Isolated polycystic liver disease revealed by surgical acute abdominal syndrome

A 65 year old woman presented with acute abdomen pain, a history of chronic abdominal swelling. Physical examination found a palpable mass of the right upper quadrant extended to the umbilical quadrant, associated to generalized abdominal tenderness. Laboratory testes demonstrated an inflammatory syndrome without perturbation of serum enzymes of cholestasis. A computed tomography scan of the abdomen revealed more than 20 liver cysts, the biggest measured 20 cm. The biliary tree and the kidneys were normal. It also showed an intraperitoneal effusion. We suspected a spontaneous rupture of hepatic hydatid cysts despite negative hydatid serology. The patient was operated upon electively and the operative findings were a polycystic liver spread through several segments of the liver with a giant thin walled cyst of the right lobe measured 20cm associated to a free fluid in the hepato-diaphragmatic space, without biliary fistula. We performed the fenestration technique which combined aspiration of 4 liters of serous fluid and chocolate-like materials and deroofing of the biggest cyst. The histology has confirmed the diagnosis of a simple hepatic cyst. The outcome until 11 months after still uneventful.