Blind ectopic hemato-megaureter: A rare association with the rare obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare complex of structural abnormalities of the female urogenital tract characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal anomaly. In this article, we report a case of an 11-year-old girl who presented with pain in lower abdomen since the onset of menarche 8 months back. Magnetic resonance imaging revealed uterus didelphys, hematometrocolpos, hematosalpinx, obstructed hemivagina, and left renal agenesis. There was an associated dilated, tortuous tubular structure with hemorrhagic contents in the midline in the pouch of douglas, with a proximal blind end in the left paravertebral region and distal ectopic insertion in the cervix (hematocolpos) suggestive of blind ectopic hemato-megaureter. To the best of our knowledge, there are very few reported cases of associated blind hemato-megaureter with ectopic insertion into the cervix in a case of OHVIRA syndrome. Imaging findings were confirmed on diagnostic laparoscopy with vaginoscopy and cystoscopy.