Cervical instability in skeletal dysplasia Report of 6 surgically fused cases

Six patients with skeletal dysplasias complicated by deformity or instability of the cervical spine were reviewed. Three patients had congenital spondyloepiphyseal dysplasia and 1 each suffered from Morquio's syndrome, pseudoachondroplasia, and chondrodystrophia calcificans congenita. Four patients had myelopathy of varying severity from slight weakness of one leg to tetraplegia. At a mean age of 6 (3-11) years, they underwent posterior fusion and immobilization in a halo brace. In all the cases the extent of postoperative neurologic recovery was, however, limited. Our cases illustrate the need of early diagnosis and fusion of cervical instability.