Scoliosis in Prader-Willi syndrome: prevalence, effects of age, gender, body mass index, lean body mass and genotype
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, approximately 30%; adolescents, 80%); 44% of children with scoliosis had a Cobb angle above 20 degrees . 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.
|Persistent URL||dx.doi.org/10.1136/adc.2007.123836, hdl.handle.net/1765/15237|
|Journal||Archives of disease in childhood|
de Lind van Wijngaarden, R.F.A, Klerk, L.W.L, Festen, D.A.M, & Hokken-Koelega, A.C.S. (2008). Scoliosis in Prader-Willi syndrome: prevalence, effects of age, gender, body mass index, lean body mass and genotype. Archives of disease in childhood, 93(12), 1012–1016. doi:10.1136/adc.2007.123836