Carolis syndrome associated with medullary sponge kidney disease and nephrocalcinosis-A case report.

Caroli’s disease is an autosomal recessive condition characterized by non-obstructive dilatation of intrahepatic biliary ducts. The exact etiology is unclear. Two variants of Caroli’s disease are- type I is simple form; in which bile ducts are dilated without hepatic fibrosis and the type II which is associated with congenital hepatic fibrosis along with its sequelae, also known as Caroli’s syndrome. The importance of recognizing this disease as a cause of biliary stasis lies in fact that it is frequently associated with recurrent cholangitis, liver abscesses, cirrhosis and cholangiocarcinoma.