- Discussion:
- is transmitted as an autosomal dominant condition;
- most patients show relatively slow progression of weakness involving primarily muscles of facial expression & proximal upper limbs;
- whistling and sucking thru straw may be impossible, & patients cannot fully bury their eyelashes when they close their eyes;
- although their arms become gradually weaker from adolescence on, patients can usually work into later life;
- in common form, facial weakness is noted in first decade, w/ main musculoskeletal problem being
related to weakness of the shoulder girdle with scapular winging;
- weakness is due to weakness of muscles that fix scapula to thoracic wall (
trapezius,
levator scapulae, and
rhomboids);
- muscles that abduct glenohumeral joint (
deltoid,
supraspinatus,
infraspinatus, and
subscapularis) remain strong;
- in absence of stabilization, there is winging of the scapula and limited abduction of the arm;
Current Concepts Review.
The Diagnosis and Orthopaedic Treatment of Inherited Muscular Diseases of Childhood.
Scapulothoracic arthrodesis for patients who have fascioscapulohumeral muscular dystrophy.
Scapulocostal stabilization for scapular winging in facioscapulohumeral muscular dystrophy.
Scapulothoracic arthrodesis in facioscapulohumeral muscular dystrophy. Review of seventeen procedures with three to twenty-one-year follow-up.
Simplified Technique for Scapulocostal Fusion in Facioscapulohumeral Dystrophy.
J. Pediatr. Orthop 13:749-751,1993 Jakab E., Gledhill R.B.
Thoracoscapular Fusion for Facioscapulohumeral Dystrophy.
Copeland S.A., Howard R.C. JBJS. 60B:547-551,1978
Scapulothoracic arthrodesis in facioscapulohumeral muscular dystrophy.
Scapulothoracic Fusion for Facioscapulohumeral Muscular Dystrophy.
