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Possibilities of Early Ultrasound Diagnostics of the Herlin — Werner — Wunderlich syndrome (Case Report)

https://doi.org/10.52560/2713-0118-2021-5-161-170

Abstract

Herlin — Werner — Wunderlich syndrome or OHVIRA (Obstructed hemivagina and ipsilateral renal anomaly) is a rare type of uterine and vaginal doubling defect (0,1 – 10,0 % of all uterine abnormalities) which is characterized by a triad of symptoms: uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal dysgenesis. The manifestation of the disease usually present after menarche, is accompanied by severe pain syndrome, leads to redoubted complications and organ-carrying operations with decreasing the fertility. At an earlier age, it can manifest itself as an infection of the genitourinary system. The diagnosis is based on clinical and anamnestic data using instrumental methods, the most accessible and safe of which is ultrasound. This article presents three clinical cases of patients 5 – 6 years old with a description of the ultrasound picture of the OHVIRA syndrome.

About the Authors

F. Sh. Mamedova
Neonatology and Pediatrics of National Medical Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Healthcare of Russia; Russian edical Academy of Continuing Professional Education, Ministry of Healthcare of Russia, the Department of Radiation Diagnostics of Children
Russian Federation

Mamedova Fatima Shapievna, Ph. D. Med., doctor of the Department of Ultrasound Diagnostics; Аssistant at the Department of Radiation Diagnostics of Children

4, ul. ac. Oparina, 117997, Moscow, Russia

Tel.: +7 (495) 438-85-42



E. A. Filippova
Neonatology and Pediatrics of National Medical Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Healthcare of Russia; Russian edical Academy of Continuing Professional Education, Ministry of Healthcare of Russia, the Department of Radiation Diagnostics of Children
Russian Federation

Filippova Elena Aleksandrovna, Ph. D. Med., Head of the Department of Ultrasound Diagnostics; Associate Professor at the Department of Radiation Diagnostics of Children

4, ul. Ac. Oparina, 117997, Moscow, Russia

Phone number: +7 (495) 438-85-42



Z. K. Batyrova
Neonatology and Pediatrics of National Medical Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Healthcare of Russia
Russian Federation

Batyrova Zalina Kimovna, Ph. D. Med., senior researcher, at the Department of Pediatric and adolescent gynecology

4, ul. Ac. Oparina, 117997, Moscow, Russia

Phone number: +7 (495) 438-85-42



Z. H. Kumykova
Neonatology and Pediatrics of National Medical Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Healthcare of Russia
Russian Federation

Kumykova Zaira Khasanovna, Ph. D. Med., senior researcher, at the Department of Pediatric and adolescent gynecology

4, ul. Ac. Oparina, 117997, Moscow, Russia

Phone number: +7 (495) 438-85-42



References

1. 1 Adamyan L. V., Sibirskaya E. V., Evdokimov A. I., Arslanyan K. N., Zhuravlev S. A., Smal T. A. Pathology of the uterus and vagina in girls and young women in violation of the outflow of menstrual blood. Obstetrics and Gynecology. 2015. No. 4. Р. 36–39 (in Russian).

2. 2 Alieva K. Kh., Kokhreidze N. A., Sukhotskaya A. A. et al. Herlyn-WernerWunderlich syndrome in the prepubescent period (literature review and clinical observations). Аndrology and genital surgery. 2020. No. 4. P. 60–67 (in Russian).

3. 3 Vasilyev A. O., Govorov A. V., Pushkar D. Y. Embryological aspects of congenital anomalies of the kidney and urinary tract (CAKUT). Vestnik Urologii. 2015. No. 2. P. 47–60 (in Russian).

4. 4 Han J. H., Lee Y. S., Im Y. J., Kim S. W., Lee M.-J., Han S. W. Clinical Implications of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome in the Prepubertal Age Group. 2016. PLoS ONE 11(11): e0166776.

5. 5 Tan Y. G., Laksmi N. K., Yap T.-L. et al. Preventing the O in OHVIRA (Obstructed Hemivagina Ipsilateral Renal Agenesis): Early Diagnosis and Management. J. of Pediatric Surgery. 2020. No. 55 (7). P. 1377–1380.


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For citations:


Mamedova F.Sh., Filippova E.A., Batyrova Z.K., Kumykova Z.H. Possibilities of Early Ultrasound Diagnostics of the Herlin — Werner — Wunderlich syndrome (Case Report). Radiology - Practice. 2021;(5):161-170. (In Russ.) https://doi.org/10.52560/2713-0118-2021-5-161-170

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ISSN 2713-0118 (Online)