Caroli disease

Caroli disease is a rare inherited disorder of the liver in which the bile ducts inside the liver become abnormally widened and cystic, a change also called ectasia. It is sometimes known as communicating cavernous ectasia or congenital cystic dilatation of the intrahepatic biliary tree. Two patterns are described. The focal or simple form affects one isolated portion of the liver, while the more diffuse form, usually combined with portal hypertension and congenital hepatic fibrosis, is often called Caroli syndrome. The reasons the two forms differ are not well understood. The disease is also associated with liver failure and with polycystic kidney disease. It affects about one person in a million, and Caroli syndrome is reported more often than Caroli disease. It is distinct from other conditions in which the ducts widen because of an obstruction, and it is not one of the choledochal cyst derivatives.