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Current Pathobiology Reports

, Volume 6, Issue 4, pp 241–246 | Cite as

Perioperative Developments in Biliary Atresia Treatment

  • O. Madadi-Sanjani
  • C. Petersen
Pathobiology of Orphan Diseases (S Ranganathan, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Pathobiology of Orphan Diseases

Abstract

Purpose of Review

This paper presents an overview of the current developments in the perioperative biliary atresia (BA) treatment.

Recent Findings

Biliary atresia is a rare cholangiopathy and the most common indication for pediatric liver transplantation. Kasai portoenterostomy is the primary therapy, leading to sufficient biliary drainage in 20–30% of patients with native liver. Multiple strategies in preoperative, operative, and postoperative care were tested to improve survival with native liver (SNL). Screening methods, centralization of care to specialized centers, laparoscopic Kasai procedure, and adjuvant steroid therapies are suggested to improve the operative outcomes.

Summary

There is no evident superiority of laparoscopic KPE nor adjuvant steroid therapy. However, screening programs and centralization of care to specialized centers are endorsed.

Keywords

Biliary atresia Kasai portoenterostomy Screening Centralization Laparoscopic Kasai 

Abbreviations

BA

Biliary atresia

BARD

Biliary atresia and related disorders

ChiLDREN

Childhood Liver Disease Research and Education Network

ERN

European Reference Networks

JF-SNL

Jaundice-free survival with native liver

KPE

Kasai portoenterostomy

SNL

Survival with native liver

START

Steroids in Biliary Atresia Randomized Trial

Notes

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.

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Center of Pediatric SurgeryHannover Medical SchoolHannoverGermany

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