Scapulothoracic Fusion for Facioscapulohumeral Muscular Dystrophy

Abstract

In developmental dysplasia of the hip, a deficient acetabulum may be augmented by placing local autogenous iliac osseous graft, or the ilium itself, over the head of the femur with the expectation that the added bone will function as a bearing surface. We analysed this bone obtained en bloc during subsequent surgery which was performed for degenerative osteoarthritis in three patients at 6, 25 and 30 years after the initial augmentation procedure. In each patient, the augmentation comprised of red cancellous bone covered on its articulating surface by a distinct layer of white tissue. Microscopy of this tissue showed parallel rows of spindle-shaped cells lying between linearly arranged collagen bundles typical of joint capsule. Biochemical analysis showed type I collagen, the principal collagen of joint capsule and bone, with no significant quantity of type II collagen, the principal collagen of cartilage. While the added bone produced by acetabular augmentation was durable, histological and biochemical analyses suggested that it had not undergone cartilage metaplasia. The augmented acetabulum articulates with the head of the femur by means of an interposed hip joint capsule.

Facioscapulohumeral muscular dystrophy assessment and treatment

“…5 Twyman et al reported that forced vital capacity decreased by an average of 21% (range, 3.2% to 30%) in all five patients in his series who had bilateral scapulothoracic fusion. 21 Others have reported minimal or no reduction in vital capacity, respiratory volume or forced expiratory volume following scapulothoracic fusion.…”

“…4 The weakness of scapular stabilisers means that the shoulder blade is abnormally mobile and rotates causing cephalad displacement and winging. 5 The increased mobility means that deltoid is also at a mechanical disadvantage and the additional effort required causes the deltoid to fatigue easily. At rest there is an elevated scapular position due to weakness of the middle and lower thirds of trapezius and internal rotation of shoulder.…”

“…16 It can also alleviate discomfort and improve the appearance of the shoulder. 5 Various methods of scapulothoracic stabilisation have been described. Although muscle transfers, including those involving trapezius, teres major, rhomboids, latissimus dorsi and pectoralis major, have been used to treat scapular winging due to poliomyelitis, these procedures are contraindicated in FSHD because of the widespread muscle weakness and uncertainties of progression of muscle involvement.…”

Inherited Myopathies and Muscular Dystrophies

“…Patients with severe arm weakness may benefit from surgical fixation of the scapulae. 48,49 Immunosuppressive medications are ineffective despite the inflammatory response seen on muscle biopsies. Respiratory insufficiency occurs rarely, usually many years after disease onset.…”

Meta-Analysis of Long Thoracic Nerve Decompression and Neurolysis Versus Muscle and Tendon Transfer Operative Treatments of Winging Scapula

“… 5 – 9 Static winging scapula 4 in muscular dystrophy, facioscapulohumeral dystrophy, is corrected mainly by scapulothoracic fusion/arthrodesis and tendon transfer procedures. 2 , 10 – 17…”