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.
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow CME: Take the exam for this article:
 Pediatrics Test 8: Winter 2006
Right arrow [Supplementary Material]
Right arrow Letters to the Editor: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when Letters to the Editor are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrowReprints and Permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Diab, M.
Right arrow Articles by Shapiro, F.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Diab, M.
Right arrow Articles by Shapiro, F.
Related Collections
Right arrow Pediatrics
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us   Add to Technorati  
What's this?

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.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Technorati Technorati    What's this?


This article has been cited by other articles:


Home page
 Clin Med ResHome page
S. Giannini, C. Faldini, S. Pagkrati, G. Grandi, V. Digennaro, D. Luciani, and L. Merlini
Fixation of Winged Scapula in Facioscapulohumeral Muscular Dystrophy
Clin. Med. Res., October 1, 2007; 5(3): 155 - 162.
[Abstract] [Full Text] [PDF]