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Perioperative Developments in Biliary Atresia Treatment

  • Pathobiology of Orphan Diseases (S Ranganathan, Section Editor)
  • Published:
Current Pathobiology Reports

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.

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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

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Authors and Affiliations

  1. Center of Pediatric Surgery, Hannover Medical School, Carl-Neuberg-Street 1, 30625, Hannover, Germany

    O. Madadi-Sanjani & C. Petersen

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  1. O. Madadi-Sanjani
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  2. C. Petersen
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Correspondence to O. Madadi-Sanjani.

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Madadi-Sanjani, O., Petersen, C. Perioperative Developments in Biliary Atresia Treatment. Curr Pathobiol Rep 6, 241–246 (2018). https://doi.org/10.1007/s40139-018-0181-y

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