Inguinal hernial sac is reducible over the inguinal ligament
whereas in femoral hernia the sac is reducible below the inguinal ligament.
Between different types of inguinal hernia - oblique hernia is reducible from
down upwards and from medial to lateral, while direct hernia is reducible on a
trajectory perpendicular to the abdominal wall.
Hydrocele - The bulge is not reducible; its consistency is elastic; the testicle
cannot be palpated; ultrasound examination highlights the fluid collection in
tunica vaginalis. Remember that inguinal hernia could be associated with
hydrocele. (Figure 37)
Varicocele - Even though the bulge is reducible by palpation, the external aspect
of scrotum is different compared to inguinal hernia. The dilated veins of
pampiniform plexus often appear visibly through the scrotum and feel like "a
bag of worms". (Figure 37, 38)
Cyst of the spermatic cord - It is sometime difficult to differentiate from a hernia
but the tumor does not reduce in supine or at palpation and ultrasonography
highlights the cyst.
Inguinal lipoma - The tumor is of soft, elastic consistency and does not reduce in
supine or at manipulation. The signs of expansion and impulsion are missing.
A tumor of the testis - The testicle itself is enlarged. The tumor cannot be
reduced by taxis and ultrasound examination reveals the tumor. There is no sign
of expansion at coughing.
Inguinal lymphadenopathy - The tumor is not reducible and sign of expansion is
missing. Ultrasound examination is helpful in diagnosis.
Ectopic testis - The testicle is missing from the scrotum but it can be present in
the inguinal canal. Ultrasound examination is of real help in finding the testicle.
[4] Some congenital hernias may be associated with ectopic testis.

Varicocele is an abnormal enlargement of the veins draining the testicles (of the
pampiniform plexus). The right testicular vein drains into the inferior vena cava, while
the left testicular vein drains into the left renal vein at a right angle. (Figure 38) This
anatomical difference favors the appearance of the varicocele almost in all cases on the
left side of the scrotum. Upward flow of blood in veins is ensured by small one-way
valves that prevent backflow. Defective valves, or compression of the vein by a nearby
structure, can cause stasis and reflux with dilatation of the veins near the testis, leading
463
to the formation of varicocele. Treatment is represented by interruption of the testicular
vein (vein sectioning and clipping) usually by laparoscopic approach.
Hydrocele testis represents the accumulation
of fluid around a testicle, fluid produced by the
tunica vaginalis. (Figure 38) It can be the result
of a cancer, trauma or orchitis. A common way
of diagnosing a hydrocele is by attempting to
shine a strong light through the enlarged
scrotum (translumination). A hydrocele will
usually pass light, while a tumor will not.
Hydrocele is usually treated surgically. A less
invasive method, but also less effective, is
represented by injection of a sclerosing agent
inside the hydrocele sac after needle aspiration
of fluid. By Lord's surgical procedure, the
tunica vaginalis is plicated by sutures around
the testicle, and it is suitable for small
hydrocele. In Vinkelmann's procedure, the
edges of tunica vaginalis are sewn together
behind the spermatic cord, and in Bergmann's
procedure, the edges of the hydrocele sac are
resected and oversewn for hemostasis.
The final diagnosis should be a complete one and should mention the followings:
Morphological type: direct or indirect hernia
Anatomoclinical type of hernia: inguino-interstitial, or inguino-pubian, or
inguino-funicular, or inguino-scrotal (labial)
Complications: uncomplicated (reducible) or complicated (what type of
complication)
Treatment. The surgical treatment of hernias is the rule, whereas wearing content
devices (belts) represents the exception. Many surgical techniques have been developed
throughout the history but the procedure invented by Bassini in 1887 was for a long
period considered the gold standard and the author the father of modern day hernia
repair.[13,14] There are two main possible approaches for inguinal hernia repair: the
classical (open) and the laparoscopic approach.
In open surgery, there are three steps in repair:
1. Finding and dissecting the sac
2. Treating the content of the sac
3. Reinforcing the inguinal wall using one of the multiple possible
procedures
Classical open surgical procedures (some of them are just of historical interest)
can be classified as:
A. Anatomical procedures (which respect or reconstruct the inguinal canal)
B. Non-anatomical procedures (which abolish the inguinal canal)
a. Retrofunicular procedures - all layers are sewn behind the
spermatic cord
b. Prefunicular procedures - all layers are sewn in front of the
spermatic cord
C. Procedures with transposition of the spermatic cord
464
D. Plastic procedures
E. Tension free procedures
In open surgery, any type of anesthesia can be used:
local, epidural, spinal, general. The patient must be tested
for allergy to the anesthetic agent before the procedure.
Regarding the incisions, the most often used is the
Bassini incision that begins at two cm medial to the iliac
spine and continues down to the tuberculum pubicum. Other
types of incisions are shown in Figure 40. The Annandale-
Lawson Tait access, through the peritoneal cavity, is used to
close the internal inguinal ring of an associated inguinal
hernia when a laparotomy is indicated for other intra-
abdom
ion in the suture line probably accounts for many
recurr
neurosis are sewn together in front of the spermatic
cord restoring the inguinal canal.
inal pathology.
A. Anatomical procedures
BASSINI procedure: the principle is the reinforcement of the posterior wall of the
inguinal canal by sewing under tension (it is not a tension free procedure) the conjoint
tendon (Henles ligament) to the inguinal ligament (Poupart). In addition, the
transversalis fascia is reinforced and the internal inguinal ring recalibrated. The inguinal
canal is restored sewing together the edges of external oblique aponeurosis in front of
the spermatic cord. (Figure 41)Tens
ences after the Bassini repair.
Technique: the skin and then the external oblique fascia are incised opening thus
the inguinal canal. The spermatic cord is isolated. Spermatic fascia and cremasteric
muscle are incised and the hernial sac is discovered. If the sac cannot be found inside
the spermatic cord, an incision through the abdominal wall above the internal ring may
be performed (LaRoque incision) and by digital exploration through the peritoneal
cavity the opening of the sac is found (Reymond maneuver). The sac is isolated and
then opened. The content of the sac is inspected and treated if necessary. The sac is
ligated at level of its neck under direct visualization. The excess of sac is then resected
(Socin). The conjoint tendon of Henle is sewn to the inguinal ligament reinforcing the
posterior wall of the inguinal canal and recalibrating the internal inguinal ring. Then the
edges of the oblique external apo

ANDREWS / HACKENBRUCH procedure restores the inguinal canal. The cranial
edge of the oblique external muscle aponeurosis is used to reinforce the posterior wall
of the inguinal canal, being sewn together with the Henles ligament to the inguinal
ligament. The inguinal canal is restored using the caudal segment of the aponeurosis
sewn over the spermatic cord, overlapping the cranial segment. (Figure 42)
465
Other procedures also restore the inguinal canal but use the ligament of Cooper,
which is the strongest anatomical element of the region for a better reinforcement of the
posterior wall of the inguinal canal, and also to prevent femoral hernia occurrence.
LOTHEISEN (1942) was the first who proposed the use of ligament of Cooper, such
procedures having been developed by HASHIMOTO and McVAY. (Figure 43) The
conjoined tendon of Henle and the transversalis fascia are anchored to the Cooper's
ligament with two or three sutures without narrowing excessively the femoral canal.
The repair is then continued like in Bassini procedure. This procedure creates a high
tension between anatomical structures and sometimes, relaxing incisions in rectus
abdominis sheath are necessary.

SHOULDICE procedure (1945) divides the
transversalis fascia from the internal ring to the
tuberculum pubicum and reinforces it by using a
continuous suture in a lapel way. The medial edge of the
fascia transversalis is sewn to the inguinal ligament.
Then, a third suture line brings the conjoined tendon to
the inguinal ligament, which is doubled by a more
superficial plane.
B. Non-anatomical procedures
These procedures are considered non-anatomical
because they abolish the inguinal canal.
1. In retrofunicular procedures all layers (the
conjoint tendon and external oblique
aponeurosis) are sewn to the inguinal ligament
behind the spermatic cord for a better
reinforcement of the posterior wall
(POSTEMPSKI, WISSE procedures). Even
though the posterior wall is reinforced, the deep
inguinal ring is weakened because the external inguinal ring is moved up in the
front of the internal ring. Thus, intra-abdominal pressure acts against a weaker
zone represented by those two overlapped inguinal rings. (Figure 45)
2. In prefunicular procedures, the external oblique aponeurosis and conjoint
tendon are sewn to the inguinal ligament in front of the spermatic cord, and thus
the deep inguinal ring is translated in front of the superficial ring. (FORGUE,
GIRARD, FERRARIS, PASOKUKOTHI, VILANDRE, TH. IONESCU,
BINET, WOFLER, MUGNAI, HALSTEDT, MARTINOV, KIMBAROVSKI,
etc.). (Figure 45)
466
C. Procedures with
transposition of spermatic cord
are only of historical interest in
nowadays. These were applied
especially in recurrent hernias
with a large internal ring. Because
the internal ring represents the
weakest zone of the abdominal
wall that caused the hernia
recurrence, authors like
SCHMIEDEN and MARIN-
POPESCU developed procedures in which
a new internal ring is performed in a more
resistant region, the old one being closed.
This involves translating the spermatic cord
with or without mobilization of the testis
from the scrotum. (Figure 46)
D. Plastic procedures use different
kind of tissues or materials to reinforce the
weakened zone of the abdominal wall:
Autologous material - (patients
own biologic material)
o Skin - LOEVE REHN
o Fascia transversalis - ZIEMANN
o Hernial sac- LISCHIED
o Cremaster muscle- BRENNER
o Aponeurosis - ADLER
o Sheath of the rectus abdominis - HALSTED, VREDEN
o Fascia lata - WANGENSTEEN, BINET
With homologous material- (from the same species)
With heterologous material - (from another species)
All these procedures are history now, because new meshes of synthetic materials,
very well tolerated by the organism, have been developed and successfully used in
modern era of surgical treatment. Meshes composition can be different (Nylon,
Polyethylene, Polypropylene, Polyester, expanded polytetrafluoroethylene, Polyglycolic
acid, Polyglactin, etc.) and also their texture. (Figure 47)

E. Tension-free procedures. The most important progress in hernia repair was
the development of tension-free procedures. The principle of tension-free techniques is
467
to avoid tension on tissues, as it happens in above-mentioned procedures. All these
repairs use a barrier prosthesis represented by a permanent mesh.
The first who described a tension-free procedure was USHER in 1958. He termed
his procedure as "tension-eliminating". He opened the posterior wall of the inguinal
canal and sutured a mesh between the conjoined tendon and the inguinal ligament.
(Figure 48) STOPPA, in 1967 used a large mesh
introduced in the preperitoneal space to repair bilateral
inguinal hernia. In this case, the mesh is held in place by
intra-abdominal pressure, an application of Pascal's
principle.[10](Figure 49)

LICHTENSTEIN in 1984 described a
tension-free technique of reinforcement of the
posterior wall of the inguinal canal, which has
become the most widely used technique around
the world and represents a milestone in the history
of inguinal hernia surgery. (Figure 50)
In 1993, RUTKOW and ROBINS described
a new technique that uses a flower-shaped
polypropylene mesh, which is introduced into the
internal ring to occlude it (like a plug) and to
prevent hernia relapse. Above it, a mesh is places
behind the spermatic cord and fixed to the
conjoint tendon and inguinal ligament. (Figure 51)

The Prolene Hernia System (PSH) (1997) is an innovation that uses a special type
of mesh with a shape of "H" letter, a mesh composed of two layers connected between
them by cylinder mesh. One layer is introduced through the internal ring into the
preperitoneal space and the other, resting on the transversalis fascia, is fixed to the
conjoint tendon and inguinal ligament. In this way a double curtain reinforcement is
performed. The connecting component of the two layers clogs the internal inguinal ring
preventing thus the relapse of oblique hernias. (Figure 52)
ProGrip Mesh is a self-fixating mesh, which has small, absorbable grips on one
side to secure to the abdominal wall tissues eliminating the need to suture the mesh into
place. It is used with Lichtensteins technique. (Figure 53)
468

In recent decades new minimally invasive approaches for hernia repair have been
developed. The main benefit is the faster recovery of the patient. However, there are
still controversies over the cost-efficiency in laparoscopic approach. Laparoscopy is a
more difficult approach for hernias, being necessary special equipment, general
anesthesia and a skilled surgeon. Many techniques were used to repair hernia, like:
1. Simple closure of the internal rings procedure reported first in 1982 by
Ger R. was associated with a high early recurrence rate.[15]
2. Plug and patch repair
3. Intraperitoneal onlay mesh repair (uses a silicone mesh)
4. Transabdominal preperitoneal mesh repair (TAPP)
5. Total extraperitoneal repair (TEP)
Bilateral inguinal hernias and recurrent
inguinal hernias are the main indications for
laparoscopic approach with definite benefit over
conventional surgery to the patients.
Contraindications are represented by
incarcerated or irreducible hernias, giant hernias,
recurrent hernia after laparoscopic approach, and
prior groin irradiation.
Laparoscopic total extraperitoneal approach
(TEP) was first described by Arregui in 1991. The
principle is the same as in Stoppas procedure: a
mesh is applied in the preperitoneal space as a
barrier that reinforces the weak zones of the
inguinal region. The dissection of extraperitoneal
space by the laparoscopic extraperitoneal approach
is technically more demanding because of the
limited working space and a different perspective
of anatomy but it is now the preferred laparoscopic
technique for the repair of inguinal hernia. (Figure
54) An inflatable balloon is used to dissect the
469
extraperitoneal space and create working room.
The hernial sac is reduced and a mesh is applied
over the inguiono-femoral region preventing thus
the inguinal and femoral hernia recurrence. The
mesh is held in place by helical tacks, or by the
intra-abdominal pressure (Pascals principle).
Laparoscopic transperitoneal approach (TAPP)
was described by GILBERT in 1985. For this
approach, the pneumoperitoneum is induced and
trocars are placed into the peritoneal cavity in
appropriate position for the inguinal region. The
hernial sac is reduced and the peritoneum is
sectioned to access the preperitoneal space where
the mesh is applied over the weak zones of the
region and held in place by stitches or tacks. The
peritoneum is sewn to its anatomical position over
the mesh. (Figure 55)
Local complications after hernioplasty are rare
being represented by:
Hematomas
Seromas
Hemorrhages from the wound
Wound infections - generally will lead to hernia recurrence
Scrotal edema
Testes necrosis - when spermatic artery is injured
Hernia recurrence - there are many causes but recurrence appear mostly after
classic procedures when tissues are sewn under tension. Wound suppuration,
obesity and physical efforts are favoring factors.
Inguinal neuralgia - when the ilioinguinal or iliohypogastric nerve is entrapped
in scar tissue, mesh or sutures.
B. Femoral Hernia
Femoral hernias occur just below the inguinal ligament, through a naturally
weakness called the femoral canal. (Figure 56) Femoral hernias are a relatively
uncommon type, accounting for only 3% of all hernias. While femoral hernias can occur
in both males and females, almost all of them develop in women because of the wider
bone structure and a more horizontalized position of the female pelvis.
Anatomy (Figure 57)
The femoral canal is a short (2 cm) conical
shape canal bordered:
superiorly by the inguinal
ligament
inferiorly by the pectineal
ligament lying anterior to the
superior pubic ramus
medially by the lacunar
ligament of Gimbernat
470
laterally by the femoral vein
Arcus iliopectineus (iliopectinal arch) divides the space below the inguinal
ligament into two compartments: lacuna musculorum (is the lateral compartment for the
passage of the iliopsoas muscle and femoral nerves) and lacuna vasculorum, the medial
compartment that contains fatty tissue, the femoral vessels and the lymph nodes of
Cloquet.
Because of narrow lumen and rigid walls of the femoral canal, femoral hernias are
more likely to strangle than inguinal hernias. The hernial sac is elongated with a long
neck and there are many layers in front of it, which makes it more difficult to dissect.

Clinical picture and diagnosis
Symptoms and signs are almost similar to any other abdominal wall hernias. The
hernia appears as a bulge in the groin below the Malgaigne line. (Figure 58) The bulge
is usually smaller than that of inguinal hernia. Cough impulse sign is often absent and
should not be relied on solely when making a
diagnosis of femoral hernia. The sac of femoral hernia
usually lies below the Malgaigne line, but there are
cases when it migrates upwards into the inguinal
region making difficult the differential diagnosis
between those two types of hernia. If reducible, the
femoral sac reduces underneath the inguinal ligament,
through a trajectory from down to upwards, whereas
the inguinal hernia sac reduces over the ligament.
More difficult is the diagnosis in case of
strangulated hernia.
The diagnosis is largely based on clinical signs and symptoms. However, in obese
patients, imaging (especially ultrasonography, but also CT or MRI) may be helpful.
For reducible hernias, differential diagnosis includes:
Tuberculous cold abscess - results from tuberculous disease of the vertebrae,
when the puss is migrating over the psoas muscle until the femoral region,
giving the aspect of a soft, fluid swelling without heat, redness, pain, or
fever. Useful investigations for diagnosis are: Tuberculin skin test which is
positive in 84 - 95 % of patients who are HIV negative, spine X-ray, CT and
MRI scans, which may show patterns of vertebral body destruction.[16]
Femoral artery aneurysm - the bulge produced by aneurism is pulsatile and
can be reduced on a direction perpendicular to the thigh. Ultrasound eco-
Doppler highlights the aneurysm.[4]
471
Ectasia of the saphena magna vein arch - produces a bulge in the groin,
which can be reduced on a perpendicular direction, presenting also the sign
of cough expansion. On palpation, during cough effort a thrill can be felt,
caused by the blood reflux from the femoral vein into the dilated saphenous
vein. Eco-Doppler highlights the ectatic vein and the reflux.[4]
Lipomas - are benign tumors of the fatty tissue, which have a soft
consistency but are not reducible nor present coughing expansion sign.
Inguino-femoral lymphadenopathy - are inflammatory or tumoral enlarged
lymph nodes in the groin region. They are not reducible, nor present
coughing expansions sign. Ultrasound examination highlights the lymph
nodes.
Differential diagnosis of incarcerated femoral hernia (painful bulge and local
inflammatory signs) includes lymphadenitis of the Cloquet lymph node and phlebitis of
the greater saphenous vein arch.
Anatomoclinical types of femoral hernias: (Figure 59)
1. Laugier hernia - through the Gimbernarts
lacunar ligament
2. Cloquet hernia - under the pectineal fascia
3. Retrovascular Serafini hernia - posterior to the
femoral vessels
4. Prevascular Moskovitz (Velpeau) hernia - in
front of the femoral vessels
5. Laterovascular
6. Miolacunar or Hesselbach hernia - through the
lacuna muscularis (psoas muscle)
Treatment
Goals are the same as in inguinal hernia repair:
finding and dissecting the sac, treating the content,
resecting the sac and reinforcement of the region to
prevent relapse. There are two possible approaches:
classical by open surgery and laparoscopic. Incisions in
open surgery are (depending on surgical technique):
(Figure 60)
Femoral - for a minimal invasive procedure
Extended femoral - for double curtain
procedure
Inguinal - for Ruggi Parlavecchio procedure
Laparotomy - the Lawson Tait procedure,
when laparotomy is recommended for other
intra-abdominal pathology
Procedures (most of them just of historical interest):
1. By femoral approach (Figure 61)
Forced descent of the femoral arch
BERGER - the femoral arch is sewn to the pectineal fascia
TRICOMINI - makes a purse string
ZATEPIN - descends the femoral arch using a wire passed under the
pubic arch
472
RANY - fixes with nails the arch to the pubis
Relaxed descent of the femoral arch
FABRICIUS - the Gimbernat ligament is cut and the femoral arch is
sewn to the pectineal fascia
DELAGENIER - uses the Coopers ligament for reinforcement

2. By extended femoral approach
Double curtains procedure CADENAT (Henles ligament is sewn to
Coopers ligament and the inguinal ligament is sewn to pectineal fascia)
(Figure 62)
KEYNE - uses the rectus abdominis sheath for reinforcement

3. By inguinal approach. The skin incision is the same as for inguinal hernia. The
sac is found in the groin region, dissected and then passed underneath the
inguinal ligament and ascended into the inguinal region. This approach offers a
better access to the neck of the sac. The sac is resected and then the femoral
opening is narrowed suturing the Henles ligament and inguinal ligament to the
Coopers ligament. (Figure 63) RUGGI - PARLAVECHIO is the procedure
most often used but there are also other procedures (CODIVILA, ROBINEAU,
etc.).
4. Plastic procedures
Procedures that use autologous tissues (STRECHI - round ligament,
POLYA - saeratius muscle, WATSON - pectineus muscle, HOFFNER -
saphenous magna vein, GOROSLOVSKI - pectineal aponeurosis,
TURNER - pectineal aponeurosis and psoas fascia, etc)
Procedures that use meshes
473

C. Umbilical Hernia
The umbilicus represents another weak zone of the anterior abdominal wall. At
the umbilicus, the peritoneum makes a condensed fold, the so called Richets fascia,
which determines two types of umbilical hernia direct hernia (in the absence of
Richets fascia) and indirect umbilical hernia (oblique inferior or superior). (Figure 64)

Overweight, multiparous woman, aged between 35 and 50 represents the typical
patient with umbilical hernia. Women are affected 3-5 times more frequently than men
are.[17] Ascitis (cirrhosis, carcinomatosis, etc.) is a major factor that contributes to the
developing of hernia and makes it more difficult to treat. In this case, we are talking
about a symptomatic umbilical hernia.
The etiology is multi-factorial, but chronic intra-
abdominal high pressure and weakness of the abdominal
wall structures are of most importance.
Some hernias can be big enough, with loss of
domain, with a parietal defect of about 10-15 cm in
diameter, but most of them are small, about 5 cm. (Figure
65) The content of the sac is represented by the greater
omentum and large bowel.

Umbilical hernias are classified as:
Congenital
Embryonic, when the defect appears before the third i.u. month when the
peritoneum is not yet developed
Fetal, when the defect appears after the fourth i.u. month. The sac is
present because the peritoneum is developed in this stage.
Acquired
Of the small child
474
Of the adult
Of weakness
Of pressure
Combined
Symptomatic hernias - 20% of patients with ascites develop umbilical hernia.[18] In
10% of cases, an ulceration of the skin will develop which causes the opening of the
hernial sac and umbilical fistula. Postoperative mortality rate is about 2% if the hernia is
repaired without treating the ascites.[18]
Treatment
The vast majority of umbilical hernias of the small child heal on their own by the
age of 3-4 years, so surgery is not recommended at this age unless in case of
complications.[19] Compression bandages and other solutions to keep the hernia
reduced, are not generally agreed because may cause irritation and ulceration of the
skin.
Umbilical hernias in adult are treated surgically. There are many procedures
depending on volume of the hernia, the patient's co-morbidities and preferences. As in
any other hernias, there are two types of approach: the classical open surgery and the
In classical approach, small hernias of the adult may benefit from umbilicus
preserving or reconstruction surgery. A small arcuate incision is performed on the
umbilicus border. Then the sac is dissected, resected and the parietal defect is closed by
interrupted sutures. Finally, the skin is closed.
In larger hernias, repair consists in omphalectomy (removal of the umbilicus)
followed by abdominal wall repair. Examples of procedures are:
EDUARD QUENU - the edges of the parietal defect are sewn together.
SAPIEJKO-PICOLI - the margins are sewn one over the other in lapel for
a better reinforcement of the abdominal wall. (Figure 66)

MAYO-MENGE - is a complex procedure consisting in omphalectomy,
closure of the peritoneum, approximation of the two rectus abdominis
muscles on median line, suture of the rectus abdominis sheath in a duplicate
way (in lapel or "vest over trousers") in craniocaudal direction.[17] (Figure
67)

475
All these procedures are burdened by a high rate of hernia relapses and were
generally abandoned.
Plastic procedures use different kind of materials (homologous or synthetic) to
reinforce the abdominal wall. Before meshes era, the resected skin was used but in
nowadays, procedures using synthetic meshes are the most frequently applied. After
omphalectomy, dissection and resection of the hernial sac, in most cases the rectus
abdominis muscles are approximated by interrupted sutures closing the peritoneal cavity
and then, the mesh is applied over the fascial plane to reinforce the suture line and the
abdominal wall. (Figure 68) This is the so-called onlay procedure but the mesh can be
applied as well under the rectus abdominis muscle into the preperitoneal space and then
the two edges of the rectus abdominis are approximated. When the parietal defect is
large and edges cannot be approximated, the mesh can be positioned inside the
peritoneal cavity. In this case, a special non-adherent (silicon) mesh is used to prevent
adhesion to intestines.

Laparoscopic approach is the new trend in hernia repair, with multiple
advantages regarding the esthetic aspect and the patient recovery. It was possible thanks
to the development of new non-adherent meshes and special instruments for mesh
fixation. The procedure is applied under general anesthesia by orotracheal intubation.
First, the pneumoperitoneum is induced introducing CO2 through the Veres needle.
Then, three trocars are used: one for optical device, one two for working instruments.
The hernial orifice is visualized, hernial sac content is withdrawn into the peritoneal
cavity and excess fat is excised. Finally, the mesh (introduced via the largest trocar) is
fixed using special helicoidally tacks (absorbable or non-absorbable) to the anterior
abdominal wall so that it will occlude the hernial orifice and the non-adherent side is
oriented towards intestines. Omphalectomy is not necessary. (Figure 69)

476
D. Epigastric Hernia
Epigastric hernias represent 0.5-5 % of all operated abdominal hernias and are 2-3
times more common in men, with a higher incidence in patients from 20 to 50 years.[20]
This type of hernia appears on the midline of the epigastric region through the
fibers of the linea alba. The first to protrude is the properitoneal fatty tissue, the so-
called prehernial lipoma, which produces a cone of the peritoneum, which will become
the future hernial sac.
Usually hernias are of small dimensions, 1-2 cm in diameter, and difficult to
diagnose in obese patients. On palpation, a small, painful bulge can be felt under the
skin of the epigastric region. There are two clinical forms: a painful form and an
asymptomatic form. Frequently epigastric hernias are associated with other pathology of
the supramesocolic organs (peptic ulcer, gastric cancer, gall stones, etc.). This is the
reason why pre- and intraoperative investigation of these organs is recommended.
Treatment consists in removing the prehernial lipoma and reinforcement of the
linea alba with sutures, with or without using a mesh.
E. Spigelian Hernia
Spigelian hernia appears under the aponeurosis layer between the rectus
abdominis muscle medially, and the semilunar line laterally, at or below the linea
arcuata site.
The bulge is not very evident because the sac does not lie below the subcutaneous
layer but under the aponeurosis. It can be highlighted by ultrasound examination.
The treatment consists in abdominal wall repair through a classical open approach
or by laparoscopic approach. In open approach, the incision is placed on the hernial
area. The muscular layer is dissected and the sac is found and reduced into the
abdominal cavity or resected. Then, the posterior rectal sheath is reinforced and the
other layers re-approximated. Using a mesh is another possible way to reinforce the
abdominal wall. By laparoscopic approach, the parietal defect is covered by a silicone-
coated mesh kept in place using tacks and threads. The procedure is similar to that
applied in umbilical hernia. Laparoscopic approach ensures a very fast recovery of the
patient who in most cases is discharged in the next day after the operation. (Figure 70)

F. Obturator Hernia
The obturator canal contains the obturator nerve and vessels. It has rigid walls,
which explains why hernias through this canal incarcerate so frequently.
Incarceration or strangulation is manifested by intestinal occlusion signs and
symptoms so that usually the patient is operated in emergency by open approach
(laparotomy) and the hernia is an intraoperative finding.
The Rombergs sign observed in this type of hernia is caused by the compression
of the obturator nerve exerted by the hernial sac. The thigh is flexed in abduction with
external rotation of the knee (antalgic position). The patient feels the pain in a region
477
just above and medial to the knee, which is the area of skin innervation of the obturator
nerve.
Treatment of this type of hernia is surgical by open or laparoscopic approach. The
herniated intestinal loop is treated, if necessary resected, and the internal obturator ring
is narrowed in different ways: suture, plugging or using meshes.
G. Ischiadic (Sciatic) Hernia
This type of hernia is very rare and the diagnosis is difficult because the sac is
located under the gluteal muscles. The sac is passing through the infrapiriform foramen,
which is crossed, by the sciatic nerve, femurocutanate nerve and inferior gluteal vessels
and nerves (the greater sciatic notch is divided into 2 holes by the pririformis muscle:
the suprapiriform foramen, crossed by the superior gluteal vessels and nerves, and the
infrapiriform foramen).
Hernia should be differentiated from a gluteal abscess and other tumors in that
region. CT scan with contrast is helps the diagnosis.
4. Incisional Hernias
Incisional hernia represents a special type of hernia, which appears after invasive
surgical procedures (incisions) that involve and destroy the integrity of the abdominal
wall. Compared to common hernias, incisional hernias represent the total or partial
protrusion of abdominal viscera, under the skin, through a defect in the abdominal
wall. - (until here the definition is almost the same as in common hernia.) but
.the integrity of the anatomical structure of the abdominal wall is being affected (in
most cases by surgery). Another difference is that incisional hernias may appear in any
zones of the abdominal wall not just in hernial zones as in case of common hernias
(where the abdominal wall is naturally weaker).
They are called incisional because appear most often after surgeries that require
laparotomy. However, the term of "incisional" hernia does not include the full spectrum
of pathology to which it relates. A better term is evntration, which besides hernia,
caused by surgical incisions, includes as well those produced by trauma (aggressions,
accidents, etc) and also the abdominal wall relaxation.
Classification
Traumatic hernias are those caused by abdominal wall trauma. In this case,
there is a breach in the abdominal wall through which the hernial sac is
protruding under the skin. The hernial sac is present and incarceration is
whenever possible. (Figure 71)
Postoperative - these are the true incisional hernias
Non surgical - accidentally, as a consequence of a blunt or penetrating
trauma of the abdomen
Non-traumatic hernias are actually relaxations of the abdominal wall
produced by various factors. There is no true hernial sac and no breach in the
abdominal wall. The wall is weakened, relaxed, and thus bulges in the
affected region. These types of hernias never lead to incarceration.
Various deficiencies (protein, vitamins, etc.) and other pathologies that
affect the abdominal muscles or their innervation.
Obstetrical hernias, the so-called diastasis of the rectus abdominis
muscles represent a weakening of the fascial membrane (linea alba)
478
connecting the rectus muscles appeared especially after multiple
pregnancies. It is asymptomatic and does not lead to incarceration.

Risk factors [21,22]
A. Local factors and surgical technique. After laparotomies, the integrity of the
abdominal wall is affected. The resulting scar is not as strong as normal tissues are and
can break on intense efforts. In addition, many other local factors could contribute to the
impairment of the healing process with the result of a breach in the abdominal wall.
Wound suppuration is the most important and frequent cause of incisional
hernias. Abdominal cavity drainage promotes local suppurative processes. The type of
abdominal incision is also an important factor. Vertical incisions predispose more
frequently to incisional hernia (the incidence is significantly higher for midline incisions
compared with transverse incisions -11% vs. 4.7%) [23-26] and oblique incisions, which
cut the nerve supply of the muscles, lead to a relaxation of the abdominal wall beneath
the incision. Other authors found that midline and transverse incisions cause similar
hernia rates.[27]
B. General factors are represented by:
Obesity - is associated more frequently with diabetes, wound suppuration
and an increased intra-abdominal pressure
Age - healing process is affected at advanced ages
Debilitating factors such as catabolic conditions, malnourishment,
hypoproteinemia, anemia, cancer, disturb the healing process
Postoperative bronchopulmonar complications by coughing effort increase
the intra-abdominal pressure
Other factors such as treatment with steroids, chemotherapy, radiation, etc.
interfere with the healing process
Intense physical efforts after surgery
More than 50% of incisional hernias develop within
the first 2 years after the primary operation. The frequency
rate is approximately 2-20%.[23]
The onset is as an asymptomatic bulge noticed by
the patient. Over the time, incisional hernia enlarges and
becomes more evident and painful. Symptoms like
vomiting, constipation, or severe pain can be associated
with incarceration or strangulation. The evident
appearance of hernia makes it easily to diagnose, often
requiring no testing outside of a physical examination.
The parietal defect may be of different sizes from
small to very large with fibrous inextensible margins. The
sac may be unique or multiple (multilocular sac). The
content of the sac is represented by abdominal viscera
479
(intestines, greater omentum) which are adherent to each other and to the sac.
Superjacent skin is thin with scars, sometimes presenting trophic disorders. (Figure 72)
Clinical evolution and complications are similar to those of common hernias
(incar
eatment has the same targets and steps as in other abdominal wall hernias:
dissec
een developed over the time. The approach may
be by
treatment:
ve asepsia
e threads for sutures
6 to 9 month from the last operation.
Contraindications for operation are:[28]
ain
ife expectancy
rtension
esence of peritoneal dialysis catheter
rials. They can be
used
of the parietal
re is applied especially in diastasis recti. The
O-PICOLI reinforces the abdominal wall using a double layer
stores the abdominal wall integrity suturing each anatomical plan
e abdominal
wall.
anterior rectus sheath after fascial
ceration, irreducibility, loss of domain, etc.).
Treatment
The tr
tion of the sac, treatment of the content (adhesiolysis, partial resection of the
greater omentum or bowel resection in incarcerated hernias) and reinforced closure of
the parietal defect to prevent relapses.
Many surgical procedures have b
classical open surgery (laparotomy) or by laparoscopy.
There are six rules that have to be respected for surgical
1. Skin infections must be treated prior to surgery
2. Rigorous compliance with intra- and postoperati
3. Perfect hemostasis
4. Use slow absorbabl
5. Use of antibiotics to prevent infection
6. Hernia repair will be performed after
This period is necessary for the parietal defect to develop strong fibrous
margins.
Major loss of abdominal dom
Severe debilitation
Fewer than 5 years l
Respiratory distress
Pregnancy
Portal hype
Renal failure with pr
Many old types of repair procedures do not use prosthetic mate
in small or medium size hernias. The tension in the suturing line in these
conventional repairs is the reason why the relapse rate reaches 50%.[29]
EDUARD QUENU procedure approximates the margins
defect with interrupted sutures.
DALAIN COTIADES procedu
procedure reinforces the median abdominal line without opening the
peritoneal cavity by approximating the margins of the rectus abdominis
muscles.
SAPJEIK
technique.
MAYDL re
separately. It is used for incisional hernias after appendectomy.
Plastic procedures use prosthetic materials (meshes) to reinforce th
There are different types and sizes of meshes, usually of polypropylene, which are
much better, adapted and accepted by human tissues.
Onlay procedure. The mesh is sutured to
defect has been closed primarily. (Figure 73)
480
Inlay procedures. After resection of
the hernial sac, the mesh is sutured to
the defect margins. Special meshes
must be used (see below) which do not
adhere to the viscera. It is a tension
free procedure.
Rectorectus procedure. The mesh is
placed between peritoneum and rectus
muscles (Rives-Stoppa technique).
Underlay procedure. The mesh is
placed on the inner surface of the abdominal wall, on the peritoneum. One
surface of these meshes that is in direct contact with intra-abdominal organs
is coated with a non-adherent material (silicone) so that tissues cannot
adhere to it. This kind of meshes are used when the parietal defect is large
and cannot be entirely closed and also in laparoscopic approach. The
technique of the laparoscopic approach is similar to that described at
umbilical hernia.
Intra and postoperative complications are represented
by:
Intestinal lesions - must be recognized and
repaired during operation
Wound infection - one of the worse
complications that might compromise the
outcome leading to hernia relapse or even
evisceration.
Skin necrosis (Figure 74)
Mesh infection - appears in about 7% of cases,
in most cases requiring removal of the mesh
[30-32]
Persistent seroma - needs a prolonged follow up
of the wound and removal of seroma by drainage or percutaneous ultrasound
guided puncture and aspiration.
Prolonged pain - anti-inflammatory drugs and myorelaxants are prescribed
Ileus - early and late intestinal occlusion is possible caused by intestinal
adhesions
Bleeding/Hematoma - suprafascial hematoma should be recognized and
evacuated early to prevent suppuration and mesh infection
Recurrence after mesh repair appears in about 10% of cases [33]
Respiratory distress - in voluminous hernias, replacing the content of the sac
back into the abdominal cavity, and suturing under tension the parietal
defect, will lead to a high intra-abdominal pressure limiting respiratory
movements of the diaphragm.
Abdominal compartment syndrome - refers to organs dysfunction caused by
intra-abdominal hypertension (a sustained intra-abdominal pressure >20
mmHg). Nearly every organ system is affected. Physiologic consequences
include impaired cardiac function, decreased venous return, hypoxemia,
hypercarbia, renal impairment, diminished gut perfusion, and elevated
intracranial pressure.[34]
481
5. Eviscerations
Evisceration represents the extrusion of the viscera outside the peritoneal cavity,
in direct contact with atmosphere, through a solution of continuity in the abdominal
wall. There is no sac like in hernia and the skin is opened.
Causes that might produce evisceration are: (Figure 75)
1. Postoperative disruption (dehiscence) of the wound
2. Posttraumatic, in case of penetrating wounds (accidental or by aggression)

Risk factors are represented by:
A. Determinant factors:
Intra-abdominal high pressure
Deficiencies in wound healing process
B. Contributing factor leading to poor healing:
Advanced age
Hypoproteinemia, hypovitaminosis, anemia, cancers
Obesity
Diabetes
C. Factors contributing to increased intra-abdominal pressure:
Chronic bronchopneumopathy
Prostate adenoma, postoperative ileus
D. Local factors wound infection
E. Deficiencies of surgical technique:
Incorrect approximation of wound margins, ischemic sutures
Inadequate suture materials
Drainage through the wound
Postoperative evisceration usually occurs between day 5 and 10 and it is
announced by a small hemorrhage through the wound. After that, during an episode of
high intra-abdominal pressure (coughing, effort) the intestines extrude outside the
abdominal cavity on the skin.
There are situations when the eviscerated organs remain enclosed under the skin.
Dehiscence appears only at muscular layer, the skin line suture remaining integer. It is
the so-called blocked evisceration. In this case, if digestive transit is preserved, and
there are no signs of peritonitis, the operation can be postponed. The evisceration will
develop later an incisional hernia, which will be operated after 6-12 month in better
conditions.
Treatment
Evisceration is an emergency condition and treatment measures must be taken as
soon as possible. Evisceration means peritonitis. General measures include hydro-
482
volemic, electrolytic, and acid-basic rebalancing; hypoproteinemia will be corrected,
antibiotherapy and other specific measures will be taken.
Eviscerated organs and peritoneal cavity are copiously washed with lukewarm
saline solution. Intestines will be thoroughly inspected and any lesions will be treated as
necessary. After repositioning the eviscerated organs into the abdominal cavity, the
wound edges are prepared, contaminated and necrotic tissues are excised and the
abdominal wall is closed by interrupted or running sutures in a single or multiple
successive layers depending on the state of the abdominal wall and the causing agent of
evisceration.
6. Diaphragmatic Hernias
The diaphragm is a fibro-muscular structure that separates the thoracic cavity
from the abdomen. Between the two cavities, thoracic and abdominal, there is a gradient
of pressure. The higher pressure in the abdominal cavity will determine the migration of
abdominal viscera into the thoracic cavity through a defect of the diaphragm.
The diaphragm develops from three anatomical structures, which merge to form
the diaphragm: the septum transversarium (for the fibrous part of the diaphragm), and
two Ustkovs folds (for the muscular part of the diaphragm). (Figure 76) Deficiencies of
diaphragm development, will lead to congenital diaphragmatic hernias.
Weak zones of the diaphragm are all diaphragmatic holes through which diverse
anatomical structures are passing from, or towards the abdominal or thoracic cavity
(aortic hiatus, esophageal hiatus, inferior cava vein, ductus thoracicus, splanchnic
nerves, azygos veins, etc.). (Figure 77)
Diaphragmatic hernia is a defect or hole in the diaphragm that allows the
abdominal contents to move into the chest cavity.

The following types of diaphragmatic
hernia may exist:
1. Congenital diaphragmatic hernia
a. Morgagni's hernia - anterior
b. Bochdalek hernia - posterior
2. Hiatal hernia
3. Iatrogenic diaphragmatic hernia
4. Traumatic diaphragmatic hernia
Classification
Congenital
o Embryonic maldevelopment
483
appears in the first 3 month of intrauterine life
o Fetal - the defect appears after the third intrauterine life
Agenesia of one or both diaphragm in most cases is incompatible with life. Over
80% are located on the left side.[35] Partial defects can be operated with better
prognosis.
Acquired
o Traumatic - diaphragmatic ruptures
Blunt trauma
Penetrating trauma stab wound (thoracic or abdominal)
o Nontraumatic
Hiatal hernia
Congenital hernias
The most frequent congenital
diaphragmatic defects through which
abdominal viscera herniate into the
thoracic cavity are: the Morgagni-
Larreys orifice and the Bochdaleks
costo-vertebral foramen.
Large congenital hernias are
manifested shortly after birth.
Clinical picture is represented by
respiratory distress, apparent
dextrocardia, a scaphoid abdomen
and radiological appearances of
bowel in the hemithorax.
Treatment involves urgent
nasogastric suction, to prevent distension of the bowel and further compression of the
lung and general resuscitation before surgical repair.
Bochdalek hernia is the most common diaphragmatic hernia in children with a
frequency of about one of every 2,500 live births. About 85% of Bochdalek
hernias occur on the left side, about 10% on the right, and approximately 5% are
bilateral.[35] It is twice as common in male as in female neonates. Mortality
ranges from 45% to 50%. [36,37]
There is a classic triad is represented by:[38] (Figure 78)
1. respiratory distress - the principal symptom
2. apparent dextrocardia, and
3. scaphoid abdomen
Treatment. Immediately after birth, neonates with Bochdalek hernia are taken to
the operating room. For hernia on the left side, the transabdominal subcostal
approach is generally preferred, whereas for hernia on the right side, a
transthoracic approach is preferred.[39] The herniated organs are restored in the
abdominal cavity and the defect is closed with interrupted nonabsorbable sutures.
Large defects may be closed with a patch of mesh. The pleural cavity is drained
with a tube placed on water seal.
Morgagni (Morgagni-Larrey) hernia is the maldevelopment of the septum
transversum, which failed to merge to the sternal and costal elements. It
comprises approximately 2% of all congenital diaphragmatic hernias and is
484
generally accompanied by a hernia sac.[35] The hernia is located anterior between
the sternal and costal attachments of the diaphragm. It is most commonly seen on
the right side. These hernias are generally asymptomatic and are usually detected
as incidental findings on radiographs. The most commonly involved abdominal
organ is the transverse colon and the great omentum. De defect is sutured with
nonabsorbable sutures.
Hiatal Hernia
Hiatal hernia represents the protrusion of the superior portion of the stomach into
the thoracic cavity through the esophageal hiatus.
Normally, the cardia and the stomach are located within the abdominal cavity
being maintained in this position by various anatomical structures and physiological
processes. Between the esophagus and the
diaphragm, there is interposed connective tissue
(the membrane of Leimer-Bertelli Treitz) and
muscle fibers that are drawn from the diaphragm
and dissolve into the esophageal wall (Rougets
muscle). (Figure 79) The visceral peritoneum and
the phrenoesophageal membrane cover the
abdominal esophagus. The phrenoesophageal
ligament is a fibrous layer of connective tissue
bridging the space between the esophageal wall
and the margins of the esophageal hiatus. The
ligament plays an important role in anchoring the
lower esophagus and maintaining gastroesophageal competence.[40] The cardia and part
of the gastric fundus is ancored by various ligaments and membranes to the
retroperitoneal plane, which provide adequate stability to the esophagogastric junction.
The size of the hiatus narrows whenever intra-abdominal pressure rises.[41]
With advancing in age, collagen fibers replace elastic fibers loosing thus elasticity
and loosening attachments. Large accumulation of adipose tissue between the
phrenoesophageal membrane and the cardia may
cause the loose of anchorage of the cardia. All
these factors associated with an enlarged hiatus
and high intra-abdominal pressure (obesity,
pregnancy, physical efforts) are contributing
factors in developing hiatal hernia.
The gastroesophageal junction acts as a
barrier to prevent reflux of the stomach content
into the esophagus and the consequent
esophagitis (GERD gastro esophageal reflux
disease). Components of this barrier are the diaphragmatic crura, the lower esophageal
sphincter, and the angle of Hiss. (Figure 80) The consequence of intrathoracic
protrusion of the cardia and/or the stomach is the loss of antireflux mechanisms.
Types of hiatal hernia (Figure 81)
1. Short esophagus (brachyesophagus) - the esophagus is shortened, and the
cardia is intrathoracic
2. Sliding hernia - is the most common (95% of cases). The length of the
esophagus is normal but the cardia slips inside the thorax.
485
3. Rolling hernia (paraesophageal hernia) - the length of the esophagus is
normal, the cardia is intra-abdominal but the stomach is protruded inside
the thorax. The widened hiatus permits the fundus of the stomach to
protrude into the thorax alongside the esophagus.

Another classification is:[42,43]
Type I hiatal hernia is the sliding hiatal hernia
Type II hiatal hernia is the classic form of paraesophageal hernia in which
part of the gastric fundus, and not the GEJ, herniates above the diaphragm
alongside the esophagus
Type III hiatal hernia is the mixture of type I and II hiatal hernia
Type IV hiatal hernia is the herniation of other abdominal organs (spleen,
colon, pancreas, etc.) through the esophageal hiatus into the posterior
mediastinum
Epidemiology
The true incidence of hiatal hernia in the overall population is unknown because
of minimal or even absence of symptoms in many individuals.[44]
Hiatal hernia is more common in females than in males probably due to the intra-
abdominal high pressure during pregnancies.[45] It is also more frequent as advancing in
age especially due to the loss of muscular tonicity and elasticity of the fixing elements
of the esophagus. Almost 60% of individuals aged 50 or older have hiatal hernia.[46]
Para-esophageal hernias tend to become more voluminous over time.
Symptoms
Most patients with hiatal hernia are asymptomatic or complain of only minor
symptoms, the disease being discovered incidentally. There is no direct correlation
between hernia size and intensity of symptoms. In 5% of cases, hernia evolves to
complications such as gastric volvulus, perforation, strangulation, all of them
threatening the life of the patient.[47] The most common complication is reflux
esophagitis.[42]
Symptoms associated with hiatal hernia are variable but generally include:
Epigastric fullness
Postprandial pain
Heartburn - 30 - 60 minutes after eating
Regurgitation - worsened with lying flat
Excessive belching
Aspiration - stomach contents refluxed into the airway
486
Asthma - chronic result of aspiration
Chest pain - burning mid-chest pain
Difficulty swallowing
Pain with swallowing
Bleeding
Stomach twisting and perforation
Obstruction
Investigations
Barium swallow in Trendelemburg position will highlight the reflux of contrast
into the esophagus and the position of cardia or stomach above the diaphragm. (Figure
81)

CT scan with oral contrast offers detailed information about the position of the
cardia and the stomach and about the size of hernia. (Figure 82)
Esophagogastroscopy has the advantage that it directly visualizes the esophageal
mucosa highlighting the esophagitis and can take biopsies. (Figure 83)

Treatment
Asymptomatic hernias are not treated as long as they remain undiagnosed. The
diagnosed hernias, if manifested by various digestive and /or thoracic symptoms could
benefit from medication and/or surgery. If there is a reflux esophagitis, treatment will
target the following aspects:
Lifestyle changes
Antacid medications
Stimulation of gastric motility and evacuation
Surgical methods that oppose to gastroesophageal reflux
SURGICAL TREATMENT
Surgery is necessary only in the minority of patients with complications of GERD
despite aggressive treatment with proton pump inhibitors (PPIs).[47] Surgery is
recommended especially for paraesophageal hernias. Goals of treatment are:
487
1. Repositioning the stomach and the cardia inside the abdomen
2. Recalibrating the esophageal hiatus
3. Making an antireflux valve
Possible approaches are:
Abdominal or transthoracic approach
Classical or laparoscopic approach
Pure endoscopic approach NOTES (Natural Orifice Translumenal
Endoscopic Surgery)
Procedure: Nowadays laparoscopic approach is the most widely used. After
entering the abdominal cavity, the esogastric junction is exposed. The hiatal hernia is
easily observed in rolling hernias. The lesser omentum is sectioned preserving the
hepatic branches of vagus nerves and vascular branches. The right diaphragmatic crus is
exposed and the dissection continues in the fatty tissue between the esophagus and crus.
The peritoneum above the esophagus is cut and dissection goes on toward the left crus.
The aim is to free up the esophagus and to reposition the cardia into the abdomen. Then,
the gastro-splenic and gastrophrenic ligaments are divided to free up the fundus of the
stomach. The both diaphragmatic pillars are approximated using non-absorbable threads
recalibrating thus the esophageal hiatus. The operation continues with the antireflux
procedure. (Figure 84) There are several types antireflux valve. Most procedures use the
gastric fundus, which is twisted around the abdominal esophagus (Nissen, Belsey,
Toupet procedures) or used as an anterior flap (Dor procedure). (Figure 85) In Hill's
procedure, the cardia is fixed posterior to the diaphragmatic crus and the angulation of
the Hiss angle is increased.

488
In thoracic approach (by left thoracotomy or thoracoscopy), a part of the stomach
is used to add length to the esophagus by cutting the stomach at level of Hiss angle
(Collis Belsey procedure). The crura are re-approximated and esophageal hiatus is
narrowed. (Figure 86)
TIF (transoral incisionless fundoplication) procedure is a new endoscopic
procedure to treat GERD. No incisions are required. It uses a special endoscope
(EsophyX) which reinforces the gastroesophageal junction by folding (plicating) the
upper portion of the stomach around the gastroesophageal junction for about 270
degrees and securing it in place by special fasteners.[48] (Figure 87)

7. Diaphragmatic Ruptures
The diaphragm is a musculo-membranous structure, relatively thin, which
represents the upper wall (ceiling) of the abdominal cavity. It separates two cavities
with different pressures: the thoracic cavity with low pressure and the abdominal cavity
with high pressure. The movement of diaphragm by contraction and relaxation changes
the pressure inside those two cavities having the main role in ventilation (inspiration
and expiration). Diaphragmatic rupture, according to the extent and localization leads to
two important consequences:
1. Protrusion of the abdominal organs into the thorax, and
2. Impairment of the ventilatory function
The mechanisms by which the diaphragm may
break are: (Figure 88)
Closed trauma or blunt abdominal trauma,
produces a sudden increase of the intra-
abdominal pressure leading to
diaphragmatic rupture
Open or penetrating trauma represented by
wounds (stab, gunshot, etc.), which can be:
o Transabdominal wounds or/and
o Transthoracic wounds
489
Due to the gradient of pressure between the abdominal (high) and pleural cavity
(low), intra-abdominal organs (colon, stomach, spleen, small bowel, omentum) protrude
into the thorax, where they become adherent to the intrathoracic organs (lung,
pericardium, etc). (Figure 89)
In cases of penetrating trauma, both intra-abdominal and intrathoracic organs may
have serious lesions, not just the diaphragm. In these cases, surgeons must carefully
inspect all organs inside the both cavities.
All these cases are considered emergencies and must be operated as soon as
possible but after a good evaluation and investigation of the patient (ultrasound, chest
radiography, CT-scan, etc.). A high index of suspect is vital for the diagnosis of
diaphragmatic injuries in an emergency setting.[49]

The type of approach is closely related to the associated injuries.[49] In case of
transthoracic penetrating wound, the surgical approach will be through a thoracotomy.
When intra-abdominal organs are also affected, a laparotomy can be performed if
lesions cannot be managed through the diaphragm. For example, a splenectomy can be
performed through the left diaphragmatic defect.
When the entrance of the traumatic agent is the abdomen, laparotomy will be
performed, and if on exploration diaphragmatic lesions are found, if necessary (when
other intrathoracic lesions are present), the laparotomy can be associated with
thoracotomy or can be transformed in thoraco-phreno-laparotomy.
The diaphragmatic defect is sutured with interrupted non-absorbable sutures.
The diaphragmatic rupture after blunt abdominal trauma is much more frequent
on the left side (the right hemidiaphragm is protected by the liver), at level of centrum
tendinosum (central tendon).
Most often, patients are polytraumatized and are unconscious, so their assessment
is more difficult. The first concern is the life support, but concomitant good evaluation
must be carried out.
Bowel sounds may be heard in the chest and the chest radiograph may reveal
bowel gas in the lung fields. A contrast study (when possible) will confirm the
diagnosis. A CT-scan must be performed.
For intrathoracic lesions, thoracotomy will be the first choice of exploration and
then, if necessary, a laparotomy can be performed. As a rule, for live saving, solving
intrathoracic lesions has priority against abdominal lesions.
Occasionally, small diaphragmatic lesions are overlooked and the patient presents
some time later with a diaphragmatic hernia. In these cases, the diaphragmatic lesion
will be solved through a thoracotomy as the test of time proved that intra-abdominal
lesions are not present (peritonitis, hemoperitoneum).
490
There are cases in which traumatic diaphragmatic hernia are incarcerated with
damage of the intra-abdominal organs, especially colon or small intestine. It happens
especially when the diaphragmatic defect is small. The diagnosis is usually difficult and
represents an intraoperative surprise. A more detailed history on these patients reveals a
history of abdominal trauma or other mechanisms of sudden increase of intra-abdominal
pressure.
There are three clinical phases of diaphragmatic injuries (described by
Grimes):[50,51]
1. The acute phase - in the same day with the trauma.
2. The second or latent phase - if the injury is not recognized in the early
phase. It is an asymptomatic phase but intra-abdominal viscera evolve into
gradual herniation.
3. The third phase is that of complications (obstruction, incarceration,
strangulation, perforation, peritonitis, pleural effusions, etc.).
7. Diaphragmatic Relaxations
The phrenic nerves, which are the only motor
supply to the diaphragm, arises from C3,4,5 roots. It lies
on the scalenus anterior muscle and enters the thorax.
The right phrenic nerve passes through the diaphragm
close to the inferior vena cava in the central tendon. The
left phrenic passes through the muscular part of the
diaphragm. (Figure 90)
Diaphragmatic relaxation represents the total or
partial relaxation of the diaphragmatic muscles due to
primary or acquired, traumatic or non traumatic, phrenic
nerves palsy or diaphragmatic muscle lesions or
degeneration. (Figure 91)

The diaphragm is the major respiratory muscle. When it contracts, it lowers
producing a negative pressure inside the thoracic cage, so inspiration takes place. When
it relaxes, the intra-abdominal pressure pushes the diaphragm upward, the intrathoracic
pressu
matic relaxation produces
si
oxical respiration with pendulating air from
one lung into the other one.
re rises and expiration takes place.
An important condition for proper ventilatory function is that both diaphragm
(left and right) to run simultaneously. A large diaphrag
gnificant breathing changes through several mechanisms.
1. First the lung volume on that side is reduced.
2. Second it produces a parad
491
The consequence is the hypoventilation,
hypoxia, dyspnea, and bronchopneumonia. The
mediastinum is also pendulating with each
respiration.[52] (Figure 92)
Diaphragmatic relaxation is a rare condition
in adults. It is oligosymptomatic when relaxation
is minor and diagnosis is made incidentally on
chest radiography. In complete relaxation (more
frequently on the left side), symptoms may be
represented by:
respiratory distress with dyspnea, tachypnea, cyanosis
tracheal and cardiac shift
decreased breath sounds over the affected area
asynchronous chest wall movement
and may evolve to:
chronic pulmonary suppuration
diaphragmatic rupture
ulcer and volvulus of the stomach
death
Treatment
Surgery is indicated only in symptomatic
relaxations. The aim of surgery is to fix the
diaphragm in inspiration position so that
paradoxical movement and mediastinal
pendulating are minimized. (Figure 93) The
operation can be performed by thoracotomy or by
minimal invasive approach (thoracoscopy).
There are three main procedures of
phrenoplasty: (Figure 94)
1. Phrenoplication
2. Incision followed by dual-layer suture
3. Dual-layer sandwich mesh repair

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Qq0I9eKXr&sig=dOjQ0ihkex26gK69BKvCfBJ0XWs&hl=en&sa=X&ei=2u7eUcijLrSa4gS9o4DYDw&ved=0CEEQ6A
EwAw
42. Jong Jin Hyun, Young-Tae Bak - Clinical Significance of Hiatal Hernia - Gut Liver 2011; 5(3):267277.
43. Kahrilas PJ, Kim HC, Pandolfino JE. - Review Approaches to the diagnosis and grading of hiatal hernia. - Best Pract.
Res. Clin. Gastroenterol. 2008; 22(4):601-616.
44. Luigi Bonavina - Paraesophageal Hiatus Hernia - in K.I. Bland et al. (eds.), Surgery of the Esophagus and Stomach,
DOI: 10.1007/978-1-84996-438-8_2, Springer-Verlag London Limited 2011
45. Rigler LG, Eneboe JB. - Incidence of hiatus hernia in pregnant women and its significance. - J. Thoracic Surg. 1935;
4:262268.
46. Goyal Raj K. - "Chapter 286. Diseases of the Esophagus" in Harrison's Principles of Internal Medicine, 17e.
47. Waqar A Qureshi - Hiatal Hernia - Medscape reference, Emedicine - http://emedicine.medscape.com/article/178393-
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48. Cadire GB, Rajan A, Rqibate M, Germay O, Dapri G, Himpens J, Gawlicka AK. - Endoluminal fundoplication (ELF)--
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current possibilities of surgical repair. - Ann. Ital. Chir. 2006; 77(2):131-135.

Surgical Pathology of the Thyroid Gland
SURGICAL PATHOLOGY OF THE THYROID GLAND

1. Surgical anatomy and physiology
2. Methods of investigation of the thyroid gland
3. Goiter - Endemic thyroid dystrophy
4. Other causes of enlarged thyroid gland
a. Nodules
b. Acute thyroiditis
c. Subacute thyroiditis - de Quervain
d. Chronic thyroiditis (Hashimoto, Riedel)
5. Hyperthyroidism
6. Thyroid cancer
1. Surgical Anatomy and Physiology
The thyroid is an endocrine gland located in
the anterior region of the neck below the larynx in
front of the first 3-4 tracheal rings. It resembles the
letter "H" consisting of two lobes connected by an
isthmus. (Figure 1) It weighs about 25-30 grams.
The gland has a reddish brown color and soft
consistency. Nearly 50% of thyroid glands exhibit
a pyramidal lobe arising from the centre of the
isthmus. [1]
Thyroid gland is wrapped by a sheath.
Between the own thyroid capsule and the sheath there is
connective tissue and blood vessels. Loose connective
tissue allows an easy dissection and enucleation of
thyroid lobes.
The gland develops from an outpouching of the
pharynx floor and migrates downward. The persisting
remnant of this migration is known as a thyroglossal
duct, which further may develop the cyst of the
thyroglossal duct. (Figure 2)
Anatomical relations - relevant surgical aspects
(Figure 3)
In front, the gland is covered by the pretracheal fascia and the infrahyoid muscles
(sternohyoid, omohyoid, sternothyroid, thyrohyoid). Sometimes, when voluminous
goiters have to be operated, cutting these muscles facilitates the removal of the gland.
Lateral, the gland has relations with the jugular vein, carotid artery and vagus
nerves. These vascular structures are very important and must be protected during
operations on thyroid gland. In very advanced cases of thyroid cancer, the jugular vein
may be compressed or even invaded and it can be resected during classical neck
dissection operation.
Medial, the gland has relations with the larynx, trachea, pharynx, esophagus and
recurrent laryngeal nerves. These relations are also very important. In voluminous
495
goiters trachea may be compressed with consecutive respiratory impairment (dyspnea).
After a long period of compression of the trachea, the cartilaginous structure of the
tracheal rings becomes weakened (tracheomalacia) and after thyroid removal, the
trachea collapses leading to asphyxia. In such cases, tracheostomy is the procedure that
saves the patients life. Injuries of the larynx or trachea during operations on thyroid are
very rare but possible.
Posterior, the gland comes in relations with the recurrent laryngeal nerves,
esophagus, cervical sympathetic chain and parathyroid glands. Compression on
laryngeal nerves will lead to hoarseness, on esophagus to dysphagia, and on the
sympathetic chain to Claude-Bernard-Horner syndrome. Injuries of these structures,
especially of the recurrent nerves, are possible during operations. Due to the very close
relations with the parathyroid glands, there is the chance to remove these glands along
with the thyroid lobes during thyroidectomy, which will induce to parathyroid
insufficiency.
Inferior, the thyroid lobes meet the inferior thyroid vascular pedicle and recurrent
laryngeal nerves. The recurrent laryngeal nerves may be damaged most often at this site,
due to their intimate relations with the inferior thyroid vessels, which are resected
during thyroidectomy. (Figure 4) Sometimes, the inferior pole of the thyroid lobe may
plunge under the sternum making the operation more difficult.
Superior, the relations are with the superior thyroid vessels and laryngeal nerves.
Damage of the superior laryngeal nerves should be avoided during resection of the
superior thyroid vascular pedicle. Due to the coiled shape of the superior thyroid artery,
it may retract under muscles after resection, which makes it difficult to find and make
the hemostasis.

496
Vascularization
Arterial supply of the thyroid gland is ensured by two main arteries represented
by: (Figure 5)
1. Superior thyroid artery - from the external carotid artery
2. Inferior thyroid artery - from the thyreocervical trunk
Venous blood is drained by: (Figure 5)
1. Superior thyroid vein - toward the internal jugular vein
2. Middle thyroid vein - toward the internal jugular vein
3. Inferior thyroid vein - toward the brachiocephalic venous trunk

Lymphatic drainage of the thyroid gland. (Figure 6) Lymphatics are arranged in two
networks: one around the thyroid follicles and the other subcapsular. Most of lymphatic
vessels have an upward trajectory draining towards the deep jugular lymph nodes
groups. Other lymphatic vessels drain the lymph downward, towards pretracheal and
mediastinal groups. Other lymph nodes groups are: perilaryngeal, deep lateral, and
submandibular. Lymphatic vessels may cross the midline draining into the contralateral
lymph nodes. These lymphatic groups should be known and removed by surgeons
during operations of neck dissection.
Innervation. The thyroid gland receives autonomic sympathetic fibers from the cervical
sympathetic chain, and parasympathetic fibers from the vagus nerves.
Histology. The histological structure of the gland is composed of many small globular
sacs called follicles. Follicles are lined by follicular cells and are filled with a fluid
known as colloid that contains a gelatinous substance consisting of proteins, mainly the
prohormone thyroglobulin and other iodoproteins and serum albumin. Between
follicles, there are fibroblasts, endothelial cells and C cells or parafollicular cells derived
from the neural crest. They secrete calcitonin.
Physiology. The thyroid is an endocrine gland that secretes several hormones of which
the most important are thyroxine (T4), triiodothyronine (T3) and calcitonin.
Calcitonin is secreted by C cells in response to the increase in calcium levels,
decreasing the bone resorption.
The thyroid gland has the ability to uptake iodine supplied by food and using it in
the synthesis of thyroid hormones. The thyroid cells, under the action of the TSH
(thyroid - stimulating hormone) and PRL (prolactin) hormones assimilate the inorganic
blood iodine. Iodine is mostly concentrated in thyroid gland. The thyroid iodine
concentration is 30-50 times higher than in blood.[2,3] The follicular cells, using some
enzymes, synthesize the thyroglobulin, which is stored in colloid. When thyroid
hormones are needed, thyroglobulin is reabsorbed from the colloid into the cells, where
it is split into its component parts, including the two thyroid hormones thyroxine (T4)
497
and triiodothyronine (T3). The hormones are then released, passing from the cells into
the blood stream. T3 is ten times more active than T4.[3] About 20% of T3 is produced
inside the thyroid gland whereas the remaining of 80% results from extrathyroidal
deiodination of T4.[4-6]
The rate of T4 production is 80-100 g per day.[3,7] Total daily production rate of
T3 is 30-40 g.[8] In the blood stream, the both T4 and T3 circulate predominantly
bounded to serum protein such as thyroxine-binding globulin (TBG), transthyretin
(TTR), albumin or lipoproteins.
Central regulation of thyroid hormone secretion. The thyroid gland is
controlled by the activity of the hypothalamic-pituitary-thyroid axis. (Figure 7)
Regulation of the biosynthesis and secretion of T3 and T4 is performed by TSH. Its
secretion is inhibited through a feedback mechanism by thyroid hormones and
stimulated by TRH.
An excessive amount of iodine administered
to a person with euthyroidism initial cause a
reduction in the synthesis and secretion of thyroid
hormone. The inhibitory effect is called Wolff-
Chaikoff [9] and is used in medical practice for
rapid reduction of thyroid function by
pharmacological doses of iodine. The iodine
administered preoperatively for 10-14 days has a
significant reducing effect of thyroid vascularity.
Thyroid hormones have vital effects on
tissue growth, brain maturation, calorigenesis or
oxygen consumption and specific effects on
multiple organs and systems:
On the cardiovascular system, hormones have a positive chronotropic and
inotropic effect.
On the respiratory system, hormones maintain the ventilatory response to the
hypoxia and hypercapnia.
On digestive system, hormones stimulate intestinal mobility leading to
diarrhea in hyperthyroidism and constipation in hypothyroidism.
On bones, high levels of hormones have a resorption stimulating effect with
hypercalciuria.
On neuromuscular system, thyroid hormones have important effects. The
fetal thyroid insufficiency is accompanied by severe irreversible mental
retardation. In adult, the excess of hormones can lead to muscles damage and
even cause proximal myopathy, hyperreflexia, tremor, and anxiety. In
thyroid insufficiency, the myxedema is associated with hyporeflexia,
depression, impaired memory, inattentiveness and somnolence.[10,11]
On reproductive system, particularly the hypothyroidism induces abnormal
uterine bleedings, amenorrhea, anovulation and subsequent infertility.
498
2. Methods of Investigation of the Thyroid Gland
Methods for investigations of thyroid gland can be classified as methods for
investigating the morphology of the gland and methods for investigating thyroid
function (functional tests).
Thyroid's morphology and its relations with surrounding anatomical structures,
besides the clinical examination, can be investigated by:
Ultrasound examination is the easiest, cheapest and noninvasive method of
investigation. Both thyroid lobes can be well visualized, assessing their
dimensions, structure and relations with neighborhood structures. It can
identify single or multiple nodular lesions and also establish the cystic or
solid nature of a nodule. (Figure 8) As well, enlarged cervical lymph nodes
are highlighted by this method. Ultrasonography also guides the fine needle
biopsy.
CT and MRI scans are valuable investigation methods especially used in
cases of thyroid cancer and retrosternal or ectopic goiters. It offers important
information about the position, dimensions and morphology of the thyroid
gland but also about regional lymph nodes involvement and the relation of
the tumor with the neighboring structures. (Figure 9)

Thyroid scintigraphy is a nuclear medicine procedure that uses radiolabeled
iodine (I
131
or I
123
) which is assimilated by the gland. At 30-60 minutes after
intravenous administration, a thyroid scan is obtained. Thyroid scintigraphy
gives information about the size and shape of the thyroid gland but also
about its function highlighting areas of hyper- or hypofunctional thyroid
tissues. It is very useful in differential diagnosis of hyperthyroidism. Another
advantage is that it can highlight the presence of ectopic or intrathoracic
thyroid tissue.
Fine needle aspiration biopsy (FNAB) or fine needle aspiration cytology
(FNAC) is an accurate diagnostic tool used in the evaluation of nodular
thyroid disease.
Thyroid biopsy was first described in 1930 by Martin and Ellis who used a thick
18-gauge needle for aspiration. Then, Silverman performed the first needle biopsy using
the Tru-cut type. Unfortunately, a long period these methods were less applied because
of fear to spread the cancer, of false negative results and of possible complications. In
the 1960
s
, Scandinavians introduced FNAB as a method that became widely accepted in
the U.S. only in 1980.[12,13]
Indications for FNAB are:[14]
A. For solitary nodule
1. Strongly consider FNA for:
499
a nodule 1.0 cm or more in largest diameter if microcalcifications are present
a nodule 1.5 cm or more in largest diameter if any of the following apply:
nodule is solid or almost entirely solid, or
there are coarse calcifications within the nodule.
2. Consider FNA for:
a nodule 2.0 cm or more in largest diameter if any of the following apply:
the nodule is mixed solid and cystic, or
the nodule is almost entirely cystic with a solid mural component, or
the nodule has shown substantial growth since prior US examination.
FNA is likely unnecessary if the nodule is almost entirely cystic, in the absence of the above-
listed features.
B. For multiple nodules. Consider FNA of one or more nodules, with selection prioritized on the basis of
the previously stated criteria in the order listed above. FNA is likely unnecessary in diffusely enlarged
glands with multiple nodules of similar US appearance without intervening normal parenchyma.
Thin needles, generally of 25-gauge, are used for FNAB (Cyba, Westcott,
Franseen, etc.). (Figure 10) The procedure can be performed without anesthesia or
under local anesthesia and under ultrasound guidance. (Figure 11) Fluid and cells, not
tissue samples, are extracted by this method. Usually a syringe is attached to the needle.
To extract cells from the thyroid nodule, active aspiration is applied, although there are
methods that are not using a syringe and nor aspiration. More types of vacuum devices
exist on the market but the principle is the same: creating a negative pressure into the
syringe. Six to ten smears are prepared from each nodule (harvested fluid is thinly
distributed across the slide) and then examined microscopically (cytology).

Regardless of the technique used, the material obtained by biopsy may be
adequate if there are at least six groups consisting of 10-15 epithelial cells. This should
be classified in one of the following five diagnostic groups:[13]
1. Non-diagnostic (either due to inadequate cellularity or technical problems) -
15%-20% of cases
2. Benign (features consistent with a multinodular goiter or thyroiditis) - 60-70%
of cases
3. Follicular lesions, including those where malignancy cannot be ruled out
4. Suspicious of malignancy - 10-12% of cases
5. Diagnosis of malignancy, with unequivocal features of papillary cancer,
medullary or anaplastic carcinoma or lymphoma or metastases - 20-25% of
cases
FNAB for thyroid nodules has a sensitivity of 83%, with a specificity of 92%.[13]
In 2007, the National Cancer Institute (NCI), Bethesda, Maryland, US, organized
the Thyroid Fine Needle Aspiration State-of-the-Science Conference, and established
guidelines using a standardized nomenclature for the interpretation of thyroid fine
needle aspirates known as the Bethesda system for reporting thyroid cytopathology.
[15,16](Table 1)

500
Table 1 - The Bethesda - correlation with the risk of malignancy and case management
Category Risk of malignancy (%) Management
Non-diagnostic 1-4 Repeat FNAB
Benign <1 To follow up
Atypia of Undetermined Significance (AUS) 5-10 Repeat FNAB
Suspicious for a Follicular Neo /Follicular
Neoplasm
15-30/15-45 Lobectomy
Suspicious for Malignancy 60-75 Lobecomy ot total
thyroidectomy
Malignant 97-99 Total thyroidectomy
Functional tests are represented by:
Dosage of thyroid hormones - free T4 (fT4) and free T3 (fT3) with normal
values of: fT4 = 9-30 pmol/L (0.7 - 2.5 ng/dL) and fT3 = 3 - 8 pmol/L (0.2 -
0.5 ng/dL).
Dosage serum TSH is the most commonly used test for assessing thyroid
function. It can be determined by the immunometric assay (IMA) or
radioimmunoassay (RIA). Normal values of TSH = 0.5 to 4.5 mIU / L.
Dosage of thyroglobulin is used in practice especially for monitoring of
thyroid remnants after thyroidectomy or recurrent thyroid cancers.
Radioactive iodine uptake test (RAIU) uses small doses of radiolabeled
iodine (I
123
isotope with a half-life time of about 13 hours, or I
131
of which
half-life time is approximately 8 days). After oral administration of iodine,
the absorption of radioactive iodine by the thyroid is studied after 46 hours
and after 24 hours with the aid of a scintillation counter. The method allows
differential diagnosis of thyrotoxicosis especially if used in combination
with thyroid scintigraphy. Normal values in countries with sufficient amount
of food iodine are 5-15% at 6 hours and 8-30% at 24 hours, for I
123
, and 20
+/- 5% at 2 hours, and 40 +/-5% at 24 hours, and 10-15% less than the
previous value at 48 hours, if using I
131
.
Other functional and dynamic tests as necessary.
3. Goiter - Endemic Thyroid Dystrophy
Goiter means the enlargement of the thyroid gland due to hyperplastic and
hypertrophic processes of dystrophic nature that affect thyroid follicles, connective and
vascular tissues.
It is considered an endemic disease because it is constantly found in certain
geographical areas with iodine deficiency in water and food (eg. Maramures region in
Romania). It is a thyropathic disease because in the centre of the disease the thyroid
glad is that whose function and structure is altered. It is a dystrophic disease because the
metabolism disorder affects the whole body.
Etiopathogenesis
1. Determinant conditions:
Exogenous factors - iodine deficiency in water and foods.[2,17,18] The
amount of iodine required is 100-150 g/day.[19,20] During pregnancy and
lactation period requirement is 200 g/day.[2,21] In the absence of iodine,
compensatory thyroid tissue hyperplasia occurs. In areas where the daily
501
iodine intake is <50 g, goiter is usually endemic, and when the daily intake
falls <25 g, congenital hypothyroidism is seen.[22]
Goitrogens such as cassava, cabbage, cauliflower, Brussels sprouts and
turnips. These interfere with T3/T4 synthesis.[23]
Endogenous factors:
Factors that interfere with intestinal absorption of iodine: intestinal flora,
excess of calcium, and magnesium salts. Fluoride causes increased
elimination of iodine.
Substances that inhibit the uptake of iodine in the thyroid (thiocyanate,
perchlorate) - cassava contains a thiocyanate which inhibits iodide
transport within the thyroid.
Substances that interfere with hormone biosynthesis: Thyouracil. In
excess, iodine administration does not have the opposite effect.
Drug induced goiter: sulfonamides and phenylbutazone inhibit
organification of iodine; iodine containing drugs such as amiodarone
interfere with thyroglobulin proteolysis; iodine or lithium interfere with
thyroglobulin breakdown and release of T3/T4 [23]
2. Adjuvant factors - socio-economic (diet)
3. Predominant biological factors - age, sex, repeated pregnancies,
breastfeeding
4. Genetic factors - unclear
Morphopathological types of goiters
1. Parenchymal goiter (diffuse hyperplasia). The gland is evenly enlarged with
smooth, regular, uniform appearance on sections. Histologically there is a
"macro- micro follicular colloid adenoma.
2. Nodular goiter - there are circumscribed nodules separated from the gland
by a fibrous tissue, which allows enucleation. Nodules may be solitary or
multiple (multinodular or polynodular goiter). (Figure12) Nodules may look
different on section: some of them have a solid consistency and others are
cystic. Histological appearance is that of acinar adenoma (vesicles of
different sizes) or trabecular adenoma (cords of epithelial cells without
vesicles with colloid).
3. Cystic goiter - there are multiple enlarged vesicles of a colloid goiter. The
content of cysts is serous or blood. The true cysts have a lining of secretory
epithelium, whereas in false cysts the epithelium is missing. Colloid goiter:
means massive storage of colloid within follicles often with flattened
epithelium.
4. Varieties: nodulocystic, calcified, vascular.

502
Another classification by location:
1) Goiters with normal location
2) Goiters with abnormal location
a) Plunged goiter. The goiter is plunged into the retrosternal space. It is
frequently encountered in elderly with pulmonary emphysema. Surgical
approach is more difficult in such cases.
b) Endothoracic goiter (medial or lateral). The goiter is migrated into
thoracic cavity but it keeps its connection with the thyroid gland. Thyroid
scintigraphy usually highlights the presence of the thyroid tissue inside
the thoracic cavity and then CT scan completes the investigation. The
approach for thyroidectomy in such cases is both cervical and thoracic
(thoracotomy).
c) Ectopic goiters are those goiters that do not have any connection with the
thyroid gland. They develop on heterotopic thyroid tissue islands. The
most common sites are the base of the tongue, larynx, trachea and rarely
the pericardium. Ectopic islands of thyroid tissue are highlighted by
scintigraphy.
d) Struma ovarii is a rare ovarian tumor defined by the predominant
presence of thyroid tissue. Most commonly, it occurs as part of the
ovarian teratoma.
Epidemiology
Almost one-third of the world's population lives in areas of iodine
deficiency.[1,18,22] It is estimated that goiters affect as many as 200 million of the 800
million people who have a diet deficient in iodine.[1]
Sex: The female-to-male ratio is 4/1 [24]
Age: The frequency of goiters decreases with advancing age, but on the other
hand, the incidence of thyroid nodules, increases with advancing age.
Clinical picture
In the initial phase, the patient is asymptomatic and there are no functional
disorders. The patient incidentally, or the doctor at a routine examination, discovers a
swelling in the neck. As the thyroid gland grows, the swelling becomes more and more
visible and different kinds of symptoms appear. (Figure 13) Symptoms are related to
compression exerted by the enlarged thyroid gland on the neighbor anatomical
structures such as the trachea, larynx, superior and inferior laryngeal nerves, and
esophagus.

503
Compressive syndromes:
On laryngeal nerves: - hoarseness,
dysphonia, bitonal voice
On larynx and trachea: - dyspnea of effort,
asphyxia
On esophagus: - dysphagia
On large vessels: - cyanosis of the face,
headache, epistaxis, tachycardia
On vagus nerves: - cardiac arrhythmias and
respiratory disorders
On cervical sympathetic chain: - Claude
Bernard-Horner Syndrome (myosis,
palpebral ptosis, enophtalmia, facial
redness). (Figure 14)
Physical examination of patient with goiter is best
performed with the patient upright, sitting or standing.
On inspection, symmetrical or asymmetrical deformation of the anterolateral neck
region can be observed. The skin is of normal appearance and non-adherent to the
gland. In very large goiters, dilated superficial venous network in visible. On palpation,
of the thyroid gland, some features of the goiter should be recognized and described.
The proof that the bulge belongs tot the thyroid gland is that it is mobile to lateral
mobilization and follows the craniocaudal
direction movements of the larynx during
swallowing. Any modification of each thyroid
lobes should be described separately (dimensions,
sensibility, consistency, etc.). Goiters are of
variable consistency. Parenchymatous goiter is
elastic; vascular goiter is depressible and soft bruit
could be heard on auscultation; colloidal goiter is
irregular, lobular and soft; nodular goiter
consistency is high and irregular.
Cervical lymph nodes must be palpated for
signs of metastatic thyroid cancer. (Figure 15)
Classification [25,26]
The classification of goiter, used in the 80
s
, concerning its size was the following:
Grade 0 no visible goiter and the thyroid gland is impalpable
Grade 1a the thyroid gland is palpable but it remains invisible, even in full
extension of the neck
Grade 1b goiter is palpable and visible in the full extension of the neck
Grade 2 goiter visible in neutral position of the neck
Grade 3 very large goiter, clearly visible from distance
WHO proposed simplified classification:[27]
Grade 0 no goiter presence is found (the thyroid impalpable and invisible)
Grade 1 neck thickening is present but not visible in normal position of the
neck. The thyroid is palpable. This grade includes also nodular goiters if
thyroid enlargement remains invisible
Grade 2 neck swelling, visible when the neck is in normal position,
corresponding to enlarged thyroid found in palpation.
504
Diagnosis of goiter is relatively simple base on physical examination (when the thyroid
is evidently enlarged) and on investigations. Investigations should appreciate the
volume of the thyroid gland, its structure (diffuse, nodular, cystic, vascular etc.) as well
its function (eu-, hyper- or hypothyroidism). Ultrasound of the thyroid gland is the most
frequent investigation used. Chest X-ray and CT or MRI scans are recommended
especially in plunged goiters, endothoracic goiters and suspicion of cancer. Whenever
cancer is suspected, a FNAB will be performed prior to operation.
Differential diagnosis usually includes:
Cyst of the thyroglossal duct - it is located above the thyroid gland.
Branchial cleft cysts - are not belonging to the thyroid gland and thus do not
follow the deglutition movements of the larynx.
Acute thyroiditis - acute inflammatory symptoms and signs are present.
Subacute and chronic thyroiditis - thyroid biopsy is important in diagnosis.
Hydatid cyst of the thyroid - very rare cases. Ultrasound may reveal a cyst
with multiple echoes, suggesting that the nodule could be a hydatid cyst.[28]
Thyroid TB - rare cases; history of TB infection may be present; usually
cervical lymph nodes are also involved [29]; acid fast bacilli (AFB) staining
and culture from fine needle aspiration (FNA) material may put the
diagnosis [30]; clinical and radiological features are nonspecific and
histological examination is required.[31]
Thyroid syphilis - rare cases; luetic history; specific serologic tests like
Wassermann, [32,33] Veneral Disease Research Laboratory tests (VDRL).
Thyroid cancer - there are some imagistic features that could suggest the
neoplastic etiology but histological examination is necessary.
Enlarged cervical lymph nodes - either inflammatory or tumoral, lymph
nodes are diagnosed by palpation (do not follow the swallow movement of
the larynx) and imagistic investigation (US, CT).
Parathyroid tumors - imagistic investigations make the difference.
Carotid artery aneurysm - the neck bulge is pulsatile and the fact that it
belong to the carotid is proven by imagistic investigations.
Cystic hygroma - is a cystic lymphatic lesion with predilection on the left
side of the neck.[34] Ultrasound examination reveals the fact that the lesion
does not belong to the thyroid gland.
Treatment
Prophylactic treatment consists of introducing iodized salt in food or
administration of potassium iodide solution.
Curative treatment is the same as in prophylaxis but with higher doses and more
prolonged. If after 2-6 months of treatment the goiter volume does not reduce, surgery is
indicated. Surgery is also indicated when there is a suspicion of malignant
transformation and in case of compressive complications.
Anesthesia. In nowadays, goiters are operated in general anesthesia by
orotracheal intubation. There have been developed special cannulas with sensors for
laryngeal nerves to monitor nerve damage during operation. In the past, local anesthesia
was used. The only advantage of this anesthesia was the possibility of intraoperative
recognition of laryngeal nerve damage indicated by changes of the patient's voice
(hoarseness).
505
The classical approach is the Kocher incision
but minimal invasive endoscopic techniques are
also available. After skin incision, the
subcutaneous hemostasis is performed and then
platysma muscle is cut and the superior skin flap is
prepared up to the thyroid cartilage. (Figure 16)
Then, follows the longitudinal sectioning of the
anterior raphe of the neck, which allows the
exploration of the both thyroid lodges.
Types of possible operations depending on
lesion extension are: (Figure 17)
Nodule enucleation
Unilateral subtotal hemithyroidectomy
Bilateral subtotal thyroidectomy
Total hemithyroidectomy (unilateral thyroidectomy or lobectomy)
Total thyroidectomy (bilateral total lobectomy)
Combinations of these operations and associated with sternotomy or
transthoracic approach for intrathoracic or plunged goiters.

The operation ends with the drainage of the
thyroid lodges, the suture of the anterior raphe of
the neck, subcutaneous suture and closure of the
skin using clips. Clips can be removed after 24
hours and drainage tubes usually after 48 hours.
(Figure 18)
Possible intraoperative complications are:
Bleeding - usually bleeding from
parenchyma is present because the
thyroid is a well-vascularized organ
and especially in hyperthyroidism
Gas embolism may occur in lesions of
the internal jugular vein
Cardiac arrest
Mechanical respiratory complication
506
Laryngeal nerves injuries
Early postoperative complications:
Bleeding, suppurations, seromas
Disorders of phonation and respiration (tracheal compression, laryngeal
edema, bilateral damage to the laryngeal nerves, tracheomalacia)
Spasmophilia crisis and tetany due to the removal of the parathyroid glands
Tracheomalacia is caused by the flaccidity of the tracheal cartilages, which
may appear in neglected cases of voluminous goiters. The anterior-posterior
airway caliber is reduced and the trachea collapses especially during of
increased airflow, such as coughing, or crying. Tracheostomy is indicated.
Thyroid storm is a rare but potentially fatal complication. It appears in
patients with hyperthyroidism, especially in those with insufficient treated
thyrotoxicosis. The complication is produced by an excess TSH release
caused by fast decreasing of blood levels of thyroxin after thyroidectomy.
The clinical picture is represented high fevers, tachycardia, tremors,
vomiting, sometimes jaundice, agitation, psychosis, coma and hypotension.
Untreated it may be fatal for the patient.
4. Other Causes of Enlarged Thyroid Gland
A. Solitary thyroid nodule
The incidence of solitary thyroid nodule is about 2-4% in adults,[35] but 50% of
cases are found in elderly. Usually women are most affected. Thyroid cancer
corresponds to 5-15% of nodules undergoing FNAB or surgery.[13,36-38]
Single and cold (on nuclear scan) nodules are risk factors for malignancy, but 80-
90% will be benign. Ultrasound features that are suggestive of malignancy
include:[14,39,40]
Micro-calcifications
Solid nodule with markedly reduced echogenicity
A nodule that is taller than it is wide
Direct invasion of the tumor into adjacent soft tissues
Metastases to lymph nodes
An ill-defined and irregular margin
Intrinsic vascularity
Risk factors for cancer of thyroid nodules are:[41,42]
Age: patients under 30 and over 60 years have a higher risk of cancer
Males
Associated symptoms such as difficulty swallowing or hoarseness
History of head and neck irradiation
Hard consistency lump
Enlarged regional lymph nodes
Previous history of thyroid cancer in the family
A history of multiple endocrine neoplasia type 2
Thyroid nodules should be evaluated with ultrasound guided fine needle
aspiration to rule out malignancy.
507
B. Acute thyroiditis
Thyroid gland infection occurs via blood or by direct seeding from upper
respiratory infections. Patient complains of sudden onset pain, fever and swelling of the
anterior region of the neck. Gross appearance is that of a normal or slightly enlarged
painful thyroid gland with Celsian signs, and suppurative areas may be present.
Treatment consists in antibiotherapy, and in case of abscess formation incision
and drainage.
C. Subacute thyroiditis (also called de Quervains thyroiditis or
granulomatous thyroiditis)
It is a rare condition, much less common than Hashimotos thyroiditis, but the
most common cause of thyroid pain. It occurs in 75% of cases in women, usually at age
of 30-50 years.[43]
The gross appearance is that of focal to diffuse enlargement of the thyroid gland
up to twice-normal size. It may be asymmetric with nodules of variable size. May be
firm, but does not adhere to surrounding structures.
The etiology may be systemic viral infection, since associated with epidemics of
measles, mumps, Coxsackie, adenovirus and influenza.
It is self-limited, and usually resolves in 6-8 weeks.
Treatment consists in administration of anti-inflammatory drugs.
D. Hashimotos thyroiditis (lymphocytic thyroiditis)
It is an autoimmune disease associated with goiter. There are elevated circulating
anti-thyroid antibodies. The disorder affects up to 2% of the general population.[44]
More than 90% of cases occur in women, aged between 30 and 50 years.[43,45,46]
Clinical picture consists in painless, gradual diffuse (symmetric) enlargement of
the thyroid gland (pyramidal lobe may be prominent) with thyroid failure
(hypothyroidism) due to autoimmune destruction of thyroid tissue.
Histological features include a dense thyroidal accumulation of lymphocytes,
plasma cells and occasional multinuclear giant cells.[45] The gland may have adhesions
but it can be easily separated from other structures. Hashimotos thyroiditis often
coexist with other autoimmune diseases such as type 1 diabetes, celiac disease,
rheumatoid arthritis, multiple sclerosis, vitiligo, etc.[45,47-50]
The treatment of choice for Hashimoto's thyroiditis is thyroid hormone
replacement with levothyroxine sodium, usually for life. Surgery is indicated only in
cases with compressive syndromes, malignant transformation and on demand for
esthetic reasons.
E. Riedels thyroiditis (fibrous or woody thyroiditis)
A densely fibrotic inflammatory process involves the thyroid gland and adjacent
neck tissues.[51] It is a rare condition (0.05% of thyroidectomy specimens) [52], with
slight female predominance, usually at ages of 40-60 years. It can be associated with
inflammatory and multifocal systemic fibrosclerosis.[51,53] The etiology is unknown.
The gross appearance is that of extensive stony hard fibrosis involving the thyroid
gland and infiltrating the adjacent anatomical structures, obliterating dissecting planes at
surgery. Fibrosis binds the soft tissues of neck in an iron collar that may compress the
trachea.[52]
508
Treatment consists in administration of corticosteroids, tamoxifen, and
levothyroxine.[51,54] Because of the high incidence of complications, surgery is
indicated only for two purposes: for establishing the diagnosis (to rule out a cancer) and
for relieving tracheal compression.[55] A wedge resection of the thyroid isthmus
remains the preferred method for accomplishing these ends.
5. Hyperthyroidism
Hyperthyroidism represents the over activity of the thyroid gland which produces
excessive thyroid hormones and accelerates metabolism in the peripheral tissues.
Clinical picture
The clinical picture depends on the stage of the disease. There are described four
stages:
1. Neurogenic with nonspecific symptoms
2. Neurohormonal with all specific symptoms due to increased thyroid
hormone secretion
3. Visceral stage - with complications especially cardio-vascular
4. Cachectic stage
GRAVES' DISEASE, which is the most common form or cause of hyperthyroidism, is
an autoimmune disorder with thyrotoxicosis. Out of all patients, 85% are women,
usually aged between 20-40 years.
Classical clinical presentation was described by Graves Basedow and
characterized by the triad:
1. Goiter
2. Exophthalmos
3. Tachycardia
The condition starts with neuro-psychological symptoms manifested by emotional
lability, irritability, psychomotor instability, hyperactivity, agitation, loss of memory.
Cardiovascular symptoms and signs are the most important being early and
constant, manifested by palpitations, tachycardia, arrhythmias, heart failure, and toxic
myocarditis. Tachycardia usually exceeds 100 beats/min and is persistent during sleep
and worse at efforts. On auscultation, functional systolic murmur can be heard. Systolic
pressure increases and diastolic pressure decreases.
Thyroid hypertrophy is almost constant. The gland consistency is elastic, the
surface is smooth and on auscultation, thyroid thrills and bruits could be heard. In
thyroid toxic adenoma (TTA) a hypersecretory solitary nodule is found.
Changes of skin and appendages are also present manifested by pink, warm,
moist, smooth skin with increased sweating. Other signs are: perithyroidian red spots
(Maranon sign), presternal red spots (spots of "shame"), pretibial myxedema. Diffuse
pigmentation disorders may appear such as: melasma (also known as "Chloasma
faciei), vitiligo, alopecy, white hairpiece (Sabouraud sign), axillary hair loss (Williams
sign). The nails are brittle with longitudinal and transverse striations.
Due to the increased metabolism, the patient presents weight loss despite of
increased appetite. Bowel movements are accelerated.
The patient also presents termophobia (heat intolerance) and increased tolerance
to cold.
509
Menstrual periods are irregular or absent and in males enlarged breasts may
develop.
Neurological manifestations can include finger tremor, eyelid tremor and tongue
tremor.
Exophthalmos includes bulging of the eyes,
eyelid retraction infrequent blinking, and light
sensitivity. The patients have a prominent staring
appearance, an expression of fright or extreme
anxiousness. (Figure 19) Protrusion of the eyes is
due to immune-mediated inflammation in the
retro-orbital fat.
Clinical forms
1. Pure hyperthyroidy (Thyrotoxicosis)
2. Graves-Basedow disease
3. Toxic adenoma (Plummers disease)
4. Hyperthyroidized goiter
Clinical forms by evolution:
Acute hyperthyroidism
Chronic hyperthyroidism
Clinical forms by symptoms:
Forms with predominant central signs (exophthalmia)
Forms without thyroid hypetrophy
Cardiothyreosis
Forms with predominant digestive syndromes
Particular forms:
Hyperthyroidism on a thyroid cancer
Iatrogenic hyperthyroidism (Jod-Basedow phenomenon - Kocher) [56]
Reactive hyperthyroidism in other diseases or poisonings
Investigations
Laboratory test:
Thyroid Stimulating Hormone (TSH), produced by the pituitary gland
will be low in hyperthyroidism
Free T4 (free thyroxine) levels are elevated
Triiodothyronine (T3) radioimmunoassay (RIA) or free T3 levels are
also elevated
Thyroxine (T4) level is elevated
Thyroid autoantibodies: TSH receptor antibodies (TRAb) or Thyroid-
stimulating immunoglobulin (TSI) contribute to the diagnosis
Thyroid Binding Globulin (TBG) testing is not very useful
PBI = protein bound iodine (4-8 gamma%) or BEI= butanol extracted
iodine
BMR (basal metabolism rate with normal values of 5+15%) is
increased
Imaging methods:
Ultrasound
510
Iodine Uptake Test. Normal: 0-45% at 10 min, 20+-5% at 2 h, and 40+-
5% at 24 h
Radioactive iodine thyroid scan-with either I
132
or 99mTc
CT scan
Electrocardiography (ECG)
Thoracic and cervical radiography
Invasive methods:
Fine Needle Aspiration Biopsy (FNAB)
Treatment
There are three recognized modalities of treatment for hyperthyroidism: anti-
thyroid drugs, surgery and radioiodine.[57]
Anti-thyroid drugs. Two common drugs, which actually interfere with the thyroid
gland's ability to produce its hormones, are used: Methimazole (Tapazole, Thyrozol) or
Propylthiouracil (PTU). Other medicines are also prescribed depending on associated
pathology, such as beta-blockers (propranolol).
Radioactive iodine treatment. This treatment takes advantage of the fact that
thyroid cells are the only cells in the body, which have the ability to absorb iodine. By
giving a radioactive form of iodine, the thyroid cells, which assimilate it, will be
damaged or killed and so the thyroid hormones synthesis is lowered.
Surgical removal of the gland or part of it, or of the hypersecretory nodule, is
another alternative. Indications for surgery are:[58-60]
Absolute:
Hyperthyroidism associated with thyroid cancer
Toxic Thyroid Adenoma
Severe forms that do not respond to conservative treatment during the
last 6 month
Visceralized hyperthyroidism
Hyperthyroidized goiters
Relative:
All other forms that do not have absolute indications
Hyperthyroidism of the child
Hyperthyroidism during pregnancy
Cosmetic reasons
Contraindications for surgery are:
Forms without thyroid hypertrophy
Multiple endocrine forms
Forms with predominance of the central component
Forms associated with other more severe illnesses
Forms associated with psychotic disease
Preoperative preparation is very important in order to prevent complications such
as thyroid storm. General measures consist of bed rest, isolation, hypocaloric diet,
eliminating stimulants. Specific measures are represented by sedative and hypnotic
medication (valium, fenobarbital, etc.), administration of Lugol (reduces the gland
volume and increases its consistency), administration of anti-thyroid medication will be
discontinued with 10-15 days before surgery, and associated medication as needed
(Hydrocortisone 100 mg/day, Cardiotonics, Propranolol, etc.).
511
When is the right moment to operate? Criteria:
Decreased heart rate below 100/min
Rebalancing of metabolism
Weight gain
Disappearance or improvement of neuropsychological agitation
Decreasing the gland volume with increasing its consistency
Surgical treatment is generally represented by subtotal bilateral thyroidectomy in
order to reduce the thyroid gland volume and hence the hormones levels. Sometimes,
even total thyroidectomy is recommended in nodular or cystic goiters especially when
cancer is suspected. Toxic adenoma will be removed by enucleation or unilateral
thyroidectomy.
6. Thyroid Cancer
Epidemiology. Thyroid cancer is the most common endocrine malignancy, accounting
for 1 % of all new malignant tumors.[61,62] In the last decades, thyroid cancer incidence
has continuously and sharply increased all over the world.[63,64] The increase mainly
regards papillary cells type tumors. It is estimated that 60,220 men and women (14,910
men and 45,310 women) will be diagnosed with, and 1,850 men and women will die of
cancer of the thyroid in 2013.[65] In 2008 there were an estimated 33,600 new cases of
thyroid cancer diagnosed in the European Union (EU-27). The highest incidence rate
was estimated to be in France, where the female rate was five times higher than the rate
of the lowest ranking country, Greece (18.6 versus 3.3 per 100,000 females).[66]
Female/male ratio is about 3:1. In about 80-90% of cases, cancer appears on a
preexisting nodular goiter. The disease is most frequent at ages between 30 and 60.
Etiology. Causing factors are not known but radiation exposure significantly increases
the risk of developing thyroid malignancies, particularly papillary thyroid carcinoma
(after the nuclear accident at Cernobil the incidence raised significantly).[63,67] Medical
radiation exposure of the thyroid at a young age is a recognized risk factor for the
development of differentiated thyroid cancer lasting for four decades and probably for a
lifetime after exposure.[68] Hashimotos chronic thyroiditis is considered a preneoplastic
condition.
Classification. From histological point of view, thyroid cancers are classified as
follows:[69,70]
Papillary carcinoma the most frequent encountered (60-86%), derived
from follicular cells
Follicular carcinoma (9-15%) - also derived from follicular cells
Anaplastic carcinoma (1-5%)- the most aggressive form
Medullary carcinoma (2-8%) derived from neuro-endocrine calcitonin
producing cells (C cells)
Lymphoma - derived from intra-thyroid lymphoid tissue
Sarcoma - derived from connective tissue
Histological type is an important determinant of prognosis in thyroid cancer.
TNM staging [71-74]
Primary tumor (T)
T0 No evidence of primary tumor is found
512
T1 Tumor size 2 cm in greatest dimension and is limited to the thyroid
T1a Tumor 1 cm, limited to the thyroid
T1b Tumor > 1 cm but 2 cm in greatest dimension, limited to the thyroid
T2 Tumor size > 2 cm but 4 cm, limited to the thyroid.
T3 Tumor size >4 cm, limited to the thyroid or any tumor with minimal extrathyroidal
extension (eg., extension to sternothyroid muscle or perithyroid soft tissues)
T4a Moderately advanced disease; tumor of any size extending beyond the thyroid capsule
to invade subcutaneous soft tissues, larynx, trachea, esophagus, or recurrent laryngeal nerve
T4b Very advanced disease; tumor invades prevertebral fascia or encases carotid artery or
mediastinal vessel
All anaplastic carcinomas are considered stage IV:
T4a Intrathyroidal anaplastic carcinoma
T4b Anaplastic carcinoma with gross extrathyroid extension
Regional lymph nodes are the central compartment, lateral cervical, and upper mediastinal lymph
nodes:
NX Regional nodes cannot be assessed
N0 No regional lymph node metastasis
N1 Regional lymph node metastasis
N1a Metastases to level VI (pretracheal, paratracheal, and prelaryngeal/Delphian lymph
nodes)
N1b Metastases to unilateral, bilateral, or contralateral cervical (levels I, II, III, IV, or V) or
retropharyngeal or superior mediastinal lymph nodes (level VII)
M0 No distant metastasis is found
M1 Distant metastasis is present
Stage grouping. Separate stage groupings are recommended for papillary or follicular (differentiated),
medullary, and anaplastic (undifferentiated) carcinoma.
For papillary and follicular thyroid cancer (age < 45y):
Stage T N M
I Any T Any N M0
II Any T Any N M1
For papillary and follicular; differentiated (age 45y):
Stage T N M
I T1 N0 M0
II T2 N0 M0
III T3 N0 M0
IVA T1-3 N1a M0
T4a N1b M0
IVB T4b Any N M0
IVC Any T Any N M1
Medullary Thyroid Cancer
Stage 0 - no tumor can be found in the thyroid.
Stage I - tumor < 2 cm
In stage II - tumor is > 2 cm confined to the thyroid gland, or tumor of any size that has
spread to tissues just outside the thyroid, but not to lymph nodes.
Stage III - the tumor is any size, has spread to lymph nodes near the trachea and the larynx,
and may have spread to tissues just outside the thyroid.
Stage IV
IVA - the tumor is any size and cancer has spread outside the thyroid to tissues under
the skin, the trachea, the esophagus, the larynx, and/or the recurrent laryngeal nerve;
cancer may have spread to lymph nodes near the trachea, or the larynx, or both sides of
the neck or between the lungs.
IVB - cancer has spread to tissue in front of the spinal column or has surrounded the
carotid artery or the blood vessels in the area between the lungs. Cancer may have
spread to lymph nodes.
513
IVC - tumor is any size with distant metastases
Anaplastic tumors are always considered as in fourth stage.
Stage IVA - cancer is found in the thyroid and may have spread to lymph nodes.
Stage IVB - cancer has spread to tissue just outside the thyroid and may have spread to
lymph nodes.
Stage IVC - cancer has spread to other parts of the body, such as the lungs and bones, and
may have spread to lymph nodes.
Preclinical clandestine life of the tumor may last more than 20 (5-35) years [75]
and without any treatment, the clinical evolution may last 12-18 months. About 10-15%
[76] (in some series 50-90%) [77,78,82] of patients with papillary and follicular cancers,
present with lymph node or lung metastases at diagnosis. Anaplastic cancer has a rapid
course and early dissemination.
Symptoms are few and unspecific. The patient observes an accelerated growth in
volume of the gland with appearance and accentuation of compressive symptoms
(dysphonia, hoarseness, dysphagia, etc.).
On physical examination, nothing special can be noticed or, the patients or
physicians, on routine palpation of the neck discover a painless, palpable, solitary
thyroid nodule. Other pathological findings may include:
Increased consistency of the gland
Palpable cervical lymph nodes
Palpable metastases
Penetrating in the surrounding tissue
Skin ulceration in very advanced cases (Figure 20)
Signs of compression on anatomical structures (eg. Claude Bernard H.
syndrome)

Investigations
Imaging:
Ultrasound - cannot distinguish benign from malignant nodules
CT and MRI scans - evaluate soft-tissue and extension of large or
suspicious thyroid masses
Radioiodine imaging - can determine the functional status of a nodule but
carcinoma cannot be excluded based on radioiodine scans.
Laboratory:
Elevated serum calcitonin is highly suggestive of medullary thyroid
carcinoma

514
Histological diagnosis:
Fine needle aspiration biopsy (FNAB)
Frozen sections examination
Histopathological examination of the operatory specimen
Treatment of thyroid cancer is multimodal and multidisciplinary depending much on
histological type, and it includes:[74]
1. Surgery
2. Radiation therapy, including radioactive iodine therapy
3. Chemotherapy
4. Thyroid hormone therapy
5. Targeted therapy
6. New types of treatment in clinical trials
Curative intention surgery serves to remove as much as possible thyroid tissue
(total thyroidectomy) and the associated metastatic lymph nodes (neck dissection).
(Figure 21 and 22) Thus, adjunctive radioiodine treatment is more likely to destroy
small amounts of remaining thyroid tissue or that from metastases.

Neck dissection is indicated
when pathological lymph nodes
enlargement are demonstrated by
clinical examination, imaging or
intraoperatively. There are
controversies over prophylactic
neck dissection in patients
without detectable nodal enlargement due to the lack of definitive evidence of improved
recurrence rates or survival.[76] Yet, some authors consider that when no macroscopic
evidence of metastasis is present, ipsilateral central neck dissection is the best treatment
strategy.[80]
Palliative surgery is addressed to advanced cases of thyroid cancers when the
gland cannot be removed, and operations are aimed to improve the patient's condition
and prolong life. Tracheostomy is performed to prevent asphyxia by tracheal
compression and feeding gastrostomy to prevent death by starvation when the
pathological process penetrates the esophagus.
Adjunctive therapy is represented by:
Radioiodine therapy in differentiated cancers in which cells uptake Iodine
(papillary and follicular cancers)
Local radiotherapy in forms without iodine uptake
515
Radioimunotherapy
Cobaltotherapy
Hormonal therapy, regional intra-arterial chemotherapy
About 4-6 weeks after surgical thyroid removal, radioiodine therapy is started to
detect and destroy any metastasis and any residual thyroid tissue. Therapy will be
administered until no further radioiodine uptake is noted.
Patients will take thyroid replacement therapy (L-thyroxine) for life, especially
after total thyroidectomy.
Treatment of anaplastic cancer is not standardised and there is not yet an effective
treatment, the results being disappointing.
Prognosis
Most thyroid cancer has a good prognosis with 90% survival at 10 years except
the anaplastic cancer, with a 5-year survival of 5%.
The prognosis depend on the following:[74,81]
The age of the patient
The type of thyroid cancer
The stage of the cancer
The patient's general health
Whether the patient has multiple endocrine neoplasia type 2B (MEN 2B)
Whether the cancer has just been diagnosed or has recurred (come back)
Tumors are slow growing and are associated with a very favorable prognosis
(except anaplastic type). (Table 1) [82]
Table 1 - Thyroid cancer prognosis
Thyroid
cancer
type
5-year survival
10-year
survival
Stage I Stage II Stage III Stage IV Overall Overall
Papillary 100% 100% 93% 51% 96% - 97% 93%
Follicular 100% 100% 71% 50% 91% 85%
Medullary 100% 98% 81% 28% 80% - 86% 75%
Anaplastic
(always stage
IV)
7% 7% - 14% (no data)

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82. Thyroid cancer - Prognosis - http://en.wikipedia.org/wiki/Thyroid_cancer

Breast Pathology
BREAST PATHOLOGY

1. The importance of breast pathology
2. Surgical anatomy of the breast
3. Clinical examination of the breast
4. Malformations
5. Inflammations
6. Benign tumors of the breasts
7. Nipple discharge
8. Malignant tumors of the breast
9. Particular forms of breast cancer

1. The Importance of Breast Pathology
The breast may be affected by a wide variety of pathology. Both benign and
malignant types of pathology affect with predilection women at any age but may affect
men also.
Breast cancer, as well as cancer in general, remains a particular health problem
despite of worldwide efforts of researchers and physicians and huge funds allocated for
prevention and treatment of this disease.
Facts:
Breast cancer is the most common cancer in women worldwide
Breast cancer is the third disease among the most common diseases in the
world
One of eight women is likely to develop breast cancer in their lifetime
In USA 2/3 of operated women for breast cancer, do not have axillary lymph
node metastases
In Romania 85% of women operated with breast cancer are in advanced
stages.
Particular factors to be considered in breast pathology
The breast, in women, beyond the role of breastfeeding (which is however
limited to a short period of lifetime), has mainly an erotic and esthetic role,
and therefore, the breast pathology has a strong echo in the mental sphere of
the woman.
Surgery, where is indicated, takes into account more and more the psycho-
affective aspect, tending to be less mutilating possible, but however it must
also consider the invasive nature of the disease.
The breast is an easy accessible organ for examination, facilitating early
detection of pathological changes. The sooner a breast lesion is discovered,
the smaller will be the unpleasant consequences of the possible surgery.

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Breast Pathology
2. Surgical Anatomy of the Breast
Solid knowledge about breast anatomy is of particular importance for clinician
especially in cases of cancer, for several reasons:
To be able to establish a correct diagnosis
To be able to perform adequate surgery
To perform the radiotherapy in indicated regions
To be able to foresee relapses of breast cancer
Embryology
Mammary glands begin to develop from the 6
th

week of intrauterine life from two ectodermic thickening
called mammary crests. These crests are located along
the lines described by Schultze in 1892 as "milk lines"
which join the axillary region to homolateral groin.
(Figure 1)
Along these mammary crests, there are 6-7
nodules, which in mammals will develop more pairs of
mammary glands. In humans, the caudal portion of the
crests will disappear remaining only the thoracic
portion. This will determine, in normal cases, the
development of one single pair of mammary glands in
pectoral region. There are cases when supernumerary
mammary glands or just components of the breast (eg.
nipples) may develop along the milk lines.
In women, initially under the action of estrogen
and subsequently in combination with progesterone,
mammary glands undergo significant changes until to
the stage of mature breast. This process generally takes
3-4 years and is completed at the age of about 16.
Subsequent physiological changes occur during
pregnancy, lactation, and menopause.
In men, normally breasts remain almost
unchanged, but size and structure changes may occur
under certain conditions under the influence of various
hormonal factors (gynecomastia).
Location. Breasts are located in the anterior-superior
region of the chest. In women, the classical breast area
is extended craniocaudal between the second and the
sixth rib and in transverse direction between the margin
of the sternum and the anterior axillary line. (Figure 2)
In men this area is limited solely to the breast areola
region, being rudimentary represented.
The breast is enclosed between two thin sheets of
superficial fascia of the pectorals.
Extension. The mammary gland area in women is
extended in craniocaudal direction between the clavicle
and the superior margin of the rectus abdominis and in
transverse direction between the margin at the sternum
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Breast Pathology
and latissimus dorsi muscle. (Figure 3)
The mammary gland has a more extended area than the breast. This aspect is very
important because on this area, pathological processes of the breast tissue may occur,
and also for surgery because these are the limits within which the surgeon must perform
a radical mastectomy for breast cancer.
The axillary extension of the mammary gland (axillary tail) is of special interest
because it is often affected by pathological processes. In some women, this extension is
well represented and can be confused with a lipoma, an axillary lymphadenopathy, or a
supernumerary breast. Usually it becomes more evident during the premenstrual period
and lactation.
The internal surface of the gland is attached to the pectoral fascia by fibrous strips
called Coopers ligaments.
External appearance. Breasts look like two hemispherical shape masses, whose size
and weight vary from person to person based on race, age, and the various physiological
stages. Generally, the left breast is bigger than the right one.
In the central zone, there is the areola, a hyperpigmented skin area, whose size
also varies from person to person.
Under the areolas skin, there are many nervous fibers and smooth muscles
arranged in circular and longitudinal layers, which by contraction decrease and wrinkle
the surface causing elongation and turgor of the nipples.
The areola has small prominences, the Montgomery tubercles, which are large
sebaceous glands, which increase in volume during pregnancy and lactation.
The nipple is a cylindrical-conical prominence, of 10-12 mm length and 8-10 mm
in diameter. There are 15-20 galactophorous pores of the lactiferous ducts.
For better guidance in locating various pathological processes, the breast was
arbitrary divided into four quadrants by two lines, one vertical and the other
perpendicular through the centre of the nipple.
1. Upper outer (superolateral or superoexternal - SE) - over 50% of breast
cancers are located in this quadrant.
2. Lower outer (inferolateral or inferoexternal - IE)
3. Upper inner (superomedial or superointernal - SI)
4. Lower inner (inferomedial or inferointernal - II)
Two more quadrants are added: one central (C) corresponding to retroareolar area
and another, which is the axillary extension quadrant (AE) of the gland.
The structure of the breast
The breast is composed of four types of tissues:
1. Milk-producing mammary gland,
2. Milk ducts,
3. Fatty tissue, and
4. Connective and fibrous tissue, blood vessels, lymphatics and nerves
The gland is compartmentalized in 15-20 lobes. It is entirely enveloped by fatty
tissue with the exception of the retroareolar region.
The adipose layer under the skin is organized in lodges separated by fibrous
bands (Coopers ligaments), which on palpation give the feeling of a granular surface.
Do not confuse these fatty lodges with tumors.
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Breast Pathology
The gland itself is fixed to the internal surface of the derma by the Duret crests,
which are of special importance because via these structures the malignant process can
spread to the skin. When these crests are invaded by the tumoral process, the skin
becomes fixed to the gland and it can be retracted, which represents a clinical sign for
cancer.
The retromammary layer is a fatty space by which the mammary gland can slide
over the surface of the pectoral major fascia. Invasion by cancer of this layer and
penetration into the pectoral muscle, leads to fixation of the gland to the underlying
muscular plane. Fixation can be highlighted by the Tillaux maneuver.
In very rare cases, the glandular tissue may cross through the retromammary fatty
layer penetrating into the pectoral muscle. This was the reason why Halsted radical
mastectomy removed the pectoral muscles along with the breast.
Microscopic anatomy. The glandular parenchyma is divided into lobes (15-20), lobules
and acini. In their delimitation contributes the stroma represented by interlobular dense
connective tissue and intralobular loose tissue, forming septa along which blood vessels
and lymphatics are passing.
The functional units of mammary gland are the acini. Each lob has a milk duct
which opens separately at the surface of the nipple. The lobes are orientated radially
around the areola. Each lactiferous (milk) ducts has a lactiferous sinus located at the
base of the nipple. The diameter of these ducts is 2 to 4 mm. The lining of the ducts is
composed of a double layer epithelium formed by cuboidal cells at the level of lobules
and cylindrical shape cells in the extra-lobular space. In the external layer,
myoepithelial cells are present. These ducts are responsible for the majority of breast
pathology. It is considered that neoplastic lesions have the starting point is these ducts
and less in the acini. The breast fibrocystic disease has also the starting point in these
ducts.
Vascularization and innervation
Breasts have a good arterial supply from many sources represented by:
Internal mammary artery (emerging from subclavian artery)
Lateral thoracic artery ( emerging from axillary artery)
Intercostal artery branches (from thoracic aorta)
Other sources such as: thoracica suprema artery, arteries for pectoral
muscles, thoracoacromial artery, subscapular artery, thoracodorsal artery
and superficial thoracic artery.
The venous drainage of the breast is organized into two networks: one superficial
and another one deep. The superficial network forms around the areola a venous plexus
called the Hallers circle. Through the venous system cancerous cells are carried to the
first filtrating station, the lungs, and then towards other organs where metastases are
relatively frequent: liver, bones, brain.
Brachial cutaneous nerve and branches from the intercostal nerves 4, 5, 6, provide
breast skin innervation. Breast parenchyma receives sympathetic branches that reach the
secretory units along the intercostal nerves 2, 3, 4, 5 and 6.
The lymphatic system is of particular interest in breast surgery because the
tumoral spread mainly on this route with particularly prognostic effect.
There is a superficial network, which collects lymph from skin, and a deep
parenchyma network. Between those two lymphatic networks, there are two areas of
connection, one at the areola, where there is a superficial lymphatic plexus and one
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Breast Pathology
retroareolar (Sappey), and the other at the breast periphery. Along these lymphatic
routes, propagation of the cancer is possible from depth to surface, which is the base for
indication to remove the areola in mastectomies for breast cancer.
There are two main lymphatic drainage routes:
1. External mammary route (axillary route) - it drains the lymph to the
ipsilateral axillary lymph nodes and 75%-97% of cancerous cells are
carried through this route. On the trajectory of the route there is a lymph
node (called Sorgius) located at the edge of the greater pectoral muscle.
2. Internal mammary route, which travels along the internal mammary
arteries towards the internal thoracic lymph nodes (internal mammary
lymph nodes), located retrosternal in the intercostal spaces 1 to 5.
The next lymph nodes stations are:
Supraclavicular lymph nodes
Cervical lymph nodes
On the left side toward the thoracic duct and left lymphatic duct
On the right side toward the right lymphatic duct
The Pirogoff jugulo-subclavian confluence
The mediastinal lymph nodes and broncho-aortic nodes
Besides these primary routes, there are other secondary routes such as:
1. Transpectoral route - starts from the inner surface of the breast, passes
through the pectoralis major to the Rotters interpectoral lymph nodes and
then to the axillary apical lymph nodes
2. Retropectoral route - starts from the inner part of the SE quadrant, passes
behind the pactoralis major towards the apical lymph nodes.
3. Intercostal route - along the intercostal vessels to the intercostal lymph
nodes and from there to the internal mammary lymph nodes
4. Contralateral axillary route - although rare, it is still possible that tumoral
cells from breast cancer to reach the lymph nodes from contralateral axilla
5. Inferior route - described by Gerota (Romanian physician and anatomist),
which drains the lymph towards the epigastric region and diaphragmatic
nodules
Anatomy of the axilla
Axilla is a pyramidal shape structure with the tip facing the cervical region, which
is the junction between the arm and the chest. It has a tip, a base and four walls. The
content of the axilla is represented by:
Arteries:
o Axillary artery,
o Lateral thoracic artery,
o Subscapular artery,
o Thoracodorsal artery
Nerves:
o Brachial plexus,
o Intercostobrachial nerve,
o Thoracicus longus (Charles Bell - respiratory) nerve,
o Subscapular nerve,
o Thoracodorsal nerve,
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Breast Pathology
o Intercostal nerves
Fatty tissue
Lymph nodes
Anglo-American surgeons use Bergs axillary lymph nodes classification that
divides lymph nodes into three main levels based on prognosis importance and their
relations with the pectoralis minor muscle:
1. Level 1 - are lymph nodes located under the lateral edge of the pectoralis
minor (lateral posterior and anterior)
2. Level 2 - are lymph nodes lying underneath the muscle between the
medial and lateral edge
3. Level 3 - lymph nodes located medial or above the medial edge of the
muscle (apical, subclavian)
In fact, this classification is also used in the TNM system for malignant breast
tumors. To this group, called regional lymph nodes (N), in the TNM classification is
also added the ipsilateral internal mammary lymph group. Intramammary lymph nodes
are encoded as axillary lymph nodes in the TNM classification. Any other metastatic
lymph nodes are coded as distant metastases (M1) including subclavian, or contralateral
cervical lymph nodes.
3. Clinical Examination of the Breast
The diagnosis of breast pathological processes is based on three elements:
1. History
2. Clinical examination
3. Investigations
Clinical examination is the most important phase of the diagnosis because:
In most cases, it is the first method of diagnosis,
In advanced cases, the method is sufficient, the physical signs of neoplasia
being obvious,
Local examination performed by woman itself (self-examination) is the
most effective method of screening for early detection of breast cancer if
other better methods are not available through a national screening
program.
Unfortunately, 40% of tumors detected at mammography cannot be detected by
palpation! Clinical examination of the breasts just is not enough! General clinical
examination of the patient should also be performed. Not only the breast considered ill
will be examined, but also:
The contralateral breast
The both axillae (armpits)
The supraclavicular and cervical lymph nodes
The examination can be performed both in orthostatic and supine position. The
standing position is preferred. Phases of clinical examination of the breasts are:
inspection and palpation.
Inspection. The patient stands in front of doctor with arms hanging. The
following aspects will be noted:
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Breast Pathology
The position of the line between the two nipples. This is supposed to be
horizontal in normal cases. If there are changes in shape and volume of the
breast the line becomes oblique.
The breast volume. Any expansive process in the breast will lead to more
or less increase in volume of the breast. This aspect is often observed in
phyllodes tumors.
The shape of the breast. As long the tumor is small enough no changes in
breast shape will be noticed. As it grows, alteration of breast shape will
appear as irregular elevations (bulges) or as depressions (skin retraction).
The aspect of the skin. The color can be normal. In some advanced cases
and in acute inflammatory breast cancer, the color turns to reddish as in an
inflammatory process making possible confusions with acute mastitis.
Skin surface may look as an orange peel (peau dorange) very suggestive
for breast cancer. It is caused by lymphatic stasis in the derma as the
lymph ducts are blocked by the tumoral process. Ulceration of the skin
appears in advanced cases. Venous network becomes visible being
augmented in advanced cases, and in phyllodes tumors. Nodules of
permeation appear also in advanced neglected cases.
The aspect of the areola. Tumoral processes like Pagets disease may
affect the areola. The aspect is similar to eczema.
The aspect of the nipple. Very suggestive for cancer is the unilateral
recently installed retraction of the nipple.
Pathological discharge from the nipple. Discharge could be serous,
brownish, lactescent, purulent, and hemorrhagic. The most suggestive for
cancer is the unilateral spontaneous hemorrhagic discharge, which appears
especially in case of intraductal papilloma.
Then, the patient rises up her arms above the head. By this maneuver, other
aspects can stand out that do not occur in initial position. This can produce changes in
the shape of the breast, nipple retraction or other modifications become more visible.
The patient bent forward with hands on hips. In this position breasts are hanging
and pathological changes can be observed in the breast bearing a malignant process. The
breast hangs less if the tumor is fixed to the pectoral muscles or chest wall.
Breast palpation. There are some rules to be considered during palpation:
The optimal period for breast palpation is between the fifth and the
seventh day after the onset of menstruation
Always start with the breast considered normal
Palpation is performed with the palmar surface of outstretched fingers 2-5
Palpation must be gentle
Palpation must explore the entire breast area
Both axillae and cervical lymph nodes should be palpated
There are four stages of palpation:
1. Gross palpation described by Velpeau. The clinician is trying to detect
any abnormal changes in the breast thickness. It is only an indicatively
palpation.
2. Palpation of finesse follows a certain scheme (circular - concentric, spiral,
or radial) not to omit any breast areas. No matter which method is chosen,
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Breast Pathology
the breast tissue should be compressed against the chest wall and all
quadrants should be explored.
3. Palpation of the tumor to determine the following features:
Number of tumors
Precise location (most tumors are located in the SE quadrant)
Dimensions of the tumor - generally, breast tumors found
incidentally on palpation are of 2-3 cm in diameter. If the breast is
not too voluminous, tumors can be detected at a diameter of 1 cm.
Form of the tumor can be spherical or irregular
Consistency - generally, breast cancer is of hard consistency
Sensitivity - malignant tumor is not painful on palpation, at least in
the early stages and if there is no inflammatory process associated
Tumor surface is irregular in cancer but smooth in fibroadenoma
Tumor delimitation - cancer is poorly demarcated from the
surrounding tissues
Tumor mobility - breast cancer in first stages is mobile, without
having the mobility of a breast fibroadenoma. As it develops, it
becomes more and more fixed because of tissue invasion.
4. Palpation of the areola and the nipple. The nipple is gently grasped
between the index finger and thumb and compressed. In case of a breast
cancer or an intracanalicular papilloma, blood may leak through the
nipple. In case of an intracanalicular papilloma, beneath the nipple a
tumor of about 1 to 1.5 cm can be felt. Nipple retraction is caused by
neoplastic infiltration and can not be reduced manually.
Assessing the tumoral penetration. Adherence to the skin can be appreciated by
two maneuvers:
Ianisevsky sign - wrinkling of the skin above the tumor is impossible
because of tumor infiltration and edema
Dupuytrain sign - to lateral displacement maneuver of the tumor, behind
it, the skin develops a depression.
Penetration in the pectoral muscles can be explored by the Tillaux maneuver. The
doctor opposes to the adduction movement of the patients arm (the pectorals muscles
will contract) and with the other hand palpating the breast he will notice that the tumor
becomes fixed as the pectorals muscles contract.
Palpation of lymph nodes. The following lymph nodes stations should be
examined in every case:
A: Cervical nodes on the neck
B: Supraclavicular nodes just above the collarbone
C: Infraclavicular nodes just behind the collarbone
D: Axillary nodes in the armpit
4. Malformations
Classification
Malformations can be classified as congenital and acquired. Another
classification considers the abnormalities of number, volume and shape of the breast.
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Breast Pathology
A. Abnormalities of number
Amastia - Represents the congenital absence of one or both breasts. It is sometimes
associated with the absence of sternal portion of the large pectoralis major (Poland
Syndrome). It may be associated with other malformations such as the absence of
internal genital organs, ribs 3-4 or the upper limb. It is more frequent in men. Amastia is
considered complete if both the areola and the nipple are absent.
Athelia - is represented by the absence of the nipple associated or not with the absence
of the areola. It may occur in a normal located breast but are more frequent in
supernumerary breast.
Amazia - refers to a condition where mammary gland is absent, either congenital or
iatrogenic acquired. The nipple and areola are present.
Polymastia. It is a frequent (1-5%) congenital malformation where more than two
breasts are present. It can be complete when all the anatomical structures of the breast
are present or incomplete when just the mammary gland is present. The favorite
locations are along the milk crests of Schultze. The most frequent location is the axillary
region. There can be present up to 8 supernumerary breasts. It may have a familial
character, being transmitted autosomal dominant from man to man in the same family,
but also can be sporadic. Supernumerary breasts may be affected by the same
pathologies as the normal breasts.
Polythelia - is the condition represented by multiple nipples associated or not with
areola. Frequency: 2%. Supernumerary nipples usually appear symmetrically along the
milk crests, but can occur anywhere. Nipples can be rudimentary or functional.
Sometimes the condition is associated with congenital urinary malformations (may
serve as cutaneous paraneoplastic markers for urogenital malignancies). Polythelia does
not represent a particular clinical importance unless morbid processes affect nipples.
B. Abnormalities of volume
Anisomastia - is caused by the uneven development of breasts and their asymmetrical
location. If the aesthetic defect is important, it can be corrected by various operations of
mammoplasty.
Atrophy of the breast (micromastia) - it is characterized by an insufficient uni- or
bilateral development of the breast. Is may be caused by hormonal insufficiency or
general illnesses such as: tuberculosis, cirrhosis, syphilis, or other factors. Treatment
consists in treating the endocrine deficiency and silicone implant may solve de defect.
Breast hypertrophy (macromastia) - is the consequence of hypertrophy based on
proliferation of breast parenchyma (acini, ducts) and the stroma edematous infiltration.
An obvious cause is unknown, hormonal factors and race being incriminated (more
common in black women). Another cause is the excessive development of the
mammary gland during pregnancy with incomplete returning to the normal volume.
Breasts gradually increase reaching impressive dimensions and weight (7-8 kg),
most often in addition to a normal silhouette. Increased breast weight will lead to
deformation by stretching and ptosis. Breasts become painful and exerted traction on the
chest. Skin redness with superficial venous network is often visible and frequently
intertrigo appears in the submammary groove.
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Breast Pathology
Treatment in less severe forms is represented only by support with suitable bras
and anti-estrogen therapy. In advanced forms, the surgical treatment is recommended
represented by mammoplasty reduction.
Gynecomastia - represents the development in excess of the breasts in men. It appears
more frequently in teenagers. Physiological gynecomastia occurs in newborn,
prepubertal and in senescence due to an excess of estrogen. The cause is the excess of
estrogen or androgen hormone deficiency. Increased estrogen levels may be caused by
either to hyperproduction (testicular or adrenal tumors) or iatrogenic (administration of
estrogens), or liver failure (cirrhosis) to metabolize the estrogens.
Testicular endocrine insufficiency may have multiple causes: genetic (Klinefelter
syndrome), hereditary (disturbances in hormones synthesis), congenital (anorchidism,
cryptorchidism), acquired (trauma, orchitis, hydrocele, varicocele, tumors).
Initial treatment is conservative trying to resolve deficiencies by administration of
testosterone hormone or tamoxifen to block the estrogen receptors. The subcutaneous
mastectomy is indicated in rebel cases.
C. Abnormalities of shape
Inverted nipple - is a relatively common abnormality and occurs mainly during
puberty. It has an incidence of 2% in women. It is bilateral in 25% of cases.
Nipple retraction may have different degrees: flattening, umbilication and
invagination. Han and Hong (in 1999) classified nipple retraction into 3 grades as
follows:
Grade 1 - retracted nipple returns easily in the normal position and this
position is maintained without the need for traction. Slight compression or
soft pinching of the skin around areola causes the nipple to return to
normal position.
Grade 2 - nipple can be brought to its normal position only by traction and
tends to retract after traction.
Grade 3 - the nipple is strongly retracted, and it is difficult to reverse back
into normal position even by forced traction.
Breastfeeding is not contraindicated in inverted nipple, but it can pose problems
that can be overcome by educating mothers on special techniques or by using of special
suction devices. Inversion may promote nipple infection.
Surgical correction is possible, but is not indicated at young ages because it may
harm galactophorous ducts.
Appeared in mature woman, nipple retraction is an alarming sign that could
mean:
most often, a neoplastic process, especially if the retraction is
circumferential
an inflammatory process underlying the nipple
a duct ectasia associated with periductal fibrosis
Breast ptosis
Because the only supports of breasts are the skin and the suspensory ligaments of
Cooper, they tend to descend over time due to their own weight. Ptosis is mainly caused
by the weakening of supportive fibrous breast elements (Coopers ligaments) as a result
of deficiencies in the structure of collagen and elastic tissue, or idiopathic (frequently is
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Breast Pathology
associated with varicose veins, hemorrhoids, hernias, flat feet, stretch marks, etc.) or
due to ovarian hormones and thyroid disorders, either due to aging processes. Another
cause is breast hypertrophy.
It occurs mainly in postmenopausal multiparous with repeated lactations. It has 4
phases of evolution: mild, marked (the nipple in normal position, breast lower pole
down), complete (nipple is also lowered) and bulky prolapse (ptosis is associated with
marked hypertrophy).
Treatment is represented only by surgery with aesthetic visa in most cases. The
aim is to restore the breast with the nipple in the normal position and to restore its
normal shape and volume. The operation can involve only the skin in mild forms, but in
forms with hypertrophy, a glandular reduction is also necessary.
5. Inflammatory Affections of the Breast
Acute mastitis
Mastitis is an inflammatory disease of the breast caused by infection, usually
having the entrance gate the milk pores of the nipple or the skin pores. The infection can
affect the mammary gland but also other extraglandular anatomical structures (skin,
subcutaneous tissue, and muscle fascia - paramastitis). Paramastitis may involve the
premammary tissues (premammary mastitis) or the retrommamary tissues
(retromammary mastitis or inframastitis). When the infectious inflammatory process
extends to the whole breast, we talk about panmastitis.
Most commonly incriminated etiologic agent is the Staphylococcus aureus and
Streptococcus. There may be fungal infections also.
Mastitis can be acute (in most cases) or chronic. It can be also specific (infection
with specific germs such as TB, syphilis) or nonspecific (common germs). Mastitis may
be secondary to an infected hematoma or may be a metastatic abscess during a general
infectious disease.
Acute mastitis may occur at any age but usually it appears during lactation
(puerperal mastitis). Puerperal mastitis is a nonspecific acute inflammation of the
mammary gland that occurs in the period of pregnancy and more frequently during
lactation. The most often involved microorganism is S. aureus, which enters the breast
through the nipples pores or through skin erosions, cracks or sores on the nipples. The
favoring factor is the stasis of the milk in the breast. Infection plays only a minor role in
the pathogenesis of both puerperal and nonpuerperal mastitis in humans and many cases
of mastitis are completely aseptic under normal hygienic conditions.
Prophylactic measures are very important: complete emptying of the breast and
rigorous local hygiene.
There are two phases of evolution:
1. Congestive phase (acute galactophoritis)
2. Phase of collection (breast abscesses)
Clinical picture
In the congestive phase, the patient experiences intense pain in the breast that
becomes swollen. Because of the pain, the patient does not empty the breast milk
leading to stasis and favors the infection. Celsian signs appear. The breast is increased
in volume, firm, very painful with local temperature increased and diffuse skin redness.
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Breast Pathology
The nipple can be modified and with purulent discharge. The patient is feverish and
inflammatory painful axillary adenopathy may be present.
In the collection phase, the inflammatory processes usually focus on a particular
region, where the touch may feel fluctuance. In untreated forms, infection may extend to
the entire breast (panmastitis) with spontaneously fistulization to the skin.
Breast abscesses are localized breast suppurations probably related to obstruction
of lactiferous ducts. They can be located:
Subcutaneous
Retroareolar - the most frequent
Interlobular (periductal inflammation),
Retromammary
Central (simple or multiple)
Diagnosis is generally simple based on history (pregnancy or breast-feeding),
symptoms and signs (pain, redness, local swelling, and hyperthermia). Drained fluid
from the nipple after compression leaves a yellow stain (Budin sign). Axillary
inflammatory adenopathy is also present. Ultrasound investigation of the breast
highlights the presence of the abscess and may guide surgery.
Acute mastitis should be differentiated from other diseases with similar
symptoms such as:
Paramastitis
Breast engorgement (due to expansion and pressure exerted by the
synthesis and storage of breast milk)
Breast sarcoma
Inflammatory breast cancer
Treatment
The prophylaxis is represented by the compliance with local hygiene measures
and complete emptying of breast milk at each feeding.
Curative treatment differs depending on evolution stage of the mastitis. In
congestive phase, broad-spectrum antibiotics are used with favorable evolution in 96%
of cases. Additional measures are represented by:
Evacuation of breast milk by milking or vacuum aspiration
Interruption of breast feeding from that breast - even weaning
Local cold compresses
Immobilization and breast suspension plus compression dressing
In collection phase (abscess formation), surgery is the main treatment associated with
antibiotherapy. General anesthesia is usually preferred. Incision of the abscess is
followed by pus evacuation, debridement, drainage and dressing. Pus samples are sent
for antibiogram. In superficial abscess, the skin incision may be arcuate, parallel with
the Langer lines of the skin, for a better aesthetic effect, but in profound abscesses radial
incisions are recommended to avoid sectioning the milk ducts.
Chronic breast abscess is the consequence of an unresolved acute mastitis.
Clinical picture is represented by:
A hard lump in the breast, which infiltrates the surrounding tissues
The lump is painful to touch and on compression, sometimes associated
with purulent nipple discharge
Peu d'orange and retraction of the nipple may be present
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Breast Pathology
Old scars may be present on the skin (previous surgery)
Axillary lymph nodes are enlarged
After incision, thick sterile pus is observed surrounded by fibrous tissue
Because of its local features, the chronic abscess is very difficult to differentiate
from a breast cancer. A thorough history of the patient could reveal an acute mastitis
treated years ago. In addition, the skin scar present on the breast may help to make the
difference. Histological examination of the infiltrated tissue will rule out the
malignancy.
The treatment consists incision or excision with histopathological examination.
6. Benign Tumors of the Breast
Breast fibroadenoma
Breasts fibroadenoma (or adenofibroma) is a benign fibroepithelial tumor of the
mammary gland (contain epithelial and stromal elements). The predominance of the
epithelial component determines the name of adenofibroma while the predominance of
stromal component determines the name of fibroadenoma.
The notion of complex fibroadenoma refers to the fibroadenomas, which have
cysts larger than 3 cm in diameter, sclerosing lesions, epithelial calcifications and
papillary apocrine changes.
The pure adenoma is very rare being an exclusive proliferation of the epithelial
elements. It has 2 forms:
Acinar form with normal or cystic acinar proliferation
Tubular form which may appear during lactation (lactation adenoma)
There are two forms of fibroadenomas:
1. J uvenile fibroadenomas, which appear in young women and teenagers
2. Myxoid fibroadenomas, in Carneys Syndrome, which is a dominant
autosomal neoplastic syndrome, which includes myxomas of the skin and
mucosa and endocrine dysfunctions
Fibroadenoma is the most frequent benign tumor of the breast. Young females
under 40 years are predominantly affected. In 10-15% of cases, they are multiple. There
is no racial predilection.
Even they are considered benign, yet they have a potential of malignant
transformation (mostly sarcomatous) in percentage of 3-4%. Patients with multiple
fibroadenomas or complex types have a higher risk (twice) of breast cancer.
Fibroadenomas represent a hyperplasic or proliferative process started from a
terminal milk duct. The cause is unknown.
About 10% of them disappear in a year and the grate majority stop their evolution
after they reach 2-3 cm in diameter. They can regress in postmenopausal period or
calcify. On the other hand, tumors can rapidly grow during pregnancy, during treatment
with substitutive feminine hormones or during immunosuppressant treatment, when
they can simulate a breast cancer.
Clinical picture
Fibroadenomas may be located anywhere in the breast. On inspection nothing can
be noticed when they are small. As they grow, a bulge under the skin can be observed.
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Breast Pathology
In giant forms (phyllodes tumors), the breast is highly modified with distended skin,
with marble like appearance, with visible vascular network.
On palpation, the fibroadenoma has some characteristic features that make it easy
to diagnose and differentiate from other tumors. There is a tumor of about 1-5 cm
diameter, of hard-elastic consistency, with smooth surface, well delimited from
surrounding tissues, painless, and the most important feature: very mobile.
In the axilla, there are no pathological enlarged lymph nodes.
Diagnosis is usually easy, based on clinical features of the tumor.
Useful investigations for diagnosis are ultrasound examination, mammography,
tru-cut biopsy and excisional biopsy.
When the diagnosis is very clear, based on clinical features, a lumpectomy may
be performed, followed by histopahological examination. When the diagnosis is not
very clear, a biopsy from the tumor (tru-cut biopsy under ultrasound guidance) is
indicated.
Clinical features of the fibroadenoma help to differentiate it from breast cancer,
which has the following features: it has a very weak delimitation from surrounding
tissues, it is not as mobile as fibroadenoma or is quite fixed in advanced stages, its
surface is irregular and frequently is associated with enlarged axillary lymph nodes.
Other affections for differential diagnosis are:
Breast cysts - elastic consistency, also mobile but on ultrasound
examination, the content is fluid in contrast with fibroadenoma, which is
solid. At fine needle aspiration, fluid can be obtained.
Sclerocystic mastopathy - the condition gives the feeling of lead shots"
under the skin on palpation; ultrasound shows multiple small cystic
formations of various sizes; it is usually symmetrical, often painful, and
sometimes with greenish nipple discharge.
Breast lipogranulomas - are represented by poorly delimitated mass with
reduced mobility due to chronic inflammatory process, possibly associated
with axillary inflammatory adenopathy.
Lipomas - have a softer consistency and are more superficial that
fibroadenomas.
Other tumors of the breast
Treatment
Simple fibroadenomas are tumors well encapsulated which can be easily
enucleated. The operation of election is the lumpectomy with a margin of security of 1
cm around the tumor, and in cases of multiple or diffuse fibroadenomas a
quadrantectomy can be performed. In complex cases, a skin-sparing mastectomy can be
performed.
The surface section of the fibroadenoma has a pearly white color, and is bulging
under the effect of elastic fibers, while in malignant tumor the section area is flat
sometimes with yellow spots and hard calcified areas.
Prognosis is favorable but should be considered the fact that the risk of breast
cancer is twice as higher in women with history of operated fibroadenoma and the risk
of sarcomatous transformation is about 3%.
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Breast Pathology
Fibrocystic mastopathy - Reclus disease
The condition represents a proliferative lesion that occurs in women around age
30, but it can be seen at any age.
Endocrine factors are involved such as: hyperfoliculinemia, hyperthyroidism,
genital or thyroid dysfunctions.
Gross appearance is that of multiple cysts of various sizes, filled with clear or
cloudy yellow-brown liquid, surrounded by sclerous tissue with the predominance of
one of the two components. Microscopic appearance is that of microcysts surrounded by
fibrotic walls, with cylindrical secretory epithelium with mitochondria and multiple
granules, and hypo or atrophic breast tissue.
Clinical picture. The condition manifests especially by local pain or embarrassment,
spontaneous or provoked, especially during menstruation. Palpation reveals tumors
(sizes from few millimeters to 2-3 cm) disseminated or grouped, usually symmetrical,
bilateral, mobile, firm, and slightly painful. The feeling is that of "led bullets". Nipples
discharge is frequent. Axillary adenopathy usually is absent.
Only in 5% of cases, changes can be considered at risk of developing cancer.
Treatment
In early or localized forms, in women under 35 years, conservative treatment is
considered represented by hormone therapy, painkillers, anti-inflammatory drugs and
local applications (Mastoprofen). After pregnancy and lactation, the disease may
regress.
Surgery is applied in rapidly growing forms, or in case of ineffective medication
treatment. Usually quadrantectomy is performed but in diffuse forms, that include the
entire gland, even subcutaneous mastectomy is recommended.
Breast cysts
Cysts are the most common "tumors" of the breast. Cysts are rare in women over
50 years and generally do not have any relationship with breast cancer.
On palpation, cysts are mobile with smooth surface and of elastic consistency.
They may be or not painful.
Cysts are related to papilloma tumor type. The histological features are of
apocrine metaplasia (the inner lining layer of large cysts is composed of apocrine cells).
Intraoperative they look dark ("blue dome cysts").
Breast cysts can be treated by simple evacuation through fine needle aspiration or
excision. Needle aspiration in most cases is guided by ultrasound. When extracted fluid
does not contain blood, cytological examination is nod needed, because there is no
suspicion of cancer, but when blood is present, smears are examined microscopically.
In most cases, cyst evacuation by aspiration is the definitive treatment method.
Galactocele
Galactocele is a cystic tumor that contains milky substance, which appears
usually during breast feeding period. Once lactation has ended, the cyst will disappear
on its own without intervention. It is usually located beneath areola, and due to abrupt
suppression of lactation.
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Breast Pathology
Intracanalicular papilloma
Intracanalicular papillomas are cell proliferation in the mammary ducts.
The gross appearance is that of intraductal vegetations (like polyps) of a few mm,
red multi ramified epithelium developed in the ducts, located in the center of the gland
and retroareolar region. Intraductal papillomas are classified into central and peripheral
types. The ducts are enlarged but with thin walls and contain a brownish fluid.
The papilloma consists of a vascular axis covered by cylindrical epithelium. At
the periphery, hyperplastic alterations are present. Focal areas of hemorrhage and
necrosis are also present. It can accommodate atypical hyperplasia and ductal
carcinoma in situ. If the epithelium is double layer columnar cell, it becomes
noninvasive papillary carcinoma.
Clinical picture
The condition is common in women aged between 30-50 years. The patients
claims serous or serosanguinous discharge for long periods of time. On palpation, a
small, round tumor under the nipple that does not adhere to the skin can be felt. Traction
of the nipple mobilizes the tumor (related to milk duct). On nipple compression, serous
or serosanguinous discharge appears. There are no enlarged axillary lymph nodes.
Multiple intraductal papillomas occur in approximately 10% of cases, tend to
occur in the younger patients and are less often associated with nipple discharge.
Inverstigations
Ultrasound examination - high-resolution ultrasound examination can reveal
the dilated duct that contains a well-defined, smooth-walled, solid, hypo-
echoic mass.
Mammography - in small papillomas is not very helpful. When imaging
findings are present, the dilated duct can be seen in the retroareolar region
containing a benign-appearing mass
Galactography - usually reveals a filling defect
Mammary ductoscopy - directly visualizes the papilloma and guides the duct
excision surgery.
Cytological examination of nipple discharge - is used to detect malignant
cells
Tratament
Deciding on the appropriate surgery is problematic due to the difficulty in
discriminating between intraductal papilloma and breast cancer.
When the lesion is located to a single duct, microdoctectomy gives satisfactory
results in younger patients with a minimal interference with the breast. In older patients
where breast-feeding is not required, major duct excision may be preferable.
When a specific duct cannot be identified then blind excision of the retroareolar
ductal system is usually performed (central quadrantectomy) followed by histological
examination.
Prognosis
The incidence of malignancy (invasive or in situ) associated to papilloma varies
between 1 and 23%. A solitary papilloma is not thought to be a pre-malignant lesion and
is considered by some to be an aberration rather than a true disease process.
Multiple intraductal papillomas are more susceptible to develop carcinoma.
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Breast Pathology
7. Nipple Discharge
Nipple discharge is an event that causes discomfort and anxiety to women. In this
area significant progress have been made in recent years through the development of
diagnostic procedures.
Physiopathology
The causes that lead to discharge from the nipple are not yet fully elucidated. In
most cases, it is associated with endocrine disorders and/or certain drug treatments. It is
often associated to ductal ectasia and/or fibrocystic changes in the mammary gland.
Changes are often bilateral.
A less common noncancerous etiology is the ductal ectasia associated with
periductal inflammatory process (galactophoritis).
The most common cause is the intraductal proliferation of ductal epithelium as a
result of a hyperplastic process, micropapillar proliferation, papillomas and/or ductal
carcinomas.
The vast majority of intraductal changes that produce nipple discharge are located
in the first 1-4 cm of the lactiferous duct from the nipple.
Subclinical nipple discharge occurs more frequently in women who use birth
control pills and substitutive hormone therapy.
Epidemiology
Nipple discharge can occur in both sexes but is more common in women. The
frequency is about 3-8% of all women and there are no differences between races. The
disease can occur at any age.
Clinical picture
In most cases, nipple discharges are bilateral. The aspect of the discharge may be:
Clear (aqueous)
Serous (yellowish)
Lactescent (white)
Serosanguinous
Sanguinolent
To be considered a nipple discharge, discharges must take place outside the
period of lactation, to be spontaneous and persistent.
Suspicion of cancer increases if the discharge appears only at one breast, from a
single pore and is sanguinolent. In addition, if a tumor can be felt on palpation and if the
patient is over 50 years old the risk of cancer is higher. Breast cancer is more common
in men with nipple discharge.
After investigations, when suspected lesions are not malignant, in 73% of cases,
nipple discharge regress spontaneously within 5 years.
Investigations
Mammography - is not always relevant but it may highlight modifications
suggestive for cancer (clusters of microcalcifications)
Galactography (ductography) is performed by injecting a contrast iodine
solution through a pore followed my mammograms. After nipple
disinfection a ductogram cannula is gently insert in the incriminated pore
and slowly injected approximately 0.2 to 0.8 ml of iodine solution. Then
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Breast Pathology
cranio-caudal and latero-lateral mammograms are performed. Galactography
is not indicated when:
o Nipple discharge is bilateral
o Secretion is not spontaneous or it cannot be obtained by compression
of the nipple and so neither the pore can be observed
o Secretion occurs from many pores
Breast ultrasonography - may visualize the intracanalicular papilloma or
other breast gland modifications
Cytological examination of nipple discharge - may reveal neoplastic cells but
the rate of false negative results (17.8%) and false positive (2.6%) is quite
high.
Ductoscopy - allows direct visual access to the mammary ducts, using
fiberoptic microendoscopes inserted through the ductal opening onto the
nipple surface
Galactorrhea is the spontaneous flow of milk from the breast, unassociated with
childbirth or breast feeding and usually not associated with breast cancer and the more,
when it is bilateral. Galactorrhea may occur due to:
local stimulation of the nipple
chest wall trauma
consumption of various drugs (contraceptives fenotiazide, antihypertensive,
etc.)
hypoparathyroidism
pituitary adenomas
amenorrhea
Treatment
Surgery is indicated when the discharge is from a single pore, unilateral.
Additional argument for surgery is palpable tumors, lesions found after investigations,
and age over 40 years.
Indication of surgical treatment is supported by the suspicion of an existing
cancer.
Recommended operation is the quadrantectomy. The breast sector (quadrant)
corresponding to the incriminated duct and pore is excised followed by frozen section
histopathology examination and eventually conversion to mastectomy.
Prognosis
The vast majority of patients heal after surgery. If the etiology is the cancer,
mortality rate is the same as in case of other breast cancers. Of course, in case of early
diagnosis (occult) the prognosis is even better.
8. Breast Cancer
Unfortunately, we not know yet the cause of breast cancer and yet we cannot
prevent it, but history does not stop there.
Features of breast cancer:
It affects a woman's organ that besides the biological role of breastfeeding
has a very important aesthetic and erotic role with deep implications on
womens psyche and personality.
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Breast Pathology
It is easy to detect by palpation or minimally invasive examination at low-
costs.
Particularly it affects older women (over 50 years). It is very rare in young.
It is one of the forms of cancer, which if early diagnosed, has one of the
highest rates of post-therapy survival.
Breast cancer is one of the slowest developing tumors. Even more than 10
years could pass since the beginning of the process until a tumor of 1 cm in
diameter.
Epidemiology
Breast cancer is the most common cancer in women worldwide, comprising 16%
of all female cancers. It is thought to be a disease of the developed world because a
majority (69%) of all breast cancer deaths occurs in developing countries (WHO Global
Burden of Disease, 2004).
Incidence rates vary greatly worldwide, with age standardized rates as high as
99.4 per 100 000 in North America. Eastern Europe, South America, Southern Africa,
and western Asia have moderate incidence rates, but these are increasing. The lowest
incidence rates are found in most African countries but here breast cancer incidence
rates are also increasing.
Morbidity and mortality
Breast cancer ranks among the most common 3 diseases in the world. A new case
appears at every 30 seconds. One death occurs at every 1.5 minutes. It is the second
cause of mortality after lung cancer
Five Year Survival Rate By Age
Younger than 45 81%
Ages 45-64 85%
Ages 65 and older 86%
In Romania breast cancer mortality has increased from 15.60/000 as it was in 1978 to
23.27/ 000 in 1996. WHO estimated for Romania, after 2000, that breast cancer
mortality increased by 7%. Annual mortality is around 2,500 cases. One percent of
women get breast cancer each year, which is about 4200 new cases per year. Two thirds
of patients are first diagnosed in advanced stages of disease (stages III and IV), in most
cases a total mastectomy being the only surgical alternative.
Etiopathogenesis
Risk factors for breast cancer can be classified as:
A. Factors that can not be modified
B. Factors that can be modified (depend on our willing)
A. Factors that cannot be modified
Gender is the most important factor. It is known that in male breast cancer
appears in a very small percentage (1-5%).
Race. White women have a slightly increased tendency to develop breast
cancer compared to black women. On the other hand, the latter are usually
diagnosed in more advanced stages with lower survival rate. Hispanic
women and Asian have a lower risk.
Age. The breast cancer incidence increases with age being the highest in the
sixth decade.
Genetic factors. About 5% -10% of cases can be considered hereditary due
to genetic mutations.
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Breast Pathology

Incidence Rates by Race
Race/Ethnicity Female
All Races 127.8 per 100,000 women
White 132.5 per 100,000 women
Black 118.3 per 100,000 women
Asian/Pacific Islander 89.0 per 100,000 women
American Indian/Alaska Native 69.8 per 100,000 women
Hispanic 89.3 per 100,000 women
Source: National Cancer Institute, SEER Cancer Statistics Review, 2007. Statistics

Probability of Developing Breast Cancer Within the Next 10 years
By age 20 1 out of 1,760
By age 30 1 out of 229
Lifetime 1 out of 8
Source: Among those cancer free at age interval. Based on cases diagnosed 2000-2002. "1 in" are
approximates. Source: American Cancer Society Breast Cancer Facts & Figures, 2008-2009.
o BRCA1 (breast cancer 1) and BRCA2 (breast cancer 2) mutations. BRCA1 is
located on the long arm of chromosome 17 (17q), and BRCA2 on chromosome
13q. Women who have inherited mutations by deletion in BRCA1 and BRCA2
have an increased risk of developing breast cancer by 56-85% rate and also
ovarian. BRCA1 is found in 3% of breast cancer in general and 70% in women
with hereditary of breast cancer. BRCA2 mutation is identified in 10-20% of
families with breast and ovarian cancer risk and only in 2.7% of women with
early breast cancer. The risk of breast cancer for women carrying BRCA2 is 25-
30%.
o HER2 (or HER2/neu) (human epidermal growth factor receptor 2) is a surface
gene that plays a key role in regulating cell growth. When HER2 is altered, more
HER2 receptors will be produced which leads to increased cell multiplication.
HER2 is found in 25-30% of women with breast cancer. It can be detected in
tissues sample collected by biopsy or surgery.
Personal history of breast cancer. A woman who was treated for breast
cancer has a 3-4 times higher risk of developing contralateral breast cancer
and on a remnant mammary gland after surgery, which is different from
tumor recurrence.
Radiotherapy in breast area in history. If women, especially in childhood,
have received chest radiation therapy in other diseases such as Hogkin, or
other types of cancers, have an increased risk (after some 12 times greater)
of developing breast cancer
Personal history of epithelial hyperplasia. In these cases, the risk increase
depending on the type of hyperplasia as follows:
Typical epithelial hyperplasia - the risk is 1.5-2 times higher
Atypical epithelial hyperplasia - the risk is 4-5 times higher
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Breast Pathology
Atypical ductal hyperplasia - risk increases 10 times over the next
10 years from diagnosis
Nonproliferative disease of the breast (adenosis, cysts, ductal ectasia,
fibroadenoma, fibrosis, mastitis, apocrine metaplasia and moderate
squamous hyperplasia) are not associated with risk of breast cancer.
Early menarche (under 12) and late menopause (over 55 years) are
considered factors that may increase cancer risk.
Anthropometric factors. Anthropometric studies on patients with breast
cancer revealed an interesting fact, namely that the rapid growth in
childhood and the adult height higher are associated with an increased risk of
breast cancer.
B. Factors that can be modified and are related to way of life.
It was found that the habits practiced in adolescence might influence the risk of
breast cancer later in life.
Substitutive hormonal therapy. It is a proven fact that using substitutive
hormones (estrogen, progesterone) after menopause increases the risk of
breast cancer. Doctors, to prevent undesirable effects of postmenopausal
hormone decline usually prescribe these hormones. Estrogens are indicated
for prevention of osteoporosis, but they can cause uterine cancer also.
Oral contraceptives. Use of oral contraceptives has a low risk in determining
breast cancer, and this usually occurs after a usage of over 10 years.
However, the risk disappears after discontinuation.
Pregnancy. Women who gave birth the first time after the age of 30, just as
those who have never given birth, have a higher risk of developing breast
cancer.
Breastfeeding. Some studies have suggested that breastfeeding, especially
continued for 1.5-2 years would had a protective role against breast cancer
but there is no unanimity of opinion in this regard.
Obesity is associated with an increased risk of breast cancer in
postmenopausal women. Fat tissue has the ability to turn other hormones
into estrogen. The risk of breast cancer is higher due to higher amount of
estrogens.
All these factors listed above, even without the same risk weight, have a common
element, namely the endogenous or exogenous hormones.
Diet and vitamins. High-fat diet, unlike in other types of cancer, in case of
breast cancer is not associated with a higher risk of carcinogenesis.
Vegetables and fruits also do not seem to influence the risk of breast cancer.
Microlelements (trace elements) and vitamins may have a role but there is no
clear data in this regard.
Alcohol. Women who consume alcohol have a higher risk of breast cancer
but this risk is relatively small for small amounts of alcohol. This relative
risk increased with 7% for every 10 grams of alcohol consumed.
Smoking - while smoking does not appear to induce breast cancer, women
who smoke have a mortality rate higher by 25%.
Ionizing radiation increases the risk of breast cancer especially if used at a
young age. Radiological chest exploration should be avoided wherever
possible at a young age. There is an increased incidence of breast cancer
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Breast Pathology
among flight attendants (stewardesses), incriminated factor being the more
intense cosmic radiation at higher altitudes.
Chemicals. Although numerous experimental studies in animals have found
mammary carcinogenic substances, none with this effect was found in
humans.
Environmental and occupational factors. It seems that the polluted
environment of large urban agglomerations and the stress are negative risk
factors.
Other factors. Breast implants. - there is no clear evidence that implants
would lead to increased incidence of breast cancer, but implants make
mammography difficult.
Classification of risk factors according to their importance
High risk:
o The existence of genetic markers of susceptibility (BRCA 1, BRCA 2)
o Family history of breast cancer unilateral or bilateral, especially in first
degree relatives
o Personal history of breast cancer
o History of hyperplastic mastopathy
o Hormone replacement therapy (to treat postmenopausal symptoms),
o History of ovarian or endometrial cancer
Moderate risk:
o Age
o Family history of breast cancer occurred before menopause
o Radiation of the chest
o Small and repeated breast trauma
Low risk:
o History of breast cancer occurred after menopause
o Nuliparity
o First birth at an older age than 30
o Early menarche before age 12
o Late menopause, occurring over the age of 55
o Obesity occurred after menopause (increases risk by 80%)
o Daily consumption of alcohol
o Diet rich in fat and carbohydrates
o Oral contraceptive used more than 10 years
Recommendations for primary prevention of breast cancer:
Avoid exposure to radiation (avoid unnecessary radiological examinations,
avoid prolonged exposure to ultraviolet radiation)
Physical activity has beneficial effects through several mechanisms
Limitation or exclusion of alcohol consumption
Maintaining an ideal weight through diet and exercise especially after
menopause
Hypocaloric, low fat diet rich in vegetables, fruits, trace elements and
vitamins, especially in adolescence
Avoid as much as possible hormone replacement therapy and birth control
pills
Giving birth and breastfeeding at a young age would be beneficial
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Breast Pathology
American Cancer Society recommends the following steps to detect early
breast tumors:
A. Women aged over 20 years - breasts self-examination should be performed
every month
B. Women between 20 and 39 years - should be clinically examined at least
once every three years
C. Women aged over 40 years - should be clinically examined at least once a
year, in addition self-examination monthly and annual one mammography
exam
Evolution and symptoms
An adult body normally produces as many new cells as are needed to replace
those lost, maintaining constant the cell mass. Tumor cells multiplication instead does
not keep this balance. They are growing at a rate faster than normal cells causing tumor
masses.
Tumor cells, unlike normal ones, are no longer so strictly linked together to form
tissues. They have the ability to spread in various ways in any region of the body. The
immune system cannot cope with this invasion and metastases appear.
The underground life of breast cancer is very long. Tumor growth is measured in
doubling time. A doubling time is the length of time required for tumor cell mass to
double in volume. It takes about 23 doubling times starting from a tumor cell to reach a
tumor mass large enough to be seen at mammography and approximately 30 times (one
billion cells) to be palpable. Doubling period can be short, sometimes for only 10 days,
or longer, for years. An average period is of 4 months. For example, if a first tumor cell
occurred in the age of 40 and if we believe it is a fast growing tumor with a doubling
period of two months, four years must pass until the tumor can be detected on
mammography, so at the age of 44 years. As the tumor can be detected by palpation
have to pass about 5 years, so when the patient will have 49 years, that is after 9 years
from first appearance of tumor cells.
Clinical picture
There is no unique clinical picture of breast cancer since there are many clinical
forms. Symptoms are closely related to stage of the tumor.
Initially, breast cancer does not have any symptoms. Pain (continuous or
intermittent, localized or irradiated) occurs rarely, in 10% of patients. The tumor is
usually detected by the patient itself during toilet.
Local evolution of breast cancer is by direct extension to the surrounding tissues
along the connective tissue septa, along ducts and invasion of lymphatic and blood
vessels.
Local extension
Cancer extends to the skin by invasion of Duret crests and Cooper ligaments. In
this stage the skin becomes fixed, infiltrated and cannot be folded (the Ianisevskis
sign). By side displacement maneuver of the tumor, a depression appears behind it (the
Dupuytrains sign).
In a later stage, the tumor blocks the local lymphatic circulation producing a local
lymph edema. The skin pores become more evident and the aspect of orange peel (peau
dorange) appears.
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Breast Pathology
Then, the tumor directly invades the skin producing ulceration, which has
irregular and endured borders, purple color and necrotic tissue on the bottom. Bleeding
and infectious complications are common in this stage.
Dermal tumor nodules may appear at some distance from the tumor due to local
lymphatic spread.
If the tumor is located in the central quadrant, it may produce nipple retraction
(pathognomonic sign) through invasion of milk ducts and connective tissue septa.
Retraction is fixed, irreducible, unilateral and acquired.
Ducts invasion also causes nipple discharge (serous, sanguinolent or lactescent)
either spontaneously or on compression.
Extension may progress in depth to the pectoral muscles and chest wall. Invasion
of these structures can be demonstrated by the Tillaux maneuver (during pectoral
muscle contraction in forced adduction the tumor becomes fixed to the chest wall).
Regional extension takes place along the main and secondary lymphatic routes.
Along the lymphatic route, there is a lymph node, which is most likely to retain first the
cancer cells: the sentinel lymph node. This lymph node can be found and removed for
examination by methods using radiotracers and dye tracers. Histopathological findings
from this are very important in choosing the type of surgery, which will be applied (with
or without axillary lymphadenectomy).
Axillary lymph nodes affected by metastases gradually increase in volume so they
begin to compress and invade the axillary vessels and nerves causing pain in the upper
limb.
Lymphatic invasion extends to the subclavian and supraclavicular lymph nodes
with the consequence of lymphedema of the upper limb but also open secondary
lymphatic channels to contralateral armpit.
Lymph node invasion is the most important prognostic factor, efforts in this area
currently being targeted to detect breast cancer before this stage.
Tumors located in the internal quadrants spread most commonly to the internal
mammary lymph nodes, which cannot be detected by clinical examination.
Remote extension is achieved by both lymphatic and venous routes. Tumor cells
invade the microcirculation and are transported by venous bloodstream towards superior
cava vein. From here, they follow the natural path to the right heart and then to the
lungs, which represent the first major systemic, filter. Liver represents the second filter.
Most tumor cells remain stuck in the first filter (the lung) and start to develop
lung metastases. Lung metastases are manifested initially by decreasing exercise
capacity, dyspnea on effort, and then even on rest. In advanced forms, irritating cough
and dyspnea is increasing more and more, leading to death, by both reducing the lungs
hematosis surface to and paraneoplastic pleurisy.
Tumor cells escaped from the lung filter enter the pulmonary artery bloodstream
from where the path is open to any region of the body. Other most common sites of
metastases are the liver and bone.
Liver metastases produce symptoms like weight loss, loss of appetite, digestive
problems and eventually jaundice. The dull pain under right costal margin is produced
by Glissons capsule distension. Liver metastases can be detected by ultrasound
examination of the liver or by CT scan.
Bone metastases are the most common sites of metastasis in breast cancer. They
are present in approximately 25% of cases. These metastases are manifested mainly by
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Breast Pathology
early pain. The most frequent complication of bone metastases is pathological fractures.
Bone metastasis can be observed on bone radiograms, which reveal bone circumscribed
demineralization, and/or on radioscintigraphy, or CT scan and PET scan.
Other regions of metastasis are the brain, spine, spinal cord, but never the
kidneys.
In conclusion, although in early stages the breast cancer does not cause pain,
other symptoms (such as those listed below) should be warning signs for women and to
determine them to contact a doctor.
The appearance of a breast tumor and/or axillary enlarged lymph nodes
Changes in breast shape and size, and nipple symmetry
Changes of skin surface (orange peel, tumor nodules, ulceration, increased
vascular drawing)
Nipple discharge, especially sanguinolent
Recent nipple retraction
Workup in breast cancer
Before treating a breast cancer, doctors are facing two important problems: the
first is to determine if the breast lesion is really a cancer and the second is to determine
the exact location and extension of the tumoral process. Several important investigation
methods are very helpful.
I. Imaging examinations
Mammography and breast ultrasound are the most frequent investigations used in
this field.
A. Mammography. It can find breast tumors in an early stage, about 2 years before
they can be detected by palpation but it does not prevent breast cancer!
Mammography uses X-rays with very low levels (0.1 Gy). Each breast is
compressed horizontally and then obliquely and x-rays are taken in each position.
There are two types of mammographies:
o Screening mammography, which is performed in women with no
complaints in the breast area, and
o Diagnostic mammography, for women who has some complaints or
modification in breast area
An improvement in this field is the digital mammography which stores and analyzes
the information on a computer. Detectable tumor size on mammography is an
average of one cm diameter. In the table below are given for comparison the
approximate sizes of mammary tumors detected by mammography and by palpation.
Mammographic features of a malignant tumor are:
Irregular whitish mass with marginal spicule
Clusters of microcalcification
Calcification less than 0.5 mm diameter
Deformation of local architecture
Density asymmetry
Skin retraction
Peritumoral edema
Mammographic features of a benign tumor are:
Circumscribed mass
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Breast Pathology
Fat-containing lesion
Macrocalcifications
Round, uniform density, large, coarse
Widely scattered
Interpretation of mammograms. Standardization system BI-RADSTM (Breast Imaging
Reporting and Database System) to characterize the mammographic images:
Category 0 - image inconclusive, it is necessary to carry out other imaging
Category 1 - negative
Category 2 - benign character changes
Category 3 - probably benign but require tracking changes
Category 4 - suspicious for cancer changes - requires biopsy
Category 5 - highly suggestive of cancer changes
B. Breast ultrasonography
Indications:
To investigate tumors detected by mammography or palpation and for biopsy
guidance.
To differentiate the cystic from solid tumors.
To explore the breast tumors that can not be evaluated by mammography (or are
not visible, either because of location, either due to dense breast tissue in young
women)
To explore the axillary lymph nodes.
To explore the breast tissue in mastitis abscess formation
To guide the biobpsy
In pregnant women because there is no radiation exposure.
Limits of the method:
It takes longer time to investigate the patient
Can not detect microcalcifications
Isnt so accurate than biopsy, there are frequently false negative and false
positive conclusions
Examiner's experience is an important related factor
Advantages of the method:
Does not use radiation
Can differentiate between a solid and a cystic structure
Offers the possibility to explore in multiple levels
It is cheap
Malignant features of the tumor:
Lesion is taller than it is wide
Decreased hyperechogenicity
Marked acoustical shadowing
Spiculation
C. Nuclear magnetic resonance imaging (MRI)
It provides valuable information about tumor extension. The main drawback is the
price far above the mammography examination.
Indications:
Preoperative staging in breast cancer for possible or multi-focal disease
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Breast Pathology
Detection of implant rupture
Patient with metastatic breast cancer
Occult breast cancer
Differentiation between scar and tumor recurrence
Screening of high-risk patients
Recommendations for Breast MRI Screening as an Adjunct to Mammography Cancer Screening
Guidelines for Breast Screening with MRI as an Adjunct to Mammography by ACS
Recommend Annual MRI Screening (Based on Evidence*)
BRCA mutation
First-degree relative of BRCA carrier, but untested
Lifetime risk 2025% or greater, as defined by BRCAPRO or other models that are largely
dependent on family history
Recommend Annual MRI Screening (Based on Expert Consensus Opinion)
Radiation to chest between age 10 and 30 years
Li-Fraumeni syndrome and first-degree relatives
Cowden and Bannayan-Riley-Ruvalcaba syndromes and first-degree relatives
Insufficient Evidence to Recommend for or Against MRI Screening
Lifetime risk 1520%, as defined by BRCAPRO or other models that are largely dependent on
family history
Lobular carcinoma in situ (LCIS) or atypical lobular hyperplasia (ALH)
Atypical ductal hyperplasia (ADH)
Heterogeneously or extremely dense breast on mammography
Women with a personal history of breast cancer, including ductal carcinoma in situ (DCIS)
Recommend Against MRI Screening (Based on Expert Consensus Opinion)
Women at <15% lifetime risk
D. CT scan
This method of investigation is not routinely used to diagnose breast tumors due
to exposure to radiation. It is however very useful in advanced stages of disease to
assess the extension of neoplastic process and penetration into the chest wall structures
or distant metastases detection.
E. CTLM (Computed Tomography Laser Mammography)
This method uses laser technology to produce three-dimensional images of the
breast. It does not create any discomfort. CTLM is a method of looking at the blood
flow to the breast and thereby should visualize tumor angiogenesis. It can perform
images through implants and dense breast tissue easily, unlike mammography.
F. Scintimammography (Sestamibi)
It is based on the fact that the radiant substance is captured at a greater extent by
tumors than normal tissue due to their increased metabolism. It is used in selected cases
such as:
For patients with dense breast tissue difficult to investigate with other imaging
methods
When a breast tumor can be felt but it cannot be detected by mammography or
ultrasound
Breast implants
When multiple, multifocal tumors are suspected
When after mastectomy tumors appear at the level of postoperative scar
To explore the axilla in detecting metastatic lymph nodes or for sentinel node
biopsy
G. P.E.T. - Positron Emission Tomography
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Breast Pathology
The principle is the same as in Sestamibi. Post-therapy is particularly useful for
detecting any remnant cancer and active areas and to detect lymph node or distant
metastases.
H. Other imaging investigations such as:
Chest radiograph is used to highlight the pulmonary metastases
Bone radiography and scintigraphy - for bone metastases
Thermography is based on the principle that the area around the cancer tissue
has a higher temperature because of rich blood supply and more intense
metabolism
Electrical impedance scanning (EIS) is based on differences of electrical
impedance between the normal tissue and the tumoral one.
II. Investigation methods of milk ducts
A. Ductography (Galactography) - is an x-ray examination that uses mammography, a
low-dose x-ray system for examining breasts, and a contrast material to obtain
pictures, called galactograms, of the milk ducts.
Indications:
Unilateral persistent sanguinolent nipple discharge
Contraindications:
Allergy to contrast substance
Difficult to achieve in the following conditions: previous operations on the
nipple and inverted nipple
B. Ductal lavage - examines the cells in wash liquid
C. Ductoscopy - it is capable of detecting smaller abnormalities than mammograms,
MRI or ultrasound tests.
III. Tumoral markers in breast cancer
Tumoral markers are substances that can be detected in small amounts in blood,
urine and various tissues. Measurement of these markers is useful in detection and
diagnosis of various cancers.
Usefulness:
Determination of cancer risk in some people
Detect cancer in the body
Reflecting the stage of the disease
Monitoring the effectiveness of cancer treatment
Early tumor recurrence detection
Prognosis estimation of the case

Marker Description
ER/PR
(estrogen/
progesteron
receptor)
Estrogen receptors bind to cancer cells stimulating their proliferation and
differentiation. Progesterone is also a mitogenic factor stimulating the
mammary epithelium.
Determination of ER and PR receptors by immunohistochemistry has become
an important standard for clinical labor as the presence of these receptors
influence therapeutic measures and prognosis of patients.
The patients with breast cancer who have both types of receptors (70% cases)
have the best remission to treatment with Tamoxifen, while those with only
one type of receptor (30%) have poor results, and those with low levels of
receptors (less than 10%) had poor results also.
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BRCA1
(breast cancer 1)
Chromozom 17q

BRCA2
(breast cancer 2)
Chromozom 13q,
Women who have inherited mutations by deletion in BRCA1 and BRCA2
have an increased risk of developing breast cancer, and also ovarian.
BRCA1 is found in 3% of breast cancer in general and 70% in women with
hereditary of breast cancer.
BRCA2 mutation is identified in 10-20% of families with breast and ovarian
cancer risk and only in 2.7% of women with early breast cancer. The risk of
breast cancer for women carrying BRCA2 is 25-30%.
HER-2/neu
(human epidermal
growth factor
receptor 2)

Gene ERBB2
localised on
chromozom 17q21.1
A surface gene plays a key role in regulating cell growth. When HER2 is
altered, more HER2 receptors will be produced which leads to increased cell
multiplication. HER2 is found in 25-30% of women with breast cancer. They
can be detected in tissues sample collected by biopsy or surgery.
In women with metastatic HER2 positive is indicated Herceptin
(Trastuzumab) - a monoclonal antibody produced by biotechnology.
CA 15-3
(Carbohydrate
Antigen 15-3)
(Cancer Antigen 15-
3) Antigen oncofetal
(from blood)
It is a marker used to monitor treatment effectiveness in advanced breast
cancer.
Rarely is increased in the early stages of the disease.
CA-125 also known
as mucin 16
Used to monitor the response to treatment and predicting prognosis after
treatment. It is especially useful for detecting the recurrence of ovarian
cancer.
CA 27-29
It is found in the blood of the vast majority of the patients with breast cancer.
It is used together with other tests to monitor the treated of breast cancer in
stage II and III
It is an independent factor for predicting tumor recurrence
IV. Invasive methods of diagnosis
Breast biopsies
The only examination that can make with certainty the diagnosis of cancer is
histopathology, which can be obtained from specimens harvested by biopsy.
Confirmation of breast cancer before surgery is useful because it influences the
magnitude of this act, if it is necessary and subsequent management as well.
Types of breast biopsies:
1) Percutaneous
a) Fine needle aspiration
b) Tru-cut biopsy
c) Vacuum assisted biopsy
d) ABBI (advanced breast biopsy instrumentation)
2) Surgical
a) Incisional
b) Excisional
PERCUTANEOUS BIOPSIES
a. Fine needle aspiration (FNA) is indicated for tumors of cystic nature and for solid
lesions in stage T3 or T4, or axillary and local recurrences. Due to possible false
negative results, this type of investigation is not very suitable for exploring tumors of
less than 1 cm. Cytological examination is required in the following situations:
Hemorrhagic fluid is extracted
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After aspiration, the tumor mass does not disappear completely
In case of a recurrent cyst
There is a suspicion for cancer on mammogram
b. Tru-cut biopsy is also a percutaneous method. The essential differences from fine
needle aspiration biopsy are:
It uses a thick needle, specially fitted with a cutting mechanism
The process of obtaining biopsy material is cutting not aspiration
The material obtained is a cylinder of tissue, enough to differentiate
between invasive and noninvasive type of cancer
Core needle biopsy is not suitable for cystic lesions
Advantages:
Sample tissue with enough cellular material to detect breast cancer
Harvested fragments can demonstrate relationship with the surrounding
tissue ad can make the difference between in situ and invasive cancer.
Disadvantages:
As a biopsy, it harvests only fragments of tissue, not the entire tumor.
Even if the fragment does not contain cancer cells is not an absolute
guarantee that the patient is not suffering from breast cancer.
The method cannot be applied to women with breast implants as the risk
for perforation the implant.
c. Vacuum assisted biopsy. The novelty of the method is that the biopsy needle is
adapted to a vacuum system. By using vacuum, breast tissue is absorbed into the
needle slot ensuring a better sampling. 3-6 specimens are extracted.
d. ABBI - Advanced Breast Biopsy Instrumentation. This type of biopsy uses a
thicker needle of 0.5 to 2 cm in diameter. The intention of this type of biopsy is to
extract as much tissue as possible, even the entire tumor if the size permits. It is
carried out only with stereotactic equipment. Rarely used in now days.
SURGICAL BIOPSIES
a. Excisional biopsy. It is the most commonly used type of biopsy. The surgeon will
remove the tumor with a safety margin of normal tissue around it.
b. Incisional biopsy. This applies when the breast tumor is larger (more than 4 cm
diameter) or diffuse, or when chemotherapy and radiotherapy are the primary
treatment. The surgeon will harvest only a portion of the tumor that is suggestive for
cancer.
Advantages of surgical biopsies:
Ensures the diagnosis in almost 100% cases being the "gold standard" in
this sense
In case of small tumors, it can be regarded as definitive surgical therapy
method (lumpectomy) if the tumor was excised with negative margins.
Staging of breast tumors - TNM classification (American J oint Committee on Cancer)
The T stages (tumor)
TX means that the tumor size cannot be assessed
T1 The tumor is no more than 2 centimeters (cm) across
T1 is further divided into 4 groups
T1mic under a microscope the cancer cells can be seen to spread less than 0.1cm into
surrounding tissue (microinvasion)
T1a the tumor is more than 0.1 cm but not more than 0.5 cm
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Breast Pathology
T1b the tumor is more than 0.5 cm but not more than 1 cm
T1c the tumor is more than 1 cm but not more than 2 cm
T2 The tumour is more than 2 centimeters, but no more than 5 centimeters across
T3 The tumour is bigger than 5 centimeters across
T4 is divided into 4 groups
T4a The tumor has spread into the chest wall
T4b The tumor has spread into the skin
T4c The tumor is fixed to both the skin and the chest wall
T4d Inflammatory carcinoma this is a cancer in which the overlying skin is red, swollen
and painful to the touch
The N stages (nodes)
NX means that the lymph nodes cannot be assessed (for example, if they were previously
removed)
N0 No cancer cells found in any nearby nodes
N1 Cancer cells are in the upper levels of lymph nodes in the armpit but the nodes are not
stuck to surrounding tissues
N2 is divided into 2 groups
N2a there are cancer cells in the lymph nodes in the armpit, which are stuck to each other
and to other structures
N2b there are cancer cells in the lymph nodes behind the breast bone (the internal
mammary nodes, which have either been seen on a scan or felt by the doctor. There is no
evidence of cancer in lymph nodes in the armpit
N3 is divided into 3 groups
N3a there are cancer cells in lymph nodes below the collarbone
N3b there are cancer cells in lymph nodes in the armpit and under the breast bone
N3c there are cancer cells in lymph nodes above the collarbone
The M stages (metastases)
M0 No sign of cancer spread
M1 Cancer has spread to another part of the body, apart from the breast and lymph nodes
under the arm
Stage grouping
Stage 0 - Tis N0 M0
Stage I - T1* N0 M0 (*T1 includes T1mic)
Stage IIA - T0 N1 M0 - T1* N1** M0 - T2 N0 M0
(*T1 includes T1mic **The prognosis of patients with pN1a disease is similar to that of patients
with pN0 disease.)
Stage IIB - T2 N1 M0 - T3 N0 M0
Stage IIIA - T0 N2 M0 - T1*N2 M0 - T2 N2 M0 - T3 N1 M0 - T3 N2 M0 ( *T1 includes
T1mic)
Stage IIIB - T4 Any N M0 - Any T N3 M0
Stage IV - Any T Any N M1
Treatment of breast cancer is a complex and multimodal one. The arsenal includes:
1. Surgical
2. Adjuvant
3. Radiotherapy
4. Chemotherapy
5. Hormonal therapy
6. Immunotherapy
7. Others
Selection of local and systemic treatment modalities and priorities of application
depends on a number of factors and prognostic predictors including:
Tumor histology
Clinical and pathological features of tumor
Lymph nodes status
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Breast Pathology
Tumor hormone receptor
HER2 marker level
Distant metastases
Existing co-morbidities
Age of patient
Menopausal status of the patient
Patient preferences
Breast cancer in men is treated in the same way as in postmenopausal women.
TREATMENT STRATEGIES BASED ON STAGE GROUPS
1) Non-invasive carcinoma (stage 0)
a) ductal carcinoma (DCIS)
b) lobular carcinoma (LCIS)
2) Operable invasive carcinomas
a) stage I
b) stage II
c) some of stage IIIA
3) Inoperable invasive cancers
a) stage IIIB
b) stage IIIC
c) some stage IIIA
d) Cancers with distant metastases or recurrent (stage IV)
1. NON-INVASIVE CARCINOMAS
The goal of treatment in carcinoma in situ is either to prevent invasion or to
diagnose invasive component as long as it is still located at the breast.
A. Lobular carcinoma (LCIS)
Treatment is simple surveillance because the risk of invasive cancer in time is
very low (about 21% to 15). Bilateral simple mastectomy with or without reconstruction
is another alternative. Tamoxifen therapy for 5 years significantly reduces (56%) the
risk of invasive cancer.
B. Ductal carcinoma in situ (DCIS)
In patients with extended DCIS, simple mastectomy is indicated without axillary
lymphadenectomy. In patients with limited DCIS, conservative surgery is enough if
margin resections are tumor free (lumpectomy, quadrantectomy). Radiotherapy is
indicated after excision in all tumors larger than 5 cm. Tamoxifen is indicated to reduce
the risk of a primary tumors in the contralateral breast and local recurrence in those with
conservative surgery
2. INVASIVE BREAST CANCER
STAGES I, IIA AND IIB
Surgery is represented by total mastectomy with axillary lymphadenectomy or
conservative surgery with axillary lymphadenectomy. Contraindications for breast-
conserving therapy requiring radiation therapy (RT) include:
Absolute:
Prior RT to the breast or chest wall
RT during pregnancy
Diffuse suspicious or malignant appearing microcalcifications
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Breast Pathology
MuIticentric disease
Relative:
Multifocal disease requiring two or more separate surgical incisions
Active connective tissue disease involving the skin (especially
scleroderma and lupus)
Tumors >5 cm (category 2B)
Axillary lymphadenectomy will remove the level I and II of lymph nodes. Sentinel node
biopsy may be considered in the following cases:
Nonpalpable axillary lymph nodes
Tumor less than 5 cm diameter
Without having had breast surgery on the same breast
Without preoperative treatment with chemotherapy, radiotherapy or
hormone therapy
If the sentinel node can not be identified or on frozen sections metastases are
found, axillary lympadenectomy should be performed.
3. INVASIVE BREAST CANCER STAGE III
A. Locally advanced cancer but operable - T3N1M0
Surgical treatment consists of total mastectomy with axillary lymphadenectomy
reconstruction. Treatment is the same as in stage II.
B. Locally advanced cancer inoperable - stages IIIB (T4, any N, M0) and IIIC (any
T, N3, M0)
Treatment begins with preoperative chemotherapy followed by mastectomy with
lymphadenectomy if remission is obtained. Breast irradiation should be started as soon
as possible after surgery and not later than 12 weeks after, except for patients in whom
radiation therapy is preceded by chemotherapy. However, the optimal interval between
BCS and the start of irradiation has not been defined.
4. ADVANCED STAGE WITH METASTASES OR LOCAL RECURRENCE
A. Recurrences
Recurrence after conservative surgery - radical mastectomy with
lymphadenectomy chemo-hormonal therapy (to keep in mind that the patient have
already received radiation!)
Relapse occurs after total mastectomy - excision without "heroic operation"
followed by local radiotherapy (if there was no previous irradiation).
If relapse cannot be removed, the patient will benefit from local radiotherapy.
B. Metastases
Palliative treatment in this stage is trying to prolong the life. Surgery comes into
discussion in the following circumstances:
Mastectomy or excision of recurrences with the purpose of "cleaning" the
ulcerated lesions which has become infected.
Oophrectomy (ovarectomy) in premenopausal patients
Bone marrow transplantation (autologous) or stem cell transplantation
combined with high dose radio-chemotherapy.
SURGICAL TREATMENT
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Breast Pathology
1) Tumor removal (+/ - Lymphadenectomy)
a) Conservative surgery
i) Lumpectomy
ii) Quadrantectomy
iii) Extended quadrantectomy
b) Mastectomy
i) Simple mastectomy
ii) Skin sparing mastectomy
iii) Mastectomy with axillary lymphadenectomy
Maden
Patey
Halsted - limited indications (tumor infiltration of the pectoral
muscles)
iv) Heroic" operations (Ugon, Dubau, etc.) have no indication in now
days
2) Surgery to remove the lymph nodes
a) Sentinel node biopsy
b) Axillary lymphadenectomy
c) Internal mammary lymphadenectomy
3) Breast reconstruction surgery
4) Endocrine surgery (oofrectomy)
Lumpectomy is a surgical method applied for early stages of cancer, which removes
the tumor with surrounding healthy tissue. Usually is followed by six weeks of
radiotherapy. The specimen is examined by pathologist and if the tumor is too close to
the margin of resection, the surgeon must perform a re-resection at the same site.
Quadrantectomy (segmental mastectomy) means the removal of a quadrant of
mammary gland.
Partial mastectomy (or extended quadrantectomy) means removal of more than a
quadrant of the breast. Usually, after these operations external radiation therapy is given
for a period of six weeks.
Skin-Sparing Mastectomy removes the entirely mammary gland and the areola but
sparing the skin. A "keyhole"like or other types of incision are performed. This type of
operation is used when a breast reconstruction is intended (an expander in introduced
under the pectoral muscle and after a while it is replaced by silicone).
Simple or total mastectomy: removes the entire breast, but without axillary lymph
nodes and underlying muscles. The skin incision is elliptical including the areola and
nipple. It may be oblique or horizontal depending on breast volume and shape.
Modified radical mastectomy removes the mammary gland between boundaries:
sternum, clavicle, latisimus dorsi and the origin of rectus abdominis, and also removes
the axillary lymph nodes of level I and II. Level III lymph nodes are not dissected.
Madden mastectomy removes the entire breast +level I and II axillary lymph nodes.
Patey mastectomy is almost the same as in Madden procedure but the pectoraslis minor
insertion is sectioned for a better access to the axilla.
Radical mastectomy or amputation of the breast (Halsted operation) presumes
mastectomy plus a wide excision of the pectoral muscles and axillary lymph nodes. In
nowadays this type of operation is no longer performed, just in cases when muscles are
invaded by tumor.
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Breast Pathology
Sentinel Lymph Node Biopsy (SLNB)
Axillary lymph node metastases remain the most important prognostic factor in
breast cancer and also a basis that guides the adjuvant therapy.
Sentinel node is the first node on the lymphatic route where the probability of
metastasis is high and early. Only about 30% of women who require axillary node
sampling actually have metastasis to the lymph nodes. Lymphatic mapping and sentinel
lymph node biopsy can identify the patients with positive nodes, thus saving the
majority of women from an axillary dissection.
In most cases, the sentinel lymph nodes are located in the axilla but there are cases
when the sentinel lymph node is located elsewhere, or there are more than a single
sentinel lymph node. These are the reasons why the lymphatic mapping prior operation
is important. Mapping is useful also for guiding the radiation therapy.
Possible locations of sentinel lymph node are:
Axillary level I, II and III
Internal mammary chain
Supraclavicular and cervical
Intramammary
Interpectoral (Rotter)
Other locations
Contraindications of SLNB:
Contraindications related to tumor:
Tumors larger than 5 cm diameter or advanced local stage
Patients with palpable axillary lymph nodes
Patients with pure ductal carcinoma in situ
Contraindications related to the patient:
Previous breast surgery or armpit surgery
Preoperative chemo-radiotherapy
Patients with multicentric tumors in the same breast that are in different
quadrants.
Pregnancy
Allergy to technetium 99m sulphur colloid
There are two methods used for sentinel lymph node detection: one using
radioisotopes (Technetium 99) and the other, which is using a dye (metilen blue or
isosulphan blue). In many cases, for better results the two methods are combined. The
advantage of radioisotope method is that it allows the preoperative mapping guiding the
surgeon and also the radiotherapy. The dye method is cheaper.
The tracer is injected around the tumor or areola. It flows via the lymphatic
network toward the first lymph node of the lymphatic route that drains also the tumor.
This first node can be detected in two ways depending on the traces used. If technetium
99 was used a special gamma detection device and probe is necessary for detection. If
dye was used the lymph node will be detected by its blue color. Blue dye is injected
around the areola; a local massage is performed for a faster diffusion of the dye; after
10-15 minutes incision in the axilla may be performed for detection of node.
The excised node will be sent to histological examination. In the result is negative
the axillary lymphadenectomy is not necessary but if tumoral cells were found the
axillary dissection should be performed. Preventing unnecessary axillary dissection is
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Breast Pathology
important in preventing associated complications (seroma, shoulder and upper limb
pain, lymphedema, scars, etc.).
Post-mastectomy
Subcutaneous hematoma
Wound infection
Skin necrosis
Chest paresthesia
Postoperative local pain
Seroma
Lymphedema - the reported prevalence rate of lymphedema is
approximately 11%. Extensive surgery, RT, and advanced age are
recognized risk factors for arm edema
Keloid scars
Granulomas
Tumor recurrence
After axillary lymph node dissection:
Lesions or thrombosis of the axillary vein
Seroma
Impairment of shoulder movements. Symptoms include decreased range
of motion of the shoulder, a problem that may be improved with early
participation in a physical therapy program.
Damage to the brachial plexus, with chronic pain and varying degrees of
decreased grip strength occurring in up to 15% of patients and lasting for
more than a year after surgery
Chest wall pain
Post-therapy follow-up program for patients with breast cancer

Year 1 Year 2 Years 3-5 > 5 Years
Clinical examination 4 month 4 month 6 month 12 month
Chest radiography Initial If necessary If necessary If necessary
Mammography 12 month 12 month 12 month 12 month
Bone scintigraphy Initial If necessary If necessary If necessary

Inflammatory Breast Cancer (IBC) - Klotz-Volkmanns disease
Inflammatory breast cancer is particularly serious invasive form of primary breast
cancer, characterized by rapid evolution and clinical appearance of a breast
inflammatory process.
The incidence is 1-6% being more common in African-American population
(10.1%) than in Caucasian population (6.2%). It tends to be diagnosed in younger
women compared to non-IBC breast cancer. Like other types of breast cancer, it can
occur in men, but usually at an older age than in women. Some studies have shown an
association between family history of breast cancer and IBC.
The characteristic aspect of inflammation is given by the obstruction of the skin
lymphatic vessels due to lymphatic invasion by tumor cells.
Signs and symptoms. The onset is often sudden. Women have breast pain and nipple
discharge may occur. On clinical examination the breast is swollen, deformed, with skin
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Breast Pathology
erythema and edema, increased local temperature. The aspect is that of peau dorange
or "orange peel". Axillary lymph nodes are increased in volume and sensitive. In more
advanced forms, contralateral axillary lymph nodes are also affected.
All this aspects are very similar to acute mastitis and confusion is not rare. In this
event, patients are treated for a long time with antibiotics and anti-inflammatory drugs
but as they do not heal, suspicion of a cancer arises.
Diagnosis. The only investigation that could make the correct diagnosis is biopsy.
Usually the first examination is breast ultrasound to reveal some collections. This is of
no use for correct diagnosis in this case. Eventually cytology of nipple discharge could
detect cancerous cells. Inflammatory cancer is characterized as a high histological grade
invasive carcinoma, the presence of molecular markers including high aggressiveness of
S phase, aneuploidy, lack of ER receptors and a large increase in markers of p53 and
epidermal growth factor.
Treatment. The treatment is complex, aggressive, including chemotherapy,
radiotherapy and mastectomy with axillary lymphadenectomy if after chemotherapy the
response is favorable, plus hormone therapy if estrogen receptors are present.
With aggressive treatment using multimodal approach, the 5-year survival rate
improved significantly from an average of 18 months to 50% at 5 years.
PAGETS DISESE OF THE BREAST
Paget's disease has an incidence of 1-3% of all breast cancers in women. It may
occur rarely in males also. The average age of patients with breast Paget's disease is 53-
59 years, 5-10 years more than for the patients with breast cancer. Age limits in which
the disease was found is between 24 and 84 years.
The diagnosis of certainty is established only by histopathological examination
that emphasizes the unique features of Paget's cells.
Pathophysiology. Although the pathophysiology of Paget's disease has long been
controversial, most authors now agree that the origin of the disease is the neoplastic
cells of the intraductal breast cancer that invade the skin retrograde through the nipples
pores.
Signs and symptoms. An itchy rash on the nipple and areola, which then ulcerates.is
the first sign. Ulceration is regularly covered by crusts leaving a false impression of
healing. Small vesicles may appear on the affected skin area. Lesions do not heal with
topical treatments and tend to extend in surface. Symptoms can last for many years until
the patient decides to consult a doctor. The lesion is often interpreted as a dermatitis,
eczema or psoriasis. The disease may be associated with nipple discharge of various
types, but the bleeding should be a warning sign for both patient and physician. Nipple
retraction is also a sign indicating the presence of a retroareolar cancer. Unlike exema,
skin lesions in Paget's disease have relatively well defined edges and are infiltrated. The
diameter of these lesions can be between 3 and 15 cm.
In 30-50% of cases is usually associated a palpable tumor which is located behind
the nipple in most cases (70% at a distance less than 2 cm from the nipple), but it can be
located anywhere in the mammary gland (about 30% are located away from the skin
changes)
Axillary adenopathy in the early stages is present in approximately 25% of cases.
The incidence of axillary lymph node metastases is 50-60% in all cases, higher when
the tumor is already palpable.
Clinical forms.
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Breast Pathology
Previously described is the typical form
There are approximately 20-30% of cases in which just a breast tumor is
present, without typical skin manifestations associated
When the single manifestation is nipple discharge, the risk of axillary lymph
node metastases is 5%
There are also cases (7% -26%) when the typical skin manifestations of
Paget's disease are not accompanied by an associated breast cancer
Pigmented mammary Paget's disease is rare, being described both in men
and women with intraductal breast cancer spread to the epidermis through a
ductal pore.
Differential diagnosis will be done between other conditions with similar symptoms
located at the areola and nipple:
Bowen's disease - squamous cell carcinoma in situ
Contact dermatitis
Nodular cutaneous amyloidosis
Malignant melanoma
Ductal adenoma
Adenomatosis
Nipple erosions
Diagnosis. It may be easy, as long the consulting physician has sufficient knowledge of
breast pathology. Otherwise, confusion with eczema or other inflammatory skin disease
is possible.
Investigation which has the highest chance of diagnosis is biopsy of the skin
lesion, followed by histology.
Mammography is the routine examination which may reveal, in some cases (50-
70%), the presence of mammary tumor, located behind the nipple either associated with
another region or even the presence of microcalcifications. A negative result does not
exclude the possibility of cancer.
Treatment. It is mainly surgical. Extension of breast excision is based on disease stage
and location of the tumor.
If there is no palpable tumors and if the mammography is negative, the
operation is excision of areola and nipple followed by radiotherapy.
If there is a retroareolar palpable tumor, and nowhere else, and if
histopathology reveals a carcinoma in situ - lumpectomy with excision of the
nipple and areola will be performed.
If tumor is invasive, sentinel node biopsy is recommended followed by
lymphadenectomy if metastases are present, and mastectomy.
In advanced cases or if the tumor is located away from the skin lesions,
mastectomy with axillary lymphadenectomy is indicated.
Radiotherapy is usually used after mastectomy. Chemotherapy and hormone
therapy depend on tumor histological and immunohistological features.
Prognosis. Patients with palpable breast tumors have a lower survival rate than those
with nonpalpable tumors, and also those with invasive forms and axillary lymph node
metastases (between 100% and 0% survival rate at five years, depending on evolution
stage).
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Breast Pathology
PHYLLODES TUMOR
Phyllodes tumor is a rare tumor, more often benign than malignant, that appears
exclusively in women.
It occurs in any human race and any age with a majority in the 5th decade. It
occurs more frequently on the left breast. It is a large (5-30 cm) and mobile tumor. It
represents less than 1% of all breast cancers.
It is the most common form of cancer derived from non-epithelial cells, occurring
only in the breast.
Symptoms. The patient notices the occurrence of a firm consistency tumor, mobile,
well circumscribed, in the breast. Tumor tends to increase rapidly in volume. Rarely
extends to the areola and nipple and ulcerates. The patients with metastases will have
the symptoms of those organs where metastases are.
On clinical examination: The presence of a tumor of firm consistency, mobile,
well circumscribed, non-adherent. Superjacent skin is stretched, shiny with visible
vascular design (marble like). The clinical appearance is very similar to breast
fibroadenoma and so mammographic images.
Investigations. The only way to correctly diagnose is surgical biopsy because there is
no marker for this type of tumor, and mammographic images can not distinguish
between malignant and benign form.
The differential diagnosis must be made with:
Angiosarcoma
Breast cancer
Giant fibroadenoma
Acute inflammatory cancer
Sclerosing adenosis
Liponecrosis
Fibrocystic mastitis
Breast abscess
Acute mastitis
Treatment. It is only surgical: mastectomy without axillary lymphadenectomy.
Prognosis. For the benign forms, the prognosis is very good. In malignant forms
recurrences are more aggressive as the primary tumor. Most frequently the metastases
are located in lungs followed by bone, heart and liver. The vast majority of patients with
metastases die within 3 years of treatment. Unfortunately, there is no cure for systemic
metastases.
OCCULT BREAST CANCER
Breast cancer is manifested from the beginning only by axillary lymph node
metastases or rarely with distant metastases without mammary tumors that can be
detected on physical examination or mammography.
Rare cases <1%
Clinical picture. The most frequent symptoms are: moderate pain in armpit, more as a
local embarrassment and eventually, found by self examination a tumor at this level
without an obvious cause.
Diagnosis. If breast lesions, which could explain the axillary adenopathy, are not found,
a careful examination of all areas that drain to the axilla must be performed. There are
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Breast Pathology
cases when minor skin lesions, sometimes apparently cured can be easily overlooked.
History is important and may reveal recent injuries in this areas.
The differential diagnosis should be made with other diseases that can cause
unilateral axillary lymph nodes enlargement such as:
Benign
Wounds (accidental wounds, scratching cat bites, insect bites, etc.).
Panaritium
Folliculitis, and other infectious lesions
Hidrosdenitis axillaris
Acute and chronic mastitis
Phlebitis spontaneous, traumatic or paratherapeutic of the upper limb.
Antiperspirants and deodorants
Malignant
Other skin cancers (melanoma)
Pleuro-pulmonary tumors
Cancers of the lymphatic system
Investigations. Mammography can reveal microcalcifications even before the tumor
becomes palpable and ultrasound can be helpful in detecting small cystic lesions. All
other necessary investigations will be performed (chest radiography, CT or better MRI
scan, PET scan, tumoral markers, etc)
If all the investigations find nothing, usually follows a therapeutic test period, of
about 10 to 15 days with anti-inflammatory drugs.
Axillary node biopsy is the next step in establishing the etiology when
inflammatory treatment fails.
Treatment. Do not forget that the presence of axillary metastases proven by
histopathology, represents at least stage II of breast cancer.
Radical mastectomy with axillary lymphadenectomy is most frequent applied.
Radiotherapy on the entire breast gland of first intention, without mastectomy, after
axillary lymhadenectomy could be another choice. To these, chemotherapy and
hormone therapy are added depending on the type and stage of breast cancer.
Prognosis. Many studies have shown that the prognosis for occult breast cancer is the
same or even better than for palpable tumors at the same stage (still more than stage II).
Most important prognostic factor in these cases is the number of lymph nodes affected
by metastases. In one study, survival rate at 5 years was 87% when the number of
lymph nodes was between 1 and 3, and decreased to half (42%) when their number was
4 or higher.
BREAST CANCER IN MEN
They represent only 1% of all breast cancers. In Western developed countries the
incidence of breast cancer in men is 1/100.000 men but in African countries the
incidence is much higher. It can occur at any age but is most commonly diagnosed after
the age of 60 years.
Determinant causes are not known.
The following types of breast cancer usually occur in men:
Infiltrating ductal carcinoma. The vast majority of patients have this type of
cancer.
Ductal carcinoma in situ (intraductal carcinoma)
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Breast Pathology
Inflammatory carcinoma
Paget's disease of the breast.
Lobular carcinoma in situ has not been found in breast cancer in men.
Symptoms and signs are similar to those in women with breast cancer.
Staging of breast cancer in men:
Stage 1 - tumor diameter is less than 2 cm. Lymph nodes are not affected and
there are no signs of distant metastases.
Stage 2 - the diameter of the tumor is between 2 and 5 cm. It may adhere to
structures such as skin and pectoral muscle. Usually there are enlarged
axillary lymph nodes but no evidence of distant metastases.
Stage 3 - Tumor more than 5 cm in diameter, can adhere to adjacent
structures (skin, muscle). Usually there are enlarged axillary lymph nodes
but no evidence of metastases.
Stage 4 - any size tumor, with enlarged to lymph nodes and distant
metastases.
Diagnosis and treatment are the same as for women.
Tamoxifen hormone therapy is also indicated in men especially in forms of cancer
with ER / PR positive receptors.
Prognosis. The survival rate is the same as for women in the same stage of evolution,
but men breast cancer generally is discovered in more advanced stages.
BREAST CANCER AND PREGNANCY
Tumors most commonly associated with pregnancy are:
Cervical cancer
Breast cancer
Malignant melanoma
Lymphomas
Thyroid cancer
Fortunately, the incidence of these cancers is quite low during pregnancy. For this
reason, there are no statistical studies on many cases, sporadic cases being reported in
the literature.
Cancer during pregnancy raises special ethical and psychology problems. The
patient must choose between maintaining the pregnancy and cancer treatment.
Breast cancer incidence is about 0.01 to 0.03% of pregnant women and are most
often found in women who delay pregnancy until the age between 30 and 40 years.
During pregnancy, significant breast changes occur, changes, which make
difficult early detection of small tumors. In pregnant women, in general tumors are
detected with a delay of five months from the nonpregnant. In addition, pregnant
women have a 2.5 times higher chance of being diagnosed with metastatic breast
cancer.
Mammographic examination is avoided during pregnancy due to exposure to
radiation. Any breast changes considered abnormal will be examined by
ultrasonography. Biopsy is encumbered with the risk of suppurations, hematoma and
bleeding in pregnant women. It is done under the protection of antibiotics.
Breast cancer treatment during pregnancy. Treatment of breast cancer in pregnant
women is mainly surgical: lumpectomy or mastectomy with or without axillary
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Breast Pathology
561
lymphadenectomy according to the presence of increased axillary lymph nodes, and
tumor stage. If the woman is in the last 2-3 weeks of pregnancy, surgery may be
postponed until after birth. If the tumor is diagnosed during the first weeks of
pregnancy, abortion and complex treatment of cancer would be the best choice.
Radio-chemotherapy and hormono therapy will not be used during pregnancy.
If breast cancer was found postpartum, the same principles of treatment as
provided in any woman will be applied, with breastfeeding discontinuation.
Prognosis of breast cancer during pregnancy is identical to that of nonpregnant
women in the same stages.
Pregnancy after breast cancer treated in history
Women who have been treated previously for breast cancer could have a normal
pregnancy in future. The minimum duration of time from diagnosis and treatment of
breast cancer to pregnancy should be at least 2 years.
Breast cancer effects on fetus
So far, no cases of metastases at the fetus from breast cancer have been cited, but
there were several cases cited in the literature of metastases in the placenta.

Surgical Pathology of the Arteries
SURGICAL PATHOLOGY OF THE ARTERIES

1. Anatomical and physiological aspects of the vascular system
2. Methods of investigation in surgical diseases of arteries
3. Arterial trauma
4. Arteriovenous fistulas
5. Arterial aneurysms
6. Acute peripheral ischemia
7. Chronic obstructive arterial disease
1. Anatomical and Physiological Aspects of the
Vascular System
Vascular system is represented by a network of blood vessels that carry the blood
from the heart to tissues and vice versa, the heart being the main pump that keeps
moving the blood.
There are two vascular circuits: pulmonary and systemic.
1. Pulmonary circuit leads venous (deoxygenated) blood collected by the right
heart to the lungs to be oxygenated and from there back to the left heart. In this
circuit, oxygenated blood is carried by veins (returning vessels pulmonary
veins) not by arteries like in systemic circuit.
2. Systemic circuit carries oxygenated blood and nutrients through the arteries to
tissues where, at level of capillary bed, takes place the exchange: blood releases
oxygen and nutrients towards tissues and is loaded with carbon dioxide and
waste.
Deoxygenated blood is transported by veins to the right heart (atrium and
ventricle) and from there to the pulmonary circuit. Wastes are transported by arteries to
the liver and kidneys where they are metabolized and eliminated.
Blood vessels leaving the heart are called arteries and those arriving are called
veins. The arterial and venous systems are interconnected by small vessels called
capillaries.
In their journey from the heart towards tissues, the arteries ramify and blood
passes through vessels of six principal types: elastic arteries, muscular arteries,
arterioles, capillaries, venules and veins.
From heart towards tissues, arteries show a progressive diminution in diameter
from about 25 mm in the aorta to 0.3 mm in some arterioles.
Structure of the vessels wall
Blood vessels are not simply rigid pipes. They are active organs, elastic, with a
complex structure. The artery wall consists of three layers:
1. Tunica Adventitia is the outer layer of arteries and veins. It is composed of
connective tissue, collagen and elastic fibers. These fibers allow the arteries
and veins to stretch to prevent overexpansion due to the pressure that is
exerted on the walls by blood flow.
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2. Tunica Media is composed of smooth muscle and elastic fibers. This layer is
thicker in arteries than in veins. The structure also depends on the type of
artery and determines the mechanical properties of the arterial wall. In larger
arteries, it consists of connective tissues, elastic tissue and elastic fibers. In
smaller arteries, smooth muscle cells replace the elastic fiber layer.
3. Tunica Intima (endothelium) is the inner layer of arteries and veins, an
interface between circulating blood and blood vessel wall. It is composed of
an elastic membrane lining and smooth endothelium (special type of epithelial
tissue).
Endothelial cells are involved in many aspects of vascular biology, including:
Atherosclerosis
Barrier function - controlling the passage of materials and white blood cells
into and out of the bloodstream.
Blood clotting (thrombosis & fibrinolysis). The endothelium normally
provides a non-thrombogenic surface.
Inflammation
Angiogenesis
Vasoconstriction and vasodilatation, and hence the control of blood pressure
Specialized 'filtering' functions in some organs (the renal glomerulus and the
blood-brain barrier)
Impaired endothelial function causes hypertension and thrombosis
The arterial system is also fundamentally involved in the physiology of blood
flow and homeostasis of blood pressure, in coagulation and fibrinolysis, in achieving the
inflammatory and immune response and also in lipoprotein metabolism.
It seems that the endothelial cell due to the roles assigned to it in various systems
of regulation is the cell with the most and perhaps most important functions in the body.
Because of the essential antithrombotic role of the endothelial cells, vascular
surgery is practiced with full protection of the vascular endothelium.
Although both carry blood, there are major differences between the arterial and
venous system both in terms of anatomic structure and physiology, which must be
known to understand the pathology of blood vessels. (Table 1)
Table 1 - Differences between arteries and veins
Arteries
Transport blood away from the heart
Carry oxygenated blood
(except in the case of the pulmonary artery)
Have relatively narrow lumen
Have relatively more muscle/elastic tissue
Transports blood under higher pressure than
veins
Do not have valves (except for the semi-lunar
valves of the pulmonary artery and the aorta)
Veins
Transport blood toward the heart
Carry de-oxygenated blood
(except in the case of the pulmonary vein)
Have relatively wide lumen
Have relatively less muscle/elastic tissue
Transports blood under lower pressure (than
arteries)
Have valves throughout the main veins of the
body.
Arteries are divided into four types:
1. Elastic - Conducting arteries. They are represented by the largest arteries:
aorta and its branches, and have a large diameter between 1 and 2.5 cm.
Because of the large diameter, they have a low resistance pathway. Their wall
contains most elastic fibers, which enables them to withstand high pressure.
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Muscular layer is not active in vasoconstriction. Inside the artery, the flow is
pulsatile. They expand in systole and recoil in diastole.
2. Muscular - Distributing arteries. Their diameter is smaller (1 cm to 0.3 cm).
They deliver blood to specific organs. Their muscular layer is well developed
being active in vasoconstriction and vasodilatation.
3. Arterioles. Their diameter is between 0.3 cm and 10 micrometer. Large
arterioles have more smooth muscles but smaller arterioles have a small
amount of muscles. They constrict and dilate in response to neural and
chemical stimuli and represent the most important site of resistance in the
whole systemic circulation.
4. Metarterioles. They are short vessels that links arterioles and venules. The
muscular layer is composed of smooth muscle cells placed a short distance
apart, each forming a precapillary sphincter that encircles the entrance to that
capillary bed. Constriction of these sphincters reduces or shuts off blood flow
through their respective capillary beds. This allows the blood to be diverted to
elsewhere in the body.
Capillaries have the endothelial layer placed on a basement membrane. The more
metabolically active the cells, the more capillaries present in the tissue. They are of
three types: continuous (endothelial cells provide an uninterrupted lining), fenestrated
(have pores in the endothelial cells) and sinusoidal (a special type of fenestrated
capillaries that have larger openings) (Ex. In the liver, spleen)
Arterial branches have anastomosis between them at different levels so that a
particular anatomical region receives blood from several sources vascular collateral
circulation. The degree of extension and function of collateral circulation differs from
one area to another (from one organ to another), being the least well represented in the
coronary arteries, renal and retina where the circulation is of terminal type ("end
arteries"). Development and functional status of collateral circulation is an issue of
utmost importance in case of arterial occlusion. In such situations, the effectiveness of
collateral circulation depends on the type of occlusion: acute or chronic occlusion.
Blood flow
The pumping action of the heart generates blood flow. Blood flow and pressure
are unsteady. The cyclic nature of the heart pump creates pulsatile conditions in all
arteries. The blood flow that passes through a given blood vessel depends directly on
gradient of pressure between the two ends of the blood vessel and indirectly on the
resistance of blood movement. Normal arterial flow is laminar with secondary flows
generated at curves and branches. Turbulent flow induces:
An increase of platelet count near the intima layer of the vessel
Increased time of contact between platelets and endothelium
A decreased rate of clearance of procoagulant factors at interface between
endothelium blood stream
Blood vessels are not simple pipes (they have the ability to adapt to differences in
pressure and also to adjust the pressure by the action of their muscular layer). On the
other hand, blood is not a simple fluid but a liquid tissue that has a certain viscosity and
can, under certain conditions to clot forming plugs that can obstruct the vessels.
In normal laminar flow, all the blood cells and platelets occupy the central (axial)
stream. The periphery of the blood stream, adjacent to the endothelium, moves more
slowly and is composed of blood plasma.
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Stasis and turbulence in this flow caused by vascular disease, chronic congestion,
and external compression and by a lack of movement of the muscles that normally
"pump" blood through the veins may cause dysfunction or damage to the endothelium
and brings blood platelets into contact with the endothelial cells permitting the build-up
of thrombi.
Blood pressure is the force exerted by blood flow on the walls of blood vessels.
(mmHg). The blood flows from high to low pressure (gradient). Pressure results when
flow is opposed by resistance. Blood pressure always refers to systemic arterial blood
pressure in the large arteries.
Pulse pressure represents the difference between systolic and diastolic blood
pressure. Systolic = 120 mmHg, Diastolic = 70 to 80 mmHg. Pulse pressure is felt as the
pulse: pulsation in an artery in systole.
Mean Arterial Pressure (MAP) represents the average of blood pressure.
Diastolic is more important because diastole is longer than systole in a single cardiac
cycle. MAP = Diastolic Pressure + 1/3 (Pulse pressure)
Peripheral Resistance depends on:
Blood viscosity: plasma proteins and blood cells (RBCs) make blood
viscous. It is normally constant. Blood is approximately four times more
viscous than water.
Blood vessel length: the longer the vessel the more resistance (normally
constant). Blood vessels cant become longer but can become shorter as a
result of arterio-venous shunts. This leads to decrease of resistance but also
to increase of venous flow and pressure.
Blood vessel diameter is the most important in determining peripheral
resistance. The smaller the diameter result in more friction and higher
resistance. Because arterioles can dilate and constrict they are the major
determinants of peripheral resistance.
Blood pressure is highest in the aorta and decreases steadily until the arterioles.
2. Methods of Investigation in Surgical Diseases of
Arteries
Arterial surgical techniques developed and diversified greatly in recent years,
supported largely by modern methods of diagnosis and preoperative evaluation of
surgical diseases of the arteries.
Methods of investigation:
Non-invasive:
o Oscillometry
o Plethysmography
o Determining the leg-arm index
o Effort test
o Ultrasound and Doppler ultrasound
o Nuclear magnetic resonance and computed tomography in some
cases
Invasive: angiography (arteriography)
The gold standard in exploring arteries remains the angiography, but it is not
mandatory in all cases.
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Oscillometry is now an outdated method that offers just indicative data about
peripheral arterial pathology. The principle of the method is the measurement of
transmitted intra-arterial pulsations to the occluding cuff surrounding the limb. It
measures the peak of oscillations of the mean arterial pressure (oscillometric index). It
compares the amplitude of oscillations making simultaneous measurement in different
parts of the body.
The instrument is called Pachon's oscillometer (1900) which is an improvement
over the oscillometer devised by Heinrich von Recklinghausen (1867-1942). It was
widely used during World War I and between the world wars. Over the time,
improvements were made represented by automated instrumentation using
plethysmographic sensors, pulse-wave velocity sensors and ultrasonic microphones.
Plethysmography measures the changes in limb volume induced by cardiac
activity. It was, for a long period, the main way of non-invasive investigation of
peripheral arteries, now replaced by direct imaging methods.
The Ankle Brachial Pressure Index (ABPI), is the ratio between the blood
pressure in the lower limbs and that in arms. Compared to the arm, lower blood pressure
in the leg is an indication of blocked arteries (peripheral vascular disease). The ABI is
calculated by dividing the systolic blood pressure at the ankle by the systolic blood
pressures in the arm. The value is usually between 0.91-1.3, the degree of alteration of
its values quantifying the degree of peripheral obstruction. Analysis can be sensitized by
performing measurements in an effort test.
A Doppler ultrasound probe and a sphygmomanometer are usually needed. The
blood pressure cuff is inflated proximal to the artery. Measured by the Doppler probe,
the inflation continues until the pulse in the artery ceases. The blood pressure cuff is
then slowly deflated. When the artery's pulse is re-detected through the Doppler probe,
the pressure in the cuff at that moment indicates the systolic pressure of that artery.
ABPI drawbacks: it is unreliable on patients with arterial calcification, which
results in less, or incompressible arteries, as the stiff arteries produce falsely elevated
ankle pressure, giving false negatives. In addition, resting ABPI is insensitive to mild
peripheral arterial disease requiring testing during effort, which is not suitable for
patients with co-morbidities. ABPI needs skilled operators, is time consuming and
there is a lack of protocol standardization.
ABPI interpretation
Measurement Interpretation
>0.90 Normal
0.71-0.90 Mild obstruction
0.41-0.70 Moderate obstruction
0.00-0.40 Severe obstruction
Ultasonography (transthoracic echocardiography or transoesophageal, Doppler
ultrasound of peripheral arteries, etc.) is the most common current method used in
diagnosis of vascular diseases. The ability to detect blood flow velocities and waveform
characteristics allow the surgeon to understand the hemodynamic significance of a
vascular disease.
Advantages of ultrasonography:
Does not require intravenous or intra-arterial access
Does not expose the patient to contrast substances
The examination device is portable, which allows evaluation in the
emergency room, intensive care unit, and/or operating room
Duplex scanning is also one-tenth of the cost of conventional arteriography.
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Standard computed tomography (CT-scan) is used in the investigation of large
vessels pathology when both transesophageal ultrasonography and magnetic resonance
are unavailable (or if MRI is contraindicated). A variant of this method, known as spiral
CT, is more accurate but more expensive.
Computed tomography angiography is a more sensitive and specific method of
investigation, with easy access and excellent diagnostic capability. A CT angiography
of the lower extremity is obtained by injecting a bolus of intravenous contrast through
the peripheral venous system. After an appropriate delay, a CT of the lower extremities
is obtained while the arteries fill with contrast. The image can then be quickly
reformatted to visualize the appropriate vessels. Computed tomography findings that
suggest arterial injury include contrast extravasation, pseudoaneurysm formation, abrupt
narrowing of an artery, loss of opacification of an arterial segment, and arteriovenous
fistula formation.
CT angiography may give more precise anatomical detail than magnetic
resonance imaging (MRI), particularly in small blood vessels. CT angiography has still
some risks and limitations:
Exposure to radiation. However, the benefit of an accurate diagnosis far
outweighs the risk.
Allergy to x-ray contrast material.
Extravasation of contrast can cause tissue damage.
Contrast nephropathy.
Very large patients may not fit into the opening of a conventional CT
scanner or may be over the weight limit
Nuclear magnetic resonance is a non-invasive exploration, which provides
significantly better resolution, but is expensive and rarely available in emergency. It is
indicated in long-term monitoring of large artery pathology.
Angiography is a procedure, which uses x-ray and contrast substances to
highlight arteries and veins and to observe the blood flow through them. While
performing this procedure a thin catheter is inserted into a blood vessel and guided to
reach the area to be examined. Through that catheter, a dye is injected and the vessel
appears highlighted when x-ray images are taken. Images can be stored also as
radiological film in a digital form.
Benefits of this method are:
It may eliminate the need for surgery
Catheter angiography presents a very detailed, clear and accurate picture of
the blood vessels
It offers the possibility to assess vessels in specific body sites (superselective
angiography)
It makes possible to combine diagnosis and treatment in a single procedure
(angioplasty, placement of a stent, embolization)
Risks of the method:
Heart attack
Stroke
Trauma to the catheterized artery
Irregular heart rhythms (arrhythmias)
Allergic reactions to the dye or medication
Perforation of heart or artery
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Kidney damage
Excessive bleeding
Infection
Blood clots
Radiation exposure from the X-rays
3. Traumatic Lesions of the Arteries
The consequences of arterial injury depend on the intensity and nature of injury,
size and location of the concerned artery, and degree of impairment.
Traumatic lesions of the arteries have two major clinical manifestations:
1. Peripheral acute ischemia
2. Hemorrhage, which is the defining characteristic of this type of pathology
The frequency of traumatic injuries of arteries is about 20% of all injuries. Leg
arteries are interested in 50-60% of cases. In most cases, the lesions affect both arteries
and veins, but can also affect other structures such as nerves, bones, muscles, etc.
The highest incidence of arterial injuries occurs during armed conflicts, when
very frequently extremities vessels are affected. Extremities amputations (as an extreme
measure for life saving) due to vessels injury reached an incidence of 40% during the
Second World War. Newer methods of reconstruction, including endovascular surgery,
are now applied to nearly half the vascular injuries.
Etiopathogenesis
In peacetime, causes of arterial lesions in order of frequency are: car accidents,
work accidents, sports accidents, domestic accidents and aggressions.
Vessels injuries may occur after two kinds of trauma: blunt trauma and
penetrating trauma. The most common injuries occur as a result of wounds.
The mechanism of injury:
Direct arterial lesions consecutive to blunt trauma or penetrating trauma
Indirect lesions usually caused by fragments of bone fractures
Iatrogenic lesions during surgery, endovascular explorations (catheter
angiography)
Morphopathology
Contusions represent the injuries, in which the structure of the arterial wall is
only partially affected, the continuity of the artery being preserved. Most frequently is
damaged the inner layer (tunica intima), which is the most brittle. Usually the tear is
circular partial or complete. Broken endothelium may reflect and act as a damper
causing occlusion and distal ischemia.
Sometimes, the tunica media breaks leading to a weakening of the arterial wall
and the appearance of an aneurysm.
A hematoma in the arterial wall can produce artery occlusion with ischemia.
Partial rupture of the artery. The tear remains opened because of intima layer
retraction, favoring bleeding. Bleeding may be exteriorized or may occur in a cavity or
hollow organ. The accumulation of blood around the artery may causes compression or
a pulsatile hematoma and then a false posttraumatic aneurysm may occur. In the early
phase of false aneurysm, the risk of breaking and of external hemorrhage is very high
(80-90% of the cases). After three weeks, in the next 6-8 weeks, the fibrous
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organization of the pulsatile hematoma walls occurs and it transforms into a false
aneurysm, which becomes stable, yet has progressive evolution.
There are cases when both artery and satellite vein are injured and an
arteriovenous fistula may result.
Complete artery rupture or section usually is the consequence of stab wounds or
fire arms wounds. Complete ruptures occur after extreme elongations usually associated
with bone fractures.
Hemorrhage may be massive (exteriorized or into a cavity) or may stop as a result
of media layer contraction, thrombus development at side of arterial stumps or outside
compression due to hematoma. Pulse disappears shortly after trauma. Proximal and
distal to lesion, an intravascular thrombosis occurs up to the first efficient collateral
artery. Ischemic lesions extension depends on collateral circulation efficiency.
Diagnosis is based on history, general and local examination and investigations.
Depending on patients status, severity of hemorrhage, and facilities of investigation,
CT angiography, Doppler ultrasound and catheter angiography may be performed in
supporting the diagnosis. In any circumstances angiography or other investigations
should not delay the surgical restoration of blood flow.
Patient assessment is rapidly performed evaluating the degree of hemorrhage and
shock (pulse, faintness, low blood pressure, sweaty cold skin, pallor, oliguria, etc.).
History (co-morbidities) and important data regarding the mechanism of trauma
can be obtained from patient, relatives or witnesses.
Involved limb examination is performed compared to the opposite limb.
Instrumental exploration of the wound is indicated to be performed only in the operating
room.
The standard care is represented by arteriogram in stable patients and operative
exploration in unstable or bleeding patients.
Classical direct signs of vascular injury include the following:
Observed pulsatile bleeding
Arterial thrill (vibration) by manual palpation
Bruit over or near the artery by auscultation
Signs of distal ischemia
Visible expanding hematoma
Posttraumatic ischemia clinical picture has some specific features:
The pain is persistent, progressive and does not diminish after antialgic
drugs administration or reduction of bone fractures or joint dislocation.
Pulse may be present in contusions. On the other hand pulse may be
absent in bone fractures or joint dislocation and reappears after reduction.
Functional impairment of the limb may be due to bone, joint, muscle or
nerve lesions not necessarily to ischemia.
The full clinical picture of peripheral ischemia appears at 4-5 hours after trauma.
Nerve lesions occur after 15-30 minutes and may be reversible until 8-12 hours.
Anesthesia and palsy and also muscles contracture (rigor mortis) appeared at 6-8 hours
are signs of severity and irreversibility. After 12-14 hours, the ischemia is irreversible
and the only chance to rescue the patients life is limb amputation.
Treatment
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Bleeding is the main problem to be solved and requires immediate surgical
intervention in any elective or urgent cases, because of the vital risk. The first main goal
is to save the patients life. When the hemorrhage is present, (almost in every cases), it
must be stopped to prevent anemia and shock.
The other main aim of the treatment is to reestablish and maintain an efficient
blood flow in the affected limb to prevent ischemia and amputation.
Other therapeutic measures will be taken to prevent thrombosis and other
postoperative possible complications.
Hemostasis must be applied at site of accident. The most effective method is the
compressive wound dressing. This is a temporary hemostasis but in some cases, when
the diameter of the artery is small it can become a definitive hemostasis. Other
temporary hemostasis methods are: digital compression, vessel clamping with forceps,
hemostasis by tourniquet.
There are many controversies regarding hemostasis by tourniquet. Negative
aspects are: it suppresses the venous return, worsens the venous hemorrhage, worsens
ischemia, blocks collateral circulation and after removing the tourniquet the shock may
appear (tourniquet-shock syndrome) as a result of passage into the bloodstream of
toxins from ischemic tissues.
A temporary method in preserving blood flow in the affected extremity is an
improvised by-pass using different kind of materials (catheter, drainage tubes, etc.),
followed by definitive hemostasis and blood flow reestablish performed in adequate
conditions by specialists.
Definitive hemostasis may be achieved in different ways depending on injured
vessel and its location.
Vessel stumps ligation could be a solution in non-important arteries, or when
collateral circulation ensures a good blood supply. It cannot be applied in large arteries
due to consecutive downstream ischemia.
In some cases, operative intervention is primarily performed for life-saving
hemorrhage control rather than for operative repair with limb salvage. In severe cases
with multiple associated injuries, hemorrhage control by ligation of actively bleeding
arterial or venous vessels may be all that is possible
Arterial reconstruction is the method used in large and important arteries.
Depending on type of lesion, it can be performed by:
Vessels wall suture
End-to-end suture
Patching the arterial defect (with autologous or prosthetic material)
Interposition of graft
Bypassing the affected area using prosthesis, vein or artery fragment
harvested from other location
Vascular reconstruction that occurs within 3 hours of injury is generally accepted
to have the best outcome. After reconstruction, the surgeon should consider the risk of
reperfusion injury and the potential for compartment syndrome (compression of nerves,
blood vessels, and muscle inside a closed space).
When there is a marked edema of the limb decompression fasciotomy (a surgical
procedure where the fascia is cut to relieve tension or pressure) is required.
In case of trauma with complex lesions (bones, nerves) most often mixed team of
surgeons (vascular surgeon, neurosurgeon, orthopedic) is required.
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In case of prolonged ischemia in which there are already signs of irreversibility,
surgeons should balance the desire to save the limb with that of preserving the patient's
life. Limb amputation remains the only choice in advanced cases of ischemia.
Thrombosis of the graft remains the most common complication of vascular
injury and blood vessel repair. On the other hand, anticoagulation and antiplatelet agents
should be balanced with the risk of fatal hemorrhage from other injuries (eg. head and
chest injuries).
4. Arterial Aneurysms
The aneurysm represents a localized permanent dilatation of the vessel produced
by decreased strength of its wall.
The incidence increases with age, aneurysms being found in about 10% of cases
of autopsies. Approximately one in every 250 people over the age of 50 will die of a
ruptured aortic aneurysm. Abdominal aneurysm affects as many as eight percent of
people over the age of 65. Males are four times more likely to have abdominal
aneurysm than females. Those at highest risk are males over the age of 60 who have
ever smoked and/or who have a history of atherosclerosis. Half of patients with aortic
aneurysm who do not undergo treatment die of a rupture.
Etiology
Congenital aneurysms are present at birth and they are due to chromosomal
abnormalities that induce degeneration of the elastic and muscular fibers. It is frequently
associated with endocrine disorders.
Acquired aneurysms appear during lifetime and comprise the all other
aneurisms
Atherosclerosis 95% of aortic aneurisms
Infectious due to nonspecific (gram positive or negative) germs or specific
agents (syphilis) and fungi, which produce ulceration of the intima
Rheumatic
Posttraumatic unlike the true aneurysm, the false aneurysm wall does not
have a muscular or elastic layer. False aneurysms usually present as a
pulsatile mass.
Anastomotic - as a late complication of vascular surgery
Morphology
Aneurysms may be fusiform or saccular.
The aneurysm may contain clots as a result of turbulent blood flow.
Dissecting aneurysm represents a special type of aneurysm localized on thoracic
or abdominal aorta in which the wall rips (splits, dissects) longitudinally and the blood
flows between layers. The etiology and pathogenesis is usually a degenerative process
due to a primary or secondary weakness of the vessel wall as in Marfans disease, cystic
medial necrosis, hypertension or atherosclerosis.
Less frequently it is seen after iatrogenic manipulations (puncture, catheter
interventions etc.), in coarctation and trauma.
Arteriovenous aneurysm results from nearby vein erosion and a direct vascular
link between an artery and a vein. Arteriovenous aneurysms are usually the result of
arteriosclerosis. Less common causes include trauma, inflammation of an artery
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(arteritis), and infection. Arteriovenous aneurysms occurs most commonly in the arms
and legs.
Clinical picture. Symptoms depend largely on the developing stage of the aneurysm,
its complications and its location.
There are no symptoms in the early stages. As volume increases, aneurysm
produces compression on adjacent structures causing various symptoms, pain being the
most important.
Symptomatic aortic aneurysms present as lower back pain and/or mid-abdominal
pain. Associated with prominent aortic pulsation it suggests rapid growth of aneurysm
with possible rupture. Severe back, abdominal or flank pain with hypotension indicates
a ruptured aneurysm. Up to 90% of patients die before reaching hospital or during or
immediately after surgery.
The cardinal manifestation of the aorta dissection is the pain, present at onset in
most cases, with acute onset and high intensity, with posterior or anterior thoracic
location radiating anywhere in the chest or abdomen. In addition to pain and pre-
existing hypertension, circulatory disorders related symptoms are caused by propagation
of dissection, proximal or distal.
Propagation towards the ascending aorta can result in:
Acute aortic insufficiency (30-50% of dissections of the ascending aorta);
Acute myocardial ischemia;
Cardiac tamponade and sudden death in case of rupture of the aorta in the
pericardium;
Hemothorax if rupture extends beyond the external layer;
Neurological deficits to stroke, by direct spread to carotid arteries or by
reducing carotid blood flow;
Horner's syndrome (superior cervical sympathetic ganglion compression),
vocal cord paresis with hoarseness (compression of left recurrent nerve).
Propagation of dissection in the descendending aorta can cause:
Splanchnic ischemia,
Renal failure,
Peripheral pulse deficit with varying degrees of peripheral ischemia,
Focal neurological deficits due to spinal ischemia.
Suspicion of aortic dissection includes some of the following aspects in elderly
patients with atherosclerosis and hypertension long history:
Acute onset of violent chest pain
Mediastinum widening on chest X-ray image
Inequality of pulse and blood pressure between upper limbs
Differential diagnosis of pain is made with acute myocardial infarction (can also
develop early circulatory failure), massive pulmonary embolism, acute pancreatitis
(some cases), pericarditis, etc.
Evolution
Abdominal aneurysms grow an average of 0.3-0.4 cm / year. Risk of rupture at 5
years depending on the size of aneurysm is:
Very low risk for aneurysms less than 4 cm
5% for aneurysms between 4-4.9 cm
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75% for aneurysms 7 cm or greater
Distal arteries aneurysms (femoral, popliteal) are frequently associated with
symptoms resulting from thrombosis, embolization or compression of adjacent
structures causing venous thrombosis or neuropathy.
Investigations
Abdominal ultrasound is recommended as screening study and follow-up for
small <5 cm aneurysms. Aortography, MRI, and CT scan are the most used
investigations.
Full assessment of cardiovascular status and cardiovascular risk factors are
necessary.
Because vasculitis and connective tissue disorders may cause aneurysms, an ESR,
CRP and autoantibody profile may be indicated.
Assessment of renal function is important because it can be compromised by
aortic aneurysm.
Assessment of peripheral circulation using Doppler.
Classification
Treatment
Prophylaxis consists in atherosclerosis prevention and treatment of hypertension.
Indications for elective surgery are:
1. Abdominal aortic aneurysms larger than 5-5.5 cm diameter
2. Variation of diameter greater than 0.5 cm in 6 months
In all patients with aneurysms which are not operated, ultrasound monitoring
should be made at every 6 months.
Surgical possibilities:
1. Resection of the aneurysm and arterial reconstruction
2. Bypassing the aneurysm
3. Resection of a portion of the artery with reconstruction
4. Endoluminal stenting (endovascular stent graft, graft inclusion) through a
percutaneous procedure
The treatment of a dissecting aneurysm initially involves lowering the blood
pressure with drugs to reduce the force on the tear in the aorta.
Generally, it is considered that the ascending aorta dissections are surgical
emergency and must be treated by surgery, while the descending aorta dissections are
treated conservatively, unless there are signs of progression of dissection or bleeding
with hemodynamic instability. The closer the dissection to the heart, the more likely is
that surgery will be performed.
Surgical therapy, even aggressive (eg. replacing the aortic arch) or in unfavorable
conditions (myocardial infarction, stroke) results in lower mortality than with
conservative attitude in the ascending aorta dissection (7-35% compared to 50% ), so all
patients with DeBakey class I and II should be treated surgically in emergency.
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5. Arteriovenous Fistulas
Arteriovenous fistulas are direct communications, through a duct or network,
between the venous and arterial system that bypasses the microcirculation.
The passage of the arterial blood (with higher pressure) into the vein will induce
an increased pressure in that vein which becomes elongated, dilated, tortuous and
pulsatile. Other effect is the decrease of arterial irrigation distal to fistula with possible
ischemia.
The general consequence is the increased blood flow into the veins with increased
blood return to the heart that will overload the heart with the consequence of increased
heart output and its dilatation. The heart may become insufficient (heart insufficiency).
Location of fistulas may be anywhere, in any artery, central or peripheral.
Classification. Fistulas may be:
Congenital Generally, they are located peripheral and are a consequence of
angiodysplasia. The appearance is that of hemangioma, looking red-purple, soft to the
touch, reducing their dimension under compression. They may be located on limbs,
abdomen, thorax, neck, head or in viscera.
The Parks-Weber syndrome is a congenital disease with multiple skin
hemangiomas characterized by the triad:
1. Elongation of the inferior limb
2. Diffuse angiodysplasia
3. Varicose veins
Acquired with various etiology
Posttraumatic (see above) Communication between artery and vein may be
unique or multiple. They are locate in most cases on limbs but may be present
at neck, penis or intraabdominal.
Spontaneous intraabdominal as a result of evolution of a tumor or aneurysm.
Post-surgical unintentional ( kidney surgery) or intended, like in portal
hypertension (portal-systemic shunts) or in chronic renal failure (radio-cubital
shunt for hemodialysis). For hemodialysis, special large core catheters are
used. Performing an arteriovenous shunt will result in venous dilatation so
that the vein will fit the catheter.
Clinical picture
Functional symptoms are represented by moderate local pain, muscular fatigue,
sometime intermittent claudication. Symptoms of heart overload such as tachycardia,
dyspnea, symptoms of heart failure may be present.
Physical examination reveals venous dilatation, limb enlargement, dilated
capillary vessels (hemangiomas), skin discoloration, dystrophic skin lesions, and soft
tissue gangrene. On palpation, the tumor is soft, compressible, with elevated local
temperature and a thrill can be felt. On auscultation, a systolic or continuous sound
(murmur) can be heard. Digital compression of the fistula reduces tachycardia because
reduces the venous return.
Investigations: Doppler ultrasound, angiography and CT scan are helpful.
Complications: thrombosis, skin ulcerations and gangrene, infections, heart failure,
distal ischemia.
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Treatment. There are various methods depending on fistula location, dimension and
debit.
Percutaneous embolization using Gelofoam
Quadruple vascular ligation
Vascular reconstruction
6. Peripheral Acute Ischemia
Peripheral arterial occlusion is an important cause of morbidity and mortality,
particularly in connection with atherosclerotic disease, a pathological condition that can
generate any form of peripheral obstruction (on small, large or medium vessels, acute or
chronic).
Acute peripheral arterial occlusion results in acute peripheral ischemia syndrome,
which is caused by the suppression or sudden decrease of blood flow to the limbs,
followed by tissue anoxia. This may happen when the artery is broken or occluded.
Ischemia is a condition of decreased tissue viability caused by a lack of perfusion
limiting the delivery of oxygen and nutrients to the tissue. This causes both
physiological and biochemical changes in the tissue.
The severity of the acute manifestation depends on the site of occlusion, presence
of collateral circulation, and nature of the occlusion (thrombus or embolus). Emboli are
associated with a higher morbidity because the limb has not had time to develop
collateral circulation.
Arterial occlusion results in both, proximal and distal thrombosis due to flow
stagnation.
Etiology. Acute peripheral ischemia syndrome may be caused by:
Organic causes
o Trauma with damage to arteries
o Embolism
o Acute arterial thrombosis
o Arterial wall dissection
Functional causes
o Neuro-vascular reaction to different kind of agents (low temperature
frost bite, or chemicals ergotamine)
o Massive venous thrombosis, with secondary ischemia (phlegmasia
coerulea dolens)
o Arteriovenous fistula
o General causes: eg. toxic shock with collapse
Embolism
Emboli are the most common cause of acute ischemia. Their origin may be:
cardiac (80%), proximal atheroma, tumors, foreign objects, gas embolism, and fatty
embolism.
Causes of cardiac embolism are: atrial fibrillation, rheumatic valvular heart
disease (especially mitral), left ventricular myocardial infarction with thrombosis, post-
myocardial infarction sequelae, valves, endocarditis vegetations on native or prosthetic
valves, cardiac tumor fragments (atrial myxoma).
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Emboli tend to lodge at artery bifurcations or in areas where vessels abruptly
narrow. The femoral artery bifurcation is the most common site (43%), followed by the
iliac arteries (18%), the aorta (15%), and the popliteal arteries (15%).
A special form of embolism is the paradoxical embolism, when a deep venous
thrombus reaches in the arterial system. This can happen when there is a communication
between the right and the left atrium (atrial septal defect) and the thrombus reached in
the right atrium from the vein passes into the left and then get in the arteries.
Acute arterial thrombosis
Atherosclerotic lesions of the artery most frequently cause it. Other causes may
be: aneurysms, hypercoagulability (thrombosis in situ), artery trauma with intima layer
injury. It may occur as a complication of diseases: thrombangeitis obliterans, lupus
erythematosus, periarteritis nodoasa, polycythemia vera, scleroderma.
Thrombosis can be initiated anywhere in the cardiovascular system, isolated or in
combination conditions of the Virchow triad: endothelial injury, blood stasis,
hypercoagulability.
Pathophysiology
Arterial occlusion will result in:
Sudden stop or important decrease of blood flow downwards the
occlusion in the affected artery and also collaterals
Extensive thrombosis downstream and upstream the occlusion
Arterial spasm to compensate the decrease of arterial pressure resulting in
increased peripheral resistance
Decrease of cardiac debit
Extension of secondary thrombosis (vicious circle)
Capillary stasis with sludge
Cellular hypoxia
Transmineralization (Na+ enters the cells and K+ exits the cells resulting
in cell edema)
The metabolism switches from aerobe to anaerobe leading to metabolic
acidosis, lysosomal lysis and cell destruction.
Tissues resist to ischemia depending on their type. Nervous tissue after 15-30
minutes presents edema, myelin degeneration and then the neuromuscular plate is
affected. Symptoms are pain, paresthesia, anaesthesia and functional impotence of the
limb. These changes are reversible until 8-12 hours after attack.
The muscular tissue after 8-12 hours presents edema, capillary stasis,
disappearance of myoglobin, rhabdomyolysis, fibroblastic infiltration and Volkmann's
ischemic retraction or contracture.
Fatty tissue and skin are more resistant to ischemia modifications being reversible
until 12 hours. Phlyctenas and gangrene appear.
Clinical picture. Symptoms appear suddenly and are usually violent. British authors
describe them as the "6p" :
1. Pain
2. Pulselessness
3. Pallor
4. Poikilothermia (perishing cold)
5. Paresthesia
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6. Paralysis
Acute ischemia develops during 3 phases: initial phase, phase of worsening and
phase of tissue damage.
The pain usually occurs suddenly, distal to lesion, it is intense and does not
disappear after the administration of painkillers. Sometimes the pain may begin
insidiously, especially in older people with atherosclerosis. The pain may be masked by
nerve damage or shock in trauma.
The abolition of tactile sensibility, the occurrence of ischemic paralysis and
muscle rigidity are signs of serious (irreversible ischemia). Tactile sensibility is the first
abolished and then the pain and thermal sensitivity.
Skin pallor is distal to the occlusion and later turns in purple-blackish color due to
cyanosis and gangrene.
Absence of pulse distal to the occlusion is an important sign as long as it was
present before the ischemic episode. There are patients with chronic peripheral arterial
disease who do not have peripheral pulse in legs. It is also difficult to assess the pulse if
an important edema is present or the anatomic region is modified by trauma.
In the worsening stage, the intravascular thrombosis is extending leading to
intense edema of the muscles and cyanosis. The compartment syndrome is worsening
the ischemia. The success of surgical treatment in this phase is doubtful.
In the last stage rigor mortis of the limb appears, the skin is intense cyanotic with
phlyctens and gangrene. The only solution in this stage is the amputation of the limb.
Determining the occlusion site is made clinically by:
The initial site of pain
The level where the pulse cannot be felt
Distribution and degree of circulatory disorders
Clinical picture must correlated with patients history, taking in account diseases
that may cause embolic or thrombotic acute ischemia.
In case of thrombotic occlusion, patients usually have prior history of
claudication, because acute thrombosis develops most often on pre-existing
atherosclerotic lesions.
Patients with embolic occlusion suffer, in many cases, from emboligene diseases.
The onset of symptoms is more brutal and clinical evolution is significantly more faster
when acute occlusion is of embolic origin.
The most useful examinations are Doppler ultrasound and arteriography.
Arteriography can provide important information about the site of arterial occlusion,
collateral circulation and status of the arteries in general.
A series of other examinations have to be performed for the underlying disease
and laboratory measurements for the patient's general condition.
Differential diagnosis is made with:
Acute venous thrombosis at onset accompanied by arterial spasm and pain and
phlegmasia cerulea dolens (a severe form of deep venous thrombosis which results from
extensive thrombotic occlusion of the main and collateral veins of an extremity which is
characterized by sudden severe pain, swelling, cyanosis and edema of the affected limb.
Foot gangrene may also occur. An underlying malignancy is found in 50% of cases.)
Arterial spasm due to poisoning (arsenic, ergotamine), arthritis or peripheral
neuropathy (sciatica) pain less violent, short duration associated with hyperhidrosis.
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Evolution
Ischemic injury causes myoglobin release from muscle into circulation, causing
acute renal failure by precipitation in the tubules. In late stages, marked by profound
irreversible cell injury, appear: hyperpotassemia, metabolic acidosis, myocardial
depression and other organs failure (MSOF) due to toxic products released from
ischemic necrotic area.
Treatment
Therapeutic measures must be taken very sun after the onset of ischemia. The
interval of time when desobstruction is considered useful is in the first 6-8 hours, with
an optimum in the first 4-6 hours.
The patient will be in absolute rest in bed, with the limb in a lower position (not
elevated) and without applying any heat or cold on the leg. All medication will be given
by vein. Medical treatment goals are:
Pain release with major painkillers (opioids)
Suppression of arterial spasm with vasodilators (Papaverine, Lydocaine,
Pentoxifylline)
Prevention of thrombosis extension with anticoagulants
Metabolic rebalancing and treatment of the underlying disease
Anticoagulant treatment is routinely established preoperatively in acute arterial
obstructions to prevent thrombosis extension and is continued postoperatively,
depending on the underlying pathology.
Current methods of treatment have improved prognosis by introducing surgical
desobstruction with Fogarty catheter and thrombolytic therapy with streptokinase.
If the desobstruction is successful, in postoperative period a tourniquet-shock like
syndrome (reperfusion syndrome) may appear threatening the life of the patient.
Patients presents hyperazotaemia, acidosis and hyperkalemia. After revascularization a
decrease of blood pressure may appear. Stasis and swelling are worsened because of
existing vasoplegia. Kalium brutally released into the blood stream may induce cardiac
arrest. Sometimes amputation is required between two dialysis sessions.
Surgical procedures
Direct or indirect embolectomy with Fogarty catheter
Thrombectomy followed by angioplasty
Bypasses or arterial grafting
Lumbar sympathectomy - in thrombosis of smaller vessels
Fasciotomy
Limb amputation if there are irreversible damages under protection of
dialysis.
Prognosis. The rate of amputations after embolectomy is up to 15% and the
postoperative mortality rate is up to 34%.
7. Chronic Peripheral Obstructive Arteriopathies
Regardless of the underlying anatomical changes, etiology and pathogenesis are
manifested by chronic peripheral ischemic syndrome.
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The disease is more common in men (6/1), over the age of 50, although it can
affect individuals under 40. All kind of arteries may be affected (large-caliber, middle
or small arteries). Lower limbs are more often affected than the upper.
Etiology. Determinant factors are:
Genetic genetic metabolic disorders that affect the arteries wall
Infectious - typhus, syphilis, typhoid fever - may cause arterial damage
Allergic - exogenous and endogenous antigens inducing antigen-antibody
reaction altering the endothelium.
Metabolic - hyperglycemia, hypercholesterolemia, hypertriglyceridemia
Endocrine pituitary gland, suprarenal gland, endocrine pancreas
Physical and mechanical: cold, humidity, repeated microtrauma
Nervous may induce vascular spasm
Chemicals: increased ratio of Ca / Na and lack of microelements (I, Br,
Mg) in food, favor arterial wall swelling and thrombosis
Toxic - nicotine, alcohol, arsenic, lead, chromium
The neighborhood venous thrombosis may cause inflammation of the
satellite artery
Predisposing factors are considered male gender; age over 40 years, sedentary
lifestyle, hyperglucidic and hyperlipidic diet.
Risk factors are represented especially by cigarette smoking. Seventy to 90% of
patients with arterial insufficiency are smokers. Risk remains increased for up to 5 years
after smoking cessation. Other risk factors include hyperlipidemia, diabetes mellitus,
obesity and hypertension.
Etiologic classification.
1. Atherosclerosis in 90% of cases.
2. Other causes:
a. Thrombangeitis obliterans (Burgers disease)
b. Temporal arteritis Horton
c. Raynoud phenomenon,
d. Arterial inflammation.
Atherosclerosis is a condition in which an artery wall thickens as a result of the
accumulation of cholesterol. It is promoted by low-density lipoproteins and caused by
the formation of multiple plaques within the arteries.
Atherosclerosis is one of the top causes of mortality in the world with 17 million
deaths per year. Of these, 20% are caused by ischemic heart disease. In Europe,
Romania ranks third, after Russia and Bulgaria, in terms of death rate caused by
cardiovascular disease. Atherosclerosis causes the narrowing of any vessels in the body.
Morphology. There is a hardening of the arteries and increased consistency with
rigidity. The lumen has an uneven caliber, with plaque buildup and thrombosis area.
Sclerosis may extend to the surrounding tissues.
Clinical picture. Peripheral arterial occlusive disease (PAD) is characterized by
intermittent claudication, consisting of exercise-induced lower extremity pain relieved
by rest. Claudication occurs when the blood supply is inadequate to meet the demand of
lower limb muscles, usually resulting from atherosclerotic arterial stenosis.
Depending on the intensity of this cardinal symptom, Leriche and Fontaine
quantify the severity of chronic arterial obstruction as follows:
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Stage I: the absence of any signs of ischemia, vascular obstruction is
diagnosed only by clinical or laboratory tests
Stage II: effort ischemia, intermittent claudication (IIa appears at more
than 200 m of walking, IIb - claudication at less than 200 m of walking);
Stage III: ischemic rest pain without trophic disorders;
Stage IV: ischemia at rest with trophic disorders.
A more recent classification by Rutherford consists of three grades and six
categories:
1. Mild claudication
2. Moderate claudication
3. Severe claudication
4. Ischemic pain at rest
5. Minor tissue loss
6. Major tissue loss
About 20% of patients may be asymptomatic. Other symptoms include: sores,
wounds, or ulcers that heal slowly or not at all; noticeable change in color (blueness or
paleness) or temperature (coolness) when compared to the other limb (termed unilateral
dependent rubor; when both limbs are affected this is termed bilateral dependent rubor)
and diminished hair and nail growth on affected limb and digits.
Clinical evaluation of claudication. Patients should be asked about the intensity of
claudication, its location, and the distance they have to walk before it begins.
Evaluation consists of determining the location, extent, and severity of disease
and the degree of functional impairment. The key clinical feature of claudication is the
reproducibility of muscular pain after a given level of activity and cessation of pain after
a period of rest.
Aortoiliac disease is manifest by discomfort in the buttock and/or thigh and may
result in impotence and reduced femoral pulses.
Leriche's syndrome occurs when impotence is associated with bilateral hip or
thigh claudication.
Iliofemoral occlusive disease is characterized by thigh and calf claudication.
Pulses are diminished from the groin to the foot.
Femoropopliteal disease usually causes calf pain. Patients have normal groin
pulses but diminished pulses distally.
Tibial vessel occlusive disease may lead to foot claudication, rest pain, non-
healing wounds, and gangrene. Rest pain consists of severe pain in the distal portion of
the foot due to ischemic neuritis. The pain is deep and unremitting, and it is exacerbated
by elevation of the foot, and the pain is relieved by dangling the affected foot over the
side of the bed.
Other clinical tests to assess chronic obstruction are:
Buerger test consists in lifting the inferior limb at 60-75 degree with the patient
in dorsal decubitus and performing movements of ankle joints and toes till the onset of
fatigue. If there is a deficiency in blood flow, the foot becomes intensely pale and the
leg dorsal veins are collapsed.
Moskowicz test consists in lifting the limb to vertical position, wrapping it with
elastic bands and remaining in that position for 5 minutes. After that, set the foot on the
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ground and loosen the bands. The column of blood will descend till the level of
obstruction, recoloring the foot except the affected area;
Coscescu test: on the affected limb perform scratches on the skin from top to
bottom. In the well irrigated area, the dermographism is positive, while in the less
irrigated area is negative and the skin is pale.
Investigations
The gold standard investigation of arteries is arteriography. The main
disadvantage of this investigation is that it is an invasive one but it offers the most
comprehensive information about arteries morphology. It is indicated mainly in patients
who are candidates for surgery.
A noninvasive procedure is the Ankle Brachial Pressure Index (ABPI). It may be
performed as a primary investigation but it has also drawbacks. (see above)
Doppler ultrasound investigation is very useful prior to angiography because it is
not invasive and can highlight the stenotic zone of artery. It has also some limitations.
CT scan angiography is useful especially to highlight the relations of the arteries
with the surrounding structures.
Etiological forms
Non-atherosclerotic peripheral chronic occlusion is represented mainly by
Takayasu's disease and Buergers disease.
Takayasu disease (a form of arteritis with giant cells) is a rare pathological
entity (most common in the Far East), which is found mainly in young women aged 20-
40 and mostly affects the aortic origin. To the aortic inflammatory lesions, stenosis and
aneurysms of the aorta and its branches are also associated.
Clinically there is unilateral or bilateral absence of pulse, signs of cerebral
ischemia, transient amaurosis, crisis of angina pectoralis, abdominal angina, renal
hypertension, etc..,
A particular form of giant-cell arteritis is represented by Hortons disease which
usually affects the cephalic arteries being most common in females. The main
symptoms are headaches, visual disturbances, weight loss, fatigue. Chewing causes pain
in the jaw. It is treated with anti-inflammatory (cortisone). Without treatment, patients
risk blindness
Buerger disease (thromangiitis obliterans) is a segmental inflammatory-
proliferative vasculopathy, usually with self-limited evolution, which occurs mainly in
young patients, male, smokers. It is characterized by the absence or minimal presence of
atheromas and involvement of small- and medium-sized arteries and veins of the upper
and lower extremities.
Cardinal clinical manifestation is pain unremitting ischemic ulcerations, and
gangrene of the fingers and toes and as the disease evolves, the patients may require
several surgical amputations. The treatment is rarely surgical, as there are distant and
diffuse arterial damages. Sometimes successfully react to surgical sympathectomy.

Differential diagnosis of the 2 leading causes of peripheral arterial chronic occlusion
Aterosclerosis Thrombangiitis Obliterans
Clinical
predominantly affects men who are
around the age of 60 years
mainly large and medium size
vessels are affected
predominantly affects young men
between 20-40 years, heavy smokers
small and medium sized arteries are
interested
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Angiographic
continuous lesions
irregular contour of the artery
asymmetrical lesions, more
advanced in the affected limb
well developed collateral circulation,
with separation of collaterals at right
angles from the main artery
segmental lesions
affected arterial segments have
smooth walls thin, filiform, with the
aspect of loss in the rain to the
ends
lesions frequently symmetrical
collateral network consists of thin
vessels that come off from the main
trunk in sharp angle
Another etiologic form of chronic peripheral occlusion of the small vessels is the
diabetic foot caused by diabetes mellitus. The clinical picture is that of ischemic lesions
of the leg going to wet gangrene. Ulceration and necrosis appear and are infected, the
infection spreading along the fascia and tendons causing cellulite. Usually patients have
diabetic neuropathy and so lesions may not be painful.
Treatment
A. Prevention: smoking cessation, rational nutrition (reduced lipids and
carbohydrate intake), avoidance of general infections, treatment of associated
morbidities (diabetes, hypertension, obesity), physical activity, prophylactic medication
with antiplatelet and hypolipemiant drugs if the persons are over 50 years old, with risk
factors.
B. Medical treatment
General measures: avoiding exposure to cold and wet, wearing
comfortable shoes and appropriate clothing, proper local hygiene.
Physiotherapy: medical gymnastics, carbonated baths, thermal cures.
Medication: administration of antiplatlet drugs (Aspirin) vasodilators,
anticoagulants, anti-atherosclerotic, pain relievers, etc..
C. Surgical treatment. Surgery is indicated in stage III-IV of the disease.
Functional operations: lumbar sympathectomy or thoracic
splanchnicectomy or adrenalectomy (especially useful in thrombangiitis),
and combinations of these. Because, in addition to atherosclerosis which
causes mechanical obstruction, in chronic peripheral ischemia, increased
sympathetic tone with vascular spasm is also involved (intricated
mechanism of atherogenesis), surgical sympathetic denervation is often an
adjuvant treatment with good result.
Reconstructive surgery:
o Angioplasty
Open - arterial patch application,
Endovascular procedures
Balloon angioplasty
Stenting angioplasty
Laser angioplasty
o Bypasses (with own saphena magna vein or prosthesis)
o Endarterectomy
o Segmental arterial resection (restoration of continuity with the
autograft or prosthesis)
Operations of necessity: amputations, necrectomies.
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583
Endarterectomy is a surgical procedure to remove the atheromatous plaque
material, or blockage, in the lining of an artery constricted by the buildup of
soft/hardening deposits. It is carried out by separating the plaque from the arterial wall.
Endovascular angioplasty uses a catheter and a balloon to open up blocked
arteries. The catheter is passed into a main artery, often in the groin, and pushed along
until it reaches the narrowed vessel. The balloon at the tip of the catheter is then inflated
so that it pushes any build-up of plaque in the artery against the vessel's wall allowing
a freer flow of blood. Angioplasty is less radical alternative to open or bypass surgery.
Stenting. The stent is inserted in the area where the artery is narrowed to keep it
open. Some stents are "coated" with medication to prevent the artery from forming clots
and stenosing again. Stents are used in most angioplasties except when an artery is too
small for a stent to fit.
Laser vaporization of atherosclerotic plaque, or laser angioplasty. A special
balloon angioplasty catheter is used which contains a fiberoptic channel through which
a laser is passed. The laser is used to vaporize the plaque and the balloon then stretches
the zone.
Sympathectomy increases peripheral blood flow by vasodilatation of arterioles in
cutaneous vascular beds. Some patients may receive sufficient increases to help heal
superficial ischemic ulcers and relieve rest pain. Usually two lumbar ganglia are
excised: L2 and L3 ganglionectomy usually sufficient. Because of abolishing basal and
reflex constriction of arterioles and precapillary sphincters, flow increases with 10-
200%. Also it produces a relief of ischemic rest pain due to loss of attenuation of
painful stimulus transmission but sympathectomy does not improve claudication.

Surgical Pathology of the Lower Limb Veins
SURGICAL PATHOLOGY OF THE LOWER LIMB VEINS

1. Anatomy
2. Lower limb venous circulation physiology
3. Varicose veins
4. Acute venous thrombosis
1. Anatomy
Veins are vessels, which collect blood from tissues and lead it toward the right
heart. Veins of the inferior limb may be classified in four types:
1. Superficial veins - under the skin
2. Deep veins - under the muscular fascia
3. Connecting veins - connect veins in the same fascial plane
4. Perforator veins - connect the superficial veins to the deep veins (crossing
fascial plane)
Superficial veins
The superficial venous system is a subcutaneous extremely variable weblike
network of interconnecting veins. A few larger superficial veins are fairly constant in
location.
The superficial venous system of the leg caries 20% of blood and the deep veins
80%.
Superficial veins do not accompany the arteries and they drain into the two main
superficial venous collectors: the greater (or internal) saphenous vein and the lesser (or
external) saphenous vein.
Digital veins of the leg flow into the dorsal venous arch of the foot. From the
medial end of the arch starts the internal saphenous vein and from the external side the
lesser (external) saphenous vein.
The internal saphenous vein starts in the front of the tibial malleolus, and then
ascends on the medial side of the calf and thigh towards the saphenous hiatus located
approximately 4 cm below the inguinal ligament in the groin and passes through this
hiatus of the fascia lata, to drain into the common femoral vein. The magna saphenous
vein receives two tributary veins below the knee in the posterior medial region:
the superficial anterior saphenous vein and
the posterior crural arch (described by Leonardo da Vinci) which sometimes
communicates with the lesser saphenous vein (saphena parva).
There are three relatively constant veins that drain into the greater saphenous vein
arch at level of saphenous hiatus, near the saphenofemoral junction:
1. The superficial inferior epigastric vein
2. The superficial external pudendal veins (two veins)
3. The superficial circumflex iliac vein
Other tributary veins are the anterolateral branch and posteromedial branch called
the vein of Giacomini.
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Many patients have a duplicated greater saphenous trunk in the thigh, which
parallel the main trunk and reconnect with it, either usually just above or below the
knee, or traverse more superficially in the distal thigh.
From surgical point of view, the most important variations occur at the
saphenofemoral junction in the groin. The anatomical arrangement of the individual
tributaries can be very different from one leg to another.
The external or lesser saphenous vein (vena saphena parva) starts from behind
the external (peroneal) malleolus and follows the straight ascending median axis of the
posterior aspect of the calf. Initially it is located superficially in the subcutaneous tissue
and then enters a splitting of the gastrocnemius fascia in the muscle. It flows into the
popliteal vein, in the popliteal space, between the two heads of the gastrocnemius
muscles.
In two-thirds of cases, it joins the popliteal vein above the knee joint, and in one-
third of cases, it joins with other veins (most often the greater saphenous vein or the
deep muscular veins of the thigh). In some patients, the vein may have two or three
different termination sites.
In only 50 to 70 % of the cases, the saphenopopliteal junction is located in the
popliteal fossa, whereas in about 10 % it is found below it.
The greater and lesser saphenous veins are interconnected by multiple
anastomoses. The greater anastomotic vein of Giacomini is one of the most important
which descends in scarf obliquely on the back of the thigh.
Deep veins
1. Posterior tibial vein carries blood from the posterior compartment of the leg and
joins the peroneal veins to form the tibioperoneal trunk
2. Peroneal vein carries blood from the lateral compartment of the leg and joins the
posterior tibial veins to form the tibioperoneal trunk
3. Tibioperoneal trunk is formed by the confluence of the peroneal veins and the
posterior tibial veins.
4. Anterior tibial vein carries blood from the anterior compartment of the leg and
joins the tibioperoneal trunk to form the popliteal vein.
5. Popliteal vein
6. Femoral vein a continuation of the popliteal vein that begins at the adductor
canal.
7. Deep femoral vein (profunda femoris vein) carries blood from the posterior
aspect of the thigh and joins the femoral vein to form the common femoral vein
8. Common femoral vein
9. External iliac vein is the continuation of the common femoral vein above the
inguinal ligament
Perforator veins
There are about 150 perforator veins but only a few have clinical importance.
They perforate the muscular aponeuroses throughout the leg linking the two systems
anatomically and hemodynamically.
A few named perforating veins are fairly constant in location and are named only
as vague groupings. The old nomenclature included:
1. Hunters perforators in the mid thigh
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2. Dodds perforators in the distal thigh
3. Boyds perforators at the knee
4. Cocketts perforators in the distal medial calf and ankle
Other perforators:
5. Bassi perforators, located posterior link the greater saphenous vein to
peroneal vein
6. Hach perforators, located posterior link the femoral vein with the superficial
system
7. Linton and Kosinski, located near the junction between the lesser saphenous
vein and popliteal vein
8. May perforators and gastrocnemius perforating group
The extrasaphenous dermal plexus has also a complex connection with the deep
venous system through the Delater perforators.
Perforator veins in the foot are avalvulated but in the calf, they have 2-3 valves
that prevent reflux of blood from the deep veins into the superficial system. One-way
valves lead the blood flow from the surface to depth. When valves are insufficient or
incontinent, the blood may flow abnormally from depth toward surface (reflux).
Perforator veins play an important role in the hemodynamic of the leg. They
regulate the blood flow between the two systems: deep (high pressure) and superficial
(low pressure) helping to maintain an efficient evacuation of blood from the leg.
Perforator veins insufficiency is caused by their enlargement and so valves
become insufficient or because of fragile valve leaflets destruction as a consequence of
thrombosis.
There is an intimate relation between the greater saphenous vein and the
saphenous nerve and also between the lesser saphenous vein and the sural nerve in the
lower leg which may result in nerve damage during operations for varicose veins.
2. Lower Limb Venous Circulation Physiology
The main functions of the venous system are: transportation of blood to the heart,
blood storage and thermoregulation.
The superficial collecting veins can dilate to accommodate large volumes of
blood with little increase in back pressure, so that the volume of blood sequestered
within the venous system can vary at any moment without interfering with the normal
function of the veins.
The correct functioning of the venous system depends on a complex system
formed by valves and muscles (which act like pumps =the "peripheral heart") that are
individually prone to malfunction or fail, yet the system as a whole performs
remarkably well under extremely adverse conditions.
Physiology of venous legs circulation is complex and varies greatly depending on
conditions such as: standing, lying, limb elevation. Motor factors that ensure the
circulation of venous return are:
Left ventricular propulsion force (vis-a-tergo)
Aspiration force of the heart and respiratory muscles (vis-a-fronta)
Leg muscle pump and especially calf muscles (peripheral heart)
Pulse transmitted by paravenous arteries
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Autonomous tone of the vein wall
Unidirectional venous valves that prevent backflow
The calf muscle pump
The passage of blood upward from the feet against gravity depends on a complex
array of valves and pumps. Squeezing of the vein segment occurs when muscle
contraction increases the pressure within a fascial muscle compartment. With normal
cycles of contraction and relaxation, the veins are alternately compressed and
decompressed ("pumped").
Inflow to a segment of deep vein is through intake valves from perforating veins
as well as from the deep vein segment below. Outflow is through an outflow valve to
the deep vein segment above.
Opposing factors of the venous circulation are:
Gravity
Blood viscosity
Abdominal pressure
The imbalance between the two categories of factors leads to venous stasis in the
legs and consecutively varicose veins developing and trophic modifications of the skin.
3. Varicose Veins
The condition represents a chronic disease of the lower limb venous system
characterized by:
Alterations of the venous walls and valvular apparatus
Reflux of blood from the deep venous system towards the superficial
Dilated superficial veins
Pathophysiology - Hydrostatic varices
In orthostatic position, blood stasis induces a higher pressure in the lower limb
veins. This will lead in the first stage to a slightly dilatation of the deep veins sufficient
to dilate the junctions sites between the superficial and deep venous system.
At junction level between the femoral vein and saphenous veins (ostium
saphenae), there are one-way valves. When ostium enlarges, the valve becomes
incompetent allowing the backflow into the superficial veins. The reflux will induce a
higher pressure into the superficial veins leading to their dilatation. A vicious circle
occurs. The superficial veins valves become insufficient as they cannot close completely
and so the blood column cannot be fragmented (by valves) further increasing the
hydrostatic pressure into the vein. This will lead to further dilatation of veins, which
become also elongated, and tortuous (varices). The high hydrostatic pressure will force
the perforator veins, especially in the calf, where the pressure is higher. The perforator
veins will expand leading to their valves incompetence and reflux from the deep to the
surface system at this level too. Intravenous high pressure will determine lesions of the
endothelium, which associated with stasis, and turbulent flow will favor the occurrence
of thrombosis.
The stasis is worsening especially in the lower third of the calf. As venous
(deoxygenated) blood stagnates here too much time, the tissues become less oxygenated
and trophic changes of the skin and subcutaneous tissues appear culminating with leg
venous ulcers.
Morphopathology
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Varicose veins are elongated, tortuous, dilated veins, in most cases located along
the trajectory of the saphenous veins and/or their tributaries. Venous valves are
atrophied, with zones of verrucous endo-phlebitis, parietal sclerosis and connective
tissue dysplasia, as evidenced microscopically. These changes are circumscribed by a
zone of sclerosis of the subcutaneous tissue, lymphatics and adjacent skin.
Etiology. Many factors have been incriminated:
Endogenous factors:
Anthropological factor - bipedal position
Anatomo-physiological factor - the superficial venous network less
valvulated and uninfluenced by muscle contractions
Genetic factor - deficiency of collagen and elastin fibers in the venous
wall (frequently associated with hemorrhoids and flatfoot)
Constitutional type
Sex - most common in women
Age - most frequently between 20 and 40
Endocrine factor - disorders of pituitary, ovarian, thyroid, adrenal gland
and endocrine pancreas function
Pregnancy
Obesity
Exogenous factors
Profession, prolonged standing or sitting, heat, humidity, dietary factors,
infections.
Classification
In terms of etiopathogenesis, varicose veins can be classified into:
1. Congenital varicose veins
Klippel-Trenaunay syndrome - characterized by the triad of varicose
veins, hypertrophic elongation of the limb and tuberous haemangioma
Parkes-Weber syndrome - congenital arteriovenous fistulas
Venous angioma - tumors containing excess of venous structures
2. Primitive varicose veins - the cause is unknown but predisposing factors are
present
3. Secondary varicose veins - the etiology is known
Post-thrombotic syndrome
Compression on the main venous trunks
External and internal venous trauma
Arteriovenous fistulas
The CEAP classification of venous disease is widely recognized, and was
developed in 1994 by an international ad hoc committee of the American Venous
Forum. The severity scoring system was based on three elements: number of anatomic
segments affected, grading of symptoms and signs, and disability.
CEAP means: Clinical severity, Etiology or cause, Anatomy, Pathophysiology
For the initial assessment of a patient, the clinical severity is the most important
and can be made by simple observation. Classification starts with the patients initial
visit, but can be better defined after further investigations. A final classification may not
be complete until after surgery and histopathologic assessment.
There are three levels of testing, depending on the severity of the disease:
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1. Level I: office visit, with history and clinical examination, which may
include use of a hand-held Doppler scanner.
2. Level II: noninvasive vascular laboratory testing, which now routinely
includes duplex color scanning, with some plethysmographic method added
as desired.
3. Level III: invasive investigations or more complex imaging studies,
including ascending and descending venography, venous pressure
measurements, computed tomography (CT), venous helical scanning, or
magnetic resonance imaging (MRI).
Grades of increasing clinical severity:
C0 - No visible or palpable signs of venous disease.
C1 - Telangiectasies or reticular veins.
C2 - Varicose veins; distinguished from reticular veins by a diameter of 3 mm or more.
C3 - Edema.
C4 - Changes in skin and subcutaneous tissue secondary to chronic venous dfisease,
o C4a - Pigmentation or eczema.
o C4b - Lipodermatosclerosis or atrophie blanche.
C5 - Healed venous ulcer.
C6 - Active venous ulcer.
S: symptomatic, including ache, pain, tightness, skin irritation, heaviness, and muscle
cramps, and other complaints attributable to venous dysfunction
A: asymptomatic
Terminology
Atrophie blanche (white atrophy) =localized, often circular, whitish and atrophic
skin areas surrounded by dilated capillaries and sometimes hyperpigmentation -
not to be confused with healed ulcer scars.
Corona phlebectatica =fan-shaped pattern of numerous small intradermal veins on
medial or lateral aspects of ankle and foot. Synonyms include malleolar flare and
ankle flare.
Eczema =erythematous dermatitis, which may progress to blistering, weeping, or
scaling eruption of skin of leg. Most often located near varicose veins, but may be
located anywhere in the leg.
Edema =perceptible increase in volume of fluid in skin and subcutaneous tissue,
characteristically indented with pressure. Venous edema usually occurs in ankle
region, but may extend to leg and foot
Lipodermatosclerosis = localized chronic inflammation and fibrosis of skin and
subcutaneous tissues of lower leg. It must be differentiated from lymphangitis,
erysipelas, or cellulitis by their characteristically different local signs and
systemic features.
Pigmentation = brownish darkening of skin, resulting from extravasated blood.
Usually occurs in ankle region, but may extend to leg and foot.
Reticular vein =dilated bluish subdermal vein, usually 1 mm to less than 3 mm in
diameter. Usually tortuous. Excludes normal visible veins in persons with thin,
transparent skin. Synonyms include blue veins, subdermal varices, and
venulectasies.
Telangiectasia =confluence of dilated intradermal venules less than 1 mm in caliber.
Synonyms include spider veins, hyphen webs, and thread veins.
Varicose vein =subcutaneous dilated vein 3 mm in diameter or larger, measured in
upright position. May involve saphenous veins, saphenous tributaries, or
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nonsaphenous superficial leg veins. Varicose veins are usually tortuous, but
tubular saphenous veins with demonstrated reflux may be classified as varicose
veins. Synonyms include varix, varices, and varicosities.
Venous ulcer =full-thickness defect of skin, most frequently in ankle region, that
fails to heal spontaneously.
Etiologic classification
Ec - congenital
Ep - primary
Es - secondary (postthrombotic)
En - no venous cause identified
Anatomic classification
As - superficial veins
Ap - perforator veins
Ad - deep veins
An - no venous location identified
Pathophysiologic classification
Pr - reflux
Po - obstruction
Pr,o - reflux and obstruction
Pn - no venous pathophysiology identifiable
Examples of classification:
Classification according to basic CEAP: C6,S, Ep,As,p,d, Pr.
Classification according to advanced CEAP: C2,3,4b,6,S, Ep,As,p,d,
Pr2,3,18,13,14 (2004-05-17, L II).
Diagnosis. Venous disease diagnosis requires a careful history, a proper clinical
examination and laboratory investigations.
History of the patient may offer important data regarding the etiology and risk
factors.
Suggestive genetic aspects are family history of varicose veins, of
thrombophlebitis, arterial or lymphatic disease.
Investigate the personal physiological antecedents for women regarding the
number of pregnancies and evolution. The personal pathological antecedents such as
thrombophlebitis, drugs (anticoagulants, diuretics, birth control pills), nicotine intake,
diabetes, hyperlipidaemia, arteriopathies, dehydration, varicose veins surgery, fractures,
immobilization diseases at risk of thrombosis with stasis, are suggestive. Important are
also heart, kidney or liver diseases for differential diagnosis of edema.
Professions requiring prolonged standing and/or effort and those with exposure to
heat (teachers, workers, trade, cooks, builders, athletes, volleyball players, weightlifters)
are accompanied by varicose veins and their complications.
Patient age, height, weight.
Details about the disease evolution: onset (acute, chronic, insidious), initial
symptoms and their evolution.
Clinical examination starts with inspection of the legs is standing and lying
position. The differences of thickness (by swelling) or length (congenital diseases
present) of the legs is appreciated. Other findings such as edema, dilated veins, corona
phlebectatica, skin pigmentation and trophic disorders (hair loss, leg ulcer) are noticed.
590
o Sicards test. The patient is in supine position and asked to cough. Suddenly a
bulge appears in the groin at level of greater saphenous vein arch. It
demonstrates the ostial insufficiency at saphenous-femoral junction.
By palpation, dilated veins and the presence of thrombosis are estimated.
Assessment of dilated veins in obese patients is more difficult, on both inspection and
palpation because they are hidden in the thigh fat.
o Schwartzs sign. In supine position, percussion of the greater saphenous vein
above the internal malleolus will produce a wave that can be felt by fingers
placed in the groin portion of the saphena magna. It demonstrates that
semilunar valves are incompetent allowing the transmission of wave through a
continuous column of blood.
On auscultation systolic-diastolic sounds are present only in secondary varicose
veins due to arteriovenous fistulas.
Clinical functional tests are still useful in assessing lower limb veins, although
they lost importance due to the new non-invasive explorations.
o Brodie-Trendelenburg-Troianov test is used to determine the site of valvular
incompetence. The patient is lied down. The leg is elevated and so veins are
emptied. A tourniquet is applied in the upper thigh. Ask the patient to stand. If
the tourniquet prevents the veins from re-filling rapidly (under 30 seconds),
the site of the incompetent valve must be at the sapheno-femoral junction. If
the veins re-fill faster with the tourniquet in place, the incompetence must be
lower down. (Mahorner Ochsner test 3 tourniquets)
Proceed with the same protocol down the leg:
above the knee - to assess the mid-thigh perforator
below the knee - to assess competence between the short saphenous vein
and popliteal vein
If re-filling cannot be controlled, the communication is probably by one or more
distal perforating veins.
Clinical tests for the deep venous system
o Perthes test. The patient is in supine position. The calf is bandaged with
elastic bands so that only the superficial veins to be collapsed and not the
deep, and then he walks 10 to 15 min. In case of deep veins occlusion, pain in
the calf will appear in this period of time.
o Delbet - Macquot test. A tourniquet is applied above the knee, making sure
the it does not interfere with the deep venous circulation. The patient is
invited to walk, watching the changes of varicose veins. If varicose veins
reduce it means that there is an insufficiency of internal saphenous vein, but
the perforators veins are competent. The persistence of varicose veins without
modification denotes perforator veins incompetence. If varicose veins
increase, it means that there is a deep venous system insufficiency.
o Linton test: The tourniquet is applied below the knee. Patient in supine
position with the leg elevated will present a fast draining of varices when deep
venous system is permeable.
o Pratt test highlights the areas of reflux in the communicating veins. Patients is
in supine and a tourniquet is applied on the upper part of the thigh to eliminate
the possibility of reflux from femuro-saphenous junction. A bandage with
elastic bands is also applied running from the ankle to the groin. In standing
591
position as the bandage is removed from thigh towards the ankle, there is a
refill of varicose veins, when perforator veins are incompetent.
Symptoms
During the onset, usually there are no symptoms. As the varicose veins develop,
patient complains of pain in the leg, tension, muscle cramping and burning. Pain is
worsened after sitting or standing for a long time. After prolonged standing, the patient
notices the edema that usually disappears after rest with the leg elevated. In advanced
stages, trophic skin disorders appear.
Acute complications:
Varicoflebitis (thromboflebitis of the superficial veins) represents the
inflammation and thrombosis of varicose veins that become swollen, painful
with redness of skin and fever.
Bleeding from varicose veins (spontaneous or posttraumatic) or from venous
ulcer.
Chronic complications are represented by chronic venous insufficiency with all
its consequences: eczema, loss of hair, edema, liposclerosis, pigmentation, and ulcer.
Investigations
Ultrasound and Doppler ultrasound are the most useful and noninvasive
investigations. These investigations highlight the dilated veins and the reflux sites,
assessing both the superficial and deep venous system.
Phlebography is rarely indicated for diagnosis of varicose veins. It is useful in
cases of deep venous system thrombosis or if other pathology is suspected.
Differential diagnosis is made between etiopathological types of varicosities. In
advanced stages of chronic insufficiency, the differential diagnosis is more difficult and
should include the following conditions:
Swelling of the legs of different etiologies
Dermatitis of legs
Dermatologic etiology of leg ulcers
Ischemic ulcer
Mal perforans ulcer
Erythema Induratum (Nodular Vasculitis) Bazin
Ulcers in hemolytic disease
Post-thrombotic ulceration
The treatment is prophylactic and curative.
Prophylactic treatment aims to eliminate factors incriminated in the occurrence of
varicose veins and prevent their further development:
Avoid prolonged sitting and standing
Wearing compression stockings
Exercise daily
Elevate legs when possible, keeping them positioned higher than heart
level
Maintain an ideal body weight
Avoid excessive heat
Curative treatment includes the following methods:
1. Physiotherapy
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2. Medication with phlebotropic drugs
3. Sclerotherapy
4. Minimally invasive procedures (endovenous ablation)
5. Surgery
For the varicose veins
For perforator veins
For venous ulcers
Sclerotherapy can bed applied for small veins only (reticular veins). A sclerosant
(irritant for the endothelium) solution is injected into the varicose veins or spider veins
in order to cause their disappearance. The most frequent used substance is Polidocanol
(Aethoxysklerol) (Table 1) which causes localized destruction of the intima layer and
fibrosis of the vein, occluding the lumen of the vessel, and reducing its appearance.
Sclerotherapy is an outpatient procedure and is performed in several sessions,
which are carried out at 4-8 week intervals. The solution must be injected strictly
intravenously otherwise, it may cause perivenous tissue necrosis and skin pigmentation.
Another condition for success is that the vein must be completely emptied of blood
before injecting the sclerosant agent. Patients are told to walk immediately after each
treatment session and graduated compression must be applied. Class II (30-40mmHg)
compression hose or elastic stocking are most commonly used for this purpose.
Possible complications of sclerotherapy are:
Hyperpigmentation - this is a common occurrence after sclerotherapy of
veins of all sizes in approximately 10-80% of patients
Swelling
Telangiectatic matting or "postsclerotherapy neovascularization. Matting is
the name given to networks of fine red blood vessels, which develop near the
site(s) of previous injections.
Other complications (pain, localized urticaria, folliculitis, localized
hirsutism, skin necrosis, systemic allergic reactions , superficial
thrombophlebitis, deep venous thrombosis, nerve damage)
Table 1 - Suggested sclerosant/concentrations for treatment of telangiectasia/reticular veins

VESSEL TYPE SCLEROSANT CONCENTRATION
Hypertonic saline 11.7%
Sodium tetradecol sulfate STS 0.2%
Telangiectasia
<1mm
Polidocanol (Aethoxysklerol) 0.25%
Sodium tetradecol sulfate STS 0.25%
Hypertonic glucose/saline
(Sclerodex)
The formula for Hypertonic
glucose/saline is 200mg/ml dextrose
100mg/ml sodium chloride 100mg/ml
propylene glycol 8mg/ml phenoxyalcohol
Venulectasia
1-2mm
Polidocanol (Aethoxysklerol) 0.5%
Hypertonic glucose/saline
Sodium tetradecyl sulfate STS 0.25%
Reticular veins
>2mm
Polidocanol 0.5-1.0%
Laser therapy for superficial small veins (spider veins) works by sending
strong bursts of light onto the vein, through the skin that makes the vein slowly fade and
disappear. No incisions or needles are used.
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Laser therapy by minimal invasive procedure is catheter-assisted procedures
(endovenous procedures). In these procedures, a thin catheter is introduced
percutaneously into the vein and advanced as necessary. In most cases, the procedure is
performed under ultrasound guidance. As the catheter is pulled out, the heat provided by
laser or ultrasound destroys the vein by causing it to collapse and seal shut. This
procedure is usually done for larger varicose veins. It can be performed in local
anesthesia on an outpatient.
Surgical treatment has three main objectives:
1. To suppress the ostial reflux at junction level between deep and superficial
system
2. To suppress reflux through the perforator veins (highlighted by ultrasound)
3. To remove the varicose veins and to heal the lesions (ulcers)
There are two major types of surgery:
1. Addressed directly to veins
2. Addressed to complications (bleeding, ulcers)
There are many types of procedures, which are applied considering the degree of
varicose veins development. The traditional technique is that of subcutaneous stripping
of the greater saphenous vein - the Babcock procedure.
Babcock procedure starts with a skin incision of 2-4 cm at groin level where the
greater saphenous vein flows into the femoral vein. The greater saphenous vein is
discovered and isolated. Then all tributaries of the saphenous arch (external pudendal,
circumflex iliac, inferior epigastric, anterior accessory veins) are ligated and resected.
The saphena vein is ligated and resected just above the junction with femoral vein. Then
Giacomini vein is ligated and resected. The next step is a small incision above and
anterior to the internal malleolus where the origin of saphena vein is discovered. The
vein is isolated and separated from the saphenous nerve, then resected. A stripper is
introduced into the upper end of the saphena and advanced upstream till the saphenous
arch in the groin. The tip of the stripper is ligated to the vein and the vein is tripped out
in an anterograde or retrograde way. Retrograde stripping is more recommended
because of less saphenous nerve damage. During avulsion of the greater saphenous vein,
collaterals and perforator veins are broken (interrupted).
If the stripper cannot progress until the saphenous arch, extra skin incisions have
to be performed and the saphenous vein is stripped out in two or more segments. Other
clusters of varices are removed through separate incisions. If necessary, the lesser
saphenous vein arch is also resected and the vein removed.
Possible complication after stripping are:
Soreness and bruising (subcutaneous bleeding and hematoma formation)
Numbness due to nerves lesions
Suppuration
Chronic leg swelling from damaged lymphatic tissue
Incision scars
Terrier-Alglave procedure: removes the saphenous arch and the entire greater
saphenous vein through a continuous linear incision from the groin until the malleolus.
The very long incision is time consuming and may result in an unaesthetic scar. The
main indication remains the case of greater saphenous vein thrombosis when stripping is
impossible.
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Operations addressed to perforator veins. In advanced stages of venous
insufficiency with trophic lesions of the skin and ulcers, interrupting the blood reflux
through the perforator veins is indicated for healing of skin lesions. There are two main
very similar operations: the Felders and Lintons technique. In both, a longitudinal
incision is performed in the posterior or posterolateral aspect of the calf sectioning the
skin and the fascia. The perforator veins are intercepted under the fascial plane, ligated
and resected. Then the fascia and skin are sutured.
New less invasive techniques have been developed in recent years. One of these is
the subfascial endoscopic perforator vein surgery. For endoscopic approach, two
ports (10 mm and 5 mm diameter) are placed in the subfascial space of the calf remote
from the area of venous ulceration. A dissector balloon is introduced and inflated to
improve access. Carbon dioxide is then insufflated to facilitate dissection. The
incompetent perforating veins are clipped and divided using endoscopic scissors or
alternatively, coagulated and divided using an ultrasonic coagulator (harmonic scalpel).
Deep venous occlusion and/or infected ulcers are usually contraindications to
subfascial perforator veins surgery
Treatment of venous ulcers
There are surgical and non-surgical treatments.
Conservative treatments use different types of ointments with epithelization
effect. Ulcers must be kept free of infection absorbing any excess discharge,
maintaining a moist wound environment and the edema must be controlled. The patient
will wear compression garments, and physical activity will be encouraged and elevated
position of the leg during rest. Conservative treatment of venous ulcers can be
frustrating and lengthy and recurrences are frequent.
Surgery provides faster healing of ulcers. The primary condition is to ensure a
normal local metabolism of the skin. Causes of venous stasis that produces hypo-
oxygenation and edema must be first resolved (saphenectomy and ligation of perforator
veins). In many cases, eliminating causes of venous stasis will result in ulcer healing
with conservative methods.
For large ulcers there are many surgical methods but the most used are those of
plastic surgery using skin grafts. Unfortunately, skin grafts have a high rate of failure.
Topical therapy with growth factors (platelet-derived growth factor and
epidermal growth factor) is also an alternative but more expensive.
4. Acute Venous Thrombosis (AVT)
AVT is represented by blood clotting inside the veins with an acute onset.
Terms:
Venous thrombosis represents the blood clotting inside the veins without signs of
acute inflammation.
Thrombophlebitis means thrombosis + symptoms and signs of acute
inflammation.
Venous thromboembolism represents the migration of the thrombus from deep
veins along the blood stream causing obstruction of pulmonary vessels and
in many cases the death of the patient.
Superficial venous thrombosis is the thrombosis that affects only the superficial
veins or varices (varicophlebitis).
595
Deep venous thrombosis appears when deep veins (of the calf or thigh) are
affected. It the most dangerous and the cause of thromboembolism.
Etiopathology. Three factors are involved in intravascular blood clotting (the
Virchows triad):
1. Stasis/turbulence
2. Endothelial lesions
3. Hypercoagulability
Causes that determine those three factors are:
Venous endothelial injury
o As a result of prolonged compression of the vein or after trauma
o Prolonged immobilization of the legs on a solid surface
o Intravenous injections or catheter
o Hypoxia due to stasis
o Endothelial infection
Venous stasis and turbulence
o Prolonged standing
o Prolonged immobilization after surgery or trauma
o Any increase in abdominal pressure situations: pregnancy,
abdominal tumors, ascites, obesity
o Irregular dilated veins (varices) with turbulent flow
Hypercoagulability
o A postoperative or posttraumatic decrease of antithrombin III
o Congenital deficiency of antithrombin III
o Increased of inhibitor factor of plasminogen activation
o Still unexplained circumstances related to duration of surgery,
obesity, advanced age, sepsis
o Polycytemia and thrombocytosis
o Malignant tumors (Trousseau syndrome) - procoagulants factors
synthesized by the tumor cells and peritumoral inflammatory
infiltrate
Factors associated with an increased risk of venous thrombosis are:
Age >40 years, male gender, obesity and cancer
A history of thrombosis or pulmonary embolism
Operations (orthopedic, neurosurgical, urological, any operation lasting
more than 2 hours)
Pregnancy
Use of oral contraceptives
Nephrotic syndrome
Dysfibrinogenemia
Hereditary deficiency or abnormal synthesis of protein C, protein S,
antithrombin III, plasminogen
A. Superficial thrombophlebitis of the lower limbs -
Varicophlebitis
Most superficial thrombophlebitis of the legs occurs in the varicose veins due to
venous stasis and blood circulation disorders with turbulent flow. In addition, local
trauma may be another cause.
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In most cases, the clinical manifestation is that of an acute thrombophlebitis with
symptoms and local signs of inflammation. The onset is sudden with pain along the
involved vein. The greater saphenous vein or other superficial veins and varices are
affected in variable lengths.
Examination of the lover limb reveals the Celsian local signs: swelling over the
affected veins and redness of the skin. On palpation, the skin is warm, and the
thrombosed vein feels like a painful hard cord under the skin.
The differential diagnosis should include lymphangitis and cellulitis.
Superficial thrombophlebitis rarely is a life threatening condition, but a thorough
investigation of the patient is mandatory because of occult deep vein thrombosis that
may be associated and carries high rates of morbidity and mortality.
The goal of treatment of superficial thrombophlebitis is to stop the spread of the
thrombotic process and to reduce the local inflammatory reactions.
Conservative treatment consists of:
Anticoagulants (heparin and then long term oral anticoagulants)
Antiplatelet drugs (Dipyridamole, Aspirin, etc)
Local ointments with heparin
Non-steroidal anti-inflammatory drugs, such as: ibuprofen, aspirin,
acetaminophen, naproxen sodium etc.
Antibiotics
Wearing compression bandages or stockings
After resolution of inflammatory phenomena, in most cases, an organized
superficial vein thrombosis remains that can be the site from where bacteria are
discharged into the bloodstream or phlebitis may relapse. In few cases, the
inflammatory process evolves to abscess formation. These are the reasons why,
thrombosed varicose veins should be surgically removed.
Surgical treatment is addressed not only to the complication of varicose veins, the
superficial thrombosis, but also to the underlying disease: the varicose veins disease
itself. The thrombosed vein is removed, if necessary with the overlying skin through an
incision along the vein taking care not to open the vein as the thrombus is considered to
be infected.
B. Deep venous thrombosis (DVT)
It is a very dangerous condition because it may endanger the patient's life by
possible evolution to pulmonary embolism. The vast majority of cases of deep vein
thrombosis and pulmonary embolism are found in patients receiving surgery.
The annual worldwide incidence of DVT of the leg is one case per 1000
population.
The etiopathogenesis of deep thrombophlebitis in surgical patient involves many
factors of which the most important is the venous stasis due to immobilization. In
addition to this, tissue factors resulting from surgical trauma are released into the blood
stream contributing to increased platelets adhesion and blood coagulation.
Obese patients, those with cancer disease, those with venous insufficiency of the
lover limbs (varicose veins), and those operated for bone fractures and pelvic pathology
are at the highest risk of developing postoperative deep venous thrombosis.
Based on these facts, all patients are encouraged to postoperative early
ambulation (to avoid stasis) and they are treated with anticoagulants as needed.
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Most commonly, thrombosis begins in the veins of the calf and pelvis. The
thrombosis process extends from distal (peripheral) to central veins (popliteal, femoral,
and iliac). Occluded veins induce a high retrograde intravenous blood pressure
manifested by edema of which extension depends on thrombosis extension.
Steps of thrombosis process are:
1. White thrombus formation (platelets are the main components) - platelet
adhesion and activation and then aggregation to form the platelet plug.
2. The red thrombus (mixed thrombus) - a fibrin gel network that includes
red cells and white cells, which fuse with the platelet plug.
3. Retraction of the thrombus
4. Organized thrombus - the fibrinolysis process dissolves the clot.
After fibrinolysis the thrombus may dissolve completely or partially resulting the
total or partial (like a sieve) recanalization of the vein. In this last situation, the blood
flow is slowed with peripheral stasis. After prolonged standing, edema and cyanosis of
the affected lower limb occur.
The major risk for embolism is on the second and third phase (between days 2-12
after surgery) and the most in the shrinking (retraction) phase of the thrombus.
Clinical picture
General signs are:
Michaelis sign - gradual increase of temperature to 38
0
C in the absence of
an obvious cause
Mahlers "climbing pulse rate" - the scale of pulse in contrast to that of
temperature
Unexplained anxiety and restlessness
Local signs:
Swelling of entire leg or calf only. There is an increased diameter of the
calf (and thigh sometimes) compared to the opposite inferior limb. More
then 2 cm difference, measured at the same level, between the two legs is
suggestive for deep venous thrombosis.
The skin is stretched, glossy with high local temperature and slightly
cyanotic.
On palpation, tenderness of the calf is found and high consistency of
muscular tissue due to edema.
Other signs:
Pratts sign =dilated pretibial vein which remains dilated even when the
leg is elevated (sign of deep femoral vein thrombosis)
Payr sign =pain at palpation of the median muscular region of the sole
Bisgaard sign =retromalleolar pain
Tschmarke sign =pain on calf compression
Ducuing sign =pain at shaking the calf musculature
Homans sign =pain in the calf induced by passive dorsiflexion of the leg
Lowenberg sign =calf pain produced by compression with blood pressure
cuff at higher values than 100 mmHg.
Meyer sign =tender points in calf over the affected vein
Clinical forms by location:
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Thrombosis of the calfs veins is manifested by increased consistency of the
calf but not excessive edema
Thrombosis of the popliteal and femoral vein - there is a calf edema
extended till the knee
Thrombosis of the ilio-femoral vein - there is a white edema of the calf and
thigh called phlegmatia alba dolens
Very extended ilio-femoral thrombosis may result in phlegmatia cerulea
dolens - a very painful edema of the entire inferior limb. There is an
associated spasm of the arteries with cyanotic cold skins, weak femoral pulse
and alteration of general status of the patient. Foot gangrene can occur.
Thrombosis extended to the hypogastric veins is manifested by pain in the
lower abdominal quadrant, lumbar pain dysuria, acute urinary retention,
rectal and genital pain.
Thrombosis of the inferior cava vein will result in edema of the inferior
limbs, low arterial blood pressure and shock.
Thrombosis may occur also simultaneously in both lower limbs. Other less
frequent possible sites of thrombosis are upper limbs, portal and splenic vein.
Unfortunately, there are many cases without any symptoms of acute inflammation
(just thrombosis and not thrombophlebitis) or just with slight symptoms as moderate
calf pain, which evolve directly to pulmonary embolism and death, before any measure
can be taken.
Diagnosis of acute phase is based on clinical picture and other investigation of
which the Doppler ultrasound is the most useful and noninvasive.
Evolution under treatment goes to remission of symptoms. In convalescence, the
edema disappears in supine but reappears in standing position. During stabilization
phase, symptoms disappear. The last phase is that of sequelae with postthrombotic
syndrome and different kinds of possible complications.
Complications:
Pulmonary embolism
Venous gangrene
Post-thrombotic syndrome
Chronic venous insufficiency (edema, cellulitis, leg ulcers)
C. Pulmonary Embolism
It is the most dangerous complication. The thrombus starts from the deep leg
veins and carried by the blood stream to the heart. If the clot is large enough, it will
occlude the pulmonary artery resulting in sudden death. If the clot is small or there are
many fragments of clots, these will pass into the smaller pulmonary arteries occluding
them and leading to pulmonary infarction.
Symptoms
In massive thromboembolism, the onset is acute with anxiety, violent retrostrernal
pain, cyanosis, dyspnea, tachycardia and death in few minutes or evolution to
cardiogenic shock.
In less massive embolism, the thrombi reach the lungs where they occlude some
arteries leading to pulmonary infarction manifested at 24-48 hours by: chest pain,
cough, hemoptysis, and dyspnea. Fever up to 38
0
C degree is present for 2-3 days.
599
Thoracic radiography and CT scan reveal a wedge-shape pulmonary condensation.
Pulmonary infarction may also develop serohematic pleural effusion, which persists 2-3
weeks.
In case of small and repeated pulmonary thromboembolism, there are short
episodes of dyspnea, tachycardia, arrhythmia, cough and hemoptysis.
Physical signs and their incidence:
Tachypnea (respiratory rate >16/min) - 96%
Pulmonary rales - 58%
Accentuated second heart sound - 53%
Tachycardia (heart rate >100/min) - 44%
Fever (temperature >37.8C) - 43%
Diaphoresis - 36%
S3 or S4 gallop - 34%
Clinical signs and symptoms suggesting thrombophlebitis - 32%
Lower extremity edema - 24%
Cardiac murmur - 23%
Cyanosis - 19%
Approximately 10% of patients who develop pulmonary embolism die within the
first hour and approximately one third of patients who survive an initial pulmonary
embolism, die from a subsequent embolic episode.
Late complication of pulmonary thromboembolism is chronic pulmonary
hypertension.
D. Post-Thrombotic Syndrome
Evolution of venous thrombus may be:
To complete resolution - this is a rare possibility
Recanalization through a single canal but with valves destruction
Recanalization through multiple canals as a sieve
Permeabilization through collateral avalvular veins
Fibrous organization as a hard cord
There are five types of post-thrombotic syndrome:
1. Obstructive - there is a persistent obstruction of the venous axis
2. Substitution - the blood flow finds other ways to avoid the occluded vein
3. Restrictive - veins loose their elasticity and the capacity of stoking blood
4. With reflux - due to valves lesions, hydrostatic pressure rises and reflux
towards superficial veins appears
5. Combinations of the above types
As a consequence of difficult blood flow, venous stasis and high hydrostatic
pressure will develop downstream the affected veins. This will lead to edema initially
reducible and then irreducible, trophic alterations of the skin, pigmentation, cellulitis
and ulcer. Venous ulcer develops more rapidly (1-2 years) then after varicose veins (10-
20 years).
Treatment of DVT
Untreated DVT will lead to embolism in 50% of cases and to death in 10-38%.
The risk lowers in treated patients to 5-20% for embolism and <8% for mortality.
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The most important treatment is prophylaxis. Preventive measures must be
applied to all surgical patients especially to those obese, with varicose veins, those with
cancer, operated for bone fractures or for pelvic organs pathology:
Applying compression stockings or elastic bandages on the legs prior to
operation and wearing it after.
Using of pneumatic socks (sequential compression device) during
operation and in the first days after in obese patients
Early ambulation
Using anticoagulant drugs (heparin or fractionated heparin such as
Clexane, Fraxiparine, Fragmin, etc.)
Antiplatelet drugs - Dextrane, Aspirin, etc.
Treatment of the acute phase
Once the deep vein thrombosis is diagnosed, the patient will be put at rest in bed
for 7-10 days with the leg elevated at 20-25 degrees. Walking is not permitted to
prevent thrombus mobilization and embolism.
Anticoagulant treatment will be started with intravenous administration of
unfractionated heparin 5000 iu and then 500-1300 U/h for 7-10 days. Oral anticoagulant
tablets will be given simultaneously for three days and then continued 3-6 month just
with tablets in doses that keep the value of plasmatic thrombin time 2-3 folds higher
than normal values, or the INR in the range of 2.0 to 3.0. Heparin may be discontinued
when the international normalized ratio (INR) is stable and greater than 2.0.
Anticoagulant treatment does not directly restore venous patency or vascular
function, it just prevent thrombus developing. On the other hand, even in patients who
are fully anticoagulated, however, DVT and pulmonary embolism can and often do
recur.
In addition, anti-inflammatory and antibiotics will be associated.
Walking will be permitted after the resume of inflammatory phenomena and
edema.
Thrombolysis (dissolves the clots) is reserved for extensive forms (phlegmasia
cerulea dolens) of DVT and iliofemoral thrombosis.
Selecting patients for thrombolytic treatment:
Patients with an expected long-term survival
Massive DVT or phlegmasia cerulea dolens
Iliofemoral DVT
Multiple segment DVT
Patients who remain symptomatic despite anticoagulation
Recent onset DVT (<10 days)
No previous DVT
Younger patient, few co-morbidities
Contraindications for thrombolysis are:
Active bleeding
Cerebrovascular accident (within 2 months)
Major surgery (within 10 days)
Pregnancy or recent delivery
Metastatic cancer to brain or spinal cord
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There are two types of thrombolysis: systemic and direct catheter guided.
Systemic thrombolysis is more expensive and less accurate. Catheter guided uses less
quantities, has less adverse effects and a higher rate of success. Catheter thrombolysis is
performed under imaging guidance, the fibrin-specific thrombolytic agent being
delivered directly to the clot through a catheter inserted in the vein.
Ultrasound (US)-accelerated thrombolysis, involves simultaneous delivery of low
intensity US and thrombolytic agent into a thrombosed vessel, and so accelerates the
thrombolysis and reduces the time of infusion and risk of bleeding.
Thrombolytic agents can be classified in two categories:
1. Fibrin-specific agents (alteplase, reteplase, and tenecteplase), which
produce limited plasminogen conversion in the absence of fibrin.
2. Nonfibrin-specific agents such as streptokinase.
Streptokinase (the first used agent in 1933) is extracted from beta-hemolytic
streptococci; it is the cheapest but also the most antigenic and it has many adverse
reactions (fever, hypotension, etc.). The usual dose regimen is an IV bolus of 250,000 U
followed by a maintenance drip at 100,000 U/h. The drip is continued for 1-3 days, until
clinical or laboratory investigation shows thrombus resolution.
Urokinase (1947), unlike streptokinase, is not antigenic and directly activates
plasminogen to form plasmin. It is produced by renal parenchymal cells. It is indicated
in case of massive pulmonary embolism. For systemic treatment, the dose is 4,400 U/kg
Urokinase as an IV bolus, followed by a maintenance drip of 4,400 U/kg/h. The drip is
continued for 1-3 days, until clinical or laboratory investigations demonstrate thrombus
resolution. For intrathrombus delivery, the dose is a loading dose of 250,000 U IV,
followed by an infusion of 500 U/kg/h. If clot lysis is inadequate, the infusion rate can
be increased gradually up to 2,000 U/kg/h.
Alteplase (the first generation) is the first recombinant tissue-type plasminogen
activator. It is fibrin specific effective only at the surface of fibrin clot. It is not
antigenic. It is used with catheter-directed infusion of 1-1.5 mg/h for 12-24 hours.
Reteplase (the second generation) works more rapidly and has a lower bleeding
risk. It is also not antigenic. Catheter-directed infusion of 1 U/h is maintained for 18-36
hours.
Tenecteplase (TNKase) (the third generation) is produced by recombinant DNA
technology. It has the advantage for a single bolus administration and decreased
bleeding side effects.
Venous thrombectomy is applied in rare cases, especially in iliac vein thrombosis
using the Fogarty catheter.
In case of pulmonary embolism, the first who tempted the surgical remove of the
clot from the pulmonary artery was Trendelemburg but the first who reported a success
was Kirschner in 1919. The method implies thoracotomy and extracorporeal circulation
and it has a high burden of mortality.
Every patient with an episode of pulmonary embolism should receive oral
anticoagulant therapy for at least 3 months.
In patients with high risk of recurrent thromboembolism (and for those whom the
anticoagulant therapy is contraindicated) inferior vena cava filter (Greenfield filter)
may be useful.
Treatment in chronic phase (pos-thrombotic syndrome) consists of:
Elevation of extremity at rest and at night
602
603
Compression stockings grade 2 should be worn during the day, while
standing but not needed to be worn at night
Weight loss and behavior modifications
Increased exercise with strengthening of extremity muscles
Pain management
Compression pump
Vascular interventional radiology procedure: balloon opening and stenting
of narrowed vein

Indications for Splenectomy
INDICATIONS FOR SPLENECTOMY

Splenectomy is the most widely used technique in surgery of the spleen.
Splenectomy means total ablation of splenic tissue, namely the spleen and any
accessory spleen. Segmental (partial) splenectomy means the removal of one or more
segments of the spleen.
Anatomy. Size of spleen is about 12 x 7 x 3 cm. The average weight is 150 g, (80 to
300 g). It has a dark purplish color being a soft organ of very friable consistence, highly
vascular, which makes it very difficult to sewn. Also because of its friability, the spleen
breaks easily during direct or indirect abdominal or left thoracic trauma.
The spleen is located in the deep left hypochondrium. It lies between the fundus
of the stomach and the diaphragm. Its projection is between the left ribs 9 and 11.
It has two surfaces (convex and visceral concave) and two borders. The anterior
border has a few clefts. The posterior border is more rounded and blunter than the
anterior.
The concave surface comes into direct contact with: the stomach, the pancreas,
the left kidney and the splenic flexure of the colon.
The spleen is covered by peritoneum, which is firmly adherent to its capsule. It is
held in position by two peritoneal folds: the phrenicolienal ligament and the gastrolienal
ligament. The lower pole of the spleen is supported by the phrenicocolic ligament. The
most cases of iatrogenic lesions of the spleen are due to this ligament (phrenicocolic),
which frequently adheres to the inferior pole of the spleen. Traction exerted on this
ligament during operations like left hemicolectomy or gastrectomy, results in avulsion
of the spleen capsule with consecutive bleeding.
Accessory spleens are frequently present in the neighborhood of the spleen in the
gastrolienal ligament and greater omentum. They are small nodules of splenic tissue of
variable size from that of a pea to that of a plum.
Vascularization. The arterial of the spleen supply comes from the lienal artery, a
branch of the celiac trunk from the aorta. The lienal artery divides in the hilum of the
spleen in several branches and provides a segmental vascularization. The venous blood
is collected by the lienal (splenic) vein, which joins with the superior mesenteric vein to
form the portal vein. The inferior mesenteric vein flows into the splenic vein.
Lymphatic vessels have their origin in the white pulp forming a network around
vessels and flowing into the lymph nodes located in the hilum of the spleen.
Because the very close relations between the tail of the pancreas and the hilum of
the spleen, injury of the tail of the pancreas is possible during splenectomy (when
ligating and resecting the vessels) and that may result in an iatrogenic pancreatitis.
The spleen is coated by a thin capsule, which in the hilum is reflected inward
upon the vessels in the form of sheaths.
The spleen is composed of two pulps: the red pulp and the white pulp. The white
pulp is lymphoid tissue that usually surrounds splenic blood vessels. The red pulp is a
network of channels (sinuses) filled with blood.
Spleen has a major hematopoietic function until the fifth intrauterine month of
life. The most important function of the spleen is the mechanical filtration that removes
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senescent red blood cells and control of infection. Abnormal blood components will be
trapped in the spleen and ingested by splenic phagocytes.
The spleen is a major site of production for the opsonins, properdin and tuftsin.
Opsonin are molecules that target an antigen for an immune response. Properdin is a
globulin protein that acts like a pathway of complement activation important for
destruction of bacteria, foreign and abnormal cells. It also helps to neutralize some
viruses. Tuftsin binds to specific receptors on the surface of macrophages and
polymorphonuclear leukocytes, stimulating their migration, phagocytic, bactericidal,
and tumoricidal activity. It also influences antibody formation.
In non-traumatic cases, the indication for splenectomy arises from an
interdisciplinary collaboration: surgeon, internist, hematologist, gastroenterologist,
parasitologist, pediatrician, etc.
Considering the important role of the spleen in immunity, the indication of
splenectomy must be very well documented to avoid situations that can lead to
unwanted complications or worsening of diseases.
A. Splenectomy for spleen abnormalities
Abnormalities of position (spleen "displaced" in other sites than splenic lodge)
may be congenital ("ectopic") or acquired and are often accompanied by abnormal
splenic mobility which can cause twisting or volvulation around the elongated pedicle
with secondary splenic infarction or compression on neighboring viscera.
Abnormalities of number: the presence of supernumerary spleens (accessory
spleen) may require splenectomy in context of hypersplenism.
Abnormalities of shape: splenectomy is indicated in case of some complications
(infarction, spontaneous or traumatic rupture, compression on the neighborhood
organs).
B. Splenectomy for vascular abnormalities
Splenic infarction represented by single or multiple lesions secondary to
emboligenic cardiovascular disease or other diseases (hematologic, inflammatory,
septic, vasculopathies,) clinically silent or manifest by complications such as
splenomegaly accompanied by pathological phenomena, splenic rupture with/without
intraperitoneal bleeding, infection with abscesses.
Splenic artery aneurysm (the second most frequent location after the aorta) -
considering the risk of rupture with vital risk, large aneurysms should be surgically
treated immediately after the discovery of the aneurysm by splenectomy (in distal
location) or aneurysm excision with restoration and preservation of spleen artery (in
troncular location).
Arteriovenous splenic fistula, which may cause a portal hypertension syndrome.
In case of intrasplenic arteriovenous large shunt, splenectomy is also indicated.
Spontaneous rupture of splenic vessels a rare clinical condition with acute
onset of intraperitoneal hemorrhage.
Volvulus of the spleen around its vascular pedicle, rarely reversible
spontaneously, with mixed infarction (arterial and venous) of the spleen, with symptoms
of acute abdomen requiring emergency laparotomy with splenectomy.
Splenectomy in portal hypertension is indicated in the following conditions:
As single gesture:
605
o In case of segmental portal hypertension with thrombosis of the
splenic vein
o In hypertension associated with splenomegaly and hypersplenism (in
the absence of esophageal varices, upper gastrointestinal bleeding
and ascites)
o In case of portal hypertension due to arteriovenous fistula
As associated procedure complementary to different types of portal
hypertension surgery: distal spleno-renal bypass, azygoportal deconnection
Sugiura-Futagawa or Hassab operation.
Completing a previous intervention (such as a non-selective porto-caval
shunt without splenectomy leaving the possibility of further development of
splenomegaly accompanied by mechanical consequences and
hypersplenism).
C. Parasitic splenopathies
Splenic echinococcosis (primitive or secondary): splenectomy is the only
resonable therapeutic solution.
Malaria manifested by intermittent febrile accesses, associated with
splenomegaly and anemia (hypersplenism predominantly on red-cells line). The
splenomegaly develops in two phases (acute, congestive, reversible and chronic phase
sclerous irreversible). The treatment is complex, medical and surgical, splenectomy
being indicated to control and prevent complications such as mechanical, vascular,
infectious and hematologic.
Visceral leishmaniasis - manifested with fever, anemia and splenomegaly. The
treatment is medication and eventually surgical (splenectomy is indicated if there are
risks of complications and to eliminate an important reservoir of parasites).
Schistosomiasis (bilharziasis, Egyptian splenomegaly) manifested by portal
hypertension with splenomegaly and hypersplenism. Splenectomy is a step of eso-
gastric devascularization during Hassab operation.
D. Septic and viral splenopathies
Splenic abscess (single or multiple) of variable etiology: microbial (digestive
bacteria) or parasitic (Entamoeba histolytica, Plasmodium) by hematogenous, from
neighborhood or direct inoculation. Diagnosis is based on clinical picture, lab
investigations and imaging. In most cases splenectomy is the optimal therapeutic
approach.
Other infectious splenopathies: typhoid fever, endocarditis, tuberculosis,
infectious mononucleosis, can benefit from splenectomy during a complex treatment.
E. Splenic tumors
The main reasons for establishing the indication for splenectomy are symptoms
and the risk or complications (tumor size plays an important role in risk especially for
cystic hemangiomas).
F. Splenopathies during hematologic diseases
Benign hematological conditions, which benefit from splenectomy, are:
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Thalassemia - inherited autosomal recessive blood disorder which results in
reduced rate of hemoglobin synthesis or formation of abnormal hemoglobin molecules,
thus causing anemia. Severe associated splenomegaly is an indication for splenectomy.
Sickle-cell anemia or drepanocytosis, is an autosomal recessive genetic blood
disorder characterized by abnormal, rigid, sickle shape red blood cells. Sickle-cell
disease may lead to various acute and chronic complications, several of which have a
high mortality rate. Indications for splenectomy in patients with sickle cell disease
include: acute splenic sequestration crisis (rapid enlargement of spleen, anemia),
hypersplenism, splenic abscess.
Hereditary spherocytosis, is an autosomal dominant disease that results from a
deficiency of spectrin, a red blood cell cytoskeletal protein. This defect causes
membrane abnormality in the red blood cells that are small, spherical, and rigid with
increased osmotic fragility. It is manifested by hemolytic anemia, occasionally jaundice,
and splenomegaly. Splenectomy decreases the rate of hemolysis and usually leads to
resolution of the anemia. It is usually performed in childhood shortly after diagnosis but
is delayed until after the 4th year of life to preserve immunologic function of the spleen
in young children.
Aplastic anemia is a condition where bone marrow does not produce sufficient
new cells (red blood cells, white blood cells, and platelets) termed pancytopenia.
Splenectomy helps some patients with severe thrombocytopenia.
Immune thrombocytopenic purpura (idiopathic thrombocytopenic purpura,
Werlhof disease) is characterized by low platelet count, a normal bone marrow, and the
absence of other causes of thrombocytopenia. There is an increased platelet destruction
due to autoantibodies to platelet membrane. Indication for splenectomy are:
refractory severe thrombocytopenia
toxic doses of steroids
relapse after initial steroids
>6 weeks of steroids and continue to have a platelet <10,000/mm 3
>3 months with incomplete response to primary therapy platelet <30,000/mm 3
2nd trimester of pregnancy with medical refractory platelet <10,000/mm 3 or who
have platelet <30,000/mm 3 and bleeding problems.
The success rate (complete and permanent response) is about 65%.
The cause of failure to respond to splenectomy or relapse after an initial response,
in many cases, is represented by accessory spleen which also must be surgical excised.
Thrombotic thrombocytopenic purpura (TTP, Moschcowitz disease) is a rare
disorder of the blood-coagulation system, causing extensive microscopic clots in the
small blood vessels. It is manifested by neurologic symptoms (alteration in mental
status, seizures, hemiplegia, paresthesias, visual disturbance, and aphasia), fever, dark
urine, pallor, jaundice, and petechiae due to anemia and thrombocytopenia. The exact
etiology of TTP is not clear. The therapy of choice is plasma exchange with fresh frozen
plasma and corticosteroids. Splenectomy is indicated in refractory cases of TTP.
Storage diseases (Gaucher disease , Niemann-Pick disease, etc.) manifested
mainly by hepatomegaly and splenomegaly with hypersplenism.
Haematological malignancies in which splenectomy may be associated in a
complex treatment: chronic lymphocytic leukemia (CLL), hairy cell leukemia,
malignant lymphoma (Hodgkin or non-Hodgkin), myeloproliferative syndromes,
(chronic myeloid leukemia (CML), myelofibrosis with myeloid metaplasia, etc..).
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G. Splenectomy for spleen trauma
Splenectomy is the choice in severe and multiple lesions of the spleen with
hemodynamic instability. Indications for splenectomy:
Parenchyma explosion
Vascular lesions in the hilum
Intra-parenchymal massive hematoma
Patients in critical condition or other severe intra-abdominal injuries
Failure of conservative surgical techniques
Other classification for splenectomy indications: (according Angelescu et al.)
A. Absolute indications
In emergency (Vital)
o Splenic trauma
o Splenic abscess with septic syndrome
o Ruptured arteriovenous aneurysms
o Splenic infarction (torsion of the opedicle, embolism)
o Intestinal obstruction (compression, colic volvulus)
o Splenic vein thrombosis with ruptured gastric varices
Elective
o Hereditary spherocytosis
o Immune thrombocytopenic purpura
o Splenic hydatid cyst
o Benign splenic tumors
o Primary malignant tumors
B. Relative indications
o Autoimmune hemolytic anemia
o Other congenital hemolytic anemia
o Thrombotic thrombocytopenic purpura
o Splenic neutropenia
o Splenic pancytopenia
o Felty syndrome
o Myeloid metaplasia
o Chronic myeloid leukemia
o Chronic lymphocytic leukemia
o Hairy cell leukemia
o Gaucher disease
o Nieman-Pick disease
o Cirrhosis with portal hypertension and hypersplenism
o Splenic tuberculosis
o Splenic syphilis
o Malaria
o Uncomplicated aneurysms
o Splenic metastatic tumors
o Splenectomy for diagnosis
o Undiagnosed splenic tumors
o Hodgkin's lymphoma (stage I, IIB)
o non Hodgkin lymphoma
o Tactical splenectomy
o Pancreatectomy (total or left)
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o Total gastrectomy
o Spleno-renal shunt
o Esophagoplasty
o Left hemicolectomy
o Left nephrectomy
H. Indications for partial splenectomy
Partial splenectomy (segmentectomy, ablation of a splenic pole,
hemisplenectomy, subtotal splenectomy) is considered for preservation of immune
functions of the spleen.
1. For hemostasis partial spleen trauma
2. For localized spleen lesions
Cysts (pseudocyst, hydatid cyst)
Benign solid tumors
Partial infarctions
Localised abscess
3. For reduction of the spleen volume
Gaucher disease (in children)
Severe hypersplenism (in children)
Myelofibrosis
Schizostomiasis
4. For diagnosis purpose
Hodgkin's lymphoma
Unspecified tumors
Isolated splenomegaly
Contraindications for splenectomy
There are cases when splenectomy may lead to negative effects in the absence of
any beneficial therapeutic outcome for the patient:
1. Limited spleen infarction, uncomplicated and asymptomatic
2. Portal hypertension: splenectomy without any other procedures lowering
the portal pressure is contraindicated (spleen serves as a buffer organ in
portal system)
3. Splenomegaly in systemic sepsis
4. Congestive splenomegaly in malaria
5. Hereditary hemolytic anemia of low or medium grade
6. Acute leukemias (splenomegaly appears in the stage of generalization of
malignancy)
7. Non-Hodgkin systemic lymphoma (advanced evolutionary stage)
8. Splenomegaly in polycythemia vera
9. Essential hemorrhagic thrombocythaemia
10. Spleen sarcomas in generalized sarcomatosis stage (stage III).
11. Spleen metastatic tumors
Surgical technique of splenectomy.
Splenectomy can be performed via laparotomy (open approach) or by
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In certain cases splenectomy can be also perform via a left thoracotomy through
the left diaphragm. This last approach is reserved especially for polytrauma or stab
wounds when there are intrathoracic lesions (more life threatening) associated with
diaphragmatic and spleen lesions but with no other intra-abdominal lesions.
Splenectomy can be easily performed through this approach and is followed by
diaphragmatic repair and thoracic drainage.
Laparotomy can be a left subcostal approach or a median laparotomy. The
subcostal approach ensure a better and direct access to the spleen but it has also some
disadvantages: it does not afford a thorough exploration of the entire abdominal cavity
and is followed by paralytic incisional hernia due to intercostal nerves lesions.
The subcostal approach is performed especially in cases where the spleen is the
single abdominal organ affected established by investigations.
Median laparotomy is performed especially in emergency cases when a thorough
exploration of the abdominal cavity is necessary such as abdominal trauma (blunt or
penetrating) with hemoperitoneum when other organs may be involved. This approach
offers the best view over the all organs and can be split in any direction.
There are two strategies to remove the spleen:
Anterior approach - starting from the spleen hilum gastro-colic and gastro-
splenic (short gastric vessels) ligament are divided, the tail of the pancreas and the
hilum of the spleen are exposed, splenic vessels are ligated and resected, and spleen is
extracted after resection of perisplenic adhesions and ligaments.
Posterior approach starts from the posterior aspect of the spleen with the
incision of the spleno-parietal reflection of the peritoneum; the spleen and the tail of the
pancreas are mobilized to the surface and vessels are legated and resected.
Laparoscopic approach is a minimal invasive procedure, which requires a
laparoscopic unit, special instruments and a skilled surgeon. It has advantages over the
open procedure. It can be performed through either a lateral or an anterior approach.
The steps are almost the same as in open procedure but the spleen must be removed
using a special bag in which the spleen is first chopped (fragmented).
Hand-assisted laparoscopic surgery refers to a laparoscopic approach performed
with the aid of one surgeons hand introduced into the abdomen through a plastic device
inserted in a 7.5 to 10 cm wound. By this procedure, the splenectomy is much easier and
also the extraction but the minimal invasive principle is violated.
Robotic splenectomy - The main advantages are a better tridimensional view and
an increased versatility of the surgical instruments.
Contraindications for laparoscopic approach
Absolute
o Contraindications of general anesthesia (severe cardiopulmonary
disease and other co-morbid conditions making laparoscopic or
even open splenectomy impossible to perform.)
Relative
o Uncontrolled coagulopathy
o Advanced myeloproliferative syndromes
o Spleen larger than 20 cm
o Aneurysms of splenic pedicle
o Cirrhosis with portal hypertension
o Pregnancy
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Possible complications after splenectomy
Immediate complications
o Bleeding from the spleen bed or other sources
o Spleen lodge hematoma and abscess
o Postoperative infections
o Fever
o Acute pancreatitis
o Thromboembolic complications
o Left pleural effusions
Late complications
o Minor infections
o Fulminant life-threatening systemic sepsis
o Hernia at the incision site
Given the important role of the spleen in immunity, whenever possible, surgeons
try to preserve a normal spleen or to perform limited resections. This became possible
with the development of hemostatic materials (TachoSil and others) or special
instruments (harmonic scalpel, LigaSure, laser, argon plasma, etc.) which ensure a
proper hemostasis.

Thoracic Trauma
THORACIC TRAUMA

1. Considerations about anatomy and physiology of the chest
2. Chest trauma - generalities
3. Blunt thoracic trauma
a. Simple contusions
b. Muscular ruptures
c. Chest compression
d. Simple rib fractures
e. Sternal fractures
f. Flail chest
4. Open chest trauma
a. Pneumothorax
b. Open pneumothorax with traumatopnea
c. Tension pneumothorax
d. Hemothorax
e. Cardiac tamponade
5. Lesions of the intrathoracic organs
a. Lungs lesions
b. Tracheo-bronchial tree lesions
c. Cardiac lesions
d. Aortic injury
e. Esophageal rupture
f. Diaphragmatic rupture

1. Considerations about Anatomy and Physiology of the
Chest
Thorax is the part of the body between the neck and abdomen. It consists of the
rib cage, an osteocartilaginous structure (which houses the vital organs like lungs, heart
and large vessels) and soft tissues (muscles, fascia, tendons, and skin). On the front of
the chest, mammary glands are located.
The osteocartilaginous elements are represented by twelve thoracic vertebrae,
sternum and ribs with costal cartilages. Between ribs, there are 11 intercostal spaces,
which are occupied by the intercostal muscles and intercostal vessels and nerves. The
role of this cage is to protect vital organs and to confer the rigidity necessary for
ventilation.
During trauma, any of these anatomical structures can suffer injuries. The less
likely are the vertebrae, which are protected by the paravertebral muscles. The most
fragile are the ribs and cartilages, structures that break most often. Types of bone lesions
and their location depend much on the nature, strength and direction of the traumatic
force.
Bone fractures have two important consequences: broken edges may injure the
nearby anatomic structures (intercostal vessels, pleura, lung, and heart) and alter the
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most important function of the thorax, namely the respiratory function, endangering the
patient's life.
The thorax cage is like a rigid box, feature that is very important in respiration.
Altering the rigidity of the cage especially after ribs fractures will alter the respiratory
function also.
The thoracic cavity is separated from the abdominal cavity by the diaphragm,
which is the most important respiratory muscle. It acts like a piston. During its
contraction, it lowers decreasing the intra-thoracic pressure allowing the inspiration.
During relaxation, the diaphragm moves upward leading to a higher pressure in the
thoracic cavity, inducing expiration.
Intercostal muscles are the other respiratory muscles, which by contraction
elevate the ribs and increase the thoracic diameter and the depth of inspiration.
Accessory muscles of breathing are considered: the sternocleidomastoid, scalene,
serratus, pectoralis, trapezius, latissimus dorsi, and others. If a breathing disorder exists,
the accessory muscles of inspiration may become overused.
Expiration is a passive action based on elastic recoil of the lungs. In forced
expiration as well in certain condition when lung elasticity is affected the abdominal
muscles and the internal intercostal muscles help expel air.
Another important aspect is the fact that between the two pleural layers (parietal
and pulmonary) there is a virtual space with negative pressure (lower than the
atmospheric pressure). This explains why traumatic lesions of these layers, and beneath
anatomical structures, will be followed by rapid accumulation of fluids or gas (or both)
into the pleural space which will lead to lung collapse and possible mediastinum
dislocation. All these will impair more or less rapidly or dramatically the respiratory
and cardiac function.
Often there is no direct relationship between the extent of injury and the
physiopathological disorders.
There are many cases when minimal gestures such as thoracocentesis, with fluid
or gas evacuation, can save the patients life.
2. Chest Trauma - Generalities
Epidemiology
Traumas represent a principal cause of deaths in peacetime but especially in
wartime. Chest and head trauma are the most dangerous. Chest traumas represent about
a quarter of traumas in general, and greatly contributes (9-10%) to the general mortality
of about 25%-50%.
In most cases, chest injuries are part of polytrauma.
Nowadays the most common injuries are caused by road traffic accidents.
Thoracic trauma is estimated to be responsible for approximately 16,000 deaths per year
in the United States. Estimates of thoracic trauma frequency indicate that injuries occur
in 12 persons per million populations per day. Approximately 33% of these injuries
require hospital admission.
Early deaths due to thoracic trauma, which occur within 30 minutes to 3 hours
after the injury, are secondary to cardiac injury with/without tamponade, great vessels
injury, airway obstruction and aspiration.
Two thirds of these patients reach the hospital prior to die. Only 10-15% of blunt
traumas require thoracic surgery, and 15-30% of the penetrating chest traumas require
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Thoracic Trauma
open thoracotomy. About eighty percent of patients with thoracic trauma can be
managed by simple lifesaving maneuvers that do not require surgical treatment.
Optimal treatment requires a through knowledge of the pathophysiology of the
thorax and expertise the therapeutic interventions.
Improved prehospital care and rapid transportation have increased the survival,
but the mortality remains high.
Classifications
A. Blunt trauma
Blunt traumas are closed thoracic trauma (there is no solution of continuity on the
skin). Blunt chest trauma may affect any component of the chest wall and thoracic
cavity (bony skeleton, lungs and pleurae, tracheobronchial tree, esophagus, heart, great
vessels of the chest, and the diaphragm).
Kinetic forces act in different ways or mechanism:
1. Blast the pressure wave can produce:
Tissue disruption
Vascular lesions
Disruption of alveolar tissue
Disruption of tracheobronchial tree
Traumatic diaphragm rupture
Direct impact by a blunt object
A hard object that hit the thorax can produce bone fractures, especially ribs and
through the fractured edges, lesions of the nearby anatomical structures (pleura,
intercostal vessels, lungs, etc).
2. Crush - compression
The thorax is compressed between two hard surfaces. Direct injury of chest wall
and internal structures occurs. It causes a marked increase in blood pressure within the
veins of the upper thorax and may result in traumatic asphyxia. Anterior-posterior
compression forces place indirect pressure on the ribs, causing lateral, mid-shaft
fractures. Lateral compression forces applied to the shoulder are common causes of
sternoclavicular joint dislocation and clavicle fractures.
3. Deceleration
The body in motion strikes a fixed object. For example during frontal collision in
car crashes (the sternum hits the steering wheel), or a fall from height. A blunt trauma
to chest wall is produced, but after the contact with the hard surface the internal
structures continue in their motion being crushed to the internal chest wall and also
anatomical structures of fixation will be broken or even organs will be broken.
The degree of external trauma may not fully predict the severity of internal
injuries and clinical suspicion of cardiac and vascular trauma should be heightened.
Consequences of closed chest trauma are highly dependent on many factors. The
first is the force intensity. Then the direction and site of action is also important. It
should also be considered if injury occurred during inspiration or expiration. Finally yet
important should be considered patients age and existing co-morbidities. The younger
thorax is more flexible and better resist to deformation while in elderly fractures occur
more easily.
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Thoracic Trauma
B. Penetrating Trauma
There is a skin solution of continuity. Depth of penetration may be limited only
to the soft tissues of the chest wall but penetration maybe deeper affecting the pleural
cavity and internal organs. When the traumatic agent penetrates the whole thorax, being
present an opening for entry and one of exit, we talk about transfixing trauma.
Low energy forces (arrows, knives, handguns) cause injury by direct contact and
cavitation.
High energy forces (military guns, high-powered hunting rifles) produce
extensive cavitation injury due to high pressure. Tissue destruction is much higher due
to bone fragments driven by traumatic agent.
Penetrating wounds consequences depend primarily on penetration depth and the
affected organs. If mediastinal organs as the heart or great vessels are affected, the
chances of quickly death are very high. Affecting other organs (pleura, lungs,
esophagus) are also life threatening but there is an interval of time when investigations
could be performed and rescue measures can be taken.
Classification according to pathophysiological criteria:
Without pathophysiological disorders
With pathophysiological disorders:
o Acute respiratory insufficiency
o Acute cardiocirculatory insufficiency
o Acute cardiorespiratory insufficiency
o With temporary stop of cardiorespiratory function
Classification according to pathogenesis
Closed chest trauma (blunt trauma)
Open chest trauma (wounds)
o Blind or transfixiant wounds
o Non-penetrating or penetrating wounds
o With or without effusions
o Mixed
Classification based on anatomical criteria
Without anatomical lesions
With anatomical lesions:
o Parietal non-skeletal lesions
o Parietal skeletal lesions
o Diaphragmatic
o Endothoracic lesions
Single organ affected, multiple organ affected
Associated with other trauma

Table 1 Classification of thoracic trauma lesions
Parietal lesions Ribs fractures
Sternum fractures
Flail chest (free-floating segment of the chest wall)
Pleural space Simple pneumothorax
Open pneumothorax
Tension pneumothorax
Hemothorax
Pulmonary parenchyma Contusions
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Thoracic Trauma
Lacerations
Trachea and bronchi
Heart and great vessels
Mediastinum
Esophagus
Lesions of these organs
Diaphragm Diaphragmatic lesions
Potential physiological consequences in thoracic trauma:
1. Hypoxia
2. Hypercapnia
3. Hypovolemic shock
4. Obstructive" shock
5. Acidosis
The 6 types of rapidly fatal chest injuries (found on primary examination):
1. Airway obstruction
2. Suffocating pneumothorax
3. Open pneumothorax
4. Massive hemothorax
5. Flail chest - free-floating segment of the chest wall
6. Cardiac tamponade
The 6 types of potentially lethal chest trauma (found at secondary
examination):
1. Rupture of the aorta (dissection)
2. Myocardial contusion
3. Tracheobronchial rupture
4. Rupture (perforation) of esophagus
5. Pulmonary contusion
6. Diaphragmatic rupture (hernia)
The 8 types of thoracic injury free of fatal potential (identified at secondary
examination):
1. Simple pneumothorax or reduced hemothorax
2. Sternoclavicular joint dislocations
3. Sternal fracture
4. Clavicle fracture
5. Scapular fracture
6. Traumatic asphyxia
7. Simple rib fractures
8. Chest wall contusion
Some aspects of pathophysiology
Chest trauma may affect vital functions: ventilation and circulation by several
mechanisms manifested by the following syndromes:
1. Compression syndrome (compression exerted on intrathoracic organs by
pleural or pericardial effusions)
Air: Pneumothorax
Fluid: blood =Hemothorax, lymphatic fluid =Chylothorax
Mixed Fluidopneumothorax
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Thoracic Trauma
2. Chest wall instability - when the chest wall looses its rigidity as a
consequence of multiple ribs fractures in two or more places (free-floating segment of
the chest wall) the ventilatory dynamics is deeply affected, resulting in acute respiratory
failure.
The following four disturbances of respiratory dynamics may appear:
1. Paradoxical respiration the floating area of the chest wall moves in
during inhalation and out during exhalation causing poor ventilation of the
lungs, oxygen depletion and severe and even fatal cardiovascular
disturbances.
2. Pendular motion of the mediastinum - still further hampers the heart and
great vessels and reduces the already impaired oxygenating power of the
lungs.
3. The "pendulum air it appears when injury crushes only one side of the
chest. On inhalation, the air is pulled out of the flailing side and exhalation
pushes the healthy side's stale air back into the flailing lung. The
paradoxical air "pendulum" only switches stale air from one lung to the
other.
4. Bronchial hypersecretion
There are two important vicious circles:
1. COURNAND - hypoxia pulmonary hypertension alveolar
hypersecretion hypoxia
2. POISVERT - paradoxical respiration hypoxia hyperventilation
increased paradoxical respiration.
When several ribs are broken on the both sides and chest loses its rigidity and
becomes soft (flail chest), the situation is more critical because the patient cannot
breathe. In this case the only solution is mechanical ventilation by orotracheal
intubation .
3. Obstructive syndrome - accumulation of fluids in tracheo-bronchial tree will
occlude the lumen impairing ventilation, gas exchanges and promoting infection.
Causes:
Bronchial hypersecretion
Bleeding into the tracheobronchial tree
Pulmonary hypertension
Aspiration in the airway of saliva or gastric contents by vomiting
Shock lung (wet lung) - insidious onset of rapid superficial breathing,
dyspnea, and productive cough; rales and wheezes; refractory cyanosis. Xray
appearance of enlarging interstitial and alveolar infiltrates, which extend
until the entire lung is enveloped in a diffuse haze.
4. Fluid, electrolytes and acid-base imbalance: Loosing electrolytes can be
caused by bleeding, sweating, tachypnea. Acidosis has a respiratory component and a
metabolic component. Initially respiratory acidosis is followed by metabolic acidosis.
5. Diaphragmatic syndrome:
Phrenic nerve injury will lead to paralysis of the diaphragm and so it will not
participate in respiratory movements.
Laceration of the diaphragm can cause the ascension into the chest of the
abdominal organs.
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Thoracic Trauma
6. The infectious syndrome - although not the most important, must always be
considered.
7. Traumatic shock - refers to pulmonary shock or ARDS (Acute Respiratory
Distress Syndrome) which is characterized by inflammation of the lung parenchyma
leading to impaired gas exchange with concomitant systemic release of inflammatory
mediators causing inflammation, hypoxemia and frequently resulting in multiple organ
failure.
8. Pain is also an important element. Because of pain patients can not breathe
well and that will lead to hypoventilation with bronchial hypersecretion and hypo-
oxygenation and so oxygenation will decrease even more.
9. Hemorrhagic shock appears as a consequence of blood loss. The most
severe and acute forms are due to cardiac and great vessels injury (hemomediastinum)
but most often it is due to intercostal vessels lesions (produces by broken costal edges)
and lung wounds. The blood accumulates into the pleural space (hemothorax) that may
also dangerously reduce the vital capacity by compressing the lung on the involved side.
In lung wounds, the pleural space contains blood and also gas giving a characteristic
image on thoracic X-ray (hemo-pneumothorax) with a horizontal line delimitation
between fluid and gas.
The pathophysiological mechanisms (acting alone or in combination) can lead to
two life-threatening syndromes: acute posttraumatic respiratory failure and acute
posttraumatic heart failure.
The mechanism of acute respiratory failure in thoracic trauma:
Ventilation deficiency - may be caused by:
o Disturbances of chest wall dynamics:
o Flail chest
o Limitation of respiratory movements due to pain
o Cancellation of tightness of chest wall:
o Pneumothorax
Disturbances of diaphragm movements:
o Traumatic diaphragmatic rupture or phrenic nerves lesions
o Exclusion from ventilation of lung parenchyma areas:
o Compression by pleural effusions or herniated abdominal viscera
o Airway obstruction
o Aspiration of foreign bodies, blood, tracheobronchial
hypersecretion
Impairment of air distribution in the lung parenchyma:
o Paradoxical breathing
o Pendular mediastinal movements
o Alterations of gas diffusion:
o Posttraumatic pulmonary edema
o Pulmonary shock
The mechanism of acute heart failure in thoracic trauma:
Compression and dislocation of the large venous trunks
Mediastinum emphysema and hematoma
Compression on atria (massive pleural effusions)
Heart trauma
Cardiac tamponade
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Thoracic Trauma
Symptoms and signs of acute respiratory failure
As a result of hematosis (venous blood oxygenation) deficiencies:
Polypnea or tachypnea (accelerated respiratory frequency) appears at
the onset of the interstitial pulmonary edema and the frequency
increases as respiratory failure progresses.
Dyspnea is increasing progressively
Cyanosis (due to hypoxemia) occurs when the de-oxygenated Hb
reaches at least 5 g/dl. It is a late sign when anemia is associated.
Cardiovascular signs (in advanced stages):
Tachycardia, (bradycardia in end stages)
Hypotension
Neurological signs:
Impaired sensitivity
Altered consciousness (dizziness, coma)
Signs of end stages:
Shallow breathing or agony breathing
Cyanosis
Sweating
Hypotension, bradycardia
Oliguria, anuria
Laboratory signs
Decreased oxygen saturation (SaO2) - normal 97.5%
Respiratory acidosis - decreased PaO2 and increased PaCO2,
decreased blood pH.
Radiological signs - according to respiratory failure causes
Examination of patient with thoracic trauma
The evaluation of patient with chest trauma is performed in three stages:
1. At site of accident (the primary evaluation) where vital function are
assessed and also the first life-saving measures are taken.
2. Then, at hospital level in the emergency receiving unit (the secondary
evaluation) where further investigations (radiography, tomography,
laboratory, etc.) are carried out and measures of stabilization and
resuscitation are applied.
3. In the department of surgery or intensive care unit if patient requires
hospitalization for surgery or surveillance. If surgery is required the
exploration and assessment of damages are continued intraoperatively.
The initial management of the patient with thoracic trauma is frequently the
responsibility of the emergency physician who is not a thoracic surgeon. It is therefore
mandatory that the emergency physician should be able to recognize the thoracic
injuries that are or will be dramatic if not treated properly.
Patients history - collect data from the patient (where possible) and/or
environment (when patient cannot communicate).
Circumstances of injury
Traumatic agent nature
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Conditions which acted
Injury time (time elapsed from the occurrence of trauma)
Patients co-morbidities and previous treatment
Patient's symptoms (pain, dyspnea, bleeding, etc.)
In unstable and critical circumstances, quick decisions and adequate maneuvers
based on recordings of vital signs and a right interpretation of clinical and diagnostic
pattern are required.
Patient examination
On inspection:
Parietal lesions (wounds, deformities, bruising, hematoma)
Abnormal movements or limitation of thoracic respiratory movements
(rib fractures with floating regions of the chest wall, paradoxical
respiration - flail chest)
Breathing disorders polypnea, dyspnea, tirage =inspiratory sinking of
the intercostal spaces due to airway obstruction
Signs of bleeding (pallor, hemoptysis, external bleeding)
Disorders of hematosis (cyanosis, sweating)
Other signs (ecchymotic mask a dusky discoloration of the head and
neck occurring when the trunk has been subjected to sudden and extreme
compression, mental disorders, other associated lesions, etc)
On palpation:
Pain
Signs of rib or sternal fractures (focal pain, bone discontinuity, bone
crepitations)
Possible floating regions of chest wall
Subcutaneous emphysema (subcutaneous crepitation accumulation of
gas in the subcutaneous tissue)
On percussion:
Hypersonority in pneumothorax
Dull sound in pleural effusions
Enlargement of cardiac area
On auscultation:
Reduction or abolition of lungs vesicular murmur (pleural collection)
Auscultatory asymmetry between the two hemithorax
Pleural or pericardial friction rub sounds
Blurred heart sounds (In the event of cardiac tamponade)
Digestive sounds of intestinal movements (in case of traumatic
diaphragmatic hernia)
Primary evaluation
The evaluation of the patient's chest trauma is only a part of the total assessment.
A general examination should also be performed to observe any associated lesions
(abdominal, limb, head, and spine). Because thoracic injuries are severe and potentially
lethal, the diagnosis and therapy go hand in hand. In unstable and critical patients, quick
decisions based on check of the following vital signs are required:
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Thoracic Trauma
Airway patency. Ensuring a free airway is a major priority in emergency
resuscitation, patients life depending largely on it. Foreign bodies must be removed
from the month and specific maneuvers are applied to prevent backwards fall of the
tongue. All the airway manipulations must be performed with respect to potential
cervical spinal injuries.
Before starting any maneuver for airway patency, some basic parameters should
be assessed:
If patient is conscious or not
Breathing is appropriate or not - check respiratory movement, and
their extension
Duration of hypoxia - cyanosis appears very late
Airway patency
Need for administration of neuromuscular blocking agents (muscle
tension, teeth clenching, severe obstructive pulmonary disease or
asthma)
Stability of cervical spine
The circulation status is evaluated by assessing patient's pulses (radial, carotideal
or femoral). In hypovolemic shock, radial pulse becomes small and may be absent when
blood pressure is below 60 mm/Hg.
The neck veins are distended when there is cardiac tamponade, if it is associated
with hypovolemic shock distension of the neck veins may be absent.
3. Blunt Thoracic Trauma
A. Contusions
1. Simple contusions
May be of variable severity, but often they are mild. Contusion is the effect of a
direct or indirect, frontal or tangential action of the traumatic agent.
Clinic picture is represented by: pain, respiratory discomfort, dyspnea associated
with different chest wall lesions at the site of impact (abrasions, bruising, hematoma,
effusions, etc.).
Radiological examination is required but in most cases nothing pathological is
found.
Treatment is symptomatic with painkiller, myorelaxants, and non-steroidal anti-
inflammatories.
2. Muscular ruptures
Rarely due to the action of a blunt object, often as a result of accidents, sports,
etc.
Clinically is manifested by violent pain with limitation of mobility. At the site of
rupture, initially a depression may be noticed followed by a hematoma.
Chest X-Ray shows nothing special but ultrasound examination can highlight the
muscular rupture and hematoma.
Treatment is symptomatic with bed rest (immobilization), myorelaxants, and non-
steroidal anti-inflammatories and in rare cases surgical repair.
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3. Chest compression
It occurs when the chest is compressed between two forces, which lead to a
sudden increase of pressure in the chest. The pressure exerts a high force on the intra-
thoracic organs (lungs, heart) which are squeezed and then transmitted then to vessels
(veins, arteries).
MORESTIN - acute thoracic compression syndrome - is characterized by:
cervico-facial cyanosis, petechiae and edema in the upper thoracic region and
conjunctival and retinal hematic extravasation (ecchymotic mask) plus neurological
signs of cerebral edema with Cheyne-Stockes breathing type.
In addition to symptomatic treatment, oxygen therapy is needed and/or assisted
ventilation and also cardiac and renal treatment may be necessary.
B. Fractures
4. Simple rib fractures are the most common lesions in the thoracic contusion.
They are produced either directly - at site of impact, or indirectly - by anterior-posterior
chest compression. Fractures may be complete or incomplete (Greenstick fractures).
Rib fractures are not always simple. Depending on traumatic agent and its force,
more than one rib may be fractured. There are many cases when rib fractures are
complicated with lesions of the nearby tissues or organs due to the dislocation of the
fractured edges.
The most frequent associated lesions are those of intercostal vessels and parietal
pleura resulting in hemothorax. If the lung is also perforated, hemothorax will be
associated with pneumothorax too.
Rarely other intrathoracic organs (heart, aorta) are injured by fractured rib edges.
In 20% of cases trauma and fractures of left ribs 9, 10, and 11 are associated with
spleen rupture and consecutive hemoperitoneum.
Even if rib fractures are not complicated, due to the intense pain exacerbated by
every respiration, the respiratory function of the patient may be impaired especially in
those with pulmonary co-morbidities. Pain prevents the patient to breathe deeply,
enough which will lead to alveolar hypo-oxygenation and bronchial hypersecretion.
Because the patient cannot cough effectively and expectorate, secretions accumulate
and lead to airway obstruction, stasis and infection. That is the reason why treating pain
in rib fractures is very important.
Diagnosis is based mainly on clinical criteria: pain at the site of fracture, bone
crepitations, limitation of respiration, decreased breath sounds on the affected side.
Complications of rib fracture may include the following:
Hypoventilation
Hypercapnia
Hypoxia
Atelectasis
Pneumonia
Damage to underlying visceral organs
Pneumothorax (immediate or delayed)
Hemothorax (immediate or delayed)
Aortic injury (immediate or delayed)
Pulmonary contusion
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Thoracic Trauma
Intra-abdominal organ injury
First rib fractures have often been associated with serious head injury, cervical
spine injury, delayed subclavian vessel thrombosis, aortic aneurysm, tracheobronchial
fistula, thoracic outlet syndrome, and Horner's syndrome.
Chest X-ray in two incidences helps much in diagnosis of rib fractures and
associated complications (hemo-pneumothorax).
Other useful investigations are: ultrasonography especially when spleen rupture is
suspected, and CT scan which is not indicated for every simple case, just for those
associated with complications or in polytrauma and unconscious patients.
Costochondral disjunction may exist alone or in combination with the broken
ribs. Without associated rib fracture, the condition is not life threatening and nothing
special must be done, but in association with ribs fractures, it causes a free-floating area
of the chest wall (flail chest) which may be very dangerous impairing the ventilation.
Simple rib fractures without complications may be managed on an outpatient
basis. Painkillers, myorelaxants and anti-inflammatory drugs will be prescribed and a
chest X-ray will be repeated at 24-48 hours. If pain is very intense, intercostal nerve
block (first described by Braun in 1907) is indicated. It can be performed with
Lidocaine but it has a short effect, or with Lidocaine associate with absolute alcohol
(9/1) in which case the effect is longer. Respiratory parameters typically show
impressive improvements upon removal of pain. Blockade of two dermatomes above
and two below the level of fracture is required.
Rib belts or binders do not control pain and are not recommended, because they
will limit the respiratory movements.
When there are complications such as hemo-pneumothorax, the patient should be
admitted in the hospital and properly monitored and treated. Also consider admission
for elderly and patients with underlying lung disease or decreased pulmonary reserve.
The injection needle with bevel faced cephalad is inserted to the rib, then
redirected until the point just clears the inferior margin of the same rib. It is then
advanced 0.5 cm and if aspiration is negative for blood, the anesthetic solution is
injected.
5. Sternal fractures
Sternal fracture occurs as a consequence of a direct impact on the sternum such as
the wheel steering during car accidents (deceleration mechanism).
In most cases, the fracture line is transversal and rarely longitudinal. The fracture
may be with or without displacement, with or without overlapping of fractured edges. In
case of displacement, there is a high risk of cardiac lesion or compression.
The main symptom is the local pain. The pain must be differentiated from angina
or cardiac infarction. On palpation, there is a local tenderness and a deformation as a
step of scale when fractured parts are overlapping.
The diagnosis is based on physical examination and imaging explorations.
The lateral radiograph is usually the most valuable view for detecting sternal
fractures and for determining the degree of displacement.
CT is particularly useful for assessing for associated injuries such as pulmonary
contusion, pneumothorax, or retrosternal hematoma.
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Thoracic Trauma
Treatment as in rib fractures, treatment is aimed at achieving analgesia and
optimization of respiration. In case of displacement with cardiac compression, surgical
reduction and fixation of the sternum may be considered.
6. Flail Chest
It represents a segment of the chest that is free-floating with the pressure changes
of respiration. It appears when there are three or more adjacent rib fractures in two or
more places or rib fractures are associated with costochondral disjunction or
longitudinal fracture of the sternum.
Variations include posterior flail segments, anterior flail segments, and flail
including the sternum with ribs on both sides of the thoracic cage fractured, mixed
forms and soft chest (totally crushed chest).
Incidence: 20% of chest trauma in most cases representing a serious chest wall
injury with underlying pulmonary injury.
Effects of flail chest are:
Paradoxical respiration
Pendulum air
Pendulum mediastinum
Clinical picture
The major symptom is the pain caused by fractures.
The degree of respiratory insufficiency is related to the underlying lung injury.
The worst respiratory insufficiency is seen when the chest is totally crushed because the
patient cannot breathe at all. In this case, there are multiple bilateral rib fractures and the
thorax looses it rigidity becoming soft. For saving patients life it must be intubated and
ventilated with positive pressure (internal pneumatic stabilization).
Tachypnea is present due to the pain.
Paradoxical movements of the affected segment of the chest wall can be
observed.
Other symptoms and signs may be present depending on the associated lesions
and the severity of respiratory insufficiency.
Diagnosis relies on physical examination (clinical observation), imaging studies
and arterial blood gas measurements (helpful to assess the need for mechanical
ventilation and to monitor the patient).
Treatment
Severity of respiratory insufficiency is less a result of the paradoxical motion of
the chest wall but rather a result of pulmonary and other associated lesions.
Priorities:
Airway patency remove the foreign bodies, blood cloths,
secretions. If necessary, orotracheal intubation or even tracheostomy
may be performed
Oxygenation through mask or intubation
Remove pleural collections to ensure lungs expansion by
thoracocentesis or thoracostomy (pleurostomy)
Cardiocirculatory support fluid rebalancing, replace lost blood.
Analgesia
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Thoracic Trauma
Stabilization of the chest wall or early intubation and mechanical
ventilation for poor gas exchange
Stabilization of chest wall to eliminate the paradoxical respiration can be
achieved by:
1. Non-surgical procedures
2. Surgical procedures, which are most commonly performed in patients
requiring a thoracotomy for other reasons
3. Internal pneumatic stabilization indicated in massive crash of the chest
(soft chest wall).
For most patients with acceptable respiratory function the simple immobilization
of the flail segment is sufficient. It can be done using bandages or better adhesive tapes
applied only on the affected hemithorax not to impair the respiratory movements.
External fixation uses a metal plate whose ends lie on the rigid thoracic wall.
Flail ribs are suspended by threads passed under the ribs and fixed to that plate.
Advantages of ribs fixation:
Decreased pain
Improved mechanics
Decreased need of mechanical ventilation
Decreased hospital stay
3. Open Chest Trauma
Non-penetrating trauma - presumes a solution of continuity in the skin with
varying degrees of damage to the anatomical structures of the thoracic wall but without
penetration into the chest cavity. The vast majority are due to weapons or accidents.
Wounds are treated like any other wounds (decontamination, suture, etc.)
Penetrating trauma
Low Energy (arrows, knives, handguns) - Injury caused by direct contact and
cavitation.
High Energy (military, hunting rifles & high powered hand guns) - extensive
injury due to high pressure cavitation.
Shotguns - Injury severity based upon the distance between the victim and
shotgun & caliber of shot:
o Type I: >7 meters from the weapon - Soft tissue injury
o Type II: 3-7 meters from weapon - Penetration into deep fascia and
some internal organs
o Type III: <3 meters from weapon - Massive tissue destruction
Penetrating chest wounds can cause damage to any intrathoracic organ but most
frequently, lungs and heart are affected. Extent of lesions depends on kinetic energy and
type of the traumatic agent. Some injuries are simple perforations, others are so massive
that are incompatible with life.
Intrathoracic organ injuries would cause accumulation of fluids (air, blood,
lymph, digestive content) into the pleural cavity, mediastinum or pericardium.
Most of these are life-threatening injury, surgical repair being mandatory for life
rescue. Surgical gestures can be very simple but there are situations in which complex
interventions are required.
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Thoracic Trauma
1. Pneumothorax
It represents the presence of air into the pleural cavity, which is an abnormal
situation because the parietal and visceral pleura loose their intimate contact, which is
necessary for a good expansion of the lungs in inspiration.
There are two possible sources from where the atmospheric air may enter the
pleural cavity:
1. through an opening of the chest wall (wound), or
2. through an opening in the lung or bronchial tree (wound or leak)
Mechanisms:
Spontaneous Usually on an emphysematous lung during an intense
efforts or cough when emphysematous bubbles burst.
Traumatic
o Closed chest trauma due to lung, bronchial or tracheal rupture or
tear
o Open chest trauma - penetrating wounds which may affect lungs
Iatrogenic during subclavian vein catheter insertion or cardiac
resuscitation maneuvers.
Simple post-traumatic pneumothorax
In most cases, simple post-traumatic pneumothorax is the consequence of lung
perforation by fractured ribs edges during blunt trauma. More rarely the
tracheobronchial tree lesions are the cause of pneumothorax in which case this is
associated with pneumomediastinum. Simple pneumothorax may be also a consequence
of penetrating chest trauma (wounds) with lesions of the lungs and/or tracheobronchial
tree but, if the parietal wound is large enough, an open pneumothorax will develop.
Accumulation of gas into the pleural space in most cases is associated with
accumulation of blood resulting in hemo-pneumothorax.
Depending on how much gas and fluid are accumulated into the pleural space, the
lung will collapse more or less and respiratory function will be affected accordingly.
If there are no adhesions between the two pleura: parietal and pulmonary, the lung
will collapse entirely. If there are adhesions, gas and fluid will be trapped in some
pleural spaces and lung will not collapse. In this last eventuality if there is a tear in the
parietal pleura, the air will spread between anatomical layers of the thoracic wall till the
subcutaneous plane resulting in subcutaneous emphysema. The air will spread in all
directions especially in the upper part of the body (chest, neck, face) but it may reach in
the lower part also (abdomen, scrotum). Air can also spread in fatty tissue of the
mediastinum (pneumomediastinum) and the irritation of recurrent laryngeal nerves will
cause hoarseness.
A simple pneumothorax may progress to a tension pneumothorax.
Symptoms may vary very much depending on pneumothorax extension and associated
lesions. In small pneumothorax, there are no symptoms except those caused by chest
trauma (pain exacerbated by respiration). In larger pneumothorax associated with blood
collection symptoms of respiratory insufficiency are intricated with those of anemia
with tachycardia, pallor, hypotension, cold sweats.
In subcutaneous emphysema swelling of the neck, chest, face, eyelids can be
observed. This can induce pain, difficulty of swallowing, wheezing and difficulty of
breathing.
Skin marks of thoracic trauma may be evident or not.
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Thoracic Trauma
On percussion of the affected side there is a tympanic sound and on auscultation
vesicular murmur is diminished or absent. In hemopneumothorax, on percussion, two
zones are found: the upper of sonority and a lower of dullness, separation line between
them being horizontal.
Diagnosis is based on clinical and imagistic investigations. On a postero-anterior
chest X-ray the pneumothorax can be seen, the lung being collapsed more or less. In
hemopneumothorax, the superior level of fluid is highlighted by a horizontal line. In
simple fluid collections without pneumothorax, this line is not horizontal but convex
downward. Subcutaneous emphysema has also a specific image on X-ray. CT scan is
helpful in assessing associated lesions.
Treatment. All patients with pneumothorax should be admitted, investigated, treated
and monitored.
Small simple pneumothoraxes (not tension pneumothorax!) often resolve on their
own by gas resorption. Gas reabsorbs from the pleural space at a rate of 1.25% of the
trapped volume per day. Therefore, a pneumothorax occupying 30% of the hemithorax
would require 24 days to resolve with the patient breathing room air. Additional oxygen
administration increases the rate of resorption.
Medication consists of painkillers, anti-inflammatory drugs, O2, myorelaxants,
antibiotics, fluid rebalancing, and administration of blood if necessary.
Surgical therapy consists of thoracostomy with pleural drainage, which in most
cases is sufficient for lung reexpansion and blood evacuation. In certain cases when
pneumothorax does not resolve with this procedure or bleeding is massive, thoracotomy
and lesions treatment (aerostasis and hemostasis) becomes necessary.
If not massive, the subcutaneous emphysema is reabsorbed by itself in a few days.
To remove the gas from subcutaneous layer there are several methods: insertion of large
bore needles, small skin incisions or subcutaneous drainage tubes.
Pleural drainage if performed will eliminate the source of the air entering the
subcutaneous space.
2. Open pneumothorax with traumatopnea
Traumatopnea represents the passage of respiratory air in and out through a
wound of the chest wall.
Due to the gradient of pressure between the pleural space and atmosphere during
respiration, the air passes through the open thoracic wound into the pleural cavity during
inspiration and leaves it during expiration through the same opening (sucking chest
wound). Air will be drawn through wound if wound is 2/3 diameter of the trachea or
larger.
Chest wound may be associated with pulmonary lesions so that air can enter the
pleural space also from the lung
The consequences are :
1. Acute respiratory failure, with reduced tidal volume and vital capacity,
caused by lung collapse and oscillation (pendulation) between the two
lungs of the deoxygenated air.
2. Circulatory failure by pendulation of mediastinum toward the healthy
hemithorax during inspiration and toward affected hemithorax during
expiration, which affects the return of venous blood by vena cava
compression.
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Thoracic Trauma
Tidal volume is the lung volume representing the normal volume of air displaced
between normal inspiration and expiration when extra effort is not applied. Typical
values are around 500ml
Vital capacity is the maximum amount of air a person can expel from the lungs
after a maximum inspiration. It is equal to the inspiratory reserve volume plus the tidal
volume plus the expiratory reserve volume.
Symptoms are represented by dyspnea with cyanosis. On inspection, the penetrating
wound is found through which air enters and exits. On percussion of the affected side
there is a tympanic sound and on auscultation vesicular murmur is absent being replaced
by the sound produced by flowing air through the thoracic wound.
Chest X-ray will show a total collapse of the lung.
Treatment
The first aid intention is to close (seal) the wound to prevent further pendulum air
and mediastinum. It can be done by dressing the wound with impermeable gauze
(soaked with ointment) and the patient must be transported urgently to the hospital.
High-flow oxygen will be administered and aggressive hemodynamic and respiratory
resuscitation should be initiated.
Patients with severe respiratory insufficiency should be intubated and ventilated.
As long the thoracic wound is opened, there is no risk of tension pneumothorax.
In hospital a chest tube drainage will be applied through thoracostomy (or
thoracotomy if necessary) into the pleural cavity to evacuate collections and allow the
lung reexpansion. The thoracic wound will be closed. The patient will be monitored and
lung reexpansion will be assessed by auscultation and chest X-ray.
3. Tension pneumothorax
Represents the progressive accumulation of air (with every inspiration) into the
pleural cavity, air which remains trapped into the cavity and gradually compresses the
lung and shifts the mediastinum to the opposite side. It is a high life-threatening
condition but life can be saved by simple maneuvers.
The mechanism is due to a lesion of the thoracic wall (external pneumothorax) or
lung (internal pneumothorax) that acts like a one-way valve letting the air to enter the
pleural cavity but not to exit.
Major vessels such as the vena cava, pulmonary artery, and aorta become kinked
or compressed, and severe hypoxemia ensues. Cardiovascular compromise develops
because the return of venous blood to the right ventricle is severely impaired, as is the
cardiac output. Circulatory collapse shortly follows.
General condition is rapidly altered and the patient can die in few minutes.
Tension pneumothorax can be a progression of a simple or open pneumothorax.
Symptoms and signs
Dyspnea with tachypnea is the first symptom. As pulmonary atelectasis by
compression progresses dyspnea becomes more and more intense with cyanosis.
On inspection, a thoracic wound may be noticed in external pneumothorax and
the flow of air through the opening may be heard. The affected hemithorax is distended
with intercostal spaces bloating. Other signs are: tachycardia, tachypnea, and
diminished breath sounds, hyperresonance to percussion, and decreased tactile
fermiums on the ipsilateral side. A significant volume of gas in the pleural space causes
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Thoracic Trauma
tracheal deviation and mediastinal shift toward the contralateral lung, hypotension,
distended neck veins, and respiratory distress.
Tension pneumothorax is a major emergency - rarely there is enough time
available to conduct investigations. The diagnosis relies on clinical symptoms and signs.
When possible, anteroposterior chest radiography while the patient assumes a
Fowler's or semi-Fowler's position shows:
Collapse of the lung
Mediastinal shift to the healthy side
Descend of the affected side diaphragm
Widening of intercostal spaces
Treatment
Pleural decompression is needed urgently before patient reaches the hospital.
If this diagnosis is suspected, do not delay treatment in the interest of confirming
the diagnosis. Immediately place the patient on 100% oxygen.
Decompression can be easily performed by inserting a 14-16-gauge needle into
the second intercostal space along the mid-clavicular line or into the fifth intercostal
space along the mid axillary line. When the needle enters the pleural space, the sound of
gas escaping is generally perceived. The needle should be placed just above the
cephalad border of the rib to avoid the intercostal vessels.
A catheter can be introduced through the needle and then the needle may be
withdrawn.
This maneuver actually establishes a communication between the pleural space
and atmosphere converting a tension pneumothorax into an open pneumothorax.
After needle decompression, (if it is not performed in the hospital) the patient will
be transported urgently to the hospital. All patients with pneumothorax will be admitted.
If a patient is to be ventilated with positive pressure following needle aspiration,
whether fluid, air or nothing was encountered, a chest drain should be inserted.
In hospital conditions, treatment will be continued by inserting a pleural drainage,
although this maneuver can be performed at site of accident by specialized rescue team.
The site of insertion depends on coexisting of fluid accumulation (blood, effusions,
lymph) into the pleural cavity. In case of pure pneumothorax, the drain may be inserted
into the second intercostal space on mid-clavicular line. If there is fluid collection too,
the drain should be inserted into the 5th-6th intercostal space on mid axillary line, or
associated to that in the second space.
The chest tube will be attached to a Heimlich valve with drainage bag or sealed
underwater (simple Bulau or Beclaire or aspiration drainage).
Mild aspiration can be applied through the drainage tube in order to reexpand the
lung but suction should be seen as the exception rather than the rule.
Most chest drains need no suction. An effective cough can generate a much
higher pressure than can safely be produced with suction. Thoracic suction should only
be used on wards where the staff is familiar with chest drain suction. A drain is safer
with no suction than suction, which is not working correctly.
Aggressive aspiration could maintain open an air leak, the better solution instead
of aspiration being the surgically closure of the air fistula (aerostasis).
After drainage, obtain a follow-up chest x-ray to assess for lung reexpansion and
thoracostomy tube positioning.
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Thoracic Trauma
Monitor the patient continuously for arterial oxygen saturation.
In case of an external tension-pneumothorax, the cause can be very easily
removed by suturing the wound chest.
In case of internal pneumothorax due to lung perforation, lung reexpansion
against the internal chest wall and adhesions formation will seal the perforation in a few
days.
4. Hemothorax
Represents the accumulation of blood into the pleural space.
The source of blood may be any anatomical structure of the thorax but in most
cases after trauma, it comes from intercostal vessels injured by rib fracture and lung
lesions.
The quantity of blood into the pleural cavity may be small, medium or large.
Blood loss can be sudden and massive like in large vessels injuries or slow and
progressive.
Symptoms and hemodynamic changes vary depending on the amount of bleeding and
the rapidity of blood loss.
Blood loss of up to 750 mL should cause no significant hemodynamic change.
Loss of 750-1500 mL will cause the early symptoms of shock (ie, tachycardia,
tachypnea, and a decrease in pulse pressure).
Significant signs of shock with signs of poor perfusion occur with loss of blood
volume of 30% or more (1500-2000 mL).
Exsanguinating hemorrhage can occur without external evidence of blood loss.
Dyspnea is often the predominant complaint associated to those caused by chest
trauma and hypovolemia.
On general examination pallor, tachycardia, cold sweats, and tachypnea can be
noticed. On chest examination, the traumatic lesions of the skin (bruising, hematoma,
wounds, etc.) can be noticed. On percussion, dull sound over the affected side may be
heard. The upper margin of the dull depends on blood quantity in the pleural cavity.
On auscultation, breath sounds are diminished if there is a large hemothorax. In
many traumatic cases, hemothorax is associated with pneumothorax.
The main imagistic investigation is the upright chest radiography. CT scan is a
valuable method in assessing lungs and other intrathoracic organs.
Possible evolution of hemothorax:
Accumulation in large quantities endangering the patients life - needs
evacuation or thoracotomy and hemostasis and blood replacement
Lysis and resorption if small hemothorax
It causes a pleural reaction with exudate and increases the volume of the
pleural fluid.
Infection with thoracic empyema
Transformation in fibrothorax causing lung adhesions, which prevent a
good lung expansion reducing their capacity
Differential diagnosis should be made with other pleural collections
hydrothorax, pleurisy, empyema, and chylothorax. In thoracic trauma context, the
chylothorax is more likely to be produced or blood can come from abdominal cavity
through a diaphragmatic rupture.
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Treatment depends on the size of hemothorax, its speed of developing and the source
of bleeding. In most cases, patients will be admitted for treatment and surveillance.
Indications for thoracotomy after trauma:
>1500 mL blood from chest tube on insertion
>200 mL blood/hour from chest tube thereafter (for 2-4 hours)
Massive air leak such that lung will not re-expand after a properly placed
and functioning chest tube has been inserted
The medical treatment will be common as for thoracic trauma plus blood
replacement if necessary and antibiotherapy. In small hemothorax aspiration of blood by
thoracocentesis can be performed. In medium and large hemothorax, pleural drainage
through thoracostomy is the method of choice. If bleeding continues or pleural drainage
is not effective, thoracotomy should be performed for hemostasis (intercostal vessels
ligation, lung suture, etc).
5. Cardiac tamponade
It is a highly life threatening condition.
Accumulation of fluid (blood in most traumatic cases) into the pericardial sac will
lead to cardiac movement limitation with cardiocirculatory insufficiency and cardiac
arrest.
The pericardial space normally contains 20-50 mL of fluid. Pericardial effusions
can be serous, serosanguinous, hemorrhagic, or chylous.
In chest trauma, intrapericardial fluid is represented by blood, which may come
from:
A penetrating (stab, shot) wound which produces a lesion of the cardiac
vessels (coronary vessels) or heart wall (heart perforation)
A contusion of the heart with consecutive heart wall necrosis and rupture
Contusion of the heart with rupture of its wall
The pathophysiological mechanism is represented by diminished diastolic filling
because ventricles cannot distend sufficiently to overcome the increased intrapericardial
pressures. Tachycardia is the initial cardiac response to these changes to maintain the
cardiac output.
The rate of fluid accumulation into the pericardial sac is very important. Rapid
accumulation of about 150 ml will develop an increased pressure that opposes filling the
heart with blood. The rapid accumulation is more likely to occur during chest trauma. In
other conditions, when accumulation produces over a long period, more than 1000 ml of
fluid will not have significant effect due to adaptive stretching of the pericardium.
Symptoms: tachycardia, tachypnea, palpitations, dyspnea, restless body movements,
unusual facial expressions, sense of impending death, dizziness, drowsiness.
Signs: distended jugular veins, hepatomegaly, enlarged cardiac dullness on percussion,
diminished heart sounds, pericardial friction rub, weak pulse, hypotension and also
other signs related to chest trauma.
The Beck triad:
1. increased jugular venous pressure
2. hypotension
3. diminished heart sounds
Kussmauls sign: Decrease or absence of jugular vein dilatation during inspiration
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Thoracic Trauma
Imaging studies
Chest X-Ray enlargement of the heart shadow as a tent, with disappearance of
heart contours (plus other possible associated modifications due to trauma)
Ultrasound reveals fluid accumulation in the pericardial sac limiting the
amplitude of cardiac movements)
CT scan may reveal fluid accumulation in pericardial sac and other lesions but
in most cases, there is not sufficient time to perform the examination
Electrocardiogram will show: sinus tachycardia, low-voltage QRS complexes and
PR segment depression.
Differential diagnosis in chest trauma should include:
Tension pneumothorax distended jugular veins is also present!
Cardiogenic shock
Pulmonary embolism
Treatment
Cardiac tamponade during chest trauma is a very serious condition with a high
mortality. Life saving depend on rapid recognition of it and rapid pericardial
decompression. After decompression, the treatment must be continued for the
underlying cause that means in majority of cases thoracotomy or sternotomy, opening
the pericardial sac and hemostasis by either cardiac suture or vascular suture.
Pericardial puncture
1. Epigastric approach Marfans point at the tip of xiphoid appendix.
2. Chest approach - may be performed on the right or left side of the
sternum
a. The left approach:
in the 4th or 5th intercostal space very close to the
sternum to avoid the internal mammary artery. The
needle is inserted perpendicularly.
Dieulafoy point in the 5th intercostal space at 6 cm
beyond the sternum
Delormes point in the 6th intercostal space at the edge
of sternum
Rendus point - in the 6th intercostal space at 8 cm
beyond the sternum
Huchards point in the 7th intercostal space at 8 - 9 cm
from sternal midline (below the Dieulafoy point)
b. The righ approach
Roths point in the 6th intercostal space, very close to
the sternum. The needle is inserted to the left and up.
The patient will be in a semi-seated position in a 45-degree inclination of the
thorax.
After the needle passes the skin, it is driven cephalad and obliquely to the left,
following the posterior face of the sternum. Then it passes the diaphragm and after a
trajectory of 4 cm for patients younger than 5 years and 6 cm for those over 15 years, it
enters the pericardial sac in its lowest region.
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Thoracic Trauma
Advantages of this technique are that it avoids the pleura and the internal
mammary vessels and may be used in small pericardial collections. The epigastric
approach is contraindicated in sternum deformities.
The possible complications of pericardial puncture:
Coronary artery damage
Laceration of the myocardium
Penetration in the lung
Echocardiographic guidance increases the success rate of pericardiocentesis by
reducing these complications.
4. Lesions of the Intrathoracic Organs
1. Lungs lesions
A. Pulmonary contusion is represented by an intraparenchymatous hematoma
surrounded by atelectasis. It is manifested by pain, dyspnea, cough, and hemoptysis. In
case of extensive contusion, hypo-oxygenation and low blood oxygen saturation may
occur with cyanosis.
The severity ranges from mild to very serious. Pulmonary contusion is the most
common type of potentially lethal chest trauma. Estimated mortality rate ranges
between 14% and 40%.
It occurs in 3075% of severe chest injuries.
On chest X-ray, a zone of pulmonary condensation (characteristic white region) is
seen. The presence of hemothorax or pneumothorax may obscure the contusion on a
radiograph.
CT scanning is a more sensitive for pulmonary contusion. Contusion can be
detected almost immediately after the injury. However, in both X-ray and CT a
contusion may become more visible over the first 2448 hours after trauma.
Differential diagnosis. If the consolidation lasts longer than 72 hours, consider:
Aspiration
Pneumonia
ARD
Treatment in most cases is just supportive. In severe extended pulmonary lesions
with hypoxia, mechanical ventilation with oxygen supplementation is needed.
Mechanical ventilation with moderate positive pressure is indicated when:
Partial pressure of oxygen is less than 60 mm Hg at a concentration
of 50% oxygen in the inspired air
Respiratory rate>24/minut
Maximum vital capacity <10 ml / kg
Antibiotherapy is used to prevent pulmonary infection.
Pulmonary contusion can progress to complete resorption in 5-10 days or with
complications such as infection and abscess formation. It can also permanently reduce
the compliance of the lungs.
B. Pulmonary laceration
Pulmonary laceration is produced in most cases by penetrating chest wounds (stab
or shot) but also it can occur during very intense blunt thoracic trauma or as a
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Thoracic Trauma
consequence of rib fractures. The injury is more serious when is closer to the pulmonary
hilum (in these cases large vessels and bronchi are damaged too).
Symptoms and signs are the same as in thoracic contusions or penetrating wounds
with hemothorax or hemopneumothorax plus hemoptysis.
Radiological images are similar to those of lung contusion +hemothorax or
hemopneumothorax.
Treatment - in patients with small lesions without respiratory failure
thoracostomy and pleural cavity drainage with mild aspiration for lung reexpansion is
sufficient.
In case of important pneumothorax or hemoptysis, bronchoscopy would be
necessary for diagnosis of tracheobronchial tree lesions.
Patients requiring mechanical ventilation may develop broncho-pleural fistulas,
sometimes requiring two independent lung ventilation.
In more serious lesions, thoracotomy is necessary for saving the life of the
patient. Lungs lesions are surgically resolved, aerostasis and hemostasis is checked and
two drainage tubes are placed in the pleural cavity. In most cases this patients are
monitored in the intensive care unit.
2. Tracheo-bronchial tree lesions
Lesions can be axial or circular, complete or incomplete.
Symptoms are dependent on the size and permeability of the affected bronchus
(fragments of lung parenchyma or blood clots can obstruct airways).
Characteristic features on which diagnosis relies are hemoptysis accompanied by
tension pneumothorax, pneumomediastinum or subcutaneous emphysema.
Suspected bronchial rupture arises whenever in a pneumothorax the lung does not
reexpand under proper suction drainage.
Bronchoscopy should be carried out promptly since it is the most reliable means
of establishing the diagnosis.
Surgical treatment is represented by thoracotomy and suture of the ruptured
bronchus or trachea under ventilatory support using double lumen tubes and selective
bronchial intubation.
3. Cardiac lesions
Cardiac lesions may be a consequence of blunt thoracic trauma (most often in
traffic accidents when the steering wheel hit the sternum) or penetrating trauma (stab,
gunshot, puncture, etc.).
Types of lesions and their severity depend on traumatic agent type, its force and
coexisting cardiac diseases. Survival depends much on the type of cardiac lesion and
time elapsed between the accident and establishment of treatment.
Blunt traumas are represented by myocardial contusion and myocardial rupture.
The rupture may interest the walls or septum (interventricular / interatrial) and valves.
The right atrium and ventricle are the most frequently injured due to their anterior
position followed by the left atrium and left ventricle. The survival rate with 1-chamber
rupture is about 40%. Two-chamber rupture has a mortality of 100%.
A sudden rise in blood pressure during compression of the chest may injure the
cardiac valves or lacerate the ventricular wall or septum.
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Thoracic Trauma
Myocardial contusion is represented by patchy areas of muscle necrosis and
hemorrhagic infiltrate.
Les extended heart muscle contusion induces cardiac arrhythmias that usually
improves with time but injury to a coronary artery can lead to myocardial infarction.
Regurgitation and cardiac insufficiency due to traumatic lesions of valvular
system tends to worsen with time within in few weeks or years.
Diagnosis
A patient with angina-like chest pain or progressive dyspnea after trauma must be
suspected of having a cardiac injury. Arrhythmias are not very specific. Systemic
hypotension and elevated venous pressure are important signs of cardiogenic shock or
tamponade.
Investigations
Thoracic X-ray reveals sternal and ribs fracture, hemothorax, enlarged cardiac
shadow ,but cannot offer information about heart.
CT scan offer more detailed aspects concerning the pleural spaces, lungs and
mediastinum, but not very much about cardiac contusion.
Ultrasound echocardiography is an important diagnostic tool that can be used to
detect anatomical anomalies (pericardial effusion, areas of ventricular dyskinesia, and
valvular dysfunction) and physiologic anomalies of the heart (abnormal blood-flow
patterns).
12-lead EKG may show abnormalities.
CPK (Creatine phosphokinase) values may be elevated, but also in skeletal and
muscular trauma so they are not very specific.
Troponins (a complex of three regulatory proteins: troponin C, troponin I and
troponin T found in skeletal and cardiac muscle, but not smooth muscle) are more
specific.
Treatment
In stable patients without evident lesions on echocardiography the evolution is
good and only a close monitoring for several hours is required. If their condition
remains stable and the ECG reveals no or only minor changes they can be admitted to a
regular ward.
A patient with angina-like chest pain, elevated enzyme levels or minor
arrhythmias should be monitored in an intermediate care unit.
A patient with progressive dyspnea, ischemic patterns on ECG, or complex
arrhythmias should be treated in an intensive care unit, receive specific therapy, and be
investigated further.
A patient in cardiogenic shock due to cardiac tamponade will be quickly
investigated and treated accordingly (see cardiac tamponade).
In case of ventricular akinesia, the patient may benefit from inotropic support or
intraaortic balloon counterpulsation.
More serious injuries of intracardiac septa and valves, require surgery and
extracorporeal circulation.
Most penetrating cardiac injuries are secondary to assaults or accidents
(industrial, traffic). Penetration with sharp objects is associated in general with a better
outcome than penetration resulting from gunshot. Iatrogenic causes are represented by
lesions produced secondary to cardiopulmonary resuscitation (fractured sternum or ribs
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Thoracic Trauma
may penetrate the heart), central venous catheterization, or percutaneous cardiac
procedures.
Survival after such lesions is very low (6-17%), very few patients reaching the
hospital alive but from those who reach alive almost can be saved.
Patients with small wounds of the heart will develop cardiac tamponade but those
with extensive lacerations die almost immediately, as a result of rapid and voluminous
blood loss.
To prevent exsanguination, any stabbing weapons still present in the chest should
not be removed before reaching the hospital.
If there are suspicions of penetrating cardiac lesion a pericardial window can be
performed by subxiphoid approach.
Penetrating cardiac trauma must be surgically resolved. The approach can be
through a left thoracotomy or by sternotomy. The pericardial sac is opened, the blood
and cloths removed and the cardiac wound is assessed. Digital compression direct on
the wound is the procedure for temporary hemostasis. Cardiac suture can be performed
with the finger still in place on the wound or using a balloon catheter introduced into the
cardiac cavity for temporary hemostasis. Larger injured coronary arteries will require
either direct repair or bypass.
4. Aortic injury
The two most common causes of this type of lesion are traffic accidents and stab
or shot wounds. In the first case the mechanism is deceleration (heart displacement will
put under tension the aorta) and in the second the direct action of the traumatic agent.
Aortic rupture is very deadly, about 90% of patients die within minutes. Of those
who arrive at the hospital alive, another 90% die.
Many patients have little external evidence of serious chest trauma.
Aortic injury should be suspected on chest radiographs when the mediastinum is
enlarged more than 8 cm and aortic knuckle is disappeared.
CT scan reveals mediastinal hematoma but not necessarily from aortic rupture.
When the diagnosis is suspected on basis of chest radiography or clinical findings
it can be confirmed by means of contrast-enhanced aortography.
Treatment is only surgical but unfortunately with a very high mortality rate.
5. Esophageal rupture
The esophagus is located in the posterior mediastinum being a well-protected
organ against traumatic agents. However, there are rare cases when esophagus may be
injured during thoracic trauma especially during penetrating trauma caused by stab
wounds or shot gun wounds. On the other hand, iatrogenic lesions are not very rare (85-
90% of cases) occurring during endoscopic procedures, gastric tubing or during
abdominal or thoracic operations. There are also self-induced esophageal lesions caused
by foreign bodies, corrosive or drug ingestion and postemetic trauma.
Esophageal lesions are a potentially devastating condition. Rapid diagnosis and
therapy provide the best chance for survival but delay in diagnosis is common, resulting
in substantial morbidity and mortality.
Spontaneous esophageal rupture is a rare entity, which is known as Boerhaave
syndrome (rupture of the esophageal wall due to vomiting).
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Thoracic Trauma
The estimated mortality is approximately 35%, making it the most lethal
perforation of the digestive tract. The best outcomes are associated with early diagnosis
and definitive surgical management within 12 hours of rupture. If intervention is
delayed longer than 24 hours, the mortality rate (even with surgical intervention) rises to
higher than 50% and to nearly 90% after 48 hours.
As a result of a tear or rupture of the esophagus, its content (saliva, food, air) will
enter the mediastinum resulting in mediastinitis.
Clinical picture is represented by retrosternal pain, dysphagia, hematemesis,
subcutaneous emphysema, pleural effusion, fever, septic shock.
The Mackler triad:
1. vomiting
2. lower chest pain
3. cervical subcutaneous emphysema
Chest radiography and CT scan may show: enlargement of the mediastinum,
pneumomediastinum, pleural effusion especially on the left side, subcutaneous
emphysema. A water-soluble contrast (Gastrografin) can be used to highlight the
extravasation of contrast and location and extent of rupture/tear.
Esophagogastroduodenoscopy is not recommended for acute esophageal rupture.
Treatment
Patients will be admitted to ICU
Nothing by mouth
Parenteral nutritional support
Nasogastric suction
Broad-spectrum antibiotics
Criteria for nonoperative treatment:
Recent iatrogenic or postemetic esophageal perforation with minimal
symptoms and absence of sepsis.
No malignancy, obstruction, or stricture in the region of the
perforation
Isolation of the leak within the mediastinum and drainage of
perforation into the esophagus
Medical contraindications to surgery (eg. severe emphysema, severe
coronary artery disease)
The aims of surgery for esophageal rupture are:
Prevent further mediastinal contamination
Drainage of the mediastinum and pleural cavity
Ensure enteral nutrition
Reestablish the esophageal integrity or replace a portion of it
(esophagoplasty)
Surgical techniques include the following:
Tube thoracostomy (alone or associated to other techniques)
Primary repair (suture plus reinforcement) of the rupture either by
thoracic or abdominal approach or by thoracoscopic approach
Diversion (cervical esophagostomy)
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Thoracic Trauma
Diversion and exclusion (cervical esophagostomy + esophagus
ligation above the cardia +feeding gastrostomy or jejunostomy)
Esophageal resection (+cervical diversion +feeding jejunostomy)
Esophageal stent
Endoscopic placement of fibrin sealant
Esophagoplasty (using stomach or colon) in a second phase after
mediastinitis resolution
6. Diaphragmatic rupture
It may be a consequence of blunt or penetrating thoracic and abdominal trauma.
The diaphragm is the main respiratory muscle, which separates the abdominal
cavity from the thoracic cavity. Between those two cavities, there is a gradient of
pressure: the intra-abdominal pressure is higher then the intrathoracic and this is the
reason why abdominal organs tend to protrude into the thoracic cavity when there is a
solution of continuity (rupture) of the diaphragm.
The right diaphragm is better protected against rupture during blunt trauma by the
liver while the left diaphragm ruptures more frequently (70-90%) especially at level of
the central tendon.
More frequently, the rupture is the consequence of a sudden rise of the intra-
abdominal pressure during blunt abdominal trauma then during thoracic trauma because
the thoracic wall is more rigid.
On the other hand, during blunt thoracic trauma, especially from lateral side, the
diaphragm (and also the nearby organs spleen, liver) may be injured by fractured ribs.
Penetrating trauma, either thoracic or abdominal, may produce tears in the
diaphragm and organs from both cavities. Even though they are not injured, abdominal
organs can protrude into the pleural cavity resulting in diaphragmatic hernia with the
possibility of strangulation and necrosis of herniated organs. Visceral herniation occurs
in 30-50% of patients with diaphragmatic tears, and the stomach is the most common
abdominal organ to become herniated, but there are not rare cases when the transverse
colon, spleen or small intestines are involved in herniation.
In large diaphragmatic ruptures, the herniated abdominal organs produce a
dislocation of the lung and heart leading to ventilatory, respiratory and cardiocirculatory
dysfunction with dyspnea, cyanosis and cardiac rhythm disturbances.
Other symptoms may be: sharp shoulder pain, digestive symptoms (dysphagia,
vomiting, intestinal obstruction) and associated symptoms depending on the associated
traumatic lesions.
The small diaphragmatic ruptures are frequently unrecognized in the first days
because they do not give any specific symptoms and may be overlooked at chest x-ray
investigation. Diagnosis may be delayed in as many as two thirds of all patients.
The plain chest radiograph is abnormal in 77% of patients, but the findings are
nonspecific and the diagnosis is initially missed in most cases.
On physical examination of the thorax, the most important sign that rises the
suspicion of diaphragmatic rupture is the bowel movements heard on auscultation.
There are 3 clinical phases of diaphragmatic injuries (described by Grimes):
1. Acute phase - in the same day with the trauma
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Thoracic Trauma
639
2. The second or latent phase if the injury is not recognized in the early
phase. It is an asymptomatic phase but intra-abdominal viscera evolve
into gradual herniation.
3. The third phase is that of complications (obstruction, incarceration,
strangulation, perforation, peritonitis, pleural effusions, etc.)
Radiographic findings include apparent elevation of the hemidiaphragm, loss of
the normal contour, distortion of the normal shape or mediastinal shift away from the
injury. The pathognomonic findings are the intrathoracic intestinal fluid-air levels and
bowel or gastric movements observed during fluoroscopy. Administered Gastrographin
will fill the herniated stomach or intestines.
CT findings of diaphragmatic rupture include the followings:
Discontinuity of the diaphragm
Herniation of abdominal organs into the chest
Pneumothorax and/or hemothorax and/or hemoperitoneum
The mortality rate in unrecognized cases is 30% as a result of delayed herniation
of abdominal viscera and bowel strangulation. Early recognition and repair of
diaphragmatic tears improves the prognosis.
Most often, the patients are polytraumatized and unconscious.
The first taken measures are those for life support, but concomitant good clinical
and paraclinical evaluation must be carried out.
Intrathoracic organs lesions are more life threatening than those intra-abdominal,
and therefore the initial approach should be the thoracotomy in these cases. The
abdominal organs can be assessed somewhat through the diaphragmatic rupture and if
there are no intra abdominal lesions, the diaphragm will be sutured without laparotomy.
Some abdominal organs lesions can be managed through the thoracic approach
(splenectomy). If necessary, laparotomy can be associated.

Pleuropulmonary Surgical Pathology
PLEUROPULMONARY SURGICAL PATHOLOGY
1. Pleural effusions
a. Pleurisy
b. Chylothorax
c. Thoracic empyema
2. Surgical Pathology of the Lungs
a. Hydatid cyst of the lungs
b. Lung abscesses
c. Lung cancer

1. Pleural Effusions
Between the two pleura layers, the parietal and the visceral one, there is a virtual
space which contains a small quantity of fluid (about 1ml) which ensure a sliding plane
and also keeps the two pleura in contact. This liquid is produced continuously but also
reabsorbed so its quantity remains constant under the control of oncotic and hydrostatic
pressure and lymphatic drainage.
The pleural effusion represents an abnormal collection of fluid into the pleural
cavity as a result of excess fluid production or reduced absorption.
Classification. Pleural effusions are classified as:
1. Transudate,s which results from an imbalance in oncotic and hydrostatic
pressures, generally as a result of systemic factors impairment.
2. Exudates, which is the result of inflammation of the pleura or decreased
lymphatic drainage (local factors).
3. Combination of these two
The mechanisms of pleural effusions production:
Alteration of pleural permeability - inflammation, malignancy,
pulmonary embolus
Increased capillary permeability - trauma, malignancy, inflammation,
infection, pulmonary infarction, drug hypersensitivity, uremia, pancreatitis
Reduction of oncotic pressure - hypoalbuminemia, cirrhosis, cachexia
Increased hydrostatic pressure in the systemic and/or pulmonary
circulation - congestive heart failure, superior vena cava syndrome
Decreased lymphatic drainage - including thoracic duct obstruction or
rupture
Migration of fluid - from pulmonary edema across the visceral pleura -
migration across the diaphragm via the lymphatics or structural defects -
cirrhosis, peritoneal dialysis
Causes
Transudates
o Congestive heart failure
o Cirrhosis (hepatic hydrothorax)
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o Atelectasis
o Hypoalbuminemia
o Nephrotic syndrome
o Peritoneal dialysis
o Myxedema
o Constrictive pericarditis
o Urinothorax - usually due to obstructive uropathy
o Rare cases ( cerebrospinal fluid leaks to the pleura, intra-pleural
migration of central venous catheter)
Exudates inflammatory fluid with elevated protein content
o Pneumonia (parapneumonic)
o Malignancy - lung or breast cancer, lymphoma, leukemia,
sarcomas, melanoma
o Pulmonary embolism
o Systemic diseases - collagen-vascular conditions - rheumatoid
arthritis, systemic lupus erythematosus
o Tuberculosis (TB)
o Trauma thoracic trauma, postcardiac injury syndrome,
esophageal perforation
o Radiation pleuritis
o Sarcoidosis
o Fungal infection
o Intra-abdominal pathological processes abscess (especially
subdiaphragmatic), pancreatitis, pancreatic pseudocyst, stomach
cancer, ovarian cancer, Meigs syndrome (benign pelvic neoplasm
with associated ascites and pleural effusion)
o Status-post coronary artery bypass graft surgery
o Pericardial disease
o Ovarian hyperstimulation syndrome - iatrogenic complication of
ovarian stimulation for assisted reproduction technology
o Drug-induced pleural disease
o Asbestos-related pleural disease
o Yellow nail syndrome (genetic disorder consisting in yellow nails,
lymphedema, recurrent pneumonia, pleural effusions)
o Uremia
o Trapped lung (unexpandable lung due to visceral pleural localized
scarring with the formation of a fibrin peel leading to pleural
effusion)
o Chylothorax (elevated triglycerides in pleural fluid, traumatic
causes, non-traumatic causes idiopathic, congenital)
o Pseudochylothorax (chronic condition with elevated cholesterol in
pleural fluid)
o Fistula (ventriculopleural, biliopleural, gastropleural, etc)
Clinical picture
Anamnesis is very important in diagnosis. Patients should provide information
about associated known illnesses (pneumonia, cancer, cirrhosis, cardiac insufficiency,
renal impairment, trauma, etc.) and underwent treatment. In addition, occupational
history aspects may be important (asbestosis). Patients will describe the onset and
evolution of symptoms.
641
Symptoms:
Dyspnea - is the most frequent and constant symptom but its degree depends
much on the quantity of fluid accumulated. Dyspnea may be caused by the condition
producing the pleural effusion or associated co-morbidity, such as lung or heart disease,
obstructing endobronchial lesions, or diaphragmatic paralysis, rather than by the
effusion itself.
Chest pain is very variable in intensity and sometimes absent. On the debut of a
pleural effusion due to pleuritis (inflammation, exudate) the pain is intense, sharp or
stabbing, exacerbated by respiratory movements due to pleural irritation. Generally,
pain is absent in transudates. Pain may be caused by the underlying disease: cancer,
pulmonary infarction, pneumonia, etc.
Cough is frequently present but unproductive. When it is productive with
purulent or bloody sputum the underlying cause may be pneumonia, pulmonary
infarction or cancer.
Other symptoms depending on the underlying disease.
Signs
Generally, there are no physical findings for effusions smaller than 300 mL.
On inspection, in small and medium collections nothing special can be noticed. In
large collections on the affected side, the thoracic wall is distended and the enlarged
intercostal spaces are bulging between ribs. The patient is lying on the affected side.
This position reduces the movements of the affected side and so the pain, permitting
better expansion of the opposite hemithorax. Orthopnea is seen especially in patients
with cardiac insufficiency.
On palpation, the pectoral fremitus is diminished.
On percussion, dullness is found with the superior margin convex downward
(Ellis Damoiseau line), decreased diaphragmatic excursions and mediastinal shift away
from the effusion.
On auscultation, diminished or inaudible breath sounds.
Investigations
Chest radiography is the most often performed investigation. Performed in
upright position, it shows a basal opacity on the affected side. Small volume of fluid
(175 ml) will just blunt of the costophrenic angle. Large volumes will compress the lung
and shift the mediastinum to opposite side.
To detect small effusions more reliable is lateral decubitus chest X-Ray. Layering
of an effusion on lateral decubitus films defines a freely flowing fluid and, if the
layering fluid is 1 cm thick, indicates an effusion of greater than 200 mL.
Chest radiography in many cases also offers information about the underlying
condition such as: pneumonia, pulmonary infarction, lung tumors, cardiac enlargement,
etc.
CT scan usually is the next investigation ordered. It is more accurate, offers a
better resolution and more details regarding the effusion and other co-morbidities. In
many cases, effusions are found on CT performed for other illnesses. It is very useful
especially in cases of loculated pleural effusion, complete opacification of hemithorax,
or associated lung parenchymal abnormalities and for guiding the interventional
procedures.
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Ultrasound examination has certain advantages: does not use radiations, can be
performed at bedside, is faster, is cheaper and can detect small quantities of pleural
fluid.
MRI is performed especially for diagnosing tumoral masses associated with
pleural effusions.
Once the fluid was highlighted by imagistic investigations the next step in
diagnosis is to determine the nature of the fluid and what is causing it. Sometimes,
especially in transudates, the underlying cause is obvious and treatment may be applied
without necessity of performing a diagnostic thoracentesis (eg. congestive heart failure)
Thoracentesis is performed for two reasons: 1. for diagnosis and, 2. as a
treatment procedure for fluid evacuation.
In most cases, it is performed based only on physical examinations but it can be
guided by imagistic investigation also.
Diagnostic thoracentesis is indicated only when there is sufficient liquid
accumulated (over 200 ml) and in case of new and unexplained pleural effusions. It can
be performed using a simple intramuscular needle attached to a syringe. In obese
patients with thick thoracic wall, a longer puncture needle is needed.
The most appropriate position of the patient is sitting on a chair with back turned
to the doctor. The superior margin of the fluid is located by percussion and marked on
skin. Puncture will be always performed in full dullness preferable on the posterior
axillary line in the sixth intercostal space. During procedure, a nurse or another doctor
will assist the patient. After skin disinfection a local anesthesia will be performed. The
puncture needle attached to the syringe will be advanced until the resistance of the rib is
encountered. Then the needle is reoriented above the rib and advanced 1-2 cm
maintaining a negative pressure in the syringe. The needle is inserted above the rib to
avoid damaging of intercostal vessels. The patient is advised not to move, cough, or
take a deep breath during the procedure. The syringe is filled with fluid, withdrawn (the
needle also) and sent for bacteriologic and chemical analysis.
If the intention is to evacuate the fluid, before insertion, an intermediate tube will
be attached between the needle and the syringe. The fluid can be withdrawn by gravity
drainage or by suction. The quantity and aspect of evacuated fluid will be documented.
After thoracentesis control chest radiography will be performed.
Precautions:
Identifying the correct landmarks if the needle is inserted too low the
liver or the spleen may be damaged. Too high insertion can result in lung
perforation and iatrogenic pneumothorax.
Never insert the needle through an area with an infection.
Patients who are on anticoagulant drugs should be carefully considered for
the procedure.
Ask the patient not to move, cough or deep breathing during the
procedure.
Assess the patient during the procedure (difficulty breathing, dizziness,
faintness, chest pain, nausea, pallor or cyanosis, weakness, sweating,
cough, alterations in vital signs, oxygen saturation levels, or cardiac
rhythm)
Avoid rapid suction and do not evacuate more than 1500 ml of fluid
because of the risk of reexpansion (ex vacuo) pulmonary edema with a
mortality rate of 20%.
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The gross aspect of extracted fluid may be:
Aqueous aspect is characteristic for transudate - Hydrothorax
Sero-citrine appearance is characteristic for exudate
Bloody aspect is frequently due to malignancy, pulmonary infarction,
post-traumatic
Frankly purulent is characteristic for empyema
A putrid odor suggests an anaerobic empyema
Milky, opalescent fluid suggests a chylothorax
Extracted liquid will be sent for laboratory tests (cytology, bacteriological and
antibiogram, and biochemistry analysis).
Other investigations:
Surgical thoracoscopy plus biopsy the procedure is burdened by the
risks of general anesthesia
Medical thoracoscopy plus biopsy is performed on a conscious sedated
patient
Closed-needle pleural biopsy
These procedures are indicated in undiagnosed cases of pleural effusion
especially when malignancy or TB is suspected.
Varieties of pleurisy by location
Diaphragmatic pleurisy the diaphragmatic pleura is affected. The effusion is
small in quantity and may be either sero-fibrinous or purulent. Frequently occurs in
intra-abdominal subdiaphragmatic septic processes (abscesses). The pain is located at
the base of the affected hemithorax being exacerbated by respiration. Nausea and
vomiting often occur. If effusion is purulent, there may be bulging of the intercostal
spaces. The temperature is high and the patient presents a septic status.
Interlobar pleurisy - The inflammatory process affects the interlobular
pulmonary pleura. The effusion is trapped into the interlobar space due to adhesions,
which separate this space from the pleural cavity. It is found more frequently in the right
side than in the left, and between the upper and lower lobe, of the lung. Symptoms are
not characteristic. Abscesses located here can perforate and evacuate into a bronchus.
According to localization, pleurisy may be:
1) costal-diaphragmatic
2) diaphragmatic
3) costal
4) interlobar
5) paramedistinal
6) apical
Encysted pleurisy is the result of adhesions between parietal and visceral pleura,
which delimit enclosed spaces in various positions. Symptoms are not characteristic.
Collection is found on chest radiography but is difficult to differentiate from other
pleuro-pulmonary condensations. The exact position of collection is better established
under fluoroscopy and CT or MRI scan that can also guide the exploratory puncture.
A. PLEURISY
Pleuritis represents an inflammation of the pleura in most cases due to infection.
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Pleurisy means a pleuritis associated with pleural effusion. The accumulated fluid
is an exudate. Depending on fluid character pleurisy can be:
Serofibrinous pleurisy (pulmonary tuberculosis, etc.)
Purulent pleurisy (Empyema) (pulmonary tuberculosis, pneumopathies)
Hemorrhagic pleurisy (heart infarction, lung cancer or pulmonary
tuberculosis)
The inflamed pleural layers rub against each other every time the lungs expand
and this causes severe sharp pain with inhalation (pleuritic chest pain).
Serofibrinous pleurisy is characterized by a fibrinous exudate on the surface of
the pleura and an extensive effusion of serous fluid into the pleural cavity.
Etiopathogenesis
Pulmonary tuberculosis is still the most common cause (50-55%). Other possible
causes may be: cancerous pleurisy (25-30%), viral pleurisy, associated to a pulmonary
infarction, rheumatismal pleurisy, secondary a serious bacterial pneumonia treated with
antibiotics, pleurisy from subdiaphragmatic collections, liver cirrhosis, collagenosis
(especially lupus erythematosus).
In tuberculosis, pleurisy appears in patients aged between 16 and 35 years and it
may be the first manifestation of the disease. The pleural cavity is seeded by
hematogenous route or by contiguity from lung or lymph nodes TB process. Favoring
factors are: exposure to cold, humidity, malnutrition, immunity impairment.
The disease starts with a pleuritis (without fluid collection). The pleura is
swollen, erythematous and covered with fibrin deposits. If the process progresses the
exudate (a clear yellow liquid) appears. In TB, miliary tubercles are present in pleura.
Inflammation can heal without sequelae but in many cases adhesions between the two
pleural layers appear representing pleural symphysis, which can involve just some
regions or the entire pleural cavity (fibrothorax).
Clinical picture
The onset of symptoms is acute in most cases (but it may be also silent) with
thoracic pain intensified by respiratory movements radiating in shoulder or abdomen.
Pain reduces in intensity when the pleural collection appears. In the next days the fever
(39-40 C degrees) and chills appear. The fever evolves constantly for 5 days till 3 weeks
and pain reduces gradually. Associated symptoms are: pallor, dyspnea and unproductive
cough.
Usually physical signs will be absent when fluid accumulation is small.
On palpation, the tactile vocal fremitus is diminished and local tenderness can be
identified on the affected side. On percussion in pleural effusion, there is a dull sound
and the location of the upper level of the dullness depends on how much fluid is
accumulated in the pleural space. On auscultation in pleuritic phase, pleural friction rub
(leathery/creaking sounds during inspiration and expiration) may be heard and when
there is a pleural collection, the breath sounds will be reduced on the affected side.
Investigations
Chest radiography reveals the pleural collection as a homogeneous basal opacity
and possible pulmonary TB process or pneumonia.
Exploratory pleural puncture will extract fluid, which is an exudate rich in
proteins with positive Rivalta reaction containing many lymphocytes. Koch bacillus is
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found exceptionally, but inoculation of mice can cause TB infection when TB is the
cause.
ESR is constantly accelerated, and leukocytosis appears in the early days.
Clinical forms
a. Tuberculosis pleurisy may evolve as an acute-fibrinous pleurisy, as a
subacute pleurisy, or become prolonged as chronic pleurisy. It may be complicated by
pericardial or peritoneal tuberculosis. Treatment consists in administration of
tuberculostatics. Immediate prognosis is generally good, but the remote is reserved as
tuberculosis recurs frequently, especially in the first three years after the disease.
b. Pleurisy that accompanies or follows bacterial pneumonia occurs in either the
first week of evolution process (parapneumonic) or in the recovering period
(metapneumonic). Exudate is rich in polynuclear cells. It may resolve spontaneously
and under antibiotherapy, but sometimes tends to progress to empyema. Indications for
urgent drainage of parapneumonic effusions include:
Frankly purulent fluid
A pleural fluid pH of less than 7.2
Loculated effusions
Presence of bacteria on Gram stain or culture
c. Rheumatic pleurisy occurs in children and adolescents. It coexists with
rheumatic fever. Exudate is reduced and contains much fibrin, albumin and endothelial
cells.
d. Malignant pleurisy usually occurs in a patient over 50 years, evolving without
fever. Exudate often is bloody and cancerous cells can be detected at cytological
examination. Fluid collection reappears soon after evacuation. Prognosis is bad due to
the underlying disease (mean survival of less than 1 year).
B. Chylothorax
It is the presence of lymphatic fluid in the pleural space secondary to leakage of
the thoracic duct or one of its main tributaries. Because the thoracic duct transports up
to 4 L of chyle per day, a tear in this duct allows a rapid and large accumulation of fluid
in the chest.
Etiology
Malignant etiologies account for more than 50% of chylothorax diagnoses and
are separated into lymphomatous and non-lymphomatous. Lymphoma is the
most common cause, representing about 60% of all cases. Non-Hodgkin
lymphoma is more likely to cause a chylothorax than Hodgkin lymphoma.
Traumatic. Frequent (25%) causes of ductus thoracicus lesions are thoracic
trauma and different kind of surgeries (thoracic, cardiac, esophageal)
iatrogenic lesions.
Congenital chylothorax is seen in neonates.
Miscellaneous causes include cirrhosis, tuberculosis, sarcoidosis, amyloidosis,
and filariasis.
Idiopathic the cause is knot known.
Pseudochylothorax (cholesterol pleurisy) results from accumulation of
cholesterol crystals in a chronic existing effusion. The most common cause of
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pseudochylothorax is chronic rheumatoid pleurisy, followed by tuberculosis and poorly
treated empyema.
Symptoms and signs are those of classical pleural effusions without fever.
Diagnosis. The diagnosis is simple in case of posttraumatic (accidental or surgical) duct
lesion. More difficult is to establish the location of lesion.
The fluid extracted by thoracentesis looks milky. Pleural fluid analysis for
triglyceride content helps to confirm a diagnosis of chylothorax. A level greater than
110 mg/dL has a 99% chance that the fluid is chyle.
When the cause of chylothorax is not obvious other investigations are needed to
rule out a malignancy, such as CT, MRI, lymphography.
Treatment. The principles of treatment are:
Treatment of the underlying cause of chylothorax
Decrease the chyle production
Draining the collection
Obliterating the pleural space
Fluid and nutritional rebalancing
In large volumes of pleural collection, the first intention is to decompress the
lung. This can be achieved by thoracostomy. This is not a suitable measure for a long
period due to the consecutive nutritional depletion and metabolic complications.
Conservative treatment can be considered because the thoracic duct leak closes
spontaneously in nearly 50% of patients.
Reduction of chyle production may be achieved by several methods:
Total parenteral nutrition or a fat-restricted oral diet
Chemoradiation in patients with malignant chylothorax who are not
surgical candidates
Somatostatin administration
Indications for surgical intervention include the followings:
Drainage of more than 1 L of chyle per day for 5 days or a persistent leak
for more than 2 weeks despite conservative management.
Nutritional or metabolic complications
Loculated chylothorax, fibrin clots, or trapped lung
Postesophagectomy chylothorax
When traumatic lesion of the thoracic duct is the cause, the best treatment is duct
ligation. This is performed by a right thoracotomy or laparoscopic approach and the
duct is discovered and ligated just above the aortic hiatus.
Other surgical methods are:
Pleuro-peritoneal shunt (the chyle is driven into the peritoneal cavity
through a siliconized tube)
Pleurodesis obliterating the pleural space can be achieved chemically
(talcum which produces inflammation and fibrosis) or surgically (pleural
abrasion or pleurectomy).
Mortality and morbidity rates are approximately 10% in major clinical medical
centers.
C. Thoracic empyema
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Empyema is a collection of pus in a natural cavity of the body unlike abscess,
which is a collection of puss in a newly formed cavity.
The puss accumulation may occupy the entire pleural cavity (generalized purulent
pleurisy) or it may be trapped in smaller cavities (encysted purulent pleurisy).
Most commonly, it is a consequence of parapneumonic exudative pleurisy but it
may develop after any bacterial inoculation, from any source, of the pleural cavity. It
may progress to pleural sclerosis and may lead to lung entrapment and then to trapped
lung, conditions in which the lung is unexpandable due to visceral pleural restriction.
The inability of the lung to fully expand and fill the thoracic cavity after drainage is
associated with a chronic pleural effusion. Another cause of lung entrapment may be
malignancy. Patient may be asymptomatic or dyspnea is the main symptom with
restriction on pulmonary function testing.
Typical symptoms of thoracic empyema include cough, fever, chest pain,
sweating and shortness of breath. Digital clubbing may be present in cases of chronic
evolution.
Diagnosis is based on history, symptoms and signs and also on imaging studies
and Thoracentesis when puss is extracted.
Pleural effusion treatment depends very much on the underlying condition that
induced the effusion and it is addressed especially to that condition but also to effusions
complications: lung compression, encysted pleurisy, thoracic empyema, lung
entrapment.
Surgical treatment may be represented by:
Fluid evacuation by repeated thoracentesis or by thoracostomy
Thoracotomy or thoracoscopic approach for thoracic duct ligation in
chylothorax or for pleuro-peritoneal shunt
Pleural abrasion or pleurectomy to seal the pleural cavity (pleurodesis)
Decortication for trapped lungs to remove a thick, inelastic pleural peel
that restricts ventilation and produces progressive or refractory dyspnea
Thoracoplasty (removal of some portions of the ribs) alone or with muscle
flaps in the treatment of chronic thoracic empyema without remaining
pulmonary tissue to obliterate the pleural space.
Prognosis depends on the underlying condition, the moment of beginning the treatment
and its accuracy. In many cases, complete healing is possible with removal of the cause
which led to the pleural effusion. However, untreated or inappropriately treated,
effusions may lead to empyema, constrictive fibrosis, sepsis and other complications.
Sometimes, unfortunately, the cause cannot be cured completely such as in cancer,
systemic or congenital diseases. In these cases, the goal of treatment is to prolong life
and maintain a good quality of life.
Mortality depends primarily on the underlying disease, but pleural effusion may
endanger life by itself due to mechanical and septic complications.
2. Surgical Pathology of the Lungs
Surgical anatomy of the lungs
The lungs, two in number, are the essential organs of respiration. They are located
one on either side within the thorax, being separated from each other by the
mediastinum.
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The right lung usually weighs about 625 gr. and the left 567 gr. Their shape is
conical and presents an apex, a base, three borders, and two surfaces.
The apex extends into the base of the neck reaching from 2.5 to 4 cm. above the
level of the sternal end of the first rib. In emphysema the apex exceeds collarbone filling
the supraclavicular fossa. It has close relationships with brachial plexus and subclavian
vessels so that tumors (Pancoast-Tobias) at this level can easily invade these anatomical
structures.
The base is concave, and rests upon the diaphragm, which separates the right lung
from the right lobe of the liver, and the left lung from the left lobe of the liver, the
stomach, and the spleen. Lungs (especially the right) may be interested in intra-
abdominal pathologic processes. Examples are bilio-bronchial fistulas due to liver
hydatid cysts or abscesses.
The costal surface is convex, in contact with the costal pleura, and ribs. This
surface is most common interested in pathological thoracic processes, traumatic
(contusions, chest wounds, iatrogenic) and non-traumatic (pleurisy, pleural tumors,
etc.).
The mediastinal surface is concave being oriented towards the mediastinum
entering into direct contact with the heart and large vessels. Here is located the
pulmonary hilum, where the vessels, airways and nerves and enter or leave the lung.
Borders
The inferior border is thin and sharp
The posterior border is broad and rounded and comes in contact with the
esophagus aorta and ductus thoracicus
The anterior border is thin and sharp, and overlaps the front of the
pericardium
Segmentation
Lungs are divided by fissures in lobes, and lobes are divided in segments
separated by connective tissue. Each bronchopulmonary segments have their own
tertiary bronchi and arterial supply (two arteries) and a vein.
The left lung is divided into two lobes, an upper and a lower which is the largest
of the two, by an interlobular fissure (oblique fissure), which extends from the costal to
the mediastinal surface of the lung both above and below the hilum.
The left lung has 8-10 segments
superior lobe
1. apico-posterior (merger of "apical" and "posterior")
2. anterior
3. lingula of superior lobe
4. inferior lingular
5. superior lingular
inferior lobe
6. superior
7. anteromedial basal (merger of "anterior basal" and "medial basal")
8. posterior basal
9. lateral basal
The right lung is divided by two fissures into three lobes: superior, middle and
inferior. There are 10 segments:
superior lobe
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1. apical
2. posterior
3. anterior
middle lobe
4. lateral
5. medial
inferior lobe
6. superior
7. medial-basal
8. anterior-basal
9. lateral-basal
10. posterior-basal
Features of the bronchopulmonary segment:
1. Has a pyramidal shape, with the apex at the lung hilum
2. Is the largest subdivision of a lobe
3. Is surrounded by connective tissue
4. Has separate arterial supply from other segments and receives its own
segmental (tertiary) bronchus
5. Is drained by intersegmental veins that lie in the connective tissue around
the segment
6. Can be removed surgically without affecting the function of other
segments
Bronchial tree
The bronchi branch from the trachea. There are two main bronchi: the left and the
right. Bronchi divide into lobar bronchi, which divide into tertiary bronchi, also known
as segmental bronchi, each of which supplies a bronchopulmonary segment.
The right bronchus is wider and shorter than the left bronchus and branches into
three secondary bronchi, one passing to each of the three lobes of the right lung.
The left bronchus is smaller in diameter and about twice as long as the right
bronchus. It is also more horizontal and more susceptible to obstruction. It branches into
the secondary bronchi for the inferior and the superior lobes of the left lung.
The segmental bronchi divide into many primary bronchioles which divide into
terminal bronchioles, each of which then gives rise to several respiratory bronchioles,
which go on to divide into 2 to 11 alveolar ducts. There are five or six alveolar sacs
associated with each alveolar duct. The alveolus is the basic anatomical unit of gas
exchange in the lung.
Arterial supply
The lungs have two arterial systems: one functional that brings deoxygenated
blood to lungs and which belongs to the lesser circulation, and the other one, that
provides lung nutrition belonging to the systemic circulatory system.
The functional arterial blood (deoxygenated) comes from the right heart directed
by the pulmonary trunk, which divides into the two pulmonary arteries. These arteries
follow a similar branching pattern to the bronchi, accompanying them as they divide.
They continue branching and terminate as capillaries in the walls of the alveoli.
Oxygenated blood supply is derived from the bronchial arteries, which arise from
the descending aorta.
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It is important not to confuse the pulmonary arteries with bronchial arteries. The
former carry the deoxygenated blood from the right side of the heart in order to pick up
oxygen. The latter are systemic, carrying oxygenated blood, like all systemic arteries, to
supply the tissues of the bronchi.
Venous drainage
There are also two types of venous drainage:
One functional, which carries the oxygenated blood from the alveolar capillaries
towards larger pulmonary veins and then into the left atrium. Each lobe of the lung is
drained by a pulmonary vein. Veins do not accompany the artery and bronchus in
segments, but instead run in the segmental boundaries.
One systemic represented by the bronchial veins which are much smaller, and
drain blood from the regions supplied by the bronchial arteries. The left vein drains into
the hemiazygos vein, which then crosses behind the carina to empty into the azygos
vein and thereby the superior vena cava. The right bronchial vein drains directly into the
azygos vein.
Lymphatics
The lymphatic drainage of the lung is important in oncology because it represents
a route of spread for cancerous cells.
There are two lymphatic plexuses: one superficial just beneath the pleura and a
deep plexus, which accompanies the bronchi.
The deep plexus drains lymph into subsegmental, segmental, interlobar and
bronchial lymph nodes. The superficial plexus usually has no nodes. The two plexuses
combine at hilum, where tracheobronchial nodes are located (station 10R/10L). In
general, lymph will pass to superior tracheobronchial nodes from the upper lobes, and to
the inferior tracheobronchial nodes from the lower lobes, but there are anastomoses
between the lymphatic channels which may cause unexpected spread. The lymph is
drained then into the mediastinal lymph nodes.
Intercongress Meeting of the European Soc. of Pathology in Prague, Czech; 8.9 -
12.9.2012
Mediastinal nodes have numerous stations to allow surgical and radiological correlation.
Station Location Description
2R / 2L Upper Paratracheal Right - Bounded superiorly by the apex of the lung, laterally
by the pleura, medially by the trachea, inferiorly by the
intersection of the caudal border of the brachiocephalic artery
and trachea.
Left As for right, except the inferior boundary is formed by
the superior part of the arch of aorta
4R / 4L Lower Paratracheal Right Bounded above by station 2R, inferiorly by the caudal
margin of the azygos vein.
Left Bounded superiorly by station 2L, laterally by the
ligamentum arteriosum, and inferiorly by the carina
5 Aortopulmonary Located lateral to the ligamentum arteriosum and above the
pulmonary artery / trunk
6 Anterior mediastinum The space located anterior to the trachea, pulmonary arteries,
aorta and ligamentum arteriosum
7 Subcarinal The mediastinum beneath the carina, medial to station 9
8 Paraoesophageal The mediastinum posterior to the trachea, on either side of the
oesophagus
9R / 9L Pulmonary Ligament Located within the pulmonary ligament, inferior to the root of
the lung
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10R / 10L Tracheobronchial Right - Superior to the carina / right main bronchus, medial to
the origin of the right upper lobe bronchus, and inferior to
station 4R
Left Lateral and superior to the carina / left main bronchus,
medial to the origin of the left upper lobe bronchus, and
inferior to station 4L
11R / 11L Interlobar Located between the junction of the lobar bronchi
12R / 12L Lobar Located along the lobar bronchi
13R / 13L Segmental Located along segmental bronchi
14R / 14L Subsegmental Located along subsegmental bronchi
Innervation
Sympathetic nervous fibers come from the paravertebral ganglia. They produce
bronchodilation, vasoconstriction and reduce secretion of mucous glands in the bronchi.
Parasympathetic fibers are derived from the vagus nerves (X) and produce
bronchoconstriction, vasodilatation and increase the mucous secretion.
Nervous fibers form plexuses, enter the lung at hilum and accompany the
bronchial tree and the vessels.
Lungs function
The lungs are part of the body's respiratory system, which is one of the most
important systems in preserving life. A person can live for weeks without food and a
few days without water but only a few minutes without oxygen.
The principal function of the lungs is to exchange gases between the air and the
blood. In the lungs, carbon dioxide is removed from the blood and oxygen from inspired
air enters the bloodstream (hematosis =the arterialization of the blood in the lungs).
A person at rest breathes about 6 liters of air a minute. Heavy exercise can increase the
amount to over 75 liters per minute. The lungs have the greatest surface exposed to air
of about 28 sqm at rest (but up to 93 sqm during a deep breath) compared to the skin
with its surface area of approximately 1.9 sqm.
The lungs are spongy organs, which in inspiration are filled with oxygenated air,
and during expiration, the air loaded with carbon dioxide is exhaled. Air movement in
and out the lung is called ventilation and several anatomical structures participate in this
process. Inspiration is an active process produced by the respiratory muscles and
exhalation is a passive process based on lung elasticity and compliance. Gas exchange
occurs through the alveolar-capillary membrane as oxygen moves into and carbon
dioxide moves out of the bloodstream.
The most common parameters of lungs function are:
Tidal Volume (TV):, the volume of air that is inhaled or exhaled with each
normal breath.
Expiratory Reserve Volume (ERV): the maximal amount of air forcefully
exhaled after a normal inspiration. The amount of exhaled air will be more
than was just inhaled.
Inspiratory Reserve Volume (IRV): the maximal amount of air forcefully
inhaled after a normal inhalation.
Residual Volume (RV): the amount of air remaining in the lungs after the
deepest exhalation possible.
Vital Capacity (VC): The maximum amount of air that can be exhaled
after the fullest inhalation possible. Vital capacity is the sum of the tidal
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volume, the inspiratory reserve volume, and the expiratory reserve
volume.
Total Lung Capacity (TLC): the sum of the vital capacity and the residual
volume. The average total lung capacity of an adult human male is about 6
liters of air.

Average lung volumes in healthy adults
Value (litres) Volume
In men In women
Inspiratory reserve volume 3.3 1.9
Tidal volume 0.5 0.5
Expiratory reserve volume 1.0 0.7
Residual volume 1.2 1.1

Lung capacities in healthy adults
Average value (litres) Volume
In women
Derivation
In men
Vital capacity 4.6 3.1 IRV plus TV plus ERV
Inspiratory capacity 3.8 2.4 IRV plus TV
Functional residual capacity 2.2 1.8 ERV plus RV
Total lung capacity 6.0 4.2 IRV plus TV plus ERV plus RV

LUNG ABSCESSES
Lung abscess is a localized infection as a consequence of liquefactive necrosis of
the lung characterized by a pus-filled cavitary lesion. The formation of multiple small
(< 2 cm) abscesses is occasionally referred to as necrotizing pneumonia or lung
gangrene.
In the pre antibiotic era, lung abscesses were a devastating disease with a
mortality over 30%.
Etiology
The most frequent cause of abscess is pulmonary aspiration of content from oral
cavity, esophagus or stomach. This was demonstrated by the presence of the same types
of bacteria in the wall of the abscess, as in mouth.
A less frequent cause is hematogenous seeding of the lungs due to suppurative
thromboembolism. In this case, usually there are multiple small abscesses in lungs.
In addition, in rare cases lung abscess develops as a secondary abscess spread
from an extrapulmonary organ or bronchiectasis.
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Other causes may be infected pulmonary cysts, or hydatid cysts, or infected
necrotized lung tumor.
Classification. Lung abscesses can be classified as:
1. Based on pathogenesis:
a. Primary abscesses
b. Secondary abscesses
2. Based on evolution
a. Acute - less than 4-6 weeks
b. Chronic
3. Based on etiologic agent
The etiologic agent
The most common aerobe pathogens are streptococci and staphylococci.
Staphylococcus aureus (MRSA) causes a very serious and fulminate necrotizing
pneumonia in young adults and children.
Anaerobe negative germs are represented especially by Actinomyces sp. and
Bacteroides sp., Fusobacteria.
The most frequent gram-negative bacteria is Klebsiella.
Patients immunocompromised may have infection with Nocardia, Mycobacterium
sp, or fungi.
Some people, especially those from developing countries, are at risk of
developing TB abscess. There are rare cases due to amebic infection (eg, with
Entamoeba histolytica), paragonimiasis, or Burkholderia pseudomallei.
Pathogenesis
The most common cause of lung abscess is aspiration of digestive content. This is
possibly accidentally, without any prior condition, or is favored by a number of
conditions such as:
Loss of cough reflex:
o Cerebral vascular accidents
o Drug overdose
o Alcoholism
o Post-op state or coma from any cause
Trouble with deglutition:
o Neurological disorders
o Esophageal diseases (DIverticulum, achalasia, GERD)
Post obstructive pneumonia:
Lung cancer
Foreign body aspiration
85% of abscesses are located in the superior segments of right lower lobes, left
lower lobes and axillary subsegments of anterior and posterior segments of the right
upper lobe.
Gravitational forces determine the site of aspiration. Position of the patient at time
of aspiration determines the segment the aspiration is most likely to occur.
The right lower lobe is the most common site for aspiration in sitting position. In
this position, gravity facilitates lodging of the aspirate to basal segments of the right
lower lobe due to the straight line with the trachea of the right main bronchus while the
left takes of at an angle.
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In supine position with the patient on back, superior segment of right lower lobe
is the most dependent segment.
Clinical Picture
The onset and symptoms depend largely on the etiologic agent. In most cases,
especially produced by anaerobic bacteria, the onset is insidious and may go even 3-4
weeks until the patient seeks medical attention. Sometimes the onset is acute with a
pneumonia, which then progresses to cavitation and formation of an abscess.
Symptoms: Cough, low grade fever, anorexia, weight loss and expectoration of
foul smelling sputum (lack of foul smell does not exclude lung abscess, as 50% of
anaerobic infections do not produce a foul smell). Patients may develop hemoptysis or
pleurisy
Physical signs are variable depending on the severity and extent of the disease,
and the patient's health status and co-morbidities.
o Inspection on clinical general examination can be observed: fever, pale
skin, edentation and gingival problems, purulent sputum, fetid halitosis,
digital clubbing and signs depending on the associated pathology
o Palpation pectoral fremitus diminished in coexisting pleurisy
o Percussion dullness over the area of abscess or basal when associated
with pleurisy
o Auscultation - decreased breath sounds, bronchial breath sounds, course
inspiratory crackles, or when pleural effusion is associated the absence of
breath sounds over the effusion
Investigations
Imagistic and bacteriologic investigations are the most important in diagnosis and
therapeutic conduit.
Chest radiography. The typical radiographic appearance is of an irregularly
shaped cavity with an air-fluid level inside. This typical image must be differentiated
from other similar images in case of: cavitating cancer, TB, simple cyst, hydatid cyst,
esophageal diverticulum, encysted pleural effusion, empyema, etc. Embolic pulmonary
disease often causes multiple cavitations, and TB typically involves the apices.
The wall thickness of a lung abscess progresses from thick to thin and from ill-
defined to well-circumscribed as the surrounding lung infection resolves. The wall of
the abscess is typically thick and the inner surface irregular but is less commonly
nodular, which raises the possibility of cavitating carcinoma.
CT scan is not routinely needed but may be useful in differential diagnosis.
Bacteriologic diagnosis
For an accurate detection of the causative germ, harvested material must not be
contaminated. Unfortunately, the expectorated sputum does not yield useful results for
anaerobic culture because the oral cavity is extensively colonized with anaerobes.
Uncontaminated material may be obtained for anaerobic culture from the followings:
Blood culture in rare cases blood cultures are positive
Pleural fluid (if empyema is present) not every abscess is associated
with empyema
Transtracheal aspirate
Transthoracic pulmonary aspirate FNA guided by CT invasive method
Surgical specimens invasive method
655
Fiberoptic bronchoscopy with protected brush limited experience
Bronchoalveolar lavage with quantitative cultures
On the other hand, if antibiotherapy was initiated most likely the culture will not
be positive.
Flexible fiberoptic bronchoscopy is performed to exclude a bronchopulmonary
carcinoma.
Cavitary pulmonary lesions are not always caused by infection. Noninfectious
causes include the followings:
Bullae with air-fluid level
Bronchiectasis
Lung cancer
Lung infarction
Nodular silicosis, nodule with central necrosis
Pulmonary embolism
Pulmonary sequestration
Sarcoidosis
Wegener's granulomatosis
Treatment
Antibiotic therapy. If uncontaminated cultures can be obtained, then antibiotic
therapy will be guided by antibiogram. Traditionally, penicillin alone was used and
produced satisfactory results but due to the developing of germs resistance, it is no
longer recommended. Clindamycin is the most popular antimicrobial due to its good
intracellular uptake and its stability in low pH and poor vascularity. Imipenem also has
excellent activity against anaerobes. Antibiotics must be given for several weeks.
As in any other abscesses, evacuation of puss is very important in healing.
Pulmonary abscesses may be drained by various methods.
Postural drainage is the most often used and in association with antibiotics is
sufficient. The patient should be trained to obtain the optimal position for an efficient
drainage. Bronchodilators and aerosols facilitate the evacuation of puss.
Drainage by bronchoscopy is very efficient but it cannot be used daily. It is
reserved when postural drainage is not efficient and the cavity is enlarging.
Percutaneous chest tube drainage. To avoid spilling of pus into pleural cavity the
tube must be inserted through a region where the parietal and pulmonary pleura are
sealed together (as a consequence of inflammatory processes or surgically induced by
different kind of procedures - pleurodesis).
Surgery
Surgery in now days is very rarely indicated. The patients will be operated only in
case of complications such as massive hemoptysis, airway obstruction, empyema,
pulmonary gangrene or if there is a suspected neoplasm, or congenital lung
malformation. The surgical procedure performed is either lobectomy or
pneumonectomy.
Lung abscess complications may include spread of infection to other lung
segments, bronchiectasis, empyema, and bacteremia with metastatic infection such as
brain abscess.
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PULMONARY HYDATID CYST
Hydatid cyst of the lungs is a parasitic disease caused by infestation with larvae
of the dog tapeworm Echinococcus granulosus. Infestation is produced by ingestion of
tapeworm eggs that contaminate the water, vegetables or other foods, or when hands are
lead to mouth without washing them after handling soil or petting a dog whose fur
contains the parasite.
The larva hatches out of the egg in the intestines and migrates through the portal
vein into liver and then to lungs and other organs where it will remain as a large larval
tapeworm cyst (hydatid cyst).
Some authors suggest that lungs should be the favorite location of hydatid cyst
because through the lung is the route of the entire blood of the body, whereas other
authors claim that the liver is the most frequent location because this is the first filter for
blood coming from intestines.
The portal circulation and so liver may be by-passed if the hexacanth embryos
enters a lacteals in the intestinal villi and then via the lymphatic duct into the cava vein.
Morphopathology
The cyst is a kind of round tumor of variable size (up to 15 or even 20 cm in
diameter) bordered by a thick wall and filled with a liquid more or less clear and
scolices (tapeworm heads).
Hydatid cysts of the lung can be central or peripheric. The latter, through their
ever increasing growth, can come into contact with the pleura.
There are 3 phases of evolution:
1. The initial phase comprised between infestation moment and diagnosable
pulmonary lesion. At 6 hours after infestation, a catarrhal alveolitis develops,
then at 48 hours, a pulmonary condensation appears and at 10 days, a
pulmonary nodule called echinococcus pseudo-tubercle (a nodule that
resembles a tuberculosis granule but is caused by a microorganism other
than Mycobacterium tuberculosis).
2. The phase of constituted cyst when it is detectable by chest radiography. In
this phase the pericyst is formed by mechanical compression on the lung
tissue and by local allergic reaction. The pericyst consists of three layers:
The inner is formed by hyaline tissue
The middle formed by connective tissue vessels and many
eosinophils
The external layer much thicker formed by alveoli condensation
Between the cyst and the pericyst, there is a virtual space that allows the
enucleation of the hydatid membrane. As the cyst continues to grow, it will compress
other pulmonary structures such as bronchi leading to fistula formation.
3. The phase of complications The most frequent complication is the rupture
of the cyst and evacuation of its content into the bronchial tree. Rupture is
also possible into the pleural cavity.
Clinical picture
There are no symptoms for a very long period, in many cases the cyst being found
incidentally on a chest radiography. Symptoms depend on many factors such as:
location, number, volume, evolution stage and patients age.
In uncomplicated stage symptoms may be:
657
Chest pain inconstant symptom present mostly in large volume cyst
Dyspnea in large or multiple cysts
Cough rare in this stage but when appears it announces a complication
Hemoptysis is also rare in this phase
Allergic reactions urticaria (hives)
Physical signs in this stage are also poor and they are represented by the same
findings as in any pulmonary condensation.
In the complicated phase, the most frequent symptoms are caused by cyst rupture.
The main symptom of rupture into the bronchial tree is the vomica when the
content of the cyst is expelled. The vomica is an acute episode preceded and associated
with intense cough. The volume of fluid expelled depends on volume of the cyst. The
fluid is watery clear and may contain the whole hydatid membrane or just part of it. If
the entire membrane is expelled spontaneous, healing of the disease may occur. If only
the fluid is eliminated, the membranes will remain in the adventitial cavity where the
retraction of the cavity's walls and the narrowness of the bronchial opening make the
expulsion of the membrane almost impossible (incarcerated membrane)
Vomica may induce more complications such as:
Aspiration of the fluid into the bronchial tree followed by Mendelson
syndrome and even death.
Anaphylactic shock that must be treated urgently with corticosteroids
Hemoptysis more or less important
Infection of the remnant cavity which is usually the rule
Diagnosis is established based on three elements: anamnesis (occupation, endemic
zones and previous operation for liver hydatid cyst, are important), clinical picture
(vomica is the most characteristic) and paraclinical examinations.
Imagistic investigations are represented especially by chest radiography and the
more reliable radioscopy that may highlight the cyst respiration sign (Brjovschi-
Linberg sign) the cyst modifies its diameters during respiration.
Radiologic investigation remains the milestone in diagnosis. It can specify the
number, location, size complication of cysts.
The simple hydatid cyst is represented by a round or polylobed mass of 2-20 cm
diameter with well defined borders. Calcifications are exceptional.
The complicated cyst has many stages with different radiological aspects
CT examination brings more information about the pulmonary mass specifying its
cystic nature, studying the membranes and complications.
Ultrasound examination may be useful especially in peripheral location of the
cyst asserting the liquid content and observing the hydatid sand and the daughter cysts.
Abdominal ultrasound examination is also useful in diagnosis of an associated liver
hydatid cyst.
Laboratory tests: are not always necessary in positive diagnosis. In uncertain
cases, it may be helpful the following:
Eosinophilia over 5%
The indirect hemagglutination test and the enzyme-linked
immunosorbent assay (ELISA) have a sensitivity of 40% in pulmonary
echinococcosis and are the initial screening tests of choice.
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Immunodiffusion and immunoelectrophoresis demonstrate antibodies to
antigen 5 and provide specific confirmation of reactivity.
The ELISA test is useful in follow-up to detect recurrence.
Differential diagnosis is made with other pulmonary or mediastinal masses such as:
Pulmonary TB tuberculum and especially TB cavern. Patients history,
nodular images, calcifications, PPD skin test and bacteriology help the
diagnosis.
Bronchopulmonary cancer CT scan and bronchoscopy with biopsy are
helpful in diagnosis.
Simple or contaminated pulmonary cysts
Lung abscess
Encysted pleurisy
Esophageal diverticulum
Aortic aneurism
Mediastinal tumors
Treatment
The single efficient treatment is the surgical one. In case of uncomplicated cyst,
the aims of surgical treatment are: to eradicate the parasite and remove the hydatid
membrane and to treat the residual cavity preserving as much lung tissue as possible.
The approach is through a thoracotomy.
There are many possible surgical techniques. Parts of them remove the membrane
by opening the cyst and other remove the entire cyst without opening it.
ARCE procedure the fluid is slowly evacuated by puncture.
FINOCHETO procedure the fluid is rapidly evacuated by aspiration
and then the membrane is removed.
BARRET procedure evacuates a small quantity of fluid and then opens
the cyst, evacuates the rest of liquid and the membrane.
Incision of the pericyst and removing the intact cyst (Barret)
Segmentectomy or lobectomy are applied in rare instances only when the
pulmonary parenchyma is very affected (advanced pericystic
pneumonitis). The most conservative treatment should be used to save as
much lung tissue as possible.
The most difficult decision is the attitude toward the residual cavity. There are a
lot of procedures, some of them do not close the cavity, other close or collapse the
cavity using different techniques of suture or seal the cavity using intercostal muscular
flaps. The cavity is thoroughly irrigated. The bronchial openings, with or without
capitonnage (the folding of the pericystic zone by sutures) of the residual cavity, are
then closed and the pleural space is drained.
Choosing one of these techniques, depend on surgeon experience, location of the
cyst and other important features of the cyst.
The WHO guidelines recommend chemotherapy with albendazole (ABZ) and
mebendazole (MBZ) for inoperable primary liver or lung echinococcosis and for
patients with multiple cysts in two or more organs.
659
Preoperative chemotherapy may reduce the risk for recurrence of echinococcosis
and facilitate the operation. Postoperative is recommended in cycles of 1 month with 2-
weeks interval between cycles.
Prognosis
With appropriate treatment, the prognosis is excellent.
Postoperative complications (12% and 19%) are influenced by the size and
number of cysts and the type of operation. The most common complications are pleural
infection and prolonged air leakage.
The operative mortality in large series doesn't exceed 2%.
The recurrence rate is also very low (0% - 3%).
BRONCHOPULMONARY CANCER
Epidemiology. Cancer of the lung is the most common cancer in the world.
It is a very life-threatening cancer and one of the most difficult cancers to treat.
The bronchial tree and the pulmonary parenchyma are the sites where tumors may
develop as primary tumors or secondary tumors (metastases). Lung cancer is one of the
most frequent locations of cancerous disease, being in many countries the leading cause
of death in men as well as in women. It was responsible for 1.3 million deaths in 2004.
Estimated new cases and deaths from lung cancer (non-small cell and small cell
combined) in the United States in 2012: 226,160 and death: 160,340.
According to the U.S. National Cancer Institute, approximately one out of every
14 men and women in the U.S. is diagnosed with cancer of the lung.
For both sexes, Hungary has the highest rate of lung cancer, followed by French
Polynesia and the United States of America. About 55 per cent of lung cancer cases
occur in less developed countries. The lowest incidence in Eastern, Western and Middle
Africa. For women the United States of America has the highest rate of lung cancer,
followed by Denmark and Canada. (http://globocan.iarc.fr/ )
Lung cancer occurs predominantly in elderly. Almost 70% of people diagnosed
with lung cancer are over 65 years of age, while less than 3% of lung cancers occur in
people under 45 years of age.
The incidence of lung cancer is greater in men than in women, but in women the
lung cancer is accounting for almost twice as many deaths as breast cancer. Women
tend to be slightly younger, by an average of two years, at the age of diagnosis than men
are. Unlike men, a great percentage of women that develop lung cancer have never
smoked (20% occur in lifelong nonsmokers).
Blacks are much more likely than whites to get lung cancer from smoking
cigarettes.
Higher incidence and mortality rates are reported among men from lower socio-
economic groups.
Risk factors:
Tobacco smoke is by far the most important risk factor.
Radon is a radioactive gas that cannot bee seen, smelled, or tasted.
People who work in mines may be exposed to radon.
Asbestos (construction) and other substances (arsenic, chromium, nickel,
soot, tar etc. - chemical industries) have an increased risk of lung cancer.
Air pollution slightly increases the risk of lung cancer.
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Family history of lung cancer.
Personal history of lung cancer.
Age over 65.
Morphopathology
Lung cancer may be primary (derived from cells of the lung parenchyma or
bronchial tree) or secondary represented by metastases from variable other primary
tumors.
The most frequent primary tumors are represented by those derived from
bronchial tree. The vast majority of lung cancers are carcinomas that arise from
epithelial cells. The cells of origin may be: basal cells, secretory cells and endocrine
cells.
There is a multitude of histological types. The World Health Organization in
2004, reviewed the histological classification of malignant lung tumors establishing two
major groups, with important clinical practice, evolution and different treatment:
4. Non small cell lung carcinoma (NSCLC),
5. Small cell lung carcinoma (SCLC)
Histological types of bronchopulmonary cancer
Carcinoma with squamous cells
(epidermoid)
Papillary carcinoma
Carcinoma with clear cells
Carcinoma with small cells
Basaloid carcinoma
Carcinoma with small cells
(micro cell)
Carcinoma with small mixed cells
Large-cell carcinoma
(macro-cell)
Large-cell neuroendocrine carcinoma
Combined large cells neuroendocrine
carcinoma
Basaloid carcinoma
Lymphoepithelioma-like carcinoma
Carcinoma with clear cells
Large-cell carcinoma with rhabdoid phenotype
Adenocarcinoma Acinar adenocarcinoma
Papillary adenocarcinoma
Mixed adenocarcinoma
Bronchioloalveolar carcinoma
Mucinous
Non-mucinous
Combined
Mucus producing solid carcinoma
Fetal adenocarcinoma
Mucinous cystadenocarcinoma
Signet cells adenocarcinoma
Adenocarcinoma with clear cells
Sarcomatoid carcinoma Pleomorphic carcinoma
Spindle cell carcinoma
Giant cell carcinoma
Carcinosarcoma
Pulmonary blastoma
Carcinoid Typical carcinoid
Atypical carcinoid
Salivary gland tumors Mucoepidermoid carcinoma
Adenoid cystic carcinoma
Epithelial-myoepithelial carcinoma
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Pre-invasive lesions Squamous carcinoma in situ
Atypical adenomatous hyperplasia
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia
The most common type is adenocarcinoma (40%), followed by squamous cell
carcinoma (25%), small cell carcinoma (20%) and large cell carcinoma (10-15%), other
types representing about 5%.
In Romania, on the first place is epidermoid carcinoma (45%), followed by
adenocarcinoma (25%), large cell carcinoma (10%) and small cell carcinomas (20%).
Morphological features
Lung cancer appears mostly in the right lung (6/4 compared to the left lung) more
frequently in the superior lobes and in ventral segments.
The macroscopic appearance of the carcinoma with central location is that of a
solid tumoral mass, with irregular shape, variable in size, generally smooth, with gray or
white aspect on section. The endobronchial surface is typically ulcerated. By grows it
produces partial or complete obstruction of the bronchial lumen, producing atelectasis,
bronchiectasis and secondary pulmonary suppurations.
Carcinoma with peripheral location looks hard, irregular and presents a clear
separation from the surrounding pulmonary tissue. It has a homogeneous aspect on
section surface. Unlike small lesions, the large tumors produce central necrosis with
cavitation.
Epidermoid cancer: two thirds appear in the central zone of the lung, grow
slowly and metastasize late. Those located at periphery produces cavitation.
Adenocarcinoma: 70% are located in the peripheral zone of the lung.
Appears on lung scars and interstitial fibrosis. It has a medium rate of grows
and rarely cavitates. Metastasize early.
Large-cell carcinoma: can be located centrally or peripherally; metastasize
rapidly; the peripheral forms produce cavitation and the prognosis is bad.
Small-cell carcinoma: are located mostly in the central region rapidly
invading the hilum and mediastinal lymph nodes. It is the most aggressive
form.
Bronchial carcinoids: are generally small (3 cm-4 cm or less) tumors when
diagnosed and occur most commonly in people under 40 years of age.
Spread of the lung cancer
There are three ways of spread: direct extension, lymphatic and via the blood
vessels.
By direct extension, the tumor invades the nearby anatomical structures: lung
parenchyma, bronchial tree, pericardium, esophagus, pleura, thoracic wall and other
structures. The bronchial extension of the tumor is about 1.9 cm above the tumor and
that is the reason for resecting the bronchus more than 2-2.5 cm above the macroscopic
limit of the tumor.
Lymphatic extension depends on histological type and the size of the tumor. The
small-cell carcinomas metastasize predominantly on this way and less the epidermoid
carcinoma. In case of tumors less then 1 cm in diameter, metastases in lymph nodes
were not found but for tumors between 1 and 2 cm, the lymph node metastases were
found in 12% of cases.
Hematogenous spread is due to blood vessels invasion. Invasion of great vessels
is a bad prognosis factor.
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The most frequent sites of metastases are: brain, liver, bones and adrenal glands.
Less frequent sites are: kidney, pancreas and other organs.
Stadialization:
Primary Tumor (T)
TX Primary tumor cannot be assessed or tumor proved by the presence of malignant cells
in sputum or bronchial washings but not visualized by imaging or bronchoscopy
Tis - Carcinoma in situ
T1 - Tumor 3 cm or less in greatest dimension, surrounded by lung or visceral pleura,
without bronchoscopic evidence of invasion more proximal than the lobar bronchus* (i.e.,
not in the main bronchus)
T2 - Tumor with any of the following features of size or extent:
More than 3 cm in greatest dimension
Involves main bronchus, 2 cm or more distal to the carina
Invades the visceral pleura
Associated with atelectasis or obstructive pneumonitis that extends to the hilar region
but does not involve the entire lung
T3 - Tumor of any size that directly invades any of the following: chest wall (including
superior sulcus tumors), diaphragm, mediastinal pleura, or parietal pericardium; or tumor in
the main bronchus less than 2 cm distal to the carina but without involvement of the carina;
or associated atelectasis or obstructive pneumonitis of the entire lung
T4 - Tumor of any size that invades any of the following: mediastinum, heart, great vessels,
trachea, esophagus, vertebral body, or carina; or tumor with a malignant pleural or
pericardial effusion or with satellite tumor nodule(s) within the ipsilateral primary tumor
lobe of the lung
Regional Lymph Nodes (N)
N1 - Metastasis to ipsilateral peribronchial and/or ipsilateral hilar lymph nodes and
intrapulmonary nodes involved by direct extension of the primary tumor
N2 - Metastasis to ipsilateral mediastinal and/or subcarinal lymph node(s)
N3 - Metastasis to contralateral mediastinal, contralateral hilar, ipsilateral or contralateral
scalene, or supraclavicular lymph node(s
Distant Metastasis (M)
MX - Presence of distant metastasis cannot be assessed
M1 - Distant metastasis present. Specify sites
Pre-invasive lesions:
A. mild dysplasia
B. moderate dysplasia
C. severe dysplasia
D. carcinoma in situ
E. atypical adenomatous hyperplasia
F. diffuse idiopathic neuroendocrine cells hyperplasia
Clinical picture
Clinical manifestations of lung cancer have a great diversity in relation to
anatomo-clinical form, histological type and stage. In some asymptomatic patients, the
tumor is discovered accidentally on chest radiographs, but most cancers are diagnosed
by the development of new or worsening of existing signs or symptoms.
There are no pathognomonic symptoms or signs for lung cancer, but they can be
classified into four categories:
1. Clinical manifestations due to local tumor growth and intrathoracic spread
2. Signs and symptoms due to distant metastases
3. Unspecific general symptoms
4. Paraneoplastic syndromes
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Frequency (%) Symptoms
Small-cell
lung carcinoma
(SCLC)
Non-small-cell
lung carcinoma
(NSCLC)
Cough 50-76 40
Dyspnea 34-40 30-40
Chest pain 35-36 25-40
Hemoptysis 15-23 15-35
Pneumonia 21-25 13-24
Vocal cord paralysis 15 unusal
Superior cava vein syndrome 12 <10
Pleurysis 10-15 15
Pancoast-Tobias syndrome rare 3
Pericarditis unusual rare
Cough - is the most frequent symptom in lung cancer especially in those with
central location. A new cough or a change in the character of the cough in a smoker or a
former smoker should raise concern for lung cancer. A cough that persists more than a
few weeks and worse over time should be suspected as caused by a lung cancer.
Hemoptysis - cough up blood or sputum streaked with blood. Lung cancer
accounts for up to 20% of cases of hemoptysis.
Dyspnea - usually is due to the blockage to the flow of air in part of the lung,
pleural effusion or the spread of the tumor throughout the lungs.
Chest pain - appears in about 25% of people with lung cancer. The pain is dull,
aching, and persistent. Lung cancers may press on nerves, resulting in pain in shoulder,
chest, back or arm even before they cause cough or dyspnea.
Wheezing or hoarseness - may be signs of tracheo-bronchial compression due to a
tumor.
Repeated respiratory infections, such as bronchitis or pneumonia, can be a sign of
lung cancer due to obstruction that predisposes to infections.
Vocal cord paralysis is due to recurrent laryngeal nerve compression or invasion
by tumor.
Superior cava vein syndrome - is the result of the direct obstruction of the
superior vena cava by right lung upper lobe tumors and/or mediastinal
lymphadenopathy. It is manifested by dyspnea, facial swelling, head fullness, cough,
arm swelling, chest pain, dysphagia, orthopnea, distorted vision, hoarseness, stridor,
headache, nasal stuffiness, nausea, pleural effusions, venous distension of the neck and
chest wall, upper extremity edema, mental changes, plethora, cyanosis, papilledema,
stupor, and even coma.
Esophageal compression by lung cancer is manifested by difficulty of swallowing
or pain with swallowing.
Heart function disorders represented by abnormal heart rhythms, blockage of
blood flow through the heart, or fluid in the pericardial sac may be other symptoms of
lung tumor extension into the mediastinum.
Pancoast-Tobias syndrome - caused by an apical (superior pulmonary) malignant
neoplasm of the lung which invades the surrounding tissues and produces: an ipsilateral
invasion of the cervical sympathetic plexus leading to Horner's syndrome (miosis,
enophthalmia, palpebral ptosis), shoulder and arm pain (brachial plexus invasion C8-
664
T2) leading to wasting of the intrinsic hand muscles and paraesthesiae in the medial side
of the arm. Less commonly unilateral recurrent laryngeal nerve palsy producing
unilateral vocal cord paralysis (hoarse voice bovine cough), and/or phrenic nerve
involvement. There may be arm oedema secondary to the compression of blood vessels.
It is estimated that approximately 60-70% of patients with lung cancer have
metastases at presentation and 1/3 of them have symptoms due to these metastases that
has an unfavorable prognostic significance. Unspecific symptoms related to advanced
stages of cancer are: anorexia, weight loss, fatigue, fever, anemia.
Paraneoplastic syndromes occur in approximately 10-20% of patients. They are
represented by a series of non-specific disorders in relation with various organs and
systems, produced in relatively early stages of the disease. These syndromes are not
related to the size or location of the lung cancer and do not necessarily indicate that the
cancer has spread outside the chest. They are due to secretion of hormones or other
substances by the tumor tissue and so may disappeared after tumoral resection or may
recur in case of relapse or metastasis.
Paraneoplastic symptoms occur more frequently in small-cell carcinoma and
rarely in epidermoid carcinoma and adenocarcinoma. Include the following major
categories: endocrine, neurological, cardiovascular, musculoskeletal and skin
manifestations.
Endocrine
Bartter syndrome (a rare inherited defect in the thick ascending limb of the
loop of Henle in kidney characterized by hypokalemia, alkalosis, and
normal to low blood pressure) appears in 5-10% of cases exclusively in
small-cell carcinoma.
Hypercalcemia in squamous cancer
Gynecomastia in clear-cell carcinoma
Others
Neurological
Eaton-Lambert syndrome - a rare autoimmune disorder that is characterized
by muscle weakness of the limbs. It is the result of an autoimmune reaction,
where antibodies are formed against presynaptic voltage-gated calcium
channels in the neuromuscular junction. It appears exclusively in small-cell
carcinoma.
Subacute sensory neuropathy - there is a degeneration of the dorsal root
ganglia manifested by ataxia, but with little development of motor weakness.
This is common in SCLC and there is no cure.
Subacute cerebral degeneration - progressive arm and leg bilateral ataxia and
other neurological signs (dementia, nystagmus, ophthalmoplegia, extensor
plantar signs, dysarthria and arm involvement). This degeneration is
progressive and disabling. The condition can precede cancer by weeks or
years appearing most commonly in breast and ovarian cancer. Improvement
is possible following successful cancer treatment.
Limbic encephalopathy is a form of autoimmune disease caused by
autoantibody against the limbic system of the brain (Anti-Hu, which is
associated with small-cell carcinoma of the lungs). It is manifested by
memory deficits, headache, irritability, sleep disturbance, delusions,
hallucinations, agitation, seizures and psychosis.
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Visual paraneoplastic syndrome - Loss of visual acuity and loss of visual
field, impaired color vision. Most often occurs with small cell carcinoma of
lung.
Subacute necrotizing myelopathy - ascending motor and sensory loss, which
are very rapid due to the loss of gray and white matter of the spinal cord.
This leads to paraplegia. It may be shown on an MRI.
Cardiovascular and blood
Thrombotic endocarditis
Migratory thrombophlebitis
Hypercoagulation
Renal
Glomerulonephritis
Nephrotic syndrome
Skin and musculoskeletal
Hypertrophic pulmonary osteoarthropathy - clubbing - an abnormal
proliferation of skin and bone tissue, primarily in the hands and feet, most
commonly associated with NSCLC (especially adenocarcinoma)
Acanthosis nigricans - brown to black hyperpigmentation of the skin usually
found in body folds such as the posterior and lateral folds of the neck, the
axilla, groin, umbilicus, forehead, and other areas.
Dermatomyositis - The main symptoms include skin rash and symmetric
proximal muscle weakness which may be accompanied by pain.
Investigations are aimed to:
1. Confirm the diagnosis
2. Determine the histological type
3. Determine the stage and extension
Imaging investigations (X-ray, computed tomography, MRI) and bronchoscopy
provide the maximum information to assess lung cancer.
In most cases, conventional chest radiography is the first that suggests the
diagnosis of lung cancer. Computed tomography (CT) is extremely useful in
determining tumor extension, to highlight adenopathies (only 64% of cases are
identified by standard radiologic examination, but 95% by CT) and the presence of
metastases (liver, adrenal, brain, etc).
Newer techniques, such as positron emission tomography (PET) and helical
(spiral) CT, are improving the ability to detect small cancers. Oncologists frequently use
PET-CT scanners, which combine the PET and CT technology in one machine, to
evaluate patients with suspected cancer.
After a lung cancer is suspected based on imaging, a sample of tissue is required
to confirm the diagnosis and determine the type of cancer.
Sputum cytology is the easiest way to do this, but its use is limited to those tumors
that extend into the airways. Sputum cytology is not always accurate and can miss some
cancer cells. Usually, a sample of tissue directly from the tumor is needed.
A way to obtain the tissue sample is with bronchoscopy.
If the tumor is too far away from the major airways, the specimen can be obtained
by percutaneous needle biopsy under CT guidance. Sometimes, a specimen can only be
obtained by a thoracotomy, thoracoscopy or thoracocentesis (associated effusions).
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Mediastinoscopy offers the possibility to take samples of enlarged lymph nodes to
determine if inflammation or cancer is responsible for the enlargement.
Virtual bronchoscopy is a 3D reconstruction of the tracheobronchial tree based
on data acquired from CT and/or MRI scanning. The advantage is that it is not invasive
but the limitation is that it cannot perform biopsy.
Magnification bronchovideoscopy is a combination of two systems: a video
system for high magnification and a fiber optic for scope's direction. The system allows
differentiation between dysplasia and other pre-invasive lesions.
Endobronchial ultrasonography appreciates the tumor extension, being used to
analyze small lesions.
Optical Coherence Tomography (OCT) is a way of obtaining images with high
resolution in real time. OCT identifies microscopic characteristics of cells, glands,
crypts, lymphatics and vessels. Unlike endobronchial ultrasonography image is limited
in depth about 2 mm compared to about 5 mm in case of ultrasound 30 MHz.
Anatomo-clinical forms of lung cancer
THE SQUAMOUS CARCINOMA
It is the most frequent histopathological form encountered in Romania (45%)
compared to Western countries where it represents only 25-30%.
The cancerous cells are derived from the ciliated cells of the bronchial epithelium
as a result of local irritation and carcinogenic effects of smoking. The tumoral growth is
relatively slow (doubling time of 90-130 days).
The macroscopic aspect on sections is gray-whitish, with keratinized areas. It can
be well or poor differentiated.
The tumor is located in central region on large bronchi in 80% of cases and
cavitation is relatively frequent.
The tumor produces cough and hemoptysis. Bronchoscopy and sputum
cytological examination are the main methods of diagnosis. It produces bronchial
obstruction with consecutive pneumonia. The main route of spread is through lymphatic
vessels and less through blood vessels. Paraneoplastic syndromes are rare represented
by hypercalcemia and clubbing.
Squamous cell carcinoma may benefit from radical surgery but it is not
responding to radio and chemotherapy.
ADENOCARCINOMA
Ranks second as frequency in Romania (25%) but it represents the main subtype
in U.S. and European Union (30-45%). The incidence has increased by 10% in the last
25 years in Europe.
It appears predominantly in young men (<50 years) and females regardless of age,
in non-and ex-smokers.
Tumor growth is relatively slow, the doubling time being 160 days. It appears as
a white-gray lobulated mass, usually located peripherally often affecting the pleura and
producing metastases in the pleural cavity. Sometimes is associated with a scar
impregnated by anthracotic pigment.
Peripheral adenocarcinomas originate from epithelium or glands of the mucosa of
small bronchi included in areas of fibrosis or old scars. The tumor may contain
667
calcospherites (tiny, spheroidal, concentrically laminated body containing accretive
deposits of calcium salts, probably as the result of degenerative changes in the
fibrovascular stroma).
Adenocarcinomas tend to metastasize early to regional lymph nodes and remotely
to brain.
A history of chronic interstitial lung disease (scleroderma, sarcoidosis,
tuberculosis, interstitial pneumonia, etc) is frequently associated with this type of cancer
and this is the reason why the term of "scar carcinoma is given to lung
adenocarcinomas.
Usually peripheral adenocarcinoma is asymptomatic being diagnosed incidentally
by imagistic methods of investigation. On chest radiography the aspect area is as a
bipolar lesion represented by the tumoral nodule and the enlarged regional lymph nodes.
Bronchoscopy can detect only tumors with central location.
Local development is without cavitation but frequently invading the pleura and
the thoracic wall.
The tumor has a low sensitivity to chemotherapy and radiotherapy.
BRONCHIOLO-ALVEOLAR CARCINOMA (BAC)
It is a subtype of adenocarcinoma, which has been extensively studied in recent
years due to rising incidence especially among younger non-smoking women. BAC is
more likely to affect non-smokers, women, and Asians than other forms of lung cancer.
BAC has three subtypes: mucinous, non-mucinous or mixed.
While bronchiolo-alveolar non-mucinous carcinomas are usually manifested as
solitary nodules and favorable prognosis, the mucinous type tends to spread and form
satellite nodules or pneumonic condensations and have a poor prognosis. Most patients
with solitary BAC, non-invasive, and size <2 cm, can be treated by only resection.
The clinical picture is represented by irritative cough and severe respiratory
failure. Radiological bronchiolo-alveolar carcinoma may be peripheral (single node,
multiple nodules, diffuse), condensation pneumonia-like or disseminated
carcinomatosis. These aspect tumors are suitable for differential diagnosis with
metastatic adenocarcinoma of the pancreas, colon, breast, stomach, and kidney. It is not
uncommon for BAC to be mistaken for pneumonia or other lung diseases before it is
diagnosed.
Peripheral located BAC determines bronchial hypersecretion (>500 ml / day),
explaining bronchogenic dissemination.
A diagnosis of BAC requires a sample of tissue, and fine-needle aspiration
biopsy.
The solitary well differentiated BAC, have a better prognosis than other forms,
while diffuse or multinodular form does not respond to therapy.
LARGE CELL CARCINOMA
It is a rare histological type, representing approximately 9% of all lung cancers.
The origin of this tumor is found in bronchial mucous glands, especially in
the peripheral bronchi. It appears as a large necrotic mass, most commonly peripheral
invading the pleura and neighboring structures.
Over 90% of giant cell carcinoma is associated with other histological types such
as: adenocarcinoma or epidermoid carcinoma.
668
Evolution is similar to squamous cell carcinoma, with cavitation and
hematogenous relatively late dissemination. Occasionally it is associated with
neuroendocrine syndromes, which seems to respond better to chemotherapy.
SMALL LUNG CELL CARCINOMA (SCLC)
It represents 20 to 25% of lung cancers.
The of origin of tumoral cells are represented by Kulchitzky cells which have
neuroendocrine activity (peptide hormones and growth factors) and are located into the
submucosal layer of the bronchial tree.
SCLC is considered a distinct pathological entity due to its aggressive biological
features (growth rate with a fast duplication time of approximately 30 days), with early
metastatic potential and rapid dissemination via lymphatic and blood vessels.
The presence of neuroendocrine activity explains the high frequency of endocrine
paraneoplastic syndromes.
Small cell carcinoma occurs in 90% of cases as a central mass, soft, friable,
whitish, with necrosis, which may produce bronchial obstruction. Less than 5% of cases
have peripheral locations.
It has rapid growth with early metastases in the liver, CNS, bone, adrenal glands,
pancreas and kidney.
Even if the tumor has an increased sensitivity to chemotherapy, the prognosis
unfavorable due to early metastases. Up to 80% of patients die in the first year after the
positive diagnosis.
Treatment is based on histological type, stage of disease (particularly in NSCLC),
associated diseases and prognosis. Bronchopulmonary cancer treatment includes
surgery, radiotherapy, and chemotherapy, curative or palliative.
Chemotherapy and radiotherapy are most widely used in both non-small cell and
small cell lung cancers. In a small number of cases, they may lead to a cure. These
therapies result in shrinking of the tumor and are effective in relieving symptoms
prolonging the life of patients.
Chemotherapy is sometimes coupled with radiation therapy, especially for small
cell lung cancer which is aggressive and has often spread to distant parts of the body by
the time of diagnosis.
The brain is sometimes treated with radiation even if no tumor is present there,
called prophylactic cranial irradiation.
In recent years, new molecular therapies for the treatment of NSCLC have been
added - epidermal growth factor receptor inhibitors (EGFR), agents that target vascular
endothelial cells, farnesyl transferase inhibitors, retinoids, proteosome inhibitors and
inhibitors of raf / MAP kinase (activated mitotic protein-kinases).
At the time of diagnosis, only about 2025% of lung cancers can potentially be
cured, primarily by surgery.
NSCLC are good candidates for surgery, which is the treatment of choice in
early-stages, but surgically removal of tumor does not always result in a cure.
Unfortunately, relapses are very frequent.
In general, surgery is not used for SCLC, because this aggressive cancer requires
chemotherapy and radiation therapy and because in most cases the cancer has spread
beyond the lungs at time of diagnosis.
669
Possible operations, aimed to remove the lung tumor are represented by more or
less extensive lung resection, such as segmentectomy, lobectomy or pneumectomy,
mainly depending on tumor location.
Even if the tumor is in early stage, having a small size, being in a good location,
and could be technically easily removed, in many cases the operation cannot be
performed due to associated diseases. The Pulmonary Function Testing (PFT)) must be
performed prior to operation to determine whether the amount of lung remaining after
surgery will be able to provide enough oxygen and breathing function.
Most patients with a preoperative forced expiratory volume in one second of
greater than 2.5 L are able to tolerate pneumonectomy. With a forced expiratory volume
in one second of 1.1-2.4 L, a lobectomy is possible. With a forced expiratory volume in
one second of less than 1 L, patients are not considered candidates for surgery.
Following lung volume reduction surgery, mortality rates are between 5% and
9%. The perioperative mortality rate is 6% for pneumonectomy, 3% for lobectomy, and
1% for segmentectomy. These rates reflect improvements in anesthesia and surgical
techniques.
In recent years, new palliative endoscopic treatment has been developed
prolonging and improving the life quality of this patients.
Different endoscopic techniques can be classified as follows: (from
http://annonc.oxfordjournals.org/ by guest on May 15, 2012, 248)
Techniques enabling rapid removal of obstruction (e.g. mechanical
debulking/resection, ND:YAG-laser resection, electrocautery) in case of life-
threatening obstruction
Techniques enabling delayed removal of obstruction (e.g. cryotherapy,
endobronchial irradiation, photodynamic therapy) in cases of non-critical
stenosis
Techniques enabling maintenance of airway patency (e.g. stenting)
Techniques enabling symptom control such as hemoptysis (e.g. argon
plasma coagulation, electrocautery, ND:YAGlaser therapy,)
Techniques for increased local tumor control (e.g. intratumoral injection of
gene therapy vectors)
Indications for interventional bronchoscopic procedures in lung cancers are:
Life-threatening obstruction of the central airways (trachea, carina, main
bronchi)
Central airway obstruction causing symptoms (dyspnea, atelectasis, post-
obstructive pneumonia, hemoptysis or reducing the airway lumen >50%)
Inoperable early lung cancer amenable to endoscopic treatment
Light-amplified stimulated emission of radiation (LASER) has been used for
several decades for airway disorders. Nd:YAG (neodymium-yttrium aluminum garnet)
laser, using an infrared wavelength of 1064 nm. The primary indication for this
technique is palliation of airway obstruction by either primary or metastatic
malignancies.
Electrocautery has the same indications but its major advantages compared with
laser therapy are its very low cost, despite equal levels of efficiency, and probably
superior levels of safety.
670
671
Cryotherapy is the application of extreme cold for local destruction of living
tissue. The main indications are treatment of non-critical malignant airway obstruction,
hemoptysis and treatment of superficial early lung cancer.
Argon plasma coagulation (APC) is a new method using ionized argon plasma
delivered through special probes with flexible bronchoscope to obtain airway patency
and haemostasis.
Airway stenosis can be managed also by stent insertion. A large variety of stents
have been used in the last two decades but the common problems associated include
migration, granulation tissue formation and mucus plugging.
Endobronchial irradiation (brachytherapy) is currently achieved by placing a
highly radioactive source (iridium 192 probe) by means of a flexible bronchoscope at
the desired location. It can be used for curative intent treatment of early superficial lung
cancer, for treatment of primary non-resectable bronchial carcinoma and for palliative
treatment involving the removal of obstruction for primary or recurrent endoluminal
lung cancer.

Despite all current efforts in prevention (smoking cessation), early detection and
early treatment, development of new chemotherapeutic agents and combined modality
of treatments, the overall prognosis of lung cancer is still dismal, with 5-year survival
remaining at about 13% over the last few decades.

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Marius Florin Coro

Assoc. Professor, MD, Ph.D.

"Capsulotomy - sectioning the pancreatic capsule allows pan

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