Caroli syndrome

Ca·ro·li syn·drome

congenital malformation of the bile ducts within the liver leading to formation of multifocal cystic dilatations.

Ca·ro·li dis·ease

, Caroli syndrome (kah-rō-lē' di-zēz', sin'drōm)
Congenital cystic dilation of the intrahepatic bile ducts, sometimes associated with intrahepatic stones and biliary obstruction.
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References in periodicals archive ?
If both the large and small ducts are affected and both features of congenital hepatic fibrosis and Caroli disease are present, this entity is termed Caroli syndrome.
5-8) More than 300 cases of Caroli disease and Caroli syndrome (Caroli disease associated with congenital hepatic fibrosis) have been reported in the world literature, (9) and cholangiocarcinoma has been found in 7% to 14% of the cases.