Natural course of scoliosis and lifetime risk of scoliosis surgery in spinal muscular atrophy
| Autor: | Camiel A. Wijngaarde, Leonard H. van den Berg, Rob C. Brink, Joyce Sombroek, W-Ludo van der Pol, René M. Castelein, Ruben P A van Eijk, Floor A.S. de Kort, Louise A.M. Otto, Fay-Lynn Asselman, Marjolein Verhoef, Renske I. Wadman, Bart Bartels, Marloes Stam, Inge Cuppen |
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| Rok vydání: | 2019 |
| Předmět: |
Adult
Male medicine.medical_specialty Adolescent Cross-sectional study Population Scoliosis Cohort Studies Muscular Atrophy Spinal Young Adult 03 medical and health sciences 0302 clinical medicine medicine Humans Orthopedic Procedures Longitudinal Studies 030212 general & internal medicine Young adult Child education Aged Aged 80 and over education.field_of_study business.industry Hazard ratio Infant Newborn Infant Spinal muscular atrophy Middle Aged medicine.disease SMA Surgery Cross-Sectional Studies Child Preschool Disease Progression Female Neurology (clinical) business 030217 neurology & neurosurgery Cohort study |
| Zdroj: | Neurology. 93:e149-e158 |
| ISSN: | 1526-632X 0028-3878 |
| DOI: | 10.1212/wnl.0000000000007742 |
| Popis: | ObjectiveTo investigate the natural course of scoliosis and to estimate lifetime probability of scoliosis surgery in spinal muscular atrophy (SMA).MethodsWe analyzed cross-sectional data from 283 patients from our population-based cohort study. Additional longitudinal data on scoliosis progression and spinal surgery were collected from 36 consecutive patients who received scoliosis surgery at our center.ResultsThe lifetime probability of receiving scoliosis surgery was ≈80% in SMA types 1c and 2. Patients with type 2 who only learned to sit (type 2a) were significantly younger at time of surgery than those who learned to sit and stand (type 2b). The lifetime risk of surgery was lower in type 3a (40%) and strongly associated with age at loss of ambulation: 71% in patients losing ambulation before 10 years of age vs 22% losing ambulation after the age of 10 years (p = 0.005). In type 3a, preserving the ability to walk 1 year longer corresponded to a 15% decrease in lifetime risk of scoliosis surgery (hazard ratio 0.852, p = 0.017). Scoliosis development was characterized by initial slow progression, followed by acceleration in the 1.5- to 2-year period before surgery.ConclusionThe lifetime probability of scoliosis surgery is high in SMA types 1c and 2 and depends on age at loss of ambulation in type 3. Motor milestones such as standing that are not part of the standard classification system are of additional predictive value. Our data may act as a reference to assess long-term effects of new SMA-specific therapies. |
| Databáze: | OpenAIRE |
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