Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA): The Lesser Known Aspects: A Mini-review and Experience from a Tertiary Care Center

INTRODUCTION: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome also known as Herlyn-Werner-Wunderlich (HWW) syndrome was first reported by Purslow in 1922. Failure of the Mullerian duct to open into the urogenital sinus results in obstructed hemivagina, when associated with Wolffian duct abnormality can lead to renal agenesis. This association leads to imperforate hemivagina and unilateral renal agenesis, which constitutes this syndrome. This has been recognized more readily in recent years due to increasing familiarity with the disorder and advanced diagnostic methods. AIM AND OBJECTIVE: To study the common clinical presentation of patients with OHVIRA and their management strategies. METHODS AND MATERIAL: This retrospective cohort study was done in a tertiary care center in South India, and the data were collected from January 2007 to December 2021. The study was approved by the institutional review board and Ethics committee. (IRB no.14576, March 2022). RESULTS: A total of 25 patients were found with OHVIRA syndrome. The mean age at presentation was 17.7 years. The most common presenting symptoms were dysmenorrhea (19;72%) followed by pelvic pain (15;60%) and menstrual irregularities (12;52%). MRI confirmed the diagnosis in 64% of patients, and the most common complication was hematocolpos (72%), followed by pyocolpos in 24%. All patients except for one underwent primary vaginal septal resection. One patient underwent diagnostic laparoscopy proceed vaginal septal resection. CONCLUSION: Patients of younger age, especially those less than 20 years presenting with dysmenorrhea and menstrual irregularities, need a thorough evaluation. For patients with pelvic pain and dysmenorrhea, it is always prudent to do advanced imaging if the symptoms are not relieved with simple medical measures.