BACKGROUND: Herlyn-Werner-Wunderlich syndrome is an uncommon entity characterized by uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis (also called OHVIRA syndrome). Due to rarity and varied presentations, often correct diagnosis is missed out during work up, leading to management problems. We describe our dependence on detailed preoperative work up and minimally invasive endoscopic approach in management of the eight patients of OHVIRA syndrome. METHODS: In this retrospective case series study, eight patients of OHVIRA syndrome were managed from January 2012 to March 2018 with the help of improved imaging facility and diagnostic work up. Precise diagnosis helped in adopting minimally invasive approach in management. Patients were reviewed, focusing on presentation, radiologic findings and surgical management. RESULTS: Median age at diagnosis was 19 years (range 13-41 years). Abdominal pain and dysmenorrhea were the main presenting complaint. All patients except one had associated ipsilateral renal agenesis. Surgical excision of the obstructed hemivaginal septum and hematometra drainage was the main treatment. In seven patients, vaginoscopic resection of vaginal septum was done with resectoscope except one 41-year-old patient, where resection of vaginal septum was performed laparoscopically along with hysterectomy. CONCLUSION: Vaginoscopic resection of obstructed hemivaginal septum is an effective method. Management has shifted to minimally invasive approach due to improved imaging, precise preoperative diagnosis and proper understanding of the disease.
BACKGROUND: Herlyn-Werner-Wunderlich syndrome is an uncommon entity characterized by uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis (also called OHVIRA syndrome). Due to rarity and varied presentations, often correct diagnosis is missed out during work up, leading to management problems. We describe our dependence on detailed preoperative work up and minimally invasive endoscopic approach in management of the eight patients of OHVIRA syndrome. METHODS: In this retrospective case series study, eight patients of OHVIRA syndrome were managed from January 2012 to March 2018 with the help of improved imaging facility and diagnostic work up. Precise diagnosis helped in adopting minimally invasive approach in management. Patients were reviewed, focusing on presentation, radiologic findings and surgical management. RESULTS: Median age at diagnosis was 19 years (range 13-41 years). Abdominal pain and dysmenorrhea were the main presenting complaint. All patients except one had associated ipsilateral renal agenesis. Surgical excision of the obstructed hemivaginal septum and hematometra drainage was the main treatment. In seven patients, vaginoscopic resection of vaginal septum was done with resectoscope except one 41-year-old patient, where resection of vaginal septum was performed laparoscopically along with hysterectomy. CONCLUSION: Vaginoscopic resection of obstructed hemivaginal septum is an effective method. Management has shifted to minimally invasive approach due to improved imaging, precise preoperative diagnosis and proper understanding of the disease.
Authors: Riccardo Del Vescovo; Sofia Battisti; Valerio Di Paola; Claudia L Piccolo; Roberto L Cazzato; Ilaria Sansoni; Rosario F Grasso; Bruno Beomonte Zobel Journal: BMC Med Imaging Date: 2012-03-09 Impact factor: 1.930