What is the triad of Herlyn‑Werner‑Wunderlich (Obstructed Hemivagina and Ipsilateral Renal Anomaly) syndrome?

Medical Advisory BoardAll articles are reviewed for accuracy by our Medical Advisory Board
Educational purpose only • Exercise caution as content is pending human review
Article Review Status
Submitted
Under Review
Approved

Last updated: February 25, 2026 • View editorial policy

Personalize

Help us tailor your experience

Which best describes you? Your choice helps us use language that's most understandable for you.

Herlyn-Werner-Wunderlich Syndrome (OHVIRA Syndrome)

Herlyn-Werner-Wunderlich syndrome consists of the triad: uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. 1, 2, 3

Clinical Definition

The syndrome, also known as OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly), represents a rare congenital malformation resulting from maldevelopment of both Müllerian and Wolffian ducts. 1, 3 The three defining components are:

  • Uterus didelphys (complete duplication of the uterus with two separate uterine cavities) 1, 2, 4
  • Obstructed hemivagina (blockage of one side of a duplicated vagina, typically by a vaginal septum) 1, 3, 5
  • Ipsilateral renal agenesis (absence of the kidney on the same side as the obstructed hemivagina) 1, 2, 3

Typical Clinical Presentation

Patients characteristically present after menarche with cyclic pelvic pain, dysmenorrhea, and palpable pelvic mass due to hematocolpos (blood accumulation in the obstructed hemivagina). 1, 3, 4 The obstructed hemivagina prevents menstrual blood drainage from one uterine cavity, leading to progressive accumulation and symptoms. 2, 5

Additional presenting features include:

  • Severe cyclical abdominal pain that worsens with each menstrual cycle 5
  • Purulent vaginal discharge (pyocolpos) if secondary infection develops 2
  • Dyspareunia (painful intercourse) 2
  • Palpable cystic mass on vaginal examination 2, 4

Atypical presentations can occur, including diagnosis in multiparous women, though this is extremely rare. 1 One case reported acute urinary retention as the presenting complaint. 1

Diagnostic Approach

MRI is the definitive imaging modality for confirming the diagnosis and characterizing all three components of the syndrome. 6, 2, 4 MRI provides superior visualization of the duplicated uterus, vaginal septum location, and confirms renal agenesis. 2, 3

Initial evaluation should include:

  • Pelvic ultrasound as first-line imaging, which can identify uterine didelphys, fluid accumulation in the hemivagina, and absent kidney 6, 2
  • Vaginal examination revealing a bulging vaginal wall with possible pinpoint opening and purulent or bloody discharge 2
  • MRI pelvis to definitively characterize the vaginal septum, extent of hematocolpos/pyocolpos, and confirm uterine and renal anatomy 2, 3, 4

Management

Surgical resection of the vaginal septum with drainage of hematocolpos is the definitive treatment and should be performed promptly to relieve symptoms and prevent complications. 2, 3, 4 The primary goal is to create drainage for the obstructed hemivagina and restore normal menstrual flow. 3, 5

Surgical approach:

  • Vaginoplasty with excision of the vaginal septum is the standard procedure 3, 4
  • Hysteroscopy-assisted vaginal septum resection with laparoscopic guidance provides minimally invasive treatment with excellent visualization 2, 4
  • Drainage of hematocolpos and pyocolpos at the time of septum resection 2, 5
  • Placement of intracervical mold may be considered to prevent re-obstruction 2

A critical pitfall is delayed diagnosis, which can lead to recurrent pyocolpos, endometriosis from retrograde menstruation, pelvic adhesions, and potential infertility. 3, 4 Early surgical intervention prevents these long-term complications and preserves fertility. 4

Renal ultrasound should be performed in any patient with uterine didelphys to screen for ipsilateral renal agenesis, as this association defines the syndrome. 6, 2 Approximately 15% of patients with complex Müllerian anomalies have genitourinary abnormalities. 7

References

3
Research

Imaging Diagnosis of Herlyn-Werner-Wunderlich Syndrome- An Extremely Rare Urogenital Anomaly.

Journal of clinical and diagnostic research : JCDR, 2015

5
Research

Herlyn Werner Wunderlich Syndrome with Hematocolpos Symptom.

Open access Macedonian journal of medical sciences, 2019

6
Guideline

Diagnostic Approach and Management of Müllerian Anomalies

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2025

7
Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Related Questions

What is the proposed mechanism linking obstructive Müllerian anomalies to endometriosis?
What is the appropriate work‑up and treatment for a 29‑year‑old with a 10‑year history of intermittent pruritus of the groin and inner thighs unresponsive to topical tioconazole?
In a 41‑year‑old woman with a hysterectomy and intact ovaries, does a follicle‑stimulating hormone (FSH) of 5.4 mIU/mL, luteinizing hormone (LH) of 7.7 mIU/mL, and estradiol of 147 pg/mL indicate preserved ovarian function and premenopausal status, and is hormone therapy indicated?
How should I manage a patient whose quetiapine (Seroquel) was increased from 200 mg to 400 mg at bedtime and who was started on aripiprazole (Abilify) 10 mg, now presenting with new hallucinations, agitation, and irritability?
What is the appropriate erythromycin prophylaxis regimen (dose and duration) for close contacts of a pertussis case, including alternatives for intolerance?
What is the recommended management for an 89‑year‑old woman with prior parathyroid adenoma resection for primary hyperparathyroidism who now has stage 3 chronic kidney disease (eGFR ≈ 45 mL/min/1.73 m²), mild albuminuria, low 25‑hydroxy‑vitamin D, elevated parathyroid hormone, normal serum calcium, normal serum phosphate, and normal alkaline phosphatase?

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

Have a follow-up question?

Our Medical A.I. is used by practicing medical doctors at top research institutions around the world. Ask any follow up question and get world-class guideline-backed answers instantly.

Ask Question