Overview
Double uterus, hemivagina, renal agenesis syndrome, also known as Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome, is characterized by Müllerian duct anomalies including a duplicated uterus with a hemivagina and ipsilateral renal agenesis or anomaly, often accompanied by contralateral urinary tract abnormalities 1.Diagnosis
Key Diagnostic Criteria: Müllerian duct duplication with hemivagina, ipsilateral renal agenysis or anomaly, contralateral urinary tract anomalies such as vesicoureteral reflux 1.
Recommended Tests:
- Imaging: Ultrasound, MRI, or CT to identify Müllerian duct anomalies and renal abnormalities 1.
- Renal Function Tests: To assess renal function and identify any urological issues 1.Management
First-Line Treatments:
- Surgical Intervention: Indicated for recurrent urinary tract infections, uncontrolled vaginal distention, urinary incontinence, or other complications 1.
Adjunctive Treatments:
- Antibiotics: For managing recurrent urinary tract infections 1.
- Voiding Dysfunction Management: Behavioral modifications or interventions for urinary incontinence 1.Special Populations
Pediatrics: Prepubertal patients require careful monitoring due to potential complications like recurrent infections and vaginal distention; early surgical intervention may be necessary 1.Key Recommendations
Regular Imaging and Monitoring in prepubertal patients diagnosed with OHVIRA syndrome to detect early complications and guide timely intervention (Evidence: Moderate 1).
Surgical Management should be considered for symptomatic patients, particularly those with recurrent urinary tract infections or significant anatomical obstruction (Evidence: Moderate 1).
Antibiotic Prophylaxis may be warranted for patients with recurrent urinary tract infections to prevent complications (Evidence: Weak 1).References
1 Han JH, Lee YS, Im YJ, Kim SW, Lee MJ, Han SW. Clinical Implications of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome in the Prepubertal Age Group. PloS one 2016. link