Skip to main content

Advertisement

Log in

Agenesis of the dorsal pancreas: a very rare entity causing diabetes mellitus

  • case report
  • Published:
Wiener Medizinische Wochenschrift Aims and scope Submit manuscript

Summary

Dorsal pancreatic agenesis is a very rare pancreatic developmental anomaly resulting in missing corpus and cauda of the pancreas. Due to improvements and more widespread use of advanced radiological techniques like CT or MRI, the possibility of finding this disorder is growing. Thus, this rare congenital condition, as well as a pancreas divisum and pseudo-agenesis secondary to chronic pancreatitis, must be considered in the differential diagnosis. Although most of the patients seem to be asymptomatic, abdominal pain and pancreatitis may develop. Moreover, this entity should be known by the treating physician, as these patients are at a high risk of developing diabetes mellitus during their lifetime. Herein, we present the case of a 65-year-old woman with complete agenesis of the dorsal pancreas. The patient was hospitalized due to weight loss, abdominal discomfort, and diabetes mellitus type 2.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  1. Fukuoka K, et al. Complete agenesis of the dorsal pancreas. J Hepatobiliary Pancreat Surg. 1999;6(1):94–7.

    Article  CAS  Google Scholar 

  2. Sakpal SV, Sexcius L, Babel N, Chamberlain RS. Agenesis of the dorsal pancreas and its association with pancreatic tumors. Pancreas. 2009;38(4):367–73.

    Article  Google Scholar 

  3. Shih HP, Wang A, Sander M. Pancreas organogenesis: from lineage determination to morphogenesis. Annu Rev Cell Dev Biol. 2013;29(1):81–105.

    Article  CAS  Google Scholar 

  4. Voldsgaard P, Kryger-Baggesen N, Lisse I. Agenesis of pancreas. Acta Paediatr. 1994;83(7):791–3.

    Article  CAS  Google Scholar 

  5. Baumeister FAM, Engelsberger I, Schulze A. Pancreatic agenesis as cause for neonatal diabetes mellitus. Klin Padiatr. 2005;217:76–81.

    Article  CAS  Google Scholar 

  6. Heiberg KA. Ein Fall von fehlender Cauda pancreatis (bei einem Diabetiker). Zentralbl Allg Pathol. 1911;22:676–7.

    Google Scholar 

  7. Cienfuegos JA, Rotellar F, Salguero J, Benito A, Solórzano JL, Sangro B. Agenesis of the dorsal pancreas: systematic review of a clinical challenge. Rev Esp Enferm Dig. 2016;108(8):479–84.

    Google Scholar 

  8. Joo YE, et al. Agenesis of the dorsal pancreas: a case report and review of the literature. Korean J Intern Med. 2006;21:236–9.

    Article  Google Scholar 

  9. Wildling R, et al. Agenesis of the dorsal pancreas in a woman with diabetes mellitus and in both of her sons. Gastroenterology. 1993;104(4):1182–6.

    Article  CAS  Google Scholar 

  10. Body-Bechou D, et al. TCF2/HNF-1beta mutations: 3 cases of fetal severe pancreatic agenesis or hypoplasia and multicystic renal dysplasia. Prenat Diagn. 2014;34(1):90–3.

    Article  CAS  Google Scholar 

  11. Schnedl WJ, et al. Agenesis of the dorsal pancreas and associated diseases. Dig Dis Sci. 2009;54(3):481–7.

    Article  Google Scholar 

  12. Sannappa RM, Buragohain J, Sarma D, Saikia UK, Choudhury BK. Agenesis of dorsal pancreas associated with periampullary pancreaticobiliary type adenocarcinoma. J Pancreas. 2014;15(5):489–92.

    Google Scholar 

  13. Gold RP. Agenesis and pseudo-agenesis of the dorsal pancreas. Abdom Imaging. 1993;18(2):141–4.

    Article  CAS  Google Scholar 

  14. Balakrishnan V, Narayanan VA, Siyad I, Radhakrishnan L, Nair P. Agenesis of the dorsal pancreas with chronic calcific pancreatitis. Case report, review of the literature and genetic basis. J Pancreas. 2006;7(6):651–9.

    Google Scholar 

  15. Uygur-Bayramiçli O, Dabak R, Kiliçoglu G, Dolapçioglu C, Öztas D. Dorsal pancreatic agenesis. J Pancreas. 2007;8(4):450–2.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Patrick Dinkhauser.

Ethics declarations

Conflict of interest

P. Dinkhauser, J. Paesold, D. Hubner, V. Steffan, R. Lampl, and H. Hofer declare that they have no competing interests.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Dinkhauser, P., Paesold, J., Hubner, D. et al. Agenesis of the dorsal pancreas: a very rare entity causing diabetes mellitus. Wien Med Wochenschr 170, 168–170 (2020). https://doi.org/10.1007/s10354-019-00717-1

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10354-019-00717-1

Keywords

Navigation