Definição:
Congenital cystic dilatation of the intrahepatic bile ducts (). It consists of 2 types: simple Caroli disease is characterized by bile duct dilatation (ectasia) alone; and complex Caroli disease is characterized by bile duct dilatation with extensive hepatic fibrosis and portal hypertension (). Benign renal tubular ectasia is associated with both types of Caroli disease.