Pseudo-Meigs syndrome: a rare cause of abdominal ascites in a patient with an ovarian mass

Cirrhosis, Gynecological cancer

Meigs syndrome is a rare phenomenon seen in approximately 1% of patients diagnosed with ovarian tumours.1 It is defined as a triad of a benign ovarian tumour, pleural effusion and ascites with complete resolution of symptoms following surgical intervention. This constellation of findings can easily be confused with several diagnoses ranging from metastatic ovarian cancer to decompensated cirrhosis. In this case, we highlight a woman in her 30s presenting with abdominal pain and new ascites, initially thought to be due to cirrhotic portal hypertension and subsequently found to have an atypical presentation of pseudo-Meigs syndrome, in the setting of a benign ovarian teratoma. Overall, this case highlights the importance of casting a broad differential and pursuing a thorough evaluation in young women with ascites to mitigate the risk of misdiagnosis. Case presentation A woman in her 30s with a medical history of cholecystectomy presented to her primary care physician due to concerns for postprandial right upper quadrant abdominal pain and nausea. The physical examination was notable for right upper quadrant tenderness without hepatosplenomegaly and abdominal distention. An initial work-up was unremarkable, including normal complete blood count, liver function and coagulation tests. Viral hepatitis panel and lipase were normal. A right upper quadrant ultrasound without Dopplers was ordered, which demonstrated normal parenchyma of the liver, but showed moderate ascites with borderline splenomegaly (13.7 cm), suspicious for portal hypertension. Given the inconsistency between laboratory and imaging findings, MRI elastography was performed. The liver was normal in morphology, and the average liver stiffness was 295 kPa, compatible with borderline stage 1–2 fibrosis. She was referred to a hepatology clinic for further evaluation. The physical examination was notable for anicteric sclera without jaundice, normal respiratory excursion, moderate abdominal distension and an abdominal fluid thrill at the time of consultation. She underwent a paracentesis where 8000 mL of straw-coloured fluid was aspirated. The serum ascites–albumin gradient (SAAG) was low at 0.8 g/dL (serum albumin 3.5 g/dL–ascites albumin 2.7 g/dL), making portal hypertension less likely. Fluid total protein was elevated at 4.5 g/dL. Total cell count was 114 (12% neutrophils). Fluid triglyceride level was 16 mg/dL and fluid lactate dehydrogenase was 94 mg/dL. Ascitic fluid cytology was negative for malignancy. The decision was made to pursue CT imaging to evaluate for potential malignancy causing low SAAG and high protein ascites. CT of the abdomen/pelvis was notable for a non-cirrhotic configuration of the liver, a hyperenhancing cystic and solid mass measuring 6.6×6.7 cm arising from the left ovary, a mildly complex right ovary with a 1.7 cm cyst, a solid mass arising from the uterine fundus measuring 4.1×5.6 cm, representing an exophytic subserosal fibroid, large volume ascites and bilateral trace pleural effusions. Since an abdominal ultrasound without pelvic imaging was initially performed, these new findings of an ovarian mass were missed on prior imaging. The patient was referred to gynaecology oncology to discuss these findings. The physical examination was pertinent for a mildly distended abdomen with a positive fluid wave and a mobile pelvic mass. No cervical, supraclavicular or inguinal lymphadenopathy was appreciated. A pelvic examination was performed and notable for normal external genitalia, normal vaginal mucosa and a normal appearing cervix. CEA was normal, and CA-125 was elevated to 967 U/mL at the time of consultation. A CT of the chest was obtained for further evaluation of potential malignancy. Imaging was notable for extensive upper abdominal ascites without evidence of metastatic disease in the chest. Hepatology and gynaecology oncology worked together throughout her diagnostic work-up. The multidisciplinary team felt her ascites were related to her complex ovarian mass as she had no evidence of chronic liver disease or portal hypertension. A decision was made to pursue a diagnostic laparoscopy with biopsies and intraoperative frozen pathology. On entry into the abdomen, the liver surface, small and large bowel, appendix, stomach edge, diaphragm and omentum were normal in appearance. Yellow straw-coloured ascites was appreciated. The uterus appeared pedunculated with a posterior fibroid measuring 4 cm in size. The right ovary and fallopian tube were grossly normal without masses. The left ovary was noted to be nodular with pink fleshy tissue measuring approximately 12 cm in diameter. No peritoneal implants or adhesive disease were appreciated. A frozen section of the left ovary was obtained intraoperatively and notable for a benign ovarian teratoma. Pelvic washings were negative for malignancy. Given the teratoma size, a decision was made to pursue a laparoscopic left salpingo-oophorectomy for symptom management. The specimen was successfully resected, placed in an EndoCatch bag and removed from the abdomen laparoscopically. Final surgical pathology was significant for a benign ovarian teratoma, struma ovarii (figure 1). Following surgical intervention, there was complete resolution of ascites with improvement in the patient’s symptoms. She was discharged from the hepatology service and continues follow-up with gynaecology for routine care without evidence of recurrent ascites or ovarian pathology to date.

Overall, Meigs syndrome is classically defined as a triad of ascites, pleural effusions and ovarian tumour with complete resolution of symptoms following surgical intervention. However, several subclassifications have been outlined in the literature including pseudo, pseudo–pseudo, Demons and atypical/incomplete Meigs syndrome based on the constellation of symptoms and tumour origin.2 Since this patient was found to have a benign ovarian teratoma on pathology, her presentation is most consistent with pseudo-Meigs syndrome.2 3 Furthermore, atypical/incomplete Meigs syndrome is defined as the presence of either ascites or pleural effusions associated with a pelvic or abdominal tumour. This patient presented with diffuse ascites in the setting of a benign ovarian teratoma with trace pleural effusions on imaging, raising the question of whether her presentation is better classified as classic or atypical/incomplete Meigs syndrome. While several cases have similarly reported the triad of Meigs syndrome in the context of a struma ovarii, few cases have outlined the initial presentation and subsequent diagnosis from the perspective of a hepatologist.3 4 Overall, this case illustrates the importance of diagnostic paracentesis in guiding next steps in management. In patients with ascites precipitated by a malignancy with peritoneal involvement, fluid studies typically demonstrate a low SAAG, as portal hypertension is not driving the development of ascites fluid. Additionally, the fluid studies will often show a high total protein, white blood cell count>500 u/L and positive cytology.5 6 While a low white blood cell count was reassuring in our patient’s case, negative fluid cytology did not rule out malignancy due to limited sensitivity.6 7 A CT scan was appropriately pursued to investigate other aetiologies and unveiled the potential cause of the patient’s symptoms. In conjunction with an elevated CA-125, a hyperenhancing solid mass on the ovary with ascites can be suspicious for metastatic disease given that CA-125 is considered a marker for ovarian carcinoma.8 However, this marker is also notoriously elevated in cirrhosis with ascites, as well as various disorders including benign ovarian tumours, endometriosis and uterine fibroids.8 Since our patient did not have objective evidence of chronic liver disease or portal hypertension (SAAG<1.1 g/dL), surgery was pursued for further evaluation of the ovarian mass. It is essential to confidently rule out these hepatic conditions prior to pursuing surgical intervention due to the morbidity and mortality associated with abdominal surgery in patients with decompensated cirrhosis.9 10 Fortunately, our patient was found to have pseudo-Meigs syndrome in the setting of struma ovarii, which has a highly favourable prognosis with resolution of symptoms immediately following surgical intervention.1 Furthermore, this rare phenomenon should be considered when evaluating women with new ascites to prevent misdiagnosis and offer treatment that effectively resolves their symptoms. Learning points. Abdominal paracentesis with fluid analysis is a useful initial tool to guide the differential diagnosis in patients with new onset ascites. It is critical to rule out cirrhosis prior to referring patients for surgery due to the high risk of morbidity and mortality associated with abdominal surgery in patients with decompensated cirrhosis. Meigs syndrome is a ‘do not miss’ diagnosis given its excellent prognosis and treatment options. Footnotes Funding: The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors. Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy. Provenance and peer review: Not commissioned; externally peer reviewed. Patient consent for publication: Consent obtained directly from patient(s). Presented at: This abstract has been presented at ACG Conference in Philadelphia, Pennsylvania, USA, in October 2024.11

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