Abstract
BackgroundPatients with Duchenne muscular dystrophy (DMD) often develop scoliosis that progresses rapidly after loss of ambulation. Management of scoliosis is crucial because it affects both life expectancy and quality of life of patients with DMD. Spinal orthosis attempts to prevent or delay scoliosis using spinal support at three points of the controlling mechanism; the curve should be flattened by the pressure. Therefore, it is assumed that spine flexibility could be a significant influencing factor for the effectiveness of braces. Hence, we attempted to investigate the flexibility of scoliosis in non-ambulant patients with DMD.MethodsWe reviewed the medical records of 273 boys who were genetically identified as having DMD, and finally, 50 boys with serial records of radiographs after loss of ambulation were finally enrolled. And among them, only 31 patients developed scoliosis. Spine radiographs in sitting and supine positions were also reviewed to obtain Cobb angle, curve flexibility, and pelvic obliquity. Flexibilities (%) were calculated by the difference in angles between the sitting and supine positions divided by the angle at the sitting position, multiplied by 100.ResultsAmong 31 boys who had scoliosis, all but 2 boys with curves went through a sequential course of 1) no scoliosis, 2) nonstructural scoliosis, when scoliosis was only measurable in the sitting position, and 3) structural scoliosis, when scoliosis was also detectable in the supine position. Flexibility decreased each year after detection of scoliosis in those who developed scoliosis the first year, from 75.5 ± 5.0% to 57.1 ± 10.5% and to 49.1 ± 10.0% (mean ± standard deviation). Spinal flexibility was significantly correlated with curve magnitude of scoliosis in both sitting and supine position (p < 0.05, respectively).ConclusionsThere is a period of fully reducible curve in DMD patients at the initial stage of scoliosis. Afterward, as spinal curve progresses, flexibility decreases over time. To detect the scoliosis when the curve is fully reducible, scoliosis curve in DMD patients should be evaluated dynamically, including radiographs of at least in two different positions.
Highlights
Patients with Duchenne muscular dystrophy (DMD) often develop scoliosis that progresses rapidly after loss of ambulation
Scoliosis is a frequent complication of Duchenne muscular dystrophy (DMD) that progresses rapidly, in the nonambulatory stage of the disease [1,2,3,4,5,6]
Pelvic obliquity is thought to be a mechanism of compensation for scoliosis [7]
Summary
Patients with Duchenne muscular dystrophy (DMD) often develop scoliosis that progresses rapidly after loss of ambulation. We attempted to investigate the flexibility of scoliosis in non-ambulant patients with DMD. Pelvic obliquity is thought to be a mechanism of compensation for scoliosis [7]. These deformities in the musculoskeletal system together make sitting difficult, limiting the use of upper extremities and hampering activities of daily living. Rapid progression of spinal deformity leads to a deterioration in pulmonary function [3, 5, 6]. According to Hsu et al, in DMD patients whose spinal curves exceeded 40 degrees, vital capacity diminished by 12 to 16% [10]. It is important to prevent, or delay spinal deformity as it leads to compromise of respiratory function
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