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[Freeman-Sheldon's syndrome. Report of five cases]
Henry Juver Vergara Fernández1, Leonori Román Capdevila
1Hospital Shriners para Niños, México, D.F. Clínica de Cirugía de Mano. vergarahenry@hotmail.com
Acta Ortopedica Mexicana
|April 5, 2008
Summary
Freeman-Sheldon syndrome, a rare arthrogryposis, often presents with adducted thumbs and clubfeet. Surgical interventions for these limb deformities can yield satisfactory outcomes in affected patients.
Area of Science:
- Orthopedics
- Genetics
- Pediatrics
Context:
- Freeman-Sheldon syndrome (FSS) is a rare congenital disorder.
- FSS is a type of arthrogryposis characterized by specific facial and limb malformations.
Purpose:
- To review clinical data of FSS patients.
- To evaluate extremity malformations and treatment outcomes in FSS.
Summary:
- A retrospective review of 5 FSS patients (4 male, 1 female) from 1990-2003.
- Common findings included adducted thumbs, treated with first web release and dorsal graft, improving grip.
- Severe bilateral clubfeet were surgically managed with posteromedial release and talectomy, achieving satisfactory results in most cases.
- Associated anomalies included undescended testis and inguinal hernia. Intellectual capacity was normal.
Impact:
- Highlights surgical management strategies for FSS-related upper and lower extremity deformities.
- Provides insights into the clinical spectrum and orthopedic challenges of Freeman-Sheldon syndrome.
- Contributes to understanding the management and outcomes for this rare condition.
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