Management of scapular dysfunction in facioscapulohumeral muscular dystrophy: the biomechanics of winging, arthrodesis indications, techniques and outcomes

Abstract

center dot Facioscapulohumeral muscular dystrophy (FSHD) is a common hereditary disorder which typically results in scapular winging due to wasting of the periscapular muscles affected by this condition.center dot Scapulothoracic arthrodesis (STA) is the current surgical treatment for FSHD patients with severe winging and preserved deltoid muscle. center dot There are several different techniques in the literature such as multifilament cables alone and cable or cerclage wires combined with single or multiple plates. We prefer cables without plates as it provides independent strong fixation points and strongly recommend utilization of autograft.center dot The functional results of studies report that regardless of the technique used, shoulder elevation and thus quality of life is improved, as shown with outcome scores.center dot There are several complications associated with STA. Pulmonary complications are common and usually resolve spontaneously. Meticulous surgical technique and effective postoperative analgesia may reduce the incidence. Scapular complications which are associated with the fixation may be encountered in the early or late period, which are related to the learning curve of the surgeon.center dot In conclusion, STA is a reliable solution to a major problem in FSHD patients that helps them maintain their activities of daily living until a cure for the disease is found. A successful result is strongly dependent on patient selection, and a multidisciplinary team of neurologists, geneticists and orthopaedic surgeons is required to achieve good results.