Beyond the Pediatric end-stage liver disease system: solutions for infants with biliary atresia requiring liver transplant

World J Gastroenterol. 2014 Aug 28;20(32):11062-8. doi: 10.3748/wjg.v20.i32.11062.


Biliary atresia (BA), a chronic progressive cholestatic disease of infants, is the leading cause for liver transplant in children, especially in patients under two years of age. BA can be successfully treated with the Kasai portoenterostomy; however most patients still require a liver transplant, with up to one half of BA children needing a transplant by age two. In the current pediatric end-stage liver disease system, children with BA face the risk of not receiving a liver in a safe and timely manner. In this review, we discuss a number of possible solutions to help these children. We focus on two general approaches: (1) preventing/delaying need for transplantation, by optimizing the success of the Kasai operation; and (2) expediting transplantation when needed, by performing techniques other than the standard deceased-donor, whole, ABO-matched organ transplant.

Keywords: ABO-incompatible liver transplantation; Biliary atresia; Kasai operation; Liver transplantation; Living-related donor transplantation; Newborn screening; Pediatric end-stage liver disease; Pediatric liver disease; Split liver transplantation; Surgical outcomes.

Publication types

  • Review

MeSH terms

  • Age Factors
  • Biliary Atresia / diagnosis
  • Biliary Atresia / mortality
  • Biliary Atresia / surgery*
  • Decision Support Techniques*
  • End Stage Liver Disease / diagnosis
  • End Stage Liver Disease / mortality
  • End Stage Liver Disease / surgery*
  • Humans
  • Infant
  • Liver Transplantation* / adverse effects
  • Liver Transplantation* / methods
  • Liver Transplantation* / mortality
  • Portoenterostomy, Hepatic* / adverse effects
  • Portoenterostomy, Hepatic* / mortality
  • Risk Factors
  • Time-to-Treatment
  • Tissue Donors / supply & distribution
  • Treatment Outcome
  • Waiting Lists