IMPROVED SURGICAL CORRECTION METHOD FOR UTERINE AND VAGINAL DUPLICATION IN HIGH OBSTRUCTIVE HEMIVAGINA
Keywords:
Anomalies in the development of the genital organs, Herlin-Werner-Wunderlich syndrome, OHVIRA syndrome, malformations of the Müllerian ductsAbstract
Relevance: Congenital anomalies (malformations) of the female reproductive organs are persistent intrauterine deviations from normal variations in size, shape, proportions, symmetry, topography, and organogenesis, leading to functional impairment.
In recent years, there has been a noticeable increase in the detection rate of developmental anomalies of various organs and systems, including genital malformations. This trend may be attributed both to the improvement of diagnostic methods and to the deterioration of environmental conditions.
Herlyn–Werner–Wunderlich syndrome (HWW syndrome, or OHVIRA syndrome) is a congenital malformation of the Müllerian ducts. The syndrome received its name nearly half a century after the initial clinical descriptions by U. Herlyn and H. Werner (1971) and M. Wunderlich (1976). The acronym OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) was introduced in 2007.
The precise population incidence of this anomaly is difficult to determine, but the syndrome accounts for up to 10% of congenital gynecological malformations. Herlyn–Werner–Wunderlich syndrome is a rare congenital anomaly characterized by uterine duplication, unilateral obstructed hemivagina, and ipsilateral renal agenesis. The syndrome presents with a wide variety of anatomical variants, and literature data indicate that vaginal obstruction and renal agenesis are more commonly found on the right side.
The rarity and diversity of the anatomical variants are associated with considerable diagnostic challenges in gynecological practice. The diagnosis of uterine and vaginal anomalies is often difficult due to the wide range of malformation types. As a result of diagnostic errors, unjustified surgical interventions are performed in 22–34% of cases, particularly in patients with uterovaginal malformations accompanied by menstrual outflow obstruction.
Existing reconstructive surgical techniques for high vaginal aplasia are often associated with a risk of injury to adjacent organs, such as the bladder and rectum. These challenges underscore the need for further study of the diverse manifestations of this syndrome to prevent diagnostic errors—an aspect of particular importance for pediatric and adolescent gynecologists.
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