Case Report

A Case Report of Dorsal Pancreatic Agenesis and Uterus Didelphys in a Young Woman: A Seldomreported Association

Dorsal Pancreatic Agenesis and Uterus Didelphys

Abstract

Agenesis of the dorsal pancreas is a rare congenital anomaly that often remains asymptomatic and is  requently discovered incidentally during imaging for other conditions. Its association with Müllerian anomalies, such as uterus didelphys, suggests that both conditions may stem from similar embryological disruptions  during early fetal development. This report aims to raise awareness of Agenesis of the Dorsal Pancreas (ADP) and its potential associations with Müllerian anomalies. By presenting a detailed case study, we seek to address the diagnostic challenges faced by healthcare professionals and the implications for patient management, emphasizing the importance of early detection and appropriate intervention. We present a case involving a 24-year-old Iranian female who presented with a 10-day history of abdominal pain and vomiting. Initial ultrasound imaging revealed moderate hydroureteronephrosis in the right kidney, prompting further investigation. A contrast-enhanced CT scan was performed, which not only confirmed ureteropelvic junction obstruction but also unexpectedly revealed dorsal pancreatic agenesis. In addition to these findings, the patient was diagnosed with uterus didelphys, highlighting a significant developmental anomaly. This case underscores the necessity for increased awareness of ADP and its associations with Müllerian anomalies among healthcare providers. Early and accurate diagnosis through advanced imaging modalities significantly improves patient outcomes and facilitates better management strategies for associated complications.

[1] Grey CA, Desai A, Nowicki MJ, Bhesania N. Agenesis of the Dorsal Pancreas: Case Report and Review of Age-Related
Differences in Presentation. JPGN Rep. 2023;4(3):e337.https://doi.org/10.1097/PG9.0000000000000337
[2] Bortoletto P, Romanski PA, Pfeifer SM. Müllerian Anomalies: Presentation, Diagnosis, and Counseling. Obstet
Gynecol. 2024;143(3):369-77. https://doi.org/10.1097/AOG.0000000000005469
[3] Heiberg K. Ein Fall von fehlender Cauda pancreatis(bei einem Diabetiker). Zbl Allg Pathol. 1911;22:676-7.
[4] Adda G, Hannoun L, Loygue J. Development of the human pancreas: variations and pathology. A tentative classification. Anat Clin. 1984;5(4):275-83. https://doi.org/10.1007/ BF01798752
[5] Schnedl WJ, Reisinger EC, Schreiber F, Pieber TR, Lipp RW, Krejs GJ. Complete and partial agenesis of the dorsal pancreas within one family. Gastrointest Endosc. 1995;42(5):485-7. https://doi.org/10.1016/S0016-5107(95)70055-2
[6] Lång K, Lasson Å, Müller MF, Thorlacius H, Toth E, Olsson R. Dorsal agenesis of the pancreas-a rare cause of abdominal pain and insulin-dependent diabetes. Acta Radiol. 2012;53(1):2-4. https://doi.org/10.1258/ar.2011.110480
[7] Herman TE, Siegel MJ. Polysplenia syndrome with congenital short pancreas. AJR Am J Roentgenol. 1991;156(4):799-800. https://doi.org/10.2214/ajr.156.4.2003448
[8] Lal A, Pavunesan SK, Mahalingam H, Garg M, Sinha SK, Khandelwal N. A triad of complete dorsal pancreatic agenesis, pancake kidney and bicornuate uterus. An association or an incidental finding: first case in literature. JOP. 2015;16(2):189- 91.
[9] Bhandari A, Mandal S. A case of dorsal agenesis of pancreas associated with unilateral renal agenesis, unicornuate uterus, and ovarian ectopia: A brief review and learning points. Indian J Radiol Imaging. 2020;30(3):395-9. https://doi.org/10.4103/ ijri.IJRI_165_19Goodarzi A and Ostovar L. Dorsal Pancreatic Agenesis and Uterus Didelphys. CRCP. 2024; 9(2):82-87 87 March/April 2024, Volume 9, Issue 2
[10] Marcal L, Nothaft MA, Coelho F, Volpato R, Iyer R. Mullerian duct anomalies: MR imaging. Abdom Imaging. 2011;36:756- 64. https://doi.org/10.1007/s00261-010-9681-x
[11] Alonso Pacheco L, Carugno J, Nieto Pascual L. Cervical and uterine congenital anomalies. In: Laganà AS, Guglielmino A, editors. Management of Infertility. Academic Press; 2023. p. 79-96. https://doi.org/10.1016/B978-0-323-89907-9.00040-5
[12] Body-Bechou D, Loget P, d’Herve D, Le Fiblec B, Grebille AG, Le Guern H, 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. https://doi.org/10.1002/pd.4264
Files
IssueVol 9 No 2 (2024): March-April QRcode
SectionCase Report(s)
DOI https://doi.org/10.18502/crcp.v9i2.16930
Keywords
Mullerian anomalies Agenesis of Pancreas Renal anomalies

Rights and permissions
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
How to Cite
1.
Goudarzi A, Ostovar L. A Case Report of Dorsal Pancreatic Agenesis and Uterus Didelphys in a Young Woman: A Seldomreported Association. CRCP. 2024;9(2):79-84.