To clarify the role of the orthopaedic surgeon in the management of
spinal muscular atrophy, the records of 130 patients were reviewed.
Seventy-three had died. Of the remaining fifty-seven, fifty were
re-examined. The clinical manifestations of the disease, particularly those
giving rise to orthopaedic problems, were studied. At the time of review
the average age of the patients was 11.5 years. Thirty-five could not walk
and all had some degree of muscle weakness, more marked proximally and in
the lower limbs. The most common medical problem was repeated respiratory
infection and the major orthopaedic problem was scoliosis, often severe.
Nine of the thirty-five patients with scoliosis had had spine fusion. Most
of them suffered some functional loss in attaining spinal stability.