[Müllerian anomalies. Obstructed hemivagina and ipsilateral renal anomaly syndrome (OHVIRA)]. / Malformaciones müllerianas. Síndrome de hemivagina obstruida y anomalía renal ipsilateral (OHVIRA).

Müllerian duct anomalies are a group of uncommon and underdiagnosed entities, which cause specific symptoms in adolescent females and may be associated with infertility as well as adverse pregnancy outcomes. These malformations occur as a result of an arrest or abnormal development of the Müllerian ducts in different stages of the female reproductive tract during gestation. Obstructed hemivagina and ipsilateral renal anomaly syndrome (OHVIRA), formerly known as the Herlyn-Werner-Wunderlich syndrome, is a rare entity characterized by the presence of a uterus didelphys with an obstructed hemivagina cause by a vaginal septum and the association of a renal anomaly (most commonly renal agenesis) ipsilateral to the obstruction. This syndrome may remain undiagnosed during childhood and usually becomes symptomatic after menarche, causing obstructive symptoms. Occasionally it may be identified after the evaluation of a patient with infertility or recurrent pregnancy loss. The clinical diagnosis is very challenging and requires imaging studies in which ultrasound and MRI play an essential role in the diagnosis, classification and treatment plan. Opportune diagnosis and treatment achieve complete improvement of symptoms, adequate reproductive prognosis and avoid major complications such as endometriosis, pelvic adhesions and infertility. The purpose of this review is to demonstrate the pathophysiology, clinical manifestations, diagnostic methods and treatment of the obstructed hemivagina and ipsilateral renal anomaly syndrome.

Las malformaciones de los conductos de Müller son un grupo de entidades raras y poco diagnosticadas que ocasionan síntomas inespecíficos en adolescentes y pueden acompañarse de problemas de infertilidad y RESULTADOS obstétricos adversos. Estas malformaciones ocurren durante la gestación como consecuencia del desarrollo anormal de los conductos de Müller en diferentes etapas del proceso de formación del aparato reproductor femenino. El síndrome de hemivagina obstruida y anomalía renal ipsilateral, antes conocido como el síndrome de Herlyn-Werner-Wünderlich, es un padecimiento poco común, caracterizado por útero didelfo con una hemivagina obstruida por un tabique vaginal, y la asociación de una anomalía renal (agenesia renal principalmente) ipsilateral a la obstrucción. Este síndrome rara vez se identifica durante la niñez y se vuelve sintomático posterior a la menarquia, ocasionado por los síntomas obstructivos. A veces se identifica posterior a la evaluación de una paciente con problemas de infertilidad o pérdidas gestacionales recurrentes. El diagnóstico clínico es difícil, por eso se requieren estudios de imagen en los que el ultrasonido y la resonancia magnética desempeñan un papel decisivo para el diagnóstico, clasificación y plan terapéutico. El diagnóstico y tratamiento oportunos logran la desaparición de los síntomas, con pronóstico reproductivo adecuado, y se evitan las principales complicaciones: endometriosis, adherencias pélvicas e infertilidad. OBJETIVO: mostrar la fisiopatología, las manifestaciones clínicas, los métodos diagnósticos y terapéuticos del síndrome de hemivagina obstruida y anomalía renal ipsilateral.

[Uterus Didelphys, obstructed hemivagina and ipsilateral renal agenesis as a presentation of a case of the Herlyn-Wemer-Wünderlich syndrome. Literature review]. / Utero didelfo, hemivagina obstruida y agenesia renal ipsilateral como presentación de un caso de síndrome de Herlyn-Werner-Wünderlich. Revisión de la bibliografía.

UNLABELLED: The Herlyn-Werner-Wünderlich syndrome is a rare congenital anomaly of the Müllerian ducts. Müllerian malformations result from defective fusion of the Müllerian ducts during development of the female reproductive system. These malformations are associated with an increased probability of adverse obstetric events. The syndrome is characterized by uterus didelphys with obstructed hemivagina, and ipsilateral renal agenesis. Patients with this syndrome are asymptomatic until menarche, and can follow other complications. CASE REPORT: A 12 year-old female patient presented with a clinical picture of acute abdomen and a previous history of cyclical dysmenorrhea. A diagnosis of uterus didelphys associated with right hematometracolpos was made following an exploratory laparotomy. Resection of the right obstructed hemivagina was subsequently performed using a vaginal approach. A subsequent computed tomography seen identified renal agenesis ipsilateral to the obstructed hemivagina. IN CONCLUSION: The Herlyn-Werner-Wünderlich syndrome is a rare Müllerian anomaly. The characteristics of the syndrome may result in missed diagnosis until detailed evaluation is carried out. We therefore advise clinicians to look for Müllerian duct anomalies whenever a renal malformation is discovered in a fetus or girl postnatal. Early diagnosis of the syndrome and its associated anomalies are essential to provide adequate therapy, and to reduce reproductive complications through radical treatments. Conservative treatment is effective and has a good prognosis for the patient's reproductive function.