Meigs syndrome is a special variant of polyserositis that occurs in patients with tumors of ovarian tissue, uterus and completely passes after removal of neoplasia. It is manifested by an increase in abdominal volume, an increase in shortness of breath, tachycardia, weakness, fatigue, pallor, weight gain with external signs of cachexia. It is diagnosed by gynecological examination, ultrasound of the abdominal and pleural cavities, pericardium, pelvic organs, cytological examination of pleural effusion and ascitic fluid, laparoscopy. Treatment involves exudate evacuation, correction of organ disorders, surgical extirpation of the tumor.
R18 J94.8 D27 C56
Meigs syndrome (Demon-Meigs, Meigs-Salmon, Meigs-Cass) is a rare paraneoplastic disorder observed in 3% of patients suffering from voluminous formations of the reproductive organs. The symptom complex, manifested by ascites and effusion into the pleural cavity in women with solid ovarian neoplasms, was described in detail by J. Meigs in 1934-1935. A little later, R.U. Light expanded the interpretation of the syndrome to all neoplasms of the pelvic organs, including malignant neoplasia without metastases. The classic combination of ovarian tumor, ascites and hydrothorax is observed in isolated cases, abdominal effusion is more often determined in patients. In the presence of pleural exudation in 70% of cases, the process is localized on the right, in 10% — on the left, in 20% — is bilateral. The disorder occurs more often in women after 45 years.
The pathological symptom complex develops against the background of neoplastic lesions of the ovarian tissue and myometrium. Most often, polyserositis is combined with ovarian fibroma, ovarian cysts, uterine leiomyoma. Pleural, peritoneal and pericardial effusion can also be formed with ovarian carcinoma without signs of metastasis, although in this case specialists in the field of obstetrics and gynecology talk about Meigs pseudosyndrome. Sporadic cases of polyserositis with an appropriate clinical picture are described against the background of degenerative changes in ovarian tissue without tumor transformation, extensive ovarian edema, their hyperstimulation syndrome with gonadotropins, gonadoliberins during in vitro fertilization.
The pathogenesis of Meigs syndrome has not yet been studied. No specific channels connecting the uterus and ovaries with the cavities of the pleura and pericardium have been identified. Several hypotheses of exudate formation in neoplasms of female reproductive organs have been proposed. According to one theory, exudative effusion in Demon-Meigs-Kass syndrome initially accumulates in the abdominal cavity as a result of the occurrence of an “alarm reaction” of the peritoneal vessels to a growing tumor. Ascites promotes stretching of the diaphragm with the formation of congenital defects (pleuroperitoneal channels), through which exudate seeps into the pleural cavity.
A number of authors do not exclude the pathogenetic role of lymphatic vessels perforating the diaphragmatic septum. The idea of a violation of venous and lymphatic outflow as a result of mechanical compression of tissues by neoplasia has not been confirmed, since some patients develop massive polyserositis characteristic of the syndrome with tumors whose diameter does not exceed 5 cm. Exudation mechanisms can also be triggered by as yet unidentified substances secreted by the neoplasm.
The clinical symptoms of the disorder increase gradually, are nonspecific and, as a rule, become a consequence of the pressure of accumulated effusion on the surrounding organs. The patient complains of intermittent or persistent minor, more often unilateral pain in the lower abdomen, usually described as discomfort. Some women perceive painful sensations as dull, aching, bursting.
Subsequently, the stomach increases in size, the feeling of lack of air increases, general malaise, weakness, fatigue, sweating, loss of appetite, paleness of the skin, puffiness appears. There is a significant weight gain against the background of signs of cachectic syndrome (sagging skin, muscle hypotrophy). The amount of urine decreases, constipation is possible. Dysfunctional uterine bleeding may occur in patients of reproductive age.
With the progression of the process and the accumulation of significant volumes of exudative effusion, Meigs syndrome is complicated by cardiac and pulmonary insufficiency, metabolic cardiomyopathy, anemia, and increasing ischemia of various organs and tissues. In severe cases, oxygen starvation of the brain caused by insufficient oxygenation of blood in the lungs contributes to the occurrence of cognitive disorders (memory impairment, inattention), emotional lability, irritability, and a decrease in criticality to one’s condition. Against the background of irreversible cachectic changes, multiple organ failure occurs, leading to a fatal outcome.
Initially, effusion in the abdominal, pleural, and pericardial cavities is detected during physical examination (percussion and auscultation). The presence of fluid is indicated by the dulling of the percussion sound over the affected half of the chest, in the right and left flanks of the abdomen, the expansion of the boundaries of the heart in both directions. Auscultation vesicular respiration in the zone of dullness is absent or sharply weakened, heart tones are muffled, accelerated. The presence of exudate is confirmed by chest radiography, ultrasound of the pleural cavity, pericardium, abdominal organs, echocardiography. Detection of peritoneal, pleural, pericardial effusion serves as the basis for in-depth oncological examination in order to exclude tumors of the uterus or ovaries. The most informative methods for suspected Meigs syndrome are:
- Examination on the chair. A bimanual gynecological examination determines the enlargement of the uterus (in the presence of fibroids), an ovoid or rounded volume formation on the right or left in the appendage area (with an ovarian tumor). Benign neoplasia usually has a smooth surface, not soldered to the surrounding tissues. Fibroids are characterized by high density, cysts are often elastic.
- Sonography of pelvic organs. Ultrasound examination allows you to determine the exact localization, size, structure of the tumor formation. To clarify the features of its blood supply, Dopplerography with color mapping is used. If necessary, ultrasound is supplemented by MRI or CT of the female genital organs, during which their layered image is created.
- Analysis of pleural effusion. The data of cytological examination of the fluid obtained by thoracocentesis confirm the exudative nature of the process. An increased amount of protein, a positive Rivalt test, and single lymphocytes are determined. Atypical cells are absent. Similar changes are noted in the cytology of ascitic fluid.
To detect the tumor process, diagnostic laparoscopy, determination of the cancer marker CA-125 can be recommended. In the general blood analysis of patients with Meigs syndrome, the number of erythrocytes and hemoglobin levels are usually slightly reduced, ESR is increased to 25-30 mm / h, there is a slight leukocytosis with a rod-shaped shift to the left. The ECG shows signs of dystrophic changes in the myocardium.
Differential diagnosis is carried out with exudative pleurisy, cirrhosis of the liver, decompensated heart failure, polyserositis syndrome in tuberculosis, rheumatism, systemic lupus erythematosus, uremia, sepsis, paraproteinemic hemoblastosis, familial Mediterranean fever (periodic illness). According to the indications, the patient is consulted by a pulmonologist, cardiologist, phthisiologist, rheumatologist, nephrologist, hematologist, oncologist.
Therapeutic tactics are aimed at the rapid elimination of symptoms of organ compression, correction of concomitant disorders and subsequent surgical removal of neoplasia. In case of decompensated multiple organ failure, the patient is hospitalized in the intensive care unit, in other cases, planned preoperative preparation in a gynecological hospital is recommended. The main stages of treatment of Meigs syndrome are:
- Removal of exudate. To quickly unload the body from accumulated fluid, thoracocentesis and laparocentesis are performed. Liquid evacuation is carried out through the drainage system using an active aspiration device. The pathognomonic sign of the Demon-Meigs syndrome is the “inexhaustibility of exudate” — its rapid accumulation after evacuation.
- Correction of multiple organ disorders. Diuretics, cardiac glycosides are used to improve cardiac activity. If-channel inhibitors of the sinus node are indicated for tachycardia, antiarrhythmic drugs are indicated for arrhythmia. The introduction of saline and oncotic solutions is recommended for patients with electrolyte balance disorders.
- Surgical treatment. The scope of intervention depends on the revealed gynecological pathology, the patient’s age, her reproductive plans. It is possible to perform a wide range of operations — from hatching an ovarian tumor, resection of a part of the ovary, hysteroresectoscopy of fibroids to cytoreduction in ovarian cancer and extirpation of the uterus with appendages.
Prognosis and prevention
Complete resorption of exudate with restoration of general well-being usually occurs within 2 weeks after surgical extirpation of the tumor. Some patients have a small number of adhesions, pleural and pericardial adhesions. In Meigs pseudosyndrome, which complicates the course of malignant neoplasms, the prognosis depends on the stage and type of the oncological process. Prevention involves routine examinations by an obstetrician-gynecologist and regular ultrasound screening for timely detection and adequate treatment of tumor lesions of the uterus, ovarian tissue.