Abstract
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.
Highlights
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
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 of asymptomatic Herlyn–Werner– Wunderlich syndrome
Authors’ contributions K.H. Alieva: inpatient management, article writing; N.A. Kokhreidze: practical and scientific supervising (obstetrics and gynecology), performing gynecological operations; A.A. Sukhotskaya: performing a surgical operation, editing the text of the article; V.G. Bairov: performing a surgical operation, scientific and practical supervising (pediatric surgery); A.Y. Skripnik: conducting diagnostic imaging and data analysis, preparing the illustrations
Summary
Синдром Herlyn–Werner–Wunderlich (синдром OHVIRA) – сочетанный порок развития мочеполовой системы, характеризую щийся различными комбинациями удвоения матки и влагалища с формированием замкнутой вагины с одной стороны и дисгенезией ипсилатеральной почки и мочеточника. 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. Иллюстрирующие те трудности, которые возникают при диагностике синдрома OHVIRA и выборе метода лечения, применении эндоскопического оборудования у девочек младшего возраста.
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