[Congenital cystic dilatation of the intrahepatic bile ducts. Report of 40 cases].

Forty cases of adult congenital cystic dilatation of the intrahepatic bile ducts were reported in this paper together with a review of the literature. The disease is characterized by repeated attack of cholangitis, and the diagnosis is dependent almost entirely upon cholangiographic findings. The authors believe that basing on the typical cholangiographic findings as well as the tendency of having a longer course, this disease can be differentiated from the acquired dilatation of the intrahepatic bile ducts secondary to hepatolithiasis. The principle of treatment is resection of the diseased focus as well as establishment of an internal drainage. The diagnosis in 50% of the cases in this series was confirmed by histopathology, which has not been reported in the previous literature.