[Alagille syndrome, a rare differential diagnosis of intrahepatic cholestasis]

In Alagille's syndrome cholestasis is caused by a congenital hypoplasia of the intrahepatic bile ducts. If cardiac and arterial malformations are present in conjunction with the typical-but not pathognomonic-facies, this rare syndrome must be considered. ERCP shows a highly rarified bile duct system. Liver histology with a paucity of interlobular bile ducts establishes the diagnosis. Inheritance appears to be autosomal dominant. The relatively good prognosis of the disease calls for caution concerning invasive procedures.