Fatal cholangitis after endoscopic retrograde cholangiopancreatography in congenital hepatic fibrosis.

A patient with congenital hepatic fibrosis is described in whom portal hypertension with variceal bleeding was the predominant symptom, accompanied by a minor episode of cholangitis Endoscopic retrograde cholangiopancreatography disclosed intrahepatic bile duct strictures, tortuosity, and tubular dilatation, but not cystic dilatation as typical of Caroli's disease, in which cholangitis is known to be the predominant feature. Following the procedure, the patient developed fulminating cholangitis and septicaemia, and died despite surgical decompression of the biliary system.