The obstructed hemivagina, ipsilateral renal anomaly, uterus didelphys triad

Abstract

The triad of obstructed hemivagina, renal anomaly and uterus didelphys is an uncommon cause of abdominal pain and menstrual discomfort in adolescent girls. Accurate diagnosis and surgical treatment can be delayed for several months or even years. Adolescent girls presenting with these symptoms should have a baseline pelvic ultrasound scan to establish uterine anatomy. In those with ultrasound findings of Müllerian anomalies appropriate follow up would include a magnetic resonance imaging, preferably at a tertiary centre with expertise in interpretation of Müllerian anatomy, as well as early consultation and referral to a centre with experience in the management of these rare conditions. Appropriate surgery would be a single stage procedure to either excise or completely divide the obstructing septum. There is currently no consensus on concurrent laparoscopy. We present a case series of four patients with the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis managed between 2005-2009 at a tertiary centre for paediatric and adolescent gynaecology.

A Rare Obstetric Outcome in Uterus Didelphys with Obstructed Hemivagina and Ipsilateral Renal Agenesis

“…Since the mid1980s, the triad has been called ''Herlyn-Werner-Wunderlich syndrome'' in several articles (first in articles in Bulgarian and then, since 2006, in articles in English). 9 Because many authors disagree with the triad being named, this particular association of congenital anomalies is also referred to as the ''no eponym'' syndrome. 11 The acronym OHVIRA has been used to describe patients with an obstructed hemivagina and ipsilateral renal anomaly, as suggested in 2007 by Smith and Laufer.…”

“…4,[6][7][8] The literature review described few women who had pregnancies in the previously obstructed uterus. [4][5][6][7][8][9]10 An unusual case in which pregnancy occurred alternatively in both hemiuteri is presented here. Moreover, the last pregnancy (contralateral to the affected side) was simultaneous with a hematocolpos and a hematometra because of vaginal stenosis consequent to an incomplete vaginal septum resection.…”

Uterine didelphys with obstructed hemivagina and ipsilateral renal anomaly - OHVIRA syndrome: a rare congenital anomaly

“…The acronym OHVIRA is used to describe the triad of obstructed hemi vagina and ipsilateral renal anomaly and any other uterine anomaly other than uterine didelphys. 3 Both the syndromes are rare and are discussed as same entities in many case reports.…”

Sonographic diagnosis of Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: a report of two cases

“…The incidence of uterine didelphys related to this syndrome is approximately 1/2,000 to 1/28,000, and it is accompanied by unilateral renal agenesis in 43% of cases 4 . It has been estimated that there would be only four to five cases of uterine didelphys and obstructed hemivagina per year in Australia 5 . Early diagnosis and surgical intervention in OHVIRA syndrome is important to relieve symptoms, prevent complications and preserve future fertility 6 .…”