Breathlessness and abdominal swelling: a classic eponymous syndromeBMJ 2017; 356 doi: https://doi.org/10.1136/bmj.j382 (Published 09 February 2017) Cite this as: BMJ 2017;356:j382
- Jennifer Kahan, clinical fellow in oncology,
- Russell Banner, consultant clinical oncologist,
- Kerryn Lutchman-Singh, consultant in obstetrics and gynaecology
- Correspondence to J Kahan
A previously well 50 year old woman developed shortness of breath over several weeks accompanied by abdominal distension and discomfort. She was unable to lie flat due to orthopnoea. She had no comorbidities and was on no medication. A computed tomography (CT) scan of the chest, abdomen, and pelvis was undertaken, followed by biopsy and definitive surgical treatment. Postoperative histology confirmed a benign neoplasia. What are the three main radiological findings seen in the CT image (fig 1) and what is the eponymous diagnosis associated with this classic triad?
The CT shows a large right pleural effusion resulting in mediastinal shift and gross ascites (fig 2). There is a large mass arising from the pelvis. A pleural effusion or ascites associated with a benign ovarian fibroma is known as Meigs’ syndrome.
Meigs’ syndrome is the association of ovarian fibroma with a pleural effusion with or without ascites. It was first reported by JV Meigs in 1934. Removal of the fibroma was found to result in elimination of ascites and pleural effusion.1 Ovarian fibromas are a sex cord stromal neoplasia and have a low metastatic potential. They account for 1%-2% of resected ovarian neoplasms.2
Meigs’ syndrome can also be associated with raised levels of Ca125,3 and this patient had Ca125 of more than 1600 (normal range <34 kU/L).
Fluid accumulation in the peritoneal and pleural space can be related to changes in the permeability of capillaries caused by substances similar to vascular endothelial growth factor (VEGF). One study showed that, after removal of the fibroma, the postoperative VEGF levels decreased in the patient's pleural fluid but not in the peritoneal fluid.4
Presentation of an ovarian mass with ascites and pleural effusion is grounds for urgent investigation to identify possible advanced malignancy. However, it is important to consider other rarer causes of the symptoms, even when accompanied by a raised Ca125. Outcomes from ovarian fibroma in Meigs’ syndrome are extremely favourable owing to the low risk of metastatic spread. Neither ultrasound nor CT are specific enough to give a definite diagnosis of fibroma, and the key for diagnosis is biopsy or histology of the surgical specimen.
Ovarian masses associated with ascites and pleural effusions do occur in metastatic disease owing to an ovarian primary malignancy or in relation to metastatic disease that affects the ovary. In this context, these findings are termed pseudo-Meigs.
After resection of the fibroma by total abdominal hysterectomy and bilateral oophrectomy the patient made an excellent recovery and both the ascites and pleural effusion resolved.
Meigs’ syndrome is related to a benign fibroma causing a pleural effusion and ascites, whereas pseudo-Meigs’ is associated with primary or secondary ovarian malignancy.
Rapid referral for investigation and diagnosis is required. Drainage of ascitic or pleural fluid can give symptomatic relief but definitive treatment is removal of the fibroma.
We have read and understood BMJ policy on declaration of interests and declare the following: none.
Patient consent obtained.