Obstructed hemivagina and ipsilateral renal abnormality (OHVIRA) is a rare disorder first identified and termed by Herlyn- Werner- Wunderlich, after whom it was initially known as HWW syndrome. It is characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly.1,2 Patients usually present after puberty and menarche when they commonly complain of dysmenorrhea, lower abdominal pain, pelvic mass, urinary retention, and frequent pelvic or urinary infection.

The incidence is unknown but quoted at 0.1%-3%,2,3,4 and the etiology is poorly understood as well. Although classically they present with ipsilateral renal agenesis, other renal anomalies, such as renal dysplasia or ectopic ureters, can occur too.2 Hence, awareness is important for the renal physician to refer early to the pediatric and adolescent gynecologist (PAG).1,4,8 In most OHVIRA cases, the septum is thick, limiting the hemivagina to distend, resulting in retrograde flow leading to endometriosis. Endometriosis has been reported in 17-35% of patients with this condition, and timely decompression may avoid this.1,2,5 Occasionally, the septum may be thin and fenestrated, resulting in only a partial obstruction and vaguer to no symptoms, which further delay the diagnosis.2,4

We do know that timely diagnosis and appropriate management will improve the quality of life, as well as preserve the fertility of these young women. Therefore, we set out to study the clinical course of the syndrome based on past data to propose a feasible screening test to improve the pick-up rate for patients with OHVIRA and hence improve the management.

Sabah is the second largest state in Malaysia, with an area of 73,904 km2, and is situated in the northern part of Borneo, in East Malaysia. It has a population of 3.39 million, which is around 10% of the entire Malaysian population (33.5 million). Malaysia as a whole is a developing nation; however, compared with other states in Malaysia, Sabah is considerably more backward in terms of expertise and infrastructure. With such a large population, there is only one public tertiary health center with subspecialty services that is situated in Kota Kinabalu, where patients with complicated issues are referred from all over the state for further management. In Sabah, there is only one PAG specialist. Given the constraints of facilities and experience, diagnostic tools are primarily based on a clinical approach, with minimal imaging, much less advanced imaging such as computed tomography (CT) or magnetic resonance imaging (MRI) scans. In spite of the widespread availability of ultrasonography in clinics and emergency departments, only complex cases will be prioritized for advanced imaging due to the high volume of patients. Therefore, we suggest a simple screening technique that is to scan the renal system in addition to pelvic ultrasound to improve the OHVIRA diagnosis rate. This is simple to execute and widely accessible, making it the more sustainable option.