OUR FIRST 100 CONSECUTIVE PEDIATRIC LIVER-TRANSPLANTS

Orthotopic liver transplantation (OLT) is nowadays accepted as the bes t therapy for end-stage liver disease. The difficulties involved are e ven greater in children than in adults, and it is debatable whether ex clusively pediatric programs are warranted. The aim of this paper is t o analyze our experience at the Children's Hospital ''La Paz'', with t he first consecutive 100 OLT in children, 61% of whom weighed less tha n 20 kg. Since 1988, 220 pediatric patients were evaluated as candidat es, 100 OLT were performed in 78 patients and 13 died on the waiting l ist, currently maintained below 15 cases. Indications were: cholestasi s (45), metabolic disease (18), fulminant hepatic failure (3), primary liver tumors (2) and cirrhosis (10). Mean age was 66 months (range = 7 to 216) with a mean weight of 21 kg (range = 6 to 60), twenty patien ts weighed less than 13 kilograms. OLT was performed by standard techn ique. Reduced or segmental grafts were necessary in 8 instances. Twent y-two patients were retransplanted and 2 received three grafts. Indica tions for retransplantation were: hepatic artery thrombosis (8), prima ry nonfunction (4), chronic rejection (7), portal thrombosis (2) and B udd-Chiari recurrence (1). Acute rejection was observed in 52 patients , and eight cases developed a chronic rejection. These episodes were t reated with ''bolus'' of steroids, monoclonal antibodies (OKT-3) and F K-506. Surgical complications included: hepatic artery thrombosis 12%, portal vein thrombosis 3% and biliary fistula or stenosis 13%. The in cidence of primary non-function was 7%. Actuarial survival rate at 5 y ears was 75%. Survival rate for children under one year was 64% and th e difference with that of the remaining patients was not statistically significant. Our results confirm that OLT may allow adequate long-ter m development for patients with end-stage liver disease. The procedure can be performed with acceptable morbidity and mortality within a pur ely pediatric program in designated institutions where these children may meet the best care for their age.