Transplantation for childhood liver disease: An overview

The advances made in surgical technique, postoperative care, and immunosupp ression during the 1980s have permitted orthotopic liver transplantation (O LT) to evolve into an effective and widely accepted therapy for infants and children with end-stage river disease. Biliary atresia, a progressive, obl iterative disease of the bile ducts, is the most common indication for OLT in children, accounting for approximately 50% of cases. Metabolic liver dis ease (MLD) accounts for 20% to 25%; other common indications for OLT includ e fulminant hepatic failure (FHF) and forms of intrahepatic cholestasis. Th e principal problem associated with the increasing application of OLT is th e burden placed on resources, particularly the availability of donor organs . The limited pool of size-matched donor organs has led to the application of a variety of alternatives to address the needs of the pediatric recipien t; these include (I) reduced-size liver transplantation, (2) "split-liver" transplantation, and (3) use of living-related organ donors. In view of the impact on overall organ availability the use of nontransplant options, inc luding liver cell transplantation, especially for FHF and MLD, deserves bro ader application. Despite the success of transplantation, major challenges in childhood liver transplantation remain, including (1) improved preoperat ive management to ensure adequate growth, (2) more precise posttransplant m anagement of immunosuppression to ensure graft viability and avoidance of l ymphoproliferative disease, (3) earlier recognition of cytomegalovirus and Epstein-Barr virus infection, and (4) provision of services in a more cost- effective manner. The ultimate solution is to prevent liver disease through vaccination and research. Copyright (C) 1998 by the American Association f or the Study of Liver Diseases.