Meigs syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: Demons Meigs syndrome; Meigs-Cass syndrome; Meigs-Salmon syndrome

Overview

Meigs syndrome (also known as "Meigs-Cass syndrome") is defined as the triad of ascites, pleural effusion, and benign ovarian tumor. Meigs syndrome was first discovered by Otto von Spiegelberg, a German gynecologist, in 1866. There is no classification system established for Meigs syndrome. Other variants of Meigs syndrome include pseudo-meigs syndrome, and atypical Meigs' syndrome. Meigs syndrome may be caused by either ovarian fibroma, Brenner tumor, ovarian thecoma, or granulosa cell tumor. The pathogenesis of Meigs syndrome is characterized by a transudative process. The prevalence of benign ovarian tumors is approximately 2-10%, and only 1-2% develop Meigs syndrome. Meigs syndrome is more commonly observed among postmenopausal women. The median age at diagnosis is approximately 50 years. The majority of patients with Meigs syndrome are asymptomatic. Early clinical features include bloating, fatigue, and shortness of breath. Computed tomography is the imaging modality of choice for Meigs syndrome. On conventional radiography, Meigs syndrome is characterized by blunting of the costophrenic angle or fluid within the horizontal or oblique fissures (250-600 ml of fluid is required before the pleural effusion becomes evident). Surgery is the mainstay of therapy for Meigs syndrome. Exploratory laparotomy with surgical staging is the most common approach to the treatment of Meigs syndrome. Once diagnosed and successfully treated, patients with Meigs syndrome are followed-up every 6 months. Follow-up testing includes; serum cancer antigen 125, ultrasound, and basic metabolic profile.

Historical Perspective

• Meigs syndrome was first discovered by Otto von Spiegelberg, a German gynecologist, in 1866.
• Meigs syndrome is named after Joe Vincent Meigs, an American gynecologist who defined Meigs syndrome as the presence of ascites, hydrothorax, association with benign ovarian tumor.
• In 1900, the first therapeutical surgical approach was developed by Albert Jean Octave Demons to treat Meigs syndrome.

Classification

• There is no classification system established for Meigs syndrome.
• Other variants of Meigs syndrome include pseudo-meigs syndrome, and atypical Meigs' syndrome.

Pathophysiology

• The pathogenesis of Meigs syndrome is characterized by a transudative process.
• The transudative process consists in the formation of peritoneal and pleural effusion to the filtration of interstitial fluid in the peritoneum through the tumor capsule, and the diffusion to the pleural space.
• There are no genes associated with the development of Meigs syndrome.
• On gross pathology, marked ovarian enlargement, watery cut surface, and no necrosis are characteristic findings of Meigs syndrome.
• On microscopic histopathological analysis, variable stromal luteinization, marked edema of stroma surrounding follicles, and stroma around vessels are characteristic findings of Meigs syndrome.

Causes

• Meigs syndrome may be caused by either ovarian fibroma, Brenner tumor, ovarian thecoma, or granulosa cell tumor.
• In general, Meigs syndrome may be caused by any benign ovarian tumor.

Differentiating Meigs syndrome from other Diseases

• Meigs syndrome must be differentiated from other diseases that cause bloating, fatigue, and shortness of breath such as:

• Ovarian cancer
• Cirrhosis
• Colon cancer
• Tuberculosis
• Milroy's disease

Epidemiology and Demographics

Prevalence

• The prevalence of Meigs syndrome is unknown.
• The prevalence of benign ovarian tumors is approximately 2-10%, and only 1-2% develop Meigs syndrome.

Age

• Meigs syndrome is more commonly observed among postmenopausal women.
• The median age at diagnosis is approximately 50 years.

Gender

• Meigs syndrome affects exclusively females.

Race

• There is no racial predilection for Meigs syndrome.
• Meigs syndrome usually affects females with higher socioeconomic status.

Risk Factors

• Common risk factors in the development of Meigs syndrome are enlarged adnexal mass ( > 5cm), co-existing pleural effusion, and family history of any form of cancer.

Natural History, Complications and Prognosis

• The majority of patients with Meigs syndrome are asymptomatic.
• Early clinical features include bloating, fatigue, and shortness of breath.
• If left untreated, the majority of patients with Meigs syndrome may progress to develop respiratory failure, ovarian torsion, and hypoproteinemia.
• Common complications of Meigs syndrome include portal vein obstruction, inferior vena cava obstruction, and thoracic duct obstruction.
• Prognosis is generally good, and the 5 survival rate of patients with Meigs syndrome is approximately 100%.

Diagnosis

Diagnostic Criteria

• The diagnosis of Meigs syndrome is made when the following 3 diagnostic criteria are met:

• Ascities
• Pleural effusion or hydrothorax
• Benign ovarian tumor

Symptoms

• Meigs syndrome is usually asymptomatic.
• Symptoms of Meigs syndrome may include the following:

• Bloating
• Fatigue
• Weight-loss
• Shortness of breath
• Cough
• Menstrual irregularity

Physical Examination

• Patients with Meigs syndrome are usually well-appearing.
• Physical examination may be remarkable for:

• Increased abdominal perimeter
• Decreased/absent breath sounds
• Reduced chest expansion
• Rapid rate of breathing

Laboratory Findings

• There are no specific laboratory findings associated with Meigs syndrome.
• In some cases, unspecific laboratory findings may include abnormal serum cancer antigen 125 test and abnormal prothrombin time.

Imaging Findings

• Computed tomography is the imaging modality of choice for Meigs syndrome.
• On conventional radiography, Meigs syndrome is characterized by blunting of the costophrenic angle or fluid within the horizontal or oblique fissures (250-600 ml of fluid is required before the pleural effusion becomes evident)
• On computed tomography, findings include: presence of ascites, and characterization of ovarian mass.
• Ultrasound may demonstrate and confirm the presence of ovarian mass and ascities.

Other Diagnostic Studies

• Meigs syndrome may also be diagnosed using thoracentesis or paracentesis.
• Findings on paracentesis include transudative ascitic fluid, and presence of reactive mesothelial cells.
• Findings on thoracentesis include transudative pleural fluid, and negative for malignant cells.

Treatment

Medical Therapy

• There is no medical treatment for Meigs syndrome.

Surgery

• Surgery is the mainstay of therapy for Meigs syndrome.
• Exploratory laparotomy with surgical staging is the most common approach to the treatment of Meigs syndrome.
• Meigs syndrome is a benign condition and the ascites and pleural effusion resolves after resection of the primary pelvic tumor.

Prevention

• There are no primary preventive measures available for Meigs syndrome.
• Once diagnosed and successfully treated, patients with Meigs syndrome are followed-up every 6 months.
• Follow-up testing includes; serum cancer antigen 125, ultrasound, and basic metabolic profile.