Coll Guillaume, Arnaud Eric, Selek Laurent, Brunelle Francis, Sainte-Rose Christian, Collet Corinne, Di Rocco Federico
Département de Neurochirurgie Pédiatrique, Hôpital Lariboisière, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
Childs Nerv Syst. 2012 Sep;28(9):1525-35. doi: 10.1007/s00381-012-1805-x. Epub 2012 Aug 8.
Though the craniovertebral junction is often abnormal in children with Crouzon's syndrome, no study had measured accurately the size of their foramen magnum (FM).
We compared the FM size (area, diameters) on computed tomography examination in 21 children with a genetically confirmed Crouzon's syndrome prior to any surgery and in 23 control children without craniofacial abnormalities. We extrapolated the growth pattern in both groups.
We found a statistically significant smaller FM area (p=0.0228), FM sagittal diameter (p=0.0287), and FM sagittal posterior diameter (p=0.0023) in children with Crouzon's syndrome. No differences were detected with regard to the transversal diameter. Hydrocephalus in children with Crouzon's syndrome was associated with a small FM area (p=0.05), small sagittal diameter (p=0.023), small sagittal posterior diameter (p=0.0173), and reduced transversal diameter (p=0.03985). No association of the aforementioned findings was found with the position of the cerebellar tonsils or the lambdoid suture functional state (open or fused). Comparable results were observed among the two genetic forms (exon 8 or 10 mutations). Concerning the growth pattern, a first phase of rapid increase and a second phase of slow increase could be recognized in all the measurements in both populations, though with some significant differences.
The growth of FM follows a biphasic pattern in both Crouzon's and control children. The sagittal diameter and the global size of the FM are mostly affected in children with Crouzon's syndrome. The small FM, especially its posterior part, is likely to play a key role in the physiopathology of hydrocephalus.
尽管克鲁宗综合征患儿的颅颈交界区常出现异常，但尚无研究准确测量过他们枕大孔（FM）的大小。
我们对21例经基因确诊的克鲁宗综合征患儿在任何手术前的计算机断层扫描检查中的枕大孔大小（面积、直径）与23例无颅面异常的对照儿童进行了比较。我们推断了两组的生长模式。
我们发现克鲁宗综合征患儿的枕大孔面积（p = 0.0228）、枕大孔矢状径（p = 0.0287）和枕大孔矢状后径（p = 0.0023）在统计学上显著较小。在横径方面未检测到差异。克鲁宗综合征患儿的脑积水与较小的枕大孔面积（p = 0.05）、较小的矢状径（p = 0.023）、较小的矢状后径（p = 0.0173）以及减小的横径（p = 0.03985）相关。未发现上述结果与小脑扁桃体位置或人字缝功能状态（开放或融合）有关。在两种基因形式（外显子8或10突变）中观察到了类似结果。关于生长模式，在两组人群的所有测量中都可识别出快速增长的第一阶段和缓慢增长的第二阶段，尽管存在一些显著差异。
克鲁宗综合征患儿和对照儿童的枕大孔生长均遵循双相模式。克鲁宗综合征患儿的矢状径和枕大孔的整体大小受影响最大。较小的枕大孔，尤其是其后部，可能在脑积水的病理生理学中起关键作用。
