Staged Management of Recurrent Obstructed Hemivagina in Herlyn-Werner-Wunderlich Syndrome: A Case Report and Literature Review.
- 1Department of Obstetrics and Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, 050000, People's Republic of China.
- 2Hebei Key Laboratory of Regenerative Medicine of Obstetrics and Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, 050000, People's Republic of China.
- 3National Clinical Key Specialty Construction Project of Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, 050000, People's Republic of China.
Abstract
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.
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BibTeX
@article{miao2026staged,
title = {{Staged Management of Recurrent Obstructed Hemivagina in Herlyn-Werner-Wunderlich Syndrome: A Case Report and Literature Review.}},
author = {Xueqian Miao and Zhongkang Li and Yiwen Dong and Yanling Xie and Yibin Liu and Yanan Li and Yanfang Du and Li Meng and Xianghua Huang},
journal = {International journal of women's health},
year = {2026},
month = oct,
doi = {10.2147/ijwh.s633875},
url = {https://europepmc.org/article/MED/42899218},
note = {CC BY-NC},
}
RIS
TY - JOUR
TI - Staged Management of Recurrent Obstructed Hemivagina in Herlyn-Werner-Wunderlich Syndrome: A Case Report and Literature Review.
AU - Xueqian Miao
AU - Zhongkang Li
AU - Yiwen Dong
AU - Yanling Xie
AU - Yibin Liu
AU - Yanan Li
AU - Yanfang Du
AU - Li Meng
AU - Xianghua Huang
PY - 2026
DA - 2026/10/15
JO - International journal of women's health
DO - 10.2147/ijwh.s633875
UR - https://europepmc.org/article/MED/42899218
AB - 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.
SN - 1179-1411
ER -