锁骨颅骨发育不全综合征(CCD)的牙面畸形特征分析

Morphologic Characteristics of Dental and Face in Cleidocranial Dysplasia(CCD).

目的:分析锁骨颅骨发育不全综合征牙面畸形的表现.方法:收集15年(1990～2004年)诊断为锁骨颅骨发育不全综合征(CCD)患者.其中男性2例、女性2例,就诊时年龄12～18岁,平均年龄14.2岁.所有病例均填写病历、询问其囟门关闭情况,并拍摄胸片,上下颌骨曲面断层片,头颅正、侧位片位,TMJ片、面(牙合)像.结果:1)4例均有多生牙,2)乳牙滞留,3)颌骨发育异常,下颌骨发育过度、上颌骨正常或发育不足,下颌肥大、反(牙合)面型.牙槽骨较一般致密,局部区域骨小梁排列致密.4)4例患者均表现面部异常,头大面小,面下1/3短.眶间距较正常人大且颧弓突出、下弯.结论:分析锁骨颅骨发育不全综合征牙面畸形的表现有助于临床诊断.

Objective： Cleidocranial dysplasia （CCD） is a rare autosomal dominant disorder characterised by the defects of bone and tooth development. This report was to study the morphologic characteristics of dental and face in cleidocranial dysplasia（CCD）. Methods： 4 CCD patients were collected in the past 15 years, panoramic photo and face photo were taken. Results： Impacted supernumerary teeth, delayed tooth eruption, hypoplasia tooth, and the absence of cellular cementum formation were exposed in the 4 CCD patients. Conclusion： Morphologic characteristics of dental and face were helpful to the diagnose of CCD.