title: [Müllerian anomalies. Obstructed hemivagina and ipsilateral renal anomaly syndrome (OHVIRA)].
abstract: 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.
authors.affiliation: Práctica privada, Cuernavaca, Morelos, México.
authors.forename: Cyrus Dean Mario
authors.initials: CD
authors.lastname: Afrashtehfar
authors.affiliation: Departamento de Ginocología y Obstetricia, Hospital General de Zona 5, IMSS, Zacatepec, Morelos, Mexico.
authors.forename: Adrián
authors.initials: A
authors.lastname: Piña-García
authors.affiliation: Division of Biomedical Research, McGill University, Montreal, Quebec, Canada. kelvin.afrashtehfar@mail.mcgill.ca.
authors.forename: Kelvin Ian
authors.initials: KI
authors.lastname: Afrashtehfar
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000196:embryology / Q000453:epidemiology / Q000503:physiopathology / Q000601:surgery; D000293:Adolescent; D003952:Diagnostic Imaging; D042241:Early Diagnosis; D004715:Endometriosis / Q000209:etiology; D005260:Female; D006399:Hematocolpos / Q000209:etiology; D006801:Humans; D007247:Infertility, Female / Q000209:etiology; D007668:Kidney / Q000002:abnormalities* / Q000196:embryology; D009095:Mullerian Ducts / Q000002:abnormalities* / Q000196:embryology / Q000473:pathology; D011247:Pregnancy; D011379:Prognosis; D013577:Syndrome; D014599:Uterus / Q000002:abnormalities* / Q000196:embryology; D014621:Vagina / Q000002:abnormalities* / Q000196:embryology; D014928:Wolffian Ducts / Q000196:embryology
keywords: Herlyn-Werner-Wünderlich syndrome; Müllerian duct anomalies; OHVIRA syndrome; malformaciones müllerianas; obstructed hemivagina and ipsilateral renal anomaly syndrome; síndrome de hemivagina obstruida y anomalía renal ipsilatera; uterus didelphys; útero didelfo