Massive hepatomegaly in adult polycystic liver disease

Abstract

In adult polycystic liver disease, the liver gradually enlarges as it is replaced by cysts. The disease rarely produces symptoms or complications. Liver cysts are thought to arise from aberrant embryonic intrahepatic bile ducts (Von Meyenburg's complexes). We present a case of adult polycystic liver disease with Von Meyenburg's complexes and unusually severe hepatomegaly (7.7 kg, 22,080 cm3). The autopsy prevalence of adult polycystic liver disease at UCLA Medical Center is 0.13%; 93% of these cases had polycystic kidney disease. Adult polycystic kidney disease had associated liver cysts in 45% of cases.