THE CERVICAL-SPINE IN CROUZON SYNDROME

Study Design. All cervical spine radiographs of 44 patients with Crouz on syndrome treated at Great Ormond Street Hospital during the past 10 years were studied. Objectives. To assess the incidence and pattern o f cervical spine abnormalities of patients with a confirmed diagnosis of Crouzon syndrome, but particularly regarding progressive fusion. Su mmary of Background Data. Previous studies into the cervical spine ano malies in those with Crouzon syndrome have shown an increased incidenc e of congenital abnormalities compared with the normal population. The re is some suggestion from previous studies that cervical spine fusion s are progressive in nature. Methods. All radiographs were reviewed by the craniofacial team, along with a single pediatric radiologist with experience in assessment of skeletal dysplasias. Results. Radiologic abnormalities included ''butterfly'' vertebrae and fusions of the bodi es and the posterior elements. Evidence of fusion was present in eight of 44 (18%) of patients. C2-C3 and C5-C6 were almost equally affected . Black fusions involving multiple vertebrae were seen. Analysis of se quential studies in 16 patients showed evidence of progression in five . Conclusions. These results reveal an incidence of fusions that is lo wer than reported previously. There is radiologic evidence from serial studies that the fusions are progressive, and because these patients are children, the fusion process may not be complete, which may accoun t for the lower incidence of fusions than in previous studies. The pat tern of fusions is different from that in earlier studies, which may b e a result of the method of diagnosis because this population is less likely to include atypical forms of other syndromes [which have a high er incidence of cervical fusions, particularly at C2-C3). Regarding ot her congenital anomalies, it appears that butterfly vertebrae are espe cially prevalent in association with Crouzon syndrome.