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Caroli DiseaseCaroli disease is a rare congenital disease. It entails cystic dilatation of the intrahepatic bile ducts. There are two types of Caroli disease. Simple Caroli disease simply entails the bile duct dilatation or ectasia that by definition is part of Caroli disease. In complex Caroli disease, hepatic fibrosis and portal hypertension are present as well.
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