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Eur J Pediatr Surg
DOI: 10.1055/a-2507-8270
Review Article

Optimizing Post-Kasai Management in Biliary Atresia: Balancing Native Liver Survival and Transplant Timing

Omid Madadi-Sanjani
1   Department of Transplant Surgery, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Marie Uecker
2   Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
,
Gordon Thomas
3   Department of Surgery, Children's Hospital at Westmead, Sydney Medical School, University of Sydney, New South Wales, Australia
,
Lutz Fischer
1   Department of Transplant Surgery, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Bianca Hegen
4   Department of Pediatric Gastroenterology and Hepatology, University Children's Hospital, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Jochen Herrmann
5   Department of Pediatric Radiology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Konrad Reinshagen
6   Department of Pediatric Surgery, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Christian Tomuschat 
6   Department of Pediatric Surgery, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
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Abstract

Background Kasai procedure (KPE) is a palliative intervention in infants with biliary atresia (BA) aiming to restore biliary drainage. While the measure of success in BA is the post-Kasai native liver survival (NLS), BA remains the most frequent indication for liver transplantation in children. While a considerable amount of children fail to clear their jaundice following KPE, resulting in early liver failure and transplantation, some children become jaundice-free after “successful” KPE. However, sequelae of chronic liver disease might affect those children, becoming a later risk for NLS. While liver transplantation is inevitable in the majority of children, various salvage procedures have been recently described to maintain NLS. This article provides a comprehensive overview of procedures performed after KPE to prolong NLS in BA patients from early childhood to late adulthood and discusses their indications and limitations.

Methods A literature-based search for surgical and radiological interventions performed in BA patients after KPE to prolong NLS (salvage procedures) was performed using PubMed. Data from case reports, retrospective studies, and registries were included.

Results Fifteen studies included 794 patients who underwent post-KPE salvage procedures. The Oxford Centre for Evidence-Based Medicine levels were IIc to IV.

Interventions included redo-Kasai's (n = 710) for cessation of bile flow post-KPE, surgical and radiological procedures (n = 14) for bile lakes, and recurrent cholangitis, shunt surgery (n = 49), and transjugular intrahepatic portosystemic shunt (n = 21) for portal hypertension in BA patients. Age at the time of salvage interventions varied considerably, including redo-Kasai's at 27 days of life and percutaneous biliary interventions in a 35-year-old.

Conclusion Salvage procedures can maintain NLS after KPE in BA patients with disease sequelae. However, indications remain scarce and liver transplantation is ultimately unavoidable in the majority of patients. While redo-Kasai numbers are globally decreasing with the advances in liver transplantation, procedures for bile lakes and portal hypertension can be viable options for patients with complications but otherwise stable liver function. Discussion on those procedures should be held by multidisciplinary expert teams, involving pediatric hepatologists, pediatric surgeons, and transplant surgeons to elaborate on the potential of maintaining NLS or proceeding with transplantation.

Keywords

biliary atresia - cholangitis - portal hypertension - redo procedure - shunt


Publication History

Received: 17 August 2024

Accepted: 22 December 2024

Accepted Manuscript online:
24 December 2024

Article published online:
03 February 2025

© 2025. Thieme. All rights reserved.

Georg Thieme Verlag KG
Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

 

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