The Kasai portoenterostomy for biliary atresia: A review of a 27-year experience with 81 patients

J Pediatr Surg. 2003 Oct;38(10):1480-5. doi: 10.1016/s0022-3468(03)00499-8.


Purpose: The aim of this study was to utilize clinical outcome methodology through multivariable analysis of perioperative factors to predict a successful Kasai-portoenterostomy (PE).

Methods: Records of 81 patients treated for biliary atresia (BA) were reviewed. Outcome was defined as successful if the patient was alive and had no liver transplant (LT). To predict future successful or failed PE, patients were categorized at 6 months post-PE into 2 groups: Success: direct bilirubin (DB) less than 2.0 mg/dL; Failure: DB greater than 2 mg/dL, or the patient was listed/had undergone LT, or had died. Groups were analyzed for positive or negative predictive values (PPV, NPV) at 2 and 5 years after PE. Cox regression was used to determine risk factors for PE.

Results: PE was successful in 38% and failed in 62%. PPV of future success was 96% at 2 years post-PE and 95% at 5 years post-PE, NPV of failure was 76% and 74%, respectively. Bridging liver fibrosis at the time of PE and postoperative cholangitic episodes were interdependent risk factors for a failed PE (P <.05). Other covariates showed no significant relationship for PE outcome.

Conclusion: Classifying of patients 6 months postoperatively allowed us to determine a successful PE outcome. Bridging liver fibrosis at the time of the Kasai, and the increased number of postoperative cholangitic episodes were predictive of a poor PE outcome.

MeSH terms

  • Biliary Atresia / epidemiology
  • Biliary Atresia / surgery*
  • Cholangitis / epidemiology
  • Comorbidity
  • Follow-Up Studies
  • Humans
  • Infant
  • Infant, Newborn
  • Liver Transplantation / mortality
  • Multivariate Analysis
  • Portoenterostomy, Hepatic / statistics & numerical data*
  • Predictive Value of Tests
  • Prognosis
  • Regression Analysis
  • Risk Assessment
  • Survival Rate
  • Treatment Outcome