[Caroli syndrome. Clinical aspects--diagnosis--therapy]

The Caroli syndrome is a rare autosomal-recessively inherited disease with cystic dilatation of the intrahepatic bile ducts. Cystic bile duct dilatations are usually accompanied by cholelithiasis and subsequent cholangitis. This is the most substantial danger implied in the Caroli syndrome. It has often been left unconsidered as a cause in differential diagnosis of cholangitis, resulting in undetected protracted course of the disease. Once an unambiguous diagnosis has been established, the authors, by their own experience from three cases and with reference to the literature, recommend subtotal hepatectomy as optional therapy.