Current studies on orthopedic management of scoliosis in most severe SMA patients are limited to type 2 and 3 SMA with scoliosis evolving between 54 and 95°Cobbangle before spinal surgery. There is not available data on the therapeutic effects of orthopedic treatment of spine in type 1b SMA patients. This study evaluated the effectiveness of use of Garches Brace (GB) on prevention and treatment of scoliosis and its impact on quality of life, in 25 patients with spinal muscular atrophy (SMA) type Ib. Data including functional aspects in activities of daily living, quality of life, respiratory management, radiological Cobb angle (taken in lying position without brace) were collected and analysed. The GB treatment started at a mean age of 2,08 years (range 0,74-5,77) and GB was worn for a mean of 10,63 years (SD 2,55) before spinal fusion. Eighteen patients (72%) had spinal fusion, of whom 16 patients (64%) had less than 50° of Cobb angle when arriving at the age of spinal fusion, with a mean preoperative Cobb angle of 46,70° (range 0-85). Evolution of Cobb angle function to age was 2,4° per year. Sixteen patients (64%) gained mobility with an electric wheelchair and 12 patients (48%) accessed to school. The GB has shown to be an effective tool in the prevention and management of spinal deformities and in the stabilization of the pelvis. Besides, it allows stable sitting and upright head position, contributing to a better quality of life and a better accessibility to activities of daily living.
