本文關(guān)鍵詞： 腦底異常血管網(wǎng)病 顱內(nèi)動(dòng)脈瘤 出血 診斷 治療　出處：《南方醫(yī)科大學(xué)》2015年碩士論文　論文類型：學(xué)位論文

【摘要】：自發(fā)性腦室出血,指出血位于腦室系統(tǒng)或者室管膜細(xì)胞的內(nèi)襯結(jié)構(gòu),沒有明顯腦實(shí)質(zhì)出血的表現(xiàn)。相比由腦實(shí)質(zhì)、蛛網(wǎng)膜下腔等部位出血破入腦室系統(tǒng)的繼發(fā)性腦室出血,自發(fā)性腦室出血的預(yù)后明顯要好。在處理自發(fā)性腦出血的病例中,及時(shí)腦室外引流、解決腦積水和顱內(nèi)壓?jiǎn)栴}后,較多的患者都獲得滿意的療效,但仍有一部分患者經(jīng)歷再出血而造成預(yù)后不良甚至短時(shí)間死亡。而在這一部分再出血的病例中,可能包括煙霧病、煙霧病合并動(dòng)脈瘤、動(dòng)靜脈畸形等的疾病。煙霧病多發(fā)于亞洲東部,發(fā)病率大約為(0.35-0.94)/10萬人。隨著人們對(duì)煙霧病的認(rèn)識(shí)不斷的加深以及MRA和DSA的廣泛應(yīng)用,患病人數(shù)呈逐年上升趨勢(shì)。煙霧病可表現(xiàn)為缺血、出血、癲癇等類型,約一半左右的成年煙霧病患者以出血為主要表現(xiàn),近年來研究表明,煙霧病合并動(dòng)脈瘤為煙霧病出血及再出血的重要原因。最初,煙霧病合并動(dòng)脈瘤被分成主干型和外周型兩大類,這種分型對(duì)于進(jìn)一步認(rèn)識(shí)煙霧病合并動(dòng)脈瘤形成機(jī)制和臨床特點(diǎn)是局限的,不利于診斷和治療。1996年,S.Kawaguchi等將動(dòng)脈瘤分成三類：(1)Willis環(huán)類,主要分布于Willis環(huán)主干；(2)基底節(jié)類,主要分布于新生的煙霧樣血管；(3)側(cè)支吻合類：主要分布于側(cè)支吻合遠(yuǎn)端。該文章展示了關(guān)于煙霧病合并動(dòng)脈瘤分型和特點(diǎn)的新觀點(diǎn),將外周型動(dòng)脈瘤進(jìn)一步分成基底節(jié)類及側(cè)支吻合類。而近年來,文獻(xiàn)報(bào)道中還存在硬腦膜類,不能被忽視。Willis環(huán)類動(dòng)脈瘤多有蛛網(wǎng)膜下腔出血的表現(xiàn),受到重視程度較高,一般都可以通過CTA、MRA或DSA等手段明確診斷,并經(jīng)過血管內(nèi)治療或者外科手術(shù)治療后,大多能療效滿意,隨訪結(jié)果理想。然而,外周型動(dòng)脈瘤的診斷、治療和預(yù)后仍參差不齊�；坠�(jié)類動(dòng)脈瘤破裂出血多表現(xiàn)為基底節(jié)區(qū)腦出血,常被誤認(rèn)為高血壓腦出血,而沒有進(jìn)一步檢查,因而治療上采取控制血壓等保守治療方案。雖然出血原因診斷不明確或者診斷錯(cuò)誤,但是治療效果尚可滿意。最大的問題來源于側(cè)支吻合類動(dòng)脈瘤的處理,是煙霧病合并動(dòng)脈瘤最危險(xiǎn)并且容易被忽視的關(guān)鍵點(diǎn)。側(cè)支吻合類動(dòng)脈瘤出血多表現(xiàn)為腦室內(nèi)出血,既往腦血管方面輔助檢查尚未廣發(fā)應(yīng)用,在傳統(tǒng)經(jīng)驗(yàn)上,多考慮出血原因?yàn)楦哐獕盒曰蛘呶⑿⊙芷屏岩?發(fā)病患者往往癥狀相對(duì)較輕,通過腦室外引流術(shù)或者腰椎穿刺術(shù)治療后,恢復(fù)較快,因而出血原因被忽略,缺乏病因治療,造成嚴(yán)重后果。經(jīng)過明確診斷的外周型動(dòng)脈瘤,采用血管內(nèi)栓塞或者外科手術(shù)的處理,成功治療動(dòng)脈瘤的報(bào)道較多,但一部分術(shù)后出現(xiàn)神經(jīng)功能缺失的情況不容忽視,同時(shí),保守治療后動(dòng)脈瘤消失的病例仍有報(bào)道,保守治療后經(jīng)歷再破裂出血的報(bào)道也不少�？梢�,煙霧病合并動(dòng)脈瘤的處理方案尚未形成共識(shí),還需要繼續(xù)探討。本研究立足于煙霧病合并動(dòng)脈瘤引起腦室出血及再出血的臨床病例,一方面對(duì)自發(fā)性腦室出血的病因進(jìn)行分析,另一方面探討煙霧病合并動(dòng)脈瘤的分型及治療策略。具體如下：第一部分 自發(fā)性腦室出血的病因分析背景 自發(fā)性腦室出血,指出血位于腦室系統(tǒng)或者室管膜細(xì)胞的內(nèi)襯結(jié)構(gòu),約占在所有顱內(nèi)出血的患者中的3.3%,以往較多觀點(diǎn)認(rèn)為高血壓是自發(fā)性腦室出血的主要原因,腦室外引流和腰椎穿刺等處理后,尤其是及時(shí)的溶栓藥物作用下血塊溶解和排出有助于患者較快恢復(fù)。傳統(tǒng)經(jīng)驗(yàn)治療后,較多的患者都獲得滿意的療效,但仍有一部分患者經(jīng)歷再出血而造成預(yù)后不良甚至短時(shí)間死亡。可見以往單純對(duì)腦室內(nèi)血腫的處理是不夠的,必須進(jìn)一步對(duì)出血原因進(jìn)行診斷和治療。目的 本研究分析自發(fā)性腦室出血的病因,提出相關(guān)的診療策略。方法 回顧性分析南方醫(yī)科大學(xué)第三附屬醫(yī)院神經(jīng)外科2011年6月至2014年6月共收治自發(fā)性腦室出血23例連續(xù)性病例(包括3例煙霧病合并動(dòng)脈瘤),排除外傷因素,其中6例僅行頭顱CT平掃,17例行頭顱CT及DSA檢查。總結(jié)其中的臨床特點(diǎn)、出血高危因素、影像學(xué)檢查,并進(jìn)行出血原因分析及治療結(jié)果隨訪。結(jié)果腦室出血的病例分析表明：(1)在23例患者中,12(52.17%)例明確了腦血管疾病引起出血,6(26.08%)例為煙霧病患者,其中3(13.08%)例為煙霧病合并動(dòng)脈瘤,3(13.08%)例為煙霧病。3(13.08%)例為動(dòng)靜脈畸形,1(4.34%)例為動(dòng)靜脈瘺,1(4.34%)例為動(dòng)脈瘤,1(4.34%)例為顱內(nèi)靜脈竇血栓形成；另外4(17.39%)例為高血壓引起腦室出血(3例為DSA檢查陰性,1例合并未破裂動(dòng)脈瘤)；(2)17例行DSA檢查,明確12例為腦血管疾病引起出血,陽性率為70.59%,余下4例未見異常,1例為未破裂動(dòng)脈瘤,與腦室出血無關(guān)；所有病例被分成兩組,年輕組(年齡≤60),陽性率為73.3%(11/15),年長(zhǎng)組(年齡60),陽性率為50%(1/2)；(3)4例再出血患者,其中2例為煙霧病,2例未行DSA檢查,4例均未對(duì)出血原因進(jìn)行處理。結(jié)論自發(fā)性腦室出血較多是來自腦血管疾病的急性發(fā)作,納入的病例資料顯示煙霧病為最重要的病因,其次為高血壓和動(dòng)靜脈畸形。在年齡≤60組別的自發(fā)性腦室出血病例中,腦血管疾病的陽性率高達(dá)73.3%。診斷高血壓引起腦室內(nèi)出血必須要排除腦血管疾病的因素。自發(fā)性腦室出血應(yīng)常規(guī)行腦血管相關(guān)檢查(CTA/DSA)。第二部分煙霧病合并動(dòng)脈瘤的分型及治療策略背景煙霧病合并動(dòng)脈瘤為煙霧病出血的重要原因。最初,煙霧病合并動(dòng)脈瘤被分成主干型和外周型兩大類,對(duì)于主干型動(dòng)脈瘤,只能通過外科手術(shù)處理,而對(duì)于外周型動(dòng)脈瘤,被認(rèn)為手術(shù)難度大、風(fēng)險(xiǎn)高、容易損傷神經(jīng)功能,采取保守治療。1996,S.Kawaguchi等將動(dòng)脈瘤分成三類：Willis環(huán)類、基底節(jié)類和側(cè)支吻合類,展示了對(duì)煙霧病合并動(dòng)脈瘤新的認(rèn)識(shí)。目前,Willis環(huán)類動(dòng)脈瘤多有蛛網(wǎng)膜下腔出血的表現(xiàn),受到重視程度較高,并經(jīng)過血管內(nèi)治療或者外科手術(shù)治療后,大多能療效滿意,隨訪結(jié)果理想。而基底節(jié)類和側(cè)支吻合類動(dòng)脈瘤可以被經(jīng)驗(yàn)豐富的神經(jīng)外科醫(yī)師診斷明確,然而,在較多的情況下被忽視而漏診,尤其是以腦室出血為主要表現(xiàn)的患者,一旦漏診,治療結(jié)果可能較差甚至死亡。治療方案包括保守治療、血管內(nèi)栓塞和外科手術(shù),尚未形成統(tǒng)一認(rèn)識(shí)、治療水平的參差不齊。文獻(xiàn)報(bào)道還存在硬腦膜類的動(dòng)脈瘤,其治療方案也面臨困難。因此煙霧病合并動(dòng)脈瘤分型和各個(gè)類型的治療策略方面仍存在較多的問題。目的本研究歸納總結(jié)出血性煙霧病合并動(dòng)脈瘤的診斷路徑、治療方案及隨訪結(jié)果,分析動(dòng)脈瘤的特點(diǎn),評(píng)估破裂風(fēng)險(xiǎn),并探討煙霧病合并動(dòng)脈瘤的診斷及治療。方法自2011年6月至2014年6月,嚴(yán)格按照診斷指南及適應(yīng)癥,納入10例(12個(gè)動(dòng)脈瘤)出血性煙霧病合并動(dòng)脈瘤患者,全部經(jīng)CT及DSA檢查,由單一的腦血管疾病治療團(tuán)隊(duì)明確診斷并根據(jù)不同動(dòng)脈瘤的位置和特點(diǎn),采取不同的治療方法。12個(gè)動(dòng)脈瘤中,7個(gè)位于Willis環(huán),2個(gè)位于基底節(jié)區(qū),3個(gè)位于側(cè)支吻合動(dòng)脈；責(zé)任動(dòng)脈瘤9個(gè),非責(zé)任動(dòng)脈瘤3個(gè)。7個(gè)Willis環(huán)動(dòng)脈瘤中,4個(gè)為責(zé)任動(dòng)脈瘤,3個(gè)行單純彈簧圈栓塞,1個(gè)行開顱動(dòng)脈瘤夾閉；3個(gè)為非責(zé)任動(dòng)脈瘤,分別為2個(gè)采取擇期行支架輔助彈簧圈栓塞,1個(gè)采取保守治療。2個(gè)基底節(jié)類動(dòng)脈瘤,均為責(zé)任動(dòng)脈瘤,給予保守治療；3個(gè)側(cè)支吻合類動(dòng)脈瘤,均為責(zé)任動(dòng)脈瘤,其中2個(gè)采用33%Glubran膠栓塞,1個(gè)給予保守治療。結(jié)果煙霧病合并動(dòng)脈瘤的治療結(jié)果：(1)積極治療的6個(gè)Willis環(huán)類動(dòng)脈瘤,均無動(dòng)脈瘤殘留;1個(gè)保守治療者,動(dòng)脈瘤情況穩(wěn)定。2個(gè)基底節(jié)類動(dòng)脈瘤,分別于保守治療后3、8個(gè)月動(dòng)脈瘤消失。3個(gè)側(cè)支吻合類動(dòng)脈瘤,2個(gè)予栓塞治療,無動(dòng)脈瘤殘留；1個(gè)予保守治療,14d后再次出血而死亡。(2)出院時(shí)改良Rankin量表評(píng)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例獲得2～36個(gè)月隨訪,經(jīng)積極治療的6個(gè)Willis環(huán)類動(dòng)脈瘤、2個(gè)側(cè)支吻合類動(dòng)脈瘤及保守治療后消失的2個(gè)基底節(jié)類動(dòng)脈瘤均無復(fù)發(fā),1個(gè)保守治療的Willis環(huán)類動(dòng)脈瘤穩(wěn)定。結(jié)論煙霧病合并動(dòng)脈瘤主要分為三類,對(duì)于Wllis環(huán)類、側(cè)支吻合類動(dòng)脈瘤需要積極處理,首選血管內(nèi)栓塞,其次考慮外科手術(shù)。而基底節(jié)類動(dòng)脈瘤破裂風(fēng)險(xiǎn)相對(duì)小,可考慮保守治療及隨訪。
[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%)渚嬩負(fù)鐑熼浘鐥呮?zhèn)ｈ�，

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