Double uterus-hemivagina-renal agenesis syndrome

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Synonyms: Double uterus and obstructed hemivagina syndrome | Herlyn-Werner syndrome | OHVIRA syndrome | Obstructed hemivagina and ipsilateral renal anomaly | Wunderlich syndrome

A rare congenital urogenital anomaly characterized by the presence of double uterus (didelphys bicornuate or septum-complete or partial) unilateral cervico-vaginal obstruction (obstructed hemivagina-communicant not communicant or septate and unilateral cervical atresia) and ipsilateral renal anomalies (renal agenesis and/or other urinary tract anomalies). Patients are usually diagnosed at puberty after menarche due to recurrent severe dysmenorrhea chronic pelvic pain excessive foul smelling mucopurulent discharge spotting and intermenstrual bleeding (depending on the existence of uterine or vaginal communications). Fever dyspareunia and a palpable abdominal pelvic or vaginal mass (mucocolpos or pyocolpos) may also be present.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version July 2024

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