The cervical spine in Crouzon syndrome
The cervical spine in Crouzon syndrome
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DOI：
10.1097/00007632-199702150-00009
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发表时间：
1997-02-15
期刊：
影响因子：
3
通讯作者：
Jones, BM
中科院分区：
文献类型：
--
作者：
Anderson, PJ;Hall, C;Jones, BM
研究设计.本文分析了近10年来在大奥蒙德街医院治疗的44例Crouzon综合征患者的颈椎X线资料。评估确诊为Crouzon综合征的患者颈椎异常的发生率和模式，特别是关于进行性融合。先前对Crouzon综合征患者颈椎异常的研究表明，与正常人群相比，先天性异常的发生率增加。之前的研究表明，颈椎融合本质上是渐进的。方法。所有的X线片均由颅面小组和一名具有骨骼发育不良评估经验的儿科放射科医师沿着进行审查。放射学异常包括“蝴蝶"椎骨和椎体与后部结构融合。44例患者中有8例（18%）存在融合证据。C2-C3和C5-C6几乎同样受到影响。可见累及多个椎骨的黑色融合。对16例患者进行的序贯研究分析显示，5例患者有进展的证据。这些结果显示融合的发生率低于先前报告的发生率。系列研究的影像学证据表明融合是进行性的，由于这些患者是儿童，融合过程可能不完全，这可能是融合发生率低于既往研究的原因。融合的模式与早期研究不同，这可能是诊断方法的结果，因为该人群不太可能包括其他综合征的非典型形式[宫颈融合的发生率较高，特别是在C2-C3）。关于其他先天性异常，蝶形椎骨似乎与Crouzon综合征相关特别普遍。
Study Design. All cervical spine radiographs of 44 patients with Crouzon syndrome treated at Great Ormond Street Hospital during the past 10 years were studied.Objectives. To assess the incidence and pattern of cervical spine abnormalities of patients with a confirmed diagnosis of Crouzon syndrome, but particularly regarding progressive fusion.Summary of Background Data. Previous studies into the cervical spine anomalies in those with Crouzon syndrome have shown an increased incidence of congenital abnormalities compared with the normal population. There is some suggestion from previous studies that cervical spine fusions 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 bodies 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 sequential studies in 16 patients showed evidence of progression in five.Conclusions. These results reveal an incidence of fusions that is lower 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 account for the lower incidence of fusions than in previous studies. The pattern of fusions is different from that in earlier studies, which may be a result of the method of diagnosis because this population is less likely to include atypical forms of other syndromes [which have a higher incidence of cervical fusions, particularly at C2-C3). Regarding other congenital anomalies, it appears that butterfly vertebrae are especially prevalent in association with Crouzon syndrome.