The Journal of Bone and Joint Surgery, Vol 71, Issue 7 1066-1074, Copyright © 1989 by Journal of Bone and Joint Surgery, Inc
Progression of scoliosis in Duchenne muscular dystrophy
AD Smith, J Koreska and CF Moseley
Hospital for Sick Children, Toronto, Ontario, Canada.
We reviewed the clinical charts and spinal radiographs of fifty-one boys
who had Duchenne muscular dystrophy, had not had surgical treatment of the
spine, and had been followed until death. All had scoliosis. None of the
following variables was useful in predicting which curves would become
severe: age when the patient initially walked, age when he ceased walking,
age at onset of spinal collapse, surgical release of the iliotibial bands,
or age at the time of death. Radiographs were made within eighteen months
before death for thirty-three patients; in thirty-one of them, the final
curve exceeded 40 degrees and in seventeen, 90 degrees. For the remaining
eighteen patients, final radiographs were made more than eighteen months
before death; at that time, eight of them already had a curve of more than
90 degrees. Although there was a relationship between extension of the
lumbar spine and severity of scoliosis at the time of final follow-up,
early maintenance of the lumbar spine in extension rarely prevented the
development of a severe curve. For most of the patients who had a severe
curve, sitting was difficult and was accompanied by breakdown of the skin
and pain. When a patient's curve exceeded 35 degrees, the vital capacity
usually was less than 40 per cent of the predicted normal value. Therefore,
when walking becomes impossible for boys who have Duchenne muscular
dystrophy, routine spinal arthrodesis should be considered.
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