Available online 21 October 2023

: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome describes a spectrum of Mullerian anomalies characterized by uterine didelphys, unilateral obstructed hemivagina and ipsilateral renal anomalies. We report the case of a neonatal complication secondary to OHVIRA syndrome with long term follow up, adding to the collective understanding of this syndrome.

: We present a 22-day-old female with an acute kidney injury secondary to post-renal obstruction from a large hydrometrocolpos. Multidisciplinary care facilitated timely diagnosis of OHVIRA syndrome and temporizing operative management. The patient was followed serially into her adolescence and ultimately underwent definitive excision of her vaginal septum.

: OHVIRA syndrome encompasses a broad spectrum of anatomical variation with different considerations in pre-pubertal and post-pubertal patients. Multidisciplinary care allows for timely diagnosis and clinical decision making within this complex patient population.

© 2023 Published by Elsevier Inc. on behalf of North American Society for Pediatric and Adolescent Gynecology.