Intracranial dural arteriovenous fistulas: association with cerebral venous thrombosis, baseline aggressiveness, and clinical outcomes. A retrospective multicenter study on 263 consecutive patients and literature review.

OBJECTIVE: The pathogenesis of intracranial dural arteriovenous fistulas (icDAVFs) is controversial. Cerebral vein thrombosis (CVT) and venous hypertension are recognized predisposing factors. This study aimed to evaluate the incidence of association between icDAVF and CVT and describe baseline aggressiveness and clinical outcomes for icDAVFs associated with CVT. The authors also performed a literature review of studies reporting icDAVF associated with CVT. METHODS: Two hundred sixty-three consecutive patients in two university hospitals with confirmed icDAVFs were included. A double-blind imaging review was performed to determine the presence or absence of CVT close or distant to the icDAVF. Location, type (using the Cognard classification), aggressiveness of the icDAVF, clinical presentation, treatment modality, and clinical and/or angiographic outcomes at 6 months were also collected. All prior brain imaging was analyzed to determine the natural history of onset of the icDAVF. RESULTS: Among the 263 included patients, 75 (28.5%) presented with a CVT concomitant to their icDAVF. For 18 (78.3%) of 23 patients with previous brain imaging available, CVT preceding the icDAVF was proven (6.8% of the overall population). Former/active smoking (OR 2.0, 95% CI 1.079-3.682, p = 0.022) and prothrombogenic status (active inflammation or cancer/coagulation trouble) were risk factors for CVT associated with icDAVF (OR 3.135, 95% CI 1.391-7.108, p = 0.003). One hundred eighty-seven patients (71.1%) had a baseline aggressive icDAVF, not linked to the presence of a CVT (p = 0.546). Of the overall population, 11 patients (4.2%) presented with spontaneous occlusion of their icDAVF at follow-up. Seven patients (2.7%) died during the follow-up period. Intracranial DAVF + CVT was not associated with a worse prognosis (modified Rankin Scale score at 3-6 months: 0 [interquartile range {IQR} 0-1] for icDAVF + CVT vs 0 [IQR 0-0] for icDAVF alone; p = 0.055). CONCLUSIONS: This was one of the largest studies focused on the incidence of CVT associated with icDAVF. For 6.8% of the patients, a natural history of CVT leading to icDAVF was proven, corresponding to 78.3% of patients with previous imaging available. This work offers further insights into icDAVF pathophysiology, aiding in identifying high-risk CVT patients for long-term follow-up imaging. Annual imaging follow-up using noninvasive vascular imaging (CT or MR angiography) for a minimum of 3 years after the diagnosis of CVT should be considered in high-risk patients, i.e., smokers and those with prothrombogenic status.

Intracranial Dural Arteriovenous Fistulas.

Dural arteriovenous fistulas (DAVFs) are abnormal communications between meningeal arteries and dural venous sinuses and/or cortical veins. Although many fistulas are benign and do not require treatment, some may carry a significant risk of bleeding or cause symptoms and warrant treatment. This review provides a review of various aspects of intracranial DAVFs including epidemiology, pathophysiology, clinical presentation, imaging characteristics, classification, natural history, and management options. By exploring these topics, we aim to enhance understanding of this condition and facilitate patient care.

Risk of Early Versus Later Rebleeding From Dural Arteriovenous Fistulas With Cortical Venous Drainage.

BACKGROUND: Cranial dural arteriovenous fistulas with cortical venous drainage are rare lesions that can present with hemorrhage. A high rate of rebleeding in the early period following hemorrhage has been reported, but published long-term rates are much lower. No study has examined how risk of rebleeding changes over time. Our objective was to quantify the relative incidence of rebleeding in the early and later periods following hemorrhage. METHODS: Patients with dural arteriovenous fistula and cortical venous drainage presenting with hemorrhage were identified from the multinational CONDOR (Consortium for Dural Fistula Outcomes Research) database. Natural history follow-up was defined as time from hemorrhage to first treatment, rebleed, or last follow-up. Rebleeding in the first 2 weeks and first year were compared using incidence rate ratio and difference. RESULTS: Of 1077 patients, 250 met the inclusion criteria and had 95 cumulative person-years natural history follow-up. The overall annualized rebleed rate was 7.3% (95% CI, 3.2-14.5). The incidence rate of rebleeding in the first 2 weeks was 0.0011 per person-day; an early rebleed risk of 1.6% in the first 14 days (95% CI, 0.3-5.1). For the remainder of the first year, the incidence rate was 0.00015 per person-day; a rebleed rate of 5.3% (CI, 1.7-12.4) over 1 year. The incidence rate ratio was 7.3 (95% CI, 1.4-37.7; P, 0.026). CONCLUSIONS: The risk of rebleeding of a dural arteriovenous fistula with cortical venous drainage presenting with hemorrhage is increased in the first 2 weeks justifying early treatment. However, the magnitude of this increase may be considerably lower than previously thought. Treatment within 5 days was associated with a low rate of rebleeding and appears an appropriate timeframe.

Dural arteriovenous fistulas in cerebral venous thrombosis: Data from the International Cerebral Venous Thrombosis Consortium: Data from the International Cerebral Venous Thrombosis Consortium.

BACKGROUND AND PURPOSE: To explore the prevalence, risk factors, time correlation, characteristics and clinical outcome of dural arteriovenous fistulas (dAVFs) in a cerebral venous thrombosis (CVT) population. METHODS: We included patients from the International CVT Consortium registries. Diagnosis of dAVF was confirmed centrally. We assessed the prevalence and risk factors for dAVF among consecutive CVT patients and investigated its impact on clinical outcome using logistic regression analysis. We defined poor outcome as modified Rankin Scale score 3-6 at last follow-up. RESULTS: dAVF was confirmed in 29/1218 (2.4%) consecutive CVT patients. The median (interquartile range [IQR]) follow-up time was 8 (5-23) months. Patients with dAVF were older (median [IQR] 53 [44-61] vs. 41 [29-53] years; p < 0.001), more frequently male (69% vs. 33%; p < 0.001), more often had chronic clinical CVT onset (>30 days: 39% vs. 7%; p < 0.001) and sigmoid sinus thrombosis (86% vs. 51%; p < 0.001), and less frequently had parenchymal lesions (31% vs. 55%; p = 0.013) at baseline imaging. Clinical outcome at last follow-up did not differ between patients with and without dAVF. Additionally, five patients were confirmed with dAVF from non-consecutive CVT cohorts. Among all patients with CVT and dAVF, 17/34 (50%) had multiple fistulas and 23/34 (68%) had cortical venous drainage. Of 34 patients with dAVF with 36 separate CVT events, 3/36 fistulas (8%) were diagnosed prior to, 20/36 (56%) simultaneously and 13/36 after (36%, median 115 [IQR 38-337] days) diagnosis of CVT. CONCLUSIONS: Dural arteriovenous fistulas occur in at least 2% of CVT patients and are associated with chronic CVT onset, older age and male sex. Most CVT-related dAVFs are detected simultaneously or subsequently to diagnosis of CVT.

Assessing the rate, natural history, and treatment trends of intracranial aneurysms in patients with intracranial dural arteriovenous fistulas: a Consortium for Dural Arteriovenous Fistula Outcomes Research (CONDOR) investigation.

OBJECTIVE: There is a reported elevated risk of cerebral aneurysms in patients with intracranial dural arteriovenous fistulas (dAVFs). However, the natural history, rate of spontaneous regression, and ideal treatment regimen are not well characterized. In this study, the authors aimed to describe the characteristics of patients with dAVFs and intracranial aneurysms and propose a classification system. METHODS: The Consortium for Dural Arteriovenous Fistula Outcomes Research (CONDOR) database from 12 centers was retrospectively reviewed. Analysis was performed to compare dAVF patients with (dAVF+ cohort) and without (dAVF-only cohort) concomitant aneurysm. Aneurysms were categorized based on location as a dAVF flow-related aneurysm (FRA) or a dAVF non-flow-related aneurysm (NFRA), with further classification as extra- or intradural. Patients with traumatic pseudoaneurysms or aneurysms with associated arteriovenous malformations were excluded from the analysis. Patient demographics, dAVF anatomical information, aneurysm information, and follow-up data were collected. RESULTS: Of the 1077 patients, 1043 were eligible for inclusion, comprising 978 (93.8%) and 65 (6.2%) in the dAVF-only and dAVF+ cohorts, respectively. There were 96 aneurysms in the dAVF+ cohort; 10 patients (1%) harbored 12 FRAs, and 55 patients (5.3%) harbored 84 NFRAs. Dural AVF+ patients had higher rates of smoking (59.3% vs 35.2%, p < 0.001) and illicit drug use (5.8% vs 1.5%, p = 0.02). Sixteen dAVF+ patients (24.6%) presented with aneurysm rupture, which represented 16.7% of the total aneurysms. One patient (1.5%) had aneurysm rupture during follow-up. Patients with dAVF+ were more likely to have a dAVF located in nonconventional locations, less likely to have arterial supply to the dAVF from external carotid artery branches, and more likely to have supply from pial branches. Rates of cortical venous drainage and Borden type distributions were comparable between cohorts. A minority (12.5%) of aneurysms were FRAs. The majority of the aneurysms underwent treatment via either endovascular (36.5%) or microsurgical (15.6%) technique. A small proportion of aneurysms managed conservatively either with or without dAVF treatment spontaneously regressed (6.2%). CONCLUSIONS: Patients with dAVF have a similar risk of harboring a concomitant intracranial aneurysm unrelated to the dAVF (5.3%) compared with the general population (approximately 2%-5%) and a rare risk (0.9%) of harboring an FRA. Only 50% of FRAs are intradural. Dural AVF+ patients have differences in dAVF angioarchitecture. A subset of dAVF+ patients harbor FRAs that may regress after dAVF treatment.

Analysis of Risk Factors for Anterior Communicating Artery Aneurysm Rupture: A Single-Center Study.

BACKGROUND: Congenital hypoplasia or aplasia of the A1 segment of the anterior cerebral artery is associated with an increased incidence of berry aneurysms at the anterior communicating artery (ACoA) complex. We analyzed the factors contributing to ACoA aneurysm rupture. METHODS: We retrospectively analyzed the data of patients with ACoA aneurysms who had undergone cerebral angiography from July 2008 to January 2020. The risk factors for rupture were identified using univariate and multivariate logistic regression models. We used the imaging data of patients without intracranial aneurysms as the control population. RESULTS: We confirmed 253 aneurysms in 253 patients, including 137 men (54.2%) and 116 women (45.8%), with a mean age of 54.6 ± 12.7 years. Of the 253 aneurysms, 218 (86.2%) were ruptured and 35 (13.8%) were unruptured, with a mean diameter of 4.56 ± 1.96 mm and 3.24 ± 1.79 mm, respectively. Of the 253 aneurysms, 176 (69.6%) were <5 mm in diameter, 146 (83%) of which were ruptured. Of the 253 patients, 141 (55.7%) exhibited A1 segment hypoplasia or aplasia: 106 (41.9%) on the right side of the circle of Willis and 35 (13.8%) on the left. Multivariate logistic regression analysis identified the aneurysm diameter (odds ratio, 4.11; 95% confidence interval, 1.601-16.07; P = 0.003) and age <65 years (odds ratio, 0.17; 95% confidence interval, 0.062-0.48; P < 0.001) as independent predictors of rupture. CONCLUSIONS: ACoA aneurysms are small (<5 mm) and have a high risk of rupture. A1 segment hypoplasia or aplasia is a risk factor for ACoA aneurysm formation; however, it was uncorrelated with aneurysm diameter or rupture risk. The strongest independent risk factors for rupture were age <65 years and aneurysm diameter.

Dural Arteriovenous Fistula Formation Secondary to Cerebral Venous Thrombosis: Longitudinal Magnetic Resonance Imaging Assessment Using 4D-Combo-MR-Venography.

BACKGROUND AND PURPOSE: Dural arteriovenous fistulae (DAVFs) can develop secondary to cerebral venous thrombosis (CVT). The incidence of DAVF has not yet been investigated prospectively. METHODS: Between July 2012 and January 2018, combined static and dynamic 4D MR venography (4D-combo-MRV) was performed in 24 consecutive patients at diagnosis of CVT and after 6 months. 3 Tesla magnetic resonance imaging with time of flight and contrast-enhanced magnetization-prepared rapid acquisition with gradient echo were performed at baseline to evaluate the extent of thrombosis and affected vessel segments. Baseline and follow-up 4D-combo-MRV were assessed for signs of DAVF. Interrater reliability of DAVF detection and the extent of recanalization were analyzed with kappa statistics. RESULTS: DAVFs were detected in 4/30 CVT patients (13.3%, 95% confidence interval [CI] 3.3-26.7). Two of 24 patients (8.3%, 95% CI: 0-20.8) had coincidental DAVF with CVT on admission. At follow-up, de novo formation of DAVF following CVT was seen in 2/24 patients (8.3%, 95% CI: 0-20.8). Both de novo DAVFs were low grade and benign fistulae (Cognard type 1, 2a), which had developed at previously thrombosed segments. Endovascular treatment was required in two high degree lesions (Cognard 2a + b) detected at baseline and in one de novo DAVF (Cognard 1) because of debilitating headache and tinnitus. Thrombus load, vessel recanalization, and frequency of cerebral lesions (hemorrhage, ischemia) were not associated with DAVF occurrence. CONCLUSION: This exploratory study showed that de novo DAVF formation occurs more frequently than previously described. Although de novo DAVFs were benign, 75% of all detected DAVFs required endovascular treatment. Therefore, screening for DAVF by dynamic MRV, such as 4D-combo-MRV, seems worthwhile in CVT patients.

Incidence of hemorrhagic cerebrovascular disease due to vascular malformations during the COVID-19 national quarantine in Italy.

BACKGROUND: hemorrhagic cerebrovascular disease due to vascular malformations represents an emergency for neurosurgery and neuro-interventional departments. During the COVID-19 pandemic, a dramatic reduction in the number of hospitalizations for acute myocardial infarction or stroke and a larger time interval from symptom onset to first medical contact have been reported. This study aims to verify the hypothesis that there would also have been a reduction of admissions for hemorrhagic cerebrovascular disease during the Italian lockdown. MATERIAL AND METHOD: s A multicenter, observational survey was conducted to collect data on hospital admissions for hemorrhagic cerebrovascular disease due to vascular malformations throughout two-months (March 15th to May 15th); the years 2020 (COVID-19 Italian lockdown), 2019 and 2018 were compared. Cases were identified by ICD-9 codes 430, 431, 432.1, 432.9, 747.81 of each hospital database. The statistical significance of the difference between the event rate of one year versus the others was evaluated using Poisson Means test, assuming a constant population. RESULTS: During the 2020 lockdown, the total number of admissions for hemorrhagic cerebrovascular disease was 92 compared with 116 in 2019 and 95 in 2018. This difference was not significant. GCS upon admission was 3-8 in 44 % of cases in 2020 (41 patients), 39.7 % in 2019 (46 patients) and 28 % in 2018 (27 patients). CONCLUSION: Reduction of admissions for hemorrhagic cerebrovascular disease due to vascular malformations during the COVID-19 lockdown was not confirmed. Nevertheless, some patients reached the emergency rooms only several days after symptoms onset, resulting in a worse clinical condition at admission.

Association of Anterior Cerebral Artery Variants and Cerebral Infarction in Patients with Balloon-Assisted Coil Embolization for Unruptured Internal Carotid Artery Aneurysms.

BACKGROUND: Balloon-assisted coiling (BAC) is an endovascular treatment that may be associated with increased complications and thromboembolic events compared with other coiling techniques. We compared clinical outcomes of endovascular treatment (simple coiling, stent-assisted coiling, and BAC) in patients with unruptured intracranial aneurysms at the internal carotid artery and assessed risk factors that could cause cerebral infarction in patients who underwent BAC. METHODS: We retrospectively reviewed the records of 528 patients with 544 aneurysms who underwent endovascular treatment for unruptured intracranial aneurysms between January 2013 and November 2019. Demographic features, clinical information, balloon inflation time, fetal posterior cerebral artery, anterior cerebral artery (ACA) variants, and angiographic results were analyzed to determine risk factors for cerebral infarction. RESULTS: There were no significant differences among the 3 groups in terms of general characteristics. In the BAC group, 14 of 39 patients showed a significantly higher incidence of cerebral infarction on diffusion magnetic resonance imaging compared with the stent-assisted coiling (37/238) and simple coiling (21/267) groups (P < 0.001). There was no significant difference between the ACA variants (normal vs. hypoplasia or aplasia) and cerebral infarction in the simple coiling and stent-assisted coiling groups, but the proportion of aplasia or hypoplasia in the BAC group was significantly higher (P = 0.001). CONCLUSIONS: There is a significant association between anatomic ACA variants and cerebral infarction occurrence after BAC. Identifying the variant of the anatomic ACA using digital subtraction angiography would help to predict cerebral infarction after BAC.

Dural Arteriovenous Fistulae After Cerebral Venous Thrombosis.

BACKGROUND AND PURPOSE: This analysis examined the frequency of dural arteriovenous fistulae (dAVF) after cerebral venous thrombosis (CVT) in patients included in a randomized controlled trial comparing dabigatran etexilate with dose-adjusted warfarin (RE-SPECT CVT [A Clinical Trial Comparing Efficacy and Safety of Dabigatran Etexilate With Warfarin in Patients With Cerebral Venous and Dural Sinus Thrombosis]), who had systematic follow-up magnetic resonance (MR) imaging. METHODS: RE-SPECT CVT was a Phase 3, prospective, randomized, parallel-group, open-label, multicenter, exploratory trial with blinded end point adjudication. We allocated patients with acute CVT to dabigatran 150 mg twice daily or dose-adjusted warfarin, for 24 weeks and obtained a standardized MR protocol including time-of-flight MR angiography, 3-dimensional phase-contrast venography, and 3-dimensional contrast-enhanced MR venography at the end of the treatment period. A blinded adjudication committee assessed the presence of dAVF in a predefined substudy of the trial. RESULTS: We analyzed development of dAVF in 112 of 120 randomized patients; 57 allocated to dabigatran and 55 to warfarin. For 3 (2.7%) of these 112 patients, quality of follow-up imaging was insufficient to evaluate dAVF. A dAVF (Borden I) was found in 1 patient (0.9%) allocated to warfarin; however, this dAVF was already present at baseline. The patient did not present with hemorrhage at baseline or during the trial and was asymptomatic at follow-up. CONCLUSIONS: Despite systematic imaging, we found no new dAVF 6 months after CVT. Routine follow-up cerebral MR angiography aiming to detect new dAVF 6 months after CVT has a very low yield. Registration: URL: https://www.clinicaltrials.gov. Unique identifier: NCT02913326.

Prevalence of MRI abnormalities in people with epilepsy in rural China.

OBJECTIVE: To assess the prevalence of brain MRI abnormalities in people with epilepsy in rural China and to compare it with that of individuals in the United Kingdom. METHODS: Brain MRI scans were obtained in people with epilepsy who participated in a rural community-based program in China between July 2010 and December 2012. Individual epileptogenic lesion types were reviewed and their associations with seizure control examined. The MRI findings were compared with 2 previous similar studies in the United Kingdom. RESULTS: Among the 597 individuals (58% male, median age 38 years) with MRI scans analyzed, 488 (82%) had active epilepsy. The MRI was abnormal in 389 individuals (65%), with potentially epileptogenic lesion in 224 (38%) and nonspecific abnormalities in 165 (28%), and 108 (18%) were potentially resectable. The potentially epileptogenic lesions were less frequently detected in children (<18 years old, 12 of 68, 18%) than in adults (212 of 529, 40%; p < 0.001). In people with potentially epileptogenic lesions, 67% (150 of 224) had failed ≥2 antiseizure medications. They had higher risk of uncontrolled epilepsy than those with normal MRI (risk ratio [RR] 1.25; p < 0.001) and those with nonspecific abnormality (RR 1.15; p = 0.002) after adjustment for age and sex. The diagnostic yield of MRI was similar to that reported in community- and hospital-based studies in the United Kingdom. CONCLUSIONS: More than one-third of people with chronic epilepsy in rural China have potentially epileptogenic lesions identifiable on brain MRI, with two-thirds fulfilling the definition of pharmacoresistance. These findings highlight the magnitude of the unmet needs for epilepsy surgery in China.

Brain capillary telangiectasias: from normal variants to disease.

BACKGROUND: Brain capillary telangiectasias (BCTs) are small, dilated capillary networks in the brain that are most commonly asymptomatic. Though rare, symptomatic cases of BCTs have been reported, and it is therefore important to understand the nature of these lesions in order to facilitate proper recognition. Relative to other intracranial vascular malformations, updated information on the various epidemiologic, radiographic, and pathologic features of BCTs within the published literature may be inadequate. METHODS: We searched the PubMed database for prior reports of symptomatically-manifested BCTs. Moreover, Google Scholar and PubMed were searched in order to review current epidemiologic, radiographic, pathologic, and pathogenetic features of BCTs. RESULTS: Forty-eight published studies were included for a total of 99 individual cases of BCTs with symptomatic manifestations. Thirty-three symptomatic BCTs were hemorrhagic in nature, while 66 were non-hemorrhagic. The mean age at presentation of hemorrhagic lesions was 25.5 years, and the most common location was the supratentorial CNS (54.5%) with motor disturbance representing the most commonly encountered presenting symptom (26.1%). 15.2% of hemorrhagic lesions were treated with surgical removal. In non-hemorrhagic lesions, the mean age at presentation was 39.8 years with the pons being the most common lesion location (78.5%) and headache being the most common presenting symptom (22.2%). 12.1% of patients with non-hemorrhagic lesions were treated with surgical removal. CONCLUSIONS: Despite their rarity, symptomatic BCTs, both hemorrhagic and non-hemorrhagic, can cause devastating neurological sequelae, potentially through multiple mechanisms. The large majority of these lesions do not require intervention, though surgical removal has been achieved with good outcome in select cases. Further documentation of symptomatic manifestations with or without surgical intervention is vital in order to further understand the clinical, surgical, and pathogenic implications of these less-appreciated vascular malformations.

Symptomatic developmental venous anomalies.

Cerebral developmental venous anomalies (DVAs) are variations of venous vascular anatomy related to an underdevelopment of either the superficial or deep venous emissary system, resulting in a dilated transmedullary vein fed by multiple smaller venous radicles responsible for drainage of normal brain parenchyma. While typically benign and found incidentally on imaging studies, DVAs can rarely be symptomatic. The radiographic appearance of DVAs, as well as their symptomatic manifestations, is diverse. Herein, we will discuss the pathophysiology of symptomatic DVAs while providing illustrative case examples depicting each of their pathogenic mechanisms.

High Prevalence of Developmental Venous Anomaly in Diffuse Intrinsic Pontine Gliomas: A Pediatric Control Study.

BACKGROUND: No link has been demonstrated between diffuse intrinsic pontine glioma and developmental venous anomaly in pediatric patients. OBJECTIVE: To determine the prevalence of developmental venous anomaly in a pediatric cohort of diffuse intrinsic pontine glioma. METHODS: We performed a retrospective cohort study (1998-2017) of consecutive pediatric patients harboring a diffuse intrinsic pontine glioma (experimental set, n = 162) or a craniopharyngioma (control set, n = 142) in a tertiary pediatric neurosurgical center. The inclusion criteria were the following: age <18 yr at diagnosis; histopathological diagnosis of diffuse intrinsic pontine glioma or craniopharyngioma according to the 2016 World Health Organization classification of tumors of the central nervous system; no previous oncological treatment; and available preoperative magnetic resonance imaging performed with similar acquisition protocol. RESULTS: We found a significantly higher prevalence of developmental venous anomaly in the experimental set of 162 diffuse intrinsic pontine gliomas (24.1%) than in the control set of 142 craniopharyngiomas (10.6%; P = .001). The prevalence of developmental venous anomalies was not significantly impacted by demographic data (sex, age at diagnosis, and underlying pathological condition), biomolecular analysis (H3-K27M-mutant subgroup, H3.1-K27M-mutant subgroup, and H3.3-K27M-mutant subgroup), or imaging findings (anatomic location, anatomic extension, side, and obstructive hydrocephalus) of the studied diffuse intrinsic pontine gliomas. CONCLUSION: We report a higher prevalence of developmental venous anomaly in pediatric diffuse intrinsic pontine glioma patients than in control patients, which suggests a potential underlying common predisposition or a causal relationship that will require deeper investigations.

Prevalence and Clinical Implications of the Primitive Trigeminal Artery and its Variants: A Meta-Analysis.

BACKGROUND: The primitive trigeminal artery (PTA) is the most common and the largest persistent carotid-basilar anastomosis. Primitive trigeminal artery variants (PTAVs) are anastomoses between the internal carotid artery and cerebellar arteries. These vessels pose a risk of hemorrhagic or ischemic complications during neurosurgical procedures in the parasellar and intrasellar regions. The aim of this study was to determine the prevalence of both PTA and PTAVs and their clinically important anatomic features. METHODS: Major electronic databases were thoroughly searched for studies on PTA and PTAV. References in the included articles were also evaluated. Data regarding prevalence, laterality, origin, course patterns, and associated anomalies were extracted and pooled into a meta-analysis. RESULTS: A total of 39 studies (110,866 patients) were included in the meta-analysis. The total pooled prevalence estimate of PTA and PTAVs combined was 0.4% (95% confidence interval [CI], 0.3-0.5). Individually, PTA was present in 0.3% of patients and PTAV in 0.2%. Both arteries most often originated from the C4 internal carotid artery and took a course lateral to the dorsum sellae. The anterior inferior cerebellar artery type was the predominant PTAV (72.1%). Basilar artery hypoplasia was found in 42.5% of patients with a PTA. CONCLUSIONS: PTA and PTAVs are rare vessels, but they are clinically important because they can contribute to trigeminal neuralgia. Knowledge of the potential course of these arteries is essential in neuroradiology and neurosurgery, especially in minimally invasive procedures such as the endoscopic endonasal transsphenoidal approach to the pituitary gland and the percutaneous gasserian ganglion procedure.

Clinical Characteristics and Endovascular Treatment for Spinal Dural Arteriovenous Fistula in Japan: Japanese Registry of Neuroendovascular Therapy 2 and 3.

A subgroup analysis of spinal vascular lesions registered in the Japanese Registry of Neuroendovascular Therapy 2 (JR-NET2) and JR-NET3 was performed. About 172 analyzable cases of spinal dural arteriovenous fistula (SDAVF) were assessed, including the characteristics, treatment strategy, and treatment outcome. SDAVF was more common in middle-aged and older males. The most commonly affected area was the thoracolumbosacral region (83.7%), and most cases had a non-hemorrhagic onset (89.0%). Complete obliteration was achieved in 54.7%. Treatment-related complications occurred in three patients (7.0%). Post-treatment neurological improvement was achieved in 48.3%. The primary endpoint [modified Rankin Scale (mRS) score of 0-2 on postoperative day 30] was achieved in 60.5% of the cases. As a new discovery, the incidence of cervical SDAVF increased from 1.8% in JR-NET2 to 19.7% in JR-NET3. Compared with non-cervical SDAVF, cervical SDAVF was characterized by a higher proportion of hemorrhagic onset (P <0.01), incomplete obliteration of the shunt (P <0.01), and embolization-related complications (P = 0.01). Overall, a mRS of 0-2 on postoperative day 30 was correlated with a pre-treatment mRS of 0-2 (P <0.01) in a univariate analysis. Complete obliteration of the shunt was the only predictor of postoperative neurological improvement (P = 0.001) in a multivariate analysis. Endovascular treatment for SDAVF has been safely administered in Japan. The incidence of cervical SDAVF, which has more aggressive features, appears to be increasing. Early diagnosis and complete obliteration of the shunt are important for improving the treatment outcomes of patients with SDAVF.

A Propensity Score-Matched Cohort Analysis of Outcomes After Stereotactic Radiosurgery in Older versus Younger Patients with Dural Arteriovenous Fistula: An International Multicenter Study.

OBJECTIVE: This study aims to evaluate the outcomes of Gamma Knife stereotactic radiosurgery (SRS) for dural arteriovenous fistulas (dAVFs) in older patients (≥65 years) compared with younger patients (age <65 years). METHODS: Two groups with a total of 96 patients were selected from a database of 133 patients with dAVF from 9 international medical centers with a minimum 6 months follow-up. A 1:2 propensity matching was performed by nearest-neighbor matching criteria based on sex, Borden grade, maximum radiation dose given, and location. The older cohort consisted of 32 patients and the younger cohort consisted of 64 patients. The mean overall follow-up in the combined cohort was 42.4 months (range, 6-210 months). RESULTS: In the older cohort, a transverse sinus location was found to significantly predict dAVF obliteration (P = 0.01). The post-SRS actuarial 3-year and 5-year obliteration rates were 47.7% and 78%, respectively. There were no cases of post-SRS hemorrhage. In the younger cohort, the cavernous sinus location was found to significantly predict obliteration (P = 0.005). The 3-year and 5-year actuarial obliteration rates were 56% and 70%, respectively. Five patients (7.8%) hemorrhaged after SRS. Margin dose ≥25 Gy was predictive of unfavorable outcome. The obliteration rate (P = 0.3), post-SRS hemorrhage rate (P = 0.16), and persistent symptoms after SRS (P = 0.83) were not statistically different between the 2 groups. CONCLUSIONS: SRS achieves obliteration in most older patients with dAVF, with an acceptable rate of complication. There was no increased risk of postradiosurgery complications in the older cohort compared with the younger patients.

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.

Cerebral Venous Malformations in a Chinese Population: Clinical Manifestations, Radiological Characteristics, and Long-Term Prognosis.

OBJECTIVE: We elucidated the clinical and radiological characteristics and analyzed the risk factors for hemorrhage and poor outcomes of cerebral venous malformations (CVMs) in a northern Chinese population. METHODS: We included 60 consecutive patients with CVM patients in Beijing Tiantan Hospital from January 2011 to February 2018. The clinical manifestations, radiological characteristics, management, and outcomes were elucidated and analyzed. The patients were followed up for 5-64 months (median, 26). Poor outcomes included repeat bleeding, secondary infarction, severe disability (modified Rankin scale score ≥3), and death. RESULTS: Infratentorial CVMs were more prone to intracranial hemorrhage (75% vs. 28.6%; P < 0.001), dizziness (37.5% vs. 10.7%; P = 0.017), and focal neurological deficits (65.6% vs. 25%; P = 0.002) than were supratentorial CVMs. Supratentorial CVMs were more prone to seizure (32.1% vs. 0%; P = 0.001) than were infratentorial CVMs. Multivariate logistic regression revealed that the major risk factors for intracranial hemorrhage in CVMs were infratentorial lesions (P = 0.003) and complicated cavernous angiomas (P = 0.016). Compared with conservative treatment, resection of hematoma or cavernous angiomas with CVM preservation did not increase the risk of poor outcomes (P = 0.646). However, CVM resection significantly increased that risk (odds ratio, 44.0; P = 0.003). CONCLUSIONS: Our results have shown that conservative treatment of CVMs results in a relatively good prognosis. For those complicated by hemorrhage or cavernous angiomas requiring surgical interventions, the integrity of the CVM should be preserved, irrespective of the treatment. In exceptional cases, before CVM resection, the CVM drainage should be comprehensively evaluated.