Orthotopic liver transplantation (OLT) has evolved over the past two decades to become the standard of care for end-stage liver disease in infants and children. Technical advances, particularly the use of technical variant allografts, have permitted extension of OLT into a much younger and smaller population than previously possible. Major centers around the world now routinely perform OLT in infants with survival success equivalent to that in older children and adults. We are beginning to see a small population of school-aged children who were infant OLT recipients. The further extension of OLT into neonates is more recent, with only a few pediatric centers reporting survival success. Very little is known about this frontier of transplantation. Our intent is to provide an overview of neonatal OLT using all available data and our experience in the field. (Liver Transpl 2003;9:783-788.)F or the purpose of this review, neonatal orthotopic liver transplantation (OLT) is defined as transplantation performed in the first three months of life. This does not coincide with the usual definition of the neonate, infants in their first month of life. The extension to three months is necessary, however, because of the manner in which data regarding pediatric OLT have been collated. This division also holds because most diseases necessitating neonatal OLT produce liver failure at or near the time of birth. United Network for Organ Sharing (UNOS) data show that in 2001, 623 pediatric OLTs were performed in the United States. Of these transplantations, 167 were performed in children younger than one year of age and only eight in neonates. The number of neonatal OLT procedures has been varied over the past four years from eight to 14, with the peak in 2000. Patient and graft survival, 57% and 38% respectively, remain significantly lower than that of older children. 1,2 UNOS data show that infants three to 12 months old have graft and patient survival of 72.8 % and 82.1%, respectively. Children aged one to five years have 76.8% graft and 84.1% patient survival, and those older than six years have greater than 82% graft and 91% patient survival. These data demonstrate that transplantation in neonates presents unique medical and technical challenges. The complexities of liver transplantation in this age group have received relatively little attention in the medical literature. This review details the common indications for transplantation, addresses the multiple obstacles to successful transplantation, and explores strategies to improve survival and long-term outcomes in neonatal recipients.
Defining the ProblemThe age distribution of reported neonatal OLTs is evenly distributed across the first three months of life. Woodle et al reported a series of 23 neonatal OLTs performed at three US centers: 28% were less than one month old, 35% were one to two months old, and 36% were two to three months old at the time of transplantation. 2 The youngest infant reported to undergo liver transplant was five days old. 3 The typical body weight...