Imaging in spine and spinal cord malformations

Imaging in spine and spinal cord malformations
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DOI:
10.1016/j.ejrad.2003.10.015
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发表时间:
2004-05-01
影响因子:
3.3
通讯作者:
Tortori-Donati, P
Tortori-Donati, P
中科院分区:
医学3区
文献类型:
--
作者:
Rossi, A;Biancheria, R;Tortori-Donati, P

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脊柱和脊髓畸形统称为脊髓闭合不全。它们是由原肠胚形成(2-3周)、初级神经胚形成(3-4周)和次级神经胚形成(5-6周)的早期胚胎阶段发生的缺陷引起的。脊柱闭合不全分为开放性脊柱闭合不全(OSDs)和闭合性脊柱闭合不全(CSD),开放性脊柱闭合不全(OSDs)通过皮肤缺损暴露异常神经组织,闭合性脊柱闭合不全(CSD)存在对潜在畸形的连续皮肤覆盖。开放性脊髓闭合不全主要包括脊髓脊膜膨出和其他罕见的异常,如脊髓膨出和半脊髓(脑膜)膨出。闭合性脊柱闭合不全根据与下背部皮下肿块的相关性进一步分类。伴有肿块的闭合性脊髓闭合不全表现为脂肪脊髓膨出、脂肪脊髓脊膜膨出、脊膜膨出和脊髓囊膨出。无肿块的闭合性脊柱缝闭包括简单缝闭状态(终丝紧密、丝状和硬膜内脂肪瘤、持续性终脑室和真皮窦)和复杂缝闭状态。后一类还包括中线脊索整合缺陷(基本上以脊髓纵裂为代表)和节段性脊索形成缺陷(以尾部发育不全和脊髓节段性发育不全为代表)。磁共振成像(MRI)是成像这些复杂的异常的首选方式。使用上述分类方案对诊断有很大帮助。(C)2003爱思唯尔爱尔兰有限公司保留所有权利。
Spinal and spinal cord malformations are collectively named spinal dysraphisms. They arise from defects occurring in the early embryological stages of gastrulation (weeks 2-3), primary neurulation (weeks 3-4), and secondary neurulation (weeks 5-6). Spinal dysraphisms are categorized into open spinal dysraphisms (OSDs), in which there is exposure of abnormal nervous tissues through a skin defect, and closed spinal dysraphisms (CSD), in which there is a continuous skin coverage to the underlying malformation. Open spinal dysraphisms basically include myelomeningocele and other rare abnormalities such as myelocele and hemimyelo(meningo)cele. Closed spinal dysraphisms are further categorized based on the association with low-back subcutaneous masses. Closed spinal dysraphisms with mass are represented by lipomyelocele, lipomyelomeningocele, meningocele, and myelocystocele. Closed spinal dysraphisms without mass comprise simple dysraphic states (tight filum terminale, filar and intradural lipomas, persistent terminal ventricle, and dermal sinuses) and complex dysraphic states. The latter category further comprises defects of midline notochordal integration (basically represented by diastematomyelia) and defects of segmental notochordal formation (represented by caudal agenesis and spinal segmental dysgenesis). Magnetic resonance imaging (MRI) is the preferred modality for imaging these complex abnormalities. The use of the aforementioned classification scheme is greatly helpful to make the diagnosis. (C) 2003 Elsevier Ireland Ltd. All rights reserved.