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

A 19-year-old nulligravid woman was admitted on 13 April 2025 with a 1-month history of lower abdominal pain after menstruation, accompanied by intermittent fever up to 38.0°C. She had regular menstrual cycles since menarche at age 12 (3–4 days every 30 days, moderate flow) and reported dysmenorrhea only before her previous surgeries, with painless cycles thereafter. Her neonatal history was notable for excision of a perineal mass, with histopathology confirming ectopic colonic tissue. Right renal agenesis and lifelong continuous urinary incontinence had been documented since infancy. At 9 years of age, cystoscopy revealed a small-capacity bladder with trabeculation, absence of the right ureteral orifice, a hypoplastic urethral sphincter, and a valve-like mucosal fold adjacent to the right bladder neck. At age 12, 6 months after menarche, she underwent ultrasound-guided hysteroscopy with transvaginal septum resection for HWWS. Five months later, recurrent pain prompted a second transvaginal septum resection. She then remained free of gynecologic symptoms (pelvic pain, fever, abnormal discharge) for more than 7 years. However, her lifelong persistent urinary incontinence continued throughout this period. One month before the current admission, she again developed lower abdominal pain and fever. Imaging at local hospitals suggested a recurrent right-sided obstructed genital tract with pelvic inflammatory changes, nonvisualization of the right kidney, and pelvic effusion. On examination at our institution, a blind depression approximately 1.5 cm in diameter and 3 cm in depth was identified at the 7–8 o’clock position posterior to the hymenal ring. Rectovaginal examination revealed a normal left cervix and uterus with right adnexal fullness. Laboratory tests on admission showed a white blood cell (WBC) count of 7.85 × 10 9 /L (reference range: 3.50–9.50 × 10 9 /L), a neutrophil count of 3.67 × 10 9 /L (reference range: 1.80–6.30 × 10 9 /L), and an elevated lymphocyte count of 3.36 × 10 9 /L (reference range: 1.10–3.20 × 10 9 /L). C-reactive protein (CRP) was 3.37 mg/L, and procalcitonin (PCT) was <0.02 ng/mL. These findings indicated only mild systemic inflammatory activity despite the presence of purulent material in the obstructed hemivagina. Pelvic MRI demonstrated uterus didelphys, complete obstruction of the right hemivagina with fluid accumulation, a patent left cervix, and a small amount of pelvic effusion ( Figure 1A and B ). Urinary tract CT confirmed right renal agenesis, compensatory enlargement of the left kidney, and mild bladder wall thickening ( Figure 1C ). No right-sided ureterocele, ectopic ureter, or blind megaureter was observed. However, imaging could not fully exclude a left ectopic ureter or other lower urinary tract abnormalities. Dynamic renal scintigraphy (99mTc-DTPA) confirmed the absence of right renal function (GFR: 0 mL/min) and compensatory hyperfunction of the left kidney (GFR: 90.74 mL/min). Given the absence of right renal function, a right ectopic ureter was unable to produce urine and was therefore highly unlikely to be the cause of her persistent incontinence. Taken together, these findings indicate that her lifelong urinary incontinence was most likely attributable to congenital lower urinary tract abnormalities. However, a left ectopic ureter had not been fully excluded. The working diagnosis was recurrent obstruction of the right hemivagina after two prior septum resections in a patient with complex duplicated vaginal anatomy, uterus didelphys, ipsilateral renal agenesis, and persistent urinary incontinence. No formal urology consultation was obtained during this admission. According to the patient’s history, she had planned further evaluation in Beijing for urinary incontinence, which might require staged urologic treatment. Figure 1 Pelvic MRI and urinary tract CT findings in a patient with recurrent HWWS. ( A ) Coronal T2-weighted pelvic MRI showing uterus didelphys and marked fluid distension of the right hemivagina, consistent with obstructed hemivagina. The left cervix and left hemivagina are patent. A small amount of pelvic effusion is also visible. ( B ) Sagittal T2-weighted pelvic 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 urinary tract CT showing right renal agenesis and compensatory enlargement of the left kidney. Mild bladder wall thickening and scant intravesical gas are also present. Three-panel radiology figure of pelvis and abdomen highlighting a fluid-filled pelvic cavity and single kidney. Pelvic MRI and urinary tract CT findings in a patient with recurrent HWWS. ( A ) Coronal T2-weighted pelvic MRI showing uterus didelphys and marked fluid distension of the right hemivagina, consistent with obstructed hemivagina. The left cervix and left hemivagina are patent. A small amount of pelvic effusion is also visible. ( B ) Sagittal T2-weighted pelvic 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 urinary tract CT showing right renal agenesis and compensatory enlargement of the left kidney. Mild bladder wall thickening and scant intravesical gas are also present. Because prior surgery had markedly distorted the anatomy, diagnostic vaginoscopy was first performed for anatomical reassessment only. The left cervix appeared normal. A pinpoint opening at the 7–8 o’clock position led into a deeply recessed cavity containing purulent mucinous material, suggesting persistent obstruction with secondary infection. No definitive reconstruction was attempted at this stage. Under combined vaginoscopic, hysteroscopic, and real-time transabdominal ultrasound guidance, the left cervical canal was confirmed to be normal ( Figure 2A and B ). A thick right longitudinal vaginal septum was then incised, allowing entry into the obstructed posterior cavity, from which purulent and mucinous material was drained and irrigated ( Figure 2C and D ). Two small ostia were identified at the dome of the cavity: the superior opening was consistent with the right external cervical os, whereas the inferior opening communicated with an adjacent microchannel ( Figure 2E-H ). The septal incision was extended inferiorly and leveled flush with the main vaginal lumen ( Figure 2I ). Figure 2 Hysteroscopic and vaginoscopic findings during the first-stage procedure. ( A and B ) Normal left cervical canal and external OS. ( C ) Thick longitudinal vaginal septum obstructing the right hemivagina. ( D ) Purulent debris within the posterior cavity behind the septum, indicating secondary infection. ( E and F ) Two small openings identified at the dome of the posterior cavity: the superior ostium showed a squamo-columnar junction consistent with the right external cervical OS, whereas the inferior ostium communicated with an adjacent microchannel near the right cervix. ( G ) Magnified view of the right external cervical OS. ( H ) Inferior accessory ostium located beneath the right cervix and communicating with the posterior cavity. ( I ) Appearance after septal incision and decompression, showing restoration of drainage and removal of the obstructing tissue. The figure consists of 9 sub-parts. Parts A and B demonstrate an unremarkable left cervical canal together with a normally configured external cervical os. Part C reveals a markedly thickened longitudinal vaginal septum that completely blocks the right hemivagina. Part D shows purulent material and debris collected within the cavity located posterior to the septum, a finding suggestive of a superimposed infection. Parts E and F document two distinct small apertures at the roof of the posterior cavity. The upper aperture displays a squamo-columnar junction, identifying it as the right external cervical os. The lower aperture, in contrast, opens into a narrow adjacent microchannel situated close to the right cervix. Part G provides an enlarged view of the right external cervical os. Part H illustrates an inferior accessory ostium positioned below the right cervix, which communicates with the posterior cavity. Part I shows the surgical field following septal incision and decompression, demonstrating re-established drainage and complete removal of the obstructing tissue. Nine-part composite of hysteroscopic and vaginoscopic views labeled A through I, showing cervical orifices, vaginal septum, purulent debris, accessory ostia, and decompression after septal incision. Hysteroscopic and vaginoscopic findings during the first-stage procedure. ( A and B ) Normal left cervical canal and external OS. ( C ) Thick longitudinal vaginal septum obstructing the right hemivagina. ( D ) Purulent debris within the posterior cavity behind the septum, indicating secondary infection. ( E and F ) Two small openings identified at the dome of the posterior cavity: the superior ostium showed a squamo-columnar junction consistent with the right external cervical OS, whereas the inferior ostium communicated with an adjacent microchannel near the right cervix. ( G ) Magnified view of the right external cervical OS. ( H ) Inferior accessory ostium located beneath the right cervix and communicating with the posterior cavity. ( I ) Appearance after septal incision and decompression, showing restoration of drainage and removal of the obstructing tissue. Because a blind accessory perineal tract lay close to the floor of the posterior cavity, a deliberate trans-septal puncture was created under direct visualization and ultrasound guidance, and a 14-Fr Foley catheter was placed across the channel to maintain drainage and reduce early re-closure. The balloon was inflated with 18 mL saline and left in place for 25 days. To minimize bladder or rectal injury and false passage formation, all key steps were performed under direct endoscopic visualization and ultrasound guidance. No intraoperative injury occurred. The intraoperative diagnosis was revised to uterus didelphys with duplicated cervices and vaginas, recurrent obstruction of the right hemivagina after prior septum resections, a right accessory blind tract/transverse septum, persistent urinary incontinence, and previous perineal ectopic-colon excision. A second-stage procedure was scheduled after the next menstrual cycle. Under general anesthesia, the relationship between the recurrently obstructed right hemivagina and the accessory blind tract was reassessed ( Figure 3A and B ). The previously placed balloon catheter was identified and removed. The distal two-thirds of the accessory tract was excised after circumferential submucosal dissection, and the proximal stump was closed with a 3–0 absorbable purse-string suture. The proximal segment was then inverted inward through the pre-existing channel into the main vaginal lumen to seal the tract. Figure 3 Schematic illustration of the anatomical configuration and staged surgical management of recurrent obstructed hemivagina in HWWS. ( A ) Preoperative schematic showing recurrent obstruction of the right hemivagina after two prior septum resections. A deeply recessed posterior cavity is present behind the longitudinal septum and lies adjacent to a blind-ending accessory perineal tract, with the two spaces in close anatomical proximity. ( B ) Schematic of the first-stage procedure performed on 17 April 2025. The right longitudinal vaginal septum was incised, followed by trans-septal puncture through the accessory blind tract and placement of a drainage catheter across the transverse barrier. This approach was intended to decompress the posterior cavity, flatten the deep recess, and prevent re-accumulation of fluid or purulent material. ( C ) Schematic of the second-stage reconstruction performed on 12 May 2025. Definitive obliteration of the posterior cavity was achieved using horizontal buttressing sutures, combined with inversion and partial excision of the accessory blind tract and limited perineal reconstruction. This approach eliminated the residual dead space, restored genital tract anatomy, and improved perineal appearance. Three-panel diagram of the uterus, hemivagina, and perineum before surgery, during trans-septal catheter drainage, and after definitive repair in recurrent obstructed hemivagina in HWWS. Schematic illustration of the anatomical configuration and staged surgical management of recurrent obstructed hemivagina in HWWS. ( A ) Preoperative schematic showing recurrent obstruction of the right hemivagina after two prior septum resections. A deeply recessed posterior cavity is present behind the longitudinal septum and lies adjacent to a blind-ending accessory perineal tract, with the two spaces in close anatomical proximity. ( B ) Schematic of the first-stage procedure performed on 17 April 2025. The right longitudinal vaginal septum was incised, followed by trans-septal puncture through the accessory blind tract and placement of a drainage catheter across the transverse barrier. This approach was intended to decompress the posterior cavity, flatten the deep recess, and prevent re-accumulation of fluid or purulent material. ( C ) Schematic of the second-stage reconstruction performed on 12 May 2025. Definitive obliteration of the posterior cavity was achieved using horizontal buttressing sutures, combined with inversion and partial excision of the accessory blind tract and limited perineal reconstruction. This approach eliminated the residual dead space, restored genital tract anatomy, and improved perineal appearance. To eliminate the residual recessed cavity, 2–0 absorbable sutures were placed from the main vaginal lumen through the septal window into the posterior cavity and across the transverse tissue toward the accessory-tract wound, thereby flattening and obliterating the residual space. Additional vertical mattress sutures reinforced closure. The final reconstructed anatomy is shown in Figure 3C . Blood loss was minimal, urine remained clear, and rectal examination confirmed intact rectal mucosa. The postoperative course was uneventful. The patient subsequently resumed regular menstruation without dysmenorrhea, intermenstrual spotting, or recurrent pelvic pain. At 10 months after the second-stage surgery, follow-up by gynecologic examination and ultrasonography confirmed no re-accumulation, recurrent obstruction, or pelvic infection. Urinary incontinence showed no obvious improvement postoperatively.

Herlyn–Werner–Wunderlich syndrome (HWWS), also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare congenital malformation of the Müllerian and mesonephric duct systems, classically characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis or other renal anomalies. 1–3 Owing to the shared embryologic origin of the Müllerian and Wolffian ducts, these anomalies frequently coexist, producing a broad spectrum of genital and urinary tract malformations. 1 , 2 Most patients become symptomatic soon after menarche, with cyclic pelvic pain, dysmenorrhea, abnormal vaginal discharge, hematocolpos, or pelvic infection; delayed diagnosis may lead to retrograde menstruation, endometriosis, pelvic adhesions, and impaired reproductive outcomes. 1–4 Although adolescent presentation is typical, a minority of patients are diagnosed only during pregnancy or after a previous pregnancy, underscoring the marked anatomical heterogeneity of the syndrome. 5 , 6 Accurate anatomical delineation is essential for diagnosis and operative planning. Magnetic resonance imaging (MRI) is particularly valuable because it defines uterine morphology, the level and extent of vaginal obstruction, the relationship between duplicated cavities, and associated renal or ureteral anomalies, while ultrasonography remains an important complementary tool, for initial evaluation and real-time intraoperative guidance. 1 , 2 , 4 , 7 Structured classification systems, including the ESHRE/ESGE consensus and the ASRM Müllerian Anomalies Classification 2021 (MAC2021), improve diagnostic consistency and communication. 8 , 9 However, cases with unusual communications, associated urinary tract defects, or distorted postoperative anatomy may still be difficult to classify completely and require individualized interpretation. 8 , 9 The standard treatment for symptomatic HWWS is vaginal septum excision or marsupialization to establish unobstructed drainage, relieve symptoms, and preserve reproductive function. 4 , 7 In most reported series, timely surgery achieves satisfactory symptom control and generally favorable fertility outcomes. 2–4 , 7 Nevertheless, persistent obstruction, infection, or re-obstruction may occur in anatomically complex cases, particularly when the obstructed cavity is deeply recessed, the septum is thick or incompletely leveled, or associated accessory channels are overlooked. 2–4 , 7 A recent literature review of 1673 cases highlighted the substantial anatomical variability of HWWS and the need for tailored surgical planning rather than a uniform operative strategy. 10 Within this context, recurrent postoperative obstruction represents an uncommon but clinically important scenario, because prior surgery may distort tissue planes, obscure the true anatomy, and increase the risk of false passage formation, incomplete drainage, and restenosis. Here, we report a complex recurrent case of HWWS with duplicated vagina and repeated right hemivaginal obstruction more than 7 years after two prior septal resections, successfully managed using MRI-based anatomical mapping, endoscopic reassessment, and staged reconstructive surgery.

Herlyn–Werner–Wunderlich syndrome (HWWS) is a rare obstructive Müllerian anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal anomaly. Although the classical triad is well recognized, the anatomical spectrum is broad, and symptom severity depends on the degree of obstruction, the presence of partial communication, and associated urinary tract malformations. Most patients present soon after menarche with cyclic pelvic pain, dysmenorrhea, abnormal vaginal discharge, hematocolpos, or pelvic infection; delayed diagnosis may predispose to retrograde menstruation, endometriosis, pelvic adhesions, and impaired reproductive outcomes. 1–3 , 5 , 6 Recently, Güneş et al reported an adolescent case of OHVIRA syndrome with uterus didelphys, right hematometrocolpos, and ipsilateral renal agenesis that was successfully managed by simple vaginal septum incision and drainage. 11 In contrast, the present case illustrates a far more complex scenario—late recurrence after two prior septal resections, duplicated vaginal anatomy, and an accessory blind tract—requiring staged reconstructive surgery rather than simple repeat incision. From an embryological perspective, HWWS is a mesonephric (Wolffian) duct anomaly, and the Embryological-Clinical Classification proposed by Acién et al classifies unilateral renal agenesis with ipsilateral blin hemivagina under Class 2 (mesonephric or Wolffian anomalies), providing a more fundamental framework than purely anatomical systems. 12 On this basis, accurate morphological classification in clinical practice remains important for diagnosis, communication, and surgical planning. The present case is best described as a complete bicorporeal uterus with double cervix and obstructing longitudinal vaginal septum. According to the ESHRE/ESGE system, this corresponds most closely to U3bC2V2. 8 In the ASRM Müllerian Anomalies Classification 2021 (MAC2021), however, descriptive terminology may be more practical than code-based labeling in complex postoperative cases; thus, this patient can be described as having uterus didelphys, duplicated cervices, obstructed right hemivagina, and ipsilateral renal agenesis. 9 The classification of HWWS has evolved from the two-type system based on complete vs incomplete obstruction proposed by Zhu et al in 2015 13 to the 2021 Chinese expert consensus, which further categorizes obstructed hemivagina into four types based on the anatomical features of the vaginal septum and the presence of fistulous communications or cervical atresia ( Figure 4 ). 14 Recently, Dou et al conducted a nationwide multicenter retrospective study of 415 patients with OHVIRA syndrome, confirming that the four-subtype classification covers most anatomic variants. They also identified a previously unreported subtype (provisionally designated subtype V), characterized by unilateral cervical atresia with a fistula between the two cervixes and incomplete obstruction. 15 In our patient, repeated previous surgery and the presence of an accessory blind tract made the anatomy more complex than the classical triad alone would suggest. This illustrates that formal classification is useful for communication, but individualized interpretation based on imaging and endoscopic findings remains essential in recurrent or anatomically distorted cases. 8 , 9 Figure 4 Classification of obstructed hemivagina according to the 2021 Chinese expert consensus. ( A ) Type A: imperforate oblique vaginal septum. Complete obstruction of one hemivagina, with the obstructed uterine cavity isolated from both the contralateral side and the external genital tract. ( B ) Type B: oblique vaginal septum with microperforation. Incomplete obstruction with a small fistulous opening within the septum, allowing limited drainage. ( C ) Type C: imperforate septum with cervical fistula. Complete hemivaginal obstruction accompanied by a small fistulous tract between the two cervices or between the obstructed cavity and the contralateral cervical canal. ( D ) Type D: cervical atresia type. Dysplastic or atretic cervix on the obstructed side, resulting in cervical-level obstruction of the ipsilateral Müllerian structures. Illustrations of four types of obstructed hemivagina: Type A, B, C and D. Classification of obstructed hemivagina according to the 2021 Chinese expert consensus. ( A ) Type A: imperforate oblique vaginal septum. Complete obstruction of one hemivagina, with the obstructed uterine cavity isolated from both the contralateral side and the external genital tract. ( B ) Type B: oblique vaginal septum with microperforation. Incomplete obstruction with a small fistulous opening within the septum, allowing limited drainage. ( C ) Type C: imperforate septum with cervical fistula. Complete hemivaginal obstruction accompanied by a small fistulous tract between the two cervices or between the obstructed cavity and the contralateral cervical canal. ( D ) Type D: cervical atresia type. Dysplastic or atretic cervix on the obstructed side, resulting in cervical-level obstruction of the ipsilateral Müllerian structures. The present case also highlights that late recurrence after prior septum resection cannot always be explained by inadequate septal opening alone. In this patient, re-obstruction developed more than 7 years after two prior procedures. Based on the intraoperative findings, several mechanisms were likely involved: first, an uneven residual septal edge may have predisposed to re-closure; second, the deeply recessed posterior cavity created a poorly drained residual space that favored fluid stasis and infection; and third, the accessory blind perineal tract likely acted as a concealed communicating compartment. Taken together, failure in this case appears to have resulted from incomplete elimination of all obstructed or communicating spaces rather than from simple luminal narrowing alone. This interpretation is consistent with previous reports emphasizing the marked anatomical heterogeneity of HWWS and the importance of addressing adjacent obstructed compartments during surgical planning. 2 , 3 , 5 , 10 Accurate preoperative and intraoperative anatomical mapping was therefore central to management. MRI is particularly valuable in HWWS because it delineates uterine morphology, the site and extent of obstruction, the relationship between duplicated cavities, and associated urinary tract anomalies. 1 , 2 Ultrasound is also highly useful, both as an initial imaging modality and as a real-time intraoperative guide. 2 , 3 , 10 In our patient, MRI identified uterus didelphys and right hemivaginal obstruction, whereas vaginoscopy and hysteroscopy clarified the geometry of the residual posterior cavity and its relationship to the accessory blind tract. This combined approach was especially important in a previously operated field, where safe identification of the true lumen and avoidance of false passage formation were critical. It should be noted that laparoscopy was not performed in this case. Given the patient’s history of two prior surgeries and severely distorted anatomy, we selected vaginoscopy combined with hysteroscopy and real-time ultrasound guidance for anatomical reassessment, which allowed safe identification of the true lumen and avoided the risks of adhesiolysis in a previously operated field. Although laparoscopy can be useful for assessing pelvic adhesions, the tissue planes in this patient had been markedly altered, and its resolution of the fine anatomy of the vaginal septum and recessed cavity is inferior to that of transvaginal endoscopy combined with ultrasound. A staged surgical strategy was selected because the patient presented not only with recurrent obstruction, but also with infection, a deep residual cavity, and altered tissue planes after prior surgery. The first stage achieved decompression, drainage, and temporary stenting with a 14-Fr Foley catheter left in place for 25 days, thereby reducing inflammatory burden and helping flatten the residual recess before definitive repair. The second stage focused on obliteration of the residual pocket and elimination of the accessory blind tract rather than simple repeat septal incision. In this respect, the key principle was not merely to enlarge the opening, but to remove the structural basis for recurrent stasis and re-obstruction. The absence of re-accumulation on gynecologic examination and ultrasonography during 10 months of follow-up supports the effectiveness of this stepwise strategy. Long-term reproductive outcome remains an important concern in young women with HWWS. Reported pregnancy rates after successful septal resection are favorable, at approximately 72%, although miscarriage, preterm birth, and cesarean delivery remain more frequent than in the general population. 5 Accordingly, early diagnosis and durable restoration of unobstructed genital tract outflow are important not only for symptom relief, but also for preservation of future reproductive potential. The urologic aspect of this syndrome should also not be overlooked. In this patient with lifelong persistent urinary incontinence and ipsilateral renal agenesis, the nonfunctioning right kidney makes a right ectopic ureter or ureterocele unlikely to account for the continuous leakage. Her incontinence is therefore more likely related to lower urinary tract abnormalities, such as urethral sphincter hypoplasia, bladder neck incompetence, or a small-capacity bladder. However, a left ectopic ureter and other lower urinary tract abnormalities have not been fully excluded. No formal urologic consultation was obtained during this admission, and the incontinence showed no obvious improvement postoperatively. After the genital tract obstruction and residual cavity have been managed, the patient will be referred to the urology department for urodynamic studies, cystourethroscopy, and imaging to establish a definitive diagnosis and guide further treatment. In selected patients with persistent urinary symptoms or complex mesonephric anomalies, staged urologic assessment should therefore remain part of long-term multidisciplinary management. The history of perineal ectopic colonic tissue is rare and its embryological relationship to the Müllerian and mesonephric anomalies in this patient remains uncertain. It may reflect a broader developmental field defect involving the cloaca, but this cannot be confirmed from the available data. If rectal symptoms develop or a broader anorectal malformation is suspected, dedicated rectal and endoscopic assessment should be considered. In conclusion, this case of recurrent HWWS with duplicated vagina demonstrates that successful treatment depends on addressing not only the obstructing septum, but also the entire system of residual recesses and accessory spaces. Although the successful outcome of a single case cannot establish the efficacy or superiority of the proposed staged approach, it does suggest its feasibility and provides a clinically useful reference for anatomically complex or recurrent cases. MRI-based mapping, endoscopic reassessment, and staged reconstruction provided durable symptom relief and restoration of normal menstruation in an anatomically complex postoperative setting. For patients with infection, deep residual cavities, recurrent obstruction, or associated urinary anomalies, individualized staged surgery should be considered.