Williams B
Midland Centre for Neurosurgery, Warley, West Midlands, U.K.
Adv Tech Stand Neurosurg. 1993;20:107-64. doi: 10.1007/978-3-7091-6912-4_4.
Syringomyelia is a condition with many possible causes, the commonest of which seems to be an abnormality at the foramen magnum. Such cases may be grouped under the heading of "Hindbrain related syringomyelia" and the principles of treatment for all such cases are largely similar. The commonest of these foramen magnum region abnormalities is hindbrain herniation which may be associated with a history of birth difficulties, a small posterior fossa, segmentation abnormalities of the cervical vertebrae or the base of the skull, arachnoiditis of the subarachnoid spaces, subarachnoid pouches, hydrocephalus and intracranial tumours or tumours partly blocking the foramen magnum. Other causes of syringomyelia include conditions which could be grouped under the heading of "non-hindbrain related syringomyelia", these mostly produce blockage of the spinal subarachnoid spaces, especially spinal "arachnoiditis" or meningeal fibrosis, including that secondary to traumatic paraplegia. Intraspinal tumours are sometimes cystic and some authors have included this association under the heading of syringomyelia. Syringomyelia of all kinds is almost always a surgical condition, the destructive forces are those of fluid distending the tissues. As a principle, treatment directed against the cause of the accumulation and the intracord propagation of the fluid by normalising the CSF pathways is more likely to be successful than drainage of the cavities. Drainage operations have an inevitable failure rate and a further incidence of complications attends myelotomy and the leaving of any drainage tube within the narrow confines of the spine. Correction of craniospinal pressure dissociation and re-establishment of a cisterna magna appears to be the most successful treatment strategy and is likely to be immediately and permanently successful in correcting not only the pressure problems such as long tract involvement and syringobulbia features but also in producing satisfactory clinical and radiological improvement in the syringomyelia. The recommended technique includes radical means to gain space at the foramen magnum by creating a large artificial cisterna magna, resecting part of the tonsils, preventing the descent of the cerebellum and avoiding the use of space occupying or fibrosis producing dural grafts. Because the pathogenesis of the cavities remains in doubt, the method by which this treatment stratagem is effective is unclear. It may be that change in the closure conditions of parts of the neuraxis, i.e., alteration in the capacitance and consequent change in pulsation characteristics afforded by the decompression may be the factor which predicates success. Surgical management of hindbrain related syringomyelia is not easy, there are hazards associated with operation, hydrocephalus demands priority in it's management.(ABSTRACT TRUNCATED AT 400 WORDS)
脊髓空洞症是一种有多种可能病因的病症,其中最常见的病因似乎是枕骨大孔处的异常。此类病例可归类于“与后脑相关的脊髓空洞症”,所有这类病例的治疗原则在很大程度上是相似的。枕骨大孔区域最常见的异常是后脑疝,它可能与出生困难史、小脑后窝狭小、颈椎或颅底的分割异常、蛛网膜下腔蛛网膜炎、蛛网膜下腔囊肿、脑积水以及颅内肿瘤或部分阻塞枕骨大孔的肿瘤有关。脊髓空洞症的其他病因包括可归类于“与后脑无关的脊髓空洞症”的情况,这些大多会导致脊髓蛛网膜下腔阻塞,尤其是脊髓“蛛网膜炎”或脑膜纤维化,包括继发于外伤性截瘫的情况。脊髓内肿瘤有时呈囊性,一些作者已将这种关联归入脊髓空洞症范畴。各类脊髓空洞症几乎总是需要手术治疗,其破坏作用是液体使组织膨胀。原则上,通过使脑脊液通路正常化来针对液体积聚及在脊髓内扩散的病因进行治疗,比引流空洞更有可能成功。引流手术不可避免地有失败率,并且脊髓切开术以及在狭窄的脊柱内留置任何引流管都会引发进一步的并发症。纠正颅颈压力分离并重建枕大池似乎是最成功的治疗策略,不仅有可能立即且永久成功地纠正诸如长束受累和延髓空洞症特征等压力问题,还能使脊髓空洞症在临床和影像学上得到令人满意的改善。推荐的技术包括通过创建一个大的人工枕大池、切除部分扁桃体、防止小脑下降以及避免使用占位或产生纤维化的硬脑膜移植物等激进方法来在枕骨大孔处获得空间。由于空洞的发病机制仍存在疑问,这种治疗策略有效的方法尚不清楚。可能是神经轴部分闭合条件的改变,即减压所带来的容量变化以及随之而来的搏动特性改变,可能是预示成功的因素。与后脑相关的脊髓空洞症的手术管理并不容易,手术存在风险,脑积水在其管理中需要优先处理。(摘要截选至400字)