Scoliosis management in Duchenne muscular dystrophy: prospective study of modified Jewett hyperextension brace

Abstract

The management of scoliosis in Duchenne muscular dystrophy is not standardized. Some centers offer no treatment, some provide customized body jackets or wheelchair inserts, and others recommend early surgical spinal fusion. Spinal orthoses are frequently prescribed at significant cost and inconvenience to the patient, with little evidence as to effectiveness in preventing scoliosis. This paper reports the preliminary findings of 22 patients involved in a study assessing the usefulness of a modified Jewett hyperextension brace. Seven brace wearers and 15 controls were entered into the study. The preliminary results indicate a mean curvature of 23.6 degrees (+/- 12.8 degrees) in the experimental group and 35.7 degrees (+/- 24.8 degrees) in the controls. Over an observation period ranging from two to seven years, the wearers have shown an average curve progression of 8.8 degrees/yr while the control group progressed at 11.2 degrees/yr. This difference is not considered statistically significant. We conclude that wearing the brace minimally slows scoliosis progression, but does not ultimately prevent a significant curve.