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Portal hypertensive biliopathy: a rare cause of childhood cholestasis

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Abstract

Portal hypertensive biliopathy (PHB) is defined as abnormal biliary changes that take place most likely secondary to extrahepatic portal vein obstruction (EHPVO) with portal hypertension. This condition may be asymptomatic or could lead to a cholestatic state, which is not well-described in children. We report a child who developed a cholestatic nature with portal hypertension some time after having neonatal surgery for duodenal atresia. We discuss the differential diagnosis and management of this rare condition. Symptomatic PHB has been only rarely reported in children. It should be suspected in patients with portal hypertension and having features of biliary obstruction. Hepaticojejunostomy may have a therapeutic role in selected patients in whom endoscopic or percutaneous manipulation of the biliary tree is unsuccessful and who have not responded to a surgical portal-systemic shunt procedure.

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Article 08 April 2021

Nagesh N. Swamygowda, Rajesh Pendlimari & Kaushik Subramanian

Abbreviations

PHB:

portal hypertensive biliopathy

EHPVO:

extrahepatic portal vein obstruction

ERC:

endoscopic retrograde cholangiography

ANCA:

anti-neutrophil cytoplasmic antibody

PSC:

primary sclerosing cholangitis

CT:

computerized tomography

USS:

ultrasound survey

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Authors and Affiliations

  1. Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, ON, Canada

    Wael El-Matary, Eve A. Roberts & Simon C. Ling

  2. Division of General Surgery, The Hospital for Sick Children, Toronto, ON, Canada

    Peter Kim

  3. Division of Diagnostic Imaging, The Hospital for Sick Children, Toronto, ON, Canada

    Michael Temple

  4. Division of Pathology, The Hospital for Sick Children, Toronto, ON, Canada

    Ernest Cutz

  5. Division of Pediatric Gastroenterology, Faculty of Medicine, University of Alberta, 9222, Aberhart Centre 1, 1402 University Avenue, Edmonton, AB, T6G 2J3, Canada

    Wael El-Matary

Authors
  1. Wael El-Matary
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  2. Eve A. Roberts
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  3. Peter Kim
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  4. Michael Temple
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  5. Ernest Cutz
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  6. Simon C. Ling
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Correspondence to Wael El-Matary.

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El-Matary, W., Roberts, E.A., Kim, P. et al. Portal hypertensive biliopathy: a rare cause of childhood cholestasis. Eur J Pediatr 167, 1339–1342 (2008). https://doi.org/10.1007/s00431-008-0675-4

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  • DOI: https://doi.org/10.1007/s00431-008-0675-4

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