Staged Treatment of Recurrent Obstructed Hemivagina in Herlyn–Werner–Wunderlich syndrome with Double Vagina: A Case Report and Mini-Review

Staged Treatment of Recurrent Obstructed Hemivagina in Herlyn–Werner–Wunderlich syndrome with Double Vagina: A Case Report and Mini-Review | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Staged Treatment of Recurrent Obstructed Hemivagina in Herlyn–Werner–Wunderlich syndrome with Double Vagina: A Case Report and Mini-Review Xueqian Miao, Zhongkang Li, Yiwen Dong, Yanling Xie, Yibin Liu, and 4 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-8223990/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Background: Herlyn–Werner–Wunderlich syndrome (HWWS) is a rare congenital Müllerian anomaly characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal malformation, and postoperative recurrence is even more uncommon. This report details a unique case of recurrent obstructed hemivagina in Herlyn-Werner-Wunderlich syndrome with double vagina, managed with a staged surgical approach. Case presentation: We report a 19-year-old woman with a history of two prior vaginal septum resections who presented with recurrent pelvic pain and fever. Imaging revealed uterine didelphys, duplicated cervices and vaginas, complete obstruction of the right hemivagina, and ipsilateral renal agenesis. Vaginoscopy demonstrated a deeply recessed recurrent cavity with purulent fluid, indicating persistent obstruction and local infection. Given the distorted postoperative anatomy, a staged surgical strategy was adopted: initial hysteroscopic decompression and septal incision under ultrasound guidance, followed by definitive reconstruction including resection of the accessory pseudo-vaginal tract, obliteration of the residual obstructed chamber, and vulvar repair. The patient recovered well, achieved regular painless menstruation, and experienced no recurrence during follow-up. Conclusions: This case highlights a complex recurrent variant of HWWS and underscores the essential role of MRI and endoscopic assessment in delineating atypical anatomy. Individualized, stepwise surgical correction is critical for preventing restenosis, controlling infection, restoring normal genital tract continuity, and preserving future reproductive potential in patients with complicated or recurrent HWWS. Herlyn–Werner–Wunderlich syndrome double vagina longitudinal vaginal septum recurrent obstructed hemivagina staged trans-septal management Figures Figure 1 Figure 2 Figure 3 Figure 4 1. Introduction Herlyn–Werner–Wunderlich (HWW) syndrome—also termed obstructed hemivagina and ipsilateral renal anomaly (OHVIRA)—is a rare congenital Müllerian malformation defined by the triad of uterus didelphys, an obstructed hemivagina, and ipsilateral renal agenesis (1–3). Owing to the shared embryologic origin of the Müllerian and Wolffian ducts, abnormalities frequently coexist, and the timing or degree of developmental arrest determines the wide clinical spectrum. Most patients present soon after menarche with cyclic pelvic pain, dysmenorrhea, pelvic mass, or abnormal discharge; delayed recognition may result in hematocolpos, hematometra, tubal distention, pelvic infection, endometriosis, and subsequent fertility impairment (1–3). To improve diagnostic consistency and guide operative planning, structured classification systems have been developed. The ESHRE/ESGE 2013 consensus provides a reproducible anatomy-based framework for uterine, cervical, and vaginal anomalies (4), while the ASRM Müllerian Anomalies Classification 2021 (MAC2021) incorporates clinically oriented subtypes and explicitly acknowledges associated urinary tract malformations (5). Imaging is central to diagnosis and surgical road-mapping. MRI remains the preferred modality in adolescents or non–sexually active patients because it reliably defines the level of obstruction, the continuity between duplicated cavities, and associated renal or ureteral anomalies (1,2). Expert ultrasonography—including transabdominal, transrectal, or selected 3D approaches—serves as a valuable adjunct when MRI is unavailable or when real-time intraoperative guidance is required (2,6,7). Surgical treatment typically consists of transvaginal or endoscopic septal resection with wide marsupialization to prevent restenosis. Large case series demonstrate rapid symptom relief and generally favorable long-term reproductive outcomes; however, restenosis, persistent obstruction, or infection may occur in anatomically complex cases or after incomplete resection (6–8). Recent systematic reviews emphasize standardized preoperative mapping, careful application of classification systems, and individualized adjunctive measures—such as temporary drainage, staged procedures, or targeted cavity remodeling—to minimize re-obstruction and preserve fertility (8). Notably, the spectrum of HWW extends beyond the classic triad: variants involving ectopic or remnant ureteral segments, Gartner duct cysts communicating with the obstructed hemivagina, vesicovaginal or ureterovaginal fistulas, or distorted postoperative anatomy may obscure diagnosis and complicate surgical decision-making (9,10). Within this context, we present a recurrent HWW case with duplicated vaginas and repeated hemivaginal obstruction years after prior septal resections, managed successfully using MRI-guided mapping and a staged trans-septal reconstructive strategy. 2. Case presentation 2.1 Case characteristics and clinical course A 19-year-old nulligravid woman was admitted on 13 April 2025 with a one-month history of lower abdominal pain occurring after menstruation, accompanied by intermittent fever up to 38 °C. She had regular menses since menarche at age 12 (3–4 days/30-day cycle, moderate flow) and reported dysmenorrhea only prior to earlier surgeries; her cycles were painless thereafter. Her neonatal history was notable for surgical excision of a perineal mass at 1 month of age, with pathology confirming ectopic colonic tissue. Right renal agenesis and lifelong continuous urinary incontinence were documented in infancy. At age 9, cystoscopy revealed a small-capacity bladder with trabeculation, absence of the right ureteral orifice, hypoplastic urethral sphincter, and a valve-like mucosal fold adjacent to the right bladder neck. At age 12 (30 May 2017), six months after menarche, she presented with cyclic abdominal pain and fever. Imaging and examination confirmed a longitudinal vaginal septum consistent with Herlyn–Werner–Wunderlich (HWW) syndrome. She underwent ultrasound-guided hysteroscopy and transvaginal septum resection. Five months later (6 November 2017), recurrent pain prompted a second transvaginal resection, after which she remained asymptomatic for more than seven years. One month before the current admission, an abdominal ultrasound at a local clinic suggested renal malformation and a right lower abdominal mixed-echo mass. Subsequent CT demonstrated pelvic fat stranding with minimal effusion, nonvisualization of the right kidney, and irregular morphology of the solitary left kidney. Gynecologic ultrasound suggested a Müllerian anomaly with suspected right-sided hematocolpos/hematosalpinx and pelvic effusion. Outpatient ultrasound at our institution similarly indicated congenital urogenital malformation with right-sided obstructed genital tract and prompted inpatient evaluation. Physical examination showed normal left labia, a well-healed perineal scar on the right, and an orthotopic urethral meatus with continuous leakage. Posterior to the hymenal ring at the 7–8 o’clock position, a 1.5-cm depression was probed to a 3-cm blind end. Bimanual and rectal examinations revealed a normal left cervix and uterus and a right adnexal fullness; mobility was preserved. Laboratory tests, including complete blood count, biochemistry, and tumor markers, were within normal limits. Pelvic MRI demonstrated findings characteristic of recurrent HWW syndrome: uterus didelphys, complete obstruction of the right hemivagina with hematocolpos, a patent left cervix, small pelvic effusion, and suspected iliac lymphatic cysts (Figure 1A, B). CT of the urinary tract again confirmed right renal agenesis, compensatory hypertrophy with irregular contour of the left kidney, and mild bladder wall thickening with scant intravesical gas (Figure 1C). Dynamic renal scintigraphy showed complete absence of right renal function and compensatory hyperfunction of the left kidney. A working diagnosis of recurrent obstructed right hemivagina following two prior septal resections, associated with complex double-vagina anatomy and congenital urinary tract malformations with persistent urinary incontinence, was established. 2.2 Interventions Exploratory mapping (15 April 2025). A diagnostic vaginoscopy was performed to clarify the distorted anatomy following two prior septal resections. The left cervix appeared normal. At the 7–8 o’clock position of the vaginal wall, a pinpoint opening led into a deep posterior cavity containing purulent, mucinous material. Due to the complexity of the obstructed tract and the need for comprehensive preoperative planning, this session was limited to anatomical mapping without further intervention. Stage 1 – Septal incision, trans-septal drainage, and balloon stenting (17 April 2025). Under combined vaginoscopy, hysteroscopy, and real-time transabdominal ultrasonography, the left cervical canal was first identified as normal (Figure 2A–B). A thick right longitudinal vaginal septum was visualized obstructing the route toward the posterior cavity (Figure 2C). A targeted incision was then made along the septum, approximately 1.5 cm in thickness, allowing entry into the obstructed posterior compartment where purulent and mucinous debris was encountered (Figure 2D). Copious irrigation was performed. At the dome of the posterior cavity, two small ostia were identified (Figure 2E–F). The superior ostium displayed a squamo-columnar junction consistent with the right external cervical os, while the inferior ostium communicated with an adjacent micro-channel. A magnified view of the right cervical os is shown in Figure 2G, and the lower accessory ostium beneath the right cervix is depicted in Figure 2H. The septal incision was further extended inferiorly to approximately 2 cm above the hymenal ring and leveled flush with the main vaginal lumen. Hemostasis was secured with bipolar coagulation. Because the accessory perineal tract (“right accessory vagina”) lay in close proximity to the posterior cavity floor yet remained a blind pouch externally, a deliberate trans-septal puncture was performed and a balloon catheter was positioned across the newly created channel (inflated with 18 mL saline) to allow drainage, equalize pressure, flatten the deeply recessed cavity, and reduce early re-closure. The final appearance following septal decompression is shown in Figure 2I. The updated intraoperative diagnosis included uterus didelphys, duplicated cervices and vaginas, recurrent obstruction of the right longitudinal septum, a right accessory transverse septum, urinary incontinence, and remote perineal ectopic-colon excision. A second-stage procedure was scheduled after the next menstrual cycle. Stage 2 – Posterior-cavity obliteration, accessory-tract inversion/excision, and perineal reconstruction (12 May 2025). Under general anesthesia in lithotomy position, the anatomic configuration of the recurrent obstructed right hemivagina and its communication with the accessory perineal tract, as illustrated in Figure 3A, was reassessed. The previously placed balloon catheter occupying both the accessory tract and posterior cavity was identified (Figure 3B). A circumferential submucosal hydrodissection plane was developed, permitting sharp excision of the lower two-thirds of the accessory tract. The proximal stump was then closed using a 3-0 absorbable purse-string suture. The suture tail was routed through the pre-existing trans-septal channel into the main (left) vaginal lumen; gentle traction inverted the proximal segment of the accessory tract into the main vaginal cavity, resulting in complete sealing of the tract. To eliminate the residual “dam effect” created by the deeply recessed posterior cavity, 2-0 absorbable sutures were passed from the main vagina through the septal window into the posterior cavity and then across the transverse tissue toward the accessory-tract wound, effectively flattening and buttressing the cavity floor. Additional vertical mattress sutures further reinforced closure of the accessory-tract bed. The final reconstructed anatomy, with complete obliteration of the posterior cavity and normalized perineal contour, is shown in Figure 3C. Blood loss was minimal; urine remained clear intraoperatively, and digital rectal examination confirmed preservation of rectal mucosal integrity. Outcomes and follow-up. Postoperative recovery was uneventful. Menstrual cycles subsequently remained regular, without dysmenorrhea, intermenstrual spotting, or clinical evidence of re-accumulation. The staged, MRI-guided, trans-septal strategy effectively resolved recurrent obstruction in this complex double-vagina variant of HWW syndrome and restored functional genital tract continuity. 3. Discussion Herlyn–Werner–Wunderlich (HWW) syndrome represents a unique subgroup of obstructed Müllerian anomalies characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal anomaly. Although the triad appears uniform, the syndrome encompasses substantial anatomic heterogeneity along both Müllerian and Wolffian duct derivatives, explaining its highly variable clinical manifestations. Patients typically present soon after menarche with cyclic pelvic pain, dysmenorrhea, purulent discharge, malodorous secretions, or intermittent fever; delayed diagnosis may result in hematocolpos, hematometra, ascending infection, pelvic adhesions, and secondary endometriosis, all of which may adversely affect future fertility (1–3). To standardize terminology and improve diagnostic precision, multiple classification systems have been developed. While the ESHRE/ESGE 2013 and ASRM MAC 2021 systems have improved communication and provided structured criteria for uterine, cervical, and vaginal anomalies (4,5), they do not fully capture the anatomical diversity within HWW syndrome. In China, the 2021 Expert Consensus further subdivides obstructed hemivagina into four clinically meaningful types—imperforate septum (Type I), micro-perforated septum (Type II), imperforate septum with cervical fistula (Type III), and cervical atresia (Type IV)—highlighting how subtle differences in septal configuration affect symptom onset, obstruction severity, and choice of operative strategy (Fig. 4 ) (6). Building on these frameworks, the KUU′V system (kidney–ureter–uterus–vaginal septum) proposed in 2022 integrates renal and ureteral anomalies with Müllerian variants, reflecting the high coexistence of urinary tract defects in HWW syndrome (7). Clinical heterogeneity in HWW syndrome is closely linked to these anatomic variations. A recent large-scale study involving 1673 patients reported dysmenorrhea (53.8%), abnormal uterine bleeding (28.9%), and vaginal discharge (26.6%) as the most common symptoms; right-sided obstruction (57.3%), hematocolpos (81.7%), uterus didelphys (88.8%), and ipsilateral renal agenesis (93.1%) were the principal anomalies (8). Endometriosis was identified in approximately 9.6% of patients, with over half exhibiting ipsilateral endometriotic ovarian cysts. This aligns with the hypothesis that prolonged retrograde menstruation due to obstruction promotes endometriosis development (8,9). Preoperative imaging plays a decisive role in defining obstruction level, identifying coexisting urinary tract anomalies, and avoiding false passages during surgery. Although ultrasonography is widely used as a first-line tool, MRI remains the most accurate modality for delineating uterine morphology, evaluating septal thickness, recognizing fistulous tracts, and identifying the precise geometry of the obstructed cavity (1,10). Consensus guidelines from ESHRE/ESGE recommend combining gynecologic examination with 2D ultrasonography as an initial evaluation and endorsing 3D ultrasonography or MRI as the diagnostic “reference standard” in complex cases (9). In this patient, MRI precisely mapped uterus didelphys, complete obstruction of the right hemivagina, and the shape and depth of the posterior compartment—information essential for safe operative navigation. The present case underscores the limitations of relying exclusively on initial classification-based management. Despite two prior childhood septal resections, the patient experienced recurrent obstruction more than seven years later—a delayed failure attributed to several possible factors: (1) an incompletely leveled or uneven septal edge functioning as a “valve,” predisposing to re-closure; (2) a deeply recessed posterior cavity acting as a persistent dead space conducive to fluid stasis, infection, and fibrosis; (3) an unrecognized accessory perineal tract functioning as a concealed reservoir for contamination; and (4) prior interventions performed amid active inflammation, increasing the risk of postoperative adhesions and scarring. These mechanisms have been corroborated by multiple studies reporting that recurrence often results not from inadequate septal width alone but from failure to manage adjacent comunicating spaces or accessory channels (6–8,11,12). Given these challenges, a staged surgical approach was deliberately selected. The first stage—MRI-guided vaginoscopy, targeted septal incision, evacuation of purulent material, and trans-septal balloon stenting—allowed decompression of the infected cavity, flattening of the posterior recess, and stabilization of the mucosa before definitive reconstruction. Similar strategies have been recommended in obstructed Müllerian anomalies where temporary stenting or marsupialization minimizes early restenosis (6–8). The second stage addressed the underlying risk factors for late recurrence by obliterating the posterior dead space, inverting and excising the accessory perineal tract, and restoring normal vulvovaginal geometry. The complete elimination of alternative outflow pathways and correction of the “dam effect” are consistent with surgical principles described in recurrent HWW variants (11–14). Long-term reproductive outcomes are a central concern for young women with HWW syndrome. Although pregnancy rates after successful septal resection are favorable (approximately 72%), adverse obstetric outcomes remain more frequent than in the general population, including higher rates of miscarriage, preterm delivery, and cesarean section (8). Early diagnosis and timely intervention are therefore essential not only for symptom control but also for reducing the risk of endometriosis, preserving fertility, and preventing chronic pelvic adhesions (2,8,9). Psychosocial factors, including anxiety related to urinary incontinence or repeated pelvic surgeries, should not be overlooked. In this case, framing the surgical plan as “two smaller, safer steps” improved patient understanding and adherence while minimizing perioperative anxiety. In conclusion, this complex case of double-vagina HWW syndrome with late recurrent obstruction illustrates that successful management requires addressing not merely the septum but the interconnected system of obstructed and accessory spaces. MRI-based mapping, staged decompression, cavity obliteration, and elimination of accessory tracts provided durable symptomatic relief and restored normal menstrual function. For patients with infection, deep posterior cavities, thick septa, or associated urinary anomalies, a staged strategy should be strongly considered. Precision imaging and tailored operative planning remain indispensable to prevent re-obstruction, reduce morbidity, and preserve long-term reproductive potential. Declarations Acknowledgements Not applicable. Funding This study was supported by the following funding sources: the Natural Science Foundation of Hebei Province, Project No. H2023206356; the Natural Science Foundation of Hebei Province, Project No. H2024206427; the Natural Science Foundation of Hebei Province, Project No. H2024206433; the Hebei Provincial Central Government-Guided Local Science and Technology Development Fund Project (Science and Technology Innovation Base Project), Project No. 236Z7756G; the 2024 Hebei Provincial Government-Sponsored Clinical Medicine Outstanding Talent Training Program, Project No. ZF2024040; and the 2025 Hebei Provincial Government-Sponsored Clinical Medicine Outstanding Talent Training Program, Project No. ZF2025102. Availability of data and materials The datasets generated and/or analyzed during the current study are available from the corresponding author upon reasonable request. Authors’ contributions Xueqian Miao and Zhongkang Li conceived the study, coordinated the project, and supervised the overall research design. Yiwen Dong, Yanling Xie, Yibin Liu and Yanan Li collected the clinical data and contributed to data curation. Yanfang Du and Li Meng assisted with methodological development and contributed to critical discussion of the manuscript. Zhongkang Li, Yibin Liu, and Xueqian Miao drafted the manuscript. Li Meng and Xianghua Huang prepared and reviewed the figures. Xianghua Huang, Li Meng, and Yanfang Du critically revised and finalized the manuscript. All authors read and approved the final version of the manuscript. Ethics approval and consent to participate Not applicable. This study is a retrospective case report and does not involve identifiable patient information. Patient consent for publication Written informed consent for publication was obtained from the patient and/or legal guardians. Competing interests The authors declare that they have no competing interests. References Del Vescovo R, Battisti S, Di Paola V, et al. Herlyn–Werner–Wunderlich syndrome: MRI findings and differential diagnosis. BMC Med Imaging. 2012;12:4. Panaitescu AM, Peltecu G, Gică N. Herlyn–Werner–Wunderlich syndrome: Case report and review of the literature. Diagnostics (Basel). 2022;12(10):2466. Wdowiarz K, Rajtar-Czyż M, Starczewski A. Diagnosis and treatment of Herlyn–Werner–Wunderlich syndrome. Pol Przegl Chir. 2021;93(2):42-48. Grimbizis GF, Gordts S, Di Spiezio Sardo A, et al. ESHRE/ESGE classification of female genital tract congenital anomalies. Hum Reprod. 2013;28(8):2032-2044. Pfeifer SM, Attaran M, Goldstein J, et al. ASRM Müllerian Anomalies Classification 2021. Fertil Steril. 2021;116(5):1238-1252. Smith NA, Laufer MR. Management and follow-up of obstructed hemivagina and ipsilateral renal anomaly. Fertil Steril. 2007;87(4):918-922. Gungor Ugurlucan F, Dural O, Yasa C, et al. Diagnosis, management, and outcome of OHVIRA syndrome. Clin Imaging. 2020;59:172-178. Moufawad G, Giannini A, D’Oria O, et al. Systematic review of OHVIRA syndrome diagnosis and surgical management. Gynecol Minim Invasive Ther. 2023;12(3):123-129. Xu X, Tian Y, Zhou J, et al. HWWS with unusual urinary anomalies: Case report and literature review. Heliyon. 2024;10:e33558. Yu JH, Lee SR, Choi H, et al. Complex urinary anomalies in HWWS. Diagnostics. 2021;11(1):7. Girardi FC, Aleixes SRJ, Atuati MA, et al. Diagnosis and treatment of atypical HWWS: MRI and surgical insights. Int J Surg Case Rep. 2019;63:129-134. Dias JL, Jogo R. Pre- and post-surgical MRI and US findings in HWWS. Abdom Imaging. 2015;40:2667-2682. Tong J, Zhu L, Chen N, Lang J. Endometriosis in HWWS. Fertil Steril. 2014;102(3):790-794. Candiani M, Vercellini P, Ferrero-Caroggio C, et al. Long-term outcomes of HWWS conservative surgery. Eur J Obstet Gynecol Reprod Biol. 2022;275:84-90. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-8223990","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":566226610,"identity":"35e5cac4-8a5e-420f-8489-8dd0b495ceaa","order_by":0,"name":"Xueqian Miao","email":"","orcid":"","institution":"The Second Hospital of Hebei Medical University","correspondingAuthor":false,"prefix":"","firstName":"Xueqian","middleName":"","lastName":"Miao","suffix":""},{"id":566226611,"identity":"c89eaf1f-4141-47df-af8c-e4089d7e89fd","order_by":1,"name":"Zhongkang Li","email":"","orcid":"","institution":"The Second Hospital of Hebei Medical University","correspondingAuthor":false,"prefix":"","firstName":"Zhongkang","middleName":"","lastName":"Li","suffix":""},{"id":566226612,"identity":"cecbb2b9-c566-4057-bcdc-ed32b6e0adfe","order_by":2,"name":"Yiwen Dong","email":"","orcid":"","institution":"The Second Hospital of Hebei Medical University","correspondingAuthor":false,"prefix":"","firstName":"Yiwen","middleName":"","lastName":"Dong","suffix":""},{"id":566226613,"identity":"5ed0127d-908f-473a-a691-4698f0c41942","order_by":3,"name":"Yanling Xie","email":"","orcid":"","institution":"The Second Hospital of Hebei Medical University","correspondingAuthor":false,"prefix":"","firstName":"Yanling","middleName":"","lastName":"Xie","suffix":""},{"id":566226614,"identity":"d28c90cb-2525-4229-983e-4cb6c163d2c1","order_by":4,"name":"Yibin Liu","email":"","orcid":"","institution":"The Second Hospital of Hebei Medical University","correspondingAuthor":false,"prefix":"","firstName":"Yibin","middleName":"","lastName":"Liu","suffix":""},{"id":566226615,"identity":"9827e806-3bc8-4875-8bc9-4e799de95dac","order_by":5,"name":"Yanan Li","email":"","orcid":"","institution":"The Second Hospital of Hebei Medical University","correspondingAuthor":false,"prefix":"","firstName":"Yanan","middleName":"","lastName":"Li","suffix":""},{"id":566226616,"identity":"d3fbde5f-72b8-47f9-a4fd-3598235fe558","order_by":6,"name":"Yanfang Du","email":"","orcid":"","institution":"The Second Hospital of Hebei Medical University","correspondingAuthor":false,"prefix":"","firstName":"Yanfang","middleName":"","lastName":"Du","suffix":""},{"id":566226617,"identity":"c7bd75dc-73de-47f4-90a7-2bea7916bada","order_by":7,"name":"Li Meng","email":"","orcid":"","institution":"The Second Hospital of Hebei Medical University","correspondingAuthor":false,"prefix":"","firstName":"Li","middleName":"","lastName":"Meng","suffix":""},{"id":566226618,"identity":"9897d85c-b310-40d3-bd6f-3144b61f58f3","order_by":8,"name":"Xianghua Huang","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAABBUlEQVRIiWNgGAWjYBACAwh1QI6NvfngYyADzJUgRosxP8+xZGOStCTOnJFjJk2UFnOJ5GcPv7bdYdxwIMGsuqDiTrTBAeaDt3kY7PJwabGckWZuLHPmGbPBgQNpt2eceZa74QBbsjUPQ3IxTofdSDCTlqg4zGZwsOHYbd62w0AtPGbSPECnNuDUkv5NWsLgMI/BYca2Yt5/IC383whoyTGT/FBxWEKyjZmNmbcBbAsbfi1n3pRJM5w5bMDPw8YszXPsWe7Mw2zGlnMMknFrOZ6+TfJn2+H6Nvn3Hz/z1NzJ7Tve/PDGmwo7nFpAgJkHlQs2Co96IGD8gV9+FIyCUTAKRjoAAPLXYYGMJhK8AAAAAElFTkSuQmCC","orcid":"","institution":"The Second Hospital of Hebei Medical University","correspondingAuthor":true,"prefix":"","firstName":"Xianghua","middleName":"","lastName":"Huang","suffix":""}],"badges":[],"createdAt":"2025-11-27 16:38:24","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-8223990/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-8223990/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":99319183,"identity":"1b4dde3c-49c5-4a69-9782-3eddb5c28667","added_by":"auto","created_at":"2025-12-31 16:36:35","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":410271,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003ePelvic MRI and urinary tract CT findings in a patient with recurrent HWW syndrome.\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e(A) Coronal T2-weighted pelvic MRI showing uterus didelphys and a markedly distended right hemivagina consistent with hematocolpos. The left cervix and hemivagina remain unobstructed. A small amount of pelvic effusion is also visible; (B) Sagittal T2-weighted MRI demonstrating the longitudinal vaginal septum, complete obstruction of the right hemivagina with fluid accumulation, and the spatial relationship between the duplicated uterine horns and the obstructed vaginal cavity; (C) Axial contrast-enhanced CT of the urinary tract revealing complete right renal agenesis and compensatory hypertrophy of the left kidney. Mild bladder wall thickening and scant intraluminal gas are present, consistent with chronic urinary dysfunction.\u003c/p\u003e","description":"","filename":"Figure1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-8223990/v1/b38b03e4638970787015145e.jpg"},{"id":99224054,"identity":"1b85c7d2-aa53-4907-8e8f-39d458888024","added_by":"auto","created_at":"2025-12-30 10:03:23","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":1192031,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eHysteroscopic and vaginoscopic findings during Stage 1 surgery.\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e(A–B) Normal left cervical canal and external os; (C) Visualization of the thick longitudinal vaginal septum obstructing the right hemivagina; (D) Purulent debris adherent to the posterior cavity behind the septum, consistent with secondary infection; (E–F) Two small openings were identified at the dome of the posterior cavity: the superior ostium displayed a squamo–columnar junction consistent with the right cervical external os, whereas the inferior ostium communicated with an adjacent microchannel near the right cervix; (G) Magnified view of the right cervical external os; (H) Lower ostium located beneath the right cervix, connecting to the posterior cavity; (I) Appearance of the cavity after septal incision and decompression, demonstrating restored patency and removal of obstructing tissue.\u003c/p\u003e","description":"","filename":"Figure2.jpg","url":"https://assets-eu.researchsquare.com/files/rs-8223990/v1/741399d385a7da7359864d17.jpg"},{"id":99224051,"identity":"a1b026bc-0797-40af-8e67-d60795aeae1b","added_by":"auto","created_at":"2025-12-30 10:03:23","extension":"jpg","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":579348,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eSchematic illustration of the anatomical configuration and staged surgical strategy for recurrent obstructed hemivagina in HWW syndrome.\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e(A) Preoperative schematic demonstrating recurrent obstruction of the right hemivagina after two prior septal resections. A deeply recessed posterior cavity is present behind the longitudinal septum, positioned adjacent to the blind-ending accessory perineal tract (“accessory vagina”), with the two spaces lying in close anatomic proximity.\u003c/p\u003e\n\u003cp\u003e(B) Illustration of the first-stage procedure performed on 17 April 2025. A targeted incision was made along the right longitudinal septum, followed by trans-septal puncture through the accessory perineal tract and placement of a drainage catheter across the transverse barrier. This strategy aimed to decompress the posterior cavity, flatten the deep recess, and prevent re-accumulation of hematocolpos or purulent fluid.\u003c/p\u003e\n\u003cp\u003e(C) Illustration of the second-stage reconstruction performed on 12 May 2025. Definitive posterior-cavity obliteration was achieved using horizontal buttressing sutures, combined with inversion and excision (“up-rolling”) of the accessory tract and limited perineal reconstruction. This approach eliminated the residual dead space, restored normal genital tract geometry, and improved external vulvar appearance.\u003c/p\u003e","description":"","filename":"Figure3.jpg","url":"https://assets-eu.researchsquare.com/files/rs-8223990/v1/aac2a1cbef7149a152ebae7b.jpg"},{"id":99224053,"identity":"302c3cc2-1bcd-4286-b654-39f33ac5e515","added_by":"auto","created_at":"2025-12-30 10:03:23","extension":"jpg","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":361961,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eClassification of obstructed hemivagina based on the 2021 Chinese expert consensus.\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e(A) Type I: Imperforate oblique vaginal septum.\u003c/p\u003e\n\u003cp\u003eComplete obstruction of one hemivagina, with the obstructed uterine cavity isolated from the contralateral side and from the external genital tract.\u003c/p\u003e\n\u003cp\u003e(B) Type II: Oblique vaginal septum with a micro-perforation.\u003c/p\u003e\n\u003cp\u003eIncomplete obstruction with a small fistulous opening (a few millimeters) within the septum allowing limited drainage.\u003c/p\u003e\n\u003cp\u003e(C) Type III: Imperforate septum with cervical fistula.\u003c/p\u003e\n\u003cp\u003eComplete hemivaginal obstruction accompanied by a small fistulous tract between the two cervices or between the obstructed cavity and the contralateral cervical canal.\u003c/p\u003e\n\u003cp\u003e(D) Type IV: Cervical atresia type.\u003c/p\u003e\n\u003cp\u003eDysplastic or atretic cervix on the obstructed side, resulting in cervical-level obstruction of the ipsilateral Müllerian structures.\u003c/p\u003e","description":"","filename":"Figure4.jpg","url":"https://assets-eu.researchsquare.com/files/rs-8223990/v1/28071316f1b3d97043cc4eb2.jpg"},{"id":105041888,"identity":"08072a71-270b-404c-ab7f-ef48681d2442","added_by":"auto","created_at":"2026-03-20 07:57:41","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":3257715,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-8223990/v1/38fd2df4-666b-4e94-b80e-dbc5118c86df.pdf"},{"id":99224091,"identity":"d8125c85-74d1-4f27-8fe4-f880e4d4cb5b","added_by":"auto","created_at":"2025-12-30 10:03:25","extension":"mp4","order_by":0,"title":"","display":"","copyAsset":false,"role":"supplement","size":77296935,"visible":true,"origin":"","legend":"","description":"","filename":"file.mp4","url":"https://assets-eu.researchsquare.com/files/rs-8223990/v1/ecc745459e7b6ca322238077.mp4"}],"financialInterests":"No competing interests reported.","formattedTitle":"Staged Treatment of Recurrent Obstructed Hemivagina in Herlyn–Werner–Wunderlich syndrome with Double Vagina: A Case Report and Mini-Review","fulltext":[{"header":"1. Introduction","content":"\u003cp\u003eHerlyn\u0026ndash;Werner\u0026ndash;Wunderlich (HWW) syndrome\u0026mdash;also termed obstructed hemivagina and ipsilateral renal anomaly (OHVIRA)\u0026mdash;is a rare congenital M\u0026uuml;llerian malformation defined by the triad of uterus didelphys, an obstructed hemivagina, and ipsilateral renal agenesis (1\u0026ndash;3). Owing to the shared embryologic origin of the M\u0026uuml;llerian and Wolffian ducts, abnormalities frequently coexist, and the timing or degree of developmental arrest determines the wide clinical spectrum. Most patients present soon after menarche with cyclic pelvic pain, dysmenorrhea, pelvic mass, or abnormal discharge; delayed recognition may result in hematocolpos, hematometra, tubal distention, pelvic infection, endometriosis, and subsequent fertility impairment (1\u0026ndash;3). To improve diagnostic consistency and guide operative planning, structured classification systems have been developed. The ESHRE/ESGE 2013 consensus provides a reproducible anatomy-based framework for uterine, cervical, and vaginal anomalies (4), while the ASRM M\u0026uuml;llerian Anomalies Classification 2021 (MAC2021) incorporates clinically oriented subtypes and explicitly acknowledges associated urinary tract malformations (5).\u003c/p\u003e \u003cp\u003eImaging is central to diagnosis and surgical road-mapping. MRI remains the preferred modality in adolescents or non\u0026ndash;sexually active patients because it reliably defines the level of obstruction, the continuity between duplicated cavities, and associated renal or ureteral anomalies (1,2). Expert ultrasonography\u0026mdash;including transabdominal, transrectal, or selected 3D approaches\u0026mdash;serves as a valuable adjunct when MRI is unavailable or when real-time intraoperative guidance is required (2,6,7). Surgical treatment typically consists of transvaginal or endoscopic septal resection with wide marsupialization to prevent restenosis. Large case series demonstrate rapid symptom relief and generally favorable long-term reproductive outcomes; however, restenosis, persistent obstruction, or infection may occur in anatomically complex cases or after incomplete resection (6\u0026ndash;8).\u003c/p\u003e \u003cp\u003eRecent systematic reviews emphasize standardized preoperative mapping, careful application of classification systems, and individualized adjunctive measures\u0026mdash;such as temporary drainage, staged procedures, or targeted cavity remodeling\u0026mdash;to minimize re-obstruction and preserve fertility (8). Notably, the spectrum of HWW extends beyond the classic triad: variants involving ectopic or remnant ureteral segments, Gartner duct cysts communicating with the obstructed hemivagina, vesicovaginal or ureterovaginal fistulas, or distorted postoperative anatomy may obscure diagnosis and complicate surgical decision-making (9,10). Within this context, we present a recurrent HWW case with duplicated vaginas and repeated hemivaginal obstruction years after prior septal resections, managed successfully using MRI-guided mapping and a staged trans-septal reconstructive strategy.\u003c/p\u003e"},{"header":"2. Case presentation","content":"\u003cp\u003e\u003cstrong\u003e2.1 Case characteristics and clinical course\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eA 19-year-old nulligravid woman was admitted on 13 April 2025 with a one-month history of lower abdominal pain occurring after menstruation, accompanied by intermittent fever up to 38 \u0026deg;C. She had regular menses since menarche at age 12 (3\u0026ndash;4 days/30-day cycle, moderate flow) and reported dysmenorrhea only prior to earlier surgeries; her cycles were painless thereafter.\u003c/p\u003e\n\u003cp\u003eHer neonatal history was notable for surgical excision of a perineal mass at 1 month of age, with pathology confirming ectopic colonic tissue. Right renal agenesis and lifelong continuous urinary incontinence were documented in infancy. At age 9, cystoscopy revealed a small-capacity bladder with trabeculation, absence of the right ureteral orifice, hypoplastic urethral sphincter, and a valve-like mucosal fold adjacent to the right bladder neck.\u003c/p\u003e\n\u003cp\u003eAt age 12 (30 May 2017), six months after menarche, she presented with cyclic abdominal pain and fever. Imaging and examination confirmed a longitudinal vaginal septum consistent with Herlyn\u0026ndash;Werner\u0026ndash;Wunderlich (HWW) syndrome. She underwent ultrasound-guided hysteroscopy and transvaginal septum resection. Five months later (6 November 2017), recurrent pain prompted a second transvaginal resection, after which she remained asymptomatic for more than seven years.\u003c/p\u003e\n\u003cp\u003eOne month before the current admission, an abdominal ultrasound at a local clinic suggested renal malformation and a right lower abdominal mixed-echo mass. Subsequent CT demonstrated pelvic fat stranding with minimal effusion, nonvisualization of the right kidney, and irregular morphology of the solitary left kidney. Gynecologic ultrasound suggested a M\u0026uuml;llerian anomaly with suspected right-sided hematocolpos/hematosalpinx and pelvic effusion. Outpatient ultrasound at our institution similarly indicated congenital urogenital malformation with right-sided obstructed genital tract and prompted inpatient evaluation.\u003c/p\u003e\n\u003cp\u003ePhysical examination showed normal left labia, a well-healed perineal scar on the right, and an orthotopic urethral meatus with continuous leakage. Posterior to the hymenal ring at the 7\u0026ndash;8 o\u0026rsquo;clock position, a 1.5-cm depression was probed to a 3-cm blind end. Bimanual and rectal examinations revealed a normal left cervix and uterus and a right adnexal fullness; mobility was preserved. Laboratory tests, including complete blood count, biochemistry, and tumor markers, were within normal limits.\u003c/p\u003e\n\u003cp\u003ePelvic MRI demonstrated findings characteristic of recurrent HWW syndrome: uterus didelphys, complete obstruction of the right hemivagina with hematocolpos, a patent left cervix, small pelvic effusion, and suspected iliac lymphatic cysts (Figure 1A, B). CT of the urinary tract again confirmed right renal agenesis, compensatory hypertrophy with irregular contour of the left kidney, and mild bladder wall thickening with scant intravesical gas (Figure 1C). Dynamic renal scintigraphy showed complete absence of right renal function and compensatory hyperfunction of the left kidney.\u003c/p\u003e\n\u003cp\u003eA working diagnosis of recurrent obstructed right hemivagina following two prior septal resections, associated with complex double-vagina anatomy and congenital urinary tract malformations with persistent urinary incontinence, was established.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e2.2 Interventions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eExploratory mapping (15 April 2025).\u003c/p\u003e\n\u003cp\u003eA diagnostic vaginoscopy was performed to clarify the distorted anatomy following two prior septal resections. The left cervix appeared normal. At the 7\u0026ndash;8 o\u0026rsquo;clock position of the vaginal wall, a pinpoint opening led into a deep posterior cavity containing purulent, mucinous material. Due to the complexity of the obstructed tract and the need for comprehensive preoperative planning, this session was limited to anatomical mapping without further intervention.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eStage 1 \u0026ndash; Septal incision, trans-septal drainage, and balloon stenting (17 April 2025).\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eUnder combined vaginoscopy, hysteroscopy, and real-time transabdominal ultrasonography, the left cervical canal was first identified as normal (Figure 2A\u0026ndash;B). A thick right longitudinal vaginal septum was visualized obstructing the route toward the posterior cavity (Figure 2C). A targeted incision was then made along the septum, approximately 1.5 cm in thickness, allowing entry into the obstructed posterior compartment where purulent and mucinous debris was encountered (Figure 2D). Copious irrigation was performed.\u003c/p\u003e\n\u003cp\u003eAt the dome of the posterior cavity, two small ostia were identified (Figure 2E\u0026ndash;F). The superior ostium displayed a squamo-columnar junction consistent with the right external cervical os, while the inferior ostium communicated with an adjacent micro-channel. A magnified view of the right cervical os is shown in Figure 2G, and the lower accessory ostium beneath the right cervix is depicted in Figure 2H.\u003c/p\u003e\n\u003cp\u003eThe septal incision was further extended inferiorly to approximately 2 cm above the hymenal ring and leveled flush with the main vaginal lumen. Hemostasis was secured with bipolar coagulation. Because the accessory perineal tract (\u0026ldquo;right accessory vagina\u0026rdquo;) lay in close proximity to the posterior cavity floor yet remained a blind pouch externally, a deliberate trans-septal puncture was performed and a balloon catheter was positioned across the newly created channel (inflated with 18 mL saline) to allow drainage, equalize pressure, flatten the deeply recessed cavity, and reduce early re-closure. The final appearance following septal decompression is shown in Figure 2I.\u003c/p\u003e\n\u003cp\u003eThe updated intraoperative diagnosis included uterus didelphys, duplicated cervices and vaginas, recurrent obstruction of the right longitudinal septum, a right accessory transverse septum, urinary incontinence, and remote perineal ectopic-colon excision. A second-stage procedure was scheduled after the next menstrual cycle.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eStage 2 \u0026ndash; Posterior-cavity obliteration, accessory-tract inversion/excision, and perineal reconstruction (12 May 2025).\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eUnder general anesthesia in lithotomy position, the anatomic configuration of the recurrent obstructed right hemivagina and its communication with the accessory perineal tract, as illustrated in Figure 3A, was reassessed. The previously placed balloon catheter occupying both the accessory tract and posterior cavity was identified (Figure 3B). A circumferential submucosal hydrodissection plane was developed, permitting sharp excision of the lower two-thirds of the accessory tract. The proximal stump was then closed using a 3-0 absorbable purse-string suture. The suture tail was routed through the pre-existing trans-septal channel into the main (left) vaginal lumen; gentle traction inverted the proximal segment of the accessory tract into the main vaginal cavity, resulting in complete sealing of the tract.\u003c/p\u003e\n\u003cp\u003eTo eliminate the residual \u0026ldquo;dam effect\u0026rdquo; created by the deeply recessed posterior cavity, 2-0 absorbable sutures were passed from the main vagina through the septal window into the posterior cavity and then across the transverse tissue toward the accessory-tract wound, effectively flattening and buttressing the cavity floor. Additional vertical mattress sutures further reinforced closure of the accessory-tract bed. The final reconstructed anatomy, with complete obliteration of the posterior cavity and normalized perineal contour, is shown in Figure 3C. Blood loss was minimal; urine remained clear intraoperatively, and digital rectal examination confirmed preservation of rectal mucosal integrity.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eOutcomes and follow-up.\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003ePostoperative recovery was uneventful. Menstrual cycles subsequently remained regular, without dysmenorrhea, intermenstrual spotting, or clinical evidence of re-accumulation. The staged, MRI-guided, trans-septal strategy effectively resolved recurrent obstruction in this complex double-vagina variant of HWW syndrome and restored functional genital tract continuity.\u003c/p\u003e"},{"header":"3. Discussion","content":"\u003cp\u003eHerlyn\u0026ndash;Werner\u0026ndash;Wunderlich (HWW) syndrome represents a unique subgroup of obstructed M\u0026uuml;llerian anomalies characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal anomaly. Although the triad appears uniform, the syndrome encompasses substantial anatomic heterogeneity along both M\u0026uuml;llerian and Wolffian duct derivatives, explaining its highly variable clinical manifestations. Patients typically present soon after menarche with cyclic pelvic pain, dysmenorrhea, purulent discharge, malodorous secretions, or intermittent fever; delayed diagnosis may result in hematocolpos, hematometra, ascending infection, pelvic adhesions, and secondary endometriosis, all of which may adversely affect future fertility (1\u0026ndash;3).\u003c/p\u003e \u003cp\u003eTo standardize terminology and improve diagnostic precision, multiple classification systems have been developed. While the ESHRE/ESGE 2013 and ASRM MAC 2021 systems have improved communication and provided structured criteria for uterine, cervical, and vaginal anomalies (4,5), they do not fully capture the anatomical diversity within HWW syndrome. In China, the 2021 Expert Consensus further subdivides obstructed hemivagina into four clinically meaningful types\u0026mdash;imperforate septum (Type I), micro-perforated septum (Type II), imperforate septum with cervical fistula (Type III), and cervical atresia (Type IV)\u0026mdash;highlighting how subtle differences in septal configuration affect symptom onset, obstruction severity, and choice of operative strategy (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003e) (6). Building on these frameworks, the KUU\u0026prime;V system (kidney\u0026ndash;ureter\u0026ndash;uterus\u0026ndash;vaginal septum) proposed in 2022 integrates renal and ureteral anomalies with M\u0026uuml;llerian variants, reflecting the high coexistence of urinary tract defects in HWW syndrome (7).\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eClinical heterogeneity in HWW syndrome is closely linked to these anatomic variations. A recent large-scale study involving 1673 patients reported dysmenorrhea (53.8%), abnormal uterine bleeding (28.9%), and vaginal discharge (26.6%) as the most common symptoms; right-sided obstruction (57.3%), hematocolpos (81.7%), uterus didelphys (88.8%), and ipsilateral renal agenesis (93.1%) were the principal anomalies (8). Endometriosis was identified in approximately 9.6% of patients, with over half exhibiting ipsilateral endometriotic ovarian cysts. This aligns with the hypothesis that prolonged retrograde menstruation due to obstruction promotes endometriosis development (8,9).\u003c/p\u003e \u003cp\u003ePreoperative imaging plays a decisive role in defining obstruction level, identifying coexisting urinary tract anomalies, and avoiding false passages during surgery. Although ultrasonography is widely used as a first-line tool, MRI remains the most accurate modality for delineating uterine morphology, evaluating septal thickness, recognizing fistulous tracts, and identifying the precise geometry of the obstructed cavity (1,10). Consensus guidelines from ESHRE/ESGE recommend combining gynecologic examination with 2D ultrasonography as an initial evaluation and endorsing 3D ultrasonography or MRI as the diagnostic \u0026ldquo;reference standard\u0026rdquo; in complex cases (9). In this patient, MRI precisely mapped uterus didelphys, complete obstruction of the right hemivagina, and the shape and depth of the posterior compartment\u0026mdash;information essential for safe operative navigation.\u003c/p\u003e \u003cp\u003eThe present case underscores the limitations of relying exclusively on initial classification-based management. Despite two prior childhood septal resections, the patient experienced recurrent obstruction more than seven years later\u0026mdash;a delayed failure attributed to several possible factors: (1) an incompletely leveled or uneven septal edge functioning as a \u0026ldquo;valve,\u0026rdquo; predisposing to re-closure; (2) a deeply recessed posterior cavity acting as a persistent dead space conducive to fluid stasis, infection, and fibrosis; (3) an unrecognized accessory perineal tract functioning as a concealed reservoir for contamination; and (4) prior interventions performed amid active inflammation, increasing the risk of postoperative adhesions and scarring. These mechanisms have been corroborated by multiple studies reporting that recurrence often results not from inadequate septal width alone but from failure to manage adjacent comunicating spaces or accessory channels (6\u0026ndash;8,11,12).\u003c/p\u003e \u003cp\u003eGiven these challenges, a staged surgical approach was deliberately selected. The first stage\u0026mdash;MRI-guided vaginoscopy, targeted septal incision, evacuation of purulent material, and trans-septal balloon stenting\u0026mdash;allowed decompression of the infected cavity, flattening of the posterior recess, and stabilization of the mucosa before definitive reconstruction. Similar strategies have been recommended in obstructed M\u0026uuml;llerian anomalies where temporary stenting or marsupialization minimizes early restenosis (6\u0026ndash;8). The second stage addressed the underlying risk factors for late recurrence by obliterating the posterior dead space, inverting and excising the accessory perineal tract, and restoring normal vulvovaginal geometry. The complete elimination of alternative outflow pathways and correction of the \u0026ldquo;dam effect\u0026rdquo; are consistent with surgical principles described in recurrent HWW variants (11\u0026ndash;14).\u003c/p\u003e \u003cp\u003eLong-term reproductive outcomes are a central concern for young women with HWW syndrome. Although pregnancy rates after successful septal resection are favorable (approximately 72%), adverse obstetric outcomes remain more frequent than in the general population, including higher rates of miscarriage, preterm delivery, and cesarean section (8). Early diagnosis and timely intervention are therefore essential not only for symptom control but also for reducing the risk of endometriosis, preserving fertility, and preventing chronic pelvic adhesions (2,8,9). Psychosocial factors, including anxiety related to urinary incontinence or repeated pelvic surgeries, should not be overlooked. In this case, framing the surgical plan as \u0026ldquo;two smaller, safer steps\u0026rdquo; improved patient understanding and adherence while minimizing perioperative anxiety.\u003c/p\u003e \u003cp\u003eIn conclusion, this complex case of double-vagina HWW syndrome with late recurrent obstruction illustrates that successful management requires addressing not merely the septum but the interconnected system of obstructed and accessory spaces. MRI-based mapping, staged decompression, cavity obliteration, and elimination of accessory tracts provided durable symptomatic relief and restored normal menstrual function. For patients with infection, deep posterior cavities, thick septa, or associated urinary anomalies, a staged strategy should be strongly considered. Precision imaging and tailored operative planning remain indispensable to prevent re-obstruction, reduce morbidity, and preserve long-term reproductive potential.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eAcknowledgements\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis study was supported by the following funding sources: the Natural Science Foundation of Hebei Province, Project No. H2023206356; the Natural Science Foundation of Hebei Province, Project No. H2024206427; the Natural Science Foundation of Hebei Province, Project No. H2024206433; the Hebei Provincial Central Government-Guided Local Science and Technology Development Fund Project (Science and Technology Innovation Base Project), Project No. 236Z7756G; the 2024 Hebei Provincial Government-Sponsored Clinical Medicine Outstanding Talent Training Program, Project No. ZF2024040; and the 2025 Hebei Provincial Government-Sponsored Clinical Medicine Outstanding Talent Training Program, Project No. ZF2025102.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe datasets generated and/or analyzed during the current study are available from the corresponding author upon reasonable request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors’ contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eXueqian Miao and Zhongkang Li conceived the study, coordinated the project, and supervised the overall research design. Yiwen Dong, Yanling Xie, Yibin Liu and Yanan Li collected the clinical data and contributed to data curation. Yanfang Du and Li Meng assisted with methodological development and contributed to critical discussion of the manuscript. Zhongkang Li, Yibin Liu, and Xueqian Miao drafted the manuscript.\u003c/p\u003e\n\u003cp\u003eLi Meng and Xianghua Huang prepared and reviewed the figures. Xianghua Huang, Li Meng, and Yanfang Du critically revised and finalized the manuscript. All authors read and approved the final version of the manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable. This study is a retrospective case report and does not involve identifiable patient information.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003ePatient consent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent for publication was obtained from the patient and/or legal guardians.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare that they have no competing interests.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eDel Vescovo R, Battisti S, Di Paola V, et al. \u003cem\u003eHerlyn\u0026ndash;Werner\u0026ndash;Wunderlich syndrome: MRI findings and differential diagnosis.\u003c/em\u003e BMC Med Imaging. 2012;12:4.\u003c/li\u003e\n\u003cli\u003ePanaitescu AM, Peltecu G, Gică N. \u003cem\u003eHerlyn\u0026ndash;Werner\u0026ndash;Wunderlich syndrome: Case report and review of the literature.\u003c/em\u003e Diagnostics (Basel). 2022;12(10):2466.\u003c/li\u003e\n\u003cli\u003eWdowiarz K, Rajtar-Czyż M, Starczewski A. \u003cem\u003eDiagnosis and treatment of Herlyn\u0026ndash;Werner\u0026ndash;Wunderlich syndrome.\u003c/em\u003e Pol Przegl Chir. 2021;93(2):42-48.\u003c/li\u003e\n\u003cli\u003eGrimbizis GF, Gordts S, Di Spiezio Sardo A, et al. \u003cem\u003eESHRE/ESGE classification of female genital tract congenital anomalies.\u003c/em\u003e Hum Reprod. 2013;28(8):2032-2044.\u003c/li\u003e\n\u003cli\u003ePfeifer SM, Attaran M, Goldstein J, et al. \u003cem\u003eASRM M\u0026uuml;llerian Anomalies Classification 2021.\u003c/em\u003e Fertil Steril. 2021;116(5):1238-1252.\u003c/li\u003e\n\u003cli\u003eSmith NA, Laufer MR. \u003cem\u003eManagement and follow-up of obstructed hemivagina and ipsilateral renal anomaly.\u003c/em\u003e Fertil Steril. 2007;87(4):918-922.\u003c/li\u003e\n\u003cli\u003eGungor Ugurlucan F, Dural O, Yasa C, et al. \u003cem\u003eDiagnosis, management, and outcome of OHVIRA syndrome.\u003c/em\u003e Clin Imaging. 2020;59:172-178.\u003c/li\u003e\n\u003cli\u003eMoufawad G, Giannini A, D\u0026rsquo;Oria O, et al. \u003cem\u003eSystematic review of OHVIRA syndrome diagnosis and surgical management.\u003c/em\u003e Gynecol Minim Invasive Ther. 2023;12(3):123-129.\u003c/li\u003e\n\u003cli\u003eXu X, Tian Y, Zhou J, et al. \u003cem\u003eHWWS with unusual urinary anomalies: Case report and literature review.\u003c/em\u003e Heliyon. 2024;10:e33558.\u003c/li\u003e\n\u003cli\u003eYu JH, Lee SR, Choi H, et al. \u003cem\u003eComplex urinary anomalies in HWWS.\u003c/em\u003e Diagnostics. 2021;11(1):7.\u003c/li\u003e\n\u003cli\u003eGirardi FC, Aleixes SRJ, Atuati MA, et al. \u003cem\u003eDiagnosis and treatment of atypical HWWS: MRI and surgical insights.\u003c/em\u003e Int J Surg Case Rep. 2019;63:129-134.\u003c/li\u003e\n\u003cli\u003eDias JL, Jogo R. \u003cem\u003ePre- and post-surgical MRI and US findings in HWWS.\u003c/em\u003e Abdom Imaging. 2015;40:2667-2682.\u003c/li\u003e\n\u003cli\u003eTong J, Zhu L, Chen N, Lang J. \u003cem\u003eEndometriosis in HWWS.\u003c/em\u003e Fertil Steril. 2014;102(3):790-794.\u003c/li\u003e\n\u003cli\u003eCandiani M, Vercellini P, Ferrero-Caroggio C, et al. \u003cem\u003eLong-term outcomes of HWWS conservative surgery.\u003c/em\u003e Eur J Obstet Gynecol Reprod Biol. 2022;275:84-90.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Herlyn–Werner–Wunderlich syndrome, double vagina, longitudinal vaginal septum, recurrent obstructed hemivagina, staged trans-septal management","lastPublishedDoi":"10.21203/rs.3.rs-8223990/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-8223990/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground: \u003c/strong\u003eHerlyn–Werner–Wunderlich syndrome (HWWS) is a rare congenital Müllerian anomaly characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal malformation, and postoperative recurrence is even more uncommon. This report details a unique case of recurrent obstructed hemivagina in Herlyn-Werner-Wunderlich syndrome with double vagina, managed with a staged surgical approach.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCase presentation:\u003c/strong\u003e We report a 19-year-old woman with a history of two prior vaginal septum resections who presented with recurrent pelvic pain and fever. Imaging revealed uterine didelphys, duplicated cervices and vaginas, complete obstruction of the right hemivagina, and ipsilateral renal agenesis. Vaginoscopy demonstrated a deeply recessed recurrent cavity with purulent fluid, indicating persistent obstruction and local infection. Given the distorted postoperative anatomy, a staged surgical strategy was adopted: initial hysteroscopic decompression and septal incision under ultrasound guidance, followed by definitive reconstruction including resection of the accessory pseudo-vaginal tract, obliteration of the residual obstructed chamber, and vulvar repair. The patient recovered well, achieved regular painless menstruation, and experienced no recurrence during follow-up.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusions:\u003c/strong\u003e This case highlights a complex recurrent variant of HWWS and underscores the essential role of MRI and endoscopic assessment in delineating atypical anatomy. Individualized, stepwise surgical correction is critical for preventing restenosis, controlling infection, restoring normal genital tract continuity, and preserving future reproductive potential in patients with complicated or recurrent HWWS.\u003c/p\u003e","manuscriptTitle":"Staged Treatment of Recurrent Obstructed Hemivagina in Herlyn–Werner–Wunderlich syndrome with Double Vagina: A Case Report and Mini-Review","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-12-30 10:03:14","doi":"10.21203/rs.3.rs-8223990/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"2ff343a2-49bf-4630-a014-fbc18b9d20d8","owner":[],"postedDate":"December 30th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2026-03-20T07:56:27+00:00","versionOfRecord":[],"versionCreatedAt":"2025-12-30 10:03:14","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-8223990","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-8223990","identity":"rs-8223990","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}