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Surgical treatment of upper extremity contractures in Emery–Dreifuss muscular dystrophy

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Emery–Dreifuss muscular dystrophy is a rare form of muscular dystrophy. In the present study we present two patients with Emery–Dreifuss muscular dystrophy and severe upper extremity contractures treated successfully with contracture release and musculotendinous lengthenings. For each of these patients a chart review was carried out and surgical technique reviewed. Patient 1 demonstrated elbow flexion contractures of 65° (right) and 60° (left) preoperatively and ∼45° (right) and 20° (left) postoperatively. Patient 2 demonstrated flexion contractures of 75° bilaterally preoperatively and 45° bilaterally postoperatively. For more severe elbow contractures, surgical treatment resulted in improved range of motion without a significant sacrifice of strength, allowing these patients to remain independent.
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Keywords: Emery–Dreifuss; contracture release; joint contracture; muscle lengthening; muscular dystrophy

Document Type: Research Article

Affiliations: 1: Hand & Upper Extremity Service, Yale Department of Orthopaedics & Rehabilitation, Yale School of Medicine, New Haven, Connecticut 2: Child Neurology Associates, Atlanta, Georgia, USA 3: The Pediatric Hand & Upper Extremity Center of Georgia, Hand & Upper Extremity Program, Children’s Healthcare of Atlanta at Scottish Rite, Atlanta Medical Center Orthopaedic Residency Program

Publication date: January 1, 2017

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