Scoliosis and lung function in spinal muscular atrophy

Eur Spine J. 1995;4(5):268-73. doi: 10.1007/BF00301032.

Abstract

The notes and radiographs of 43 patients with a confirmed diagnosis of spinal muscular atrophy were reviewed. A significant inverse linear relationship between the severity of scoliosis and the percentage of predicted vital capacity and peak flow was found. The patients who stood had a significantly better lung function than patients who were confined to a wheelchair, and their scoliosis deteriorated significantly more slowly. Sixteen patients underwent surgical spinal stabilisation, 4 with Harrington instrumentation and 12 with segmental spinal instrumentation, at an average age of 12 years and 11 months. The average curve correction achieved was 40%. The decline in lung function seen pre-operatively was not only reversed, but a significant improvement was found at final follow-up.

MeSH terms

  • Adolescent
  • Case-Control Studies
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Lung / physiopathology*
  • Lung Volume Measurements
  • Male
  • Muscular Atrophy, Spinal / complications
  • Muscular Atrophy, Spinal / physiopathology*
  • Posture
  • Retrospective Studies
  • Scoliosis / etiology
  • Scoliosis / physiopathology*
  • Scoliosis / surgery
  • Spinal Fusion
  • Spinal Muscular Atrophies of Childhood / physiopathology
  • Surveys and Questionnaires