J Korean Soc Radiol.  2025 Jan;86(1):129-140. 10.3348/jksr.2024.0018.

Anatomical Variations, Genitourinary Anomalies and Clinical Presentations in Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: Case Series

• Kim HJ
• Lee EJ
• Chang YW
• Hong SS
• Hwang J
• Nam B
• Bae SH

• KMID: 2564835
• DOI: http://doi.org/10.3348/jksr.2024.0018

Abstract

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare Müllerian duct anomaly, commonly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. While these are the three most common genitourinary anomalies in OHVIRA syndrome, a spectrum of urogenital anomalies can be present. Knowledge of this spectrum is crucial for proper patient management and treatment planning. In this case series, we report on five patients with OHVIRA syndrome, each presenting with a urogenital anomaly other than the typical renal agenesis or uterus didelphys. We highlight the gynecological complications encountered, which clinicians and radiologists should be aware of.

Keyword

OHVIRA Syndrome; Urogenital Anomaly; Ectopic Ureter; Classification; Vaginal Cancer