RT Journal Article
SR Electronic
T1 Scoliosis in Prader–Willi syndrome: prevalence, effects of age, gender, body mass index, lean body mass and genotype
JF Archives of Disease in Childhood
JO Arch Dis Child
FD BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health
SP 1012
OP 1016
DO 10.1136/adc.2007.123836
VO 93
IS 12
A1 R F A de Lind van Wijngaarden
A1 L W L de Klerk
A1 D A M Festen
A1 A C S Hokken-Koelega
YR 2008
UL http://adc.bmj.com/content/93/12/1012.abstract
AB 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.