A 32 year-old Peruvian woman presented with a 2 year history of dyspnea, dyspepsia and abdominal distension. Physical examination revealed diminished vesicular sounds and painless hepatomegaly. Liver function lab tests were normal. HBV, HCV and Equinococcus serologies were negative. Ultrasonography and computed tomography (Fig. 1) showed many well-delimited liver cysts, the largest of which measured 20×16cm, without septa or membranes inside. Echocardiogram (Fig. 2) revealed compression of right atrium, inferior vena cava and suprahepatic veins. TC showed data of chronic congestive liver.
Polycystic liver disease (PCLD) was diagnosed. This disease is defined by the presence of multiple hepatic cysts that microscopically resemble simple hepatic cysts, but they are larger and more numerous.1 PCLD is usually asymptomatic except for very large or multiple cysts. They can develop complications such as bleeding, infection, portal hypertension or from mass effect, as in our case.2
Study of genetic PRKCSH mutation was negative; however, only 1/3 of PCLD cases present this mutation.1
Four liters of bile secretion were evacuated and four sessions of polidocanol foam injection were performed for sclerosis. Currently, patient's symptoms have improved, the main cyst measures 9cm, and treatment with lanreotide is being evaluated to reduce liver volume.3
FundingNo organization funded this research.
Conflict of interestThere is no conflict of interest.