Abstract
Seventy-six patients with biliary atresia (BA) were seen at the Kobe Children's Hospital during the period from 1971 to 1989. We reviewed the medical records of these patients to clarify the timing of orthotopic liver transplantation (OLT) and to elucidate the role of re-do portoenterostomy. Seventy-six patients were divided into three groups according to their postoperative courses; Group I (n=35): good bile flow and serum bilirubin≦1.0 mg/dl within 2 yrs after operation. There were 3 deaths. The cause of death was ruptured esophageal varices. Another three developed intrapulmonary shunt. A few patients even without jaundice in this category, cirrhosis gradually progressed, ultimately requiring OLT. Portal vein thrombosis and intrapulmonary shunt should be carefully followed. Group II (n=27): fair bile flow but serum bilirubin>1.0mg/dl 2 yrs after operation. There were 19 deaths. Growth retardation, ascites accumulation and variceal bleeding sequentially developed in these patients when serum bilirubin exceeded 10 mg/dl and chE became low (150-200 IU/l). This seems to be the proper timing of OLT because nothing is gained from awaiting OLT at this point. Group III (n=14): no bile flow. All died except for one who had OLT. Nutritional managent is mandatory in this category for a successful OLT. Re-do Kasai operation was done in 22 patients and 11 of these are now alive including one who survived OLT. Re-do portoenterostomy for failed Kasai is encouraging because it can achieve biliary drainage good enough in about half of the patients to survive long-term in a satisfactory quality of life. OLT can be deferred, allowing the patients to grow in the meantime.
