(Video Poster 33) Robotic Removal of Hematocolpos & Vaginal Septum in an OHVIRA Patient

Objectives: We will review robotic assisted surgical techniques for the difficult removal of congealed blood from a longstanding hematocolpos as well as the transection of a longitudinal vaginal septum. Our case involves a patient with Obstructed Hemivagina, Ipsilateral Renal Agenesis (OHVIRA) syndrome. We will explore this unique syndrome and its surgical management through the lens of gender affirming care.

Methods: OHVIRA syndrome, also known as Herlyn-Werner-Wunderlich syndrome, is a congenital anomaly in part due to the failed fusion of Mullerian ducts around 10-12 weeks gestational age (Moufawad et al., 2023). It presents as a didelphic uterus, obstructive vaginal septum, and ipsilateral renal anomalies including hypoplasia or agenesis (Mendoza & Nicer, 2022). Its incidence is rare, affecting between 0.1%-3.8% of the population (Friedman et al., 2018). 
We present a 28-year-old transgender male with OHVIRA syndrome. In adolescence the patient underwent a nephrectomy of the right hypoplastic kidney, right supracervical abdominal hysterectomy, and right salpingo-oophorectomy of the obstructed side. Following initiation of testosterone therapy in adulthood he became amenorrheic, however pelvic pain persisted. An MRI revealed a left uterine horn, bilateral cervices, and 9 cm hematocolpos in the obstructed right hemivagina. 
Intraoperatively, we employed a number of techniques to aid in surgical dissection of this altered anatomy. A foley catheter was placed vaginally and inflated in the obstructed hemivagina for identification purposes. The hematocolpos was left intact throughout the hysterectomy of the remaining uterine horn as the mass effect provided tension which aided in the dissection. Multiple different techniques  were unsuccessfully employed in attempts to remove the copious amount of congealed blood from the longstanding hematocolpos. Successful removal was finally accomplished by using a “top down” technique in which a raytec was inserted laparoscopically, and used to “sweep” the blood out as it was delivered vaginally. The remaining longitudinal vaginal septum was then transected robotically using the scissors and vessel sealer starting proximally and extending distally.

Clinical Relevance: This surgery was successful due to the understanding of OHVIRA syndrome and careful identification of the distorted anatomy. Innovative surgical approaches were employed such as maintaining the hematocolpos to improve visualization during the dissection. The manual removal of copious amounts of congealed blood using a rayec, as well as the excision of the vaginal septum in a “top down” fashion contributed to the success. Through the combination of these skills, the patient’s chronic pain and gender dysphoria was successfully addressed in a minimally invasive fashion.