Digestive Diseases and Sciences

, Volume 54, Issue 3, pp 481–487 | Cite as

Agenesis of the Dorsal Pancreas and Associated Diseases

  • Wolfgang J. Schnedl
  • Claudia Piswanger-Soelkner
  • Sandra J. Wallner
  • Pia Reittner
  • Robert Krause
  • Rainer W. Lipp
  • Hans E. Hohmeier
Review

Abstract

Background Agenesis of the dorsal pancreas is a very rare congenital pancreatic malformation and is associated with some other diseases. Methods A PubMed search revealed 53 cases of agenesis of the dorsal pancreas. Results In 28 patients with this congenital malformation hyperglycemia was demonstrated, 27 had abdominal pain, 16 had pancreatitis, 14 had an enlarged or prominent pancreatic head visible on computed tomography, and in a few cases, polysplenia, which may occur with various congenital anomalies of visceral organs, was described. Conclusions Difficulties involved in obtaining a firm diagnosis have led to a variety of terms being used to describe this congenital disease. Diagnosis of agenesis of the dorsal pancreas is inconclusive without demonstration of the absence of the dorsal pancreatic duct. Here we describe the embryological development of the pancreas, the so-far known cases of agenesis of the dorsal pancreas with associated medical problems, and the diagnostic measures to find the right conclusions.

Keywords

Pancreas development Agenesis of dorsal pancreas Pancreas hypoplasia Short pancreas Diabetes mellitus Abdominal pain Pancreatitis 

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Copyright information

© Springer Science+Business Media, LLC 2008

Authors and Affiliations

  • Wolfgang J. Schnedl
    • 1
    • 2
  • Claudia Piswanger-Soelkner
    • 1
  • Sandra J. Wallner
    • 3
  • Pia Reittner
    • 4
  • Robert Krause
    • 1
  • Rainer W. Lipp
    • 1
  • Hans E. Hohmeier
    • 5
  1. 1.Department of Internal MedicineMedical UniversityGrazAustria
  2. 2.Practice for General Internal MedicineLiezenAustria
  3. 3.Institute of PathophysiologyCenter for Molecular MedicineGrazAustria
  4. 4.Diagnostikum Sued-WestGrazAustria
  5. 5.Division of Endocrinology, Metabolism, and Nutrition, Department of MedicineDuke University Medical Center Durham 3813DurhamUSA

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