Surgical treatment of vertebral deformities in neurofibromatosis.

Vertebral deformity in neurofibromatosis can present in two basic forms which determine the prognosis and treatment. In the first form, which is rare, there are no gross dystrophic changes, while in the second, which is the more common, such changes are widespread. Neurofibromatosic scoliosis where there are no signs of bone dystrophy poses no particular therapeutic problems. However, the surgical treatment of the dystrophic form is much more complex and the results far more uncertain. There is a high incidence of pseudarthrosis following posterior arthrodesis and fixation with Harrington rods, and this has shown the need for combined posterior and anterior arthrodesis in all cases of neurofibromatosic kyphoscoliosis. Furthermore, posterior arthrodesis alone does not provide protection against future relapse even when kyphosis is initially absent. Delayed kyphosis, due to subsidence of the vertebral bodies, frequently appears after six months and necessitates a second operation to reinforce the posterior arthrodesis. However, a greater guarantee of stability can be achieved simply by performing combined anterior and posterior arthrodesis at the outset.