OHVIRA Syndrome with Incomplete Vaginal Obstruction, Hematocolpos, and Pyocolpos Despite Regular Menstruation in an Adolescent: A Case Report

Abstract

Background

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare congenital Müllerian anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal anomaly. Patients with complete vaginal obstruction typically present shortly after menarche with severe dysmenorrhea and cyclic pelvic pain. Incomplete obstruction may permit partial menstrual drainage, resulting in atypical manifestations and delayed diagnosis. We report incomplete OHVIRA syndrome presenting with preserved menstrual flow despite hematocolpos and pyocolpos. The Zhu classification distinguishes complete obstruction (Class 1) from incomplete obstruction (Class 2), in which some menstrual outflow may occur despite persistent obstruction. In this case, findings supported incomplete obstruction, although a specific small septal opening was not documented.

Case Presentation

A 13-year-old postmenarchal girl was referred with persistent brown vaginal discharge following menstruation and recent right lower abdominal pain. She reported regular menstrual cycles for one year without dysmenorrhea. Examination revealed normal external genitalia with an intact hymen. Pelvic ultrasonography demonstrated uterus didelphys, hematocolpos, and non-visualization of the left kidney, raising suspicion of OHVIRA syndrome. Pelvic magnetic resonance imaging confirmed uterus didelphys with duplicated cervices and left-sided hematocolpos. According to the Zhu classification, the case was classified as Class 2 OHVIRA syndrome, indicating incomplete vaginal obstruction. According to the ESHRE/ESGE classification, the uterine, cervical, and vaginal anomalies were described as U3b-C2-V2, corresponding to a complete bicorporeal uterus, double cervix, and longitudinal obstructing vaginal septum, respectively. The patient underwent genitoscopy, transverse incision and complete excision of an oblique vaginal septum, drainage of retained collections, vaginal irrigation, and hymenorrhaphy. Approximately 50 mL of purulent material mixed with retained menstrual blood was evacuated, consistent with pyocolpos associated with chronic incomplete obstruction. Microbiological culture was not performed; therefore, the causative organism could not be identified. Cefazolin was administered preoperatively and postoperatively. Postoperative ultrasonography demonstrated complete resolution of the hematocolpos without residual pelvic fluid. During 6 months of telephone follow-up, the patient reported no recurrent symptoms; in-person reassessment was limited by school commitments and distance from the hospital.

Conclusion

Incomplete OHVIRA syndrome should be considered in postmenarchal adolescents with persistent postmenstrual brown vaginal discharge, even when menstruation is regular and dysmenorrhea is absent. Preserved menstrual flow does not exclude obstructive Müllerian anomalies. Pelvic ultrasonography, renal evaluation, magnetic resonance imaging, and endoscopic assessment are important for diagnosis and surgical planning. Early recognition and complete vaginal septum excision can relieve obstruction and treat retained collections; long-term reproductive outcomes cannot be determined from the available follow-up.

Introduction

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, also known as Herlyn–Werner–Wunderlich syndrome, is a rare congenital Müllerian anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal anomaly. Patients with complete vaginal obstruction typically present shortly after menarche with cyclic pelvic pain, severe dysmenorrhea, and hematocolpos. However, incomplete obstruction may permit partial menstrual drainage, resulting in atypical presentations and delayed diagnosis.

The Zhu classification further distinguishes OHVIRA syndrome according to the degree and pattern of vaginal obstruction. Class 1 represents complete vaginal obstruction, whereas Class 2 represents incomplete vaginal obstruction, in which partial menstrual outflow may occur. In the present case, the preserved regular menstrual flow despite accumulation within the left hemivagina was consistent with Class 2 OHVIRA syndrome. Using the ESHRE/ESGE classification, the associated Müllerian anomaly can be described as U3b-C2-V2, indicating a complete bicorporeal uterus, double cervix, and longitudinal obstructing vaginal septum, respectively.

Because menstrual cycles may remain regular, patients with incomplete OHVIRA syndrome often present with nonspecific symptoms, including persistent postmenstrual brown vaginal discharge, recurrent genital tract infection, or chronic pelvic pain. These atypical manifestations may delay diagnosis, increasing the risk of hematocolpos, pyocolpos, endometriosis, pelvic adhesions, and subsequent reproductive complications. Pelvic ultrasonography and magnetic resonance imaging (MRI) play complementary roles in confirming the diagnosis and guiding surgical management.

We report an adolescent with incomplete OHVIRA syndrome presenting with persistent postmenstrual brown vaginal discharge despite regular menstruation, who was subsequently diagnosed with hematocolpos and pyocolpos. Unlike the classic presentation characterized by severe dysmenorrhea and complete vaginal obstruction, our patient maintained regular menstruation despite progressive hematocolpos and secondary pyocolpos, illustrating a diagnostic pitfall of incomplete OHVIRA syndrome.

Case Presentation

A 13-year-old postmenarchal girl was referred to our tertiary referral hospital with suspected congenital genital tract anomaly after experiencing persistent brown vaginal discharge following menstruation and intermittent right lower abdominal pain. She had attained menarche one year earlier and reported regular menstrual cycles with normal menstrual duration and volume. Notably, she denied progressive dysmenorrhea, excessive menstrual bleeding, fever, urinary symptoms, or bowel complaints. The persistent postmenstrual brown vaginal discharge had gradually become her primary concern, prompting further evaluation.

The patient had no previous history of pelvic surgery, trauma, or known congenital anomalies. Physical examination revealed stable vital signs and normal secondary sexual characteristics. Abdominal examination demonstrated mild tenderness over the right lower quadrant without guarding or a palpable pelvic mass. External genital examination showed normal vulvar anatomy with an intact hymen and no obvious vaginal bulging or active discharge (Figure 1), making the diagnosis difficult based on clinical examination alone.

Figure 1.

External genital examination. External genital examination demonstrating normal vulvar anatomy with an intact hymen. No obvious vaginal bulging, external genital abnormalities, or active vaginal discharge were observed, despite the presence of an obstructed hemivagina.

Pelvic ultrasonography demonstrated uterus didelphys with a fluid-filled left hemivagina consistent with hematocolpos. In addition, the left kidney could not be visualized, raising suspicion of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome (Figure 2). Pelvic magnetic resonance imaging (MRI) further demonstrated uterus didelphys with two separate uterine bodies and duplicated cervices. The right cervix communicated normally with the vagina, whereas the left cervix was associated with a distended left hemivagina containing hyperintense fluid on both T1- and T2-weighted images, consistent with hematocolpos. No fistulous communication between the urinary bladder, vagina, and rectum was identified. In addition, no pelvic fluid collection or pelvic lymphadenopathy was observed. These findings confirmed the diagnosis of incomplete OHVIRA syndrome and provided detailed anatomical information for surgical planning (Figure 3). Based on the documented anatomical findings, the anomaly was classified as Class 2 OHVIRA syndrome according to the Zhu classification, indicating incomplete vaginal obstruction. According to the ESHRE/ESGE classification, the anomaly was described as U3b-C2-V2, representing a complete bicorporeal uterus, double cervix, and longitudinal obstructing vaginal septum, respectively.

Figure 2.

Pelvic ultrasonographic findings. Pelvic ultrasonography demonstrating uterus didelphys with a fluid-filled left hemivagina consistent with hematocolpos. The left kidney was not visualized, raising suspicion of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome.

Figure 3.

Pelvic magnetic resonance imaging findings. Pelvic magnetic resonance imaging demonstrating uterus didelphys with duplicated cervices and left-sided hematocolpos, providing detailed anatomical information for surgical planning.

The patient subsequently underwent genitoscopy under general anesthesia. Endoscopic evaluation demonstrated a right patent vaginal canal with a normal cervix and a bulging left lateral vaginal wall caused by an oblique vaginal septum. Following needle aspiration to confirm the retained collection, a transverse incision was made over the septum, resulting in evacuation of retained chocolate-colored fluid mixed with approximately 50 mL of purulent material, confirming hematocolpos complicated by pyocolpos. Complete excision of the obstructing vaginal septum was performed, followed by copious saline irrigation and approximation of the vaginal mucosa. Given the intact hymen at presentation, hymenorrhaphy was subsequently performed as an individualized additional procedure to preserve hymenal integrity (Figure 4).

Figure 4.

Genitoscopic appearance of the bulging vaginal septum. Genitoscopy demonstrated a patent right vaginal canal and a bulging left vaginal wall caused by the oblique vaginal septum.

The postoperative course was uneventful. The patient remained hemodynamically stable without fever, excessive pain, or postoperative bleeding. Follow-up pelvic ultrasonography demonstrated complete resolution of the hematocolpos with no residual pelvic fluid collection (Figure 5). She was discharged in good clinical condition and scheduled for outpatient follow-up to monitor vaginal patency, reproductive tract development, and renal function. A summary of the patient’s clinical course, diagnostic evaluation, surgical management, and postoperative follow-up is presented in Table 1.

Figure 5.

Postoperative pelvic ultrasonography. Follow-up ultrasonography demonstrated complete resolution of the hematocolpos without residual pelvic fluid collection.

Table 1.

Clinical Timeline

Time Point	Clinical Event
Approximately 1 year before presentation	Menarche; regular menstrual cycles without dysmenorrhea were reported.
Before diagnosis	Persistent postmenstrual brown vaginal discharge and intermittent/recent right lower abdominal pain.
Initial assessment	Examination showed normal external genitalia and an intact hymen. Ultrasonography demonstrated uterus didelphys, a fluid-filled left hemivagina, and non-visualization of the left kidney.
Further diagnostic assessment	MRI demonstrated duplicated uterine bodies and cervices and a distended left hemivagina containing blood-product-like material; no fistulous communication with the bladder or rectum was identified.
Surgical management	Genitoscopy showed a patent right vaginal canal and a bulging left vaginal wall due to an oblique septum. Incision and complete septal excision evacuated chocolate-colored fluid mixed with approximately 50 mL of purulent material, followed by irrigation and mucosal approximation.
Postoperative assessment	The postoperative course was uneventful. Ultrasonography showed resolution of hematocolpos without residual pelvic fluid.
6 months postoperatively	Telephone follow-up: no recurrent symptoms or new complaints were reported. In-person reassessment was not performed because of school commitments and distance from the hospital.

Discussion

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare congenital Müllerian anomaly characterized by uterus didelphys, obstructed hemivagina, and an associated ipsilateral renal anomaly. In this patient, the left kidney was not visualized on ultrasonography; renal agenesis was not definitively confirmed. Clinical manifestations usually become apparent shortly after menarche because complete obstruction of one hemivagina results in progressive hematocolpos, cyclic pelvic pain, and severe dysmenorrhea. Consequently, the diagnosis is often suspected when adolescent girls present with the characteristic combination of cyclic pelvic pain and a pelvic mass. Nevertheless, increasing evidence suggests that the clinical spectrum of OHVIRA syndrome is considerably broader than previously recognized, particularly in patients with incomplete vaginal obstruction, in whom menstrual outflow remains partially preserved and symptoms become less specific, resulting in delayed diagnosis. In the present case, the diagnosis of Zhu Class 2 OHVIRA syndrome is supported by the coexistence of a duplicated uterus and cervices with an incompletely obstructed left hemivagina, allowing sufficient menstrual outflow to maintain apparently regular menstruation. The ESHRE/ESGE descriptor U3b-C2-V2 further provides a standardized anatomical description of the complete bicorporeal uterus, double cervix, and longitudinal obstructing vaginal septum.^1–8

The most distinctive feature of our case is that preserved menstrual flow masked the obstructive anomaly, allowing progressive retention of menstrual blood until secondary infection developed. Unlike the classic presentation of complete vaginal obstruction, our patient maintained apparently regular menstruation despite the presence of hematocolpos and pyocolpos. This clinical feature highlights an important diagnostic pitfall: partial menstrual outflow does not necessarily indicate adequate drainage of the obstructed hemivagina or exclude the development of secondary infection. Akay et al reported a case of OHVIRA syndrome characterized by delayed diagnosis and a complicated clinical course. Although both cases illustrate the diagnostic and management challenges associated with OHVIRA syndrome, their clinical presentations and treatment courses differed. Our patient was a 13-year-old adolescent who presented with preserved menstrual flow and pyocolpos, whereas the case reported by Akay et al involved an adult patient with a complicated course that ultimately required hysterectomy. The occurrence of pyocolpos despite apparently regular menstruation is therefore a key distinguishing feature of our case. It underscores the importance of investigating persistent postmenstrual brown vaginal discharge in adolescents, particularly when accompanied by unilateral pelvic pain or findings suggestive of a Müllerian anomaly.^2,4,5,9–12

The embryological basis of OHVIRA syndrome explains the coexistence of genital and urinary tract anomalies observed in this patient. During normal embryogenesis, coordinated development of the Müllerian and mesonephric ducts is required for uterine fusion, vaginal canalization, and normal renal formation. Disruption of mesonephric duct development may impair ureteric bud formation and result in an ipsilateral renal anomaly, while simultaneously altering the position and fusion of the Müllerian ducts, leading to uterus didelphys and obstructed hemivagina.^6,7 In the present case, this developmental pattern is reflected by the combination of uterus didelphys, incomplete obstructing vaginal septum, and non-visualization of the left kidney on ultrasonography. The patient’s apparently regular menstruation despite progressive accumulation of retained blood suggests that menstrual outflow was at least partially preserved; however, the precise anatomical mechanism permitting this outflow was not definitively established.

Imaging played a pivotal role in establishing the diagnosis. Pelvic ultrasonography served as the initial modality by demonstrating uterus didelphys, hematocolpos, and non-visualization of the left kidney, findings that strongly suggested OHVIRA syndrome. Magnetic resonance imaging subsequently provided superior delineation of uterine duplication, duplicated cervices, and the configuration of the obstructing vaginal septum, thereby facilitating preoperative planning. In this patient, MRI was particularly valuable for preoperative planning because it delineated the duplicated uterine bodies and cervices, the distended left hemivagina, and the configuration of the oblique vaginal septum. This detailed anatomical assessment helped characterize the obstructed compartment and informed planning for surgical correction. Similar diagnostic pathways have been consistently recommended because ultrasonography is an excellent screening tool, whereas MRI remains the reference standard for comprehensive evaluation of Müllerian anomalies and associated urinary tract abnormalities.^1,3,5,11,12

Definitive treatment of symptomatic OHVIRA syndrome consists of surgical excision of the obstructing vaginal septum with adequate drainage of retained contents, aiming to relieve obstruction while preserving future reproductive function.^2,9,10,13,14 In our patient, complete septal excision resulted in evacuation of hematocolpos and approximately 50 mL of purulent material, confirming secondary pyocolpos caused by prolonged retention and ascending infection. Although hematocolpos is frequently reported, pyocolpos remains an uncommon complication that generally reflects delayed diagnosis.

Surgical management of OHVIRA syndrome aims to relieve the obstruction and establish adequate drainage of the affected hemivagina while maintaining vaginal patency. Both septal incision and complete excision have been described, and the choice of technique should be individualized according to the septal anatomy and intraoperative assessment. In the present case, the oblique vaginal septum was incised and completely excised, followed by drainage of the retained chocolate-colored fluid and purulent material and copious saline irrigation. Hymenorrhaphy was additionally performed based on the operating surgeon’s technical and clinical judgment. The family had been counseled regarding the procedure and available treatment options, and hymen-preserving vaginoscopic techniques were considered. Following septal excision, the vaginal mucosal edges were sutured and approximated to help maintain the patency of the surgically opened compartment. Although the patient reported no recurrent symptoms during six months of telephone follow-up, the absence of an in-person vaginal examination limits assessment for asymptomatic vaginal stenosis, adhesions, or re-obstruction.¹⁵

Vaginal septum resection is the most commonly reported surgical treatment for OHVIRA syndrome. In a systematic review and meta-analysis of 35 studies involving 526 patients, Bonetti et al reported vaginal septum resection in 83% of cases, supporting its role as the established primary surgical approach. Hysteroscopic septum resection was also reported as a minimally invasive alternative, although the available evidence was based on a smaller number of studies. In the present case, the obstructing septum was incised and completely excised, with drainage of the retained hematocolpos and purulent material followed by vaginal irrigation. The surgical approach should be individualized according to the anatomy of the septum, the degree of obstruction, associated complications, and the surgeon’s assessment.¹⁶

In addition to benign long-term sequelae, the obstructed or non-visible hemivagina and hemicervix may constitute an anatomical diagnostic blind spot, potentially limiting direct examination and delaying the recognition of pathology. Although exceptionally rare, gynecologic malignancies have been reported in association with OHVIRA/HWWS. A 2026 systematic review by Parisi et al identified reported cases of gynecologic malignancies involving patients with OHVIRA/HWWS, with tumors predominantly involving the cervix and vagina. Given the limited, case-based nature of the available evidence, these reports do not establish a causal relationship or permit reliable estimation of malignancy risk. Nevertheless, they highlight the importance of careful anatomical assessment and appropriate imaging of the affected compartments. Persistent postmenstrual discharge, even in patients with apparently regular menstruation, should therefore prompt further evaluation rather than be dismissed. Continued follow-up after septum resection is also important to assess vaginal patency, recurrent obstruction, and any persistent or new symptoms.¹⁷

This report has several limitations. First, although the clinical and imaging findings support incomplete vaginal obstruction, the precise anatomical mechanism permitting partial menstrual outflow was not definitively established, limiting further specification of the anatomical subtype. Second, microbiological culture of the purulent material was not performed; therefore, the causative organism could not be identified. Third, postoperative follow-up was limited to six months by telephone, and no in-person vaginal examination was performed to assess vaginal patency or exclude asymptomatic stenosis, adhesions, or recurrent obstruction. Early postoperative ultrasonography demonstrated resolution of the hematocolpos, providing evidence of anatomical improvement following surgery. However, preservation of reproductive anatomy should not be interpreted as evidence of preserved fertility, and the patient’s long-term reproductive outcomes remain unknown. Further clinical follow-up is warranted to assess vaginal patency, identify recurrent symptoms, and provide appropriate reproductive counseling as the patient matures.

This case therefore emphasizes that apparently normal menstruation does not exclude obstructive Müllerian anomalies. Recognition of persistent postmenstrual brown vaginal discharge, appropriate pelvic imaging, and timely intervention may help address obstruction and reduce the risk of further complications. This case does not provide evidence regarding long-term reproductive outcomes.

Conclusion

This case emphasizes that apparently normal menstruation does not exclude obstructive Müllerian anomalies. Persistent postmenstrual brown vaginal discharge, particularly when accompanied by unilateral pelvic pain, warrants careful evaluation and appropriate pelvic imaging. Timely intervention may relieve obstruction and reduce the risk of further complications. Structured long-term follow-up after septum resection is recommended to assess vaginal patency, detect possible stenosis or recurrent obstruction, monitor persistent or new symptoms, and address associated urinary tract abnormalities. Age-appropriate reproductive counseling should also be provided as the patient matures. Although early postoperative ultrasonography demonstrated resolution of the hematocolpos, this report does not establish long-term reproductive outcomes.

Funding Statement

This publication charge is funded by Unpad through the Indonesian Endowment Fund for Education (LPDP) on behalf of the Indonesian Ministry of Higher Education, Science and Technology and managed under the EQUITY Program (Contract No. 4303/B3/DT.03.08/2025 and 3927/UN6.RKT/HK.07.00/2025).

Abbreviations

MRI, Magnetic Resonance Imaging; OHVIRA, Obstructed Hemivagina and Ipsilateral Renal Anomaly; US, Ultrasonography.

Ethics Approval and Consent to Participate

This study was conducted in accordance with the Declaration of Helsinki. Ethical approval was not required for this case report in accordance with institutional policies.

Consent for Publication

Written informed consent for publication of the patient’s case details and accompanying images was obtained from her parent/legal guardian.

Disclosure

The authors report no conflicts of interest in this work.