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Nasobiliary drainage prior to surgical biliary diversion in progressive familial intrahepatic cholestasis type II

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Abstract

Progressive familial intrahepatic cholestasis (PFIC) can cause intense pruritus that is refractory to medical therapy. Surgical biliary diversion techniques, including partial internal biliary diversion (PIBD), have been developed over the years to relieve pruritus without requiring liver transplantation. No clinical or genetic features can currently predict postoperative pruritus response. We present three PFIC type 2 (PIFC 2) patients who underwent transient endoscopic nasobiliary drainage (NBD) prior to PIBD surgery. Two patients repeatedly responded to NBD and presented with complete pruritus resolution after subsequent PIBD. NBD failed technically in the third patient, and PIBD was partially successful. Mild post-endoscopic biological pancreatitis occurred in 2/6 NBD procedures and resolved spontaneously. The only adverse effect observed within 7 years post-PIBD was very mild transient osmotic diarrhea.

Conclusion: Our limited data suggest that NBD is a safe and effective way to predict pruritus response before performing permanent biliary diversion surgery in PFIC patients.

What is Known:

• Surgical biliary diversion techniques have been developed to relieve intractable pruritus in progressive familial intrahepatic cholestasis (PFIC).

• No clinical or genetic features can currently predict pruritus response to surgery.

What is New:

• Our data suggest that nasobiliary drainage could be a safe and effective tool to predict pruritus response to biliary diversion and avoid unnecessary surgery in PFIC patients.

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Abbreviations

ERCP:

Endoscopic retrograde cholangiopancreatography

MARS:

Molecular adsorbents recirculating system

NBD:

Nasobiliary drainage

PEBD:

Partial external biliary diversion

PFIC:

Progressive familial intrahepatic cholestasis

PIBD:

Partial internal biliary diversion

TBA:

Serum total bile acids

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Funding

GJ is supported by the Fonds National de la Recherche Scientifique et Médicale (FNRS, Belgium) through an FRIA grant (number FC29559). IS is supported by FNRS and Fondation contre le Cancer grants.

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Authors and Affiliations

  1. Department of Pediatrics, Pediatric Gastroenterology and Hepatology Unit, Cliniques Universitaires Saint-Luc, Avenue Hippocrate 10, Brussels, 1200, Belgium

    Giulia Jannone, Xavier Stephenne, Isabelle Scheers, Françoise Smets & Etienne M. Sokal

  2. Department of Surgery, Pediatric Surgery and Transplantation Unit, Cliniques Universitaires Saint-Luc, Brussels, Belgium

    Catherine de Magnée & Raymond Reding

Authors
  1. Giulia Jannone
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  2. Xavier Stephenne
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  3. Isabelle Scheers
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  4. Françoise Smets
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  5. Catherine de Magnée
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  6. Raymond Reding
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  7. Etienne M. Sokal
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Contributions

GJ contributed to study conception and design, to data collection and analysis, drafted the manuscript and approved the final manuscript. XS contributed to study conception and design and to material preparation, revised the manuscript and approved the final manuscript. IS contributed to study conception and design and to material preparation, revised the manuscript and approved the final manuscript. FS contributed to study conception and design and to material preparation, revised the manuscript and approved the final manuscript. CDM contributed to study conception and design and to material preparation, revised the manuscript and approved the final manuscript. RR contributed to study conception and design and to material preparation, revised the manuscript and approved the final manuscript. ES contributed to study conception and design and to material preparation, revised the manuscript and approved the final manuscript. 

Corresponding author

Correspondence to Giulia Jannone.

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These procedures were considered as standard medical care and did not require prior ethical committee approval.

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The authors declare that they have no conflicts of interest.

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Communicated by Peter de Winter

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Jannone, G., Stephenne, X., Scheers, I. et al. Nasobiliary drainage prior to surgical biliary diversion in progressive familial intrahepatic cholestasis type II. Eur J Pediatr 179, 1547–1552 (2020). https://doi.org/10.1007/s00431-020-03646-z

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  • DOI: https://doi.org/10.1007/s00431-020-03646-z

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