Straus David, Foster Kimberly, Zimmerman Frank, Frim David
Pritzker School of Medicine, Chicago, IL, USA.
Pediatr Neurosurg. 2009;45(5):384-9. doi: 10.1159/000260909. Epub 2009 Nov 26.
Chiari I malformation (CM1) is characterized by impaired CSF flow through the foramen magnum. Dysfunctional autonomic cardiovascular regulation may result in syncope. Syncope may be the primary presenting symptom of CM1: a syndrome termed Chiari drop attack. It has been postulated that Chiari drop attack is secondary to dysautonomia caused by hindbrain compression. There has been recent debate regarding the association between CM1, dysautonomia and Chiari drop attack.
We selected patients with Chiari drop attacks who had negative workups for cardiac syncope, followed by tilt table testing and subsequent surgical decompression. We report test results and clinical outcomes following CM1 decompression.
Ten patients met the inclusion criteria: 5 patients had positive and 5 negative tilt table tests. Following decompression, 7 had symptomatic improvement or resolution and 3 failed to improve. The sensitivity and specificity of the tilt table test for detecting clinical improvement with surgical decompression was 43 and 33%, respectively. Tilt table testing had 40% accuracy in predicting clinical response to decompression.
In this short series, surgical decompression of CM1 has a high success rate (70%) for patients with Chiari drop attacks. Tilt table testing has poor predictive value in judging the clinical response to surgical decompression and is not a useful test to guide surgical decision- making.
Chiari I 畸形(CM1)的特征是脑脊液通过枕骨大孔的流动受损。自主神经心血管调节功能障碍可能导致晕厥。晕厥可能是 CM1 的主要首发症状:一种称为 Chiari 跌倒发作的综合征。据推测,Chiari 跌倒发作继发于后脑受压引起的自主神经功能障碍。最近关于 CM1、自主神经功能障碍和 Chiari 跌倒发作之间的关联存在争议。
我们选择了 Chiari 跌倒发作且心脏性晕厥检查结果为阴性的患者,随后进行倾斜试验及后续手术减压。我们报告 CM1 减压后的检查结果和临床结局。
10 名患者符合纳入标准:5 名患者倾斜试验结果为阳性,5 名患者为阴性。减压后,7 名患者症状改善或消失,3 名患者未改善。倾斜试验检测手术减压后临床改善情况的敏感性和特异性分别为 43%和 33%。倾斜试验预测减压临床反应的准确率为 40%。
在这个简短系列研究中,CM1 手术减压对 Chiari 跌倒发作患者有较高成功率(70%)。倾斜试验在判断手术减压临床反应方面预测价值较差,不是指导手术决策的有用检查。