Summary
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.
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Paper read at the ESDS meeting, Birmingham, 1994, and selected for full publication.
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Robinson, D., Galasko, C.S.B., Delaney, C. et al. Scoliosis and lung function in spinal muscular atrophy. Eur Spine J 4, 268–273 (1995). https://doi.org/10.1007/BF00301032
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DOI: https://doi.org/10.1007/BF00301032