Donauer E, Rascher K
Department of Neurosurgery, University of the Saarland, Homburg, Fed. Rep. of Germany.
Neurosurg Rev. 1993;16(1):7-13. doi: 10.1007/BF00308604.
The generally accepted definition of syringomyelia is that it is a chronically progressive illness characterized by the presence of cavities or syrinxes in the spinal cord. As manifold as the terminology of syringomyelia are the hypotheses of the etiology. Nowadays with MRI without and with gadolinium it is possible to recognize intramedullar cavities safely, the MR especially the cine-MR provides information on pathophysiological details of the flow and intracavitary pressure dependent pulsations of the CSF. Animal models and the findings of own experimental studies have enabled us to study a form of syringomyelia which very closely resembles that brought about by dysrhaphic malformations in the human being and to examine the effectiveness of certain types of surgical therapy. In this paper the term syringomyelia is only used for dysrhaphic cavities in the medulla. After our experience with 61 patients with syringomyelia now we perform the operative decompression of the craniocervical transition as the first step in the operative treatment of the progressive syringomyelia combined with severe craniocervical malformations. In cases with insufficient treatment response we suggest the syringoarachnoid shunting of persisting large intramedullar cavities.
一般认为，脊髓空洞症的定义是一种慢性进行性疾病，其特征为脊髓内存在空洞或管腔。脊髓空洞症病因的假说和其术语一样繁多。如今，借助有无钆对比剂的磁共振成像（MRI），能够安全地识别髓内空洞，尤其是电影磁共振成像（cine-MR）能提供关于脑脊液流动及与腔内压力相关的搏动的病理生理细节信息。动物模型和自身实验研究结果使我们能够研究一种与人类脊柱裂畸形所致脊髓空洞症极为相似的脊髓空洞症，并检验某些手术治疗方法的有效性。在本文中，脊髓空洞症一词仅用于延髓内的脊柱裂空洞。根据我们对61例脊髓空洞症患者的经验，现在我们将颅颈交界区的手术减压作为进行性脊髓空洞症合并严重颅颈畸形手术治疗的第一步。对于治疗反应欠佳的病例，我们建议对持续存在的大髓内空洞进行脊髓蛛网膜分流术。
