Management of Spinal Deformities in Prader-Willi Syndrome

Summary: Fifty-one patients with genetically proved Prader-Willi syndrome were analyzed to determine the prevalence and the evolution of spinal deformities, to evaluate the impact of growth hormone treatment, body mass index and analyze the results of conservative and operative treatment. It is essential to keep body mass index values below twenty-five. Growth hormone treatment helps to diminish body mass index value and decrease the risk of scoliosis. Surgical treatment is related to significant postoperative complications.

Objectives: To determine the prevalence and the evolution of spinal deformities in patients with Prader-Willi syndrome (PWS), to evaluate the impact of growth hormone treatment and to analyse the results of conservative and operative treatments.

Methods: Data from 51 patients with genetically proved PWS were reviewed. The different types of scoliosis were classified with the SRS classification. Body mass index was calculated (BMI) and related to age and growth hormone administration. Statistical analysis was carried out with the coefficient correlation test. Chi square test was used to compare qualitative values.

Results: Twenty-seven patients were female and twenty-four were male. The prevalence of scoliosis was 52%. The age of patients at the last follow-up was 10,7 ± 6,7 years. Scoliotic deformity was more often presented in patients up to ten year (p<0 ,01). Patients with a BMI less than 25 had lower risk for scoliosis. Among the scoliotic patients seventeen (65%) were belonging in female group and nine (35%) male group. Growth hormone treatment significantly decreases patients, ten scoliosis major curvature angle, bellow fifteen degrees : 120 ± 30 observed. Eleven Cobb angles higher degrees: 310±110 braced. Five curvatures up to 500±300 trunk imbalancement surgically treated. Four of these complications postoperatively.

Discussion: Controlled weight gain is of great importance in Prader-Willi patients. It is essential to keep body mass index values below twenty-five. Growth hormone treatment helps to diminish body mass index value and to decrease the risk of scoliosis. Scoliotic deformity is more often present in patients above the age of ten. Surgical treatment is related to significant postoperative complications.