#393 : Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) — A Series of 6 Cases at a Tertiary Referral Centre

Background and Aims: OHVIRA is a rare congenital urogenital anomaly. Diagnosis is often delayed because patients have normal onset of puberty and menstruation if there is partial obstruction with a communicating fenestration in the hemivagina. Symptoms can be non-specific such as abdominal pain and dysmenorrhoea, but urinary retention and a pelvic mass have been reported. Haematocolpos can cause anatomical distortion, making imaging difficult. Definitive management is by vaginoplasty but that can be complex, especially in an anatomically small child, or cases with chronic inflammation. Retrograde bleeding during obstruction is common, leading to endometriosis and ascending infection. This is associated with complex surgery, increased morbidity and subfertility, which is why early diagnosis and timely management are important. Method: Medical records were retrieved from 6 patients with OHVIRA treated between 1 April 2021 and 31 March 2023 at a tertiary referral centre. For each patient, age of diagnosis, referral source, symptoms, diagnostic investigations, diagnostic challenges, anatomical findings, surgical managements, complications and outcomes were reviewed. Results: Patient’s ages ranged from 10 to 33 years. Median age of diagnosis was 30. Cases were referred from paediatric surgery (n=1), gynaecology (n=2), general surgery (n=2) and urology (n=1). Symptoms included abdominal pain (n=4), pyrexia (n=2), intra-menstrual bleeding (n=2) and vaginal discharge (n=1). 3 patients had been misdiagnosed before referral. All had unilateral haematocolpos and underwent ultrasound (n=6) and either MRI (n=4) or CT (n=2), followed by vaginoplasty. 2 patients had emergency surgery for pelvic abscesses. There were no intra- or post-operative complications, or adverse outcomes. Conclusion: Median age at diagnosis was advanced, and symptoms and referral source were variable. Misdiagnosis was frequent. Surgical management was successful with a single stage vaginoplasty. Ascending infection can be a consequence of delayed diagnosis and management. It is important to evaluate any female with dysplastic or absent kidneys for uterovaginal abnormalities.