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°
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° (range,
110° to 160°) and 144° (range, 130° to 160°),
respectively, from a preoperative mean of 75° (range, 70° to 90°).
At the time of the final assessment (mean, 6.3 years postoperatively),
abduction and flexion were maintained at a mean of 139° and 134°,
respectively, in seven shoulders; however, in the remaining four shoulders,
both of these motions had decreased to a mean of 48° 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.
Level of Evidence: Therapeutic Level IV. See Instructions
to Authors for a complete description of levels of evidence.