Uterovaginal duplication with blind hemivagina and ipsilateral renal agenesis: Review of unusual presentation

Congenital abnormalities of the Mullerian duct system can result in various urogenital anomalies, including uterus didelphys with blind hemivagina and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome). It usually presents after the menarche with progressive pelvic pain during periods secondary to haematocolpos. However, rarely presentation is unusual with consequent diagnostic delay. This paper attempts to review the medical literature for rare presentations that make diagnosis and treatment difficult, including our cases. Clinicians must be aware of the importance of this rare congenital anomaly of the female genital tract, especially many years after the menarche. This condition should be considered to prevent misdiagnosis or suboptimal treatment.