Of 47 consecutive infants with extrahepatic biliary atresia, effective bile drainage with the return of the serum bilirubin concentration to normal, was achieved in 17 (38%). Direct bile duct-to-bowl anastomosis, attempted in 15 infants, produced bile drainage in only those 4 (9%) in whom bile could be seen within the bile duct remnants at laparotomy. 13 (45%) of 29 infants subjected to portoenterostomy (direct liver-to-bowel anastomosis) had satisfactory prolonged bile drainage with normal serum bilirubin values. Although a correct preoperative diagnosis was made in each case, in 3 (6%) the 72-hour faecal rose bengal 131I excretion was greater than 10% of the injected dose, and in 5 (11%) the hepatic histology did not indicate bile duct obstruction, showing that both investigations are necessary for preoperative diagnosis. Preoperative clinical, laboratory, and hepatic histological features in the 16 jaundice-free survivors showed no significant difference when compared with the 31 infants in whom surgery was successful. Cholangitis occurred in only 7 (43%) of 16 infants with satisfactory bile drainage and was easily controlled with antibiotic treatment. No cutaneous enterostomies were performed. In most survivors liver function tests remain abnormal, but the patients are symptom-free. While it is too early to predict a long-term prognosis for these children, our eldest survivors are healthy and show normal development.
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