[Effect of thoracic bracing on lung function in children with neuromuscular disease]. / Impact du corset thoracique sur la fonction respiratoire chez des enfants atteints de maladie neuromusculaire.

ABSTRACT

UNLABELLED Respiratory muscle weakness associated with scoliosis in neuromuscular disease leads to respiratory impairment. Children with scoliosis are usually treated with spinal bracing to delay the progress of disease. We studied the impact of spinal bracing on lung function in these children. METHODS: Retrospective study of patient data from January 1997 to January 2003. Spirometry and measurement of lung volume involved 32 observations, corresponding to 17 children with neuromuscular disease, including 14 with spinal muscular atrophy. Data for children with and without a brace were studied. RESULTS: A total of 72% of the children had severe scoliosis (Cobb score>30 degrees ); 40% wore a Garchois brace. Children without a brace showed a mean vital capacity of 65% of predicted value, with a restrictive syndrome in 44% of observations. Children with a brace showed significantly reduced vital capacity (-4.6%; P<0.001) and forced expiratory volume in 1 s (-4.6%; P=0.002). The reduced vital capacity was lower in children with the Garchois brace -1% (P=0.02). Severity of scoliosis and measured volumes were not related. CONCLUSION: Spinal bracing in children with neuromusclar disease leads to significant respiratory impairment. Assessment of pulmonary function is necessary when a brace is indicated. The Garchois brace might lead to less impairment of respiratory function.