A case study of Herlyn Werner Wunderlich Syndrome (OHVIRA)

Herlyn Werner Wunderlich syndrome, also known as Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA), is a rare congenital condition affecting 0.16% of females. It involves malformation of the Mullerian and mesonephric ducts, with two forms: complete and incomplete OHVIRA. The incomplete type may go undetected, while the complete type can lead to noticeable symptoms that necessitate prompt diagnosis and treatment.

In this case, a 14-year-old girl who began menstruating two years ago presented with persistent lower abdominal pain, worsening during her menstrual cycle, and increased swelling in her abdomen. Her secondary sexual characteristics were appropriate for her age. Imaging studies, including an abdominal ultrasound and transrectal scan, revealed a left-sided hematometra and hematocolpos caused by complete hemivaginal obstruction. An MRI confirmed the absence of the left kidney and identified two separate uterine horns, fallopian tubes, cervix, and upper vagina, with the left upper vagina not connecting to the vaginal opening.

Surgical intervention involved removing the vaginal septum to establish communication between the hemivaginas and drain the accumulated blood. This procedure transformed the condition from OHVIRA to uterine didelphys, improving the patient's obstetric outlook. Diagnosing both complete and partial obstructions can be difficult due to nonspecific symptoms and the possibility of regular menstruation from the functioning uterine horn. Complete obstruction often manifests after the onset of menstruation with symptoms like abdominal pain and swelling due to trapped blood. Therefore, early identification and treatment are vital to avoid complications such as endometriosis, which can lead to infertility and an increased risk of ectopic pregnancy.

Source: Madhumitha J, Charumathi R, Sundaram, M. et al. Herlyn Werner Wunderlich Syndrome/OHVIRA: A Rare Case Report with Successful Laparoscopy Assisted Vaginoplasty. J Obstet Gynecol India (2024). https://doi.org/10.1007/s13224-024-02039-8

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