Posterior Cerebral Artery Insufficiency in Pediatric Moyamoya Disease

The majority of clinical studies on moyamoya disease (MMD) have focused on anterior circulation. The disease involvement of posterior circulation in MMD, mainly in the posterior cerebral artery (PCA), has been mentioned since the early 1980s, and it has been repeatedly emphasized as one of the most important factors related to poor prognosis in MMD. However, its clinical features and outcome have only been elucidated during the last few years. In this review, the angiographic definition of PCA stenosis is summarized. The clinical features are elucidated as being either early-onset or delayed-onset, according to the time of PCA stenosis diagnosis in reference to the anterior circulation revascularization surgeries. The surgical strategy and hypothesis on the mechanism of PCA stenosis is also briefly mentioned. It appears that some MMD patients may show PCA stenosis during the early or late course of the disease and that the presenting symptoms may vary. Because the hemodynamic compromise caused by PCA stenosis may respond well to surgical treatment, clinicians should be aware of the condition, especially during follow-up of MMD patients.     

Posterior cerebral artery involvement in moyamoya disease: initial infarction and angle between PCA and basilar artery

Purpose

Moyamoya disease (MMD) is a chronic cerebrovascular occlusive disease, and progressive involvement of the posterior cerebral artery (PCA) has been reported. However, majority of MMD articles are presenting classic anterior circulation related issues. This study investigates the preoperative factors related to the long-term outcome of posterior circulation in MMD.

Methods

Retrospective review of 88 MMD patients (166 PCAs in either hemisphere) without symptomatic disease involvement of PCA at initial diagnosis was done. Data at initial diagnosis regarding age, presence of infarction, status of the PCA, type of posterior communicating artery, and the angle between PCA and basilar artery were reviewed. Progressive stenosis of PCA was evaluated by symptom or radiological imaging during follow up.

Results

During an average follow up of 8.3 years, 29 out of 166 (18 %) evaluated PCAs showed progressive disease involvement. The average time of progression from the initial operation was 4.9 years, with the latest onset at 10.8 years. The patients who showed progressive stenosis of the PCA tended to be younger, present with infarction, have smaller angle between PCA and basilar artery, and have asymptomatic stenosis of the PCA at initial presentation. However, multivariate analysis confirmed only the presence of initial infarction and a smaller angle between PCA and basilar artery to be significantly associated with progressive stenosis of PCA.

Conclusions

Involvement of PCA in MMD may occur in a delayed fashion, years after the completion of revascularization of anterior circulation. Persistent long-term follow-up regarding the posterior circulation is recommended.     

10例双侧颈动脉重度狭窄或闭塞患者的临床特点分析

目的探讨双侧颈动脉重度狭窄或闭塞患者的临床特点。方法回顾性分析10例双侧动脉重度狭窄或闭塞患者临床特点及血流代偿情况。结果 10例患者中,双侧颈总动脉闭塞和颈内动脉闭塞各1例,双侧颈内动脉重度狭窄5例,一侧闭塞合并一侧重度狭窄3例。临床表现为后循环缺血5例,后循环合并前循环缺血3例,前循环缺血2例。7例行DSA患者,有7例后交通动脉开放,4例前交通动脉开放。结论双侧颈动脉重度狭窄或闭塞患者临床表现以后循环缺血多见,后交通动脉可能为其主要代偿模式。     

缺血型烟雾病脑硬膜动脉血管融通术后血管重建效果及影响因素

目的 探讨缺血型烟雾病脑硬膜动脉血管融通术（encephalo-duro-arterio-synangiosis,EDAS）后血管重建效果及影响因素。方法 回顾性分析在我院接受EDAS术治疗的120例缺血型烟雾病患者的临床资料,对性别、首次手术年龄、术前脑糖代谢情况、脑梗死类型、手术侧别、颈内动脉分期、大脑后动脉分期、前后循环间代偿以及左右侧代偿与术后动脉向颅内生长情况之间进行多因素logistic回归分析。结果 本组病例中102例患者接受双侧手术,18例接受单侧手术,总共手术为222例侧。术后动脉向颅内生长的有174例侧,血管重建有效率78.4%。多因素logistic回归分析显示首次手术年龄［优势比（odds ratio,OR）为1.03,95%可信区间（confidence interval,CI）1.012～1.049］,大脑后动脉分期（OR =0.767,95%CI 0.601～0.977,P =0.0319）,左右侧代偿（OR =0.499,95%CI 0.280～0.891,P =0.0188）及脑梗死类型与血管重建效果有关。相对于正常脑组织,皮层梗死（OR =0.275,95%CI 0.133～0.569,P =0.0005）、皮层下白质梗死（OR =0.317,95%CI 0.131～0.763,P =0.0104）和点状梗死（OR =0.392,95%CI 0.193～0.796,P =0.0096）血管重建效果更好。结论 EDAS可以有效建立颅内外血管重建。首次手术年龄越低、大脑后动脉分期越高、有左右侧代偿可以获得更好的术后血管重建;相对于正常脑组织,出现皮层梗死、皮层下白质梗死和点状梗死类型脑组织可获得更好血管重建效果     

大脑后动脉区梗死与后循环血管状态及危险因素关系

目的探讨大脑后动脉区梗死与后循环血管状态及危险因素的关系。方法回顾性收集2010年1月-2014年6月在首都医科大学宣武医院神经内科住院的经头部MRI证实的首次新发大脑后动脉区梗死的患者192例,其中171例行CT血管成像,21例行DSA,排除有可疑心源性栓子来源的患者。根据头部MRI将梗死部位分为A(中脑组)、B(丘脑组)、C(颞叶内侧组)、D(胼胝体压部组)、E(枕叶组)、F(多部位梗死组)6组。比较PCA区梗死与后循环血管状态及危险因素的关系。结果 (1)PCA区梗死病变分布:192例患者中A、B、C、D、E、F组分别为12例(6.3%)、74例(38.5%)、15例(7.8%)、11例(5.7%)、28例(14.6%)、52例(27.1%)。(2)椎-基底动脉系统血管状态:椎动脉病变66例(34.4%)合并大脑后动脉狭窄13例,基底动脉病变27例(14.1%)合并大脑后动脉狭窄2例,大脑后动脉病变45例(23.4%),未发现血管病变69例(35.9%)。(3)对PCA区梗死病变分布与后循环血管病变行单因素卡方检验:B组与基底动脉病变相关(χ~2=5.318,P=0.021);E组与PCA P4段病变相关(χ~2=18.556,P0.001);F组与椎动脉、基底动脉病变相关(χ~2=4.386,7.059;P=0.036,0.008)。(4)血管病变组与无血管病变组比较:合并高血压病差异有统计学意义(11.126,P=0.001)。结论大脑后动脉区梗死与后循环血管病变关系密切,尤其合并导致血管病变的危险因素时,更应重视血管检查,减少卒中再发。     

Posterior circulation abnormalities in moyamoya disease : a radiological study.

Moyamoya disease (MMD) is an uncommon entity outside Japan. Though the clinical and radiological features are well described, involvement of the posterior circulation has not been highlighted. Out of 10 patients of MMD studied, the posterior circulation was involved in 9 (3 bilateral, 6 unilateral). The P1 segment was most commonly affected. Interestingly, no infarcts were seen in the territory of the posterior circulation in any patient. Five patients showed recent haemorrhages on scan. It was thalamic haemorrhage in four and subarachnoid in one patient. The posterior circulation is frequently involved in MMD as evident on angiography. However, ischaemic events of the posterior circulation are not frequent, as the posterior circulation acts as collateral pathway for the diseased anterior circulation till later stages of the disease.     

急性脑梗死患者脑动脉狭窄的分布及危险因素分析

目的分析急性脑梗死患者脑动脉狭窄的具体分布特点以及相关危险因素。方法选取焦作煤业集团中央医院2010-02—2017-02收治的516例急性脑梗死脑动脉狭窄患者为研究对象,所有患者经DSA(数字减影血管造影)证实,将其归为观察组,再选取同时期焦作煤业集团中央医院诊治的386例无脑动脉狭窄的脑梗死患者为对照组,分析观察组患者的脑动脉狭窄分布情况,同时对脑动脉狭窄形成的影响因素行单因素与多因素Logistic回归分析,明确影响脑动脉狭窄的相关危险因素。结果320例为单纯颅内动脉狭窄,构成比为62.0%;88例为单纯颅外动脉狭窄,构成比为17.1%;108例为颅内合并颅外动脉狭窄,构成比为20.9%。376例为前循环动脉狭窄,构成比为72.9%;140例为后循环动脉狭窄,构成比为27.1%。脑动脉狭窄以颅内动脉狭窄、前循环动脉狭窄为主。多因素Logistic回归分析显示,脑动脉狭窄形成的独立危险因素包括尿酸(UA)、收缩压、吸烟(P<0.05)。结论急性脑梗死患者脑动脉狭窄以颅内动脉狭窄、前循环动脉狭窄为主,UA、收缩压、吸烟为脑动脉狭窄形成的独立危险因素。     

Fetal posterior cerebral artery in pediatric and adult moyamoya disease: A single-center experience of 480 patients

PurposeIn past pediatric and adult cohort studies of moyamoya disease, the fetal posterior cerebral artery has received less attention. Its relationship with the clinical manifestations and collateral circulation of moyamoya disease or ipsilateral cerebral hemispheres remains unclear.MethodWe summarize the clinical features of patients with and without fetal posterior cerebral artery moyamoya disease from consecutive cases.We explored the relationship between fetal posterior cerebral arteries and collateral circulation in the ipsilateral cerebral hemispheres, as well as differences among different subgroups of patients.According to the morphology, the fetal posterior cerebral artery is divided into complete fetal posterior cerebral artery and partial fetal posterior cerebral artery. Clinical features were classified as: infarction,hemorrhage,and non-stroke in unilateral/bilateral cerebral hemispheres. Collateral circulation is divided into extracranial vascular compensation and leptomeningeal collateral circulation. Digital subtraction angiography and CT/MR were used to evaluate the blood flow status and clinical characteristics of patients with moyamoya disease.ResultA total of 960 cerebral hemispheres from 142 pediatric patients and 338 adult patients were included in the study. A total of 273 (56.9%) patients had 399 cerebral hemispheres (41.6%) with fetal posterior cerebral arteries. Adults with fetal posterior cerebral arteries had lower rates of infarction (24.6%vs37.3%, P =0.005) and were less likely to have bilateral stroke (8.4%vs11.5%, P =0.038). Cerebral hemispheres with fetal posterior cerebral artery were more likely to have anterior cerebral artery and middle cerebral artery stenosis and less likely to have occlusion (P =0.002, 0.001), and less likely to involve the posterior circulation (P < 0.001). The cerebral hemispheres of the fetal posterior cerebral artery had higher leptomeningeal collateral circulation scores. There are significant differences in extracranial vascular compensation between cerebral hemispheres with and without fetal posterior cerebral artery. Adult patients with fetal posterior cerebral artery were more advanced in Suzuki stage (P =0.017).ConclusionsOur results suggest that fetal posterior cerebral artery is associated with infarct manifestations in pediatric and adult moyamoya disease. In the cerebral hemispheres, the fetal posterior cerebral artery is associated with ipsilateral hemispheric anterior and posterior circulation artery injury, extracranial vascular compensation, leptomeningeal collateral circulation compensation, and infarction. Adult patients with fetal posterior cerebral artery were more advanced in Suzuki stage.     

Posterior Cerebral Artery Aneurysm Re-Rupture Following Revascularization for Moyamoya Disease

ObjectivesAneurysms associated with hemorrhagic moyamoya disease (MMD) are reported to stabilize or recede following revascularization.Case report/resultsA 29 year-old male with no past medical history presented obtunded with diffuse intraventricular hemorrhage and vascular imaging demonstrating bilateral MMD without any associated aneurysms. He underwent a delayed right-sided STA-MCA bypass and pial synangiosis, and was subsequently discharged on hospital day 24 with a modified Rankin Scale score (mRS) of 2. He returned eleven days later from a rehabilitation facility with recurrent IVH. A saccular 5 mm right P4 segment posterior cerebral artery aneurysm was seen on a diagnostic angiogram and embolized with Onyx glue.ConclusionsDistal posterior circulation artery aneurysmal rupture is a rare cause of hemorrhagic MMD. This case demonstrates the capacity of these aneurysms to re-rupture following revascularization and underscores the importance of treating the aneurysms directly.     

64层螺旋CT对头颈部血管病变的临床价值探讨

目的探讨64层螺旋CT血管成像对头颈部血管病变的临床价值。方法34例疑头颈部血管病变的患者行64层螺旋CT头颈部血管成像(CTA),扫描数据在ADW 4.3工作站用不同的后处理技术进行血管后重建。结果34例患者的双侧颈内动脉(ICA)、椎动脉(VA)、大脑前动脉(ACA)、大脑中动脉(MCA)、大脑后动脉(PCA)及Willis环均能显示,共检出狭窄76段,闭塞2段,动脉瘤5例;血管异常起源3例,正常者14例。结论64层螺旋CT血管成像可以清晰显示头颈部血管病变,对临床诊疗起重要指导作用。     

颅底动脉环异常与老年人缺血性脑血管病关系临床探讨

目的探讨颅底动脉环(Willis)的形态变异与缺血性脑血管病的关系,为临床治疗方法选择提供解剖依据。方法采用数字减影血管造影技术(DSA)对64例老年缺血性脑血管病患者行主动脉弓和全脑血管造影,应用A1优势征判断Willis前环的完整性,以大脑后动脉供血类型判断Willis后环的完整性。结果本组病人中A1优势征者43.8%;大脑后动脉供血为基底动脉供血型者40.6%;颈动脉供血型者21.9%;混合供血型者37.5%。共检出血管狭窄31例,临床症状和血管狭窄部位符合者23例(74.2%),不符者8例(25.8%)。结论Willis前环的A1优势征和Willis后环中大脑后动脉供血为颈动脉供血型、混合供血型者提示Willis动脉环形态变异;形态异常的Willis动脉环与缺血性脑血管病的发病及发病后的血流动力学代偿密切相关;血流动力学的改变可通过侧支循环造成临床症状和血管狭窄部位不符,可能影响脑梗死后血管活性药物的选择及超选择性动脉溶栓时溶栓部位的选择。     

螺旋CT 血管生长技术及脑血管造影在烟雾病诊断中的价值

目的 探讨使用多排螺旋CT 血管生成技术(MSCTA)及脑数字减影(DSA)血管造影在烟雾病(MMD)中的诊断价值.方法 采用8 排螺旋CT 进行头部螺旋容积扫描,分析23 例烟雾病的CT 平扫及增强的影像表现,利用"血管生长(add vessel)"法对图像进行后处理,采用多平面体积重组及容积重建图像对脑血管进行三维重建并和DSA 结果进行比较分析.结果 CT 平扫13 例患者显示不同部位的脑出血,5 例患者表现为多发性的脑梗死,3 例患者仅见局限性的脑萎缩,2 例患者CT 平扫未发现明显异常.MSCTA 均清晰显示不同程度的颈内动脉颅内段和(或)大脑前动脉、大脑中动脉狭窄或闭塞,23 例均显示纤细迂曲的"烟雾"状异常血管网.其中15 例经股动脉插管行全脑数字血管减影(DSA)证实.结论 采用血管生成、容积重建技术,MSCTA 是明确诊断烟雾病的有效非创伤性首选检查方法,可替代DSA 进行烟雾病的筛选、诊断和随访观察.     

血管内支架植入术治疗脑动脉盗血综合征16例疗效观察

目的探讨支架成形术治疗因锁骨下动脉、颈内动脉狭窄导致的脑内盗血综合征的疗效和安全性。方法应用血管内支架植人术对16例经内科药物治疗无效的脑动脉重度狭窄引起的盗血综合征患者进行治疗，其中11例为锁骨下动脉盗血，5例为颈内动脉盗血；术后随访并采用Malek评分法对所有患者进行评价。结果支架植人后即行DSA检查，影像学见7例狭窄血管基本恢复正常，6例狭窄〈10％，3例残余狭窄〈20％，平均随访10个月，其中发生前循环梗死2例，后循环梗死3例。结论支架成形术可有效治疗脑动脉重度狭窄引起的脑内盗血综合征。     

儿童缺血型烟雾病的临床和影像分析

【摘要】
目的 分析儿童缺血型烟雾病患者的临床和影像特点,探讨脑梗死的相关因素。
方法 回顾性分析2011年6月～2012年6月收治的27例儿童缺血型烟雾病患者的临床及影像学资料。
大脑半球分为无脑梗死半球32侧和脑梗死半球22侧,将患者性别、年龄、造影特点（包括54侧半球
的Suzuki分期、大脑后动脉分期,眼动脉、颞浅动脉、脑膜动脉、枕动脉、上颌动脉和后循环向前循
环代偿）与脑梗死的发生进行统计分析。
结果 27例患者的临床资料显示性别和年龄与脑梗死的发生差异不具有显著性（性别χ2=2.095,
P =0.148;年龄χ2=4.286,P =0.453）。根据半球计算：皮质脑梗死17侧（17/22）,额叶梗死14侧
（14/22）,皮质下脑梗死9侧（9/22）。无梗死侧半球和梗死侧半球Suzuki分期以及大脑后动脉分期
的差异具有显著性（Suzuki分期Z=－3.054,P =0.002;大脑后动脉分期Z=－4.557,P <0.001）。眼动脉、
颞浅动脉、脑膜动脉、枕动脉、上颌动脉和后循环向前循环代偿与脑梗死的发生差异不具有显著性。
结论 本研究显示儿童缺血型烟雾病皮质脑梗死较皮质下脑梗死常见。在皮质脑梗死中,额叶脑
梗死最常见。脑梗死的发生与Suzuki分期和大脑后动脉受累程度有关。     

Multiple encephalogaleoperiosteal synangiosis for bilateral carotid artery stenosis in a 13-year-old girl: a case report

Introduction

Encephalogaleoperiosteal synangiosis (EGS) has been widely used to treat children with moyamoya disease (MMD). We present the first case of successful multiple EGS in a patient with brain ischemic disease who presented with different cerebrovascular findings from MMD.

Methods

A 13-year-old girl had an increased frequency of transient ischemic attacks that affected her right extremities. Digital subtraction angiography showed tapering of the internal carotid artery (ICA). The anterior cerebral artery (ACA) and middle cerebral artery (MCA) were visible on vertebral angiogram, but not on carotid angiogram. The intact circle of Willis and lack of hypervascularity of the lenticulostriate arteries were observed. Decreased regional cerebral blood flow (CBF) in the bilateral ACA and MCA territories quantified by ¹²³I-N-isopropyl-p-iodoamphetamine-single photon emission computed tomography indicated the need for extracranial-intracranial bypass surgery. Multiple EGS procedures were performed instead of direct anastomosis, which is the standard procedure for intracranial ICA stenosis, because the space for the craniotomy was limited by transdural anastomosis.

Results

Despite the fact that the diagnosis of MMD was questionable, the hemispheres were well vascularized, and the neurology and CBF improved postoperatively.

Conclusion

The preserved circle of Willis and lack of moyamoya vessels were inconsistent with the features of MMD. However, childhood onset, bilateralness, chronic intracranial ICA stenosis, and transdural anastomosis indicated the same underling pathogenicity as MMD. It is hypothesized that ICA stenosis occurred immediately proximal to the posterior communicating artery in this case. This would have produced the atypical finding of the remaining circle of Willis without growth of the basal moyamoya vessels.

     

烟雾病的MSCTA分析研究

目的进一步提高对烟雾病的理解和认识。方法收集本院30例证实为烟雾病患者MSCTA影片(包括原始轴位影片、VR、MIP、MPR后处理影片),结合脑血管造影结果及文献资料进行分析。结果 30例患者中均有不同程度颈内动脉系统狭窄及闭塞,单侧者10例,双侧者20例;大脑后动脉狭窄或闭塞者9例;大脑后动脉及颈外动脉系统侧支循环形成20例;合并动脉瘤者11例。所有病例均可见异常血管网,部分患者行DSA检查,结果基本一致。结论烟雾病为脑底动脉进行性狭窄、闭塞及广泛侧支循环形成的疾病,它不仅累及颈内动脉系统,还累及椎-基底动脉系统和颈外动脉系统。MSCTA可代替DSA作为烟雾病的首选检查方法。     

DSA在烟雾病诊断中的临床应用研究

目的探讨烟雾病DSA检查的影像学表现及临床意义。方法回顾性分析26例确诊烟雾病患者临床及DSA影像学资料。结果26例患者DSA影像学特征如下:脑底异常烟雾状血管网形成;受累动脉狭窄或闭塞;丰富侧支循环形成。其中单/双侧大脑前、中及后动脉均存在不同程度的狭窄或闭塞性病变者24例(92.3%);双侧颈内动脉床突上段狭窄或闭塞者18例(69.2%),单侧者7例(26.9%)。结论烟雾病患者有显著的DSA影像学特征,DSA是诊断烟雾病的主要手段,临床上对疑似病例应早行DSA检查明确诊断。     

An institutional experience of 26 patients with Moyamoya disease: A study from Northwest India

Aim:

Moyamoya disease (MMD) is a slowly progressive bilateral stenocclusive process of the distal internal carotid and proximal portions of the anterior and middle cerebral arteries and the formation of an abnormal vascular network at the base of the brain. The purpose of this retrospective study was to identify clinical features, salient features, radiological features and yield of diagnostic cerebral angiography in MMD.

Materials and Methods:

We analyzed the records of 26 patients with MMD evaluated and treated at our institute from August 2010 until March 2013. Diagnosis of MMD was made on the basis of features of angiographic findings. Cerebral angiography showed typically fine network of vessels at the base of the brain with puff of smoke appearance suggestive of MMD. CT angiography (CTA) was done in 25 (96.15%) patients where as Digital substraction angiography (DSA) was done in 18 (69.23%) patients.

Results:

Out of the 26 patients 13 were in the pediatric age group and 13 were adults. At presentation 14 patients had infarcts and 10 patients had hemorrhages. Among the hemorrhagic group 20% had isolated intracerebral hemorrhage (ICH), 50% patients had ICH with intraventricular extension (IVE) and 30% patients had primary intraventricular hemorrhage (PIVH). 50 % of the patients had involvement of the posterior circulation.

Conclusion:

Posterior circulation involvement is frequent in MMD. Though parenchymal bleed with/without intraventricular extension is the usual presentation of hemorrhagic MMD, isolated intraventricular hemorrhage could also be the mode of presentation.     

Magnetic resonance angiography in moyamoya disease

Spontaneous occlusion of the circle of Willis, i.e., moyamoya disease (MMD), is a clinical disease entity angiographically characterized by progressive and bilateral stenosis of the carotid bifurcation, with a hazy collateral network at the base or convexity of the brain. Although the importance of computed tomography (CT) and conventional magnetic resonance (MR) imaging in diagnosing MMD has already been determined, conventional arteriography is still regarded as necessary for definitive diagnosis. Magnetic resonance angiography (MR-A) is a very recent vascular imaging technique which allows noninvasive and direct imaging of vessels without the use of contrast medium. We present four pediatric cases of MMD, evaluated by conventional angiography, CT, MR imaging, and MR-A. Our data demonstrate thatMR-A is successful both in allowing detection of occlusive disease of the basal portion of the internal carotid artery and large branch basal cerebral vessels and demonstrating the collateral vessels at the base of the brain. As a noninvasive procedure, MR-A promises to become a valuable alternative to classical angiography in the diagnosis of MMD.     

Bihemisphere Ischemia Due to a Unilateral Lesion: A Case Report

Bihemispheric ischemic strokes secondary to unilateral vessel disease are uncommon. We present the case of a 70-year-old man with multiple acute/subacute bilateral infarcts. The patient was found to have stenosis of the left internal carotid artery secondary to herpes zoster ophthalmicus vasculopathy, with involvement of the left proximal middle and anterior cerebral arteries. Angiographic studies also revealed A1 segment aplasia of the right anterior cerebral artery (ACA), thus indicating dependence on the left-sided circulation for perfusion of the bilateral ACA vascular territory. This case illustrates how A1 segment aplasia, an anatomic variant of the circle of Willis detected by angiographic studies, can contribute to bilateral infarction in the ACA vascular territory.