1992 Volume 28 Issue 4 Pages 897-901
Two infants with syndromatic paucity of interlobular bile ducts associated with concurrent extrahepatic biliary obstruciton are reported. Agenesis of extrahepatic bile ducts was documented by surgical exploration of the porta hepatis in both patients during hepatic portoenterostomy at the age af 2 and 3 months. They were suspected of syndromatic paucity of interlobular bile ducts because their chest films showed butterfly-like vertebral anomalies and additionally because a heart murmur was audible in one. Hepatic biopsy specimens revealed paucity of interlobular bile ducts, and minimal portal fibrosis. As these findings were characteristic of syndromatic paucity of interlobular bile ducts, both infants were diagnosed as having paucity as well as extrahepatic biliary atresia. Operative outcome was poor in terms of postoperative extended bile excretion. However both are still living at one and 4 ytears 5 months of age. Although it was hoped that hepatic portoenterostomy might have effectively prolonged their lives, retrospectively this procedure can not be considered definitive. In conclusion, it is quite likely that some etiologic factor which is predisposing to paucity of interlobular bile ducts in Alagille's syndrome affected extrahepatic as well as intrahepatic biliary tracts in certain cases.
