Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome should be redefined as ipsilateral renal anomalies: Cases of symptomatic atrophic and dysplastic kidney with ectopic ureter to obstructed hemivagina

This study reports additional cases to the literature that do not fit the classic definition of OHVIRA syndrome. In the cases in this study, there was no ipsilateral renal agenesis, but an ipsilateral dysplastic and atrophic kidney with an ectopic ureter to the obstructed hemivagina. In addition, these cases demonstrate that the dysplastic and atrophic kidney may cause symptoms such as persistent vaginal drainage after resection of vaginal septum. The dysplastic and atrophic kidneys may not be detected by any imaging modality including MRI and DMSA scan and may be ectopic in location. All three of our cases had persistent vaginal drainage after resection of the vaginal septum which was cured by removal of the dysplastic and atrophic kidney ipsilateral to the obstructed hemivagina. The limitations of this study include the small number of patients and retrospective nature. While the classic definition of OHVIRA syndrome includes ipsilateral renal agenesis, there is increasing evidence that the absence of a kidney on imaging is due to renal dysplasia and atrophy and not true renal agenesis. In addition, we have observed that this dysplastic and atrophic kidney may cause clinically significant issues such as persistent vaginal drainage through an ectopic ureter to the ipsilateral hemivagina after resection of vaginal septum. This small dysplastic and atrophic kidney may not be visualized on imaging studies and laparoscopy can be diagnostic and therapeutic if indicated. Ipsilateral renal anomalies, not ipsilateral renal agenesis, should be considered part of the definition of OHVIRA syndrome. Pediatric urologists need to be aware of the spectrum of renal anomalies in OHVIRA syndrome.