The Kasai procedure in the treatment of biliary atresia.

From 1978 to 1992, 62 patients were diagnosed as having extrahepatic biliary atresia (EHBA) at Childrens Hospital Los Angeles. The patients presented with either persistent jaundice, alcoholic stools, and/or hepatomegaly. Hepatobiliary IDA scans were performed in 47 of the patients; 46 had results typical of EHBA. Fifty-nine of the 62 patients underwent a Kasai portoenterostomy; three patients were more than 4 months of age at initial presentation and were referred directly for liver transplantation. The preoperative total bilirubin level for all patients averaged 8.6 mg/dL (range, 4.1 to 18.1). All patients underwent a standardized Kasai procedure using a 40-cm Roux-en-Y intestinal segment performed in the end-to-side fashion. Postoperative management included oral antibiotics and fat-soluble vitamins for at least 9 months. Long-term complications included cholangitis in 20 cases and portal hypertension in 25, which led to variceal hemorrhage in 12 cases. Growth rates were at or above the 50th percentile for age in 59% of the Kasai patients. Forty-one of the 59 Kasai patients survived (69.5%); six deaths occurred within 2 years after the Kasai procedure. Twelve patients were lost to follow-up within 2 years after surgery, and for calculation of mortality were presumed dead. Seventeen patients had follow-up for 5 or more years, 13 had follow-up for 2 to 5 years, and 29 had follow-up for less than 2 years. The average total bilirubin level for the patients with less than 2 years of follow-up was 7.9 mg/dL (0.3 to 20.8), and that for the patients with more than 2 years of follow-up was 1.6 mg/dL (0.3 to 18.1). Orthotopic liver transplantation was performed in 11 Kasai patients, in whom chronic liver failure eventually developed. Rejection occurred in one of these patients, which required retransplantation. Based on these results, the Kasai portoenterostomy procedure continues to offer palliation, if not long-term success, in a large percentage of patients with EHBA.