Liver transplantation in babies and children with extrahepatic biliary atresia.

Orthotopic liver transplantation (OLT) is a life-saving procedure for end-stage liver failure. We reviewed 39 children (24 girls, 15 boys) who received OLT for biliary atresia from 1987 to 1991. Twenty had unsuccessful portoenterostomy, 6 were referred too late for a drainage operation, and the remaining 13 achieved bile drainage but developed portal hypertension. At transplant 37 had decompensated liver disease with varices (28), ascites (24), encephalopathy (17), and gastrointestinal bleeding (12). The median weight and age at transplant were 8 kg and 12.6 months, respectively. The median waiting time was 65 days. Forty-eight grafts (30 reduced and 18 whole) were performed; graft loss was 33% and 27%, respectively. Of the 30 segmental grafts, 15 were reduced conserving the left lateral segment and hepatic vein (Brisbane technique)--13 were from the left lobe and 2 from the right lobe. The overall subject survival rate is 72%. Eleven deaths occurred: primary nonfunction (3), sepsis (3), perioperative bleed (3), and other causes (2). Early complications included: hepatic artery thrombosis (5), hepatic vein thrombosis (2), bowel perforation (3), biliary leak (3), and acute rejection (8). Later complications were chronic rejection (4) and biliary stricture requiring reconstruction (3). Follow-up at 12 months confirms good quality of life for both child and family with catch up growth and normal development. Technical advances in reduction hepatectomy have allowed us to treat small babies under 1 year with an urgent requirement for OLT, with comparable results to those obtained with whole grafts. In conclusion, in the future size and age need not be a contraindication to OLT in children with biliary atresia.