title: Scoliosis and lung function in spinal muscular atrophy.
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.
authors.affiliation: Department of Orthopaedic Surgery, University of Manchester, Royal Manchester Children's Hospital, UK.
authors.forename: D
authors.initials: D
authors.lastname: Robinson
authors.forename: C S
authors.initials: CS
authors.lastname: Galasko
authors.forename: C
authors.initials: C
authors.lastname: Delaney
authors.forename: J B
authors.initials: JB
authors.lastname: Williamson
authors.forename: J L
authors.initials: JL
authors.lastname: Barrie
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008168:Lung / Q000503:physiopathology*; D008176:Lung Volume Measurements; D008297:Male; D009134:Muscular Atrophy, Spinal / Q000150:complications / Q000503:physiopathology*; D011187:Posture; D012189:Retrospective Studies; D012600:Scoliosis / Q000209:etiology / Q000503:physiopathology* / Q000601:surgery; D013123:Spinal Fusion; D014897:Spinal Muscular Atrophies of Childhood / Q000503:physiopathology; D011795:Surveys and Questionnaires