Scapulothoracic Arthrodesis in Facioscapulohumeral Dystrophy with Multifilament Cable

Abstract

Patients with facioscapulohumeral dystrophy (FSHD) are affected mostly by impaired shoulder function. Scapulothoracic arthrodesis was introduced to improve shoulder function. We evaluated the outcomes of scapulothoracic arthrodesis using multifilament cables, performed on 13 patients with FSHD (18 shoulders). There were eight males and five females (mean age, 29 years; range, 20-50 years). Outcome criteria were active shoulder forward flexion and abduction, the Disabilities of the Arm, Shoulder, and Hand (DASH) score, respiratory function tests, and a new shoulder function score. Patients were followed for a minimum of 24 months (average, 35.5 months; range, 24-87 months). Solid fusion was obtained in all shoulders (two after revision); active abduction range increased from 47.2°± 11.6°to 102.2°± 10.0°(mean ± standard deviation) and anterior flexion range from 55.6°± 16.1°to 126.1°± 20.9°. The DASH score decreased from 33.6 ± 8.9 points preoperatively to 11.6 ± 8.0 points postoperatively. Shoulder function score increased from 15.9 ± 2.4 points to 22.2 ± 1.3 points. Scapulothoracic arthrodesis provides satisfactory function in patients with FSHD. Our data suggest use of multifilament cables for fixation is a reasonable option with an acceptable complication rate.

Medium to Long-Term Outcome of Thoracoscapular Arthrodesis with Screw Fixation for Facioscapulohumeral Muscular Dystrophy

“…However, the fusion rate in the present series was 86% (thirty of thirty-five), equal to the rate in our previous series 1 . This is higher than the range of 58% to 83% in other published series involving wire fixation (see Appendix) 2,11,14,19 . Four shoulders in the present series had failure of the fixation during the early postoperative period.…”

“…The rate of revision for nonunion has been reported to be 9% to 11% in some previous series 2,10 . Demirhan et al 2 attributed most of the nonunions in their series to vigorous activity early during the recommended immobilization period. Our patient followed the postoperative regimen adequately and his nonunion had no clear cause.…”

“…Selecting the number of ribs involved in the arthrodesis is controversial 2,11 . We fused three consecutive ribs, providing adequate stability of the initial fixation.…”

“…It was first described by Landouzy and Dejerine in 1884 1 and is a genetic disorder characterized by weakness in facial and shoulder girdle musculature 2 . It is an autosomal dominant myopathy, although 10% to 30% of cases arise from a de novo mutation 3,4 .…”

Clinical practice considerations in facioscapulohumeral muscular dystrophy Sydney, Australia, 21 September 2015

“…A solid fusion is anticipated after 20 weeks with improvement in shoulder function expected by 6 months, although this can continue to improve up to 1 year after surgery. Complications are infrequent but include hemothorax or pneumothorax, rib or scapula facture, pain, infection, nonunion, reduced lung capacity, and stiff shoulder [1,4,[24][25][26]. Scapular fixation surgery may be considered in patients with FSHD who have normal deltoid strength at 45°abduction, a positive response to a compression test (gain of function when the scapulae are manually fixed), normal pulmonary function, and who have decreased QoL due to weakness in the muscles of scapular fixation or from pain.…”