Zinner Syndrome: Case Series and Literature Review
https://doi.org/10.20514/2226-6704-2023-13-5-392-400
Abstract
Zinner syndrome is a rare congenital anomaly of the mesonephric duct, characterized by a triad of symptoms: seminal vesicle cysts, ipsilateral renal agenesis and ejaculatory duct obstruction. This leads to a severe complication — oligozoospermia/azospermia, which can subsequently cause infertility. The widespread use of medical imaging increases the probability of incidental detection. Namely, magnetic resonance imaging (MRI) is the imaging modality of choice for making a diagnosis.
Study purpose: to optimize patient routing in Zinner syndrome, as well as to minimize the risk of misdiagnosis or missed pathology, by providing strong and weak points for each modality.
Materials and methods: we present two clinical cases of Zinner syndrome. The first one is a complicated course in a 25-year-old patient, and the second one is accidentally discovered in a 27-year-old patient. The patients underwent a comprehensive diagnostic panel, including: ultrasound (US), computed tomography (CT), magnetic resonance imaging (MRI). The results obtained were analyzed in the light of available literature data.
Results: in most cases, Zinner syndrome is an incidental finding during. The diagnosis based on these imaging methods and the correct patient routing allowed us to make a timely and correct diagnosis, followed by decisions on further treatment tactics.
Conclusion: Zinner syndrome is a rare disease and is often diagnosed based on imaging findings only. A radiologist and clinician need to know about the diagnostic criteria for this syndrome in order to successfully diagnose and determine the optimal treatment tactics.
About the Authors
D. A. KushnerovaRussian Federation
Moscow
V. S. Tikhonova
Russian Federation
Moscow
I. A. Blokhin
Russian Federation
Moscow
A. P. Gonchar
Russian Federation
Anna Pavlovna Gonchar
Moscow
References
1. Sofia F., Vitor O., Diogo R. Zinner syndrome presenting with intermittent scrotal pain in a young man. Radiol Case Rep. 2018 Dec; 13(6): 1224–1227. doi: 10.1016/j.radcr.2018.08.012
2. Amman Y., Hafiz M.F., Mohammed H.S., et al.. Zinner Syndrome Unmasked by Workup for Renal Colic and Uncontrolled Hypertension. Cureus. 2020 May. 12(5): e8381. doi: 10.7759/cureus.8381
3. Moore K.L., Persaud T.V.N., Torchia M.G. The developing human. Clinically oriented embryology. 10th ed., 2016,243-255.
4. Shibani M., Rajeev R., Umesh C.G. Zinner syndrome—a rare developmental anomaly of the mesonephric duct diagnosed on magnetic resonance imaging. Radiol Case Rep. 2016 Dec; 11(4): 313–317. doi: 10.1016/j.radcr.2016.04.002.
5. Ahmed I., Abdelmoughit H., Idriss Z., et al. Zinner’s Syndrome: A Rare Diagnosis of Dysuria Based on Imaging. Case Rep Urol. 2020; 2020: 8826664. doi: 10.1155/2020/8826664
6. Djidda A., Fatima-Ezzahrae B., Mouna S., et al. Zinner Syndrome. European Journal of Case Reports in Internal Medicine. 2021; 8(6): 002628. doi: 10.12890/2021_002628
7. Abakar D., Badi F., Sabiri M., et al. Zinner syndrome. EJCRIM 2021;8: doi: 10.12890/2021_002628.
8. Valerio D.P., Riccardo G., Angelo T., et al. Zinner syndrome: two cases and review of the literature. BMJ 8 June 2021; 14(6): e243002. doi: 10.1136/bcr-2021-243002.
9. Aybike H. et al. Int J Impot. Zinner syndrome and infertility – a literature review based on clinical case. Int J Impot Res. 2021; 33(2): 191–195. doi: 10.1038/s41443-020-00360-0.
10. Demaeyer L., Holz S., Pamart D., et al. Robotic management of painful Zinner syndrome, case report and review of literature. Int. J. Surg. Case Rep. 2020:73:61-64. doi: 10.1016/j.ijscr.2020.06.078.
11. Ostrowska M., Grześk M., Kaczyński S., et al. Zinner Syndrome - A Rare Cause of Recurrent Epididymitis and Infertility. Clin Pract. 2021 Dec 9; 11(4):942-946. doi: 10.3390/clinpract11040108.
12. Cito G., Sforza S., Gemma L., et al. Infertility case presentation in Zinner syndrome: Can a long-lasting seminal tract obstruction cause secretory testicular injury? Andrologia. 2019 Dec; 51(11): e13436. doi: 10.1111/and.13436.
13. Canales-Casco N., Dominguez-Amillo A., Arrabal-Polo M.A., et al. Hematospermia as a Rare Form of Presentation of Zinner Syndrome. Urology. 2017;99: e15–e16. doi: 10.1016/j.urology.2016.09.030
14. Liu T., Li X., Huang L., et al. Zinner syndrome: an updated pooled analysis based on 214 cases from 1999 to 2020: systematic review. Ann Palliat Med. 2021;10: 2271–2282. doi: 10.21037/apm-20-1997.
15. Pinhas M.L., Jules D., Edmond T. Gonzales. Genetic Etiology of Posterior Urethral Valves. The American Urological Association Education and Research, 1983 Oct;130(4):781-4. doi: 10.1016/s0022-5347(17)51460-3.
16. Gabrielle L., Bixia Zheng, Grace U. Ediae, et al. Homozygous WNT9B variants in two families with bilateral renal agenesis/hypoplasia/dysplasia. Am J Med Genet A. 2021 Oct; 185(10): 3005–3011. doi: 10.1002/ajmg.a.62398
17. Gianmartin C., Luca G., Claudia G., et al. Sperm retrieval by conventional testicular sperm extraction for assisted reproduction in patients with Zinner syndrome. Clin Exp Reprod Med. 2021 Mar; 48(1): 85–90. doi: 10.5653/cerm.2020.03769
Review
For citations:
Kushnerova D.A., Tikhonova V.S., Blokhin I.A., Gonchar A.P. Zinner Syndrome: Case Series and Literature Review. The Russian Archives of Internal Medicine. 2023;13(5):392-400. https://doi.org/10.20514/2226-6704-2023-13-5-392-400