目的 探讨颅-锁骨发育不全综合征（cleidocranial dysplasis, CCD）的临床及影像表现，以提高对本病的认识。 方法 回顾性分析本院确诊的2例CCD典型病例的临床及影像表现，结合文献对该病的临床特征及影像表现进行总结 分析。结果 2例患者有不同程度的颅骨、牙齿、锁骨发育障碍，主要表现为颅骨发育异常、牙颌面畸形、锁骨发育 不全和“锥形”胸等。结论 CCD具有较特征性临床及影像表现，有助于临床诊断。

Objective To investigate the clinical features and radiologic findings of cleidocranial dysplasis(CCD). Methods Two patients with a clinically verified CCD were reviewed retrospectively. Results All patients expressed developmental abnormality of crania, teeth and clavicle. The radiologic features of CCD are:①multiple impacted permanent and supernumerary teeth, hypoplastic maxillary sinus and slender coronoid process. ②open sutures of the skull, small maxillary sinuses, missing nasal bone, numerous wormian bones, large fontanelles, hypoplastic maxilla, and prognathic mandible. ③dysplasia of both the clavicles and cone shaped thorax. Conclusion Clinical and radiologic characteristics of CCD are distinctive in certain extent, wich help the diagnosis.