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DOI:
https://doi.org/10.57187/5278

Clinical reasoning: Case report

Vol. 156 No. 7 (2026)

Dorsal pancreatic agenesis revealed by acute biliary pancreatitis in a postpartum woman with gestational diabetes: a case report

Cite this as:
Swiss Med Wkly. 2026;156:5278
Published
28.07.2026

Summary

Dorsal pancreatic agenesis is an exceptionally rare congenital anomaly involving the partial or complete absence of the pancreatic body and tail. Metabolic dysfunction, including impaired glucose tolerance and insulin-dependent diabetes, is frequently reported; however, the relationship between dorsal pancreatic agenesis and gestational diabetes mellitus has been reported only sporadically.

The objective of this report is to present a rare case of dorsal pancreatic agenesis revealed by acute biliary pancreatitis and recurrent gestational diabetes mellitus and to review the relevant literature concerning its metabolic and clinical implications.

We report the case of a postpartum woman in her early 30s who presented with acute biliary pancreatitis. Imaging revealed complete agenesis of the pancreatic body and tail, with preservation of the pancreatic head derived from the ventral bud. She had a history of recurrent gestational diabetes, raising the possibility that pregnancy-associated insulin resistance had unmasked a compensated beta-cell deficit due to dorsal pancreatic agenesis. The patient underwent an uncomplicated laparoscopic cholecystectomy with symptom resolution.

This case highlights three clinically significant observations: (1) recurrent gestational diabetes mellitus may represent an early clinical clue to reduced pancreatic endocrine reserve in dorsal pancreatic agenesis; (2) acute biliary pancreatitis can incidentally reveal congenital pancreatic anomalies; and (3) structural pancreatic abnormalities should be considered in patients with atypical or recurrent gestational diabetes mellitus. The association between dorsal pancreatic agenesis and gestational diabetes has been reported, albeit rarely, but the interaction between recurrent gestational diabetes mellitus, obesity, and reduced pancreatic endocrine reserve remains insufficiently characterised.

References

  1. 1. Shubhankar G, Panwar V. Incidentally detected dorsal pancreatic agenesis: hidden clinical implications. Abdom Radiol (NY). 2026 Apr;51(4):1792–6. 10.1007/s00261-025-05167-5
  2. 2. Mei W, Cao F, Li F. Two cases of agenesis of the dorsal pancreas and a review of the literature. BMC Gastroenterol. 2020 Apr;20(1):94. 10.1186/s12876-020-01245-8
  3. 3. Xia LZ, Bu XF, Jiang PC, Yu F, Zhang YJ, Meng NN. Agenesis of the dorsal pancreas with chronic suppurative pancreatitis: case report and literature review. Medicine (Baltimore). 2021 Dec;100(49):e28137. 10.1097/MD.0000000000028137
  4. 4. Schnedl WJ, Piswanger-Soelkner C, Wallner SJ, Reittner P, Krause R, Lipp RW, et al. Agenesis of the dorsal pancreas and associated diseases. Dig Dis Sci. 2009 Mar;54(3):481–7. 10.1007/s10620-008-0370-3
  5. 5. Pasaoglu L, Vural M, Hatipoglu HG, Tereklioglu G, Koparal S. Agenesis of the dorsal pancreas. World J Gastroenterol. 2008 May;14(18):2915–6. 10.3748/wjg.14.2915
  6. 6. 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 Aug;108(8):479–84. 10.17235/reed.2016.4474/2016
  7. 7. Liang K, Ou X, Huang X, Lan Q. Agenesis of the dorsal pancreas: a rare cause of insulin-dependent diabetes without abdominal pain: Case report. Medicine (Baltimore). 2018 Mar;97(9):e0046. 10.1097/MD.0000000000010046
  8. 8. Yang T, Yang X, Wang L, Mo J. Agenesis of the dorsal pancreas presenting with diabetic ketoacidosis - a case report and literature review. BMC Endocr Disord. 2019 Nov;19(1):120. 10.1186/s12902-019-0449-1
  9. 9. Denson AM, Rodriguez KE, Letourneau-Freiberg LR, Kandasamy B, Naylor RN, Abdullatif HD, et al. Children With Pancreatic Hypoplasia Experience Poor Weight Gain and Labile Diabetes but Low Incidence of DKA [Erratum in: J Clin Endocrinol Metab. 2026 Mar 17;111] [4] [:e1216-e1221. doi: 10.1210/clinem/dgag013. PMID: 41006196; PMCID: PMC13017217]. J Clin Endocrinol Metab. 2026 Mar;111(4):e1164–73. 10.1210/clinem/dgaf473 10.1210/clinem/dgaf473
  10. 10. Oldenburg B, van Leeuwen MS, van Berge Henegouwen GP, Koningsberger JC. Pancreatitis and agenesis of the dorsal pancreas. Eur J Gastroenterol Hepatol. 1998 Oct;10(10):887–9. 10.1097/00042737-199810000-00013
  11. 11. Sakpal SV, Sexcius L, Babel N, Chamberlain RS. Agenesis of the dorsal pancreas and its association with pancreatic tumors. Pancreas. 2009 May;38(4):367–73. 10.1097/MPA.0b013e318196c401
  12. 12. Rittenhouse DW, Kennedy EP, Mascaro AA, Brumbaugh JL, Stein LH, Rosenberger LH, et al. The novel triad of dorsal agenesis of the pancreas with concurrent pancreatic ductal adenocarcinoma and nonalcoholic chronic calcific pancreatitis: a case series and review of the literature. J Gastrointest Surg. 2011 Sep;15(9):1643–9. 10.1007/s11605-011-1542-6
  13. 13. Low JP, Williams D, Chaganti JR. Polysplenia syndrome with agenesis of the dorsal pancreas and preduodenal portal vein presenting with obstructive jaundice—a case report and literature review. Br J Radiol. 2011 Nov;84(1007):e217–20. 10.1259/bjr/27680217
  14. 14. Zhong JW, Yu LM, Ying QJ, Peng WW, Xu CZ, Cai ZZ, et al. Performance of endoscopic ultrasound for diagnosis of agenesis of the dorsal pancreas: a case report. J Int Med Res. 2020 May;48(5):300060519884148. 10.1177/0300060519884148
  15. 15. Mehta V, Hopson PE, Smadi Y, Patel SB, Horvath K, Mehta DI. Development of the human pancreas and its exocrine function. Front Pediatr. 2022 Sep;10:909648. 10.3389/fped.2022.909648
  16. 16. Allen HL, Flanagan SE, Shaw-Smith C, De Franco E, Akerman I, Caswell R, et al.; International Pancreatic Agenesis Consortium. GATA6 haploinsufficiency causes pancreatic agenesis in humans. Nat Genet. 2011 Dec;44(1):20–2. 10.1038/ng.1035
  17. 17. Weedon MN, Cebola I, Patch AM, Flanagan SE, De Franco E, Caswell R, et al.; International Pancreatic Agenesis Consortium. Recessive mutations in a distal PTF1A enhancer cause isolated pancreatic agenesis. Nat Genet. 2014 Jan;46(1):61–4. 10.1038/ng.2826
  18. 18. Wildling R, Schnedl WJ, Reisinger EC, Schreiber F, Lipp RW, Lederer A, et al. Agenesis of the dorsal pancreas in a woman with diabetes mellitus and in both of her sons. Gastroenterology. 1993 Apr;104(4):1182–6. 10.1016/0016-5085(93)90290-s doi: https://doi.org/10.1016/0016-5085(93)90290-S