Chern Joshua J, Gordon Amber J, Mortazavi Martin M, Tubbs R Shane, Oakes W Jerry
Pediatric Neurosurgery, Children's Hospital, Birmingham, Alabama 35233, USA.
J Neurosurg Pediatr. 2011 Jul;8(1):1-5. doi: 10.3171/2011.4.PEDS10528.
In 1998 the authors identified 5 patients with syringomyelia and no evidence of Chiari malformation Type I (CM-I). Magnetic resonance imaging of the entire neuraxis ruled out other causes of a syrinx. Ultimately, abnormal CSF flow at the foramen magnum was the suspected cause. The label "Chiari 0" was used to categorize these unique cases with no tonsillar ectopia. All of the patients underwent posterior fossa decompression and duraplasty identical to the technique used to treat patients with CM-I. Significant syrinx and symptom resolution occurred in these patients. Herein, the authors report on a follow-up study of patients with CM-0 who were derived from over 400 operative cases of pediatric CM-I decompression.
The authors present their 12-year experience with this group of patients.
Fifteen patients (3.7%) were identified. At surgery, many were found to have physical barriers to CSF flow near the foramen magnum. In most of them, the syringomyelia was greatly diminished postoperatively.
The authors stress that this subgroup represents a very small cohort among patients with Chiari malformations. They emphasize that careful patient selection is critical when diagnosing CM-0. Without an obvious CM-I, other etiologies of a spinal syrinx must be conclusively ruled out. Only then can one reasonably expect to ameliorate the clinical course of these patients via posterior fossa decompression.
1998年，作者发现了5例患有脊髓空洞症且无I型Chiari畸形（CM-I）证据的患者。对整个神经轴的磁共振成像排除了脊髓空洞症的其他病因。最终，怀疑枕骨大孔处脑脊液流动异常是病因。“Chiari 0”这一标签被用于对这些无扁桃体异位的独特病例进行分类。所有患者均接受了与治疗CM-I患者相同技术的后颅窝减压和硬脑膜成形术。这些患者的脊髓空洞症和症状得到了显著缓解。在此，作者报告了对源自400多例小儿CM-I减压手术病例的CM-0患者的随访研究。
作者介绍了他们对这组患者的12年经验。
共识别出15例患者（3.7%）。手术时发现，许多患者在枕骨大孔附近存在脑脊液流动的物理障碍。其中大多数患者术后脊髓空洞症明显减轻。
作者强调，这一亚组在Chiari畸形患者中占比非常小。他们强调，诊断CM-0时仔细选择患者至关重要。若无明显的CM-I，必须彻底排除脊髓空洞症的其他病因。只有这样，才有望通过后颅窝减压改善这些患者的临床病程。
