Objective

To evaluate the effectiveness of use of Garches Brace (GB) on prevention and treatment of scoliosis and its impact on quality of life, in a group of patients with spinal muscular atrophy (SMA) type Ib.

Material/patients and methods

We identified 102 patients with SMA not acquiring sitting position, but acquiring head dress (type Ib SMA) followed at our center between 1975 and 2009. We reviewed the charts and identified 25 patients that were treated by GB and had a follow-up in our department of three years or more. We collected and analyzed data including functional aspects in activities of daily living, quality of life, respiratory management, radiological Cobb angle (taken in lying position without brace).

Results

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.

Discussion – conclusion

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 °Cobb angle before spinal surgery. There is not available data on the therapeutic effects of orthopedic treatment of spine in type 1b SMA patients. The Garches Brace 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. The Garches Brace is generally well tolerated in children without significant side effects.