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Liver Transplantation for Biliary Atresia: Is There a Difference in Outcome for Infants?
Ronen Arnon1, Rachel A Annunziato, Guiseppe D'Amelio
1*Recanati/Miller Transplantation Institute †Department of Pediatrics, Mount Sinai Medical Center, New York ‡Department of Psychology, Fordham University, Bronx, NY §Department of Pediatrics, Baylor College of Medicine, Houston, TX.
Insights
Pediatric liver transplantation (LT) for biliary atresia (BA) is most common in infants. However, infants transplanted for BA face higher mortality rates, highlighting the need for improved bile drainage strategies post-Kasai hepatoportoenterostomy.
Area of Science:
- Pediatric Hepatology
- Transplant Surgery
- Gastroenterology
Background:
- Biliary atresia (BA) is a leading cause of pediatric liver transplantation (LT).
- Poor bile drainage after Kasai hepatoportoenterostomy is a primary indication for LT in infants with BA.
- LT is performed in children with BA due to complications like biliary cirrhosis or recurrent cholangitis.
Purpose of the Study:
- To compare clinical characteristics and outcomes of first isolated LT for BA in infants (transplanted at infancy [TAI], <2 years) versus children (transplanted at childhood [TAC], 2-<18 years).
Main Methods:
- Utilized the United Network for Organ Sharing Standard Transplant Analysis and Research data set.
- Identified children with BA who underwent first isolated LT between 2002 and 2012.
- Compared outcomes between TAI and TAC groups.
Main Results:
- 1818 children underwent LT for BA; 77.4% were TAI and 22.6% were TAC.
- One and 5-year patient survival was 95.2%/93.8% (TAI) and 97.8%/97.1% (TAC).
- One and 5-year graft survival was 87.6%/84.6% (TAI) and 93.2%/90.7% (TAC).
- Waitlist removal for disease progression or death was higher in TAI (6.3%) vs TAC (3.7%).
- Cold ischemic time predicted death in TAI; life support status predicted death in TAC.
Conclusions:
- The majority of LT for BA occurs in children under 2 years old.
- Younger BA patients (TAI) exhibit significantly higher waitlist and post-LT mortality.
- Improved bile drainage methods after Kasai hepatoportoenterostomy are crucial for better outcomes in infants with BA.
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.
