Biliary tract abnormalities in patients with arteriohepatic dysplasia

Arteriohepatic dysplasia is a congenital syndrome associated with life-long cholestasis. Because of symptoms suggesting extrahepatic biliary tract obstruction, we studied three patients with this syndrome by endoscopic retrograde cholangiopancreatogram (ERCP). All patients showed a decrease in the number of intrahepatic ducts. In addition, the intrahepatic ducts show attenuation with focal areas of dilatation. In one subject, this latter finding appeared to be a localized process. The extrahepatic ducts were also narrowed. One patient in this series was found to have gallstones and another cirrhosis. Although the relationship of these anomalies to the cholestasis seen in these patients is unclear, arteriohepatic dysplasis can be added to the list of processes associated with biliary tract abnormalities.