Obstructed hemivagina and ipsilateral renal anomaly syndrome: a case series of 4 patients with insights into diagnosis and treatment strategies

Our first and second patients were 2 girls, aged 14 and 15 years, respectively. Both patients presented with signs of an acute abdomen. Menarche occurred approximately 1 year before presentation, and both patients had experienced regular menstrual cycles characterized by minimal bleeding in the form of spotting. They also reported progressive dysmenorrhea, a sensation of vaginal fullness, and pelvic pain, which intensified with each menstrual cycle. Abdominal and pelvic ultrasonography revealed right renal agenesis and an accumulation of hemorrhagic fluid in the proximal segment of the right vagina. Subsequent lower abdominal MRI demonstrated findings consistent with left-sided hematocolpos, hematometra, and hematosalpinx. Figure 1 presents the pelvic ultrasound findings. Figure 2 presents an axial pelvic MRI demonstrating a didelphic uterus with bilateral uterine horns and a distended, obstructed hemivagina containing hematocolpos. Figure 1 Pelvic magnetic resonance imaging showing a bicornuate uterus with a distended blind hemivagina, right renal agenesis, and compensatory left renal hypertrophy Figure 1 dummy alt text Mouiman. Obstructed hemivagina and ipsilateral renal anomaly syndrome. Am J Obstet Gynecol Glob Rep 2026. Figure 2 Axial pelvic magnetic resonance imaging demonstrating a didelphic uterus with bilateral uterine horns and a distended obstructed hemivagina containing hematocolpos Figure 2 dummy alt text Mouiman. Obstructed hemivagina and ipsilateral renal anomaly syndrome. Am J Obstet Gynecol Glob Rep 2026. Pelvic magnetic resonance imaging showing a bicornuate uterus with a distended blind hemivagina, right renal agenesis, and compensatory left renal hypertrophy Axial pelvic magnetic resonance imaging demonstrating a didelphic uterus with bilateral uterine horns and a distended obstructed hemivagina containing hematocolpos After obtaining informed consent, the patients underwent hymenotomy and resection of a left transverse vaginal septum under general anesthesia during menstruation. A transverse incision measuring approximately 0.5 to 1.0 cm was made, resulting in the drainage of 400 to 450 mL of coagulated blood from the uterus and vagina ( Figure 3 ). The incision site was marsupialized. Hymenal reconstruction was performed at the end of the procedure. The patients were discharged on postoperative day 2 without complications. No vertebral anomaly was identified on imaging in either patient. Figure 3 Hysteroscopic view of the blind hemivagina with hysteroscopic drainage of the associated hematocolpos Figure 3 dummy alt text Mouiman. Obstructed hemivagina and ipsilateral renal anomaly syndrome. Am J Obstet Gynecol Glob Rep 2026. Hysteroscopic view of the blind hemivagina with hysteroscopic drainage of the associated hematocolpos A follow-up examination was conducted 3 weeks after surgery. At that time, ultrasonography revealed normal uterine morphology and endometrial thickness in both uterine cavities. Our third patient was an 11-year-old girl who presented to the emergency department with severe dysmenorrhea associated with nausea and vomiting. On rectal examination, a tense mass approximately 10 cm in size was palpated in the upper-middle third of the vagina. Transrectal ultrasound revealed a didelphic uterus with a hematometra in the left cavity. Gynecologic examination under anesthesia revealed a complete vaginal septum on the left side with a fluctuating mass adjacent to it. The septum was incised, draining a substantial amount of dark, hemorrhagic material. Postoperative ultrasound images showed the left hemiuterus with a slightly opened cavity and contents flowing into the vagina. No vaginal collection or hematosalpinx was observed at follow-up. After 6 months, the patient attended a follow-up consultation and reported regular menstruation. No vertebral anomaly was identified on imaging. Our fourth patient was a 16-year-old girl with no significant medical history who presented to the hospital with severe cyclical pelvic pain since menarche that was unrelieved by standard analgesics. The history revealed intense dysmenorrhea and a recurrent sensation of pelvic heaviness without abnormal bleeding or urinary symptoms. Clinical examination revealed hypogastric tenderness, and digital rectal examination revealed a palpable pelvic mass. Pelvic ultrasound revealed a didelphic uterus with left-sided hematocolpos and ipsilateral renal agenesis. Pelvic MRI confirmed the presence of a didelphic uterus, an obstructed blind hemivagina on the left side causing hematocolpos, and a left renal agenesis, consistent with the diagnosis of OHVIRA syndrome (Herlyn-Werner-Wunderlich syndrome). Surgical resection of the obstructing vaginal septum was performed via a vaginal approach, and the postoperative course was uneventful ( Figure 4 ). The patient’s symptoms resolved, and follow-up imaging revealed regression of the hematocolpos. Ongoing gynecologic and nephrological monitoring was established. No vertebral anomaly was detected on imaging. Figure 4 Results after correction of the malformation as part of an obstructed hemivagina and ipsilateral renal anomaly syndrome Figure 4 dummy alt text Mouiman. Obstructed hemivagina and ipsilateral renal anomaly syndrome. Am J Obstet Gynecol Glob Rep 2026. Results after correction of the malformation as part of an obstructed hemivagina and ipsilateral renal anomaly syndrome

In this case, a fistula connects the 2 cervices of the obstructed vagina and the contralateral cervix. 4 In OHVIRA syndrome, the incidence of unilateral renal agenesis is described as 1:1000 live births. The association between renal anomalies and Müllerian anomalies is described as being between 30% and 70% and is explained by an interaction between the paramesonephric and mesonephric ducts during renal development. 5 A defective interaction is believed to be the cause of renal anomaly in OHVIRA syndrome. It was previously presumed that this leads to renal agenesis in patients with OHVIRA syndrome. However, there is increasing evidence that renal dysplasia leads to renal atrophy. The OHVIRA syndrome is a Wolffian-induced Müllerian anomaly. 5 Renal anomalies can be associated with an ectopic ureter connected to the ipsilateral obstructed hemivagina. This case can create diagnostic and therapeutic challenges if clinicians are not aware of this possibility. 6 The lack of prepubertal presentation is likely because the external genitalia appear normal, and there is a small prepubertal uterus, even with a mild degree of hydrocolpos, if any. 4 Patients usually present after menarche with progressive dysmenorrhea, lower abdominal pain, a paravaginal mass, foul mucopurulent discharge, and intermenstrual bleeding due to hemihematocolpos. Accurate identification is important because a delay in or lack of treatment may increase the risk of endometriosis, pelvic adhesions, and infertility. 7 The diagnosis of OHVIRA syndrome requires a multimodal approach, integrating the patient’s history; clinical presentation; imaging studies, including ultrasonography, computed tomography, and MRI; and direct visualization via hysteroscopy or laparoscopy. First, early and accurate diagnosis allows adequate and timely surgical treatment, providing symptom relief and preventing further complications. Second, whenever a multicystic-dysplastic kidney or the absence of a kidney in a fetus or newborn is detected, genital abnormalities should be looked for. 7 Ultrasound examinations allowed the correct diagnosis of uterovaginal duplication, hematocolpos, hematometrocolpos, and ipsilateral renal agenesis. However, MRI provided a more detailed description of uterine morphology and the continuity with each vagina, whether obstructed or nonobstructed. Ugurlucan et al. 8 presented a study comparing the MRI measurements of the distance from the hematocolpos to the perineum between patients who underwent single-stage vaginoplasty and those who underwent hemihysterectomy. 9 The authors support a therapeutic role of surgery in OHVIRA syndrome management and advise against its use for diagnosis. When the diagnosis is established, in the absence of symptoms or complications, patients should undergo both clinical and radiological follow-up until menarche. The frequency of radiological investigations may decrease after diagnostic confirmation and then increase before the onset of puberty. In the presence of symptoms or complications, surgery should be considered. In our experience, patients were diagnosed after menarche. In our opinion, the ideal time for surgery is when the vagina begins to fill but before hematometrocolpos develops. After surgery, long-term follow-up with periodic checks is needed to evaluate renal and gynecologic outcomes. In cases of preserved renal function, serum creatinine measurement, blood pressure monitoring, and renal ultrasound should be performed periodically. 3 In cases of impaired renal function or problems, a nephrological follow-up should be considered. From a gynecologic point of view, the presence of vaginal stenosis and endometriosis should be evaluated periodically. Patients with obstructive Müllerian anomalies have a higher risk of endometriosis due to retrograde menstruation (approximately 40%). 4 Some studies have shown a resolution of endometriosis after the treatment of OHVIRA. Given the possible hidden progression of endometriosis and its potential negative effect on fertility, it is important to consider laparoscopy and medical therapy in patients with persistent pelvic pain and/or dysmenorrhea. Common complications include postoperative adhesions, tearing of the vaginal wall, reformation of the obstruction, and vaginal stenosis. In our procedure, the septum is incised using a hysteroscope rather than being excised, as occurs during traditional surgery. Incision is preferred over excision because the latter may lead to scar formation and is more likely to be complicated by reobstruction and vaginal stenosis. 3

Of note, 4 female patients aged between 11 and 16 years, all diagnosed with OHVIRA syndrome, were included in this retrospective case series. The most common clinical presentation was progressive dysmenorrhea accompanied by a sensation of pelvic fullness or a vaginal mass, which typically occurred after menarche in 3 patients, and acute abdominal pain, which occurred in 2 patients. The median age of the patients was 14 years (interquartile range, 11–16). All patients were female. The main symptoms included severe dysmenorrhea (100%), pelvic mass or fullness (75%), nausea and vomiting (25%), and cyclical pelvic pain (100%). Menarche occurred approximately 1 year before presentation in 3 patients, who had irregular or scanty menstrual bleeding. Pelvic and abdominal ultrasounds revealed ipsilateral renal agenesis in all patients (100%). MRI confirmed the presence of a uterus didelphys with an obstructed left hemivagina in all cases. Hematocolpos and hematometra were identified in 3 patients. All patients underwent surgical management involving incision or resection of the obstructing vaginal septum under general anesthesia via a vaginal approach. In the most severe cases, the drainage volumes ranged from 400 to 450 mL of coagulated blood. No intraoperative complication was reported. Clinical and imaging follow-ups were conducted over a period of up to 6 months. The complete resolution of hematocolpos and restoration of normal uterine morphology were observed in all patients. Significant improvement in symptoms, including dysmenorrhea and pelvic pain, was noted. No case of obstruction recurrence or major postoperative complications was reported. Of note, 1 patient reported regular menstruation at 6 months postoperatively.

This case series was reported in accordance with the Preferred Reporting Of CasE Series in Surgery 2020 criteria. 3 We retrospectively reviewed the medical records of 4 female patients diagnosed with OHVIRA syndrome. The inclusion criteria were as follows: female patients with clinical and radiological features consistent with OHVIRA syndrome, confirmed by pelvic ultrasound and/or magnetic resonance imaging (MRI), and managed surgically. We collected demographic data, presenting symptoms, imaging findings, intraoperative findings, surgical techniques, postoperative courses, and short-term follow-up outcomes. All procedures were performed under general anesthesia using a surgical approach involving hymenotomy and hysteroscopic resection of the vaginal septum, with the specific technique adapted to each patient’s anatomic presentation. Postoperative follow-up included clinical examinations and imaging to assess the resolution of hematocolpos and uterine morphology and to monitor for complications or recurrence. Ethical approval for this study was granted by the ethics committee. Written informed consent for publication was obtained from the patients’ legal guardians. A copy of the consent form is available upon request.

Early diagnosis of OHVIRA syndrome relies primarily on a high index of clinical suspicion in menarchal-age females presenting with cyclical pelvic pain, dysmenorrhea, or a pelvic mass, supported by targeted radiological evaluation, including pelvic ultrasound and MRI. Surgical correction via hysteroscopic resection of the vaginal septum was effective in all 4 cases, with resolution of symptoms and no major complications observed during a follow-up period of up to 6 months. Regular follow-up is essential for monitoring renal function and gynecologic outcomes, although conclusions regarding long-term fertility preservation await data from extended follow-up.

In 1980, the triad of obstructed hemivagina, uterus didelphys, and ipsilateral renal anomaly was named the Herlyn-Werner-Wunderlich syndrome. The incidence of these disorders is estimated at 0.1% to 3.8%. The incidence of these disorders is separated into 3 categories (types I, II, and III). In this case, the uterine horn behind the septum has no connection to the outside or to the contralateral uterus, and menstrual blood accumulates in the cavity behind the vaginal septum. In this case, there is a pinpoint-sized hole in the septum through which a limited amount of menstrual blood drains out. The ipsilateral uterus is separate from the other horn.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome was first identified by Purslow in 1922 as a result of the aberrant embryologic development of the paramesonephric (Müllerian) and mesonephric (Wolffian) ducts. Under normal circumstances, the Wolffian ducts play a crucial role in the formation of Müllerian structures and are essential for renal development. 1 With growing awareness and significant advancements in imaging technologies, diagnoses have become increasingly frequent. The condition typically presents in postpubertal females with symptoms such as cyclical lower abdominal pain and abnormal vaginal discharge. There is no clear recommendation for the optimal time and method of surgical treatment for all patients. However, most published articles refer to the most minimally invasive option of vaginal septum resection. 2 We present 4 previously asymptomatic patients with OHVIRA syndrome who presented with acute abdomen shortly after menarche. These patients were treated with hysteroscopic resection of the vaginal septum.