LONG-TERM RESULTS WITH THE KASAI OPERATION FOR BILIARY ATRESIA

Objective: To evaluate long-term outcome in a series of children with biliary atresia treated by portoenterostomy. Design: Case series of co nsecutive infants with biliary atresia with 10-year follow-up. Data we re obtained by retrospective chart review or phone interview.Setting: A tertiary academic medical center and regional children's hospital. P atients: A consecutive series of 104 infants diagnosed with biliary at resia more than 10 years ago were evaluated. Eighty-nine had totally o bliterated extrahepatic ducts, 4 had proximal hilar cysts (correctable type), and 11 had patency of the gallbladder and distal common duct. Interventions: Ninety-eight patients underwent biliary reconstruction and 6 had exploration only. Seventy-four infants underwent reconstruct ion using a Roux-en-Y with exteriorization. The 11 infants with distal patency underwent a portocholecystostomy (''gallbladder Kasai''). The remainder had various modifications of the Kasai operation. Main Outc ome Measures: Survival, liver function, complications, growth, and dev elopment. Results: The 6 patients who did not have a portoenterostomy died. Of the 98 who had a reconstruction, 63 died (mean age at death, 27 months; median, 13.4 months), 10 following liver transplantation. T welve of the 35 survivors ultimately required liver transplants. Twent y-three children are alive more than 10 years after portoenterostomy w ithout the need for transplantation. Two thirds have experienced some manifestation of portal hypertension (ie, variceal bleeding, hypersple nism, or ascites). Nineteen patients (79%) are anicteric with normal l iver synthetic function and are in an age-appropriate school grade or working and living independently. Conclusions: We found that surgical correction of biliary atresia offers long-term survival for about one quarter of patients, provides palliation until liver transplantation b ecomes necessary, and that if surgical correction is not feasible, bil iary atresia is uniformly fatal. The outlook is good for those childre n who survived more than 10 years and justifies continued attempts to establish bile flow in infants with biliary atresia.