Long-Term Management of the Pediatric Liver Transplant
AASLD develops evidence-based practice guidelines and practice guidances which are updated regularly by a committee of hepatology experts and include recommendations of preferred approaches to the diagnostic, therapeutic, and preventive aspects of care.
Pediatric Liver Transplantation (LT) has dramatically changed the prognosis for many infants and children with liver failure and metabolic disease. As survival increases, long-term maintenance resources exceed perioperative care requirements. The commonest indication for LT is biliary atresia, which accounts for 50% of children requiring transplantation in the United States and for 74% in Europe. Most early deaths occur within 3 months after transplantation. The main causes of graft loss in the first week include primary nonfunction, hepatic artery thrombosis (HAT) or portal vein thrombosis (PVT), systemic sepsis, and multiorgan failure (<10%). Other significant complications are acute rejection (AR; 50%), chronic rejection (CR; 10%), biliary leaks and strictures (5%-25%), viral infections [especially cytomegalovirus (CMV) and Epstein-Barr virus (EBV)], acute kidney injury, and fluid imbalance. The 1-year patient survival rate is 90%, and the survival rate is 75% at 15 to 20 years with good quality of life. Survival after transplantation for acute liver failure has improved from 70% at 1 year to 87%, with 5-year survival rates of 67% to 80%. The documented 5-year survival rates for transplantation are >90% for chronic liver disease and 89% for metabolic liver disease. Vital to survival are improved selection (prioritization and management of candidates with the Pediatric End-Stage Liver Disease score), better preoperative management of hepatic complications and nutritional support, innovative surgical techniques for expanding the donor pool, and improved postoperative immunosuppression and management.