青少年Chiari畸形Ⅰ型患儿脊髓的萎缩样改变

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目的:比较青少年Chiari畸形Ⅰ型(Chiari malformation type I,CMI)患儿与健康青少年脊髓形态的差异,探讨脊髓空洞对CMI患儿脊髓形态的影响。方法:回顾性分析2012年6月至2019年3月期间诊断为CMI的292例患儿病历资料,其中不伴脊髓空洞的15例CMI患儿纳入CM组;余下277例中274例患儿脊髓空洞上缘低于Cn 3-4椎间盘平面,按照最佳匹配原则选择年龄与CM组最为相近(±18个月)伴脊髓空洞的CMI患儿,按照1∶2比例将30例患儿纳入CMS组;再以同样方式选取30名健康青少年志愿者纳入对照组。在MRI测量所有纳入对象Cn 2水平脊髓前后径、Cn 2水平椎管前后径、中脑-桥脑交界处前后径、小脑扁桃体顶端至枕骨大孔的距离和脊髓空洞的最大直径。所有影像学参数由2名研究者分别独立测量2次后取首次测量结果平均值纳入统计,采用组内相关系数(intraclass correlation coefficient,n ICC)检验观察者内和观察者间的信度。三组间影像学参数及年龄的比较采用方差分析和SNK-n q检验,三组间性别分布比较采用n χ2检验,CM组及CMS组中影像学参数间进行Pearson相关分析。n 结果:观察者内和观察者间的n ICC为0.91~0.95,表明测量可靠性“极好”。三组纳入对象的年龄及性别分布的差异均无统计学意义。CM组和CMS组患儿Cn 2水平脊髓前后径相似(n P=0.254),且均明显低于健康对照组(均n P0.05)。n 结论:与健康青少年相比,CMI患儿颈髓、中脑-脑桥连接处及Cn 2水平脊髓有明显萎缩性改变;伴脊髓空洞的CMI患儿存在更加明显的中脑-桥脑连接处萎缩性改变和椎管扩张。n “,”Abstract:To compare the morphology of spinal cord between healthy adolescents with adolescent Chiari malformation type I (CMI) patients and investigate the impact of syringomyelia on the morphology of spinal cord in CMI patients.Methods:The clinical and radiological data of 292 CMI patients diagnosed by our center between June 2012 and March 2019 were retrospectively reviewed. Among them 15 CMI patients without syringomyelia were recruited in the CM group. Among the remaining 277 CMI patients, 274 patients had syringomyelia below the Cn 3-4 intervertebral disc. According to the principle of best matching, CMI patients with syringomyelia were selected with the closest age to the CM group (±18 months), and 30 CMI patients with syringomyelia were included in the CMS group according to a ratio of 1∶2. Thirty healthy adolescents were enrolled as the control group (NC group) in the same way. The anteroposterior diameters of spinal cord at Cn 2 (DSCO-Cn 2), spinal canal at Cn 2 (DSCA-Cn 2), midbrain-pontine junction (DPJ), the distance between the tip of cerebellar tonsils and the foramen magnum (AB) and the maximal diameter of the syrinx (D-syrinx) were measured on MRI. All radiographic parameters were measured twice independently by two spine surgeons, and intraclass correlation coefficient (ICC) were determined to demonstrate intra- and inter-observer reliability. One-way ANOVA and SNK-n q test were used to compare the above radiographic parameters and age between CM, CMS and NC group. The distribution of genders was compared between the three groups using Chi-square tests. Pearson correlation analysis were conducted to demonstrate the relationship between radiographic parameters in CM and CMS group.n Results:ICC ranged between 0.91 and 0.95 in the current study, demonstrating “excellent” reliability of radiographic measurements. No significant difference was noted regarding age and the distribution of genders among the three groups. Patients in CM and CMS groups showed similar DSCO-C n 2 values (n P=0.254), both of which were significantly lower than that in NC group (n P0.05).n Conclusion:CMI adolescents have significant atrophic change of cervical spinal cord and midbrain-pontine junction compared with healthy adolescents, regardless of the existence of syrinx. Moreover, syrinx in CMI patients indicated more obvious atrophic change of midbrain-pontine junction and dilated spinal canal compared with isolated CMI patients.
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