Perioperative Evaluation in Herlyn-Werner-Wunderlich Syndrome

BACKGROUND: Herlyn-Werner-Wunderlich syndrome is defined by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. We report the presentation, radiographic findings, and outcomes of three patients with Herlyn-Werner-Wunderlich syndrome who underwent surgical management at a tertiary care academic medical center. CASES: Two patients were diagnosed by magnetic resonance imaging and one by ultrasonography. All three underwent successful transvaginal septoplasty and drainage of the hematocolpos and hematometra. One required septoplasty revision with temporary vaginal stent placement. CONCLUSION: Herlyn-Werner-Wunderlich syndrome is a rare condition. The most urgent concern is the obstruction of menstrual effluent. Unlike an isolated transverse vaginal septum, the vaginal septum with Herlyn-Werner-Wunderlich syndrome is parallel to the patent vaginal canal, less pliable, and closer to the cervix. These anatomical differences result in pronounced distortion with significant diagnostic and surgical challenges.