Background and Clinical Significance: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare Müllerian duct anomaly that is frequently misdiagnosed as primary dysmenorrhea, resulting in delayed intervention and preventable complications such as hematocolpos, pelvic infection, adnexal damage, and endometriosis. Accurate early diagnosis relies on imaging, yet the complementary roles of ultrasound and magnetic resonance imaging (MRI) remain underemphasized in routine clinical pathways. This study highlights contemporary diagnostic strategies and surgical outcomes to support timely, fertility-preserving management. Case Presentation: We report a retrospective case series of four adolescent patients diagnosed with OHVIRA syndrome between 2020 and 2025. All patients underwent pelvic ultrasound as an initial diagnostic modality, followed by MRI for definitive anatomical characterization. Imaging findings, surgical management, and clinical outcomes are presented and compared with a systematically screened review of recent literature. One case represents a rare prepubertal variant with ectopic ureteral communication. Surgical management primarily involved single-stage hemivaginal septal resection, with individualized approaches based on anatomy and clinical presentation. Conclusions: Ultrasound remains an effective first-line screening tool for suspected obstructive Müllerian anomalies, while MRI is essential for definitive diagnosis, identification of associated urinary tract variants, and surgical planning. Early MRI-guided intervention enables symptom resolution and may reduce long-term reproductive morbidity. Standardized longitudinal follow-up into reproductive age is required to confirm fertility outcomes and optimize evidence-based management of OHVIRA syndrome.
Bakhsh et al. (Wed,) studied this question.
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