[Cystic dilatation of the biliary tract]

Cystic dilatations of the biliary tree are defined by cysts which communicate with the biliary channels. Caroli's disease is characterized by macroscopic dilatations of segmental bile ducts (who drain the segments of the liver). It is not familial. Kidneys are usually normal. The main symptom is cholangitis. Complications are intrahepatic stones and cholangiocarcinoma. Treatment of cholangitis is antibiotics. In resistant cases, partial hepatectomy (if cysts are localized in one part of the liver) or hepatic transplantation (if cysts are diffuse) should be considered. Congenital hepatic fibrosis is characterized by microscopic dilatations of interlobular bile ducts and portal fibrosis. It is an autosomal recessive disease associated with renal abnormalities. The major clinical manifestation is digestive haemorrhage due to portal hypertension. Caroli's syndrome is the association of Caroli's disease and congenital hepatic fibrosis. Choledocal cyst is characterized by a cystic dilatation of the common bile duct or hepatic duct. The main manifestations are jaundice and cholangitis. Cholangiocarcinoma may occur. Treatment is surgical resection.