The natural history, associated anomalies, and response to operative and
nonoperative treatment were reviewed in 102 patients with neurofibromatosis
and spine deformity. Eighty patients were found to have curvatures
associated with dystrophic changes in the vertebrae and ribs. The presence
of dystrophic changes such as rib penciling, spindling of the transverse
processes, vertebral scalloping, severe apical vertebral rotation,
foraminal enlargement, and adjacent soft-tissue neurofibromas was found to
be highly significant in prognosis and management. Brace treatment of
dystrophic curves was unsuccessful. Posterior fusion, with or without
internal fixation, was the procedure of choice for problems due purely to
scoliosis. Patients with dystrophic kyphoscoliosis required both anterior
and posterior fusion to achieve stability. Sixteen patients had compression
of the spinal cord or cauda equina.