Caroli Disease, Caroli Syndrome, and Congenital Hepatic Fibrosis

TL;DRAbstract

The cystic diseases of the liver are mostly autosomal recessive disorders with variable intrahepatic biliary dilatation and increased hepatic fibrosis either as part of the underlying defect or as a result of liver damage from recurrent episodes of ascending cholangitis. Caroli disease (CD) denotes congenital saccular intrahepatic dilatation of the biliary tree. Caroli syndrome (CS) is a more common disorder in which the bile duct dilatation is associated with congenital hepatic fibrosis (CHF). The clinical manifestations of CS are related to the biliary abnormalities and portal hypertension. Recurrent bacterial cholangitis, liver abscesses, intra- and extrahepatic stones, and increased risk of cholangiocarcinoma are the main complications of CD and CS. Treatment is supportive and directed toward treating the biliary tract infections and the complications of portal hypertension. Surgical treatment consists of liver resection or liver transplantation. The prognosis of CD and CS is varia

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