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成人 Chiari I 型畸形:后颅窝的形态学分析

Chiari type I malformations in adults: a morphometric analysis of the posterior cranial fossa.

作者信息

Aydin Sabri, Hanimoglu Hakan, Tanriverdi Taner, Yentur Ercument, Kaynar Mehmet Yasar

机构信息

Department of Neurosurgery, Cerrahpasa Medical Faculty, Istanbul University, Istanbul 34301, Turkey.

出版信息

Surg Neurol. 2005 Sep;64(3):237-41; discussion 241. doi: 10.1016/j.surneu.2005.02.021.

Abstract

OBJECTIVE

Chiari type I malformation (CMI) is a congenital disorder characterized by caudal displacement of the cerebellar tonsils through the foramen magnum into the spinal canal. Recent studies suggest that overcrowding in the posterior cranial fossa (PCF) because of underdeveloped bony structures in the intrauterine life is the main cause of this malformation. For this reason, the authors want to contribute to the current literature, which focuses on bone abnormalities in the PCF in patients with CMIs.

METHODS

We examined a retrospective cohort of 60 adult patients with CMIs, and multiple measurements were made on magnetic resonance imaging. The results were compared to 30 healthy adult control subjects. Mann-Whitney U test was used as a statistical method.

RESULTS

All measurements except mean anteroposterior diameter of the foramen magnum were reduced in patients compared to control. An increase in the anteroposterior mid-sagittal distance of the foramen magnum in patients reached statistically significant difference compared to control. All patients had tonsillar herniation at least 5 mm below the plane of the foramen magnum. Chiari type I malformation in this study was associated with syringomyelia in 46 patients.

CONCLUSION

This study with a limited number of patients suggests that the bony components of the PCF are not developed fully, supporting the current concept that CMI is a disorder of the para-axial mesoderm.

摘要

目的

Chiari I型畸形(CMI)是一种先天性疾病,其特征是小脑扁桃体通过枕骨大孔向椎管尾侧移位。最近的研究表明,由于子宫内生活中骨结构发育不全导致后颅窝(PCF)拥挤是这种畸形的主要原因。因此,作者希望为当前聚焦于CMI患者PCF骨异常的文献做出贡献。

方法

我们检查了60例成年CMI患者的回顾性队列,并在磁共振成像上进行了多项测量。将结果与30名健康成年对照者进行比较。采用曼-惠特尼U检验作为统计方法。

结果

与对照组相比,患者除枕骨大孔平均前后径外的所有测量值均降低。患者枕骨大孔前后矢状中距离的增加与对照组相比达到统计学显著差异。所有患者的扁桃体疝均至少低于枕骨大孔平面5毫米。本研究中的Chiari I型畸形与46例患者的脊髓空洞症相关。

结论

这项患者数量有限的研究表明,PCF的骨成分发育不完全,支持了CMI是一种轴旁中胚层疾病的当前概念。

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