Sim K B, Hong S K, Cho B K, Choi D Y, Wang K C
Department of Neurosurgery, Seoul Red Cross Hospital, Korea.
J Korean Med Sci. 1996 Dec;11(6):509-16. doi: 10.3346/jkms.1996.11.6.509.
Though several pathogenetic theories concerning the frequent association of Chiari malformation and hydrocephalus with myeloschisis have been suggested, none of them explains all the aspects of the disorder. To investigate whether myeloschisis is the direct cause of Chiari malformation and hydrocephalus or these conditions are the results of another basic event, we observed the morphological changes of the posterior cranial fossa and its components in the chick embryos with surgically induced myeloschisis. To make myeloschistic lesions, we opened the neural tube for a length of 9-11 somites in Hamburger and Hamilton stage 16-19 chick embryos. They were divided into cervicothoracic (C-T) and lumbosacral(L-S) groups according to the area of incision. The embryos were re-incubated until postoperative day 11. In the control group, embryos were incubated with the eggshell window open as their experimental counterparts. The survival rates of each group were as follows; 11% (9 survivors/85 operated embryos), 8% (7/83), and 17% (10/60) in the C-T, L-S and control groups, respectively. Myeloschisis positive rates were 100% in the operated groups and 0% in the control group. The heads of embryos were sectioned along the sagittal plane to observe the morphological changes in the posterior cranial fossa and its components. Of the survivors, five in the C-T group, two in the L-S group and six in the control group were available for light microscopic inspection. In the majority of embryos with myeloschisis, without difference between the C-T and L-S groups, the fourth ventricles were smaller than those of the control group and the subarachnoid spaces in the posterior cranial fossa were also narrower. In embryos with severe changes, the cerebellum displaced downward comparing with that of the control embryos. No evidence of hydrocephalus was present Though not always typical, morphological changes similar to Chiari malformation were observed in chick embryos with surgically induced myeloschisis. It suggests a strong direct causal relationship between the two conditions and supports the theories of derangements in cerebrospinal fluid dynamics rather than those of primary mesenchymal or neural origin as a pathogenetic mechanism of Chiari malformation.
尽管已经提出了几种关于Chiari畸形和脑积水与脊髓裂经常伴发的发病机制理论,但没有一种理论能够解释该疾病的所有方面。为了研究脊髓裂是否是Chiari畸形和脑积水的直接原因,或者这些情况是否是另一个基本事件的结果,我们观察了手术诱导脊髓裂的鸡胚后颅窝及其组成部分的形态变化。为了造成脊髓裂损伤,我们在Hamburger和Hamilton 16 - 19期鸡胚中打开神经管9 - 11个体节的长度。根据切口区域将它们分为颈胸(C - T)组和腰骶(L - S)组。将胚胎重新孵化至术后第11天。在对照组中,胚胎与实验组一样,蛋壳窗口打开进行孵化。每组的存活率如下:C - T组为11%(9只存活/85只手术胚胎),L - S组为8%(7/83),对照组为17%(10/60)。手术组的脊髓裂阳性率为100%,对照组为0%。将胚胎头部沿矢状面切开,以观察后颅窝及其组成部分的形态变化。在存活的胚胎中,C - T组有5只、L - S组有2只、对照组有6只可用于光镜检查。在大多数有脊髓裂的胚胎中,C - T组和L - S组之间没有差异,第四脑室比对照组小,后颅窝蛛网膜下腔也更窄。在变化严重的胚胎中,小脑与对照胚胎相比向下移位。没有脑积水的证据。尽管并不总是典型的,但在手术诱导脊髓裂的鸡胚中观察到了与Chiari畸形相似的形态变化。这表明这两种情况之间存在强烈的直接因果关系,并支持脑脊液动力学紊乱理论,而不是原发性间充质或神经起源理论作为Chiari畸形的发病机制。