IMPROVED SURVIVAL IN BILIARY ATRESIA PATIENTS IN THE PRESENT ERA OF LIVER-TRANSPLANTATION

Therapy for patients with biliary atresia (BA) has become controversial, with orthotopic liver transplantation (OLTx) suggested in place of portoenterostomy. This is based on the unpredictable success of portoenterostomy, and the increased difficulty of the OLTx procedure following prior extensive liver surgery. The survival rate reported here for infants transplanted after unsuccessful protoenterostomy does not support this approach. OLTx was undertaken in 37 patients when end-stage liver failure followed primary portoenterostomy. Recipient age ranged from 6 months to 14 years (median, 13 months), and weight ranged from 5 to 45 kg (median, 8 kg) at the time of OLTx. Reduced-size allografts were used as the primary allograft in 25 patients (23 left lobe), and 12 received whole-organ allografts. Retransplantation was required in 5 patients, each received a reduced-size allograft. There was no increased incidence of vascular complications, primary nonfunction, irreversible rejection, intestinal perforation, biliary complications, sepsis, or lymphoma comparing the BA patients with all other non-BA patients who had undergone OLTx (all P = .16). There was no statistically significant difference in mean operative blood loss between BA patients (EBL = 1.99 BV) and non-BA patients (1.50 BV) (P = .14). Actuarial survival for the series of BA patients was 89% at 1 year, and 80% at 2 years. Following the introduction of reduced-size allografts, donor organs were selected for use with a priority on donor stability. The actuarial survival for BA patients during this time has improved to 96% at 1 year, and 91% at 2 years. Patient survival at 1 year following OLTx was 78% for whole organ allograft recipients, and 100% for reduced-size allograft recipients. No patient with BA died on the waiting list since the introduction of reduced-size OLTx procedures in 1988. This series supports the sequential use of primary portoenterostomy and subsequent liver transplantation if hepatic failure appears to be progressive or portal hypertensive complications occur in the treatment of BA. OLTx can be successfully and safely undertaken if portoenterostomy does not prevent the development of end-stage liver failure. Reduced-size liver transplant techniques have improved survival and helped eliminate waiting list mortality. These two procedures are complimentary rather then competitive alternatives in the care of this complex disease. © 1993.