Abstract / Summary
Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare congenital urogenital anomaly with variable Müllerian and renal manifestations.While the classic presentation includes postmenarchal pelvic pain and hematocolpos, incompletely obstructed forms may remain asymptomatic and be detected incidentally.We report the case of a 16-year-old girl with regular menstrual cycles and no prior gynecological symptoms who presented with acute urinary retention in the setting of a urinary tract infection.Ultrasonography showed non-visualization of the left kidney, a small cystic structure in the left renal fossa, and two uterine cavities.Computed tomography confirmed apparent left renal agenesis with compensatory hypertrophy of the right kidney and a probable involuted dysplastic renal remnant.Pelvic magnetic resonance imaging demonstrated a bicornuate bicollis uterus and a single non-distended vaginal canal, without hematocolpos, hematometra, or hematosalpinx.The overall findings were considered compatible with an atypical incompletely obstructed OHVIRA variant, although a small hemivaginal communication was not directly visualized.This case highlights the broad anatomical and clinical spectrum of OHVIRA syndrome and emphasizes the importance of evaluating the genital tract in adolescent girls with unilateral renal anomalies, even in the absence of typical gynecological symptoms.