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Polycystic liver disease presenting with an exudative pleural effusion: a case report

DOI: 10.1186/1752-1947-6-107

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Abstract:

A 50-year-old Caucasian woman presented with a two-week history of increasing dyspnoea. An examination revealed a large right pleural effusion and gross hepatomegaly. An ultrasound confirmed a large polycystic liver and diagnostic thoracocentesis revealed an exudate, which was sterile to culture. The pleural effusion proved refractory to drainage and our patient underwent surgery to deroof the main hepatic cysts in an attempt to reduce the pressure on her right diaphragm. The histology was compatible with that of polycystic liver disease. No evidence of malignancy was found. After surgery, our patient had no recurrence of her effusion and, to date, has remained asymptomatic from her polycystic liver disease.The case in this report illustrates that an exudative pleural effusion is a rare complication of polycystic liver disease. We feel that the mechanical effects of a large polycystic liver, and subsequent disruption of sub-diaphragmatic capillaries, resulted in a persistent exudative pleural effusion. Thus, surgical debulking of the hepatic cysts is required to manage these effusions.Polycystic liver disease is a relatively benign condition, causing significant symptoms in less than 5% of patients. The most common presentations necessitating surgical intervention are due to the mass effect of cysts within the liver, causing abdominal pain and distension, obstructive jaundice, portal hypertension and recurrent ascites [1-4]. There have been several reports in the literature describing the complications of polycystic liver disease [5]; however, presentation with a recurrent exudative pleural effusion, only resolving after debridement of hepatic cysts, has rarely been described. We feel that this should be considered as a recognised complication of polycystic liver disease and also be an indication for surgical intervention.A 50-year-old Caucasian woman presented with increasing dyspnoea over a two-week period and a two-month history of right upper quadrant distension

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