Tags: Disease.

Caroli disease is a rare inherited disorder characterized by dilatation of the intrahepatic bile ducts. There are two types of Caroli disease the most common being the simple or isolated case where the bile ducts are widened by ectasia. The second more complex cause is commonly known as Caroli Syndrome. This complex form is also linked with portal hypertension and congenital hepatic fibrosis. The differences between the causes of the two cases have not yet been discovered.

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