Kreiborg S, Cohen M M, Skovby F
Department of Pediatric Dentistry, Royal Dental College, Copenhagen, Denmark.
Proc Finn Dent Soc. 1991;87(1):183-8.
The dysmorphic growth patterns of the craniofacial skeleton are summarized in five patients with the Proteus syndrome. Two different modes of abnormal growth are present. One mode involves focal overgrowth of membrane bones, producing multiple hyperostoses which result in progressive craniofacial disfigurement and asymmetry. The second mode involves overgrowth of cartilage in the condyle, resulting in dentofacial asymmetry. The phenotype of the craniofacial skeleton results from both processes. In terms of appearance, the mode involving hyperostosis seems more dramatic in most cases than the mode involving cartilage.
本文总结了5例患有变形综合征患者的颅面骨骼畸形生长模式。存在两种不同的异常生长模式。一种模式涉及膜性骨的局灶性过度生长，产生多个骨质增生，导致进行性颅面畸形和不对称。第二种模式涉及髁突软骨过度生长，导致牙颌面不对称。颅面骨骼的表型是这两种过程共同作用的结果。在外观方面，涉及骨质增生的模式在大多数情况下似乎比涉及软骨的模式更为显著。
