Published online July 7, 2008
PEDIATRICS (doi:10.1542/peds.2007-3487)
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ARTICLE

Scoliosis in Patients With Prader-Willi Syndrome

Thierry Odent, MDa, Franck Accadbled, MD, PhDb, Georges Koureas, MDa, Maxime Cournot, MDc, Alexandre Moine, MDb, Gwenaelle Diene, MDd, Catherine Molinas, MDd, Graziella Pinto, MDe, Maïthé Tauber, MDd, Bruno Gomes, MBBSf, Jérôme Sales de Gauzy, MDb and Christophe Glorion, MDa

a Department of Orthopaedic Surgery
e Centre de Référence Pour le Syndrome de Prader-Willi, Department of Endocrinology, Hôpital des Enfants-Malades Assistance-Publique Hôpitaux de Paris, Faculté de Médicine Paris Descartes, Paris, France
b Department of Orthopaedic Surgery
d Centre de Référence Pour le Syndrome de Prader-Willi, Department of Endocrinology, Hôpital des Enfants, CHU Toulouse, Toulouse, France
c Department of Epidemiology, INSERM U558, CHU Toulouse, Toulouse, France
f Department of Orthopaedic Surgery, Royal Newcastle Centre, Newcastle, Australia

OBJECTIVE. 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.

PATIENTS AND METHODS. 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.

RESULTS. 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.

CONCLUSIONS. 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.

Key Words: growth and nutrition • growth-hormone treatment • Prader-Willi syndrome • scoliosis

Abbreviations: PWS—Prader-Willi syndrome • GH—growth hormone • IQR—interquartile range • SG—scoliosis group • NSG—nonscoliosis group

Accepted Apr 11, 2008.