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[Role and indications for surgery in Duchenne de Boulogne muscular dystrophy with rapid development].

[Article in French]

Abstract

One hundred and fifteen children suffering from muscular dystrophy had been treated surgically during the previous 12 years. The aim of the treatment was to slow the progression of development of spine deformity and obtain a prolongation of walking and standing ability. The indications for surgery were in relation to trauma in 17 cases or for progressive lower limb or spine deformity. In walking patients (21 cases) surgery was indicated only for contractures limiting walking ability. Surgery was also indicated in 35 cases soon after walking capability had ceased. In these cases correction of deformity allowed children to walk again with calipers for an average of 3 years. In 37 cases surgery was performed only to allow standing or to prevent or delay the development of spinal deformity. The indications for extensive spine fusion are discussed in relation to the speed of development of deformity, the pulmonary vital capacity, the dynamometric estimation of muscle strength, and the estimated duration of survival. Human, family and ethical factors are discussed.

PMID:
6224272
[PubMed - indexed for MEDLINE]
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