RESUMEN

El síndrome OHVIRA o de Herlyn Werner Wünderlich es una malformación congénita rara, causada por una alteración en los conductos de Müller y mesonéfricos que se caracteriza por la triada: útero didelfo, hemivagina obstruida y agenesia renal ipsilateral, aún de etiología incierta. Las pacientes suelen permanecer asintomáticas hasta la menarquia, cuando la presentación clínica más común es el dolor pélvico, un crecimiento tumoral vaginal o abdominal, periodos menstruales normales, infertilidad y descarga vaginal excepcional. Se comunica el caso clínico de una paciente de 15 años de edad, virgen, con descarga vaginal fétida crónica que inicialmente se diagnosticó y trató como enfermedad pélvica inflamatoria; sin embargo, por tratarse de una afección rara y por los antecedentes de la paciente, se realizaron estudios de imagen complementarios, donde el ultrasonido pélvico reportó piocolpos y ausencia del riñón izquierdo, útero didelfo y hemivagina obstruida. Con los datos previos y el resto de los estudios de gabinete se estableció el diagnóstico de síndrome de OHVIRA o de Herlyn Werner Wünderlich.

Palabras clave: útero didelfo, hemivagina, agenesia renal, piocolpos.

ABSTRACT 

OHVIRA (Obstructed hemivagina and ipsilateral renal anomaly) by acronym and abbreviations in English or Herlyn Werner Wunderlich syndrome is a rare congenital malformation caused by an alteration in the Mullerian ducts and Wolffian Ducts. Which is characterized by a triad: uterus didelphys, obstructed and ipsilateral renal agenesis hemivagina still uncertain etiology. Patients are usually asymptomatic until menarche where the most common clinical presentation is pelvic pain, followed by a vaginal or abdominal mass, normal menstrual periods, infertility, and vaginal discharge rarely appears. The case of a female patient of 15 years, nubile with chronic fetid vaginal discharge, initially diagnosed and treated as pelvic inflammatory disease occurs, however because it is an exceptional condition with the background of the patient, by complementary studies were conducted where pelvic ultrasound revealed pyocolpos and absence of left kidney, uterus didelphys, blind hemivagina by other imaging studies, where we could integrate Herlyn-Werner-Wunderlich syndrome. 

In conclusion, abnormalities in the development of the Müllerian ducts are difficult to diagnose early, so you must have the embryological knowledge, conduct thorough clinical assessment and detailed picture in whom the condition is suspected to identify malformations coexisting urinary tract and vaginal defects with the importance of preserving reproductive success through appropriate planning of surgical approach, given that the fertility rate in these patients is comparable to the average.

Key words: Uterus didelphys, hemivagina, renal agenesis, pyocolpos.