Acute urinary retention caused by OVHIRA syndrome.

Obstructed hemivagina and ipsilateral renal anomaly (OVHIRA) syndrome is a congenital urogenital malformation that associates a bifid uterus with a longitudinal vaginal septum, resulting in a blind hemivagina and an ipsilateral renal agenesis. The clinical presentation is highly variable, delaying diagnosis and leading to important complications. An 18-year-old woman was diagnosed with OVHIRA syndrome following acute urinary retention. An agenesis in the left-sided renal system and an enormous pelvic mass compressing the adjacent structures were revealed in the intravenous urography. Nuclear magnetic resonance was the imaging modality that provided most diagnostic information, defining the pelvic mass as a giant left hematometrocolpos contained with a longitudinal vaginal septum. Resection of the septum was performed draining all reduced hematic content and both hemiuteri communicated with a single vagina, resulting in an asymptomatic patient. OVHIRA syndrome is a little known entity that can occur atypically, which makes the diagnosis difficult and delays the treatment. It is important to maintain a high degree of clinical suspicion to avoid irreversible complications.