Biliary atresia--surgical management. A 10-year review

The surgical results in 39 children operated on for biliary atresia at Red Cross War Memorial Children's Hospital between January 1975 and January 1985 are reported. The mean age at operation was 12.8 weeks (range 6 weeks-6 months). In the first 4 years no patient had sustained bile drainage after operation. Since 1979 16 of 32 patients (50%) have had significant bile drainage. Of these, 8 are alive and have been followed up from 6 months to 6 years after operation. Five are jaundice-free and well. The other 3 are mildly jaundiced, have evidence of ongoing liver damage, and have a poor prognosis. The deaths were due to progressive liver damage with liver failure, portal hypertension, and intercurrent infection. Cholangitis after 'successful' porto-enterostomy was the main cause of poor prognosis and can be related to the age at referral and the presence of cirrhosis of the liver at the time of surgery; thus bile drainage does not necessarily equate with cure. Early referral of infants with conjugated hyperbilirubinaemia to a major centre is of paramount importance if we are to improve the prognosis.