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Volume 10
Issue 6
10.3390/children10060949
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Review

Surgical versus Medical Management of Progressive Familial Intrahepatic Cholestasis—Case Compilation and Review of the Literature

by
Maria Noelle Hüpper
1,
Judith Pichler
2,
Wolf-Dietrich Huber
2,
Andreas Heilos
2,
Rebecca Schaup
2,
Martin Metzelder
1 and
Sophie Langer
1,*
1
Department of Paediatric Surgery, Medical University of Vienna, 1090 Vienna, Austria
2
Department of Paediatric Gastroenterology, Department of Paediatrics, Medical University of Vienna, 1090 Vienna, Austria
*
Author to whom correspondence should be addressed.
Children 2023, 10(6), 949; https://doi.org/10.3390/children10060949
Submission received: 14 April 2023 / Revised: 13 May 2023 / Accepted: 23 May 2023 / Published: 26 May 2023
(This article belongs to the Special Issue Abdominal Surgery in Pediatrics Update)
Review Reports Versions Notes

Abstract

(1) Background: Progressive familial intrahepatic cholestasis (PFIC) is a rare cause of liver failure. Surgical biliary diversion (SBD) and ileal bile salt inhibitors (IBAT) can delay or prevent liver transplantation (LTX). A comparison of the two methodologies in the literature is lacking. The combination has not been investigated. (2) Methods: We performed a literature survey on medical and surgical treatments for PFIC and reviewed the charts of our patients with PFIC of a tertiary hospital. The end points of our analysis were a decrease in serum bile acid (sBA) levels, reduction of pruritus and delay or avoidance of (LTX). (3) Results: We included 17 case series on SBD with more than 5 patients and a total of 536 patients. External or internal SBD, either conventional or minimally invasive, can reduce pruritus and sBA, but not all PFIC types are suitable for SBD. Six publications described the use of two types of IBAT in PFIC with a total of 118 patients. Treatment response was dependent on genetic type and subtype. Patients with PFIC 2 (nt-BSEP) showed the best response to treatment. Four out of eleven PFIC patients underwent SBD at our centre, with two currently receiving IBAT. (4) Conclusions: Limited data on IBAT in selected patients with PFIC show safety and effectiveness, although surgical methods should still be considered as a successful bridging procedure. Further studies to evaluate a possible combination of IBAT and SBD in PFIC are warranted and treatment decision should be discussed in an interdisciplinary board.
Keywords: progressive familial intrahepatic cholestasis; surgery; children; odevixibat; maralixibiat; biliary diversion; bile acid; pruritus progressive familial intrahepatic cholestasis; surgery; children; odevixibat; maralixibiat; biliary diversion; bile acid; pruritus

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MDPI and ACS Style

Hüpper, M.N.; Pichler, J.; Huber, W.-D.; Heilos, A.; Schaup, R.; Metzelder, M.; Langer, S. Surgical versus Medical Management of Progressive Familial Intrahepatic Cholestasis—Case Compilation and Review of the Literature. Children 2023, 10, 949. https://doi.org/10.3390/children10060949

AMA Style

Hüpper MN, Pichler J, Huber W-D, Heilos A, Schaup R, Metzelder M, Langer S. Surgical versus Medical Management of Progressive Familial Intrahepatic Cholestasis—Case Compilation and Review of the Literature. Children. 2023; 10(6):949. https://doi.org/10.3390/children10060949

Chicago/Turabian Style

Hüpper, Maria Noelle, Judith Pichler, Wolf-Dietrich Huber, Andreas Heilos, Rebecca Schaup, Martin Metzelder, and Sophie Langer. 2023. "Surgical versus Medical Management of Progressive Familial Intrahepatic Cholestasis—Case Compilation and Review of the Literature" Children 10, no. 6: 949. https://doi.org/10.3390/children10060949

APA Style

Hüpper, M. N., Pichler, J., Huber, W.-D., Heilos, A., Schaup, R., Metzelder, M., & Langer, S. (2023). Surgical versus Medical Management of Progressive Familial Intrahepatic Cholestasis—Case Compilation and Review of the Literature. Children, 10(6), 949. https://doi.org/10.3390/children10060949

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