PALLIATIVE TREATMENT OF MYOPATHIES - CONTRIBUTION OF SURGERY

The knowledge acquired over the last few years on Duchenne's muscular dystrophy (DMD) serves as the basis for management of all types of myo pathy. After reviewing the current classification of muscle dystrophie s and the principles of orthopedic management of these conditions, a h istorical 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 allowe d 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 signi ficant improvements in quality of life. Surgical stabilization of the scoliosis ensured preservation of comfortable sitting in every case, a s well as preservation of autonomous upper limb motion, with a low rat e of complications. Surgical indications in DMD are well-established a nd serve as the basis for making surgical decisions in the other myopa thies, except during early infancy. The principles and orientations of treatment of the other myopathies are defined, in the light of the va riability of lesions and of the rate of muscle function loss. In these non-DMD myopathies, medical and surgical treatments are of as yet unq uantifiable benefit but provide a substantial improvement in quality o f life.