From the Chinese Academy of Medical Sciences, Peking Union Medical College, Plastic Surgery Hospital; the Division of Plastic and Reconstructive Surgery, Mayo Clinic Florida; the Division of Plastic and Reconstructive Surgery, Yale School of Medicine; and the Department of Plastic Surgery, University of São Paulo.
Plast Reconstr Surg. 2019 Jun;143(6):1233e-1243e. doi: 10.1097/PRS.0000000000005643.
Considerable craniofacial features of Crouzon syndrome are attributed to the dysmorphology of the cranial base. As cephalometric studies have focused mainly on the facial deformity, rather than the cranial base, the underlying cause of deformity is not as well understood. Therefore, the authors compared the cranial base development of Crouzon syndrome to controls to trace the timing of deformity in the cranial base and face, to analyze their temporal correlation.
Ninety computed tomographic scans were included (Crouzon, n = 36; controls, n = 54) and divided into five age subgroups. Craniofacial cephalometric measurements were analyzed by Materialise software.
The overall cranial base length in Crouzon syndrome compared with controls decreased 8 percent (p = 0.014) on average. The posterior cranial fossa shortening accounted for most of this reduction. The cranial base displaced with the distances from basion, sella, and ethmosphenoid to posterior nasal spine shortened by 21%, 18%, and 16%, respectively (all p < 0.01) during life. Although the cranial base angle on intracranial surface remains normal, the angles on facial surface narrowed were reduced.
The cranial base deformity of Crouzon syndrome consists of the whole skull base and particularly anterior skull base shortening early, leading to a compensatory widened anterior skull base. However, when this widening did not compensate fully for the rapid enlargement of the brain, the posterior skull base displaced inferiorly and became kyphotic. The cranial base deformity develops sequentially anterior to posterior in a probable cascade of influence pattern.
CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.
颅面特征明显的颅缝早闭综合征（Crouzon 综合征）的颅底畸形归因于颅底发育不良。由于头影测量研究主要集中在面部畸形，而不是颅底，因此对畸形的潜在原因了解得还不够。因此，作者将 Crouzon 综合征患者的颅底发育与对照组进行比较，以追溯颅底和面部畸形的发生时间，分析其时间相关性。
共纳入 90 例 CT 扫描（Crouzon 组 36 例，对照组 54 例），并分为 5 个年龄亚组。使用 Materialise 软件分析颅颌面头影测量值。
与对照组相比，Crouzon 综合征患者的颅底总长度平均缩短了 8%（p = 0.014）。其中后颅窝缩短占大部分。颅底在生命过程中随以下各点与后鼻孔棘的距离缩短而发生移位：基线、蝶鞍和筛骨垂直板分别缩短 21%、18%和 16%（均 p < 0.01）。尽管颅底内表面的角度仍正常，但面颅底角度变窄。
Crouzon 综合征患者的颅底畸形包括整个颅底，尤其是早期颅前底缩短，导致颅前底代偿性增宽。然而，当这种增宽不能完全补偿大脑的快速增大时，颅后底向下移位并呈角状后凸。颅底畸形以前后顺序发展，可能呈级联影响模式。
临床问题/证据等级：风险，II 级。
