agxАндрология и генитальная хирургияAndrology and Genital Surgery2070-97812412-890210.17650/2070-9781-2020-21-4-60-67agx-459Research ArticleОРИГИНАЛЬНАЯ СТАТЬЯORIGINAL REPORTСиндром Herlyn–Werner–Wunderlich в препубертатном периоде (обзор литературы и клинические наблюдения)Herlyn–Werner–Wunderlich syndrome in the prepubescent period (literature review and clinical observations)https://orcid.org/0000-0003-0083-3689АлиеваК. Х.AlievaK. Kh.

Кяниз Ханкишиевна Алиева

197341 Санкт-Петербург, ул. Аккуратова, 2

alieva_kkh@almazovcentre.ruhttps://orcid.org/0000-0002-0265-9728КохреидзеН. А.KokhreidzeN. A.СухоцкаяА. А.SukhotskayaA. A.https://orcid.org/0000-0002-8446-830XБаировВ. Г.BairovV. G.https://orcid.org/0000-0003-4396-4486СкрипникА. Ю.SkripnikA. Yu.ФГБУ «Национальный медицинский исследовательский центр им. В. А. Алмазова» Минздрава РоссииРоссияV. A. Almazov National Medical Research Center, Ministry of Health of RussiaRussian Federation2020120220212146067Copyright © Алиева К.Х., Кохреидзе Н.А., Сухоцкая А.А., Баиров В.Г., Скрипник А.Ю., 20212021Алиева К.Х., Кохреидзе Н.А., Сухоцкая А.А., Баиров В.Г., Скрипник А.Ю.Alieva K.K., Kokhreidze N.A., Sukhotskaya A.A., Bairov V.G., Skripnik A.Y.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://agx.elpub.ru/jour/article/view/459

Синдром Herlyn–Werner–Wunderlich (синдром OHVIRA) – сочетанный порок развития мочеполовой системы, характеризу ющийся различными комбинациями удвоения матки и влагалища с формированием замкнутой вагины с одной стороны и дисгенезией ипсилатеральной почки и мочеточника. Причинами ошибок диагностики и лечения являются относительная редкость порока, недостаточная информированность практических врачей о синдроме, отсутствие мультидисциплинарного подхода. Несвоевременная и тактически хаотичная диагностика синдрома Herlyn–Werner–Wunderlich приводит к неверному представлению о клинической ситуации, ошибочному выбору объема лечения и, как следствие, к таким осложнениям, как стриктуры, распространенный спаечный и воспалительный процесс, а также к необратимым изменениям топографии органов малого таза с последующим ухудшением репродуктивного потенциала пациентки. В данной статье представлен обзор литературы по проблеме, рассмотрены клинические случаи диагностики данного порока у пациенток в препубертатном периоде.

Herlyn–Werner–Wunderlich syndrome (OHVIRA syndrome) is a combined malformation of the genitourinary systeme, characterized by various combinations of uterus dydelphys with unilateral obstructed (or blind) hemivagina and ipsilateral renal agenesis. The causes of mistakes in diagnosis and treatment are common because of relative rarity of anomaly, insufficient awareness of practitioners about the syndrome and the lack of multidisciplinary approach. Untimely and tactically chaotic diagnosis of Herlyn–Werner–Wunderlich syndrome leads to a misconception about the clinical situation, wrong choice in treatment, and, as a consequence, to complications such as strictures, widespread adhesions and inflammation, as well as irreversible changes in the topography of organs of the small pelvis with a subsequent deterioration in reproductive status of patient. This article provides a review of the literature on the problem, considers the clinical cases of diagnosing this defect in prepubertal patients.

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The authors declare that there are no conflicts of interest present.