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Surgical treatment of upper extremity contractures in Emery-Dreifuss muscular dystrophy
Felicity G Fishman1, Edward M Goldstein, Allan E Peljovich
1aHand & Upper Extremity Service, Yale Department of Orthopaedics & Rehabilitation, Yale School of Medicine, New Haven, Connecticut bThe Pediatric Hand & Upper Extremity Center of Georgia cHand & Upper Extremity Program, Children's Healthcare of Atlanta at Scottish Rite dAtlanta Medical Center Orthopaedic Residency Program eChild Neurology Associates, Atlanta, Georgia, USA.
Abstract:
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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