The cervical spine in the Klippel-Feil syndrome. A report of 57 cases

We report 57 cases of the Klippel-Feil syndrome. Five had type I congenital cervical fusion, 48 type II and 4 type III. The symptoms and signs varied considerably. Patients with C2-C3 fusion often had symptoms associated with odontoid dysplasia and occipito-cervical instability. Twenty-one had progressive neurological symptoms and 19 were operated on: 3 had occiput-C3 posterior arthrodeses, 2 a single level fusion, 1 a laminectomy and anterior arthrodesis, and 13 had laminoplasties. Degenerative changes at the unfused segment and a narrow bony canal are high risk factors in the development of neurological compromise.