The Journal of Bone and Joint Surgery (American). 2005;87:2267-2275.
doi:10.2106/JBJS.D.02952
© 2005 The Journal of Bone and Joint Surgery, Inc.
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 Pediatrics Test 8: Winter 2006
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Scapulothoracic Fusion for Facioscapulohumeral Muscular Dystrophy

Mohammad Diab, MD1, Basil T. Darras, MD2 and Frederic Shapiro, MD2

1 Department of Orthopaedic Surgery, University of California, San Francisco, School of Medicine, 500 Parnassus Avenue, San Francisco, CA 94143
2 Departments of Neurology (B.T.D.) and Orthopaedic Surgery (F.S.), Children's Hospital Boston, 300 Longwood Avenue, Boston, MA 02115. E-mail address for F. Shapiro: frederic.shapiro{at}childrens.harvard.edu

Investigation performed at the Departments of Neurology and Orthopaedic Surgery, Children's Hospital Boston, Boston, Massachusetts

The authors did not receive grants or outside funding in support of their research or preparation of this manuscript. They did not receive payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity. No commercial entity paid or directed, or agreed to pay or direct, any benefits to any research fund, foundation, educational institution, or other charitable or nonprofit organization with which the authors are affiliated or associated.

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.


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