Scapulopexie dans la myopathie facio-scapulohumérale

Facio-scapulohumeral (FSH) muscular dystrophy, first described by Landouzy and Déjerine (1885) [2], represents one of the most frequent forms of congenital myopathy. FSH muscular dystrophy is characterized by prevalent weakness of facial and proximal upper limb muscles that progresses very slowly. Palsy preferentially affects the scapulothoracic joint muscle. During upper limb movements, this leads to a winged scapula and to an absence of scapula stability on the thorax. This does not allow normal abduction and flexion of the shoulder. We suggest that the scapula be fixed to the rib cage without arthrodesis, thereby achieving a scapulopexy by using absorbable material, to obtain a fibrous scar. Both sides are operated at the same time. During postoperative care, an orthesis is worn permanently for 3 weeks, then only during daytime for 2 months. Physiotherapy lasts for 3 months in order for patients to learn how to use their upper limbs again.