A case of caroli's disease confined to the left lateral segment of the liver

Abstract

Caroli's disease is a rare congenital hepatobiliary disorder characterized by multifocal segmental dilatation of intrahepatic bile ducts affecting all or parts of the liver. Two forms of Caroli's disease have been described ; the pure form and that associated with periportal fibrosis. This disease may diffusely affect the liver or be localized to one lobe or segment. About 20% of all reported Caroli's disease cases are of the monolobar type. We report a case of simple type Caroli's disease confined to the left lateral segment of the liver in a 45-year-old man. He was initially admitted due to slightly elevated transaminase 1. Computed tomography (CT) scanning, magnetic resonance imaging (MRI) and positron emission tomography with 18F-fluorodeoxyglucose (FDG-PET) findings indicated a diagnosis of biliary cystadenoma. The diagnosis of Caroli's disease was ultimately confirmed by pathological findings. He underwent left hepatic lateral segmentectomy and recovered completely.