Background: Müllerian duct anomalies are rare congenital malformations resulting from abnormal development, fusion, or resorption of the Müllerian ducts. The coexistence of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis is known as Herlyn–Werner–Wunderlich syndrome (OHVIRA syndrome), a rare developmental anomaly. Case Presentation: A 14-year-old girl presented with acute urinary retention of 8–10 hours duration associated with lower abdominal pain. Physical examination revealed a hemodynamically stable adolescent with normal secondary sexual characteristics and recent attainment of menarche. Ultrasonography demonstrated a solitary enlarged right kidney, left renal agenesis, bicornuate/didelphys uterus, and hematocolpos. Magnetic resonance imaging confirmed a Müllerian duct anomaly characterized by uterine didelphys, duplication of vaginal cavities due to a longitudinal vaginal septum, mild-to-moderate left-sided hematocolpos, bilateral polycystic ovaries, and absence of the left kidney. The patient was referred to the obstetrics and gynaecology department for definitive management and discharged in stable condition. Conclusion: This case highlights the importance of considering Müllerian duct anomalies in adolescent females presenting with pelvic pain and urinary retention following menarche. Early imaging and multidisciplinary management are crucial to prevent complications and preserve reproductive function.
Patidar et al. (Tue,) studied this question.