Pellerin Philippe, Vinchon Matthieu, Guerreschi Pierre, Anastassov Yuri, Zhang Zhi Yong, Tang Xiao Jun, Alonso Nivaldo
French National Center for Rare Cranio Maxillo Facial Malformations (2008-2013).
Department of Pediatric Neurosurgery, Lille University Hospital, Lille, France.
J Craniofac Surg. 2022 Sep 1;33(6):1914-1923. doi: 10.1097/SCS.0000000000008644. Epub 2022 Mar 14.
Spatial resolution of computerized tomographic scanner has reached a level to which accurate anatomic measurements could be done in. Three-dimensional accurate measurements require a reliable referential system. In craniology landmarks are usually selected in the skull base. For craniofacial malformation the classical landmarks are of no help so the authors have used the vestibular orientation to study a series of 50 Crouzon syndrome computerized tomographic scan and compare the results (shape, position, linear mensuration, volumes…) with 122 controls in unaffected patients. The authors have confirmed Crouzon description of a high level of polymorphism in phenotypes, the authors propose an organigram to understand the abnormal growth patterns in crouzon syndrome, which lead to such consequences. This polymorphism implies that the planning of surgical treatment should be tailored for each case.
计算机断层扫描仪的空间分辨率已达到能够进行精确解剖测量的水平。三维精确测量需要一个可靠的参照系统。在颅骨学中，地标通常选在颅底。对于颅面畸形，经典地标并无帮助，因此作者利用前庭定位法研究了50例克鲁宗综合征患者的计算机断层扫描，并将结果（形状、位置、线性测量、体积等）与122例未受影响患者的对照结果进行比较。作者证实了克鲁宗对表型高度多态性的描述，作者提出了一个器官图谱来理解克鲁宗综合征中异常的生长模式，正是这种生长模式导致了这些后果。这种多态性意味着手术治疗方案应针对每个病例量身定制。
