Ongoing cirrhosis after successful porticoenterostomy in infants with biliary atresia

Abstract

Thirty-one patients were treated by portoenterostomy procedures for extrahepatic biliary atresia. In 20, there was unequivocal evidence of bile drainage. Eleven of these 20 were restudied from 3 to 23 mo postoperatively by liver biopsy. The clinical condition of most of these infants was satisfactory. Despite absence of clinical jaundice, normal rose bengal scan, and normal liver function studies, eight showed progressive degrees of hepatic fibrosis when compared to the results of the biopsy taken at the time of portoenterostomy. Two patients showed stable histology and one, after progressing on a second biopsy, had normal liver histology on a third study. The implications of progressive hepatic histopathology despite bile drainage are discussed.