COMPARATIVE 3-DIMENSIONAL ANALYSIS OF CT-SCANS OF THE CALVARIA AND CRANIAL BASE IN APERT AND CROUZON SYNDROMES
COMPARATIVE 3-DIMENSIONAL ANALYSIS OF CT-SCANS OF THE CALVARIA AND CRANIAL BASE IN APERT AND CROUZON SYNDROMES
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DOI：
10.1016/s1010-5182(05)80478-0
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发表时间：
1993-07-01
影响因子：
3.1
通讯作者：
VANNIER, MW
中科院分区：
文献类型：
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作者：
KREIBORG, S;MARSH, JL;VANNIER, MW
本研究的目的是通过计算机断层扫描（CT）的三维（3 - D）重建来描述和分析阿佩尔（Apert）和克鲁宗（Crouzon）颅骨。研究纳入了12名阿佩尔患者和19名克鲁宗综合征患者。年龄范围为0至23岁。所有的CT扫描均按照相同的方案进行，层厚为2毫米或4毫米，颅面区域的三维重建包括正中矢状面和水平切面。记录了颅骨和颅底的一些定性特征，并在三维模型上测量了颅底角。我们的研究结果表明，阿佩尔综合征和克鲁宗综合征在颅骨发育方面有很大差异，其畸形高度依赖于年龄。我们认为，软骨异常，尤其是在前颅底，从子宫内极早期的生命阶段就在阿佩尔综合征的颅骨发育中起主要作用。然而，出生后观察到的一些颅骨异常是由由此产生的畸形和代偿性生长导致的，并且可能因早期颅骨变形而加重。克鲁宗综合征的主要异常似乎是骨缝和软骨结合过早融合。根据出生时和婴儿早期的发现，这种融合似乎在胎儿期相对较晚发生。克鲁宗患者的成人颅骨形态可以通过由此产生的畸形和代偿性生长变化来解释。在这两种综合征中都提倡尽早松解冠状缝区域并推进额骨，但原因略有不同。在阿佩尔综合征中，这种治疗的主要指征是减少颅骨和颅底进一步的畸形生长变化，而考虑到婴儿早期发现的较大的中线颅骨缺损，颅内压升高的风险较低。相比之下，克鲁宗综合征的新生儿会出现多处骨缝融合和软骨结合融合，并且通常需要早期手术以预防或治疗颅内压升高。
The purpose of this study is to describe and analyze Apert and Crouzon skulls from three-dimensional (3-D) reconstructions of CT-scans. 12 Apert patients and 19 with Crouzon syndrome were included in the study. The age range was 0 to 23 years. All CT-scannings were carried out according to the same protocol with a slice thickness of 2 or 4 mm and 3-D reconstructions of the craniofacial region included midsagittal and horizontal cuts. A number of qualitative characteristics of the calvaria and cranial base were recorded and the cranial base angle was measured on the 3-D models.Our results showed that Apert and Crouzon syndromes are very different in cranial development and their dysmorphology is highly age dependent. We suggest that cartilage abnormalities, especially in the anterior cranial base, play a primary role in cranial development in the Apert syndrome from very early intrauterine life. Several cranial anomalies observed postnatally, however, are caused by the resultant dysmorphic and compensatory growth and are probably compounded by early cranial deformation. The primary abnormality in Crouzon syndrome appears to be premature fusion of sutures and synchondroses. Based on the findings at birth and early infancy it would seem that such fusions occur relatively late in fetal life. The adult cranial form in Crouzon's patients is explainable by resultant dysmorphic and compensatory growth changes.Very early release of the coronal suture areas with advancement of the frontal bone is advocated in both syndromes but for somewhat different reasons. In Apert syndrome, the primary indication for such treatment is to reduce further dysmorphic growth changes in the calvaria and cranial base, whereas the risk for increased intracranial pressure is low considering the large midline calvarial defect found in early infancy. In contrast, newborns with Crouzon syndrome develop multiple suture synostoses and fused synchondroses, and early surgery is most often indicated to prevent or treat increased intracranial pressure.