OHVIRA-syndrome in a newborn

• Main Authors: J. Kueppers (Author), L. Wehrli (Author), S. Zundel (Author), S. Shavit (Author), N. Stahr (Author), P.O. Szavay (Author)
• Format: Book
• Published: Elsevier, 2021-06-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 2213-5766; 10.1016/j.epsc.2021.101859
• Keywords: OHVIRA-Syndrome; Herlyn-Werner-Wunderlich syndrome; Renal agenesis; Müllerian duct anomalies; Pediatrics; RJ1-570; Surgery; RD1-811
• Source: Journal of Pediatric Surgery Case Reports, Vol 69, Iss , Pp 101859- (2021)

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.