Current therapeutic guidelines in Duchenne Muscular Dystrophy to prolong life.

Duchenne's myopathy is an X-linked disease with well defined evolutionary phases, characterized by degradation of the walking function, development of evolutive scoliosis and progressive decline of the respiratory function leading patients to premature death. In 1985 Y. Rideau in France carried out a new global therapeutic strategy for treatment of lower limb deformities, scoliosis deformity and progressive restrictive syndrome. The indication for surgery at the lower limbs is made very early, at the onset of the first signs of disease. The procedures are carried out at the same time and always bilaterally; they include: (i) hip section of superficial flexors; (ii) iliotibial band resection; (iii) subcutaneous tenotomy of semitendineous and gracilis; (iv) subcutaneous lengthening of Achilles tendons. In the post-operative period, the patient begins exercises of active and passive mobility in few days and after three weeks recovers his performances; ambulation will remain almost normal for several years. A comparison of two groups of patients, the first precociously operated on the lower limbs, the other one not operated, shows better performances in the operated group. The indications for surgical treatment of Duchenne scoliosis must be made after the loss of ambulation and not too late, to avoid the concurrent respiratory restrictive syndrome makes the patient inoperable. Over ten years ago, in Poitiers, a specific instrumentation for Duchenne scoliosis was created, providing for cylindrical rods fixed by peduncular screws at the sacro-lumbar level. On the dorso-lumbar level, the rod becomes flat to allow more flexibility of the trunk. The complications observed in a group of 55 patients operated for scoliosis, consisted in 2 cases of breaking of rods and 1 superficial infection. The surgery approach in DMD has the double aim to prolong the time of the autonomous ambulation and to avoid the evolution of scoliosis, limiting the harmful effects of the scoliosis on the respiratory function. However, the surgery alone is unable to prolong the life expectancy in these patients, without treating the restrictive respiratory syndrome, first by nasal ventilation and then by elective tracheotomy, essential for the survival of the patient