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Scoliosis in Prader–Willi syndrome: prevalence, effects of age, gender, body mass index, lean body mass and genotype
  1. R F A de Lind van Wijngaarden1,
  2. L W L de Klerk2,
  3. D A M Festen1,
  4. A C S Hokken-Koelega1,3
  1. 1
    Dutch Growth Research Foundation, Rotterdam, The Netherlands
  2. 2
    Erasmus University Medical Centre Rotterdam/Sophia Children’s Hospital, Department of Paediatric Orthopaedics, Rotterdam, The Netherlands
  3. 3
    Erasmus University Medical Centre Rotterdam/Sophia Children’s Hospital, Department of Paediatrics, Rotterdam, The Netherlands
  1. Mr R F A de Lind van Wijngaarden, Dutch Growth and Research Foundation and Erasmus University Medical Centre Rotterdam/Sophia Children’s Hospital, Westzeedijk 106, 3016 AH Rotterdam, The Netherlands; r.delindvanwijngaarden{at}erasmusmc.nl

Abstract

Background: The reported prevalence of scoliosis in children with Prader–Willi syndrome varies from 15% to 86%.

Objective: To study the prevalence of scoliosis and the effects of age, gender, body mass index (BMI), total lean body mass (LBM), LBM of the trunk (trunkLBM) and genotype.

Design: Radiographs were taken, length and weight were measured (BMI standard deviation scores (BMI SDS) and body surface area (BSA)), and dual energy x-ray absorptiometry was performed, measuring LBM and trunkLBM.

Patients: 96 children, median (interquartile range) age 4.8 years (2.1 to 7.5), were included in a multicentre study. None received growth hormone treatment.

Main outcome measures: Two types of scoliosis were identified: (1) long C-curve type scoliosis (LCS) and (2) idiopathic scoliosis (IS). Children were divided into age categories (infants, 0–3 years; juveniles, 3–10 years; adolescents, 10–16 years).

Results: The prevalence of scoliosis was 37.5% and increased with age (infants and juveniles, ∼30%; adolescents, 80%); 44% of children with scoliosis had a Cobb angle above 20°. Children with scoliosis were significantly older than those without. Children with LCS were younger and more hypotonic than those with IS: median (interquartile range) age 4.4 years (1.7–5.9) vs 11.1 years (6.5–12.1) (p = 0.002) and trunkLBM/BSA ratio 7080 (6745–7571) vs 7830 (6932–8157) (p = 0.043).

Conclusions: The prevalence of scoliosis in children with Prader–Willi syndrome is high (37.5%). Many children with scoliosis (13%) had undergone brace treatment or surgery. The type of scoliosis is affected by age and trunkLBM/BSA ratio.

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Footnotes

  • See Editorial, p 1004

  • Competing interests: None.

  • Ethics approval: Obtained.

  • Patient consent: Obtained/parental consent obtained.

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