Cognitive impairment in asymptomatic carotid artery stenosis is associated with abnormal segments in the Circle of Willis.

OBJECTIVE: Our group has previously demonstrated that patients with asymptomatic carotid artery stenosis (ACAS) demonstrate cognitive impairment. One proposed mechanism for cognitive impairment in patients with ACAS is cerebral hypoperfusion due to flow-restriction. We tested whether the combination of a high-grade carotid stenosis and inadequate cross-collateralization in the Circle of Willis (CoW) resulted in worsened cognitive impairment. METHODS: Twenty-four patients with high-grade (≥70% diameter-reducing) ACAS underwent carotid duplex ultrasound, cognitive assessment, and 3D time-of-flight magnetic resonance angiography. The cognitive battery consisted of nine neuropsychological tests assessing four cognitive domains: learning and recall, attention and working memory, motor and processing speed, and executive function. Raw cognitive scores were converted into standardized T-scores. A structured interpretation of the magnetic resonance angiography images was performed with each segment of the CoW categorized as being either normal or abnormal. Abnormal segments of the CoW were defined as segments characterized as narrowed or occluded due to congenital aplasia or hypoplasia, or acquired atherosclerotic stenosis or occlusion. Linear regression was used to estimate the association between the number of abnormal segments in the CoW, and individual cognitive domain scores. Significance was set to P < .05. RESULTS: The mean age of the patients was 66.1 ± 9.6 years, and 79.2% (n = 19) were male. A significant negative association was found between the number of abnormal segments in the CoW and cognitive scores in the learning and recall (ß = -6.5; P = .01), and attention and working memory (ß = -7.0; P = .02) domains. There was a trend suggesting a negative association in the motor and processing speed (ß = -2.4; P = .35) and executive function (ß = -4.5; P = .06) domains that did not reach significance. CONCLUSIONS: In patients with high-grade ACAS, the concomitant presence of increasing occlusive disease in the CoW correlates with worse cognitive function. This association was significant in the learning and recall and attention and working memory domains. Although motor and processing speed and executive function also declined numerically with increasing abnormal segments in the CoW, the relationship was not significant. Since flow restriction at a carotid stenosis compounded by inadequate collateral compensation across a diseased CoW worsens cerebral perfusion, our findings support the hypothesis that cerebral hypoperfusion underlies the observed cognitive impairment in patients with ACAS.

Morphology and morphometry of the anterior cerebral-anterior communicating artery complex.

PURPOSE: The variations of Cerebral arterial circle (Circle of Willis) are not rare. The study is done to assess the pattern of the component vessels of its anterior part with regard to Right- Left variations and compare with other studies. MATERIAL AND METHOD: 56 formalin fixed cadavers were analyzed. The diameter, length and variations of the following vessels were observed: Anterior Cerebral Artery (proximal A1 segment to distal A2 segment) and anterior communicating artery. Statistical analysis was done by Statistica 14.0.1 software. RESULT: 53.57% had normal anterior cerebral artery- anterior communicating artery complex. Length and diameter were slightly more on left side. Unilateral hypoplasia, Azygos and Tripple Anterior Cerebral Artery was present in 21.43%, 3.57 and 1.78% respectively. Aplastic, double and fenestration type anterior communicating artery were present in 5.3%, 8.92% and 1.78% respectively. CONCLUSION: Anatomical knowledge of variations should be updated regularly for neurosurgeons, radiologists and clinicians to avoid unexpected consequences.

Association between circle of Willis and ischemic stroke: a systematic review and meta-analysis.

BACKGROUND: Circle of Willis is the main structure that provides constant and regular blood flow to the brain, protects the brain from ischemia. Stroke has remained the second leading cause of death globally in the last fifteen years. It is the fifth leading cause of death in the United States. It is also the leading cause of serious adult disability. Interlinked problems related to ischemic stroke are become increasing nowadays. Strong evidence is needed about the pooled measure of association between the circle of Willis (COW) and ischemic stroke. Therefore, this systematic review and meta-analysis were intended to provide compressive and up to date evidence on the association between the variations of COW and ischemic stroke using the available studies. METHODS: PubMed, Google Scholar, Science Direct, and Cochrane Library databases were systematically searched. All essential data were extracted using a standardized data extraction template. The heterogeneity across studies was assessed by using the Cochrane Q test statistic, I2 test statistic, and P-values. A fixed-effect model was used to estimate the pooled effect of the measure association between COW and ischemic stroke. RESULTS: In this meta-analysis, 2,718 participants were involved. The pooled measure of association between COW and ischemic stroke was 1.38 (95% CI 0.87, 2.19). Therefore, this indicated that the presence of any variation in COW was 1.38 times more likely to develop ischemic stroke as compared to the patent COW. The presence of hypoplasia/incompleteness in a posterior communicating artery (PcomA) [Pooled OR: 1.34 (95% CI 0.80, 2.25)] and anterior communicating artery (AcomA) [Pooled OR: 1.32 (95% CI 0.81, 2.19)] were a contributing factor for the development of ischemic stroke. Hypertension was the most common comorbid condition, followed by diabetes mellitus, smoking, coronary artery disease, and hyperlipidemia. CONCLUSIONS: There was a non-significant positive association between COW variation and ischemic stroke in this meta-analysis.

Morphologic variants of the Cerebral Arterial Circle on computed tomographic angiography (CTA): a large retrospective study.

PURPOSE: To confirm and illustrate the great variability of morphology of the Cerebral Arterial Circle (CAC)-also commonly called "Circle of Willis"-in current clinical Computed Tomography Angiography (CTA) practice. METHODS: Computed Tomographic Angiographic 3D Volume Rendering reconstructions of the CAC performed in a series of 511 patients were retrospectively reviewed and classified following their anatomic configuration. RESULTS: An amount of 27 CAC configurations were listed. Complete and "nearly complete" (1 missing segment) CACs were found in 115 (22.58%) and 157 (28.6%) patients. The posterior arch was much more frequently incomplete (374 patients = 73.18%) than the anterior arch (96 patients = 18.4%). The main cause was a high prevalence of missing posterior communicating arteries (PCoAs). The left or right PCoA were unilaterally lacking in 156 patients (30.53%) and both PCoAs were lacking in 179 patients (35.02%). Cases with 2 and 3 missing segments were observed in 184 (36%) and 44 patients (8.6%). Precarious situations were also identified including 7 cases (1.4%) of complete isolation of the middle cerebral artery (MCA), 11 cases (2.15%) of absence of interhemispheric supply, 205 cases (40.1%) of full separation of the carotid and vertebra-basilar (VB) territories and 44 cases (8.6%) of full separation of the three main arterial axes (both ICAs and VB). The prevalence of Fetal Posterior Cerebral Arteries (FPCA) variants was also reported. A "Full" FPCA was found unilaterally in 48 (9.4%) and bilaterally in 13 (2.54%) of patients. Apart from agenesis and hypoplasia reported in our study, various other variations of the anterior complex of the CAC (ACoA and A2 segments of the ACA) were also noted. CONCLUSION: CTA with 3D Volume Rendering may powerfully assess the numerous variations of the CAC. This assessment is of prime importance for the evaluation of patients presenting with risk factors or in whom neurosurgery, cardiac surgery, interventional radiology or carotid endarterectomy (CEA) are being considered.

A unique finding of the basilar artery.

Variants of the posterior intracranial circulation are important for surgeon, interventionalists and radiologists. Herein, a unique configuration of the basilar artery is reported. A 54-year-old man with a history of COPD, hypothyroidism, smoking, and hyperlipidemia presented to an outside institution with nausea, confusion, altered mental status, and ataxia. The patient was evaluated for stroke. Imaging revealed rotation of the basilar apex of 180 degrees, fetal configuration of the posterior communicating artery, right posterior cerebral artery filling from the left vertebral artery, and duplication of the left and right superior cerebellar arteries. The patient continued to deteriorate neurologically and MRI revealed multifocal and symmetric signal abnormalities in the brain stem, thalami, basal ganglia, and hippocampi. The differential diagnosis included acute disseminated myeloencephalitis. Despite plasma exchange and steroid therapy, the patient died a few days later. This case report demonstrates a rare variation of the basilar apex.

Correlation of internal carotid artery diameter and carotid flow with asymmetry of the circle of Willis.

BACKGROUND: The purpose of this study was to clarify the effect of asymmetric COW variants on carotid flow changes, and proposed an easy estimate of the representative carotid flow volume for accurate numerical simulation. METHODS: A total of 210 healthy adults receiving magnetic resonance angiography and carotid duplex sonography were included. Three anterior cerebral artery asymmetry (AA) groups were defined based on the diameter ratio difference (DRD) of bilateral A1 segments: AA1 group, one-side A1 aplasia; AA2, A1 DRD ≥ 50%; AA3, A1 DRD between 10 and 50%. Similarly, 3 posterior communicating artery (PcomA) asymmetry (PA) groups were defined: PA1 group, one fetal-origin posterior cerebral artery and absent contralateral PcomA; PA2, PcomA DRD ≥ 50%; PA3, PcomA DRD between 10 and 50%. RESULTS: With A1 asymmetry, the ICA diameter of the dominant A1 is significantly greater than the contralateral side. Significant differences of bilateral ICA flow were present in the AA1 and AA2 groups (mean flow difference 42.9 and 30.7%, respectively). Significant bilateral ICA diameter and flow differences were only found in the PA1 group. Linear regression analysis of ICA diameter and flow found a moderately positive correlation between ICA diameter and flow in all AA groups, with a 1 mm increment in vessel diameter corresponding to a 62.6 ml increment of flow volume. The product of bilateral ICA diameter and flow volume difference (ICA-PDF) could be a potential discriminator with a cutoff of 4.31 to predict A1 asymmetry ≥50% with a sensitivity of 0.81 and specificity of 0.76. CONCLUSIONS: The study verifies that A1 asymmetry causes unequal bilateral carotid inflow, and consequently different bilateral ICA diameters. Adjustment of the inflow boundary conditions according to the COW variants would be necessary to improve the accuracy of numerical simulation.

Atherosclerotic ICA stenosis coinciding with ICA asymmetry associated with Circle of Willis variations can mimic near-occlusion.

Differentiating carotid near-occlusion (tight atherosclerotic stenosis causing distal artery size reduction) from conventional stenosis is the first step when grading carotid stenoses with NASCET method. The internal carotid artery (ICA) can be asymmetrically associated with Circle of Willis variations. When such ICA asymmetry coincides with stenosis, it may mimic near-occlusion. We studied ICA anatomical variant prevalence in 4042 consecutive CTA exams from all indications, 53 excluded due to carotid occlusion, 814 with any ≥ 50% steno-occlusive disease intra- or extracranially, 3228 without. Of the 3989 included cases, 568 (14%) had ICA asymmetry, of which 335 (59%) were from associated with Circle of Willis variations. Of 3228 patients without ≥ 50% stenosis or other steno-occlusive disease intra- and extracranially; 257 (8.0%) demonstrated ICA asymmetry associated with Circle of Willis variations, equally common among sexes and age unrelated and most frequently attributed to an ipsilateral A1 hypoplasia/aplasia, less often attributed to large contralateral posterior communicating artery. As ICA asymmetry associated with Circle of Willis variations are common, caution should be exercised diagnosing near-occlusion on asymmetry alone.

Cerebrovacular Reserve Predicts the Cerebral Hyperperfusion Syndrome After Carotid Endarterectomy.

BACKGROUND: Cerebral hyperperfusion syndrome is a rare but potentially severe complication of carotid artery revascularisation that develops under conditions of resistant postoperative hypertension and impaired cerebrovascular autoregulation. OBJECTIVE: Was to determine which preoperative and operative factors affect the development of cerebral hyperperfusion syndrome after carotid endarterectomy. METHODS: This prospective observational study enrolled 93 asymptomatic patients who underwent carotid endarterectomy. Cerebral hyperperfusion was registered in patients who had 100% postoperative increase in mean flow in middle cerebral artery registered by Transcranial Doppler ultrasound. Cerebral hyperperfusion syndrome was diagnosed in patients with cerebral hyperperfusion who postoperatively developed at least one of the symptoms. Pre-operative and operative risk factors for cerebral hyperperfusion syndrome were analysed by multivariate binary logistic regression. RESULTS: Out of 93 operated patients, cerebral hyperperfusion was registered in 23 and cerebral hyperperfusion syndrome in 18 patients. Risk factors for cerebral hyperperfusion syndrome were included in the binary logistic regression model. Incomplete Circle of Willis morphology on 3D TOF magnetic resonance image (p = 0.002), Breath holding index below the 0.69 cut-off (p = 0.006), positive criteria for insufficient collateral flow through circle of Willis registered by TCD (p = 0.03), and poorly controlled hypertension (p = 0.023) showed statistically significant independent predictive value for cerebral hyperperfusion syndrome. The model was statistically significant (p = 0.012) and correctly classified 90.3 % of patients. CONCLUSIONS: Incomplete circle of Willis and insufficient collateral flow, low cerebrovascular reserve, and poorly regulated hypertension are significant predictors of post- carotid endarterectomy hyperperfusion development.

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.

Association Between Incomplete Circle of Willis and Carotid Vulnerable Atherosclerotic Plaques.

Objective- Carotid high-risk plaque, characterized by intraplaque hemorrhage, fibrous cap rupture, and large lipid-rich necrotic core, is associated with cerebrovascular events. This study sought to investigate the relationship between high-risk carotid plaque and an incomplete circle of Willis (COW). Approach and Results- Patients were recruited from a multicenter study, Chinese Atherosclerosis Risk Evaluation (CARE-II) and underwent 3-dimensional time-of-flight magnetic resonance angiography for intracranial arteries and 2-dimensional multicontrast magnetic resonance vessel wall imaging for carotid arteries on a 3.0T magnetic resonance scanner. The integrity of the COW in anterior and posterior portions was evaluated. Characteristics of carotid plaques were assessed. Correlation between incomplete COW and carotid plaque features was determined. Of 482 eligible patients, patients with carotid intraplaque hemorrhage showed significantly higher prevalence of an incomplete anterior COW (52.7% versus 38.5%; P=0.022) compared with those without. An incomplete anterior COW was associated with intraplaque hemorrhage before (odds ratio, 1.781; 95% CI, 1.083-2.931; P=0.023) and after adjusted for clinical risk factors (odds ratio, 1.945; 95% CI, 1.139-3.321; P=0.015). The unilateral carotid artery stenosis showed no correlation with incomplete anterior COW and posterior COW (all P>0.025). No significant associations were found between other plaque features and any type of incomplete COW (all P>0.025). Conclusions- An incomplete COW is independently associated with intraplaque hemorrhage of carotid atherosclerotic plaques. Clinical Trial Registration- URL: http://www.clinicaltrials.gov . Unique identifier: NCT02017756.

Repaired coarctation of the aorta, persistent arterial hypertension and the selfish brain.

BACKGROUND: It has been estimated that 20-30% of repaired aortic coarctation (CoA) patients develop hypertension, with significant cardiovascular morbidity and mortality. Vertebral artery hypoplasia (VAH) with an incomplete posterior circle of Willis (ipCoW; VAH + ipCoW) is associated with increased cerebrovascular resistance before the onset of increased sympathetic nerve activity in borderline hypertensive humans, suggesting brainstem hypoperfusion may evoke hypertension to maintain cerebral blood flow: the "selfish brain" hypothesis. We now assess the "selfish brain" in hypertension post-CoA repair. METHODS: Time-of-flight cardiovascular magnetic resonance angiography from 127 repaired CoA patients (34 ± 14 years, 61% male, systolic blood pressure (SBP) 138 ± 19 mmHg, diastolic blood pressure (DBP) 76 ± 11 mmHg) was compared with 33 normotensive controls (42 ± 14 years, 48% male, SBP 124 ± 10 mmHg, DBP 76 ± 8 mmHg). VAH was defined as < 2 mm and ipCoW as hypoplasia of one or both posterior communicating arteries. RESULTS: VAH + ipCoW was more prevalent in repaired CoA than controls (odds ratio: 5.8 [1.6-20.8], p = 0.007), after controlling for age, sex and body mass index (BMI). VAH + ipCoW was an independent predictor of hypertension (odds ratio: 2.5 [1.2-5.2], p = 0.017), after controlling for age, gender and BMI. Repaired CoA subjects with VAH + ipCoW were more likely to have difficult to treat hypertension (odds ratio: 3.3 [1.01-10.7], p = 0.049). Neither age at time of CoA repair nor any specific repair type were significant predictors of VAH + ipCoW in univariate regression analysis. CONCLUSIONS: VAH + ipCoW predicts arterial hypertension and difficult to treat hypertension in repaired CoA. It is unrelated to age at time of repair or repair type. CoA appears to be a marker of wider congenital cerebrovascular problems. Understanding the "selfish brain" in post-CoA repair may help guide management. JOURNAL SUBJECT CODES: High Blood Pressure; Hypertension; Magnetic Resonance Imaging (MRI); Cardiovascular Surgery; Cerebrovascular Malformations.

Rare Bilateral Caudate Infarction in a Patient with a Common Circle of Willis Variant.

A 62-year-old male presented to hospital with acute aphasia. His past medical history was significant for a previous left middle cerebral artery stroke, from which he fully recovered, hypertension, dyslipidemia, coronary artery disease, one episode of atrial fibrillation postoperatively, and thalidomide exposure in utero. Although initially he was thought to be aphasic, on further examination, he demonstrated significant abulia. His level of consciousness was normal, and neurological examination was otherwise unremarkable. A CT angiogram of the head and neck was performed. The patient was not a candidate for acute therapy, as he had established stroke on imaging, and the time of onset was unclear.

Incomplete circle of Willis is associated with a higher incidence of neurologic events during carotid eversion endarterectomy without shunting.

OBJECTIVE: A complete circle of Willis (CoW) is considered an important collateral network to maintain blood flow during cross-clamping in carotid endarterectomy (CEA). The aim of this study was to evaluate the impact of an incomplete CoW with isolated middle cerebral artery (iMCA) on immediate neurologic events (INEs) after CEA. METHODS: We prospectively collected the clinical data and outcomes of 902 patients who underwent CEA under general anesthesia between 2013 and 2015. All patients had preoperative computed tomography angiography of the extracranial and intracranial cerebral circulation. Indications were asymptomatic (52%) and symptomatic (48%) carotid artery disease. Patients who had CEA with shunt (n = 35) and those with inadequate intracranial imaging to assess CoW were excluded (n = 322) only. Computed tomography angiography images were reviewed retrospectively and independently by two vascular radiologists who were blinded for treatment outcomes. Imaging assessment included the vertebral and carotid circulation and each segment of the CoW, which was classified as normal, hypoplastic (diameter < 0.8 mm) or absent. The ipsilateral MCA was considered isolated if there was an absence of the anterior and posterior communicating branches from the contralateral carotid or posterior circulations. INE was defined as any transient ischemic attack (TIA) and stroke diagnosed immediately after the procedure. RESULTS: Of the 545 included patients (331 males; mean age, 69 ± 8 years), 12 (2.2%) had a stroke in the postoperative period. There were 20 INEs (8 strokes and 12 TIAs). A complete CoW was rare; it was only detected in 19 patients (3.5%) and an iMCA was found in 34 patients (6.3%). When at least one collateral circulation was complete (in 330 patients), we observed only four INEs (1.2%). Of the 34 patients with an iMCA, 8 (24%) had INE (6 TIAs and 2 strokes). Overall, iMCA was an independent predictor of INEs (odds ratio, 11.12; 95% confidence interval, 3.57-35.87; P < .001). With logistic regression, the model included hypertension, smoking, diabetes, hyperlipidemia, carotid clamping time (minutes), contralateral significant internal carotid artery stenosis of greater than 90%, ipsilateral significant internal carotid artery stenosis of greater than 90%, preoperative symptoms in 6 months, and iMCA; above iMCA only symptomatic patients had significant risk (odds ratio, 3.34; 95% confidence interval, 1.19-9.73; P = .02), whereas all other parameters were not significant. CONCLUSIONS: An iMCA carries more than a 10-fold higher the risk of INEs after CEA with cross-clamping without shunt protection. In these patients, routine shunting is recommended to prevent INEs.

Importance of variants in cerebrovascular anatomy for potential retrograde embolization in cryptogenic stroke.

OBJECTIVES: To test the hypothesis that variants in cerebrovascular anatomy will affect the number of patients demonstrating a plausible retrograde embolization mechanism from plaques in the descending aorta (DAo). METHODS: Thirty-five patients (aged 63 ± 17 years) with cryptogenic stroke underwent 4D flow MRI for the assessment of aortic 3D blood flow and MR angiography for the evaluation of circle of Willis, posterior circulation, and aortic arch architecture. In patients with proven DAo plaque, retrograde embolization was considered a potential mechanism if retrograde flow extended from the DAo to a supra-aortic vessel supplying the cerebral infarct territory. RESULTS: Retrograde embolization with matching cerebral infarct territory was detected in six (17%) patients. Circle of Willis and aortic arch variant anatomy was found in 60% of patients, leading to reclassification of retrograde embolization risk as present in three (9%) additional patients, for a total 26% of cryptogenic stroke patients. CONCLUSION: 4D flow MRI demonstrated 26% concordance with infarct location on imaging with retrograde diastolic flow into the feeding vessels of the affected cerebral area, identifying a potential etiology for cryptogenic stroke. Our findings further demonstrate the importance of cerebrovascular anatomy when determining concordance of retrograde flow pathways with vascular stroke territory from DAo plaques. KEY POINTS: • Retrograde embolization from descending aortic plaques constitutes a plausible etiology in cryptogenic stroke. • Common variants of cerebrovascular anatomy are important in determining retrograde embolization mechanism. • Variant cerebrovascular anatomy can link retrograde flow pathways with vascular stroke territory.

Basilar Artery Diameter Is Inversely Associated with Fetal Type Circle of Willis.

BACKGROUND: The relationship between basilar artery (BA) diameter and Circle of Willis (CoW) configuration has been scarcely investigated. We aimed to assess this association in community-dwelling older adults. METHODS: MRAs of 346 individuals were reviewed. Using generalized linear models adjusted for demographics and cardiovascular risk factors, we assessed the relationship between BA diameter and fetal and non-fetal types CoW, as well as the impact of this relationship on BA ectasia prevalence. RESULTS: In the total population, the mean BA diameter was 3.13 ± 0.68 mm and 7 subjects (2%) had ectasia (BA diameter >4.5 mm). In 248 subjects with non-fetal types CoW, the mean BA diameter was 3.32 ± 0.62 mm, and 2.8% had ectasia. In 98 subjects with fetal type CoW, the mean BA diameter was 2.66 ± 0.58 mm, and no individual had ectasia. The BA diameter was smaller in subjects with fetal type CoW than in those with non-fetal types (ß 0.65; 95% CI 0.51-0.79; p < 0.001). Individuals with fetal type CoW have an 18% reduction in BA diameter compared to those with non-fetal types, independently of demographics and cardiovascular risk factors. CONCLUSION: This study shows an inverse relationship between the BA diameter and the presence of fetal type CoW.

Circle of Willis variations and artery diameter measurements in the Turkish population.

BACKGROUND: The aim of this study was to evaluate the frequency of circle of Willis (COW) variations and the measurements of the COW vessel diameters in the Turkish population. MATERIALS AND METHODS: The patient population for this single-centre, retrospective study was formed of patients who had brain magnetic resonance angiography (MRA). A total of 384 subjects were included in the study. The three-dimensional time-of-flight (TOF) technique was used for MRA imaging to evaluate the anatomy of the COW. Variations in the COW were recorded. The diameters of the critical arteries of the COW were measured. RESULTS: Adult configuration of the COW was determined in 326 (85%) cases, foetal configuration in 50 (13%) cases and transitional configuration in 8 (2%) cases. The COW was normal in 328 (85.4%). In the remaining 56 (14.6%) cases, agenesis was determined in 62 vessels. The mean calibrations of the vessels were measured as 2.85 mm in the basilar artery, 4.24 mm in the right internal carotid artery (ICA), 4.32 mm in the left ICA, 1.58 mm in the right A1, 1.64 mm in the left A1, 2.13 mm in the right M1, 2.10 mm in the left M1, 1.80 mm in the right P1, 1.88 mm in the left P1, 1.12 mm in the right posterior communicating artery, and 1.12 mm in the left posterior communicating artery. CONCLUSIONS: Circle of Willis variations may show geographic and ethnic differences. Knowledge of the frequency and types of variation in the population is important for neurosurgeons and in radiological interventional procedures as a guide for entry and in respect of collateral which could develop later.

Completeness of the circle of Willis and risk of ischemic stroke in patients without cerebrovascular disease.

INTRODUCTION: We investigated circle of Willis (CoW) completeness in relation to the risk of future ischemic stroke in patients without prior cerebrovascular disease. METHODS: We included 976 patients with atherosclerotic disease, but no previous TIA/stroke, from the Second Manifestations of ARTerial disease (SMART) study. All patients underwent MR angiography of the CoW. Cox regression was used to determine whether anterior CoW completeness (anterior communicating artery or A1 segments) and posterior CoW completeness (posterior communicating arteries or P1 segments) were related to future stroke, and whether CoW completeness influenced the relation between internal carotid artery (ICA) stenosis/occlusion and future stroke. RESULTS: Thirty patients (3.1 %) had ischemic stroke after 9.2 ± 3.0 years of follow-up. Twenty-four patients (80 %) had anterior circulation stroke. An incomplete anterior CoW was related to future anterior circulation stroke (HR 2.8 (95 % CI 1.3-6.3); p = 0.01), whereas a one-sided and two-sided incomplete posterior CoW were not (HR 2.2 (95 % CI 0.7-7.1; p = 0.19) and 1.9 (95 % CI 0.6-5.9; p = 0.29), respectively). In stratified analyses, patients with an incomplete anterior CoW had the highest risk of future anterior circulation stroke when they also had a one-sided (HR 7.0 (95 % CI 1.3-38.2; p = 0.02)) or two-sided incomplete posterior CoW (HR 5.4 (95 % CI 1.0-27.8; p = 0.04). CoW completeness did not change the relation between asymptomatic ICA stenosis/occlusion and future ischemic stroke (p = 0.68). CONCLUSIONS: An incomplete anterior CoW combined with an incomplete posterior CoW is related to future anterior circulation stroke. CoW completeness has no large effect on the relation between asymptomatic ICA stenosis/occlusion and future stroke.

PHACE(S) Syndrome With Absent Intracranial Internal Carotid Artery and Anomalous Circle of Willis.

The authors present a case of PHACE(S) (posterior fossa malformations, hemangioma, arterial anomalies, cardiac defects, eye anomalies, and sternal cleft or supraumbilical raphe) syndrome with a right-sided segmental infantile hemangioma, and describe in detail, the associated absent ipsilateral intracranial internal carotid artery and anomalous Circle of Willis. Propranolol therapy led to accelerated, complete involution. Nadolol may reduce the theoretical risk of treating PHACE(S) patients with ß-blockers.

A1 segment hypoplasia accompanied by AcomA aneurysms assessed with magnetic resonance angiography.

PURPOSE: This study is to evaluate the use of three-dimensional time-of-flight magnetic resonance angiography (3D-TOF MRA) at 3.0 T for the detection and classification of A1 segment hypoplasia based on a large case series and investigates the potential links with AcomA aneurysm occurrence. METHODS: Between January 2009 and June 2012, 8,013 patients underwent MRA examination at 3.0 T in our hospital. MRA was performed using 3D-TOF with volume rendering technique. The presence and location of A1 segment hypoplasia and AcomA aneurysm was reviewed. RESULTS: Among the 8,013 patients examined, 138 patients were identified with AcomA aneurysms. 425 patients were defined with A1 segment hypoplasia, among whom 303 right-sided A1 hypoplasia and 122 left-sided A1 hypoplasia. 60 of these 425 patients were confirmed with AcomA aneurysms, among them were 49 right-sided A1 hypoplasia. The prevalence of AcomA aneurysm with A1 segment hypoplasia was 14.1 %, which was much higher compared with that (1.0 %) of AcomA aneurysm without A1 segment hypoplasia (P < 0.001). CONCLUSIONS: The incidence of right-sided A1 segment hypoplasia either accompanied with AcomA aneurysm or not was much greater than that of left-sided. Intracranial AcomA aneurysm development appeared to be associated with A1 segment hypoplasia.

Circle of Willis abnormalities in children with neurofibromatosis type 1.

BACKGROUND AND PURPOSE: The aim of the study was to assess anatomical variants and abnormalities in cerebral arteries on magnetic resonance angiography in 67 children with neurofibromatosis type 1 (NF1). MATERIALS AND METHODS: The study included 67 children aged 9 months to 18 years (mean 6.6 years). Control group comprised 90 children aged 2-18 years (mean: 11.8 years). All patients were examined at 1.5T scanner. RESULTS: We found cerebral arteriopathy (moyamoya disease) in one child (1.5%) in the study group. No aneurysms were found. Twenty-nine NF1 children (43.3%) had arterial anatomical variants. In 13 of them, more than one variant was diagnosed (44.8% of group with variants, 19.4% of study group). In control group, 19 children (21.1%) had variants, including four children with more than one variant (21% of group with variants, 4.4% of control group). Arterial variants were more common in NF1 patients compared with control group (p=0.026, binomial test for two proportions). Percentage of multiple variants was higher in study group than in control group, but this difference was not significant. Variants were more frequent on left side than on the right one (significant difference in control group; p=0.022, McNemara test). In study group, the number of left-sided anomalies (25) was similar to that of right-sided ones (22). There was no correlation between gender and variants, unidentified bright objects and variants or between optic gliomas and variants. CONCLUSIONS: Occurrence of arterial variants in NF1 patients was twofold higher than in control group. Multiple variants were more frequent in the study group although the difference did not reach statistical significance. Features of cerebral arteriopathy were found in one child with NF1.