Late-onset OHVIRA syndrome in a 49-year-old woman with severe dysmenorrhea: a case report on vaginoscopic management

TL;DR

A 49-year-old woman with severe menstrual pain was diagnosed with a rare Müllerian duct anomaly and successfully treated with minimally invasive surgery.

Contribution

This case documents one of the latest known presentations of OHVIRA syndrome and confirms the efficacy of minimally invasive surgical management.

Findings

MRI confirmed OHVIRA syndrome in a 49-year-old woman with a didelphic uterus and right renal agenesis.

Symptoms resolved after vaginoscopic and hysteroscopic resection of the vaginal septum.

No recurrence was observed at 6-month follow-up, supporting long-term surgical efficacy.

Abstract

OHVIRA syndrome is a rare Müllerian duct anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Although typically diagnosed in adolescence, it can rarely present later in life. We present the case of a 49-year-old woman who presented with severe dysmenorrhea and intermittent spotting. Imaging studies, particularly MRI, confirmed the diagnosis of OHVIRA syndrome by revealing a didelphic uterus with hematometra and hematocolpos secondary to a right-sided obstructed hemivagina, along with right renal agenesis. The patient was successfully managed with vaginoscopic and hysteroscopic resection of the vaginal septum. Postoperatively, her symptoms resolved completely, with no recurrence observed at 6-month follow-up. This case contributes to the literature by documenting one of the latest known presentations of OHVIRA syndrome, reinforcing that a high index of suspicion for Müllerian anomalies must be maintained regardless of patient age. It demonstrates that a partially draining orifice can mask classic symptoms for decades and confirms the long-term efficacy of a minimally invasive surgical approach for both diagnosis and treatment.

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