Revisiting Caroli Syndrome in a Tanzanian Patient

Caroli disease and Caroli syndrome are two rare congenital diseases of the intrahepatic bile ducts. Caroli syndrome is characterized by the saccular dilatation of intrahepatic bile ducts associated with congenital hepatic fibrosis. It is rarely diagnosed in childhood. We hereby describe a case of Caroli syndrome in a young Tanzanian female who had abdominal pain and distension since childhood. Her history suggested the presence of portal hypertension possibly from congenital hepatic fibrosis. The diagnosis was reached based on ultrasound, computed tomography (CT) scan of the abdomen, and magnetic resonance cholangiopancreatography (MRCP).