title: Surgical treatment of upper extremity contractures in Emery-Dreifuss muscular dystrophy.
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.
authors.affiliation: aHand & 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.
authors.forename: Felicity G
authors.initials: FG
authors.lastname: Fishman
authors.forename: Edward M
authors.initials: EM
authors.lastname: Goldstein
authors.forename: Allan E
authors.initials: AE
authors.lastname: Peljovich
mesh_terms: D000293:Adolescent; D003286:Contracture / Q000601:surgery*; D004550:Elbow / Q000601:surgery; D005260:Female; D006801:Humans; D008297:Male; D020389:Muscular Dystrophy, Emery-Dreifuss / Q000601:surgery*; D010375:Pedigree; D011184:Postoperative Period; D016059:Range of Motion, Articular; D034941:Upper Extremity / Q000601:surgery*