Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: institutional case series and a systematic literature review

Uterus didelphys with an obstructed unilateral vagina by a transverse vaginal septum associated with ipsilateral renal agenesis, duplication of inferior vena cava, high-riding aortic bifurcation, and intestinal malrotation: a case report

“…This condition could be due to embryologic arrest at the 8th gestational week that simultaneously affects the m€ ullerian and metanephric ducts (7). Usually, on examination, a unilateral pelvic mass is detected, with the right side affected nearly twice as frequently as the left (16). Our case showed the right-sided involvement as well.…”

The Obstructed Hemivagina, Ipsilateral Renal Anomaly, and Uterine Didelphys Triad and the Subsequent Manifestation of Cervical Aplasia

“…7 The etiology is unclear, but right-sided anomalies are known to occur more frequently than left, and in accordance with literature estimates of 65%, 7 of our 10 cases had an affected right side. 8 The finding of postoperative vaginal constriction in 1 patient in our case series would appear to support simple, complete division of the obstructed hemivaginal septum as the more appropriate approach over complete excision; however, this issue can not be clarified in a small case series. Vaginal stenosis has been described as an uncommon postoperative complication in a larger case series, although no clear reason was identified by the authors as all patients underwent the same procedure.…”

Uterus Didelphys with Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA Syndrome)

“…In our study, ten patients had a vaginal septum obstructing the left hemivagina and nine subjects had obstructed right hemivagina, suggesting that the syndrome does not preferentially involve one of the sides of the body. A study carried out more than 30 years ago (Rock & Jones, 1980) noted a more common occurrence of obstructed hemivagina and renal agenesis on the right side, as also observed more recently by other authors (Vercellini et al, 2007). Although this is not a common anomaly, prognosis is excellent when patients are diagnosed early on and adequately managed.…”

“…The exact etiology of this malformation is not entirely known, but the simultaneous occurrence of malformations in the Mullerian and Wolffian ducts has been considered. The association between uterovaginal and renal malformations is frequent as the Wolffian ducts play a role in the formation of the kidneys and the internal genitalia (Gholoum et al, 2006;Zurawin et al, 2004;Vercellini et al, 2007;Coskun et al, 2008;Acien et al, 2009;Prada Arias et al, 2005) superior portion of the vagina and the uterus. Uterus didelphys occurs due to a failure in the fusion between both Mullerian ducts resulting in the duplication of the uterine body, the canal, and also possibly of the vagina.…”