H-R Weiss1, S Bohr. 1. Asklepios Katharina Schroth Spinal Deformities Rehabilitation Centre, Korczakstr. 2, 55566 Bad Sobernheim, Germany. hr.weiss@asklepios.com
Abstract
UNLABELLED: Patients with Prader-Willi syndrome often suffer from scoliosis of major degrees. Due to current literature surgical intervention seems the gold standard of treatment although the rate of complications in this condition are reported to be significantly higher than in patients with Adolescent Idiopathic Scoliosis. Purpose of this study was to reveal the effects of conservative treatment in this rare patient population. MATERIALS AND METHODS: A case series of patients with this condition has been investigated to estimate as to whether Prader-Willi patients with scoliosis may benefit from conservative scoliosis management. 9 Patients with this condition have been found in our out-patient database. 5 of these retarded patients (3 girls, two boys) today are 19 years and older and therefore are without any significant residual growth. Average Cobb angle was 47 degrees (34 - 66 degrees) at 12 years, average observation time was 6.4 years. RESULTS: Two of the five patients progressed. Average Cobb angle after follow-up was 52 degrees. No progression beyond 70 degrees has been found after cessation of growth. In one patient the curve deteriorated clearly after reducing brace wearing time and therefore was due to non-compliance. CONCLUSIONS: Stabilisation of scoliosis due to Prader-Willi syndrome is possible by means of conservative management. To expose this patient population to the risks of surgical management seems not to be justified.
UNLABELLED: Patients with Prader-Willi syndrome often suffer from scoliosis of major degrees. Due to current literature surgical intervention seems the gold standard of treatment although the rate of complications in this condition are reported to be significantly higher than in patients with Adolescent Idiopathic Scoliosis. Purpose of this study was to reveal the effects of conservative treatment in this rare patient population. MATERIALS AND METHODS: A case series of patients with this condition has been investigated to estimate as to whether Prader-Willipatients with scoliosis may benefit from conservative scoliosis management. 9 Patients with this condition have been found in our out-patient database. 5 of these retardedpatients (3 girls, two boys) today are 19 years and older and therefore are without any significant residual growth. Average Cobb angle was 47 degrees (34 - 66 degrees) at 12 years, average observation time was 6.4 years. RESULTS: Two of the five patients progressed. Average Cobb angle after follow-up was 52 degrees. No progression beyond 70 degrees has been found after cessation of growth. In one patient the curve deteriorated clearly after reducing brace wearing time and therefore was due to non-compliance. CONCLUSIONS: Stabilisation of scoliosis due to Prader-Willi syndrome is possible by means of conservative management. To expose this patient population to the risks of surgical management seems not to be justified.