Scapulothoracic fusion for facioscapulohumeral muscular dystrophy.
BACKGROUND
Facioscapulohumeral muscular dystrophy causes winging of the scapula and weakness and discomfort of the shoulder. Surgical stabilization of the scapula to the posterior part of the chest wall permits shoulder abduction and flexion by the deltoid muscle. In the present retrospective study, we describe our experience with eleven scapulothoracic fusion procedures that were performed for the treatment of the infantile and adolescent forms of the disease.


METHODS
Eleven procedures were performed in eight patients, including four male patients (one of whom had bilateral involvement and three of whom had unilateral involvement) and four female patients (two of whom had bilateral involvement and two of whom had unilateral involvement). One of the female patients had the infantile variant, whereas all other patients had the adolescent form of the disease. The mean age at the time of the eleven operations was seventeen years. The scapula was fused to the thorax in 25 degrees of abduction with use of 16-gauge wires, a plate or washers on the posteromedial scapular surface to prevent wire pull-out, and iliac crest autograft. After a mean duration of follow-up of 6.3 years, all patients were assessed clinically and radiographically.


RESULTS
In all cases, scapular winging and shoulder fatigue and pain were initially eliminated. In the first year after the operation, active abduction and flexion of the shoulder improved to a mean of 145 degrees (range, 110 degrees to 160 degrees ) and 144 degrees (range, 130 degrees to 160 degrees ), respectively, from a preoperative mean of 75 degrees (range, 70 degrees to 90 degrees ). At the time of the final assessment (mean, 6.3 years postoperatively), abduction and flexion were maintained at a mean of 139 degrees and 134 degrees , respectively, in seven shoulders; however, in the remaining four shoulders, both of these motions had decreased to a mean of 48 degrees because of progressive loss of deltoid muscle strength. In two cases, prominent subcutaneous wires required trimming. There were no other complications.


CONCLUSIONS
Scapulothoracic fusion relieves shoulder fatigue and pain, allows for smooth functional abduction and flexion of the upper extremity, and improves the appearance of the neck and shoulder in patients who have symptomatic scapular winging due to facioscapulohumeral muscular dystrophy. The procedure is associated with a low risk of complications. Progression of the disease affecting the deltoid muscle can cause loss of abduction, but the other benefits of stabilization persist.


METHODS
Therapeutic Level IV.
Diab
Mohammad
M
Department of Neurology, Children's Hospital Boston, Boston, Massachusetts 02115, USA.
Darras
Basil T
BT
Shapiro
Frederic
F
D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D020391:Muscular Dystrophy, Facioscapulohumeral / Q000601:surgery*; D019637:Orthopedic Procedures / Q000379:methods*; D012189:Retrospective Studies; D012540:Scapula / Q000601:surgery; D035441:Thoracic Wall / Q000601:surgery; D016896:Treatment Outcome