Scoliosis in Duchenne muscular dystrophy

Abstract

The x-linked Duchenne muscular dystrophy (DMD) is the most frequent generalized muscle disorder arising from a lack of the sarcolemmic protein "dystrophin". Patients with DMD develop in the majority a progressive scoliosis when they cease walking and/or standing at the age of 10 years and become confined to a wheelchair. Increasing muscle weakness leads to a progression of the curvature, the pelvic tilt and problems in sitting. Together with the simultaneous progressive weakness of the respiratory muscles a restrictive pulmonary insufficiency will occur. Surgical stabilization of the spine (> 20 degrees Cobb, forced vital capacity > 35%) by an adequate multisegmental instrumentation enabling early mobilization is now the treatment of choice. However, orthotic treatment may offer an acceptable compromise in exceptional cases, if the patient rejects surgical intervention or is in the late (inoperable) stages of the disease. Such a treatment is superior to a primary sitting support provision with insufficient possibilities of correction. The authors' experiences with 48 scoliosis orthoses made for 28 patients with DMD are reported. A "double plaster" cast has emerged as the best method to optimize adaption, especially in severe curvatures and the time taken for manufacturing the orthosis. A great deal of experience, patience and the consideration of the patients' individual demands are inevitable for a successful orthotic treatment.