Spinal stabilisation in Duchenne muscular dystrophy

Royal Manchester Children's Hospital, England.

Of 55 patients with Duchenne muscular dystrophy offered surgical stabilisation of the spine, 32 accepted and 23 refused. We compared both groups pre-operatively and at six-month intervals in respect of survival, forced vital capacity, peak expiratory flow rate and severity of scoliosis. In the nonoperated patients, the forced vital capacity deteriorated by a mean of 8% per annum; in the operated group it remained static for 36 months and diminished slightly thereafter. Spinal stabilisation resulted in an improvement in the peak expiratory flow rate which was maintained for up to five years. In the nonoperated patients the scoliosis progressed from a mean of 37 degrees to a mean of 89 degrees at five years; in the stabilised spines it was improved from a mean of 47 degrees to a mean 34 degrees at five years. There was significantly improved survival in the patients who had undergone spinal stabilisation.