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Herlyn-Werner-Wunderlich syndrome: a case report.
Pedro Salomao Piccinini1, John Doski2
1Department of Surgery, University of Texas Health Science Center, San Antonio, TX, USA.
Herlyn-Werner-Wunderlich (HWW) syndrome, a rare congenital disorder, involves uterus didelphys, obstructed hemivagina, and kidney agenesis. Delayed diagnosis is possible even with normal menses, highlighting the need for awareness.
Area of Science:
- Reproductive medicine
- Pediatric surgery
- Medical imaging
Background:
- Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly affecting Müllerian duct development.
- It classically presents with uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis.
- Typical symptoms include abdominal mass, pain, and dysmenorrhea due to hematocolpos.
Observation:
- This case presents a 13-year-old girl with HWW syndrome.
- Her menses were normal, leading to a potential diagnostic delay.
- Diagnosis was achieved through computed tomography (CT) scan, pelvic ultrasound, and surgical exploration.
Findings:
- The study details the evaluation and surgical management of a patient with HWW syndrome.
- It emphasizes that normal menstruation does not exclude the condition.
- Diagnostic imaging modalities like CT and ultrasound are crucial for identification.
Implications:
- This case highlights the importance of considering HWW syndrome even with atypical presentations like normal menses.
- Early and accurate diagnosis through imaging and surgical confirmation is vital for effective management.
- Understanding this rare condition aids in timely intervention and improved patient outcomes in pediatric gynecology.
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