Dorsal pancreas agenesis and polysplenia/heterotaxy syndrome: A novel association with aortic coarctation and a review of the literature

Suraj Kapa, Ferga C. Gleeson, Santhi Swaroop Vege

Research output: Contribution to journalArticle

32 Scopus citations

Abstract

Context: Agenesis of the dorsal pancreas is very rare and may be associated with other congenital disease states. It has a rare association with polysplenia/heterotaxy syndrome. Most commonly, these states occur due to errors in development of the asymmetric organs and may be associated with benign to severe congenital cardiac malformations. Case report: We report a case of a 25-year-old male with known coarctation of the aorta who was otherwise asymptomatic. Following a routine cardiac examination, he was incidentally discovered to have an absent body and tail of the pancreas on imaging. Further testing demonstrated findings consistent with a diagnosis of polysplenia/ heterotaxy syndrome with agenesis of the dorsal pancreas. Conclusions: In patients with congenital heart disease, there is increased likelihood for the presence of other congenital malformations. In particular, polysplenia/heterotaxy syndrome, while very rare, has been shown to be associated with cardiac abnormalities. The importance in diagnosis lies in the potential clinical consequences of polysplenia/ heterotaxy syndrome and agenesis of the dorsal pancreas, including late-onset diabetes mellitus, pancreatitis, and intestinal volvulus.

Original languageEnglish (US)
Pages (from-to)433-437
Number of pages5
JournalJournal of the Pancreas
Volume8
Issue number4
StatePublished - Jul 1 2007

Keywords

  • Aortic coarctation
  • Congenital
  • Embryonic development
  • Heart defects
  • Pancreas

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Hepatology
  • Endocrinology

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