Different interventions for OHVIRA/Herlyn-Werner-Wunderlich syndrome based on age and symptoms: a single-institution cohort study.

We retrospectively analyzed existing medical records of patients admitted to Oita University Hospital between April 2014 and March 2023. Data were extracted from obstetric and gynecological records of patients diagnosed with OHVIRA/HWW syndrome. This study adhered to the guidelines of the Declaration of Helsinki in conjunction with the prevailing ethical regulations. Written informed consent was obtained from all patients for publication. All cases were evaluated for uterine morphological abnormalities using magnetic resonance imaging (MRI), which showed duplicated uterus, duplicated vagina, and unilaterally obstructed vagina. Additionally, ipsilateral renal agenesis was confirmed by MRI, computed tomography, or ultrasonography. Patient background information, including the age of onset, age at diagnosis, age at menarche, chief complaint, history of pregnancy and delivery, medical history, family history, degree of dysmenorrhea, hematoma size, gynecologic complications, and communicating tract, was collected. The degree of dysmenorrhea was defined based on previous studies as the degree of daily life disturbance with menstrual pain during menstruation [ 8 , 9 ]. Patients were asked about the intensity of symptoms (“no symptoms/none,” “do not bother/mild,” “can be tolerated/moderate,” or “difficulties in daily life/severe”). Participants were divided into two groups: the non-severe group, which reported “no symptoms,” “do not bother,” or “can be tolerated,” and the severe group, which reported “difficulties in daily life.” Furthermore, the age at the time of intervention, method of intervention, and pathological diagnosis were recorded for cases where surgical intervention was performed. Statistical analysis was performed using SPSS version 25.0 (IBM Corp., Armonk, NY, USA). Clinical characteristics and interventions were examined using Pearson’s chi-square or Fisher’s exact probability test, whichever befitted the situation. Statistical significance was set at p  < 0.05.

During the study period, nine patients were diagnosed with OHVIRA/HWW syndrome (Table  1 ). They had an average age of 23.1 years, with a mean age at diagnosis of 20.3 years; six cases (67%) exhibited left-sided abnormalities, whereas three had right-sided abnormalities. OHVIRA syndrome was diagnosed in six cases (67%), and three were diagnosed with HWW syndrome. Four cases had severe dysmenorrhea. Four cases had dysmenorrhea as their chief complaint, while six cases had a chief complaint other than dysmenorrhea. Patients were categorized into two groups based on the timing of diagnosis: diagnosis before menarche (three cases) or diagnosis after menarche (six cases). Communicating tracts were detected in three cases, and therapeutic interventions (surgery or hormone therapy) were performed in five cases. Statistically, diagnosis after menarche and severe dysmenorrhea are positively correlated with therapeutic interventions ( p  = 0.048 and p  = 0.040, respectively) (Table  2 ). Other factors (OHVIRA/HWW syndrome, laterality of the anomaly, chief complaint excluding dysmenorrhea, hematoma size, and communicating tract) showed no correlations with therapeutic interventions. Notably, one patient developed symptoms following amenorrhea due to pregnancy and was diagnosed with HWW syndrome for the first time (Case 4). Treatment approaches varied among cases: three underwent traditional transvaginal septum resection, one received vaginoscopic surgery (Case 7), and one was treated with hormone therapy without surgery (Case 6). Detailed information on three notable cases (Cases 4, 6, and 7) is provided below. Table 1 Summary of the OHVIRA/HWW syndrome Case Diagnosis Laterality of anomaly Age at menarche Age at onset Age at diagnosis Chief Complaint Degree of dysmenorrhea Hematoma size (mm) Complication Communicating tract Intervention Management 1 OHVIRA Left 13 14 9 Dysmenorrhea Moderate 33 × 30 × 16 Hemosalpinx Not detected No Careful observation 2 OHVIRA Left 11 11 9 Dysmenorrhea, abdominal pain Mild None None Not detected No Careful observation 3 HWW Left 13 17 17 Dysmenorrhea, increased vaginal discharge Severe 37 × 30 × 25 None Not detected Yes Vaginal septum resection 4 HWW Right 13 28 28 Incomplete drainage of lochia, abdominal pain Severe 25 × 22 × 18 None Detected Yes Vaginal septum resection 5 OHVIRA Left 10 41 37 Abdominal pain, purulent vaginal discharge Severe 40 × 30 × 23 Myoma Not detected Yes Vaginal septum resection 6 OHVIRA Left 12 38 38 Abdominal pain, atypical genital bleeding Severe None Endometriosis, Adenomyosis, myoma Detected Yes Conservative treatment with dienogest 7 HWW Left 10 10 10 Dysmenorrhea Moderate 47 × 34 × 24 None Detected Yes Vaginoscopic vaginal septum resection 8 OHVIRA Right 12 26 26 Lower abdominal pain due to PID Moderate None PID Not detected No Careful observation (only antibacterial therapy) 9 OHVIRA Right 10 None 9 None None 39 × 36 × 31 None Not detected No Careful observation Abbreviations: OHVIRA, obstructed hemivagina and ipsilateral renal anomaly; HWW, Herlyn-Werner-Wunderlich Summary of the OHVIRA/HWW syndrome Conservative treatment with dienogest Vaginoscopic vaginal septum resection Lower abdominal pain due to PID Abbreviations: OHVIRA, obstructed hemivagina and ipsilateral renal anomaly; HWW, Herlyn-Werner-Wunderlich Table 2 Comparison of clinical characteristics and therapeutic interventions Valuables Intervention p -value No Yes Diagnosis OHVIRA 4 2 0.119 HWW 0 3 Laterality of anomaly Left 2 5 0.167 Right 2 0 Timing of diagnosis After menarche 1 5 0.048 before menarche 3 0 Degree of dysmenorrhea Non-severe 4 1 0.040 Severe 0 4 Chief Complaint exclude dysmenorrhea No 2 1 0.405 Yes 2 4 Hematoma size  3 cm 2 3 Complication No 2 3 0.643 Yes 2 2 Communicating tract Not detected 4 2 0.119 Detected 0 3 Abbreviations: OHVIRA, obstructed hemivagina and ipsilateral renal anomaly; HWW, Herlyn-Werner-Wunderlich Comparison of clinical characteristics and therapeutic interventions Abbreviations: OHVIRA, obstructed hemivagina and ipsilateral renal anomaly; HWW, Herlyn-Werner-Wunderlich The patient had been symptom-free since her menarche. She was diagnosed with a bicornuate uterus after a natural pregnancy, which occurred in the left uterus, and she also had right renal agenesis. The patient underwent an emergency cesarean section at 32 weeks of gestation due to non-reassuring fetal status. The infant, a male, weighed 1,150 g. During the surgery, an enlarged right uterus, approximately the size of a goose egg, was observed. Postoperative recovery was uneventful. MRI indicated a bicornuate uterus with a double cervix and right renal agenesis (Fig.  1 a and b). Fig. 1 Clinical images from Case 4 ( a–c ), Case 6 ( d–f ), and Case 7 ( g–i ). ( a, d, g ) Bicornuate uterus detected by pelvic MRI. ( b, e, h ) Left renal agenesis identified by abdominal MRI ( b, h ) and computed tomography scan ( e ). ( c, f, i ) Cervical to vaginal blood retention observed by pelvic MRI Abbreviations: MRI, magnetic resonance imaging Clinical images from Case 4 ( a–c ), Case 6 ( d–f ), and Case 7 ( g–i ). ( a, d, g ) Bicornuate uterus detected by pelvic MRI. ( b, e, h ) Left renal agenesis identified by abdominal MRI ( b, h ) and computed tomography scan ( e ). ( c, f, i ) Cervical to vaginal blood retention observed by pelvic MRI Abbreviations: MRI, magnetic resonance imaging Additionally, MRI revealed localized dilation in the right cervical area with retained blood components (Fig.  1 c). Approximately 11 months postpartum, the patient underwent vaginal septum excision surgery due to worsening abdominal pain. Pathological analysis of the excised tissue confirmed the presence of endocervical tissue, leading to a diagnosis of HWW syndrome. Her postoperative recovery was uneventful. The patient was diagnosed with a bicornuate uterus at 21 years old. At 38, she presented with abnormal genital bleeding and abdominal pain, leading to the discovery of a 6 cm uterine mass. MRI revealed a duplicated uterus, left vaginal closure, left renal agenesis, and uterine adenomyosis (Fig.  1 d and e). Although no significant communicating tracts were observed, there was evidence of left-sided vaginal fluid retention (Fig.  1 f). Additionally, a left ovarian endometriotic cyst (65 × 10 mm-sized) was detected, leading to the diagnosis of OHVIRA syndrome. Given her mild abdominal pain and concurrent conditions, such as endometriosis and adenomyosis, oral dienogest therapy was initiated. This treatment approach effectively managed her abdominal pain and menstrual discomfort for over eight years. The patient experienced severe dysmenorrhea shortly after her first menstruation at age nine, requiring early surgical intervention. Initially, she presented with the bicornuate uterus and a 2 cm-sized hematocolpos. Her condition worsened over three months, with the cyst enlarging to 5 cm. MRI revealed a duplicated uterus, left vaginal obstruction, left vaginal hematocolpos, and left renal agenesis (Fig.  1 g and i). Abnormal genital bleeding due to insufficient communicating tract formation, not large enough to drain the menstrual blood, necessitated surgery one year after menarche. A novel approach using vaginoscopy revealed a pinhole-like opening with brownish discharge on the left side of the right cervix. Vaginal septum excision was performed, and pathological examination confirmed the diagnosis of HWW syndrome. Her postoperative recovery was uneventful.

Herlyn-Werner-Wunderlich (HWW) syndrome was first described by Wunderlich in 1976 [ 1 , 2 ], and obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome was first reported in 1992 [ 3 ]. HWW syndrome is characterized by ipsilateral renal agenesis in a duplicated uterovaginal uterus with a unilateral obstructed cervix and cyst [ 1 , 2 ]. Conversely, OHVIRA syndrome includes not only ipsilateral renal agenesis but also conditions like multicystic dysplastic kidney and hypodysplastic kidney [ 3 ]. Although typically asymptomatic during childhood, OHVIRA/HWW syndrome becomes symptomatic after the onset of menstruation, causing menstrual difficulties and lower abdominal pain [ 1 – 3 ]. Management strategies for these syndromes are tailored based on age, symptom severity, obstructions, and communicating tracts [ 1 – 5 ]. Treatments for symptom relief involve addressing the point of obstruction, often achieved through excision of the vaginal septum. Although traditional vaginal surgery was previously common, recent years have seen an increased use of vaginoscopy, especially for younger patients [ 4 – 7 ]. Additionally, OHVIRA/HWW syndrome has been associated with pelvic inflammatory disease and endometriosis [ 1 – 3 ], necessitating both surgical intervention and long-term management to preserve future fertility [ 1 ]. Herein, we report the clinical characteristics and management of nine patients with OHVIRA/HWW syndrome, with varying onsets, diagnoses, and intervention timings at our facility.

This study reveals three novel findings. First, early diagnosis before menarche reduces the risk of therapeutic intervention, and severe dysmenorrhea is a risk factor for such interventions. To date, no studies have evaluated risk factors for therapeutic interventions through statistical analysis. Zarfati et al. report that two cases did not undergo surgical intervention, both of which were diagnosed before menarche in 26 cases of OHVIRA/HWW syndrome [ 10 ]. Lim et al. show that there are no correlations between age (10–19 vs. 20 and above) and surgical intervention in OHVIRA syndrome [ 11 ]. It is better to decide on intervention based on menarche rather than age, and surgical intervention can be postponed before menarche. Furthermore, if dysmenorrhea is not severe after menarche, surgical intervention can be avoided. Second, amenorrhea due to pregnancy may lead to the re-closure of an existing communicating tract, thereby aggravating the symptoms of OHVIRA/HWW syndrome. Case 4 is the first well-documented instance where symptoms appeared after amenorrhea during pregnancy, leading to a diagnosis of OHVIRA/HWW syndrome. Retrospectively, the patient was asymptomatic because menstrual blood was draining through the communicating tracts, which naturally dilated due to menorrhea. However, amenorrhea from pregnancy caused the communicating tracts to re-close. This phenomenon is similar to cervical stenosis observed after conization. Tanaka et al. [ 12 ] reported that conization within 12 months of childbirth is an independent risk factor for cervical stenosis. Additionally, Penna et al. [ 13 ] found that menopause is an independent predictor of cervical stenosis. These studies suggest that the lack of natural dilatation of communicating tracts by menstrual blood promotes the re-closure of natural or artificial (therapeutic) communicating tracts. Li et al. [ 14 ] suggested that vaginal stents or Foley balloons are useful in preventing re-stenosis. Third, we successfully performed two types of hymen-sparing treatments for OHVIRA/HWW syndrome: hormone therapy without surgery (Case 6) and vaginoscopic incision (Case 7). Hymen-sparing treatments can reduce both mental and physical pain [ 6 ] and may alleviate social distress in cultures that value hymenal integrity [ 4 , 5 ]. Case 6 demonstrated the potential for symptom control through minimally invasive treatment without surgery for incomplete atresia. The standard treatment for OHVIRA/HWW syndrome is transvaginal surgery to facilitate menstrual blood drainage [ 14 ]. Although hormone therapy has been suggested as a treatment option, such as using a gonadotropin-releasing hormone agonist/antagonist and hormonal contraception to postpone surgery [ 15 , 16 ], there are no previous reports of using dienogest as the primary treatment for OHVIRA/HWW syndrome. Dienogest is a viable option for patients with OHVIRA/HWW syndrome with incomplete obstruction, as it can control menstrual blood loss, pelvic pain, and endometriosis, which are often complications of this syndrome [ 1 – 3 ]. Vaginoscopic surgery was first reported for HWW syndrome in 2012 by Nassif et al. [ 7 ] and for OHVIRA syndrome in 2018 by Ludwin et al. [ 6 ]. Consistent with our findings, Cheng et al. [ 4 ] also performed vaginoscopic surgery on 14 patients with OHVIRA syndrome without intraoperative complications or postoperative re-stenosis. It was particularly found to be useful for patients with a restrictive vaginal opening or a narrow vaginal canal. This study has some limitations, such as a small number of cases, selection bias, and lack of long-term outcomes. Although OHVIRA/HWW syndrome commonly presents with right-sided abnormalities (approximately 60%) [ 1 , 3 ], 67% of the abnormalities observed in this study were on the left side, suggesting that uncommon cases were preferentially referred to our institution. However, this study provides useful information for patients and their physicians due to its relatively large number of cases, statistical analysis, and detailed individual information.

Early diagnosis before menarche reduces the risk of therapeutic intervention, and severe dysmenorrhea is a risk factor for such interventions. Amenorrhea due to pregnancy can result in the re-closure of a pre-existing communicating tract. Additionally, we successfully performed minimally invasive management, including hormone therapy without surgery and vaginoscopic incision.