Yarbrough Chester K, Powers Alexander K, Park Tae Sung, Leonard Jeffrey R, Limbrick David D, Smyth Matthew D
Department of Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri, USA.
J Neurosurg Pediatr. 2011 Mar;7(3):244-7. doi: 10.3171/2010.11.PEDS1097.
A subset of patients with Chiari malformation Type I (CM-I) presented with acute onset of a neurological deficit. In this study the authors summarize their experience with these patients' clinical presentation, imaging results, timing of surgery, and outcome following decompression.
The authors reviewed clinical records, imaging studies, and operative notes from all patients undergoing posterior fossa decompression for CM-I at St. Louis Children's Hospital from 1990 to 2008. Of the 189 patients who underwent surgery, 6 were identified with the acute onset of a neurological deficit at presentation.
All 6 children (age range 3-14 years, 3 boys and 3 girls) had either syringomyelia (5 patients) or T2 signal changes in the spinal cord (1 patient) and CM-I on initial MR imaging. Three patients presented after minor trauma (1 with paraparesis, 2 with sensory deficits). Three patients presented without a clear history of trauma (1 with abrupt onset of spontaneous dysphagia and ataxia, 2 with sensory deficits). Decompression was performed at a mean 7.7 ± 4.9 days after symptom onset (7.0 ± 1.6 days after neurosurgical evaluation). In 1 patient, symptoms had resolved by the time of surgery; in the remainder of the patients, clear improvements were noted within 2 weeks of surgery, with complete resolution of symptoms by 12 months postoperatively. Follow-up MR images were obtained in 4 patients, demonstrating improvement in the extent of the syrinx in each patient.
Children with CM-I and syringomyelia can develop acute spinal cord or bulbar deficits with relatively minor head or neck injuries. The prognosis for symptomatic improvement in the observed deficit is good, with each patient in our series showing resolution of deficits over time. However, based on this relatively limited experience, the authors suggest that patients who present with an acute neurological deficit and are found to have CM-I be managed with early posterior fossa decompression. Patients with CM-I and syringomyelia may be at higher risk of acute neurological deficit than those without a syrinx.
一部分I型Chiari畸形（CM-I）患者会急性出现神经功能缺损。在本研究中，作者总结了这些患者的临床表现、影像学结果、手术时机以及减压术后的结局。
作者回顾了1990年至2008年在圣路易斯儿童医院接受CM-I后颅窝减压手术的所有患者的临床记录、影像学研究和手术记录。在189例接受手术的患者中，有6例在就诊时急性出现神经功能缺损。
所有6名儿童（年龄范围3至14岁，3名男孩和3名女孩）在初次磁共振成像时均患有脊髓空洞症（5例）或脊髓T2信号改变（1例）以及CM-I。3例患者在轻微外伤后就诊（1例双下肢轻瘫，2例感觉障碍）。3例患者就诊时无明确外伤史（1例突然出现自发性吞咽困难和共济失调，2例感觉障碍）。减压手术在症状出现后平均7.7±4.9天（神经外科评估后7.0±1.6天）进行。1例患者在手术时症状已缓解；其余患者在术后2周内症状明显改善，术后12个月症状完全消失。4例患者进行了随访磁共振成像，显示每个患者脊髓空洞的范围均有改善。
患有CM-I和脊髓空洞症的儿童在相对轻微的头部或颈部损伤后可能会急性出现脊髓或延髓功能缺损。观察到的功能缺损症状改善的预后良好，我们系列中的每个患者随着时间的推移症状均得到缓解。然而，基于这一相对有限的经验，作者建议，对于急性出现神经功能缺损且被发现患有CM-I的患者，应尽早进行后颅窝减压治疗。与没有脊髓空洞的患者相比，患有CM-I和脊髓空洞症的患者可能有更高的急性神经功能缺损风险。
