Long-term prognosis and factors affecting biliary atresia from experience over a 25 year period.

BACKGROUND The purpose of this study was to delineate the long-term prognosis and factors we have noted in our 25 years of experience treating patients suffering from biliary atresia (BA) who have undergone Kasai's operation. METHODS We studied 141 patients (69 male and 72 female infants) who underwent Kasai's operation at the National Taiwan University Hospital between 1976 and 2000. Factors analyzed included age at time of surgery, postoperative bile flow, frequency of cholangitis, prophylactic long-term oral antibiotics given, and intrahepatic biliary cyst formation. RESULTS The 5-year and 10-year survival rates for native liver patients were 34.8% (49/141) and 30.5% (43/141). If surgery was performed before the patient was 60 days old, the results were 44.8% (26/58) and 39.7% (23/58). Good bile flow occurred in 115 patients (81.6%) and 88 (62.4%) became jaundice-free. Cholangitis was encountered in 77 patients (54.6%) within the first two postoperative years. Prophylactic oral antibiotics lowered the rate of recurrent cholangitis (p = 0.011). Multiple intrahepatic biliary cysts occurred in 11.3% (16/141) of patients, who had a higher mortality rate when compared with the other patients (p = 0.037). The factors that had a positive effect include: (1) less than 60 days of age at time of surgery, (2) good bile flow, (3) low frequency of cholangitis, (4) long-term prophylactic antibiotics, and (5) no multiple intrahepatic cyst formation. CONCLUSION The long-term survival rate can be improved if Kasai's operation is performed early, there is a detailed dissection producing good bile flow, and long-term prophylactic antibiotics are given to prevent recurrent cholangitis and formation of multiple intrahepatic cysts.