Meigs Syndrome (Including Pseudo-Meigs Syndrome)

Acquired disorder associated with a triad presentation of benign tumors of the ovary or other female pelvic organs, leading to ascites and pleural effusion. Histologically, the benign ovarian tumor might be a fibroma, thecoma, cystadenoma, or granulosa cell tumor.

Meigs Salmon Syndrome.

In 1934, Salmon described the association of pleural effusion with benign pelvic tumors. In 1936, Joe Vincent Meigs, an American obstetrician, and John W. Cass, an American physician, described seven cases of ovarian fibromas associated with ascites and pleural effusion.

In the United States, ovarian tumors are more prevalent in upper socioeconomic groups. Ovarian fibroma is found in 2 to 5% of surgically removed tumors and Meigs syndrome represents 1%. The prevalence is unknown. It is seen from the third decade, with a peak in the seventh decade.

Acquired disorder.

Meigs syndrome is characterized by the presence of ascites and pleural effusion in the presence of a benign ovarian tumor. Pseudo-Meigs syndrome presents in a similar fashion and is associated with benign tumors of other pelvic organs. The cause of the ascites is uncertain but may be a result of mechanical irritation of the peritoneum, venous or lymphatic obstruction, or production of vasoactive substances by the tumor. The development of pleural effusions, which are usually right sided and may be massive, probably is caused by passage of ascitic fluid via transdiaphragmatic lymphatics or directly via diaphragmatic defects. Classically, the ascites and pleural effusions disappear following tumor removal. Development of ascites may be caused by release of mediators (e.g., activated complements, histamines, fibrin degradation products) from the tumor, leading to increased capillary permeability.

The presence of a pleural effusion and ascites in the presence of a benign pelvic tumor and the disappearance of effusions following excision of the tumor are a sine qua non in making the diagnosis. Other causes of ascites and/or pleural effusions must be excluded. The presence of elevated CA125 levels usually is suggestive of ovarian malignancy. Elevated levels of CA125 have been reported in Meigs syndrome in the absence of malignancy.

Meigs Syndrome: Usually presents as abdominal pain and/or distension. If the ascites is significant or in the presence of a pleural effusion, dyspnea may be a presenting feature. Nonproductive cough, malaise, and weight loss may accompany the other clinical features.

Pseudo-Meigs Syndrome: Consists of pleural effusion, 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.

Atypical Meigs Syndrome: Characterized by a benign pelvic mass with right-sided pleural effusion but without ascites. As in Meigs syndrome, pleural effusion resolves after removal of the pelvic mass.

Respiratory status must be evaluated. A recent chest radiograph is mandatory; if the pleural or peritoneal fluid is interfering with respiratory mechanics, preoperative drainage of the collections must be considered. Other causes of pleural effusions must be excluded, including other malignancies and infections.

Respiratory function must be optimized. Laparotomy will be required for removal of the ovarian or pelvic tumor.

No known specific implications with this condition.