Caroli disease (Q87266)

Caroli's disease is a rare congenital disorder characterised by multifocal, segmental dilatation of the large intrahepatic bile ducts. It presents with recurrent bacterial cholangitis, biliary stones causing biliary pain or episodes of pancreatitis. The more common variant of this disease, named Caroli's syndrome, is characterised by dilatations of the large bile duct associated with congenital hepatic fibrosis, with signs and symptoms of portal hypertension or bacterial cholangitis.