Biliary atresia: review of experience with twenty-three patients

Prior to the Kasai procedure of hepatic porto-enterostomy in 1959, biliary atresia was a bleak chapter in paediatric surgery. It was only after many years, however, that the procedure became widely accepted. During the past 8 years 23 cases of biliary atresia have been treated at the Royal Alexandra Hospital for Children (RAHC). Twenty-one had the noncorrectable type of atresia. Sustained drainage was obtained in ten patients, all of whom had the noncorrectable type anomaly. Nine are still alive, eight having survived more than one year after operation and all are free from jaundice. Cholangitis has been a problem in six of these patients and six have evidence of fibrosis or cirrhosis on subsequent liver biopsy. Two patients have shown improvement in liver histology since operation. Sustained biliary drainage is related to age at operation, the size of biliary ductules at the porta and the subsequent development of cholangitis. Long term prognosis still remains uncertain.