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

Journal of Pediatric Urology
Bruce SchlomerLaurence Baskin

Abstract

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. 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. 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 resectio...Continue Reading

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Citations

Feb 24, 2016·Urology·Amanda Saltzman, Christopher Roth
Jul 2, 2016·Journal of Pediatric Urology·K D SusonJ P Gearhart
Aug 9, 2019·Journal of Obstetrics and Gynaecology of India·Alka KriplaniIsha Kriplani
May 11, 2020·Urology·Charlotte Q WuEdwin A Smith
Aug 14, 2021·Urology Case Reports·Hussam NassarRita Gobet
Oct 2, 2021·Radiographics : a Review Publication of the Radiological Society of North America, Inc·Mark D SugiHailey H Choi

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