Herlyn-Werner-Wunderlich Syndrome /OHVIRA Syndrome; A Rare Urogenital Anomaly with Unusual Presentation in Two Cases with Review of Literature

The OHVIRA syndrome classically occurs in the setting of uterine didelphys or more rarely, a septate uterus with incidence in various case series is 0.1-3.8%. Renal agenesis is the most commonly reported urologic anomaly. The typical patient with this rare condition usually presents after menarche with nonspecific symptoms of recurrent pelvic pain or dysmenorrhea but both of our patients had atypical presentation. Sonography is frequently the initial imaging modality for evaluation of suspected Müllerian duct anomalies, MR imaging is an excellent modality for evaluating the frequently complex Müllerian duct anomalies. Treatment invariably requires surgical intervention in the form of excision of vaginal septum to relieve obstruction. In addition to relief of pain due to obstruction, surgery also reduces chances of pelvic endometriosis due to retrograde menstrual seeding. We are reporting these cases being their atypical presentation in adulthood life.