[Palliative treatment of myopathies. Contribution of surgical treatments].

Abstract

The knowledge acquired over the last few years on Duchenne's muscular dystrophy (DMD) serves as the basis for management of all types of myopathy. After reviewing the current classification of muscle dystrophies and the principles of orthopedic management of these conditions, a historical perspective of surgical procedures used in DMD is presented. The various modes of discovery of myopathy are described. Deformities of the limbs and spine related to DMD are reviewed and their outcome is detailed. In DMD patients, the surgical procedures described allowed to delay loss of the ability to walk by 1 year 7 months to 3 years 8 months according to the severity of the condition, and provided significant improvements in quality of life. Surgical stabilization of the scoliosis ensured preservation of comfortable sitting in every case, as well as preservation of autonomous upper limb motion, with a low rate of complications. Surgical indications in DMD are well-established and serve as the basis for making surgical decisions in the other myopathies, except during early infancy. The principles and orientations of treatment of the other myopathies are defined, in the light of the variability of lesions and of the rate of muscle function loss. In these non-DMD myopathies, medical and surgical treatments are of as yet unquantifiable benefit but provide a substantial improvement in quality of life.