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1:4000 births
Brief history:
1670: W. Durston first description of an EA
1696: T. Gibson first description of an EA with TEF
1939: Ladd i Leven first doctors who treated using
Embryology
4th week:
laringo-tracheal bud;
tracheo-esophageal
septum;
separation of the
diverticulumby the
primitive foregut, in a
cranial-caudal direction
Genetic factor
- families known with more members
having EA
- 9% of twins with EA
Esophageal malformations
classification
- Gross EA without TEF (6-8%)
Esophageal malformations
classification
- Gross EA with distal TEF (85%)
EA with distal
and proximal TEF
Esophageal malformations
classification
- Gross -
TEF without
AE H-type (3
- 5%)
esohageal stenosis
membranous atresia
Prenatally - ultrasonography!
Failure of nasogastric tube insertion
X-ray
- gastric air
- associated pulmonary
malformations
- contrast agent +/ esophagoscopy?
bronchoscopy ?
swallowing disorders
Functional defects in breathing-swallowing
coordination
Esophageal diverticula
Hydro-electrolytic balance
Venous access by central catheter
Approach
- transpleural
- extrapleural
b) spiral KIMURA
Delayed primary
anastomosis techniques
Howard bougienage of the
superior end
colon esophagoplasty
Postoperative complications
Anastomotic fistula
Anastomotic stenosis
TEF recurrence
Swallow disorders
Gastroesophageal reflux
Esophageal motility disorders, dysphagia
Prognosis
Waterston - 1962
I
II
III
> 2500 gr
pneumonia (-)
Associatied anomalies (-)
1800 2500 gr
Moderate pneumonia OR
Associated anomalies
< 1800 gr OR
> 1800 gr
Severe pneumonia
Severe anomalies
survival
95%
68%
6%
Prognostic
Spitz - 1994
survival
1500 gr
major CCM (-)
97%
II
< 1500 gr or
Major CCM (+)
59%
III
22%
Definition
Incidence:
1:4000 births
Brief history:
1679 Riverius first description of a CDH
1796 Morgagni describes the anterior defect in CDH
1848 Bochdalek describes the postero-lateral defect in CDH
Embriology
4 components are responsible of diaphragmatic development:
1. Septum transversum
2. Pleuroperitoneal membranes
3. Dorsal mesoesophagus
4. Thoracic wall
2. posterolateral BOCHDALEK
3. esophageal hiatus
Causes:
- Latent closure of the pleuroperitoneal duct
- Hepatic protection on the right
Physiopathology
Lung mass
Surfactant
Pulmonary compliance
Oxigenation
CO2 discharge
Pulmonary a. hypoplasia
Hypoxia
Acidosis
Pulmonary a. pressure
Physiopathology
Vasoactive substances:
prostaglandins
tromboxanes
leucotrienes
Cardiac function and cardiac outpout worsens
Fiziopatologie - 3
Ventilation in
pressures
Iatrogenic barotrauma
Fragile alveola
breaking
Pulmonary
tension
Emphysema
treatment
4. Pulmonary hypertension can be reversible in the
Radiologic examination
Thoraco-abdominal plain x-ray
hydroaeric images
deviation of the heart and
trachea
grey abdomen, opaque
X-ray examination
Contrast subtance use:
Tratament
-
- nitroglycerin
- nitroprusside
- prostaglandines
wanted side effects (pulmonary HYPO tension).
High frequency with low pressures ventilations
Extracorporeal membrane oxygenation (ECMO)
- cardiopulmonary by-pass, most frequently venous-arterial, carotid a.
and jugulary v.
- ECMO using in severe cases of superior digestive bleeding can
decrease mortality from 80% to 60%
Observations
Surgery must wait as long as possible in order to
decrease pulmonary hypertensions and raise
pulmonary compliance
Surgical treatment
abdominal approach
Large defect:
- prosthetics
Prognostic:
- rezervat
Complicaii:
- gastroesophageal reflux
- pulmonary complications
- mechanical occlusion,
volvulus, relapse
Retrosternal hernia
dimension
Discreet clinical signs respiratory and/or digestive
Herniated gut strangulation
- pain
- vomiting
- bloating
- hiccups
- no stools or gas
- retrosternal or prehepatic sounds
Retrosternal hernias
Anterior-posterior position and profile plain X-ray
Hiatal hernias
AKERLUND classification:
1. brachioesophagus
2. paraesophageal hernias
3. sliding hernias:
most frequent in children
they also gather cardioesophageal relaxation
mobile cardia
Tablou clinic
Vomiting with blood secondary anemia
Retrosternal pain
Hiccupe
Dyspnea
Cough
Aspiration pneumopathy
Radiologic examination
Gastroesophageal reflux
Easy to see dilation on plain x-ray
Contrast substance
Treatment
1. Medical and postural
- good results, tried first before surgery
2. Surgery
Objectives:
1` abdominal reduction hernia
2` diaphragm reconstruction
3` anti-reflux procedure
- NISSEN fundoplication wrapping the gastric
fundus around the terminal esophagus reinforcing
the closing function of the lower esophageal
sphincter