title: Liver transplantation in infants younger than 1 year of age.
abstract: OBJECTIVE
  The authors report on experience with liver transplantation for infants younger than 1 year of age.
  
  
  BACKGROUND
  Over the last 15 years, orthotopic liver transplant has become the only lifesaving procedure available for infants with end-stage liver disease. Many transplant centers initially required infants to reach a specific weight or age to minimize morbidity and mortality. Size-appropriate infant donors also were uncommon. As a result, many children, in the first few years of life, died of their disease. The availability of reduced-size cadaveric and living-related liver transplants has offered the ability to transplant the young infant with liver failure.
  
  
  METHODS
  The authors instituted a program to aggressively transplant infants with liver failure in the first year of life using both cadaveric and living-related liver donors.
  
  
  RESULTS
  Between June 1991 and January 1995, 13 infants were transplanted for rapidly progressive liver failure. Infant age ranged from 4 to 11 months (mean, 7.5 months). The cause of liver failure included biliary atresia (11), alpha 1-antitrypsin deficiency (1), and liver failure secondary to echovirus 7 (1). The United Network for Organ Sharing status at the time of transplant ranged from status 4, intensive care unit bound (4 patients); status 3, hospitalized (4 patients); or status 2, failing at home (5 patients). Six patients (46%) received cadaveric whole organ (2) or segmental transplants (4). Seven patients (54%) received left lateral segment living-related transplants from parental donors. After operation, patients received cyclosporine or FK506-based immunosuppression. Three patients (23%) required four retransplants (two cadaveric for primary nonfunction; one living-related for graft thrombosis in the face of fungal infection and bile leak). Postoperative complications included primary nonfunction (15%), rejection (85%), graft vascular thrombosis (15%, two of three revascularized successfully), bacterial and fungal infections (77%), and viral infections (46%). Epstein-Barr virus-associated lymphoproliferative developed in two patients (15%). Intestinal perforation requiring reoperation developed in two patients (15%). Bile leaks requiring reoperation or transhepatic stinting or both developed in three patients (23%). Two patients died in the perioperative period (< 1 month) from a combination of primary nonfunction or graft thrombosis and sepsis. Overall survival was 85%, ranging from 11.0 months to 4.5 years.
  
  
  CONCLUSIONS
  Orthotopic liver transplantation in infants younger than 1 year of age poses significant challenges from technical and infectious complications. Despite these barriers, overall patient survival is comparable to that of older children and adults.
authors.affiliation: Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: P M
authors.initials: PM
authors.lastname: Colombani
authors.forename: F G
authors.initials: FG
authors.lastname: Cigarroa
authors.forename: K
authors.initials: K
authors.lastname: Schwarz
authors.forename: B
authors.initials: B
authors.lastname: Wise
authors.forename: W E
authors.initials: WE
authors.lastname: Maley
authors.forename: A S
authors.initials: AS
authors.lastname: Klein
mesh_terms: D001656:Biliary Atresia / Q000150:complications / Q000601:surgery; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D017093:Liver Failure / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D016031:Liver Transplantation / Q000401:mortality; D011183:Postoperative Complications; D012189:Retrospective Studies; D015996:Survival Rate; D016559:Tacrolimus / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D016559:Tacrolimus