Abstract
Fascioscapular humeral dystrophy (FSHD), an autosomal dominant genetic disorder affecting 1 in every 20,000 people, is characterized by development of dystrophic changes in muscles of the face, shoulder girdle, and upper limb, along with potential involvement of the lower limbs and spine. 6 Felice K.J. Moore S.A. Unusual clinical presentations in patients harboring the fascioscapulohumeral dystrophy 4q35 deletion. Muscle Nerve. 2001; 24: 352-356 Crossref PubMed Scopus (50) Google Scholar , 7 Grogan D.P. Stanley E.A. Bobechko W.P. The congenital undescended scapula: Surgical correction by the Woodward procedure. J Bone Joint Surg Br. 1983; 65: 598-605 PubMed Google Scholar , 8 Jeannopoulous C.L. Congenital elevation of the scapula. J Bone Joint Surg Am. 1952; 34: 883-892 PubMed Google Scholar , 11 Robinson A.R. Braun R.M. Mack P. Zadek R. The surgical importance of the clavicular component of Sprengel’s deformity. J Bone Joint Surg Am. 1967; 49: 1468-1484 Google Scholar Symptoms typically present in the second decade of life and progress slowly through adulthood, resulting in limited functional capacity and vocational opportunities. 1 Brouwer O.F. Padberg G.W. Wigmenga C. Frants R.R. Fascioscapular humeral dystrophy in early childhood. Arch Neurol. 1994; 51: 387-394 Crossref PubMed Scopus (91) Google Scholar In a study on careers of patients with FSHD, 23% indicated being unemployed at one time, 20% were wheelchair-dependent, 11% had obtained jobs through a sheltered workplace for the disabled, and 17% needed to change jobs as their disease progressed. 15 Wevers C.W. Brouwer O.F. Padberg G.W. Nijboer I.D. Job perspectives in fascioscapular humeral dystrophy. Disabil Rehab. 1993; 15: 24-28 Crossref PubMed Scopus (29) Google Scholar
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