Obstructed hemivagina (OHVIRA syndrome) or uterovaginal septum and ipsilateral renal anomaly?

Batyrova Z.K., Uvarova E.V., Chuprynin V.D., Asaturova A.V., Kumykova Z.Kh., Kruglyak D.A., Gautier M.S.

1) Academician V.I. Kulakov National Medical Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Health of Russia, Moscow, Russia; 2) I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia, Moscow, Russia; 3) National Medical Research Center for Children’s Health, Ministry of Health of Russia, Moscow, Russia
This review analyzes an update on the clinical presentations of female genitourinary malformations (OHVIRA or Herlyn–Werner–Wunderlich syndrome) and new approaches to their diagnosis and treatment.
The review includes data from foreign and Russian articles found in PubMed on the topic under consideration and published in recent years.
The state-of-the-art in the area under study is analyzed. The data on the diagnosis and management of patients with the rare associative state are systematized; further areas that require in-depth study are identified.
Conclusion: The presented studies indicate that there is a need to revise the name of the Herlyn–Werner–Wunderlich syndrome and the acronym OHVIRA in favor of uterovaginal septum and ipsilateral renal anomaly. The important role of and need for the widespread introduction of visual diagnostic methods and minimally invasive surgical treatment technologies in the management of these patients in order to improve the efficiency of medical care are emphasized.

Authors’ contributions: Batyrova Z.K., Gautier M.S. – concept; Batyrova Z.K., Chuprynin V.D., Asaturova A.V., Kruglyak D.A. – material collection and processing; Batyrova Z.K. – writing the text; Uvarova E.V., Kumykova Z.Kh. – editing.
Conflicts of interest: The authors declare that there are no conflicts of interest.
Funding: The investigation has not been sponsored.
For citation: Batyrova Z.K., Uvarova E.V., Chuprynin V.D., Asaturova A.V.,
Kumykova Z.Kh., Kruglyak D.A., Gautier M.S. Obstructed hemivagina (OHVIRA syndrome) or uterovaginal septum and ipsilateral renal anomaly?
Akusherstvo i Ginekologiya/Obstetrics and Gynecology. 2023; 1: 35-40 (in Russian)
https://dx.doi.org/10.18565/aig.2022.250

Keywords

OHVIRA
obstructed hemivagina
uterovaginal septum
ipsilateral renal anomaly
Herlyn–Werner–Wunderlich syndrome
genital malformation

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Received 21.10.2022

Accepted 28.12.2022

About the Authors

Zalina K. Batyrova, PhD, Senior Researcher at the Department of Pediatric and Adolescent Gynecology, Academician V.I. Kulakov NMRC for OG&P,
Ministry of Health of Russia, +7(495)438-85-42, linadoctor@mail.ru, https://orcid.org/0000-0003-4997-6090, 4, Oparina str., 117997, Moscow, Russia.
Elena V. Uvarovа, Corresponding member of the RAS, Dr. Med. Sci., Professor, Head of the Department of Pediatric and Adolescent Gynecology, Academician V.I. Kulakov NMRC for OG&P, Ministry of Health of Russia; President of Interregional Public Organization "Association of Pediatric and Adolescent Gynecologists";
Professor at the Department of Obstetrics, Gynecology, Perinatology and Reproductology, Institute of Professional Education, +7(495)438-85-42, elena-uvarova@yandex.ru,
https://orcid.org/0000-0002-3105-5640, 4, Oparina str., 117997, Moscow, Russia.
Vladimir D. Chuprynin, PhD, Head of the Surgical Department, Academician V.I. Kulakov NMRC for OG&P, Ministry of Health of Russia, +7(495)438-85-42,
v_chuprynin@oparina4.ru, 4, Oparina str., 117997, Moscow, Russia.
Aleksandra V. Asaturova, PhD, Head of the Department of Anatomic Pathology, Academician V.I. Kulakov NMRC for OG&P, Ministry of Health of Russia, +7(495)438-23-11, a.asaturova@gmail.com, https://orcid.org/0000-0001-8739-5209, 4, Oparina str., 117997, Moscow, Russia.
Zaira Kh. Kumykova, PhD, Senior Researcher at the Department of Pediatric and Adolescent Gynecology, Academician V.I. Kulakov NMRC for OG&P,
Ministry of Health of Russia, +7(495)438-85-42, zai-kumykova@yandex.ru, https://orcid.org/0000-0001-7511-1432, 4, Oparina str., 117997, Moscow, Russia.
Diana A. Kruglyak, doctor at the Department of Pediatric and Adolescent Gynecology, Academician V.I. Kulakov NMRC for OG&P, Ministry of Health of Russia,
+7(495)438-85-42, diana.kruglyak@yandex.ru, 4, Oparina str., 117997, Moscow, Russia.
Marina S. Gautier, Pediatrician, Department of Rheumatology, Children's Health Research Center, Ministry of Health of Russia, +7(499)134-03-12,
marina.gautier@gmail.com, 2-1, Lomonosovsky Prospekt, 119296, Moscow, Russia.

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