Transvaginal flexible endoscopic fenestration with endoscopic ultrasound guidance for Herlyn–Werner–Wunderlich syndrome in a young patient

Background and Aims Herlyn–Werner–Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly syndrome, is rare, and preoperative identification of the obstructed cavity can be challenging in young patients. We describe transvaginal flexible endoscopic drainage and fenestration using a gastrointestinal endoscope and endoscopic ultrasound guidance. Methods A 12-year-old girl with severe lower abdominal pain underwent computed tomography and magnetic resonance imaging, which showed uterus didelphys, a 6-cm obstructed right hemivaginal/cervicovaginal cavity, and right renal agenesis. Endoscopic treatment was performed. Results Conventional transvaginal endoscopy under carbon dioxide insufflation did not identify a visible bulge on the right vaginal wall. Endoscopic ultrasound guidance enabled visualization and safe puncture of the obstructed cavity. After which, a guidewire-assisted drainage catheter was placed, followed by balloon dilation and electrosurgical incision to create a 2-cm fenestration. A larger catheter and, subsequently, a pigtail stent were placed to prevent restenosis. Her symptoms resolved, and no recurrence has occurred during 2 years of follow-up. Conclusions Transvaginal flexible endoscopy with endoscopic ultrasound guidance may provide a minimally invasive option for drainage and fenestration in selected patients with Herlyn–Werner–Wunderlich syndrome.