Biliary tree in cystic fibrosis. Biliary tract abnormalities in cystic fibrosis demonstrated by endoscopic retrograde cholangiography.

Two female adolescents with cystic fibrosis were investigated by endoscopic retrograde cholangiography for recurrent abdominal pain. The cholangiogram of 1 patient demonstrated multiple irregular filling defects throughout the biliary tree representing thickened bile and mucus as well as stones. The cholangiogram of the other case illustrated cystic dilatation of the intrahepatic bile ducts with intrahepatic cholelithiasis as well as extensive irregularities of the smaller proximal ductules secondary to recurrent cholangitis or focal biliary cirrhosis, or both. Endoscopic papillotomy resulted in drainage of tenacious bile and mucus and stones in both cases. Sustained clinical improvement did not follow papillotomy in either case, whereas radiologic improvement was demonstrated in 1 case.