http://www.ncbi.nlm.nih.gov/pubmed/26418214
Liver Transplantation for Biliary Atresia: Is There a Difference in Outcome for Infants?

Arnon R, Annunziato RA, D'Amelio G, Chu J, Shneider BL. Liver Transplantation for Biliary Atresia: Is There a Difference in Outcome for Infants? J Pediatr Gastroenterol Nutr. 2016 Feb;62(2):220-5.

Abstract

OBJECTIVES:
Liver transplantation (LT) in children with biliary atresia (BA) is often performed because of poor bile drainage, complications of biliary cirrhosis, or recurrent cholangitis. Poor bile drainage after a Kasai hepatoportoenterostomy is the primary driver for LT in infancy. The aim of the present study was to compare the clinical characteristics and outcome of first isolated liver transplantation for infants with BA who underwent transplant before 2 years of age (transplanted at infancy [TAI]) with children transplanted later in life (age 2-<18 years = transplanted at childhood [TAC]).

METHODS:
Children with BA who underwent LT between 2002 and 2012 were identified from the United Network for Organ Sharing Standard Transplant Analysis and Research data set files.

RESULTS:
A total of 1818 children underwent first isolated liver transplantation for BA (TAI 1408 [77.4%]; TAC 410 [22.6%]). One and 5-year patient survival of the TAI and TAC patients was 95.2%, 93.8%, and 97.8 %, 97.1%, respectively (P < 0.01 for both periods). One and 5-year graft survival of the TAI and TAC patients was 87.6%, 84.6 % and 93.2%, 90.7%, respectively (P < 0.01 for both periods). Removal from the waitlist for disease progression or death was significantly higher for TAI compared with TAC (120 patients [6.3%] vs 21 [3.7%], P = 0.02). Cold ischemic time was found to be the prognostic factor for death after LT in TAI, whereas being on life support was a poor prognostic factor in TAC by multivariate risk factor analysis.

CONCLUSIONS:
The vast majority of transplants for BA occur in children <2 years of age. Younger patients with BA had significantly higher waitlist and posttransplant mortality. Given the consistently poorer outcomes, there is an urgent need to find methods to improve bile drainage after the Kasai hepatoportoenterostomy.