Article Text

Download PDFPDF
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.

Statistics from Altmetric.com

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

Footnotes

  • See Editorial, p 1004

  • Competing interests: None.

  • Ethics approval: Obtained.

  • Patient consent: Obtained/parental consent obtained.

Linked Articles