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Survival after first esophageal variceal hemorrhage in patients with biliary atresia

D Miga1, R J Sokol, T Mackenzie

  • 1Pediatric Liver Center and Liver Transplantation Program, Section of Pediatric Gastroenterology, Hepatology and Nutrition, the Department of Pediatrics, University of Colorado School of Medicine and The Children's Hospital, Denver, Colorado 80218, USA.

The Journal of Pediatrics
|August 7, 2001
PubMed

Insights

Esophageal variceal hemorrhage (EVH) in children with biliary atresia significantly impacts transplant-free survival. Higher bilirubin levels at the time of EVH indicate a poorer prognosis, highlighting the need for careful monitoring.

Area of Science:

  • Pediatric Gastroenterology
  • Hepatology
  • Transplantation Surgery

Background:

  • Biliary atresia is a leading cause of pediatric liver disease requiring transplantation.
  • Esophageal variceal hemorrhage (EVH) is a serious complication in patients with biliary atresia.
  • Predicting outcomes after EVH is crucial for managing these complex cases.

Purpose of the Study:

  • To assess the impact of new-onset EVH on transplant-free survival in children with biliary atresia.
  • To identify predictors of survival following an initial EVH episode.

Main Methods:

  • Retrospective chart review of 134 pediatric patients with biliary atresia who underwent portoenterostomy.
  • Analysis of data from patients treated between 1973 and 1992 at a single institution.
  • Evaluation of the incidence and prognostic factors of EVH.

Main Results:

  • The risk of death or liver transplantation was 50% at 6 years post-EVH.
  • Transplant-free survival varied significantly with serum bilirubin levels at the time of EVH.
  • Patients with bilirubin >10 mg/dL had a 50% survival at 4 months, while those with bilirubin <=4 mg/dL had >80% survival at 4 years.

Conclusions:

  • The prognosis for children with biliary atresia experiencing EVH is strongly influenced by total serum bilirubin concentration at the time of the bleeding episode.
  • Serum bilirubin levels serve as a critical prognostic marker for transplant-free survival after EVH in this population.
Abstract

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