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 language | English (US) |
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Pages (from-to) | 433-437 |
Number of pages | 5 |
Journal | Journal of the Pancreas |
Volume | 8 |
Issue number | 4 |
State | Published - Jul 2007 |
Keywords
- Aortic coarctation
- Congenital
- Embryonic development
- Heart defects
- Pancreas
ASJC Scopus subject areas
- Endocrinology, Diabetes and Metabolism
- Hepatology
- Endocrinology