How to diagnose OHVIRA syndrome in adult patients

What is it about?

A patient in her early 20s presented with constant and progressive lower abdominal and back pain, mainly on the right side of the abdomen, purulent vaginal discharge and pyrexia. A radiological assessment revealed a possible tubo-ovarian abscess and the incidental diagnosis of ipsilateral renal agenesis. The patient was treated for pelvic inflammatory disease (PID); however, after antibiotic administration and since the symptoms did not resolve, an abdominal MRI was requested, which revealed uterus didelphys with two cervices, an obstructed haemivagina and evidence of haematocolpos. The diagnosis of Obstructed Hemi-Vagina with Ipsilateral Renal Agenesis (OHVIRA) syndrome was confirmed, and the patient underwent the excision of the vaginal septum, the drainage of the haematopyocolpos and the laparoscopic drainage of the tubo-ovarian abscess. She achieved a good recovery.

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Why is it important?

This case clearly demonstrates the challenges clinicians can have in diagnosing this condition in adult patients and how crucial cooperation and communication between clinicians from different specialties is. An increased awareness of the symptomatology of this syndrome can lead to a timely diagnosis and prevent years of dysmenorrhea and chronic pelvic pain, as well as complex surgical interventions with significant morbidity for the patients. Clear guidelines could ensure the prompt diagnosis and treatment of OHIVRA syndrome in the majority of cases, starting from the postnatal screening of uterine anomalies for babies that were diagnosed with renal agenesis and leading to age-specific treatment based on the specific anomaly