Meigs’ Syndrome - Symptoms, Diagnosis, and Treatment

Introduction:

Meigs’ syndrome is uncommon in women less than 30 years of age. The condition most commonly affects women of postmenopausal age (after 50 years). The incidence is high in women between 60 years to 70 years of age. Joe Vincent Meigs and John W Cass were the first to describe the association of benign ovary tumors with ascites and pleural effusion. The condition was later named Meigs’ syndrome by Rhodes and Terrel.

What Is Meigs Syndrome?

A syndrome is a collection of different signs and symptoms. The classical indications for Meigs’ syndrome are as follows:

• Benign ovary tumor: A non-cancerous growth on the ovaries such as fibroma, thecoma, and Brenner tumor.

• Ascites refer to abnormal fluid build-up in the abdomen, primarily due to underlying liver damage.

• Pleural effusion: Fluid collection between the lungs and chest cavity is called pleural effusion.

• Pericardial effusion is abnormal fluid accumulation in the sac surrounding the heart. It occurs with other signs of Meigs’ syndrome in a few individuals.

• The ascites and pleural effusion clear off after removing the ovarian tumor.

• It is also known as Demons-Meigs’ syndrome.

What Is Atypical Meigs Syndrome?

Rarely, in a few women, either pleural effusion or ascites occurs in association with benign ovarian tumors. It is called atypical or incomplete Meigs’ syndrome.

What Are the Signs and Symptoms?

The typical symptoms of Meigs syndrome are listed below:

• Benign Ovarian Tumor: It rarely occurs in children and adolescents associated with Gorlin syndrome (a condition that affects various body parts and increases the risk of developing cancer or non-cancerous growth).

1. The ovarian tumor produces little or no symptoms in women until it starts secreting hormones. If the tumor produces excess androgen hormone, it increases the development of male characteristics (virilization) in women.
2. If estrogen is produced in excess, it causes abnormal bleeding, endometrial cancer, and early puberty in children.
3. The other features include fatigue, weight loss, swollen feet, the abdomen swells due to an enlarged ovarian tumor, leaking of urine (urinary incontinence), and uterine prolapse (the pelvic muscles weaken and do not provide support to the uterus).

• Pleural Effusion: The fluid usually builds up on the right side of the chest cavity. However, it can occur on the left side or both. It causes dry cough, breathing difficulties, and pleurisy (inflammation of the tissue that separates the lungs from the chest wall).

• Ascites cause abdominal swelling.

How Is Meigs Syndrome Diagnosed?

• Physical Examination: It is essential to examine women showing ascites and pleural effusion features. The critical aspects that are evaluated during physical examination are:

1. Palpable abdominal masses.

2. Abnormal breath sounds and changes in the quality of voice are detected using a stethoscope.

3. Abdominal swelling.

4. Dullness of the chest and bulged neck veins are a few other signs of ascites that the physician evaluates.

5. Skin is also examined for any skin cancer, especially in children.

6. The ascites and pleural effusion are checked for resolution after removing the ovarian tumor, the hallmark of Meigs’ syndrome.

• Laboratory Investigations: Blood and urine are analyzed to detect any abnormality that indicates liver failure, anemia, heart failure, and excess excretion of protein in the urine (proteinuria). The blood is examined for complete blood count, prothrombin time, cholesterol levels, and pro-BNP (B-type natriuretic peptide). The high levels of pro-BNP are suggestive of heart failure. CA 125 (cancer antigen 125) is a specific protein found to be increased in the serum of women with ovarian cancer.

• Radiographic Investigations: The various imaging modalities used to diagnose Meigs’ syndrome are:

1. Ultrasound of the pelvis aids in determining any small lesions in the ovaries, uterus, fallopian tube, etc.
2. Computed tomography (CT) of the abdomen is taken to evaluate any other cause for ascites like cancer, liver disorders, etc.
3. Chest X-ray is more commonly used in the diagnosis of pleural effusion. It also helps in determining any cancer spread.
4. Positron emission tomography-computed tomography (PET-CT) is also essential in differentiating Meigs’ syndrome from other cancers.

• Thoracentesis is a minimally invasive procedure of removing fluid around the lungs and examining it for protein and lactate dehydrogenase (LDH) levels. It also gives temporary relief to the patients as fluid is removed.

• Paracentesis involves removing fluid in the abdomen, and it is analyzed for protein levels. It helps in determining the cause of ascites.

• Endoscopy: A thin, long tube with a camera is passed through the mouth to view the digestive tract. It aids in diagnosing any gastrointestinal tumor.

What Are the Related Disorders?

The disorders that have to differentiate from Meigs’ syndrome are listed down:

• Liver Cirrhosis: It is severe liver damage that occurs due to scarring of liver tissue. The individual suffers from fatigue, weight loss, jaundice, swelling of legs and feet, and ascites. Laboratory tests and biopsy are used in the diagnosis.

• Pseudo-Meigs’ Syndrome is characterized by abdominal or pelvic tumors, ascites, pleural effusion, and benign ovarian tumors.

• Ovarian Cancer: Malignant tumor of the ovaries occurs due to an unknown cause. It leads to weight loss, abdominal swelling, back pain, and an urge to urinate. It is usually treated by surgical removal of ovaries and or uterus.

How Is Meigs Syndrome Treated?

The treatment of Meigs’ syndrome is carried out after the diagnosis is confirmed. It involves:

• Paracentesis: Removing fluid from the abdomen relieves patients from breathing difficulties and abdominal discomfort.

• Thoracentesis: The fluid around the lungs from the chest cavity is removed at regular intervals to help in managing breathing difficulties.

• Surgery: The various abdominal surgery carried out in women with Meigs’ syndrome include:

1. Total hysterectomy is the surgical removal of the whole uterus and ovaries, and fallopian tubes on both sides. It is carried out in women over 50 years of age who have attained menopause.
2. Unilateral salpingo-oophorectomy is the surgical excision of the ovaries and fallopian tubes on one side. It is suggested for younger women in whom fertility preservation is essential.

Conclusion:

Meigs’ syndrome is rare and accounts for 1% of all ovarian tumors. However, the pleural effusion and ascites completely resolve after the excision of the ovarian tumor. The affected women show a good prognosis and lead a normal life post-surgery. Therefore, the healthcare specialist must diagnose such conditions appropriately.