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脑静脉性血管畸形(CVM)
http://www.100md.com 2004年10月14日 本会
     天津环湖医院介入中心 (300060)

    又称为发育性静脉异常(developmental venous anomaly,简称为DVA)及脑静脉血管瘤(Cerebral venous angiocer)简称为CVA)。有可能是一系列脑血管畸形中的一个环节。发生率不详,尸检发现率高达1-3%。在各型脑血管畸形中所占比例各家报道差别较大7.3-63%,国内报道占各类脑血管畸形的1.7-6.5%。

    临床表现:癫痫,出血,神经功能缺损,颅压高,占位等。但大多数CVM可能无症状。

    辅助检查:CT高密度(出血),混杂密度包括钙化(不同时期出血),脑室周围圆形高密度(静脉影)脑室扩大等。MRI高低信号混杂,类似海绵状血管瘤,未出血病变低信号管状结构,T2加权较粗大的静脉可能流空信号其偶数回波重聚为密度一致高信号影。脑血管造影:三联征:表浅静脉缺如,深静脉汇集(形成水母头状medusa)。大引流静脉。其他表现:异常静脉,静脉湖等。
, 百拇医药
    病理:肉眼可见三联征,静脉血管团,脑萎缩等,镜下:静脉血管壁不规则,扩张,壁厚处玻璃样变,缺乏平滑肌及弹力纤维,不同时期陈旧性出血。

    治疗:1、抗癫痫。2、手术清除血肿,VM切除。3、脑室分流。4、放疗。5、对症,大多数CVM不需要处理。

    Cerebral Venous Malformation

    Cerebral venous malformation (CVM) was also termed as developmental venous anomaly (DVA) or cerebral venous angioma (CVA), and was believed to represent an anatomical variant of the series of cerebral vascular malformations. The incidence of CVM reported previously was quite different, comprised approximately 7.3-63% of cerebral vascular malformations, which was reported to occupy 1.7-6.5% of CVMs in China.
, 百拇医药
    Clinical Presentations: most of CVMs were asymptomatic, on rare occasions, CVMs presented symptoms such as seizures, hemorrhage, intracranial hypertension and neurological defects.

    Neuroradiological Characteristics: CT scan showed hyperintensity (hemorrhage), mixed intensity lesions including calcification (different stages of hemorrhage), periventricular hyperintensity (veins), enlarged ventricles. MRI findings in patients with hemorrhage were mixed hypo- and hyperintensity lesions resembling cavernous angiomas, and in those without hemorrhage showed low signal enlarged vascular structures. On T2-WI, large veins demonstrated empty stream signals, which showed high signals on double-echo sequence. Cerebral angiography showed three characteristics (“triple signs”): lack of superficial veins, Medusa’s sign of deep veins and big draining veins, lacunae and abnormal veins were also encountered.
, http://www.100md.com
    Pathology: macropathology showed previously mentioned “triple signs”, focal venous mass, and atrophy. Micropathology demonstrated dilated veins with irregular wall, lack of smooth muscles and elastic fibers, glassy degeneration was seen at thickened parts. Different stages of hemorrhage were also seen.

    Management: 1. Anti-epileptic treatment. 2. Surgical evacuation of hematomas and resection of VM. 3. Ventricle-peritoneal shunt. 4.Radiotherapy. 5. Conservative treatment., http://www.100md.com(焦德让)