Robotic approach for OHVIRA syndrome: case report of an early diagnosis in a prepubertal girl

INTRODUCTION: OHVIRA (obstructed hemivagina with ipsilateral renal agenesis) syndrome, also known as Herlyn-Werner-Wunderlich syndrome, is a rare congenital anomaly of the female urogenital tract characterized by uterus didelphys, an obstructed hemivagina, and ipsilateral renal agenesis. It is typically diagnosed at puberty due to cyclic pelvic pain or hematocolpos. Early recognition in childhood is uncommon but may prevent serious complications. CASE PRESENTATION: We describe a 1-year-old girl in whom OHVIRA syndrome was incidentally identified during imaging performed for unrelated findings. Ultrasonography and magnetic resonance imaging demonstrated right renal agenesis, uterine duplication, and an obstructed hemivagina. Diagnostic endoscopy confirmed vaginal duplication with a right atretic hemivagina and absence of the right ureteral orifice, suggesting an ectopic ureteral insertion. The patient underwent robotic-assisted right nephroureterectomy, partial hysterectomy, and excision of the right hemivagina without complications. She was discharged on postoperative day 2 and remains asymptomatic after 1 year of follow-up. CLINICAL DISCUSSION: Early identification of OHVIRA syndrome is essential to prevent complications such as infection, pelvic abscess, endometriosis, and infertility. Robotic surgery offers excellent visualization, precise dissection, and safe reconstruction, particularly in deep pelvic regions. Compared with conventional approaches, it provides reduced postoperative pain, shorter hospital stays, and faster recovery. CONCLUSION: Robotic-assisted surgery represents a safe and minimally invasive approach for the management of OHVIRA syndrome, even in very young patients, ensuring complete anatomical correction with minimal morbidity and rapid recovery.