The surgery of "correctable" biliary atresia

Seven of 114 (6%) patients treated in Denver for biliary atresia had a variant of the disease in which only microscopic bile ducts or ductules were identified at the porta hepatis, hepatic lesions were present, even in the neonatal example, and intrahepatic biliary hypoplasia was uniformly present. The excised bile duct cysts consisted of fibrosis or scar tissue and with little or no epithelial lining. Six patients had corrective surgery. The first patient was treated by choledochoenterostomy and became totally obstructed. Definitive operation in this patient and all subsequent patients consisted of excision of all extrahepatic duct structures and Roux-en-Y portoenterostomy. Two patients died, the one who did not have surgical correction and a second who had correction at age 35 weeks. The other five patients have been followed for 18 to 158 months and are anicteric but have some degree of residual liver damage. We conclude that so-called correctable biliary atresia occurs in less than 10% of cases, that because of coincident biliary hypoplasia, "cure" is not a possible outcome, and that the treatment of choice is complete surgical excision of the extrahepatic bile ducts and Roux-en-Y portoenterostomy.