自发性脑室出血与烟雾病合并动脉瘤的诊断及治疗策略

发布时间：2018-02-24 16:22

  本文关键词： 脑底异常血管网病 颅内动脉瘤 出血 诊断 治疗　出处：《南方医科大学》2015年硕士论文　论文类型：学位论文

【摘要】：自发性脑室出血,指出血位于脑室系统或者室管膜细胞的内衬结构,没有明显脑实质出血的表现。相比由脑实质、蛛网膜下腔等部位出血破入脑室系统的继发性脑室出血,自发性脑室出血的预后明显要好。在处理自发性脑出血的病例中,及时脑室外引流、解决脑积水和颅内压问题后,较多的患者都获得满意的疗效,但仍有一部分患者经历再出血而造成预后不良甚至短时间死亡。而在这一部分再出血的病例中,可能包括烟雾病、烟雾病合并动脉瘤、动静脉畸形等的疾病。烟雾病多发于亚洲东部,发病率大约为(0.35-0.94)/10万人。随着人们对烟雾病的认识不断的加深以及MRA和DSA的广泛应用,患病人数呈逐年上升趋势。烟雾病可表现为缺血、出血、癫痫等类型,约一半左右的成年烟雾病患者以出血为主要表现,近年来研究表明,烟雾病合并动脉瘤为烟雾病出血及再出血的重要原因。最初,烟雾病合并动脉瘤被分成主干型和外周型两大类,这种分型对于进一步认识烟雾病合并动脉瘤形成机制和临床特点是局限的,不利于诊断和治疗。1996年,S.Kawaguchi等将动脉瘤分成三类：(1)Willis环类,主要分布于Willis环主干；(2)基底节类,主要分布于新生的烟雾样血管；(3)侧支吻合类：主要分布于侧支吻合远端。该文章展示了关于烟雾病合并动脉瘤分型和特点的新观点,将外周型动脉瘤进一步分成基底节类及侧支吻合类。而近年来,文献报道中还存在硬脑膜类,不能被忽视。Willis环类动脉瘤多有蛛网膜下腔出血的表现,受到重视程度较高,一般都可以通过CTA、MRA或DSA等手段明确诊断,并经过血管内治疗或者外科手术治疗后,大多能疗效满意,随访结果理想。然而,外周型动脉瘤的诊断、治疗和预后仍参差不齐。基底节类动脉瘤破裂出血多表现为基底节区脑出血,常被误认为高血压脑出血,而没有进一步检查,因而治疗上采取控制血压等保守治疗方案。虽然出血原因诊断不明确或者诊断错误,但是治疗效果尚可满意。最大的问题来源于侧支吻合类动脉瘤的处理,是烟雾病合并动脉瘤最危险并且容易被忽视的关键点。侧支吻合类动脉瘤出血多表现为脑室内出血,既往脑血管方面辅助检查尚未广发应用,在传统经验上,多考虑出血原因为高血压性或者微小血管破裂引起,发病患者往往症状相对较轻,通过脑室外引流术或者腰椎穿刺术治疗后,恢复较快,因而出血原因被忽略,缺乏病因治疗,造成严重后果。经过明确诊断的外周型动脉瘤,采用血管内栓塞或者外科手术的处理,成功治疗动脉瘤的报道较多,但一部分术后出现神经功能缺失的情况不容忽视,同时,保守治疗后动脉瘤消失的病例仍有报道,保守治疗后经历再破裂出血的报道也不少。可见,烟雾病合并动脉瘤的处理方案尚未形成共识,还需要继续探讨。本研究立足于烟雾病合并动脉瘤引起脑室出血及再出血的临床病例,一方面对自发性脑室出血的病因进行分析,另一方面探讨烟雾病合并动脉瘤的分型及治疗策略。具体如下：第一部分 自发性脑室出血的病因分析背景 自发性脑室出血,指出血位于脑室系统或者室管膜细胞的内衬结构,约占在所有颅内出血的患者中的3.3%,以往较多观点认为高血压是自发性脑室出血的主要原因,脑室外引流和腰椎穿刺等处理后,尤其是及时的溶栓药物作用下血块溶解和排出有助于患者较快恢复。传统经验治疗后,较多的患者都获得满意的疗效,但仍有一部分患者经历再出血而造成预后不良甚至短时间死亡。可见以往单纯对脑室内血肿的处理是不够的,必须进一步对出血原因进行诊断和治疗。目的 本研究分析自发性脑室出血的病因,提出相关的诊疗策略。方法 回顾性分析南方医科大学第三附属医院神经外科2011年6月至2014年6月共收治自发性脑室出血23例连续性病例(包括3例烟雾病合并动脉瘤),排除外伤因素,其中6例仅行头颅CT平扫,17例行头颅CT及DSA检查。总结其中的临床特点、出血高危因素、影像学检查,并进行出血原因分析及治疗结果随访。结果脑室出血的病例分析表明：(1)在23例患者中,12(52.17%)例明确了脑血管疾病引起出血,6(26.08%)例为烟雾病患者,其中3(13.08%)例为烟雾病合并动脉瘤,3(13.08%)例为烟雾病。3(13.08%)例为动静脉畸形,1(4.34%)例为动静脉瘘,1(4.34%)例为动脉瘤,1(4.34%)例为颅内静脉窦血栓形成；另外4(17.39%)例为高血压引起脑室出血(3例为DSA检查阴性,1例合并未破裂动脉瘤)；(2)17例行DSA检查,明确12例为脑血管疾病引起出血,阳性率为70.59%,余下4例未见异常,1例为未破裂动脉瘤,与脑室出血无关；所有病例被分成两组,年轻组(年龄≤60),阳性率为73.3%(11/15),年长组(年龄60),阳性率为50%(1/2)；(3)4例再出血患者,其中2例为烟雾病,2例未行DSA检查,4例均未对出血原因进行处理。结论自发性脑室出血较多是来自脑血管疾病的急性发作,纳入的病例资料显示烟雾病为最重要的病因,其次为高血压和动静脉畸形。在年龄≤60组别的自发性脑室出血病例中,脑血管疾病的阳性率高达73.3%。诊断高血压引起脑室内出血必须要排除脑血管疾病的因素。自发性脑室出血应常规行脑血管相关检查(CTA/DSA)。第二部分烟雾病合并动脉瘤的分型及治疗策略背景烟雾病合并动脉瘤为烟雾病出血的重要原因。最初,烟雾病合并动脉瘤被分成主干型和外周型两大类,对于主干型动脉瘤,只能通过外科手术处理,而对于外周型动脉瘤,被认为手术难度大、风险高、容易损伤神经功能,采取保守治疗。1996,S.Kawaguchi等将动脉瘤分成三类：Willis环类、基底节类和侧支吻合类,展示了对烟雾病合并动脉瘤新的认识。目前,Willis环类动脉瘤多有蛛网膜下腔出血的表现,受到重视程度较高,并经过血管内治疗或者外科手术治疗后,大多能疗效满意,随访结果理想。而基底节类和侧支吻合类动脉瘤可以被经验丰富的神经外科医师诊断明确,然而,在较多的情况下被忽视而漏诊,尤其是以脑室出血为主要表现的患者,一旦漏诊,治疗结果可能较差甚至死亡。治疗方案包括保守治疗、血管内栓塞和外科手术,尚未形成统一认识、治疗水平的参差不齐。文献报道还存在硬脑膜类的动脉瘤,其治疗方案也面临困难。因此烟雾病合并动脉瘤分型和各个类型的治疗策略方面仍存在较多的问题。目的本研究归纳总结出血性烟雾病合并动脉瘤的诊断路径、治疗方案及随访结果,分析动脉瘤的特点,评估破裂风险,并探讨烟雾病合并动脉瘤的诊断及治疗。方法自2011年6月至2014年6月,严格按照诊断指南及适应症,纳入10例(12个动脉瘤)出血性烟雾病合并动脉瘤患者,全部经CT及DSA检查,由单一的脑血管疾病治疗团队明确诊断并根据不同动脉瘤的位置和特点,采取不同的治疗方法。12个动脉瘤中,7个位于Willis环,2个位于基底节区,3个位于侧支吻合动脉；责任动脉瘤9个,非责任动脉瘤3个。7个Willis环动脉瘤中,4个为责任动脉瘤,3个行单纯弹簧圈栓塞,1个行开颅动脉瘤夹闭；3个为非责任动脉瘤,分别为2个采取择期行支架辅助弹簧圈栓塞,1个采取保守治疗。2个基底节类动脉瘤,均为责任动脉瘤,给予保守治疗；3个侧支吻合类动脉瘤,均为责任动脉瘤,其中2个采用33%Glubran胶栓塞,1个给予保守治疗。结果烟雾病合并动脉瘤的治疗结果：(1)积极治疗的6个Willis环类动脉瘤,均无动脉瘤残留;1个保守治疗者,动脉瘤情况稳定。2个基底节类动脉瘤,分别于保守治疗后3、8个月动脉瘤消失。3个侧支吻合类动脉瘤,2个予栓塞治疗,无动脉瘤残留；1个予保守治疗,14d后再次出血而死亡。(2)出院时改良Rankin量表评分,0分5例,1分2例,2分1例,3分1例,4分0例,6分1例。(3)9例获得2～36个月随访,经积极治疗的6个Willis环类动脉瘤、2个侧支吻合类动脉瘤及保守治疗后消失的2个基底节类动脉瘤均无复发,1个保守治疗的Willis环类动脉瘤稳定。结论烟雾病合并动脉瘤主要分为三类,对于Wllis环类、侧支吻合类动脉瘤需要积极处理,首选血管内栓塞,其次考虑外科手术。而基底节类动脉瘤破裂风险相对小,可考虑保守治疗及随访。
[Abstract]:Spontaneous intraventricular hemorrhage, hemorrhage in the ventricular system or the ependymal cells lining structure, showed no obvious cerebral parenchymal hemorrhage. Compared the brain parenchyma, subarachnoid hemorrhage breaking into the ventricle system of secondary intraventricular hemorrhage, better prognosis of spontaneous intraventricular hemorrhage. In the treatment of spontaneous intracerebral hemorrhage cases. Timely ventricular drainage and solve the problems of water and intracranial brain pressure, more patients can obtain satisfactory results, but there are still some patients experienced bleeding caused by poor prognosis even death in short time. And in this part the rebleeding cases, may include moyamoya disease, intracranial aneurysms associated with moyamoya disease, animal venous malformation disease. Multiple moyamoya disease in eastern Asia, the incidence rate of about /10 million (0.35-0.94). With the understanding of moyamoya disease continues to deepen, MRA and DSA widely used in The number is increasing year by year. Moyamoya disease can be manifested as ischemia, hemorrhage, epilepsy and other types of adult patients with moyamoya disease about half with hemorrhage as the main performance, recent studies show that an important reason for intracranial aneurysms associated with moyamoya disease moyamoya disease hemorrhage and rebleeding. Initially, intracranial aneurysms associated with moyamoya disease were divided into the trunk type and peripheral type two categories, this type is limited for a further understanding of moyamoya disease with aneurysm formation mechanism and clinical characteristics, is not conducive to the diagnosis and treatment of.1996, S.Kawaguchi and the aneurysm is divided into three categories: (1) Willis rings, Willis rings are mainly distributed in the main substrate (2); class, smoke like blood vessels are mainly distributed in the newborn; (3): collateral anastomoses are mainly distributed in the distal anastomoses. The article shows the new viewpoint about the type and characteristics of intracranial aneurysms associated with moyamoya disease of peripheral aneurysms further Into the basal ganglia and collateral anastomoses. In recent years, there are still such dural reported in the literature can not be ignored,.Willis ring aneurysm subarachnoid hemorrhage showed high degree of attention, can generally be through CTA, MRA or DSA and other means of diagnosis, and after endovascular treatment or surgery after surgical treatment, most satisfactory curative effect and follow-up results. However, the diagnosis of peripheral artery aneurysms, treatment and prognosis of basal ganglia is still uneven. Such aneurysms showed hemorrhage in basal ganglia, is often mistaken for high blood pressure cerebral hemorrhage, without further examination, and take treatment conservative treatment for control of blood pressure. Although the cause of hemorrhage diagnosis is not clear or incorrect diagnosis, but the treatment effect is satisfactory. The biggest problem comes from the processing class collateral anastomoses of the aneurysm is intracranial aneurysms associated with moyamoya disease The key point of the most dangerous and easily overlooked. As collateral anastomoses showed hemorrhage intraventricular hemorrhage type aneurysm, history of cerebral vascular examination has not been widely used, in the traditional experience, consider the bleeding due to hypertension or microvascular rupture caused by disease, patients often relatively mild symptoms, through external ventricular drainage or the lumbar puncture after treatment, rapid recovery, and the cause of bleeding is ignored, the lack of etiological treatment, causing serious consequences. After peripheral aneurysms diagnosed and treated by endovascular embolization or surgery, successful treatment of aneurysms are reported, but a part of postoperative neurologic deficit situation can not be ignored, at the same time, the aneurysm disappeared after conservative treatment cases have been reported, after conservative treatment experienced rebleeding reports. Many visible, intracranial aneurysms associated with moyamoya disease Treatment plan has not yet formed a consensus, but also need to continue to explore. This research is based on the intracranial aneurysms associated with moyamoya disease caused by clinical cases of intraventricular hemorrhage and rebleeding, analyze the cause of spontaneous intraventricular hemorrhage on the one hand, on the other hand, discuss the classification and treatment strategy of intracranial aneurysms associated with moyamoya disease. The details are as follows: the first part is the analysis of the cause the background of spontaneous intraventricular hemorrhage of spontaneous intraventricular hemorrhage, hemorrhage in the ventricular system or the ependymal cells lining structure, accounted for in all patients with intracranial hemorrhage in 3.3%, before most people think that high blood pressure is the main cause of spontaneous intraventricular hemorrhage, ventricle drainage and lumbar puncture treatment, especially thrombolytic drugs the effect of timely clot lysis and discharge helps patients recover quickly after treatment. The traditional experience, more patients can obtain satisfactory curative effect, but there is still a Patients experience bleeding caused by poor prognosis even death in short time. The past of intraventricular hematoma visible processing is not enough, we must further diagnosis and treatment of bleeding. The purpose of this study is analysis of the causes of spontaneous intraventricular hemorrhage, propose treatment strategy. Methods: a retrospective analysis of the Third Affiliated Hospital of Southern Medical University from June 2011 to the Department of neurosurgery in June 2014 a total of 23 consecutive patients with spontaneous intraventricular hemorrhage (including 3 cases of intracranial aneurysms associated with moyamoya disease), excluding traumatic factors, of which 6 cases underwent CT plain scan, 17 cases underwent CT and DSA examination. The clinical features of bleeding, risk factors, imaging examination, and analysis of the causes of hemorrhage the results show that analysis and treatment follow-up. Results: intraventricular hemorrhage (1 cases) in 23 patients, 12 (52.17%) cases of the cerebrovascular disease caused bleeding, 6 (26. 08%)渚嬩负鐑熼浘鐥呮偅鑰，

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