Stabilization of Scoliosis in Subjects with Prader-Willi Syndrome Treated with GH-therapy         

L. Ragusa, M. Bianco, D. Greco, F. La Barbera, F. Scannella, P. Occhipinti, A. Costanzo, S. Buono, C. Romano

Department for Mental Retardation, Oasi Institute (IRCCS), Via Conte Ruggero,73 - 94018 Troina (EN)- ITALY

Orthopedic problems are often present in Prader-Willi syndrome (PWS) such as:  flat feet, scoliosis, knock knees, hip dysplasia, osteoporosis, patello-femoral instability, bowlegs, clubfeet, and leg-length inequality.  Scoliosis in patients with PWS has many of the clinical characteristics encountered in idiopathic scoliosis. It is present from an early age and remains stable during childhood, but progresses in 15 to 20 per cent of cases during adolescence.

Growth hormone therapy (GHT) in PWS normalizes height and increases lean body mass, both of which are beneficial to weight management. These patients should be monitored for development or acceleration of scoliosis. Our patients with PWS (3 females, 1 male) had mild to moderate (5-20 degrees) scoliosis before GHT.  There was no significant difference in scoliosis progression and spine curve measurement during subsequent GH therapy in these patients.