OHVIRA-syndrome in a newborn

OHVIRA-syndrome in a newborn

OHVIRA-Syndrome (Obstructed hemivagina with ipsilateral renal anomaly) is a rare congenital malformation. Diagnosis of this malformation is usually made during adolescence. Delayed diagnosis may result in serious complications with potential future obstetric problems. We report on a newborn girl pre...

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Bibliographic Details
Published in:Journal of pediatric surgery case reports Vol. 69; p. 101859
Main Authors: Kueppers, J., Wehrli, L., Zundel, S., Shavit, S., Stahr, N., Szavay, P.O.
Format: Journal Article
Language:English
Published: Elsevier Inc 01-06-2021
Elsevier
Subjects:
Herlyn-Werner-Wunderlich syndrome
Müllerian duct anomalies
OHVIRA-Syndrome
Renal agenesis
OHVIRA-Syndrome
Renal agenesis
Herlyn-Werner-Wunderlich syndrome
Müllerian duct anomalies
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Summary:OHVIRA-Syndrome (Obstructed hemivagina with ipsilateral renal anomaly) is a rare congenital malformation. Diagnosis of this malformation is usually made during adolescence. Delayed diagnosis may result in serious complications with potential future obstetric problems. We report on a newborn girl presenting with an interlabial mass formation, a vaginal introitus on the right lateral aspect and an orthotopic urethral meatus on primary inspection. Ultrasound examination revealed left sided renal agenesis and a hydrocolpos posterior to the bladder. OHVIRA-Syndrome was assumed and a surgical incision of the obstructed left hemivagina for drainage of the hydrocolpos was performed. Further examination confirmed anatomical conditions corresponding to OHVIRA-Syndrome. Aside from routine clinical follow-up and regular ultrasound examination, further interventions were postponed until puberty. In this case report we aim to share our experience of a newborn girl with OHVIRA-Syndrome. We discuss appropriate medical decision-making regarding surgical management of patients with this malformation during each stage of development with a corresponding literature review.
ISSN:2213-5766
2213-5766
DOI:10.1016/j.epsc.2021.101859