LONGITUDINAL-STUDY OF SPINAL DEFORMITY IN DUCHENNE MUSCULAR-DYSTROPHY

To investigate the natural course of the spinal deformity in Duchenne muscular dystrophy (DMD) and its clinical relevance, longitudinal seri es of spinal radiographs and medical records of 46 patients with DMD w ere reviewed. The natural course of the deformity was classified into three types; type 1 (n = 21), unremittent progression of scoliosis wit h kyphosis; type 2 (n = 18), transition from kyphosis to lordosis befo re age 15 years; and type 3 (n = 7), less deformity without prominent longitudinal changes. Age at loss of ambulatory ability was not a pred ictor of type. Neither was the age at which the Cobb angle was 30-degr ees correlated with the rate of subsequent progression. Because the sp inal deformity always progresses, we consider spinal surgery justifiab le in type 1, when a certain strict indication exists, such as spinal deformity >30-degrees and age <15 years in patients with >35% predicte d value of vital capacity. In type 2, operation may be necessary in pa tients in whom Cobb angle will progress unremittently. There is no sur gical indication for patients with type 3.