Meigs’

Syndrome
I. Introduction

Meigs’ syndrome is defined as the triad of benign ovarian tumor with

ascites and pleural effusion that resolves after resection of the tumor. The
ovarian tumor in Meigs’ syndrome is a fibroma.

In 1934, Salmon described the association of pleural effusion with benign

pelvic tumors. In 1937, Meigs and Cass described 7 cases of ovarian
fibromas associated with ascites and pleural effusion.1 In 1954, Meigs
proposed limiting true Meigs’ syndrome to benign and solid ovarian tumors
accompanied by ascites and pleural effusion, with the condition that removal
of the tumor cures the patient without recurrence. Histologically, the benign
ovarian tumor may be a fibroma, thecoma, cystadenoma, or granulosa cell
tumor.

Pseudo-Meigs syndrome consists of pleural effusion (an example of which

can be seen in the image below), ascites, and benign tumors of the ovary
other than fibromas. These benign tumors include those of the fallopian tube
or uterus and mature teratomas, struma ovarii, and ovarian leiomyomas.2
This terminology sometimes also includes ovarian or metastatic
gastrointestinal malignancies.
 Chest radiograph showing left-sided pleural effusion.

Atypical Meigs characterized by a benign pelvic mass with right-sided pleural

effusion but without ascites has been reported at least twice. As in Meigs
syndrome, pleural effusion resolves after removal of the pelvic mass.

Pseudo-pseudo Meigs syndrome includes patients with systemic lupus

erythematosus and enlarged ovaries.

Etiology of ascitic fluid

The pathophysiology of ascites in Meigs syndrome is speculative. Meigs
suggested that irritation of the peritoneal surfaces by a hard, solid ovarian
tumor could stimulate the production of peritoneal fluid. Samantha and Black
studied ovarian tumors accompanied by ascites and found that only tumors
larger than 10 cm in diameter with a myxoid component to the stroma are
associated with ascites.4 These authors believe that their observations favor
secretion of fluid from the tumor as the source of the ascites.

Other proposed mechanisms are direct pressure on surrounding lymphatics

or vessels, hormonal stimulation, and tumor torsion. Development of ascites
may be due to release of mediators (eg, activated complements, histamines,
fibrin degradation products) from the tumor, leading to increased capillary
permeability.

Origin of pleural effusion

The etiology of pleural effusion is unclear. Efskind and Terada et al theorize
that ascitic fluid is transferred via transdiaphragmatic lymphatic channels.
The size of the pleural effusion is largely independent of the amount of
ascites.

• Efskind's study: Efskind injected ink into the lower abdomen of a

woman with Meigs syndrome and found that the ink particles
accumulated in the lymphatics of the pleural surface within half an
hour. Blockage of these lymphatics prevented accumulation of pleural
fluid and caused an increase in ascitic fluid.
• Terada and colleagues' study: In 1992, Terada and colleagues injected
labeled albumin into the peritoneum and found that the maximum
concentration was detected in the right pleura within 3 hours.

Nature of the ascitic and pleural fluid

Ascitic fluid and pleural fluid in Meigs syndrome can be either transudative or
exudative. Meigs performed electrophoresis on several cases and
determined that pleural and ascitic fluids were similar in nature. Tumor size,
rather than the specific histologic type, is thought to be the important factor
in the formation of ascites and accompanying pleural effusion.

Frequency
United States
Ovarian tumors are more prevalent in upper socioeconomic groups. Ovarian
fibroma is found in 2-5% of surgically removed ovarian tumors, and Meigs
syndrome is observed in about 1%. Ascites is present in 10-15% of those
with ovarian fibroma and hydrothorax in 1%, especially with larger lesions.

International
Prevalence is unknown.

Mortality/Morbidity
Although Meigs syndrome mimics a malignant condition, it is a benign
disease and has a very good prognosis if properly managed. Life expectancy
after surgical removal of the tumor mirrors that of the general population.

Age
The incidence of ovarian tumor begins to increase in the third decade and
increases progressively to peak in the seventh decade. Meigs syndrome in
prepubertal girls with benign teratomas and cystadenomas has been
reported.

Treatment
Medical
Medical care of patients with Meigs syndrome is intended to provide
symptomatic relief of ascites and pleural effusion by means of therapeutic
paracentesis and thoracentesis.

Surgical Care

• Exploratory laparotomy with surgical staging is the treatment of

choice.
o Perform a frozen section of the ovarian mass during exploratory
laparotomy. If the frozen section is consistent with benign tumor,
conservative surgery (salpingo-oophorectomy or oophorectomy)
is appropriate.
o Findings of lymph node biopsies and omentum and pelvic
washings are negative for malignancy if these procedures are
performed during surgery.
• In women of reproductive age, perform unilateral salpingo-
oophorectomy.
• In postmenopausal women, options include bilateral salpingo-
oophorectomy with total hysterectomy and unilateral or occasionally
bilateral salpingo-oophorectomy.
• In prepubertal girls, options include wedge resection of ovary and
unilateral salpingo-oophorectomy.
• The cure rate after either type of surgery is high and recurrence is
rare.
III. History
Patients with Meigs syndrome may have a family history of ovarian cancer.
The chief complaints are vague and generally manifest over time.

• Fatigue
• Shortness of breath
• Increased abdominal girth
• Weight loss
• Nonproductive cough
• Bloating
• Amenorrhea for premenopausal women
• Menstrual irregularity

History of Present illness

Four months prior to admission, the patient claimed that she was
suffering from menstrual irregularity and after two months her monthly
period never came. The patient also reported feelings of bloating.

Past Medical History

The patient has never been admitted to any hospital except for routine
check-ups for minor complaints like cough and colds.

Family History
Birth order of Patient: 2nd

Total no. of siblings: 3

No. of living siblings: 2

Serious diseases/illnesses of siblings: None

Cause of death/serious illnesses of siblings: None

Mother side: Has known history of ovarian cancer.

Father side: No known history of any disease.

IV. Physical Assessment
Positive signs include the following:

• Vital signs
o Tachypnea
o Tachycardia
• Lungs
o Dullness to percussion
o Decreased tactile fremitus
o Decreased vocal resonance
o Decreased breath sounds are noted, suggesting pleural effusion.
Pleural effusion is mostly observed on the right side, but it can
also be left sided.
• Abdomen
o Examination may reveal a small or large pelvic mass, or no mass
may be felt.
o Ascites is present, with shifting dullness and/or fluid thrill.
• Pelvis: Examination reveals a pelvic mass.
VI. Pathophysiology

Predisposing Precipitating
factors: factors:
Age: 64 years old Unknown
Gender: Female

Etiology:
Idiopathic

Ascitic Pleural Benign

fluid effusion ovarian
tumor
Irritation to the Through the route of
peritoneal transfer via
surfaces transdiaphragmatic Tumors larger than
lymphatic channels 10 cm in diameter
with myxoid
Production of
component to the
peritoneal fluid
stroma

Direct pressure on
Associated with
surrounding pai
ascites
lymphatics or n
vessels Lung
Favored secretion of
compression
Hormonal fluid from the tumor
stimulatio Weight gain as the source of the
n ascites

Tumor
torsion
Increase in
abdominal
Release of mediators Difficulty of
girth
like histamines, breathing
activated components
and fibrin degradation
products

Increased
capillary
permeability
VII. Laboratory

Lab studies for patients with Meigs syndrome include the following:
• CBC count: This study provides information about hemoglobin,
hematocrit, and platelet levels. A low hemoglobin count requires
further workup, including reticulocyte count, total iron-binding
capacity, and iron and ferritin levels. Anemia in patients with Meigs
syndrome is most likely due to iron deficiency. Anemia can be
corrected emergently by blood transfusion in patients undergoing
surgery for Meigs syndrome. Anemia can be treated with iron
supplementation postoperatively.
• Basic metabolic profile: Studies of sodium, potassium, chloride,
bicarbonate, blood urea nitrogen, creatinine, and glucose levels are
included. These electrolytes are checked before the patient undergoes
surgery. If necessary, corrections of these electrolytes are made.
• Prothrombin time: Prothrombin time is checked before surgery. If
elevated, it is a marker of coagulopathy. Elevated prothrombin time is
corrected before surgery, either by administering vitamin K to the
patient or by transfusing fresh frozen plasma.
• Other than serum electrolytes and CBC count, the study of interest is
the serum cancer antigen 125 (CA-125) test. Tumor marker serum
levels of CA-125 can be elevated in Meigs syndrome, but the degree of
elevation does not correlate with malignancy. In fact, a normal CA-125
level does not exclude the possibility of malignancy.8 The CA-125 level
is not used as a screening test. The highest reported level of CA-125
after laparotomy is 1808 U/mL. This would be a false-positive result.
Imaging Studies

• Chest radiography confirms pleural effusion.

• Abdominal and pelvic ultrasound confirms the ovarian mass and
ascites.
• CT scan of the abdomen and pelvis
o CT scan confirms ascites and ovarian, uterine, fallopian tube, or
broad ligament mass.
o No signs of distant metastasis are observed.

Procedures

• Paracentesis: Ascitic fluid is mostly transudative. Findings are negative

for malignant cells but can be positive for reactive mesothelial cells.
• Thoracentesis: Pleural fluid is usually transudative. Findings can be
exudative and negative for malignant cells.