烟雾病颈内动脉颅外段狭窄的临床特点分析

doi:10.11817/j.issn.1673-9248.2022.03.002

目的

对烟雾病颈内动脉颅外狭窄临床特点进行分析总结。

方法

回顾性分析2018年1月1日至2019年9月30日于北京大学国际医院住院的177例烟雾病患者的临床资料。分析患者年龄、性别、是否为出血型以及术后是否有新发梗死等基本资料，以及血管造影资料包括Suzuki分期、烟雾血管量、颈内动脉狭窄和大脑后动脉（PCA）病变情况，脑CT灌注（CTP）通过梗死前分期系统进行分析。采用Spearman相关分析颈内动脉颅外段狭窄与上述资料的相关性。

结果

177例烟雾病患者中，包括儿童43例和成人134例。颈内动脉颅外段狭窄共83侧，占所有侧的23.45%（83/354）。颈内动脉颅外段的狭窄全部为全程性狭窄（非局灶性）。颈内动脉狭窄与年龄、Suzuki分期、CTP分期、PCA病变呈正相关（r=0.122，P=0.020；r=0.333，P＜0.001；r=0.373，P＜0.001；r=0.131，P=0.010）。高分辨率MRI检查显示狭窄呈向心性狭窄，无斑块形成。

结论

颈内动脉颅外段狭窄是烟雾病病变独立的特点，应引起重视。烟雾病患者颈内动脉颅外段狭窄的典型特征是全程性、向心性狭窄。高分辨率MRI在颈内动脉颅外段狭窄中有重要意义。Suzuki分期越高，颈内动脉颅外段狭窄的可能性越大，但Suzuki早期也可能出现颈内动脉颅外段狭窄。烟雾病患者颈内动脉颅外段狭窄可能导致脑血流灌注降低。

关键词: 烟雾病, 颈内动脉颅外段狭窄, 临床特点

Objective

To analyze clinical characteristics extracranial stenosis of internal carotid artery (ICA) in moyamoya disease (MMD).

Methods

The cases of MMD admitted to Peking University International Hospital from January 1, 2018 to September 30, 2019 were retrospectively analyzed. A total of 177 patients with MMD were included. Further analysis included basic data including age, sex, presence of hemorrhage, presence of a new infarction after surgery. The angiographic data were also analyzed, including Suzuki stage, moyamoya vessel volume, ICA stenosis, and posterior cerebral artery (PCA) lesions. CT perfusion (CTP) was analyzed by a pre-infarction staging system. Spearman's correlation was used to analyze the corre lation of intracranial stenosis of internal carotid artery with the above date.

Results

There were 43 children and 134 adults included. A total of 83 sides of ICA had extracranial stenosis, accounting for 23.45% of all sides. All of the stenosis of the extracranial ICA were whole-course (non-focal). ICA stenosis was positively correlated with age, Suzuki stage, CTP grading, and PCA lesion, with correlation coefficients of (r=0.122, P=0.020; r=0.333, P＜0.001; r=0.373, P＜0.001; r=0.131, P=0.010) respectively. High resolution magnetic resonance imaging (Hr-MRI) examination showed that the stenosis was centripetal without plaque formation.

Conclusion

As an independent part of MMD, extracranial ICA stenosis should be paid more attention to. The typical characteristics of stenosis are whole course, uniform distribution and centripetal stenosis. Hr-MRI has important significance in examining extracranial stenosis of ICA. The higher the Suzuki staging, the greater the likelihood of extracranial carotid stenosis. But stenosis was also seen in Suzuki's early stage. The stenosis may result in decreased cerebral perfusion, which exacerbates the symptoms.

Key words: Moyamoya disease, Extracranial internal carotid artery stenosis, Clinical features

图1 烟雾血管量分类。图a和d为颈内动脉造影正位和侧位，显示少量烟雾血管；图b和e为颈内动脉造影正位和侧位，显示中量烟雾血管；图c和f为颈内动脉造影正位和侧位，显示大量烟雾血管

表1 烟雾病患者临床、影像资料汇总（例）

临床资料	儿童（n=43）		成人（n=134）
临床资料	男	女	男	女
颈内动脉颅外段狭窄
左侧	2	4	14	17
右侧	4	5	18	19
出血缺血分型
缺血型	21	19	41	60
出血型	2	1	17	16
Suzuki分期
左侧
1	3	0	13	8
2	0	1	1	3
3	13	8	12	11
4	4	6	20	31
5	1	6	8	15
6	1	0	5	7
右侧
1	2	4	8	4
2	1	3	3	4
3	10	4	12	17
4	7	7	14	19
5	3	1	18	17
6	0	1	3	15
大脑后动脉病变
左侧	17	9	12	19
右侧	8	5	16	17
烟雾血管量
左侧
0	5	2	18	15
1	6	9	30	41
2	7	5	9	13
3	4	5	2	6
右侧
0	3	5	13	20
1	10	9	36	39
2	7	4	4	13
3	3	2	5	4
术后新发梗死
左侧	0	1	10	4
右侧	1	4	6	6
CT灌注分期
左侧
1	5	2	17	21
2	11	10	21	28
3	5	5	14	15
4	1	4	7	11
右侧
1	5	6	15	19
2	8	7	19	40
3	6	3	17	7
4	4	4	7	10

表1 烟雾病患者临床、影像资料汇总（例）

临床资料	儿童（n=43）		成人（n=134）
临床资料	男	女	男	女
颈内动脉颅外段狭窄
左侧	2	4	14	17
右侧	4	5	18	19
出血缺血分型
缺血型	21	19	41	60
出血型	2	1	17	16
Suzuki分期
左侧
1	3	0	13	8
2	0	1	1	3
3	13	8	12	11
4	4	6	20	31
5	1	6	8	15
6	1	0	5	7
右侧
1	2	4	8	4
2	1	3	3	4
3	10	4	12	17
4	7	7	14	19
5	3	1	18	17
6	0	1	3	15
大脑后动脉病变
左侧	17	9	12	19
右侧	8	5	16	17
烟雾血管量
左侧
0	5	2	18	15
1	6	9	30	41
2	7	5	9	13
3	4	5	2	6
右侧
0	3	5	13	20
1	10	9	36	39
2	7	4	4	13
3	3	2	5	4
术后新发梗死
左侧	0	1	10	4
右侧	1	4	6	6
CT灌注分期
左侧
1	5	2	17	21
2	11	10	21	28
3	5	5	14	15
4	1	4	7	11
右侧
1	5	6	15	19
2	8	7	19	40
3	6	3	17	7
4	4	4	7	10

图2 烟雾病患者颈内动脉颅外狭窄的高分辨率磁共振特点。图a右侧颈内动脉血管造影侧位显示颈内动脉颅外段小于颈总动脉直径的50%（箭头）。图b~d为高分辨率磁共振图像。图a、b均表现出狭窄的全程性和均一性特征。图b箭头示颈内动脉狭窄部分。图c箭头为颈内动脉管腔分叉处，管径相对较粗。图d箭头指示颈内动脉管腔狭窄段，为向心狭窄，无斑块形成

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Clinical features of extracranial internal carotid artery stenosis in moyamoya disease

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Clinical features of extracranial internal carotid artery stenosis in moyamoya disease