Hutchins G M, Meuli M, Meuli-Simmen C, Jordan M A, Heffez D S, Blakemore K J
Department of Pathology, Johns Hopkins Medical Institutions, Baltimore, Maryland, USA.
Pediatr Pathol Lab Med. 1996 Sep-Oct;16(5):701-12.
Experimental studies have shown that there is a potential to attempt in utero repair of myelomeningocele in human fetuses. To provide a better understanding of the pathology of these lesions we prospectively studied eight stillborn human fetuses with myelomeningocele autopsied at The Johns Hopkins Hospital. The intact vertebral column with surrounding structures was removed, processed as a single block, and prepared as serial histologic sections. Study of the slides showed in all cases that in the center of the myelomeningocele the vertebral arch was open, the arrangement of meninges was such that the dura mater was open and in continuity with the deep layers of the dermis, and the pia mater was open and in continuity with a layer consisting of the superficial dermis and the epidermis. These meningeal relationships created an abnormally configured arachnoid space containing cerebrospinal fluid ventral to the spinal cord, which rested on the open pia mater and was exposed on the dorsal aspect of the sac. At the level of the myelomeningocele the naked cord had undergone varying degrees of injury up to complete loss of neural tissue. Where ventral remnants of the cord remained it was evident that a large degree of normal development of the cord had occurred. In most instances it appeared that the injury or destruction of the dorsal spinal cord was recent and consistent with occurrence during delivery. The results of this study support the concept that in utero surgery could preserve and protect the exposed spinal cord in a myelomeningocele of a human fetus and thus could reduce the severity of the neurologic deficit at birth.
实验研究表明，对人类胎儿的脊髓脊膜膨出进行子宫内修复具有一定潜力。为了更好地了解这些病变的病理情况，我们对在约翰霍普金斯医院进行尸检的8例患有脊髓脊膜膨出的死产人类胎儿进行了前瞻性研究。将带有周围结构的完整脊柱取出，作为一个整体进行处理，并制成连续的组织学切片。对切片的研究显示，在所有病例中，脊髓脊膜膨出的中心部位椎弓开放，脑膜的排列方式是硬脑膜开放并与真皮深层连续，软脑膜开放并与由浅表真皮和表皮组成的一层连续。这些脑膜关系形成了一个异常构型的蛛网膜下腔，其中含有位于脊髓腹侧的脑脊液，脊髓位于开放的软脑膜上，并暴露于囊的背侧。在脊髓脊膜膨出水平，裸露的脊髓遭受了不同程度的损伤，直至神经组织完全丧失。在脊髓腹侧残余部分仍存在的地方，很明显脊髓已经发生了很大程度的正常发育。在大多数情况下，似乎脊髓背侧的损伤或破坏是近期发生的，与分娩过程中发生的情况一致。这项研究的结果支持这样一种观点，即子宫内手术可以保护人类胎儿脊髓脊膜膨出中暴露的脊髓，从而可以减轻出生时神经功能缺损的严重程度。
