A rare variant of obstructed hemivagina and ipsilateral renal agenesis and its improvement of classification

Abstract

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) is a rare congenital malformation of the female reproductive organs, including uterine dysplasia, blind hemivagina, and ipsilateral renal dysplasia. It has a serious impact on patients' quality of life and fertility. We report a rare case of OHVIRA with a complete septate uterus, ectopic dysplasia of the left kidney, left ectopic ureter, and three oblique vaginal septa. The dysplastic left kidney and the ectopic ureter were removed during laparoscopy. In addition, the two layers of vaginal septa, which were ignored in the past 10 years, were removed by hysteroscopy. As the detection rate of OHVIRA has gradually increased, manifestations that previous classification systems could not recapitulate have emerged. By retrieving the relevant cases, we have tried to propose a new classification system, which would allow clinicians to have a complete understanding of the manifestations of OHVIRA and would be helpful in the treatment of patients.