Herlyn Werner Wunderlich Syndrome/OHVIRA: A Rare Case Report with Successful Laparoscopy Assisted Vaginoplasty.

Abstract

Herlyn Werner Wunderlich syndrome is a rare congenital disorder affecting 0.16 percent of the female population, characterized by mal-development of Mullerian and mesonephric ducts. It is also known as "Obstructed Hemivagina and Ipsilateral Renal Agenesis" abbreviated as OHVIRA. The two types of OHVIRA based on the degree of obstruction, complete or incomplete OHVIRA. While incomplete may go undiagnosed, complete OHVIRA presents with varying symptoms and complications requiring early diagnosis and intervention. We present a case of a 14-year-old girl who started menstruating two years ago and has regular periods till 6 months ago. She visited the gynecology outpatient department with persistent lower abdominal pain that worsened during menstruation, along with increased swelling and heaviness in her lower abdomen. Her secondary sexual characteristics matched her age. An abdominal ultrasound and transrectal scan showed a left-sided hematometra and hematocolpos with an 800 cc collection due to a complete hemivagina obstruction. An MRI confirmed the absence of the left kidney, two separate uterine horns, fallopian tube, cervix, and upper vagina. The left upper vagina did not connect with the vaginal opening, causing the syndrome. Surgery was performed to remove the vaginal septum, allowing communication between both hemivaginas and draining the hematocolpos and hematometra. Both uterine horns were equally developed. This surgery effectively changed OHVIRA to a uterine didelphys, improving the obstetric prognosis. Diagnosis is challenging for both complete and partial obstructions without a high level of suspicion because symptoms can be vague, and regular periods can still occur from the other functioning uterine horn. Complete obstruction may present after menarche with abdominal pain and swelling due to trapped blood. Early diagnosis and treatment are crucial to prevent endometriosis from blood spilling into the peritoneal cavity, which can lead to infertility issues due to potential damage to the fallopian tube and increased risk of ectopic pregnancy.