Management








Liver transplant













First performed in a pediatric patient in 1963, more than 10,000 children have now undergone successful liver transplantation in the U.S. alone. Reported 1 year survivals are over 90%, with 5 year survival rates close to 80%. Almost half of the pediatric liver transplants are in infants less than 2 years old and 75% of those are for cholestatic diseases. Liver transplant is currently a well-accepted treatment for both chronic and acute pediatric liver failure. Even with improved surgical and postoperative management, though, mortality rate is around 10%. There remain a few contraindications.

Long term postoperative management problems are many. Side effects of the prolonged immunosuppression include hypertension, growth failure, hirsutism and nephro-, neuro- and cardiotoxicity. Although as many as 20% of adults may develop graft tolerance allowing withdrawal from immunosuppression, this has not been reported in children.

Posttransplant infection with CMV occurs in 20% of patients and EBV infection in up to 50%. The latter is especially worrisome because of the possibility of developing EBV-associated lymphoproliferative disorders posttransplant. These range from benign lymphoid hyperplasia to malignant lymphoma requiring chemotherapy. Bacterial and fungal infections can also occur posttransplant.

Late surgical complications include bile duct strictures or leaks, as well as PV stenosis leading (again) to portal hypertension. Hepatic artery or PV thrombosis can also occur acutely in the immediate postoperative period.

Long term quality of life depends on the original liver disease. Many metabolic defects are not confined to the liver (e.g. organic acidemias); therefore treatment of the underlying disorder, commonly dietary restrictions, may need to continue posttransplant. Recurrence of the original liver disease may also occur posttransplant, typically chronic hepatitis or autoimmune hepatitis.

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