The evolving nature of "infantile obstructive cholangiopathy"

We report four patients with neonatal conjugated hyperbilirubinemia in whom the nature of the disease varied from that predicted by the initial diagnostic work-up. In two the initial liver biopsy showed paucity of the intrahepatic bile ducts; one cleared her conjugated hyperbilirubinemia and appeared normal at 3 years, but the other proved to have extrahepatic biliary atresia. The initial liver biopsy in two additional patients showed bile duct proliferation; both patients had a paucity of intrahepatic bile ducts later in the course of their disease. These cases illustrate the necessity of carefully following patients with neonatal conjugated hyperbilirubinemia to be certain that their courses continue to conform to the original diagnoses.