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Natural course of scoliosis and lifetime risk of scoliosis surgery in spinal muscular atrophy.
Wijngaarde, Camiel A; Brink, Rob C; de Kort, Floor A S; Stam, Marloes; Otto, Louise A M; Asselman, Fay-Lynn; Bartels, Bart; van Eijk, Ruben P A; Sombroek, Joyce; Cuppen, Inge; Verhoef, Marjolein; van den Berg, Leonard H; Wadman, Renske I; Castelein, René M; van der Pol, W-Ludo.
Affiliation
  • Wijngaarde CA; From the Department of Neurology and Neurosurgery (C.A.W., F.A.S.d.K., M.S., L.A.M.O., F.-L.A., R.P.A.v.E., J.S., I.C., L.H.v.d.B., R.I.W., W-L.v.d.P), Department of Rehabilitation (M.V.), Department of Orthopedic Surgery (R.C.B., R.M.C.), Department of Pediatrics, Child Development and Exercise Cen
  • Brink RC; From the Department of Neurology and Neurosurgery (C.A.W., F.A.S.d.K., M.S., L.A.M.O., F.-L.A., R.P.A.v.E., J.S., I.C., L.H.v.d.B., R.I.W., W-L.v.d.P), Department of Rehabilitation (M.V.), Department of Orthopedic Surgery (R.C.B., R.M.C.), Department of Pediatrics, Child Development and Exercise Cen
  • de Kort FAS; From the Department of Neurology and Neurosurgery (C.A.W., F.A.S.d.K., M.S., L.A.M.O., F.-L.A., R.P.A.v.E., J.S., I.C., L.H.v.d.B., R.I.W., W-L.v.d.P), Department of Rehabilitation (M.V.), Department of Orthopedic Surgery (R.C.B., R.M.C.), Department of Pediatrics, Child Development and Exercise Cen
  • Stam M; From the Department of Neurology and Neurosurgery (C.A.W., F.A.S.d.K., M.S., L.A.M.O., F.-L.A., R.P.A.v.E., J.S., I.C., L.H.v.d.B., R.I.W., W-L.v.d.P), Department of Rehabilitation (M.V.), Department of Orthopedic Surgery (R.C.B., R.M.C.), Department of Pediatrics, Child Development and Exercise Cen
  • Otto LAM; From the Department of Neurology and Neurosurgery (C.A.W., F.A.S.d.K., M.S., L.A.M.O., F.-L.A., R.P.A.v.E., J.S., I.C., L.H.v.d.B., R.I.W., W-L.v.d.P), Department of Rehabilitation (M.V.), Department of Orthopedic Surgery (R.C.B., R.M.C.), Department of Pediatrics, Child Development and Exercise Cen
  • Asselman FL; From the Department of Neurology and Neurosurgery (C.A.W., F.A.S.d.K., M.S., L.A.M.O., F.-L.A., R.P.A.v.E., J.S., I.C., L.H.v.d.B., R.I.W., W-L.v.d.P), Department of Rehabilitation (M.V.), Department of Orthopedic Surgery (R.C.B., R.M.C.), Department of Pediatrics, Child Development and Exercise Cen
  • Bartels B; From the Department of Neurology and Neurosurgery (C.A.W., F.A.S.d.K., M.S., L.A.M.O., F.-L.A., R.P.A.v.E., J.S., I.C., L.H.v.d.B., R.I.W., W-L.v.d.P), Department of Rehabilitation (M.V.), Department of Orthopedic Surgery (R.C.B., R.M.C.), Department of Pediatrics, Child Development and Exercise Cen
  • van Eijk RPA; From the Department of Neurology and Neurosurgery (C.A.W., F.A.S.d.K., M.S., L.A.M.O., F.-L.A., R.P.A.v.E., J.S., I.C., L.H.v.d.B., R.I.W., W-L.v.d.P), Department of Rehabilitation (M.V.), Department of Orthopedic Surgery (R.C.B., R.M.C.), Department of Pediatrics, Child Development and Exercise Cen
  • Sombroek J; From the Department of Neurology and Neurosurgery (C.A.W., F.A.S.d.K., M.S., L.A.M.O., F.-L.A., R.P.A.v.E., J.S., I.C., L.H.v.d.B., R.I.W., W-L.v.d.P), Department of Rehabilitation (M.V.), Department of Orthopedic Surgery (R.C.B., R.M.C.), Department of Pediatrics, Child Development and Exercise Cen
  • Cuppen I; From the Department of Neurology and Neurosurgery (C.A.W., F.A.S.d.K., M.S., L.A.M.O., F.-L.A., R.P.A.v.E., J.S., I.C., L.H.v.d.B., R.I.W., W-L.v.d.P), Department of Rehabilitation (M.V.), Department of Orthopedic Surgery (R.C.B., R.M.C.), Department of Pediatrics, Child Development and Exercise Cen
  • Verhoef M; From the Department of Neurology and Neurosurgery (C.A.W., F.A.S.d.K., M.S., L.A.M.O., F.-L.A., R.P.A.v.E., J.S., I.C., L.H.v.d.B., R.I.W., W-L.v.d.P), Department of Rehabilitation (M.V.), Department of Orthopedic Surgery (R.C.B., R.M.C.), Department of Pediatrics, Child Development and Exercise Cen
  • van den Berg LH; From the Department of Neurology and Neurosurgery (C.A.W., F.A.S.d.K., M.S., L.A.M.O., F.-L.A., R.P.A.v.E., J.S., I.C., L.H.v.d.B., R.I.W., W-L.v.d.P), Department of Rehabilitation (M.V.), Department of Orthopedic Surgery (R.C.B., R.M.C.), Department of Pediatrics, Child Development and Exercise Cen
  • Wadman RI; From the Department of Neurology and Neurosurgery (C.A.W., F.A.S.d.K., M.S., L.A.M.O., F.-L.A., R.P.A.v.E., J.S., I.C., L.H.v.d.B., R.I.W., W-L.v.d.P), Department of Rehabilitation (M.V.), Department of Orthopedic Surgery (R.C.B., R.M.C.), Department of Pediatrics, Child Development and Exercise Cen
  • Castelein RM; From the Department of Neurology and Neurosurgery (C.A.W., F.A.S.d.K., M.S., L.A.M.O., F.-L.A., R.P.A.v.E., J.S., I.C., L.H.v.d.B., R.I.W., W-L.v.d.P), Department of Rehabilitation (M.V.), Department of Orthopedic Surgery (R.C.B., R.M.C.), Department of Pediatrics, Child Development and Exercise Cen
  • van der Pol WL; From the Department of Neurology and Neurosurgery (C.A.W., F.A.S.d.K., M.S., L.A.M.O., F.-L.A., R.P.A.v.E., J.S., I.C., L.H.v.d.B., R.I.W., W-L.v.d.P), Department of Rehabilitation (M.V.), Department of Orthopedic Surgery (R.C.B., R.M.C.), Department of Pediatrics, Child Development and Exercise Cen
Neurology ; 93(2): e149-e158, 2019 07 09.
Article in En | MEDLINE | ID: mdl-31164393
ABSTRACT

OBJECTIVE:

To investigate the natural course of scoliosis and to estimate lifetime probability of scoliosis surgery in spinal muscular atrophy (SMA).

METHODS:

We 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.

RESULTS:

The 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.

CONCLUSION:

The 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.
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Scoliosis / Muscular Atrophy, Spinal Type of study: Etiology_studies / Incidence_studies / Observational_studies / Prevalence_studies / Prognostic_studies / Risk_factors_studies Limits: Adolescent / Adult / Aged / Aged80 / Child / Child, preschool / Female / Humans / Infant / Male Language: En Journal: Neurology Year: 2019 Document type: Article
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Scoliosis / Muscular Atrophy, Spinal Type of study: Etiology_studies / Incidence_studies / Observational_studies / Prevalence_studies / Prognostic_studies / Risk_factors_studies Limits: Adolescent / Adult / Aged / Aged80 / Child / Child, preschool / Female / Humans / Infant / Male Language: En Journal: Neurology Year: 2019 Document type: Article