Endoscopic retrograde cholangiopancreatography in adult patients with biliary atresia

Jong Jin Hyun, Shayan S. Irani, Richard A. Kozarek

Research output: Contribution to journalArticle

Abstract

Introduction: Biliary atresia is a progressive inflammatory disease of the bile duct that eventually results in biliary cirrhosis. It is a rare neonatal disease that mandates treatment within the first 2 years of life in order for the infant to survive. Patients usually undergo palliative Kasai portoenterostomy. Even when Kasai portoenterostomy has been performed in a timely manner, progression is still inevitable. In fact, the majority of patients require curative liver transplantation at a later stage before reaching adulthood. Methods: Two jaundiced biliary atresia patients who have lived well beyond 20 years with their native liver after undergoing Kasai portoenterostomy and underwent endoscopic retrograde cholangiopancreatography (ERCP) were identified. The data on patients' clinical information, procedures performed, and outcomes were retrospectively collected by chart review. Results: Presence of a long Roux limb and acute angulation from external adhesions along with ductal anomaly from disease itself rendered ERCP challenging, and intraoperative ERCP had to be performed in 1 patient. As enteroscopes had to be used, availability of accessory devices was limited. Conclusion: Management of adult biliary atresia patients with biliary obstruction with ERCP is feasible, at times, through multidisciplinary means.

Original languageEnglish
Article numbere0603
JournalMedicine (United States)
Volume97
Issue number18
DOIs
Publication statusPublished - 2018 May 1

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Biliary Atresia
Endoscopic Retrograde Cholangiopancreatography
Infant, Newborn, Diseases
Bile Duct Diseases
Biliary Liver Cirrhosis
Rare Diseases
Jaundice
Liver Transplantation
Extremities
Equipment and Supplies
Liver

Keywords

  • ascending cholangitis
  • biliary atresia
  • endoscopic retrograde cholangiopancreatography
  • Kasai portoenterostomy

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Endoscopic retrograde cholangiopancreatography in adult patients with biliary atresia. / Hyun, Jong Jin; Irani, Shayan S.; Kozarek, Richard A.

In: Medicine (United States), Vol. 97, No. 18, e0603, 01.05.2018.

Research output: Contribution to journalArticle

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