Portal de Periódicos da CAPES

Scoliosis in Patients With Prader-Willi Syndrome

2008; American Academy of Pediatrics; Volume: 122; Issue: 2 Linguagem: Inglês

10.1542/peds.2007-3487

1098-4275

Thierry Odent, Franck Accadbled, Georges Kouréas, Maxime Cournot, Alexandre Moine, Gwénaëlle Diene, Catherine Molinas, Graziella Pinto, M. Tauber, Bruno Gomes, Jérôme Sales de Gauzy, Christophe Glorion,

Genomic variations and chromosomal abnormalities

Our goals were to determine the prevalence and estimate the evolution of spinal deformities in patients suffering from Prader-Willi syndrome; find out which kind of spine deformity predominates regarding genotype and clinical patterns; and evaluate the affect of growth-hormone treatment on the onset and progression of spinal deformities.This was a retrospective longitudinal, clinical, and radiologic study. One hundred forty-five children followed between 1980 and 2006 were studied in 2 referral centers for Prader-Willi syndrome. Genetic testing confirmed the diagnosis in 133 patients. Ninety-three patients (64%) received growth-hormone therapy. For statistical analysis, age-adjusted comparison between groups was performed by using multivariate logistic regression.Mean age of the patients was 10.2 +/- 6.2 years. Sixty-three (43.4%) patients were afflicted with scoliosis. Scoliosis frequency steadily rose with age, and a large majority of patients were affected at skeletal maturity (66.7%). Scoliosis prevalence was not affected by the genotype or by growth-hormone treatment. Patients with higher BMI values had an increased risk of developing a kyphotic deformity in association with scoliosis. We found a statistical association between kyphotic deformity and the need for surgical treatment.Scoliosis is a major concern for patients with Prader-Willi syndrome, and a regular (annual) systematic back examination is mandated. The role of growth-hormone treatment on the natural history of scoliosis could not be determined, and careful monitoring during treatment is recommended.