Obstructed hemivagina

Abstract

Obstructed hemivagina in association with a double uterus and renal anomaly has been described as early as 1922. Since then, this anomaly has been described in the literature associated with several different names: double uterus with obstructed hemivagina and ipsilateral renal agenesis, obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, and Herlyn-Werner-Wunderlich syndrome. Diagnosis should be suspected in an adolescent female with escalating dysmenorrhea as menstruation occurs from the patent outflow tract, but hematocolpos, hematometra, and retrograde menstruation occur in the obstructed side leading to pain. Treatment is typically primary resection of the vaginal septum with marsupialization to unite the vaginal cavities and relieve the obstruction. Alternative therapies include hemihysterectomy with hemocolpotomy, and drainage and decompression of the hematocolpos with hormonal suppression of menses. Fertility may be affected by extent of endometriosis and adhesive disease, but infertility is not common. Pregnancies occur more commonly in the non-obstructed horn, but may be present in the horn ipsilateral to obstruction in a significant percentage of cases.