Staged Management of Recurrent Obstructed Hemivagina in Herlyn-Werner-Wunderlich Syndrome: A Case Report and Literature Review.

BACKGROUND: Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital Müllerian anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal anomaly. Recurrence after prior surgical treatment is particularly rare. We report a complex recurrent case of obstructed hemivagina in a patient with HWWS and duplicated vagina, successfully managed with a staged surgical approach.

CASE PRESENTATION: A 19-year-old woman with a history of two prior vaginal septum resections and excision of a perineal mass (histopathology confirming ectopic colonic tissue) presented with recurrent pelvic pain and fever. MRI and endoscopic assessment demonstrated uterus didelphys, duplicated cervices and vaginas, complete obstruction of the right hemivagina, ipsilateral renal agenesis, and a deeply recessed obstructed cavity containing purulent fluid. Because of the markedly distorted postoperative anatomy, a staged surgical strategy was adopted. The first stage involved hysteroscopic and vaginoscopic decompression with ultrasound-guided septal incision and drainage. The second stage consisted of definitive reconstruction, including resection of the accessory blind tract, obliteration of the residual obstructed cavity, and perineal reconstruction. The patient recovered uneventfully, resumed regular painless menstruation. No recurrence of hemivaginal obstruction was observed during follow-up.However, the diagnosis was not definitive. The patient had lifelong persistent urinary incontinence that showed no improvement after surgery. Although a right ectopic ureter or ureterocele was unlikely to account for the continuous leakage because the right kidney was nonfunctioning, a left ectopic ureter and other lower urinary tract abnormalities had not been fully excluded.

CONCLUSIONS: This case highlights a complex recurrent presentation of HWWS and emphasizes the importance of MRI and endoscopic evaluation in delineating atypical postoperative anatomy. Individualized staged surgical management may help prevent re-obstruction, control infection, restore functional genital tract continuity, and preserve future reproductive potential in patients with complicated or recurrent HWWS. Persistent urinary incontinence and a history of perineal ectopic colonic tissue warrant further urological and multidisciplinary evaluation, a left ectopic ureter and other lower urinary tract abnormalities should be carefully evaluated in this patients.