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

Abstract

Objective: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare syndrome characterized by mullerian and renal anomalies. Renal agenesis is classically considered to be part of the definition of OHVIRA syndrome. There are increasing reports that the lack of an ipsilateral kidney on imaging studies is not from true renal agenesis but renal dysplasia and atrophy. We have observed cases where this ipsilateral dysplastic and atrophic kidney leads to clinical symptoms. The objective of this study is to report cases of OHVIRA syndrome where an ipsilateral dysplastic and atrophic kidney led to clinical symptoms, and to raise awareness in the pediatric urology community of this variant of OHVIRA syndrome.

Material and methods: All cases of OHVIRA syndrome with an ipsilateral dysplastic and atrophic kidney were identified from January 2010 to June 2013. The patient's presentation, clinical course, surgical management, radiologic findings, and pathologic finding were reviewed.

Results: There were three cases of OHVIRA syndrome with a symptomatic ipsilateral dysplastic and atrophic kidney identified. All three of these dysplastic and atrophic kidneys had an ectopic ureter to the obstructed hemivagina and led to persistent vaginal drainage after resection of the vaginal septum. These dysplastic and atrophic kidneys were not visualized on any imaging studies. Laparoscopic removal of the dysplastic and atrophic kidney led to cessation of vaginal drainage in all cases.

Conclusion: 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.

Keywords: Duplicated vagina; Ectopic ureter; Mullerian; Multicystic dysplastic kidney; Obstructed hemivagina; Solitary kidney.