Dystrophic kyphoscoliosis in neurofibromatosis type I: a report of two cases and review of the literature.

Abstract

We report two cases of dystrophic scoliosis in neurofibromatosis, each of particular interest. In the first, kyphosis was present with vertebral rotatory subluxation but no neurologic impairment, while the second patient showed manifest paraplegia due to rapidly progressive kyphoscoliosis. The importance of early surgical stabilisation, both front and back if possible, is stressed. Very sharp curves with progressive myelopathy should not be treated with halo-femoral traction because of the potential danger of evoking permanent paraplegia.