We have treated 101 patients with scoliosis secondary to muscular dystrophy over a 13-year period; 64 had Duchenne’s muscular dystrophy, 33 spinal muscular atrophy and four congenital muscular dystrophy.

The patients underwent a modified Luque (87) or Harrington-Luque instrumentation (14) combined with a limited Moe fusion in all except 27 cases. A mean of 13 levels was instrumented. The mean preoperative sitting Cobb angle was 84° (10 to 150) and the mean postoperative angle 40° (52% correction). Most patients (96%) were able to discard their braces and there was a high level of patient satisfaction (89.6%).

Less correction was seen for severe curves, and there was a greater recurrence of postoperative pelvic tilt in those patients not instrumented to the sacrum. Although the incidence of minor or temporary complications was high, these occurred chiefly in the early high-risk patients with very severe curves and considerable pre-existing immobility.

We reviewed retrospectively the role of monitoring of somatosensory spinal evoked potentials (SSEP) in 99 patients with neuromuscular scoliosis who had had operative correction with Luque-Galveston rods and sublaminar wiring.

Our findings showed that SSEP monitoring was useful and that a 50% decrease in the amplitude of the trace optimised both sensitivity and specificity. The detection of true-positive results was higher than in cases of idiopathic scoliosis, but the method was less sensitive and specific and there were more false-negative results. In contrast with the findings in idiopathic scoliosis, recovery of the trace was associated with a 50% to 60% risk of neurological impairment.

Only one permanent injury occurred during the use of this technique, and any temporary impairment resolved within two months.