Clinical case of OHVIRA syndrome in emergency gynecological practice: features of differential diagnosis
L. V. Adamyan,
E. V. Sibirskaya,
S. A. Korotkova,
G. M. Turgunova,
E. G. Moksyakova,
G. T. Osipova,
A. Yu. Kurmangaleeva,
E. Yu. Dorofeeva https://doi.org/10.17749/2313-7347/ob.gyn.rep.2026.790
Abstract
OHVIRA syndrome (Herlyn-Werner-Wunderlich syndrome) is a combination of unilateral renal agenesis and uterus didelphys with obstructed hemivagina, with populational prevalence comprising 0.1–3.8 %. In emergency gynecological practice, it typically manifests as spontaneous drainage of hematocolpos requiring conducting careful differential diagnosis with abnormal uterine bleeding. The optimal diagnostic methods include gynecological examination, magnetic resonance imaging, 3D ultrasonography, and endoscopic studies.
About the Authors
L. V. Adamyan
Russian University of Medicine, Ministry of Health of the Russian Federation
Russian Federation
Russian Federation
Leyla V. Adamyan, MD, Dr Med Sci, Prof, Academician of RAS
Scopus Author ID: 7006372422
WоS ResearcherID: T-4156-2017
eLibrary SPIN-code: 9836-271
4 Dolgorukovskaya Str., Moscow 127006
E. V. Sibirskaya
Russian University of Medicine, Ministry of Health of the Russian Federation; Pirogov Russian National Research Medical University, Ministry of Health of the Russian Federation; Morozov Children's City Clinical Hospital, Moscow Department of Health; Russian Children’s Clinical Hospital – Branch of Pirogov Russian National Research Medical University, Ministry of Health of the Russian Federation
Russian Federation
Russian Federation
Elena V. Sibirskaya, MD, Dr Sci Med, Prof.
Scopus Author ID: 57193231362
WоS ResearcherID: KFS-7201-2024
eLibrary SPIN-code: 1356-9252
4 Dolgorukovskaya Str., Moscow 127006
1 Ostrovityanova Str., Moscow, 117513
1/9 4th Dobryninsky Lane, Moscow 119049
117 bldg. 1, Leninsky Prospekt, Moscow 119571
S. A. Korotkova
Morozov Children's City Clinical Hospital, Moscow Department of Health
Russian Federation
Russian Federation
Svetlana A. Korotkova, MD
1/9 4th Dobryninsky Lane, Moscow 119049
G. M. Turgunova
Morozov Children's City Clinical Hospital, Moscow Department of Health
Russian Federation
Russian Federation
Gulnoza M. Turgunova, MD, PhD
Scopus Author ID: 57218556481
eLibrary SPIN-code: 1433-2880
1/9 4th Dobryninsky Lane, Moscow 119049
E. G. Moksyakova
Morozov Children's City Clinical Hospital, Moscow Department of Health
Russian Federation
Russian Federation
Ekaterina G. Moksyakova, MD
1/9 4th Dobryninsky Lane, Moscow 119049
G. T. Osipova
Morozov Children's City Clinical Hospital, Moscow Department of Health
Russian Federation
Russian Federation
Guzel T. Osipova, MD, PhD
Scopus Author ID: 57210874000
eLibrary SPIN-code: 8980-0414
1/9 4th Dobryninsky Lane, Moscow 119049
A. Yu. Kurmangaleeva
Morozov Children's City Clinical Hospital, Moscow Department of Health
Russian Federation
Russian Federation
Aliya Yu. Kurmangaleeva, MD
eLibrary SPIN-code: 2739-8291
1/9 4th Dobryninsky Lane, Moscow 119049
E. Yu. Dorofeeva
Sklifosovsky Research Institute for Emergency Medicine, Moscow Healthcare Department
Russian Federation
Russian Federation
Ekaterina Yu. Dorofeeva, MD
3 bldg. 1, Bolshaya Sukharevskaya Square, Moscow 129010
References
1. Adamyan L.V., Sibirskaya E.V., Pivazyan L.G. et al.Developmental anomalies of the female genital tract from classifications to diagnosis: current issue state. RMZh. Mat' i ditya. 2023;6(4):425–32. https://doi.org/10.32364/2618-8430-2023-6-4-16/.
2. Rezai S., Bisram P., Alcantara I.L. et al. Didelphys uterus: A case report and review of the literature. Case Rep Obstet Gynecol. 2015;2015:865821. https://doi.org/10.1155/2015/865821.
3. Che Musa A.A., Husin M.H., Aziz M.E. et al. Undiagnosed uterine didelphys, concomitant right renal agenesis, and left nephrolithiasis in a primigravida with breech pregnancy: A case report. Cureus. 2024;16(7):e65573. https://doi.org/10.7759/cureus.65573.
4. Adamyan L.V., Sibirskaya E.V., Arslanyan K.N. et al. Pathology of the uterus and vagina in girls and young women in violation of the outflow of menstrual blood. Akusherstvo i ginekologiya. Novosti. Mneniya. Obuchenie. 2015;(4):36–40. (In Russ.).
5. Heinonen P.K. Renal tract malformations associated with Müllerian duct anomalies. Clin Obstet Gynecol Reprod Med. 2018;4(1):2–5. https://doi.org/10.15761/COGRM.1000207.
6. Buttram V.C, Gibbons W.E. Müllerian anomalies: a proposed classification. An analysis of 144 cases. Fertil Steril. 1979;32(1):40–6. https://doi.org/10.1016/s0015-0282(16)44114-2.
7. Kobayashi A., Behringer R.R. Developmental genetics of the female reproductive tract in mammals. Nat Rev Genet. 2003;4(12):969–80. https://doi.org/10.1038/nrg1225.
8. Sharkov S.M., Sibirskaya E.V., Tarbaya N.O., Shchukina N.D. Organization of measures for the prevention and early diagnosis of gynecological diseases in children and adolescents. Rossijskij pediatricheskij zhurnal. 2019;22(1):38–41. (In Russ.). https://doi.org/10.18821/1560-9561-2019-22-1-38-41.
9. Pfeifer S.M., Attaran M., Goldstein J. et al. ASRM müllerian anomalies classification 2021. Fertil Steril. 2021;116(5):1238–52. https://doi.org/10.1016/j.fertnstert.2021.09.025. Erratum in: Fertil Steril. 2023;119(6):1088. https://doi.org/10.1016/j.fertnstert.2023.04.001.
10. Fox N.S., Roman A.S., Stern E.M. et al. Type of congenital uterine anomaly and adverse pregnancy outcomes. J Matern Fetal Neonatal Med. 2014;27:949–53. https://doi.org/10.3109/14767058.2013.847082.
11. Passos I.M.P.E., Britto R.L. Diagnosis and treatment of müllerian malformations. Taiwan J Obstet Gynecol. 2020;59(2):183–8. https://doi.org/10.1016/j.tjog.2020.01.003.
12. Acién P., Acién M. The presentation and management of complex female genital malformations. Hum Reprod Update. 2016;22:48–69. https://doi.org/10.1093/humupd/dmv048.
13. Ćwiertnia A., Borzyszkowska D., Golara A. et al. The impact of uterus didelphys on fertility and pregnancy. Int J Environ Res Public Health. 2022;19(17):10571. https://doi.org/10.3390/ijerph191710571.
14. Alieva K.Kh., Kokhreidze N.A., Sukhotskaya A.A. et al. Herlyn–Werner–Wunderlich syndrome in the prepubescent period (literature review and clinical observations). Andrologiya i genital'naya hirurgiya. 2020;21(4):60–7. (In Russ.). https://doi.org/10.17650/2070-9781-2020-21-4-60-67.
15. Vercellini P., Daguati R., Somigliana E. et al. Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: institutional case series and a systematic literature review. Fertil Steril. 2007; 87 (Suppl. 4): 719-724.
16. Angotti R., Molinaro F., Bulotta A.L. et al. Herlyn-Werner-Wunderlich syndrome: an “early” onset case report and review of literature. Int J Surg Case Rep. 2015;11:59–63. https://doi.org/10.1016/j.ijscr.2015.04.027.
17. Zhu L., Chen N., Tong J.-L. et al. New classification of Herlyn-Werner-Wunderlich syndrome. Chin Med J (Engl). 2015;128(Suppl 2):222–25. https://doi.org/10.4103/0366-6999.149208.
18. Wdowiarz K., Skrajna A., Reinholz-Jaskólska M. Diagnosis and treatment of Herlyn-Werner-Wunderlich syndrome: a case report. Prz Menopauzalny. 2021;20(1):52–6. https://doi.org/10.5114/pm.2021.104034.
19. Smith N.A., Laufer M.R. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril. 2007;87(4):918–22. https://doi.org/10.1016/j.fertnstert.2006.11.015.
20. Kapczuk K., Friebe Z., Iwaniec K., Kedzia W. Obstructive Müllerian anomalies in menstruating adolescent girls: a report of 22 cases. J Pediatr Adolesc Gynecol. 2018;31(3):252–7. https://doi.org/10.1016/j.jpag.2017.09.013.
21. Kim T.H., Lee H.H., Byun D. Unusual case of OHVIRA syndrome with a single uterus, unrecognized before labor and followed by an intrapartal rupture of obstructed hemivagina. Arch Gynecol Obstet. 2014;290(5):1049–50. https://doi.org/10.1007/s00404-014-3426-y.
22. Adamyan L.V., Bogdanova E.A., Glybina T.M., Sibirskaya E.V. Childhood and adolescence abdominal syndrome due to gynecological pathology. Diagnostic and treatment errors. Akusherstvo i ginekologiya. 2012;(2):96–101. (In Russ.).
Review
For citations:
Adamyan L.V., Sibirskaya E.V., Korotkova S.A., Turgunova G.M., Moksyakova E.G., Osipova G.T., Kurmangaleeva A.Yu., Dorofeeva E.Yu. Clinical case of OHVIRA syndrome in emergency gynecological practice: features of differential diagnosis. Obstetrics, Gynecology and Reproduction. (In Russ.) https://doi.org/10.17749/2313-7347/ob.gyn.rep.2026.790
Views:
67
JATS XML
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Email this article 