Symptomatic prepubertal obstructed hemivagina and ipsilateral renal anomaly (OHVIRA)-spectrum anomalies: two case reports with individualized management and no recurrence during follow-up.

BACKGROUND: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a congenital anomaly characterized by uterus didelphys, obstructed hemivagina, and an ipsilateral renal anomaly, most commonly renal agenesis. Most patients become symptomatic after menarche and require surgical treatment. In prepubertal girls, asymptomatic fluid collections are often managed conservatively, whereas symptomatic cases have often prompted concern for ectopic ureter-related urinary accumulation requiring intervention. We report two early-childhood cases to show that integrated assessment of imaging, fluid analysis, and follow-up findings can help clarify the cause of symptomatic fluid collection and thereby guide management.

CASE DESCRIPTION: Two early-childhood girls with left renal agenesis presented with abnormal genital findings, including a protruding vaginal mass in one case and persistent vaginal discharge in the other. In the first case, imaging revealed a vaginal/paravaginal cystic lesion without definite uterine anomaly, suggesting an OHVIRA-like developmental anomaly rather than classic OHVIRA. Vaginoscopic aspiration was performed; symptoms resolved, and no recurrence was observed during follow-up. In the second case, imaging demonstrated uterine didelphys, obstructed hemivagina, and fluid distension of the left uterine cavity, cervix, and vagina, consistent with OHVIRA. The patient underwent vaginoscopy-guided partial septectomy with no recurrence during follow-up, and fluid analysis supported infected retained secretions rather than ectopic ureter-related urinary accumulation as the likely cause of the early presentation.

CONCLUSIONS: Symptomatic prepubertal OHVIRA is uncommon and may present with abnormal vaginal discharge. Such cases are not always caused by ectopic ureter-related urinary accumulation; infection may also underlie retained fluid. Integrated assessment can help clarify fluid origin and support individualized management.