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.
Practice Guideline
Long-Term Management of the Successful Pediatric Liver Transplant [updated August 2013]
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.