What is Meigs Syndrome?
Meigs syndrome is a condition where non-cancerous tumors on the ovaries cause fluid to accumulate in the abdomen (a condition known as ascites) and around the lungs (known as pleural effusions). This condition is a rare presentation of ovarian tumors, comprising roughly 1% of all cases. Because Meigs syndrome presents similarly to cancerous tumors, it can be tricky to differentiate the two. The syndrome was named after Joe Vincent Meigs, who first described it in 1937 through his work with seven patients. Although doctors had noted a link between benign ovarian tumors and fluid around the lungs (hydrothorax) in the past, Meigs was the first to observe that the fluid accumulation resolves when the tumor is removed, without further issues.
In 1954, Meigs established specific criteria to diagnose the syndrome. These include the presence of a benign ovarian tumor (such as fibroma, thecoma, granulosa cell tumor, or Brenner tumor), ascites and pleural effusion, and resolution of these conditions upon removing the tumor. Notably, the definition does not include fluid accumulation around the heart (pericardial effusion), although there have been cases where this anomaly has resolved following removal of the benign ovarian tumor.
Meigs syndrome most commonly develops in women after menopause. It’s characterized by shortness of breath, dry cough, and a swollen abdomen that can be painful. A doctor may be able to feel a mass near the ovaries during a physical examination and might observe dampened breath sounds or signs of ascites. When found in younger individuals, Meigs syndrome might indicate the presence of Gorlin syndrome, a hereditary condition that increases the risk of developing various types of cancer.
To diagnose Meigs Syndrome, doctors typically conduct a thorough medical history, physical examination, and imaging to confirm the presence of a mass in the pelvis and rule out malignant tumors. Lab tests and fluid analysis also play a crucial role in the diagnosis. Notably, the resolution of the ascites and pleural effusion after removing the tumor confirms the condition.
Treatment for Meigs Syndrome usually involves surgically removing the mass from the ovary. For young women who want to preserve their ability to have children, a procedure (called a unilateral salpingo-oophorectomy) that removes only the affected ovary and fallopian tube is preferred. Post-menopausal women, on the other hand, may have a total hysterectomy that includes the removal of both ovaries and fallopian tubes. If surgery isn’t a suitable option, alternative treatments focus on managing symptoms. These might include procedures (paracentesis and thoracentesis) to drain the excess fluids, or even the placement of catheters for ongoing fluid management.
What Causes Meigs Syndrome?
Meigs syndrome is a medical condition defined by the presence of fluid buildup in the abdomen (known as ascites) and the area around the lungs (known as a pleural effusion). However, the reasons why this happens are not very clear. Sometimes, a pleural effusion can happen without ascites, which is actually called atypical or incomplete Meigs syndrome. This scenario hints to the possibility that these two conditions can develop independently from each other. Therefore, when diagnosing and investigating these conditions, doctors must also rule out other conditions that could potentially cause similar symptoms.
Risk Factors and Frequency for Meigs Syndrome
Meigs syndrome is linked with about 1% of ovarian tumors. The majority of these tumors turn out to be harmless fibromas, according to medical examinations. These benign ovarian fibromas figure in 2% to 5% of ovarian tumors that are surgically removed. Among women with ovarian fibromas, 10% to 15% show signs of ascites, which is fluid buildup in the abdomen, and 1% show signs of fluid accumulation in the chest (hydrothorax).
- Approximately 70% of pleural effusions (fluid accumulation in the chest) occur on the right side,
- 15% occur on the left side, and
- 15% occur on both sides of the chest.
While Meigs syndrome is rarely diagnosed before a person’s thirties, the number of cases gradually increases with age and reaches its peak in the seventies.
Signs and Symptoms of Meigs Syndrome
Meigs syndrome is a medical condition that often appears in women after menopause. Symptoms related to this syndrome are often vague and can include breathing difficulties, dry cough, and abdominal discomfort due to fluid accumulation, known as ascites. More often than not, these symptoms may not immediately point to an ovarian issue, potentially leading to a delayed diagnosis. Therefore, it’s really important for doctors to keep Meigs syndrome in mind when examining a patient with these symptoms.
- Difficulty breathing
- Dry cough
- Abdominal discomfort from fluid build-up
During a physical exam, a doctor may also identify a palpable mass in the ovary area, signs of fluid accumulation in the lungs such as reduced breath sounds, changes in chest resonation, and vein enlargement in the neck. They may also observe a distended abdomen due to fluid accumulation.
- Palpable ovarian mass
- Reduced breath sounds
- Changes in chest resonation
- Vein enlargement in the neck
- Distended abdomen due to fluid accumulation
Although Meigs syndrome typically presents in postmenopausal women, it can, on rare occasions, appear in children and teenagers. In these younger patients, if an ovarian mass is found along with ascites and hydrothorax (fluid collection in the chest cavity), it’s important to rule out another condition called Gorlin syndrome. Gorlin syndrome is a genetic disorder that can lead to multiple tumors from a young age, including ovarian and heart fibromas. This syndrome is also associated with numerous other abnormalities, including skin cancers and issues related to the bones, eyes, and nervous system. If Gorlin syndrome is suspected, genetic testing should be done so that proper monitoring and screening can start promptly. Girls diagnosed with Gorlin syndrome should have a pelvic ultrasound when they start their first period or by the age of 18 to check for ovarian fibromas.
Testing for Meigs Syndrome
To diagnose Meigs syndrome, which is usually accompanied by abdominal swelling and shortness of breath, your doctor will ask you about your symptoms and examine you thoroughly. If these symptoms are present, your doctor will order imaging tests to check for a pelvic mass and signs of other conditions related to Meigs syndrome, including lung nodules, liver lesions, or signs of cancer.
Meigs syndrome can only be confirmed after removing the tumor through surgery. If the examination of the tumor shows that it is harmless and if the symptoms like fluid accumulation in the abdomen (ascites) and in the space surrounding the lungs (hydrothorax) go away following the removal, then it is additional proof of Meigs syndrome.
In addition to imaging tests, laboratory tests are conducted to rule out other conditions that may be causing your symptoms. These tests include a complete blood count, liver function tests, blood clotting tests, a test measuring a substance called brain natriuretic peptide (which can indicate heart failure), and a urine test. No specific diagnostic markers indicate ovarian tumors, which are often involved in Meigs syndrome. However, a substance known as CA-125, typically elevated in ovarian cancer, may also be increased in some cases of Meigs syndrome.
Imaging tests used to investigate suspected Meigs syndrome include ultrasound of the abdomen and pelvis, which helps evaluate any ovarian masses and associated fluid accumulation. A chest x-ray can be used to check for fluid around the lungs and potential lung tumors. Computed tomography (CT) scans of the chest, abdomen, and pelvis are used to rule out other potential causes of fluid accumulation in the belly, such as tumors spreading from other sites, masses from the digestive tract, lung diseases, or liver cirrhosis.
A special type of scan, known as positron emission tomography (PET)/CT, maybe used to confirm if the ovarian mass is benign and check for other cancerous lesions. A substance called fludeoxyglucose F18 (18F-FDG) is injected into your veins for this test. Benign ovarian tumors will show only a mild uptake of this tracer, while malignant masses will show more uptake.
If you have risk factors for cancers of the digestive system, you might undergo an endoscopy. This is a procedure where a flexible tube with a light and camera at the end is inserted to take a closer look at your esophagus, stomach, and colon.
In individuals experiencing ongoing symptoms, thoracentesis and paracentesis, procedures to remove fluid from the chest and abdomen respectively, can not only help relieve the symptoms but also aid in the diagnosis of Meigs syndrome.
Since Meigs syndrome symptoms can be similar to those of tuberculosis, if you have risk factors for tuberculosis, your doctor may recommend tuberculosis testing. This can be done through skin tests, or testing of the fluid from your abdomen or around your lungs for bacteria causing tuberculosis.
Treatment Options for Meigs Syndrome
When managing Meigs syndrome, once it has been confirmed that the patient does not have cancer or any other similar conditions, treatment usually involves two primary methods: addressing the cause of the syndrome, and relieving the symptoms.
The most effective cure for Meigs syndrome tends to be surgical removal of the ovarian mass that caused it. This can be done through an incision in the abdomen (laparotomy) or through smaller, less invasive procedures like laparoscopy. Once removed, the tissue is sent for examination to confirm the diagnosis. For young women who want to preserve their fertility, removal of one ovary and its associated fallopian tube (unilateral salpingo-oophrectomy) is the best treatment. For women who have gone through menopause, doctors often recommend total removal of the uterus, both fallopian tubes and both ovaries (total abdominal hysterectomy with bilateral salpingo-oophrectomy). The removal of the mass generally leads to the disappearance of ascites (fluid in the abdomen) and pleural effusion (fluid around the lungs), and to a return to normal of the CA-125 levels (a protein often present at high levels in ovarian cancer).
However, surgery isn’t an option for everyone, particularly if a person has other serious health conditions or doesn’t want to undergo a major surgical procedure. In these situations, the focus is on managing and palliating the symptoms. Large amounts of fluid can be drained from the abdomen (paracentesis) or chest cavity (thoracentesis), depending on where fluid buildup is causing problems. Another option is to place a catheter in the abdomen that lets the patient or caregiver drain fluid as needed at home. For symptom relief related to the buildup of fluid around the lungs, options include repeated thoracentesis, placement of a catheter in the pleural cavity, or a procedure called pleurodesis, which closes the space between the two layers of the pleura to prevent fluid buildup. Several studies have shown that placement of a permanent chest drain not only helps control symptoms, but may also lead to spontaneous pleurodesis.
Therefore, doctors need to discuss all the potential treatment options with patients who have Meigs syndrome but who might not be good candidates for surgery, to help them make the best decision for managing their symptoms.
What else can Meigs Syndrome be?
For a confirmed diagnosis of Meigs syndrome, it is necessary to obtain a tissue sample to identify the cause of the ovarian mass. There are other health conditions with similar symptoms that need to be ruled out before planning for surgery. Here are some of these conditions:
- Ovarian cancer
- Liver cirrhosis
- Cancers like pancreatic, gastrointestinal, and lung, which can also cause ascites and hydrothorax
- Congestive heart failure
- Nephrotic syndrome, which is a kidney disorder
- Tuberculosis
- Thoracic endometriosis
- Pseudo-Meigs syndrome: This has similar symptoms to Meigs syndrome, but it is observed in patients with other types of pelvic or abdominal tumors. These can include benign ovarian cysts like struma ovarii, mucinous cystadenoma, and teratomas; uterine leiomyoma; and ovarian tumors that have resulted from metastasis
There is also a condition called Tjalma syndrome, also known as Pseudo-pseudo Meigs syndrome. It’s a manifestation of systemic lupus erythematosus. Its symptoms include ascites (fluid in the abdomen), pleural effusions (fluid around the lungs), and high levels of CA-125, a protein in the blood. This happens without the presence of an ovarian tumor, whether the tumor is benign or malignant.
What to expect with Meigs Syndrome
Meigs syndrome is a harmless condition, and if detected and treated early, the outlook is fantastic. Fluid buildup around the lungs (pleural effusion) and excess fluid in the space between tissues lining the abdomen and abdominal organs (ascites) completely go away once the tumor is removed. After the operation, the life expectancy of patients with Meigs syndrome is the same as the general population.
Possible Complications When Diagnosed with Meigs Syndrome
If patients with Meigs syndrome aren’t diagnosed and treated in time, they may undergo multiple procedures to drain fluid around the lungs and abdomen. These procedures come with the risk of complications like infections, bleeding, dehydration, lung collapse, accidental injury to the intestine during the drainage procedure and a condition called hypoalbuminemia that causes low levels of albumin in the blood. If the cause of the syndrome is misdiagnosed as cancer, the patients could face a secondary illness. They might develop widespread and advanced disease, suffer severe weight and muscle loss, blood clots in deep veins, blockages in the intestine due to a growing mass, and other complications from untreated cancer.
Common Complications:
- Multiple procedures to drain fluid
- Infections
- Bleeding
- Dehydration
- Lung collapse
- Accidental injury to the intestine during procedure
- Hypoalbuminemia
- Widespread and advanced disease
- Severe weight and muscle loss
- Blood clots in deep veins
- Blockages in the intestine due to a growing mass
- Other complications from untreated cancer
Preventing Meigs Syndrome
If patients or their caregivers notice symptoms like breathlessness, a swollen belly, especially when they’re losing weight or these symptoms show up within a few months, it’s crucial to seek medical attention. This could signal the presence of Meigs syndrome, a condition that often improves after treatment. A complete medical check-up, taking a detailed account of your health history, a comprehensive physical examination and necessary lab tests and scans are essential to accurately diagnose this condition.
Once diagnosed with Meigs syndrome, patients should take comfort in the fact that it’s usually not a dangerous condition. Being referred to a specialist in female reproductive cancers promptly can help alleviate their symptoms and bring relief. However, until a clear diagnosis is delivered, the possibility of other serious cancers, such as ovarian, colon and other digestive system cancers, should be considered. This is to make sure that these possible conditions are not overlooked, which could delay critical treatment and lead to severe health risks.
