ATRESIA OF THE INTRAHEPATIC BILE DUCTS

Case histories of four patients with atresia of the intrahepatic interlobular bile ducts are reviewed. Three of the four also had anomalies of the extrahepatic bile ducts. All had secondary biliary cirrhosis. The course of disease was distinguished by a relatively long life span, all patients surviving more than three years and one remaining alive at five years. Generalized skin xanthomatosis occurred in all patients, together with a marked and characteristic elevation of the serum lipids. There was no indication on postmortem examination that intrahepatic bile ducts had developed and then become obliterated. The evidence presented in this study suggests that absence of interlobular bile ducts is a developmental anomaly, and that the embryologic development of biliary epithelium is normally independent of the growth of hepatic trabeculae.