Partial internal biliary diversion for Alagille syndrome: Case report and review of the literature

Shari Sheflin-Findling, Ronen Arnon, Samantha Lee, Jaime Chu, Fiona Henderling, Nanda Kerkar, Kishore Iyer

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14 Scopus citations

Abstract

This is a case report of the first patient with Alagille syndrome (AGS) to undergo a partial internal biliary diversion (PIBD) for the treatment of symptoms refractory to medical therapy. Alagille syndrome is a hereditary disease resulting in chronic cholestasis and hypercholesterolemia that can lead to severe and intractable pruritus and disfiguring and debilitating xanthomas. PIBD has proven to be an effective treatment option for other causes of cholestatic liver disease. This report reviews the immediate and 2-year follow-up of a patient after this surgical procedure. The results suggest that PIBD has potential to provide relief of intractable symptoms and improve the quality of life in patients with AGS while avoiding an external stoma. It does not, however, appear to prevent the progression of liver disease. Long-term follow-up is still needed.

Original languageEnglish
Pages (from-to)1453-1456
Number of pages4
JournalJournal of Pediatric Surgery
Volume47
Issue number7
DOIs
StatePublished - Jul 2012

Keywords

  • Alagille syndrome
  • Hypercholesterolemia
  • Liver transplantation
  • Partial internal biliary diversion
  • Xanthoma

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