Cinalli G, Renier D, Sebag G, Sainte-Rose C, Arnaud E, Pierre-Kahn A
Department of Pediatric Neurosurgery, Hôpital Necker-Enfants Malades, Université René Descartes, Paris, France.
J Neurosurg. 1995 Oct;83(4):575-82. doi: 10.3171/jns.1995.83.4.0575.
The incidence of chronic tonsillar herniation (CTH) was evaluated with magnetic resonance imaging in 44 patients with Crouzon's syndrome and 51 with Apert's syndrome; the incidence was 72.7% in Crouzon's syndrome and 1.9% in Apert's syndrome. All the patients with Crouzon's syndrome and progressive hydrocephalus had CTH, but of 32 individuals with Crouzon's syndrome and CTH, only 15 had progressive hydrocephalus. Five patients with Apert's syndrome were treated for progressive hydrocephalus; none had CTH. The patterns of suture closure in these two groups of patients were studied, and significant differences in coronal, sagittal, and lambdoid sutures were found between patients with Crouzon's and Apert's syndromes. In Crouzon's syndrome, significant differences in the pattern of lambdoid suture closure were found between the groups with and without CTH; in the group with CTH, the lambdoid closure appeared earlier. The authors propose that the high incidence of individuals with CTH who have Crouzon's syndrome is related to the premature synostosis of the lambdoid suture in the first 24 months of age.
采用磁共振成像对44例克鲁宗综合征患者和51例阿佩尔综合征患者的慢性扁桃体疝（CTH）发生率进行评估；克鲁宗综合征患者的发生率为72.7%，阿佩尔综合征患者的发生率为1.9%。所有患有克鲁宗综合征和进行性脑积水的患者均有CTH，但在32例患有克鲁宗综合征和CTH的个体中，只有15例有进行性脑积水。5例阿佩尔综合征患者因进行性脑积水接受治疗；均无CTH。研究了这两组患者的缝线闭合模式，发现克鲁宗综合征和阿佩尔综合征患者在冠状缝、矢状缝和人字缝方面存在显著差异。在克鲁宗综合征中，有CTH组和无CTH组在人字缝闭合模式上存在显著差异；在有CTH组中，人字缝闭合出现得更早。作者提出，患有克鲁宗综合征的CTH个体发生率高与1岁前24个月人字缝过早融合有关。
