Symptomatic prepubertal obstructed hemivagina and ipsilateral renal anomaly (OHVIRA)-spectrum anomalies: two case reports with individualized management and no recurrence during follow-up

Introduction

Background

OHVIRA syndrome, also known as Herlyn-Werner-Wunderlich syndrome, is a rare congenital malformation characterized by uterine didelphys, obstructed hemivagina, and an ipsilateral renal anomaly (1-3). Reported prevalence ranges from 0.1% to 3.8%, and a recent systematic review and meta-analysis found that most published cases originated from centres in Asia, Europe, and North America, with a pooled mean age at diagnosis of 16.36 years (4). Most patients are diagnosed after menarche, when obstructed menstrual outflow typically causes hematocolpos, pelvic pain, pelvic mass, or abnormal vaginal discharge (5-8). Ultrasound and MRI are central to diagnosis, and vaginal septectomy is generally sufficient to relieve obstruction in typical postmenarchal cases, with low reported rates of recurrent stenosis during follow-up (5,9-12).

Rationale and knowledge gap

With the routine use of prenatal ultrasonographic screening, congenital genitourinary malformations are increasingly recognized earlier in life (13). This broader age range has also made the clinical presentation of OHVIRA more heterogeneous, complicating diagnostic and management decisions (14). Asymptomatic prepubertal patients are generally managed conservatively because most can be relieved without invasive interventions, as hydrocolpos may resolve spontaneously (12). More recently, symptomatic prepubertal cases associated with ectopic ureteral opening into the genital tract have been described, often requiring combined gynecologic and urologic intervention (10,12,13,15-19). However, other causes of symptomatic retained fluid in prepubertal OHVIRA remain insufficiently characterized. In particular, the value of integrating imaging, fluid analysis, and follow-up findings to clarify fluid origin and guide individualized management has not been well described.

Objective

Our aim was to describe the diagnostic and management challenges of prepubertal symptomatic OHVIRA and related developmental anomalies, and to illustrate how integrated assessment of imaging, cyst fluid analysis, and follow-up findings may help distinguish ectopic ureter-related urinary accumulation from other causes of retained fluid, including uterovaginal infection, thereby guiding individualized management. We present this article in accordance with the CARE reporting checklist (available at https://gpm.amegroups.com/article/view/10.21037/gpm-25-51/rc).

Case presentation

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patients’ legal guardians for the publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Case 1

A 19-month-old girl was admitted for evaluation of a vaginal cyst first noted more than 1 year earlier. Her parents had observed a smooth cystic mass at the vaginal introitus that retracted at rest. Five months before admission, she had been treated for a febrile urinary tract infection with symptom resolution. Transabdominal ultrasound findings of the two cases are shown in Figure 1. In Case 1, pelvic ultrasonography performed 12 days before admission demonstrated a cystic lesion within the vaginal cavity (1.4 cm × 1.6 cm × 0.8 cm) and an elongated cystic lesion in the left pelvis (3.1 cm × 0.5 cm × 0.8 cm) that appeared to partially communicate with the vaginal lesion on some planes (Figure 1A,1B). No definite uterine anomaly was identified on imaging. Left renal agenesis was also noted. Three days after that, a pelvic MRI performed at an outside hospital (images unavailable) was reported to show two partially merged elongated cystic lesions in the vaginal and left pelvic regions. On admission, a 1.5 cm cystic mass was visible at the vaginal introitus. The patient had no significant medical history, no relevant family history of disease, and had not undergone genetic testing. Routine blood tests and serum tumor markers were within reference ranges. Given the combination of left renal agenesis and a vaginal/paravaginal cystic lesion, an OHVIRA-like developmental anomaly was suspected. Vaginoscopy was performed after written informed consent had been obtained. Intraoperatively, a cystic bulge arising from the left upper vaginal wall was identified (Figure 2), and a cervix-like structure was visualized at the vaginal apex. No definite obstructing hemivaginal septum was identified. Puncture of the cyst yielded 1.5 mL of pale yellow fluid. Analysis of the aspirated fluid showed markedly elevated creatinine (4,939 µmol/L; reference range, 17.3–54.6 µmol/L), suggesting a urinary component. Elevated levels of CA19-9 (>70,000 U/mL), CA125 (2,302 U/mL), and lactate dehydrogenase (859.9 U/mL) were also detected, whereas carcinoembryonic antigen remained within the reference range. To further evaluate a possible urinary contribution, urinalysis and CT urography (CTU) were performed. Urinalysis was normal. CTU confirmed left renal agenesis (Figure 3) and demonstrated a short linear hyperdense structure along the expected left ureteral course, suggesting a possible ureteric remnant, without hydronephrosis or ureteral dilatation. Since there were no obstructive symptoms, no additional intervention was undertaken. At the 3-month review and recent follow-up, no cyst enlargement, vaginal discharge, abdominal pain, or other discomfort was reported. The timeline of key clinical events in Case 1 is summarized in Table S1.

Figure 1 Transabdominal ultrasound findings of the two cases. (A) Color Doppler image in Case 1 showing a cystic lesion adjacent to the vagina. (B) Image in Case 1 showing an elongated cystic lesion in the left pelvis, with possible partial communication with the vaginal lesion. (C) Image in Case 2 showing a fluid-filled distended genital tract structure in the pelvis, extending caudally toward the vagina.

Figure 2 Vaginoscopic view of Case 1. A smooth-surfaced cystic mass, approximately 1.5 cm in diameter, is visible at the vaginal introitus, originating from the upper left lateral vaginal wall.

Figure 3 CTU of Case 1. The coronal view confirms left renal aplasia. The red arrows indicate the normal right kidney and the site of the absent left kidney, respectively. CTU, computed tomography urography.

Case 2

A 16-month-old girl was admitted with a 1-month history of intermittent vaginal discharge, described as yellowish or blood-tinged. Prenatal ultrasonography had suggested left renal agenesis, and she had been followed up regularly since birth. She had no surgical history, no relevant family history, and had not undergone genetic testing. Pelvic ultrasonography performed 35 days before admission showed uterine duplication, with the right uterus measuring 1.0 cm in anteroposterior diameter, and a fluid collection extending from the left uterine cavity into the vagina to 1.2 cm above the introitus (3.1 cm × 6.0 cm × 3.8 cm) (Figure 1C). Pelvic MRI performed 12 days before admission confirmed uterine didelphys with left-sided hydrometrocolpos (6.9 cm × 2.8 cm) and a blind-ending distal left vagina (Figure 4A,4B). A left paravaginal tubular structure measuring 0.5 cm in width was also identified, consistent with a possible ureteric remnant. Left renal agenesis with compensatory enlargement of the right kidney was noted, and the right ureter was unremarkable. CTU showed no contrast opacification of the tubular structure or the vaginal cavity (Figure 4C). Rectal examination identified a fluctuating mass 1.5 cm from the vaginal introitus. Laboratory testing showed leukocytosis (white blood cell count 17.4×10⁹/L). Serum tumor markers were largely unremarkable except for elevated CA125 (145.9 U/mL). Taken together, these findings were consistent with OHVIRA. The patient underwent vaginoscopy-guided drainage with partial septectomy, together with laparoscopy. Intraoperatively, a bulge was observed in the left vaginal wall (Figure 5A). Puncture yielded 20 mL of brownish-red turbid fluid. The septum was incised at the puncture site (2.0 cm) with partial excision of septal tissue (Figure 5B,5C), followed by drainage of a large volume of retained fluid. A dilated cervix-like structure was visualized at the top of the cavity (Figure 5D). Laparoscopy confirmed uterine didelphys with asymmetric uterine bodies (Figure 6). The surgical procedure was completed uneventfully. Analysis of the drained fluid showed markedly elevated CA19-9 (300.8 U/mL), CA125 (9,579 U/mL), lactate dehydrogenase (>4,500 U/mL), and carcinoembryonic antigen (60.3 ng/mL), whereas creatinine was not significantly elevated (6 µmol/L). Aerobic culture grew Escherichia coli. The patient received antibiotic therapy and was discharged after observation. At the 3-month review and recent follow-up, no recurrent discharge or other discomfort was reported. The timeline of key clinical events in Case 2 is summarized in Table S2.

Figure 4 Radiologic findings of Case 2. (A) Coronal pelvic MRI shows fluid distension of the uterine cavity. The cervix and vagina are cystically dilated, with the distal end of the vagina being blind-ended. A hypoplastic uterus is present on the right side. The white arrowhead indicates the duplicated uterine structures. (B) Sagittal pelvic MRI shows a cystically dilated vagina with fluid accumulation, indicated by the white arrowhead. (C) Axial CTU shows vaginal fluid collection and a possible ureteric remnant, indicated by the two white arrowheads. CTU, computed tomography urography; MRI, magnetic resonance imaging.

Figure 5 Intraoperative images from septal incision with partial excision in Case 2. (A) Bulging lesion of the left vaginal wall before incision. (B) White flocculent material visible within the lesion after puncture. (C) Under vaginoscopic guidance, the oblique vaginal septum was incised at the puncture site (approximately 2.0 cm) with partial excision of septal tissue. (D) A cervix-like structure was observed posterior to the septum.

Figure 6 Laparoscopic view in Case 2. Two uterine bodies are visualized. The left uterine body is larger with enlargement at the uterine horn, while the right uterine body is smaller and elongated. Each uterus is connected to its ipsilateral fallopian tube and ovary, which appear normal.

Discussion

Key findings

Our report highlights two main findings. First, the mechanisms underlying symptomatic presentation in prepubertal OHVIRA may be heterogeneous and are not necessarily attributable to ectopic ureter-related urinary accumulation. In Case 2, integrated assessment of imaging, cyst fluid analysis, and postoperative course supported infected retained secretions rather than a urinary source as the most likely explanation for the early symptoms. Second, these cases illustrate the diagnostic heterogeneity of prepubertal presentations associated with ipsilateral renal agenesis: whereas Case 2 represented classic OHVIRA, Case 1 was more consistent with an OHVIRA-like mesonephric/paramesonephric developmental anomaly. In both cases, fluid analysis contributed to etiologic interpretation when drainage was clinically indicated. Elevated cyst-fluid CA19-9, CA125, and LDH were also observed, underscoring that such abnormalities may occur in benign obstructive collections and should not be interpreted in isolation as evidence of malignancy (20).

Strengths and limitations

This report adds to the limited literature on symptomatic OHVIRA in early childhood and highlights that retained fluid in prepubertal patients may arise through heterogeneous mechanisms, including secondary infection. However, the diagnosis of OHVIRA in Case 1 could not be established with certainty. As the patient had no symptoms other than a vaginal mass, laparoscopy was not performed to identify potential uterine anomalies that may have been missed on imaging (21). Likewise, no tissue was resected for histologic confirmation of lesion origin. Etiologic confirmation of the retained fluid was also limited: although fluid biochemistry suggested urinary admixture, we did not directly demonstrate an ectopic ureteral insertion endoscopically, which has been reported as confirmatory (13). In Case 2, laparoscopy was performed despite informative MRI and provided limited additional diagnostic yield. Routine laparoscopy is not essential, and we did not perform dye-assisted techniques, which may help when anatomic clarification is needed (15).

Comparison with published reports

OHVIRA frequently coexists with ipsilateral renal anomalies, most commonly renal agenesis, consistent with its embryologic basis and supported by large series and systematic reviews (3,10,22). Most published patients are diagnosed after menarche, typically in late adolescence or adulthood (4,8,10). By contrast, prepubertal presentation is uncommon and appears more heterogeneous (10). Early symptomatic cases have been described in the neonatal or infant period, often identified during postnatal evaluation prompted by prenatal renal anomalies or by early imaging for hydrometrocolpos and related obstructive complications (7,23-26). In the existing literature, asymptomatic prepubertal patients are usually managed conservatively, as genital tract fluid collections may regress spontaneously during follow-up in the hormonally influenced early months of life (8,10,12,24). Among reported prepubertal cases requiring intervention, symptomatic retained fluid has often raised concern for ectopic ureteral opening into the genital tract, with some patients benefiting from combined gynecologic and urologic surgery (12,23-28). Our Case 2 differs from this pattern. Although imaging suggested a possible ureteric remnant, CTU showed no contrast opacification of either the tubular structure or the vaginal cavity. Cyst fluid creatinine was not elevated, and aerobic culture grew Escherichia coli. Together with sustained symptom resolution after drainage and antibiotic treatment, these findings support infected retained secretions rather than ectopic ureter-related urinary accumulation as the likely explanation for the early symptoms.

Similar to the diagnostic overlap between OHVIRA and Gartner duct cyst reported by Zhao et al. (21), Case 1 initially raised suspicion for OHVIRA because of ipsilateral renal agenesis and a vaginal/paravaginal cystic lesion. However, the absence of a definite uterine anomaly and a clear obstructing septum precluded definitive classification as OHVIRA. In the absence of direct evidence of ectopic ureteral insertion or histopathologic confirmation, Case 1 was considered more consistent with a mesonephric/paramesonephric developmental anomaly.

From the perspective of earlier recognition, prenatal clues also warrant attention. Previous reports suggest that prenatal suspicion of OHVIRA usually arises in late pregnancy, following detection of unilateral renal agenesis, particularly when associated with a fetal pelvic cystic mass (29). Prenatal ultrasound and MRI can raise suspicion but are highly operator-dependent. Recognition of these antenatal clues may facilitate earlier postnatal diagnosis and help prevent complications.

Interpretation of findings

The embryologic basis of OHVIRA has been attributed to abnormal development of the mesonephric duct, which plays an inductive role in the proper development, fusion, and septal resorption of the ipsilateral paramesonephric duct. Accordingly, absence or distal injury of one mesonephric duct may lead to ipsilateral renal agenesis, blind or atretic hemivagina, and associated uterine fusion or resorption defects (3,22,30). Anatomical variations associated with mesonephric and paramesonephric duct anomalies are highly heterogeneous (30,31). In Case 1, although left renal agenesis and a vaginal mass were present, evidence of a uterine anomaly was lacking. It is possible that the patient had a normal uterus, or that an existing anomaly was not diagnosed because the prepubertal uterus is small (12,21). However, given the patient’s young age and the absence of symptoms other than a vaginal mass, laparoscopy was not performed to further evaluate potential uterine anomalies (21). No definite oblique vaginal septum was identified and septectomy was not undertaken. Aspiration was performed for diagnostic and symptomatic relief. Fluid analysis suggested a urinary component. However, the absence of recurrence or urinary incontinence after the procedure, together with the lack of contrast opacification in the vaginal and paravaginal lesion on CTU, argued against ongoing urinary drainage and supported follow-up rather than urological intervention. Taking all findings together, Case 1 was considered more likely to represent a mesonephric/paramesonephric developmental anomaly than definitively classified OHVIRA (30).

In contrast, the diagnosis of OHVIRA in Case 2 was supported by preoperative imaging and intraoperative findings, and septectomy with drainage was indicated (32,33). In a recent literature review, only 2.2% of patients required repeat surgery (12), although long-term outcome data in prepubertal patients remain limited. Preoperative ultrasound, MRI, and CTU showed no communication between the urinary and genital tracts. Fluid analysis did not support a urinary source, and no recurrence of vaginal discharge or urinary incontinence was observed after septectomy. Although specialized investigations such as cystoscopy were not performed (16,19,21), the integrated assessment of previous findings and postoperative course argued against an ectopic ureteral opening into the genital tract and supported follow-up rather than urological intervention. The retained fluid was more likely to represent hormonally stimulated secretions with secondary infection (34), as suggested by the growth of Escherichia coli in the aspirate.

Implications for diagnosis and management

Asymptomatic prepubertal OHVIRA is usually managed conservatively (10). Once symptoms develop, further evaluation should focus not only on confirming the anatomic diagnosis but also on determining the cause of the symptoms. When drainage is indicated, integrated assessment, including fluid analysis, should be considered. In addition to the more commonly recognized ectopic ureter-related urinary accumulation, clinicians should also consider secondary infection of retained fluid, even in prepubertal patients. Based on these considerations, we propose a pragmatic diagnostic and management pathway for prepubertal patients with suspected OHVIRA-spectrum anomalies (Figure 7). Initial assessment should combine pelvic and renal ultrasonography, with additional imaging when needed. Prompt drainage and anti-infective treatment are indicated in patients with infection or obstruction, whereas those with uncertain anatomy or fluid origin may require further evaluation, including fluid analysis and selective endoscopic or laparoscopic assessment. Management should ultimately be individualized according to symptoms, underlying cause, and the presence or absence of infection, and careful follow-up remains essential.

Figure 7 Suggested diagnostic and management pathway for prepubertal patients with suspected OHVIRA-spectrum anomalies. CTU, computed tomography urography; MRI, magnetic resonance imaging; MRU, magnetic resonance urography; OHVIRA, obstructed hemivagina and ipsilateral renal anomaly.

Conclusions

Symptomatic prepubertal OHVIRA is uncommon and may present with abnormal vaginal discharge. Such cases are not always caused by ectopic ureter-related urinary accumulation; infection may also underlie retained fluid. Integrated assessment can help clarify fluid origin and support individualized management.

Acknowledgments

The authors extend their sincere gratitude to the patients and their parents for kindly providing their medical data. The authors also thank all the staff who contributed to the preparation of this manuscript. A preliminary version of this case report was presented orally at the 2025 Symposium on Adolescent Health and Medicine of the Sichuan Medical Doctor Association.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://gpm.amegroups.com/article/view/10.21037/gpm-25-51/rc

Peer Review File: Available at https://gpm.amegroups.com/article/view/10.21037/gpm-25-51/prf

Funding: This work was supported by the National Key R & D Program of China (grant No. 2021YFC2009100), and the Science & Technology of Sichuan (grant No. 2023YFS0158).

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://gpm.amegroups.com/article/view/10.21037/gpm-25-51/coif). P.W. and T.C. serves as an unpaid editorial board member of Gynecology and Pelvic Medicine from January 2026 to December 2026. L.M. serves as an unpaid editorial board member of Gynecology and Pelvic Medicine from March 2026 to February 2027. X.N. serves as an unpaid Executive Editor-in-Chief of Gynecology and Pelvic Medicine from May 2018 to May 2028. The other authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patients’ legal guardians for the publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

References

1. Herlyn U, Werner H. Simultaneous occurrence of an open Gartner-duct cyst, a homolateral aplasia of the kidney and a double uterus as a typical syndrome of abnormalities. Geburtshilfe Frauenheilkd 1971;31:340-7.
2. Wunderlich M. Unusual form of genital malformation with aplasia of the right kidney. Zentralbl Gynakol 1976;98:559-62.
3. Gruenwald P. The relation of the growing Müllerian duct to the Wolffian duct and its importance for the genesis of malformations. Anat Rec 1941;81:1-19.
4. Bonetti E, Anderson G, Duranti S, et al. Clinical features and surgical options of obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome: A systematic review and a meta-analysis of prevalence. Int J Gynaecol Obstet 2025;171:152-64. [Crossref] [PubMed]
5. Orazi C, Lucchetti MC, Schingo PM, et al. Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatr Radiol 2007;37:657-65.
6. Mandava A, Prabhakar RR, Smitha S. OHVIRA syndrome (obstructed hemivagina and ipsilateral renal anomaly) with uterus didelphys, an unusual presentation. J Pediatr Adolesc Gynecol 2012;25:e23-5. [Crossref] [PubMed]
7. Pansini L, Torricelli M, Gomarasca A, et al. Acute urinary retention due to didelphys uterus associated with an obstructed hemivagina in a 5-month-old infant. J Pediatr Surg 1988;23:984-5. [Crossref] [PubMed]
8. Zarfati A, Lucchetti MC. OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly or Herlyn-Werner-Wunderlich syndrome): Is it time for age-specific management? J Pediatr Surg 2022;57:696-701. [Crossref] [PubMed]
9. Abdelmoula G, Ragmoun H, Bezzine M, et al. A Complex Case Report of OHVIRA syndrome: Uterine didelphys, obstructed hemivagina, and renal agenesis. Radiol Case Rep 2025;20:5065-9. [Crossref] [PubMed]
10. Kudela G, Wiernik A, Drosdzol-Cop A, et al. Multiple variants of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome - one clinical center case series and the systematic review of 734 cases. J Pediatr Urol 2021;17:653.e1-653.e9. [Crossref] [PubMed]
11. Li L, Adeyemi-Fowode O, Bercaw-Pratt JL, et al. Surgical Management of OHVIRA and Outcomes. J Pediatr Adolesc Gynecol 2024;37:198-204. [Crossref] [PubMed]
12. Liu Y, Li Z, Dou Y, et al. Anatomical variations, treatment and outcomes of Herlyn-Werner-Wunderlich syndrome: a literature review of 1673 cases. Arch Gynecol Obstet 2023;308:1409-17. [Crossref] [PubMed]
13. Han JH, Lee YS, Im YJClinical Implications of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome in the Prepubertal Age Group, et al. PLoS One 2016;11:e0166776. [Crossref] [PubMed]
14. Kim YN, Han JH, Lee YS, et al. Comparison between prepubertal and postpubertal patients with obstructed hemivagina and ipsilateral renal anomaly syndrome. J Pediatr Urol 2021;17:652.e1-7.
15. Smith NA, Laufer MR. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: management and follow-up. Fertil Steril 2007;87:918-22. [Crossref] [PubMed]
16. Telecan T, Capras RD, Filip GA, et al. OHVIRA Syndrome and Ureteral Ectopy Draining in the Ipsilateral Hemiuterus, Diagnosed in the Prepubertal Age Group: Case-Report and Literature Review. Medicina (Kaunas) 2024;60:1922. [Crossref] [PubMed]
17. Nassar H, Horst M, Gobet R. A rare case of symptomatic hydrometrocolpos in a 5y old female. Urol Case Rep 2021;39:101789. [Crossref] [PubMed]
18. Nakahara Y, Nakada S, Hitomi K, et al. Urological anomalies associated with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, a case series. J Pediatr Surg Case Rep 2020;52:101358.
19. Nakamura M, Kanda S, Kajiho Y, et al. A case of right hypodysplastic kidney and ectopic ureter associated with bicornuate uterus in a prepubertal girl. CEN Case Rep 2023;12:122-9. [Crossref] [PubMed]
20. Sarandakou A, Phocas I, Botsis D, et al. Vaginal fluid and serum CEA, CA125 and SCC in normal conditions and in benign and malignant diseases of the genital tract. Acta Oncol 1997;36:755-9. [Crossref] [PubMed]
21. Zhao J, Bao Y, Gong L, et al. Case Report: Diagnostic overlap of OHVIRA syndrome and Gartner duct cyst: challenges in imaging and management. Front Pediatr 2025;13:1536314. [Crossref] [PubMed]
22. Acién P. Embryological observations on the female genital tract. Hum Reprod 1992;7:437-45. [Crossref] [PubMed]
23. Sijmons A, Broekhuizen S, van der Tuuk K, et al. OHVIRA syndrome: Early recognition prevents genitourinary complications. Ultrasound 2023;31:61-4. [Crossref] [PubMed]
24. Khanal L, Deora K, Gaihre SR, et al. Obstructed Hemivagina and Ipsilateral Renal Agenesis Syndrome in a Neonate. Cureus 2023;15:e36822. [Crossref] [PubMed]
25. Bloomfield V, Kives S, Allen L. Case Report: Neonate presenting with acute kidney injury secondary to obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: long-term follow-up. J Pediatr Adolesc Gynecol 2024;37:213-6. [Crossref] [PubMed]
26. Quintela C, Figueiredo AS, Dias A, et al. Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome: A Diagnosis in the First Year of Life. Cureus 2025;17:e77265. [Crossref] [PubMed]
27. Song Z, Tang D, Wu D, et al. Clinical characteristics of children with Herlyn-Werner-Wunderlich syndrome. Chin J Pediatr Surg 2019;40:456-9.
28. Liu W, Xie J, Xu Z. Congenital oblique vaginal septum in children. Chin J Pediatr Surg 2003;24:48-50.
29. Kim SJ, Shim SY, Cho HH, et al. Prenatal Diagnosis of Fetal Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA) Syndrome. Medicina (Kaunas) 2023;59:703. [Crossref] [PubMed]
30. Acién P, Navarro V, Acién M. Embryological-clinical classification of female genital tract malformations - a review and update. Reprod Biomed Online 2025;51:104751. [Crossref] [PubMed]
31. Jiang J, Yi S. Clinical features of 102 patients with different types of Herlyn-Werner-Wunderlich syndrome. Zhong Nan Da Xue Xue Bao Yi Xue Ban 2023;48:550-6. [Crossref] [PubMed]
32. Zhu S, Zhu Y. Diagnosis and treatment of oblique vaginal septum syndrome. J Int Obstet Gynecol 2017;44:262-4,270.
33. Gholoum S, Puligandla PS, Hui T, et al. Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome). J Pediatr Surg 2006;41:987-92. [Crossref] [PubMed]
34. SPENCER R. LEVY DM. Hydrometrocolpos: report of three cases and review of the literature. Ann Surg 1962;155:558-71. [Crossref] [PubMed]