Early and Peri-operative Prognostic Indicators in Infants Undergoing Hepatic Portoenterostomy for Biliary Atresia: a Review

Pediatric Gastroenterology (S Orenstein, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Pediatric Gastroenterology


Purpose of Review

Biliary atresia is the most common indication for liver transplantation among children. In recent years, prospective, multi-centre collaboration has been underway with the aim of providing high-quality data on the natural history of the condition, prior to and following hepatic portoenterostomy.

Recent Findings

There is increasing evidence that specific histological findings, and age, at the time of portoenterostomy have relevance as prognostic indicators. Recent data suggest that the sub-type of biliary atresia, its co-existence with other anomalies and concurrent infection may also be important variables.


This review provides a comprehensive summary of the histological predictors of outcome following portoenterostomy. Later age at portoenterostomy, advanced degree of hepatic fibrosis and co-existence with other congenital anomalies are strongly correlated with poor outcomes following portoenterostomy. There is increasing evidence to suggest that common serological indices and the presence or absence of cytomegalovirus (CMV) co-infection may have utility as early prognostic indicators.


Atresia Biliary Histology Kasai Outcome Predictors 


Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


Papers of particular interest, published recently, have been highlighted as: • Of importance

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Copyright information

© Springer Science+Business Media New York 2017

Authors and Affiliations

  • Robert N. Lopez
    • 1
  • Chee Y. Ooi
    • 1
    • 2
  • Usha Krishnan
    • 1
    • 2
  1. 1.Department of GastroenterologySydney Children’s HospitalSydneyAustralia
  2. 2.Discipline of PaediatricsSchool of Women’s and Children’s Health, Medicine, University of New South WalesSouth WalesAustralia

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