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中华脑血管病杂志(电子版) ›› 2020, Vol. 14 ›› Issue (05) : 268 -274. doi: 10.11817/j.issn.1673-9248.2020.05.004

所属专题: 经典病例 文献

论著

脑静脉系统血栓形成合并硬脑膜动静脉瘘临床影像分析四例
曹宇泽1, 张君怡1, 董立羚1, 柳青1, 郝红琳1, 朱以诚1, 倪俊1,()   
  1. 1. 100730 中国医学科学院北京协和医院神经科
  • 收稿日期:2020-06-15 出版日期:2020-10-01
  • 通信作者: 倪俊

Clinical and imaging analysis of 4 cases of cerebral venous thrombosis accompanied by dural arteriovenous fistula

Yuze Cao1, Junyi Zhang1, Liling Dong1, Qing Liu1, Honglin Hao1, Yicheng Zhu1, Jun Ni1,()   

  1. 1. Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China
  • Received:2020-06-15 Published:2020-10-01
  • Corresponding author: Jun Ni
  • About author:
    Corresponding author: Ni Jun, Email:
引用本文:

曹宇泽, 张君怡, 董立羚, 柳青, 郝红琳, 朱以诚, 倪俊. 脑静脉系统血栓形成合并硬脑膜动静脉瘘临床影像分析四例[J/OL]. 中华脑血管病杂志(电子版), 2020, 14(05): 268-274.

Yuze Cao, Junyi Zhang, Liling Dong, Qing Liu, Honglin Hao, Yicheng Zhu, Jun Ni. Clinical and imaging analysis of 4 cases of cerebral venous thrombosis accompanied by dural arteriovenous fistula[J/OL]. Chinese Journal of Cerebrovascular Diseases(Electronic Edition), 2020, 14(05): 268-274.

目的

总结分析脑静脉系统血栓形成合并硬脑膜动静脉瘘患者临床表现、诊疗过程及预后,以提高临床医师对二者合并病例的认识。

方法

回顾性分析北京协和医院2010年3月至2020年3月住院诊断为脑静脉系统血栓形成合并硬脑膜动静脉瘘病例,对其临床症状、头磁共振(包括磁共振成像、磁共振血管成像、磁共振静脉成像、磁敏感加权成像)及数字剪影血管造影(DSA)表现、治疗及转归进行分析总结。

结果

共确诊4例脑静脉系统血栓形成合并硬脑膜动静脉瘘患者,均为中青年男性,亚急性起病,其中3例患者明确诊断前病程迁延,临床表现包括视物模糊、癫痫发作、记忆力下降、性格改变等。影像学表现:3例患者以双侧丘脑病变为主异常信号,1例额顶叶皮层异常信号,4例患者头磁共振静脉成像均可见静脉或静脉窦显影异常,且DSA最终确诊为脑静脉系统血栓形成合并硬脑膜动静脉瘘。

结论

临床上中青年患者不明原因脑静脉系统血栓形成尤其血栓迁延不愈时,需警惕合并硬脑膜动静脉瘘可能;临床症状缺乏特异性,常与血栓形成及静脉回流障碍部位相关,双侧丘脑是最常见受累部位,针对性的影像学检查对于及时诊断治疗非常重要。

Objective

The clinical manifestations, diagnosis, treatment and prognosis of cerebral venous thrombosis accompanied by dural arteriovenous fistulas were summarized and analyzed, so as to improve the clinicians' knowledge of the combined cases.

Methods

The clinical symptoms, MR (including MRI, MRA, MRV, SWI) and DSA manifestations, treatment and outcome of cerebral venous thrombosis accompanied by dural arteriovenous fistula in Peking Union Medical College Hospital from March 2010 to March 2020 were analyzed retrospectively.

Results

Four cases of cerebral venous thrombosis accompanied by dural arteriovenous fistula were diagnosed in our hospital, all of whom were middle-aged men with subacute onset. The clinical manifestations included blurred vision, epileptic attack, memory decline, character change, etc. One patient had abnormal signal of frontal parietal cortex on MRI, and three patients had abnormal signal of bilateral thalamic lesions on MRI. The head MRV of 4 patients were abnormal. Four patients were diagnosed as cerebral venous thrombosis accompanied by dural arteriovenous fistula by DSA.

Conclusion

In clinic, young and middle-aged patients with cerebral venous thrombosis of unknown cause, especially those with protracted course of disease, need to be considered with dural arteriovenous fistula. There is not specific clinical which is often related to the location of thrombosis and venous reflux disorders. Bilateral thalamus is the most common affected area, and the targeted imaging examination is very important for the timely diagnosis and treatment.

图1 病例1影像学表现 图a为头磁共振静脉成像上矢状窦前部未见显影(白色圆圈);图b为数字减影血管造影可见颈外动脉系统-上矢状窦动静脉瘘(白色箭头)
图2 例2影像学表现 图a、b为头MRI可见双侧丘脑长T1、长T2异常信号(白色箭头);图c为头磁共振静脉成像直窦未见显影(白色圆圈)
图3 例3影像学表现 图a、b为头MRI可见双侧丘脑长T1、长T2异常信号(白色箭头);图c为头磁共振静脉成像直窦未见显影(白色圆圈)
图4 例4影像学表现 图a、b为头MRI可见双侧丘脑长T1、长T2异常信号(白色箭头);图c为T2*WI可见双侧丘脑多发微出血(白色圆圈)。图d为头磁共振静脉成像直窦未见显影(白色圆圈)
图5 患者数字减影血管造影(DSA)图 图a为病例2 DSA可见大脑后动脉-大脑大静脉动静脉瘘(白色箭头),图b为病例3 DSA可见大脑后动脉-基底静脉动静脉瘘(白色箭头),图c为病例4 DSA可见颈内动脉发出脑膜支与大脑深静脉形成动静脉瘘(白色箭头)
表1 脑静脉系统血栓形成合并硬脑膜动静脉瘘4例患者临床资料汇总
病例 性别 年龄(岁) 临床表现 头颅MRI表现 DSA 脑脊液 治疗 转归
例1 50

病程:10个月

起病形式:亚急性起病

主要症状:视物模糊、肢体抽搐

常规:额顶叶皮层FLAIR稍高信号

MRA:未见异常

MRV:上矢状窦局限狭窄

上矢状窦未显影,上矢状窦动静脉瘘形成,瘘口附近皮层静脉血流逆向

外观:无色透明

压力:>330 mmH2O

常规、生化:正常范围

脱水降颅压、抗癫痫治疗;外院行动静脉瘘栓塞术 症状好转
例2 43

病程:半个月

起病形式:亚急性起病

主要症状:记忆力下降、性格改变

常规:双侧丘脑长T1、长T2信号

MRA:大脑后动脉区异常血管团

MRV:直窦未显影

直窦未显影,左大脑后动脉-大脑大静脉动静脉瘘,大脑内静脉血流逆向 未行腰穿 脱水降颅压;外院行动静脉瘘栓塞术 症状好转
例3 52

病程:3年余

起病形式:亚急性起病

主要症状:记忆力下降、性格改变、步态异常

常规:双侧丘脑、基底节、颞叶内侧长T1长T2、边缘短T2信号

MRA:未见异常

MRV:直窦未显影

SWI:双侧丘脑、右内侧颞叶多发低信号

直窦未显影,右侧大脑后动脉-基底静脉动静脉瘘

外观:无色透明

压力:170 mmH2O

常规:正常范围

生化:蛋白质浓度0.82 g/L,糖、氯正常

脱水降颅压 症状好转
例4 42

病程:4个月

起病形式:亚急性起病

主要症状:睡眠增多、记忆力下降、精神行为异常

常规:双侧丘脑长T1、长T2信号

MRA:未见异常

MRV:下矢状窦、大脑内静脉、大脑大静脉、直窦未显影

T2*WI:双侧丘脑多发微出血灶

直窦未显影,双侧颈内动脉-大脑深静脉动静脉瘘

外观:无色透明

压力:150 mmH2O

常规:正常范围

生化:蛋白质浓度0.53 g/L,糖、氯正常

抗凝、脱水降颅压 回当地医院
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