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Herlyn-Werner-Wünderlich syndrome: Two case report
Victoria Fontana1, Gabriela S Aboud1, Liliana Sabbaj2
1Clinical Pediatrics; Hospital General de Niños Ricardo Gutiérrez, City of Buenos Aires, Argentina.
Archivos Argentinos De Pediatria
|January 10, 2024
Summary
Herlyn-Werner-Wunderlich syndrome (OHVIRA) is a rare congenital condition affecting the Müllerian ducts. Early diagnosis and surgical treatment of the vaginal septum are crucial to prevent complications like infertility and endometriosis.
Area of Science:
- Reproductive Medicine
- Pediatric Surgery
- Medical Imaging
Background:
- Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare congenital Müllerian duct anomaly.
- It is characterized by a combination of septate uterus, obstructed hemivagina, and ipsilateral renal agenesis.
Observation:
- The most common clinical presentation includes abdominal mass due to hematocolpos, pain, and dysmenorrhea.
- Associated conditions include infertility, endometriosis, and menstrual and obstetric complications.
Findings:
- Ultrasound is the primary imaging technique for initial assessment.
- Magnetic resonance imaging (MRI) is the most accurate diagnostic method.
- Surgical resection of the vaginal septum is the recommended treatment.
Implications:
- Early diagnosis is vital to prevent long-term complications such as infertility and endometriosis.
- This condition requires a multidisciplinary approach involving gynecologists, radiologists, and surgeons.
- Understanding OHVIRA is crucial for effective management and improved patient outcomes.
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