Herlyn-Werner-Wunderlich syndrome: Case report

Abstract

Introduction: Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation of the urogenital tract characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis, which is usually diagnosed after menarche. It is treated by resecting the vaginal septum and draining the obstructed hemivagina and has a favorable postoperative prognosis. Case presentation: This is the case of 22-year-old woman with a 9-year history of dysmenorrhea, pelvic pain, and irregular menstrual cycles that started after she had her first menstruation, who visited the gynecology service of a secondary care hospital in Sogamoso, Boyacá (Colombia). The patient reported having visited multiple specialists and being treated with vitamin E, metformin, and contraceptives, without experiencing an improvement of symptoms and signs. After being assessed, and taking into account ultrasound and MRI findings, she was diagnosed with HWW syndrome. She underwent a colpotomy plus resection of right paracervical mass of approximately 60x60mm and vaginal septum, achieving complete resolution of symptoms. Finally, 10 months after the surgery, she reported being pregnant without complications. Conclusion: HWW syndrome is a rare malformation that should be considered as a differential diagnosis in women of any age with paramesonephric duct anomalies, pelvic pain, dysmenorrhea, and presence of masses in the genital tract, since its early diagnosis and timely treatment considerably improve the quality of life of these patients by reducing the severity of symptoms, decreasing the incidence of complications, and improving obstetric prognosis.