McComb J G
Division of Neurosurgery, Children's Hospital Los Angeles, CA, USA.
Semin Pediatr Neurol. 1997 Sep;4(3):156-66. doi: 10.1016/s1071-9091(97)80034-4.
Developmental lesions of the central nervous system with failure of normal midline fusion are often referred to as being dysraphic and vary from inapparent and insignificant to a massive deformity incompatible with survival. Several different schemata are used to classify this wide variety and often complex set of malformations; however, the nomenclature is confusing and even contradictory. As most of these congenital lesions of clinical significance involve an aberration in the formation of the neural tube, it is suggested that the term neural tube defects (NTD) be used to characterize this entire group of anomalies. From a practical clinical standpoint, NTD can be subdivided into three main groupings: open spinal NTD, closed spinal NTD, and cranial NTD. This article briefly covers the epidemiology, embryology, classification, clinical presentation, and management of this group of congenital lesions.
中枢神经系统发育性病变伴正常中线融合失败常被称为神经管闭合不全,其严重程度不一,从不易察觉和无足轻重到严重畸形以至无法存活。有几种不同的分类方法用于对这一广泛且常复杂的畸形组进行分类;然而,命名法既令人困惑又相互矛盾。由于这些具有临床意义的先天性病变大多涉及神经管形成异常,因此建议使用术语神经管缺陷(NTD)来描述这一整组异常情况。从实际临床角度来看,NTD可细分为三个主要类别:开放性脊柱NTD、闭合性脊柱NTD和颅脑NTD。本文简要介绍了这组先天性病变的流行病学、胚胎学、分类、临床表现及治疗。