Park T S, Cail W S, Maggio W M, Mitchell D C
J Neurosurg. 1985 Mar;62(3):367-75. doi: 10.3171/jns.1985.62.3.0367.
Seventeen myelodysplastic patients with progressive extremity spasticity and scoliosis underwent radiological evaluation and surgical treatment. All but one were under 18 years of age at the time of surgical treatment. Duration of the clinical presentation ranged from 1 1/2 to 7 years. Metrizamide was instilled into the subarachnoid space in 12 patients, the lateral ventricle in two, and the hydromyelic cavity in three. Sequential computerized tomography scanning after intrathecal instillation of the contrast material clearly demonstrated hydromyelia in nine patients and compression of the brain stem in five. Posterior fossa decompression with plugging of the obex was performed in 12 patients, posterior fossa decompression alone in three, and ventriculoperitoneal (VP) shunting procedures in two. Of the 12 patients who underwent the obex plugging procedures, eight have shown partial or complete resolution of spasticity and an increase in motor strength with no significant postoperative complications. In contrast, posterior fossa decompression or VP shunting procedures alone have not led to a favorable neurological outcome. Hydromyelia may occur more commonly among myelodysplastic patients than previously recognized and may be treated most effectively by the obex plugging procedure.
17例患有进行性肢体痉挛和脊柱侧弯的骨髓发育异常患者接受了放射学评估和手术治疗。除1例患者外,其余患者在接受手术治疗时均未满18岁。临床表现的持续时间为1.5至7年。12例患者在蛛网膜下腔注入了甲泛葡胺,2例注入侧脑室,3例注入积水性脊髓空洞腔。鞘内注入造影剂后进行的连续计算机断层扫描清楚地显示,9例患者存在脊髓空洞症,5例患者脑干受压。12例患者进行了后颅窝减压并封闭闩部,3例仅进行了后颅窝减压,2例进行了脑室腹腔(VP)分流术。在接受闩部封闭手术的12例患者中,8例患者的痉挛部分或完全缓解,肌力增强,术后无明显并发症。相比之下,单纯的后颅窝减压或VP分流术并未带来良好的神经学结果。脊髓空洞症在骨髓发育异常患者中的发生率可能比之前认为的更高,通过封闭闩部的手术治疗可能最为有效。