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.
Similar content being viewed by others
References
Fukuoka K, et al. Complete agenesis of the dorsal pancreas. J Hepatobiliary Pancreat Surg. 1999;6(1):94–7.
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.
Shih HP, Wang A, Sander M. Pancreas organogenesis: from lineage determination to morphogenesis. Annu Rev Cell Dev Biol. 2013;29(1):81–105.
Voldsgaard P, Kryger-Baggesen N, Lisse I. Agenesis of pancreas. Acta Paediatr. 1994;83(7):791–3.
Baumeister FAM, Engelsberger I, Schulze A. Pancreatic agenesis as cause for neonatal diabetes mellitus. Klin Padiatr. 2005;217:76–81.
Heiberg KA. Ein Fall von fehlender Cauda pancreatis (bei einem Diabetiker). Zentralbl Allg Pathol. 1911;22:676–7.
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.
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.
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.
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.
Schnedl WJ, et al. Agenesis of the dorsal pancreas and associated diseases. Dig Dis Sci. 2009;54(3):481–7.
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.
Gold RP. Agenesis and pseudo-agenesis of the dorsal pancreas. Abdom Imaging. 1993;18(2):141–4.
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.
Uygur-Bayramiçli O, Dabak R, Kiliçoglu G, Dolapçioglu C, Öztas D. Dorsal pancreatic agenesis. J Pancreas. 2007;8(4):450–2.
Author information
Authors and Affiliations
Corresponding author
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
About this article
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
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10354-019-00717-1