Scoliosis in patients with Prader-Willi Syndrome.
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.


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.
Odent
Thierry
T
Department of Orthopaedic Surgery, Hôpital des Enfants-Malades Assistance-Publique Hôpitaux de Paris, Faculté de Médicine Paris Descartes, Paris, France. thierry.odent@nck.ap-hop-paris.fr
Accadbled
Franck
F
Koureas
Georges
G
Cournot
Maxime
M
Moine
Alexandre
A
Diene
Gwenaelle
G
Molinas
Catherine
C
Pinto
Graziella
G
Tauber
Maïthé
M
Gomes
Bruno
B
de Gauzy
Jérôme Sales
JS
Glorion
Christophe
C
D000293:Adolescent; D017677:Age Distribution; D000704:Analysis of Variance; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D015897:Comorbidity; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D013006:Growth Hormone / Q000627:therapeutic use; D006801:Humans; D016015:Logistic Models; D008137:Longitudinal Studies; D008297:Male; D015999:Multivariate Analysis; D011218:Prader-Willi Syndrome / Q000175:diagnosis* / Q000453:epidemiology* / Q000628:therapy; D015995:Prevalence; D011336:Probability; D011859:Radiography; D012016:Reference Values; D012189:Retrospective Studies; D012600:Scoliosis / Q000000981:diagnostic imaging* / Q000453:epidemiology* / Q000628:therapy; D012720:Severity of Illness Index; D017678:Sex Distribution
D013006:Growth Hormone