The outcome after portoenterostomy for biliary atresia is determined by age at surgery and anatomy of the atretic extrahepatic bile ducts. Liver transplant will salvage patients with failed Kasai with 10-year posttransplant survival of 71%.
Differentiating intrahepatic cholestasis from extrahepatic biliary tract obstruction may be difficult. Four patients with intraoperative cholangiographic evidence of extrahepatic ductal atresia who underwent hepatoportoenterostomy are described. All were ultimately shown to have arteriohepatic dysplasia with hypoplastic but patent extrahepatic ductal systems. The difficulty in establishing an accurate diagnosis, hazards associated with hepatoportoenterostomy, and suggestions for evaluation and management are discussed.
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