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BACKGROUND AND PURPOSE: AVFs of the foramen magnum region, including fistulas of the marginal sinus and condylar veins, have complex arterial supply, venous drainage, symptoms, and risk features that are not well-defined. The purpose of this study was to present the angioarchitectural and clinical phenotypes of a foramen magnum region AVF from a large, single-center experience. MATERIALS AND METHODS: We retrospectively reviewed cases from a 10-year neurointerventional data base. Arterial and venous angioarchitectural features and clinical presentation were extracted from the medical record. Venous drainage patterns were stratified into 4 groups as follows: type 1 = unrestricted sinus drainage, type 2 = sinus reflux (including the inferior petrosal sinus), type 3 = reflux involving sinuses and cortical veins, and type 4 = restricted cortical vein outflow or perimedullary congestion. RESULTS: Twenty-eight patients (mean age, 57.9 years; 57.1% men) had 29 foramen magnum region AVFs. There were 11 (37.9%) type 1, nine (31.0%) type 2, six (20.7%) type 3, and 3 (10.3%) type 4 fistulas. Pulsatile tinnitus was the most frequent symptom (82.1%), followed by orbital symptoms (31.0%), subarachnoid hemorrhage (13.8%), cranial nerve XII palsy (10.3%), and other cranial nerve palsy (6.9%). The most frequent arterial supply was the ipsilateral ascending pharyngeal artery (93.1% ipsilateral, 55.5% contralateral), vertebral artery (89.7%), occipital artery (65.5%), and internal carotid artery branches (48.3%). CONCLUSIONS: We present the largest case series of foramen magnum region AVFs to date and show that clinical features relate to angioarchitecture. Orbital symptoms are frequent when sinus reflux is present. Hemorrhage was only observed in type 3 and 4 fistulas.

BackgroundPediatric spinal arteriovenous shunts (SAVS) are rare lesions with heterogeneous pathogenesis and clinical manifestations.ObjectiveTo evaluate the clinical characteristics, angioarchitecture, and technical/clinical outcomes in SAVS through a large single-center cohort analysis and meta-analysis of individual patient data.MethodsA retrospective institutional database identified children (aged 0–21 years) who underwent digital subtraction spinal angiography (DSA) for SAVS between January 1996 and July 2021. Clinical data were recorded to evaluate angioarchitecture, generate modified Aminoff-Logue gait disturbance scores (AL) and McCormick grades (MC), and assess outcomes. We then performed a systematic literature review following PRISMA-IPD (Preferred Reporting Items for Systematic Reviews and Meta-Analyses for individual patient data) guidelines, extracting similar data on individual patients for meta-analysis.ResultsThe cohort consisted of 28 children (M:F=11:17) with 32 SAVS lesions, with a mean age of 12.8±1.1 years at diagnosis. At presentation, SAVS were most highly concentrated in the cervical region (40.6%). Children had a median AL=2 and MC=2, with thoracolumbar AVS carrying the greatest disability. Among treated cases, complete obliteration was achieved in 48% of cases and median AL scores and MC grades both improved by one point. Systematic literature review identified 161 children (M:F=96:65) with 166 SAVS lesions with a mean age of 8.7±0.4 years. Among studies describing symptom chronicity, 37/51 (72.5%) of children presented acutely. At presentation, children had a median AL=4 and MC=3, with thoracolumbar AVS carrying the highest MC grades. After intervention, median AL and MC both improved by one point.ConclusionsThis study provides epidemiologic information on the location, onset, and presentation of the full spectrum of pediatric SAVS, highlighting the role of targeted treatment of high-risk features.

BackgroundNo randomized trial of intracranial aneurysm coiling has compared long-term efficacy of polymer-modified coils to bare metal coils (BMCs). We report 5-year results comparing Matrix2 coils to BMCs. The primary objective was to compare the rates of target aneurysm recurrence (TAR) at 12 months. Secondary objectives included angiographic outcomes at TAR or 12 months and TAR at 5 years.MethodsA total of 626 patients were randomized to BMCs or Matrix2 coils. Detailed methods and 1-year results have been published previously.ResultsOf 580 patients eligible for 5-year follow-up, 431 (74.3%) completed follow-up or reached TAR. Matrix2 coils were non-inferior to BMCs (P=0.8) but did not confer any benefit. Core lab reported post-treatment residual aneurysm filling (Raymond III) correlated with TAR (PConclusionsAfter 5 years Matrix2 coils were non-inferior to BMCs but no benefit was demonstrated. Post-treatment residual angiographic aneurysm filling (Raymond III) is strongly associated with TAR (P

Background and purposeAutomated CTP software is increasingly used for extended window emergent large-vessel occlusion to quantify core infarct. We aimed to assess whether RAPID software underestimates core infarct in patients with an extended window recently receiving IV iodinated contrast.Materials and methodsWe reviewed a prospective, single-center data base of 271 consecutive patients who underwent CTA ± CTP for acute ischemic stroke from May 2018 through January 2019. Patients with emergent large-vessel occlusion confirmed by CTA in the extended window (>6 hours since last known well) and CTP with RAPID postprocessing were included. Two blinded raters independently assessed CT ASPECTS on NCCT performed at the time of CTP. RAPID software used relative cerebral blood flow of

The pharyngo-tympano-stapedial middle meningeal artery (PTS-MMA) variant has been described in one case report and never in the setting of arterial supply to a dural arteriovenous fistula, to our knowledge. We report the case of a middle-aged patient with severe, daily headache who presented to our institution for angiography and treatment. CT angiography and MRI demonstrated an enlarged left middle meningeal artery coursing to a large venous varix in the falcotentorial region. Dural arteriovenous fistula was confirmed by subsequent cerebral angiography. Endovascular treatment was performed but without complete obliteration of the fistula. Follow-up angiography demonstrated parasitized arterial supply from a right middle meningeal artery arising from the proximal cervical internal carotid artery coursing through the middle ear consistent with a PTS-MMA variant. The fistula was then treated surgically without recurrence at the 6-month follow-up.

Background Giant cell arteritis (GCA) is a systemic vasculitis that may cause ischemic stroke. Rarely, GCA can present with aggressive intracranial stenoses, which are refractory to medical therapy. Endovascular treatment (EVT) is a possible rescue strategy to prevent ischemic complications in intracranial GCA but the safety and efficacy of EVT in this setting are not well-described. Methods A systematic literature review was performed to identify case reports and series with individual patient-level data describing EVT for intracranial GCA. The clinical course, therapeutic considerations, and technique of seven endovascular treatments in a single patient from the authors’ experience are presented. Results The literature review identified 9 reports of 19 treatments, including percutaneous transluminal angioplasty (PTA) with or without stenting, in 14 patients (mean age 69.6 ± 6.3 years). Out of 12 patients 8 (66.7%) with sufficient data had > 1 pre-existing cardiovascular risk factor. All patients had infarction on MRI while on glucocorticoids and 7/14 (50%) progressed despite adjuvant immunosuppressive agents. Treatment was PTA alone in 15/19 (78.9%) cases and PTA + stent in 4/19 (21.1%). Repeat treatments were performed in 4/14 (28.6%) of patients (PTA-only). Non-flow limiting dissection was reported in 2/19 (10.5%) of treatments. The indications, technical details, and results of PTA are discussed in a single illustrative case. We report the novel use of intra-arterial calcium channel blocker infusion (verapamil) as adjuvant to PTA and as monotherapy, resulting in immediate improvement in cerebral blood flow. Conclusion Endovascular treatment, including PTA with or without stenting or calcium channel blocker infusion, may be effective therapies in medically refractory GCA with intracranial stenosis.

BACKGROUND Carotid cavernous fistulas (CCF) often present with diplopia secondary to cranial nerve palsy (CNP). Immediate development of postoperative CNP has been described in the literature. This study described delayed-onset of CNP after complete and reconfirmed obliteration of the CCF and resolution of initial CNP. METHODS A retrospective analysis was performed on patients with indirect CCF between 1987 and 2006 at 4 academic endovascular centers. Details of the endovascular procedures, embolic agents used, and complications were studied. Partial or complete obliteration was determined. Immediate and delayed cranial nerve palsies were independently assessed. RESULTS A total of 267 patients with symptomatic indirect CCF underwent transvenous endovascular treatment. Four patients (1.5%) developed delayed abducens nerve (VI) palsy after complete resolution of presenting symptoms after embolization. Delayed presentation ranged between 3 and 13 months after complete resolution of initial double vision and cranial nerve palsies. Transvenous coil embolization through the inferior petrosal sinus was performed in all 4 affected patients. All had follow-up angiography confirming durable closure of their CCF. MRI did not show new mass lesions or abnormal soft tissue enhancement. In all 4 patients, their abducens nerve (VI) palsy remained. CONCLUSIONS Delayed CNP can develop despite complete endovascular obliteration of the CCF. The cause of delayed CNP is not yet determined, but may represent fibrosis and ischemia. Long-term follow-up is needed even after complete neurological and radiological recovery is attained in the immediate perioperative period.

BACKGROUND AND PURPOSE: Arterial access is a technical consideration of mechanical thrombectomy that may affect procedural time, but few studies exist detailing the relationship of anatomy to procedural times and patient outcomes. We sought to investigate the respective impact of aortic arch and carotid artery anatomy on endovascular procedural times in patients with large-vessel occlusion. MATERIALS AND METHODS: We retrospectively reviewed imaging and medical records of 207 patients from 2 academic institutions who underwent mechanical thrombectomy for anterior circulation large-vessel occlusion from January 2015 to July 2018. Preintervention CTAs were assessed to measure features of the aortic arch and ipsilateral great vessel anatomy. These included the cranial-to-caudal distance from the origin of the innominate artery to the top of the aortic arch and the takeoff angle of the respective great vessel from the arch. mRS scores were calculated from rehabilitation and other outpatient documentation. We performed bootstrap, stepwise regressions to model groin puncture to reperfusion time and binary mRS outcomes (good outcome, mRS ≤ 2). RESULTS: From our linear regression for groin puncture to reperfusion time, we found a significant association of the great vessel takeoff angle (P = .002) and caudal distance from the origin of the innominate artery to the top of the aortic arch (P = .05). Regression analysis for the binary mRS revealed a significant association with groin puncture to reperfusion time (P

BackgroundPercutaneous sclerotherapy is an effective treatment for lymphatic malformations (LM) of the head and neck in adults. The purpose of this study was to examine the indications and efficacy of sclerotherapy for head/neck LM in the neonate and infant population.MethodsWe retrospectively reviewed patients treated with percutaneous sclerotherapy for LM of the head/neck at age ≤12 months at a single vascular anomalies clinic. The clinical, anatomic, and technical aspects of each treatment, complications, and post-treatment clinical and imaging outcomes were analyzed.Results22 patients underwent 36 treatments during the first year of life. Median age at first treatment was 6.2 months (range 2–320 days). Severe airway compromise was the most frequent indication for treatment (31.8%). Sclerosants included doxycycline (80.5%), sodium tetradecyl sulfate (55.5%), bleomycin (11.1%) and ethanol (2.8%). There were no immediate procedure-related complications; sclerosant-related laboratory complications included transient metabolic acidosis (8.3%) and hemolytic anemia (5.5%). Median follow-up was 3.7 years (IQR 0.6–4.8). 47.6% of patients showed >75% lesion size reduction and 19.0% showed minimal response (ConclusionsPercutaneous sclerotherapy is a safe and effective treatment for symptomatic LM of the head and neck in neonates and infants. Treatment strategy and management of recurrent symptoms requires consensus from an experienced, multidisciplinary team.

The simultaneous growth of robotic-assisted surgery and telemedicine in recent years has only been accelerated by the recent coronavirus disease 2019 pandemic. Robotic assistance for neurovascular intervention has garnered significant interest due to opportunities for tele-stroke models of care for remote underserved areas. Lessons learned from medical robots in interventional cardiology and neurosurgery have contributed to incremental but vital advances in medical robotics despite important limitations. In this article, we discuss robot types and their clinical justification and ethics, as well as a general overview on available robots in thoracic/abdominal surgery, neurosurgery, and cardiac electrophysiology. We conclude with current clinical research in neuroendovascular intervention and a perspective on future directions.

Percutaneous sclerotherapy is an effective technique for treating lymphatic malformations of the head and neck, with clinical success rates exceeding 84%.1 Sodium tetradecyl, which damages lipid membranes and stimulates free radical-induced local damage, and doxycycline, which inhibits angiogenesis, have emerged as the safest and most effective of several available sclerosants.2–4 Although severe periprocedural morbidity is rare, temporary local complications are reported in 14% and skin necrosis or scarring in up to 0.8–5.8% of sclerotherapy procedures.5 As these lesions are frequently located in the face and/or neck, even minor complications can be disfiguring and must be avoided. This technical video describes a ‘dual-agent’ approach for percutaneous sclerotherapy of macrocystic lymphatic malformations using sodium tetradecyl as a ‘primer’ followed by doxycycline as a definitive sclerosant (video 1). This technique emphasizes meticulous backtable preparation and effective use of ultrasound and fluoroscopy to minimize complications.Video 1

Although enthusiasm for transradial access for neurointerventional procedures has grown, a unique set of considerations bear emphasis to preserve safety and minimize complications. In the first part of this review series, we will review important anatomical considerations for safe and easy neuroendovascular procedures from a transradial approach. These include normal and variant radial artery anatomy, the anatomic snuffbox, as well as axillary, brachial, and great vessel arterial anatomy that is imperative for the neuroendovascular surgeon to be intimately familiar prior to pursuing transradial access procedures. In the next part of the review series, we will focus on safety and complications specific to a transradial approach.

Large, wide-necked basilar apex aneurysms are difficult to treat. Microsurgical clipping can result in neurologic morbidity and mortality. Endovascular treatment often leaves remnants that need retreatment and/or stent placement with dual antiplatelet therapy. The Woven EndoBridge (WEB) is an intrasaccular flow disruption device that can be used without dual antiplatelet therapy. However, the WEB cannot typically be used in large or giant aneurysms > 10 mm because the largest diameter device is the 11 × 9.6 mm single layer sphere (SLS). We present a case in which we use a PulseRider aneurysm neck reconstruction device in the basilar artery to assist in WEB deployment within a 22 mm basilar apex aneurysm with 14 mm neck, thereby permitting aspirin monotherapy postoperatively.

Central venous stenosis in hemodialysis patients rarely causes venous hypertension and intracranial hemorrhage. A 54 year-old male with right arm arteriovenous fistula was transferred to our institution in a comatose state following right parietal venous infarction. Fistulography showed right brachiocephalic vein (BCV) occlusion with reflux into the right transverse sinus and obstruction of left internal jugular vein outflow due to the styloid process. Balloon venoplasty of the right BCV occlusion failed to improve the patient's status because of the delayed diagnosis. Headaches and neurologic symptoms in hemodialysis patients can herald intracranial hypertension due to central venous occlusion and needs prompt assessment with fistulography.

Background Heterogenous central nervous system (CNS) neurologic manifestations of polyarteritis nodosa (PAN) are underrecognized. We review three cases of patients with PAN that illustrate a range of nervous system pathology, including the classical mononeuritis multiplex as well as uncommon brain and spinal cord vascular manifestations. Case presentation Case 1 presented with mononeuritis multiplex and characteristic skin findings. Case 2 presented with thunderclap headache and myelopathy due to spinal artery aneurysm rupture. Both patients experienced disease remission upon treatment. Case 3 presented with headache and bulbar symptoms due to partially thrombosed intracranial aneurysms, followed by systemic manifestations related to visceral aneurysms. She demonstrated clinical improvement with treatment, was lost to follow-up, then clinically deteriorated and entered hospice care. Conclusions Although the peripheral manifestations of PAN are well-known, PAN association with CNS neurovascular disease is relatively underappreciated. Clinician awareness of the spectrum of neurologic disease is required to reduce diagnostic delay and promote prompt diagnosis and treatment with immunosuppressants.

BACKGROUND Endovascular surgery is the first-line treatment for indirect cavernous carotid fistulae (CCFs). This study compares multiple treatment techniques. OBJECTIVE To compare endovascular techniques for indirect CCF treatment. METHODS Retrospective analysis was performed of prospectively maintained records at 4 centers, identifying patients undergoing indirect CCF embolization. Demographics, symptoms, and lesion characteristics were recorded. Medical records were reviewed for changes in symptoms, delayed complications, and angiographically proven recurrence. Univariate and multivariate analyses were performed to identify impacts of the above characteristics on outcomes. RESULTS Sufficient records were available for 267 patients treated between January 1987 and December 2016. Obliteration was achieved in 86.5% patients, occurring in 86.9% of exclusively transvenous treatments and 79.5% of other treatments. Obliteration rates were highest following transvenous embolization using coils compared to all other materials (likelihood ratio [LR] 5.0, P = .024). Complications were less common with coil embolization compared to other materials (LR 0.070, P

BackgroundDural arteriovenous fistulas (AVF) of the foramen magnum region (FMR) are technically challenging lesions to treat. Transvenous (TV), transarterial (TA), and surgical approaches have been described, but the optimum treatment strategy is not defined.ObjectiveTo report treatment strategies and outcomes for FMR-AVF at a single, high-volume referral center.MethodsA retrospective review from January 2010 to August 2020 identified patients with FMR-AVF at a single referral center. Angiographic features, treatment (observation, endovascular, surgical), and follow-up of angiographic and clinical results were recorded. The technical aspects of TV embolization are then presented in detail.Results29 FMR-AVF were identified in 28 patients. Of these, 24/29 (82.8%) were treated and 5/29 (17.2%) were observed. Treatment was endovascular in 21/24 (87.5%), combined (endovascular+surgical) in 2/24 (8.3%), and surgical in 1/24 (4.2%). Endovascular treatments were 76.2% TV, 14.3% TA, and 9.5% combined TV/TA. Sufficient follow-up data were available for 20/28 (71.4%) with mean follow-up of 16.8 months. No AVF recurrence was seen for TA/TV, combined endovascular/surgical, or surgical groups, and there was one recurrence (7.1%) in the TV group. Symptomatic improvement was seen in all groups: TV (71.4% complete, 28.6% partial), TA (66.7% complete, 33.3% no follow-up), TV+TA (100% partial), endovascular/surgical (100% complete), and surgical (100% partial). Minor non-neurologic complications included 1/14 (7.1%) in the TV group and 1/3 (33.3%) in the TA/TV group.ConclusionEndovascular treatment is safe and effective for most FMR-AVF. TV embolization has a high cure rate with few complications.

Although enthusiasm for transradial access for neurointerventional procedures has grown, a unique set of considerations bear emphasis to preserve safety and minimize complications. In the first part of this review series, we reviewed anatomical considerations for safe and easy neuroendovascular procedures from a transradial approach. In this second part of the review series, we aim to (1) summarize evidence for safety of the transradial approach, and (2) explain complications and their management.

BACKGROUND AND PURPOSE: Hemodynamic features of brain AVMs may portend increased hemorrhage risk. Previous studies have suggested that MTT is shorter in ruptured AVMs as assessed on quantitative color-coded parametric DSA. This study assesses the interrater reliability of MTT measurements obtained using quantitative color-coded DSA. MATERIALS AND METHODS: Thirty-five color-coded parametric DSA images of 34 brain AVMs were analyzed by 4 neuroradiologists with experience in interventional neuroradiology. Hemodynamic features assessed included MTT of the AVM and TTP of the dominant feeding artery and draining vein. Agreement among the 4 raters was assessed using the intraclass correlation coefficient. RESULTS: The interrater reliability among the 4 raters was poor (intraclass correlation coefficient = 0.218; 95% CI, 0.062–0.414; P value = .002) as it related to MTT assessment. When the analysis was limited to cases in which the raters selected the same image to analyze and selected the same primary feeding artery and the same primary draining vein, interrater reliability improved to fair (intraclass correlation coefficient = 0.564; 95% CI, 0.367–0.717; P

Background and Purpose: Do children have an increased risk for brain arteriovenous malformation (AVM) recurrence compared with adults and does this risk vary depending on initial presentation with AVM rupture? Methods: We retrospectively studied 115 patients initially presenting with brain AVM under age 25 years who underwent complete surgical resection of the AVM as documented by digital subtraction angiography (DSA) and had delayed follow-up DSA to evaluate for AVM recurrence after apparent initial cure. Results: The mean time from baseline DSA to follow-up DSA was 2.3 years, ranging from 0 to 15 years. Twelve patients (10.4% of the 115 patient cohort and 16.7% of 72 patients with hemorrhage at initial presentation) demonstrated AVM recurrence on follow-up DSA. All patients with recurrence initially presented with intracranial hemorrhage, and intracranial hemorrhage was a significant predictor of recurrence (log rank P =0.037). Among patients with initial hemorrhage, the 5-year recurrence rate was 17.8% (95% CI, 8.3%–35.7%). All recurrences occurred in patients who were children at the time of their initial presentation; the oldest was 15 years of age at the time of initial AVM surgery. The 5-year recurrence rate for children (0–18 years of age) with an initial presentation of hemorrhage was 21.4% (95% CI, 10.1%–41.9%). Using Cox regression, we found the risk of AVM recurrence decreased by 14% per each year increase in age at the time of initial surgical resection (hazard ratio=0.86 [95% CI, 0.75–0.99]; P =0.031). Conclusions: There is a high rate of recurrence of apparently cured brain AVMs in children who initially present with AVM rupture. Imaging follow-up is warranted to prevent re-rupture.

Author(s): Gautam, Ayushi; Sun, Zhengda; Winkler, Ethan; Su, Hua; McCalmont, Timothy H; Kim, Helen; Tihan, Tarik; Hoffman, William Y; Dowd, Chris F; Frieden, Ilona J; Cooke, Daniel L | Abstract: BackgroundWe report a rare case of a 19-year-old female progressively affected by a peripheral arteriovenous malformation (pAVM), a midline cerebellar astrocytoma, and a brain arteriovenous malformation (bAVM).Case descriptionShe presented with a pulsatile mass on her left cheek, which was classified as a pAVM through angiography. Following treatment with embolization and surgical resection, she returned with enlargement of the mass and imaging incidentally identified a cerebellar astrocytoma. Suboccipital craniotomy, C1 laminectomy, and endoscopic third ventriculostomy were subsequently performed. She was later treated again for growth of her pAVM, and angiography revealed the presence of a left temporal bAVM, which was resected via a pterional craniotomy.ConclusionsPathological staining identified activation of mTOR and RAS/MAPK pathway in the patient's pAVM and bAVM tissue samples. Furthermore, genetic sequencing demonstrated an activating MAPK21 (K57N) mutation in the pAVM and a gain of distal chromosome 7q in the pilocytic astrocytoma. No germline mutation was identified to explain all pathologies. This case demonstrates the need for continued development and further integration of multi-disciplinary genetic, radiological, and neurological treatment teams to effectively care for such complex presentations.

Introduction Magnetic resonance imaging (MRI) is most sensitive and specific for characterizing venous malformations (VMs). VMs typically demonstrate central enhancement on delayed-contrast imaging. Fluid-fluid levels (FFLs) are uncommon in VMs and common in lymphatic malformations (LMs). Technology has advanced since the initial description of these findings. Rates of detection of these MRI findings in VMs may have changed as MRI technology and techniques have evolved. Methods and methods A prospectively maintained database from a multidisciplinary vascular anomalies clinic was reviewed to identify patients with final diagnosis of VM or LM. Patients with reviewable contrast-enhanced MRIs were selected, reviewing the oldest MRI studies in the database against the newest MRI studies to identify equal numbers of patients from the temporal extremes. Imaging was reviewed to assess for presence of FFLs. Enhancement was quantified by measuring signal in the same location of the lesion both on pre- and postcontrast sequences Results Forty patients were identified for analysis. Twenty studies with sufficient archived imaging for review were performed between 1995 and 2006; 20 such studies were performed between 2011 and 2012. The new imaging cohort had higher rates of FFL visualization ( p = 0.001). Correlation was found between time to imaging following contrast and degree of enhancement ( p Conclusion FFLs should no longer be considered exclusionary for the diagnosis of VMs. Timing following contrast administration should be maximized to increase degree of enhancement to confirm the diagnosis of VMs.

ObjectiveTo evaluate the safety and effectiveness of the Flow Redirection Endoluminal Device (FRED) flow diverter in support of an application for Food and Drug Administration approval in the USA.Methods145 patients were enrolled in a prospective, single-arm multicenter trial. Patients with aneurysms of unfavorable morphology for traditional endovascular therapies (large, wide-necked, fusiform, etc) were included. The trial was designed to demonstrate non-inferiority in both safety and effectiveness, comparing trial results with performance goals (PGs) established from peer-reviewed published literature. The primary safety endpoint was death or major stroke (National Institutes of Health Stroke Scale score ≥4 points) within 30 days of the procedure, or any major ipsilateral stroke or neurological death within the first year. The primary effectiveness endpoint was complete occlusion of the target aneurysm with ≤50% stenosis of the parent artery at 12 months after treatment, and in which an alternative treatment of the target intracranial aneurysm had not been performed.Results145 patients underwent attempted placement of a FRED device, and one or more devices were placed in all 145 patients. 135/145 (93%) had a single device placed. Core laboratory adjudication deemed 106 (73.1%) of the aneurysms large or giant. A safety endpoint was experienced by 9/145 (6.2%) patients, successfully achieving the safety PG of 46% aneurysm occlusion was also achieved, with the effectiveness endpoint being met in 80/139 (57.6%)ConclusionAs compared with historically derived performance benchmarks, the FRED flow diverter is both safe and effective for the treatment of appropriately selected intracranial aneurysms.Clinical registration numberNCT01801007

Introduction: Arterial access is a technical consideration of mechanical thrombectomy that may affect procedure time. Few studies exist detailing the impact of aortic arch and carotid anatomy on procedure times and patient outcomes. Methods: We included 56 patients who underwent embolectomy with successful revascularization for acute ischemic stroke in the anterior circulation from a period of 01/2016-05/2018. The average age was 71 (+/- 17 years) with 39% being male. We calculated anatomic variables on the affected side from CT angiograms immediately prior to embolectomy including the medial-to-lateral span, as well as the anterior-to-posterior span, of both the aortic arch and carotid arteries. In addition, the take-off angle of the respective vessel (left common carotid or right brachiocephalic) was calculated. Charts were reviewed for procedural times and epidemiologic information (HTN, HLD, DM, CAD and Afib). Modified Rankin Scale (mRS) was calculated from PT/OT and outpatient neurology notes. Partial correlation coefficients were performed between anatomic variables, temporal variables and outcome variables after adjustment for age, gender and epidemiologic information. Results: There was a significant positive correlation between procedure time (time at groin puncture to time at reperfusion) and take-off angle. There were no other significant correlations between anatomic measures and procedure time. In addition, there was as a significant positive correlation between both procedure time and time from last seen normal to reperfusion and delta mRS (the difference between pre-stroke and post-stroke mRS). See table 1 for correlations. Conclusion: These results suggest that patients with larger take-off angles have an association with longer procedural times and worse outcomes. If these patients can be effectively identified prior to the procedure, operators could feasibly use a non-femoral access method initially to reduce procedure time.

IntroductionIndirect cavernous carotid fistulae (ICCFs) can present with insidious, non-specific symptoms and prove difficult to diagnose. This study evaluates associations among ICCF symptoms and angiographic findings.MethodsA retrospective analysis was performed of prospectively maintained records at four medical centers to identify patients with ICCFs evaluated with angiography. Patient demographics, symptoms, and angiographic findings were tabulated. Univariate and multivariate analyses were conducted to identify associations among these variables.ResultsRecords sufficient for review existed for 267 patients evaluated with angiography. Patients were most commonly women, in the sixth or seventh decade of life, and had symptoms for months before a definitive diagnosis. The most common symptoms included proptosis, diplopia, cranial nerve palsy, and chemosis. Cortical venous reflux was most common in patients with chemosis, orbital pain, or bruit. Intracranial hemorrhage was associated with cortical reflux and bilateral inferior petrosal sinus occlusion. Patients with loss of symptoms demonstrated higher rates of inferior petrosal sinus occlusion and a trend towards rupture.ConclusionA high index of suspicion is needed to promptly diagnose patients with ICCFs. High risk features are more common in the setting of chemosis, orbital pain, bruit, or spontaneous loss of symptoms. Patients with such symptoms warrant expedited angiographic evaluation.

In the endovascular treatment of cerebral arteriovenous malformations, ethanol sclerotherapy is seldom used due to safety concerns. However, when limited reflux of an embolic agent is permissible or when there is a long distance to the target, ethanol may be preferable. We reviewed 10 patients with 14 cerebral AVM feeding artery aneurysms or intranidal aneurysms treated with intra-arterial ethanol sclerotherapy at our institution between 2005 and 2014. All patients presented with acute intracranial hemorrhage. Thirteen of 14 aneurysms were treated primarily with 60%-80% ethanol into the feeding artery. Complete target feeding artery and aneurysm occlusion was seen in all cases; 8/13 (62%) were occluded by using ethanol alone. No retreatments or recurrences were seen. One permanent neurologic deficit (1/13, 7.7%) and no deaths occurred. In a subset of ruptured cerebral AVMs, ethanol sclerotherapy of feeding artery aneurysms and intranidal aneurysms can be performed with a high degree of technical success and a low rate of complication.

BACKGROUND AND PURPOSE: Although intracranial dural arteriovenous fistulas are principally supplied by dural branches of the external carotid, internal carotid, and vertebral arteries, they can also be fed by pial arteries that supply the brain. We sought to determine the frequency of neurologic deficits following treatment of intracranial dural arteriovenous fistulas with and without pial artery supply. MATERIALS AND METHODS: One hundred twenty-two consecutive patients who underwent treatment for intracranial dural arteriovenous fistulas at our hospital from 2008 to 2015 were retrospectively reviewed. Patient data were examined for posttreatment neurologic deficits; patients with such deficits were evaluated for imaging evidence of cerebral infarction. Data were analyzed with multivariable logistic regression. RESULTS: Of 122 treated patients, 29 (23.8%) had dural arteriovenous fistulas with pial artery supply and 93 (76.2%) had dural arteriovenous fistulas without pial arterial supply. Of patients with pial artery supply, 4 (13.8%) had posttreatment neurologic deficits, compared with 2 patients (2.2%) without pial artery supply ( P = .04). Imaging confirmed that 3 patients with pial artery supply (10.3%) had cerebral infarcts, compared with only 1 patient without pial artery supply (1.1%, P = .03). Increasing patient age was also positively associated with pial supply and treatment-related complications. CONCLUSIONS: Patients with dural arteriovenous fistulas supplied by the pial arteries were more likely to experience posttreatment complications, including ischemic strokes, than patients with no pial artery supply. The approach to dural arteriovenous fistula treatment should be made on a case-by-case basis so that the risk of complications can be minimized.

BACKGROUND AND PURPOSE: A minority of intracranial dural arteriovenous fistulas progress with time. We sought to determine features that predict progression and define outcomes of patients with progressive dural arteriovenous fistulas. MATERIALS AND METHODS: We performed a retrospective imaging and clinical record review of patients with intracranial dural arteriovenous fistula evaluated at our hospital. RESULTS: Of 579 patients with intracranial dural arteriovenous fistulas, 545 had 1 fistula (mean age, 45 ± 23 years) and 34 (5.9%) had enlarging, de novo, multiple, or recurrent fistulas (mean age, 53 ± 20 years; P = .11). Among these 34 patients, 19 had progressive dural arteriovenous fistulas with de novo fistulas or fistula enlargement with time (mean age, 36 ± 25 years; progressive group) and 15 had multiple or recurrent but nonprogressive fistulas (mean age, 57 ± 13 years; P = .0059, nonprogressive group). Whereas all 6 children had fistula progression, only 13/28 adults ( P = .020) progressed. Angioarchitectural correlates to chronically elevated intracranial venous pressures, including venous sinus dilation (41% versus 7%, P = .045) and pseudophlebitic cortical venous pattern ( P = .048), were more common in patients with progressive disease than in those without progression. Patients with progressive disease received more treatments than those without progression (median, 5 versus 3; P = .0068), but as a group, they did not demonstrate worse clinical outcomes (median mRS, 1 and 1; P = .39). However, 3 young patients died from intracranial venous hypertension and intracranial hemorrhage related to progression of their fistulas despite extensive endovascular, surgical, and radiosurgical treatments. CONCLUSIONS: Few patients with dural arteriovenous fistulas follow an aggressive, progressive clinical course despite treatment. Younger age at initial presentation and angioarchitectural correlates to venous hypertension may help identify these patients prospectively.

Introduction: Cerebral aneurysms affect 1-6% of the population and their rupture carries significant mortality and morbidity. The paucity of data related to histopathological features of aneurysms stems largely from the risks associated with aneurysm tissue collection. We set out to establish a feasible technique to isolate viable endothelial cells (EC) and to characterize the differences in genetic expression (GE) between iliac arterial cells vs. aneurysm cells and non-ruptured vs. ruptured aneurysm cells. Methods: 10 patients (5 non-ruptured and 5 ruptured) undergoing aneurysm treatment were enrolled under IRB approval. ECs from aneurysms and iliac arteries were sampled using an appropriately sized coil and a 0.035 “J” wire respectively. Coils and wires were processed via FACS and single cell qPCR to quantify expression of 48 genes implicated in EC function and aneurysm pathogenesis. Mixed models were performed using each GE level as the outcome with multiple predictors. Unsupervised cluster analysis including: hierarchical heat-map clustering, K-medoids, principle components, cART, and self-organizing maps (SOM) were used to find cell clusters based on coordinately expressed GE. Results: 437 FACS-sorted cells were collected and 319 demonstrated CD31, CD34 and CD105 triple-positive expression. Of these, 94 were aneurysmal cells. The average number of extracted aneurysm cells per patient was 9.4 (1-24, median 6.5) with a trend for higher cell yields from ruptured aneurysms (p = 0.116). Mixed model analyses revealed a history of smoking showed the strongest associations with GE (median p-value 0.104 with 11/48 analyses p < 0.05); 5 genes significantly predicted rupture status (median p-value 0.363 with 5/48 analyses p < 0.05); and one gene significantly differentiated cell type (median p-value 0.557, with 1/48 analyses p < 0.05). SOM analysis revealed clustered GE profiles suggestive of distinct functional EC sub-populations. Conclusion: Cerebral endovascular sampling is a safe and reliable means for targeted cell collection for genetic analysis. Smoking was strongly associated with GE levels. Our analysis provides evidence that GE levels may be associated with cell type and rupture status.

The surgical management of Cushing's disease is often complicated by difficulties detecting corticotropic adenomas. Various diagnostic modalities are used when conventional magnetic resonance imaging (MRI) is negative or inconclusive. We sought to analyze our use of two such modalities in the surgical management of Cushing's disease: (1) cavernous/inferior petrosal sinus sampling (central venous sampling, CVS) for adrenocorticotropic hormone and (2) dynamic MRI (dMRI). We conducted a single-center, retrospective review of all patients with Cushing's disease treated by a single neurosurgeon with endonasal transsphenoidal surgery. Accuracy of adenoma localization with CVS and dMRI was analyzed. Ninety-one consecutive patients were included. Pathology confirmed an adenoma in 66. Preoperative dMRI and CVS were performed in 40 and 37 patients, respectively, with 20 undergoing both studies. Surgical pathology was positive for adenoma in 31 dMRI patients, 25 CVS patients, and 13 who underwent both. Among patients with pathology confirming an adenoma, dMRI identified a lesion in 96.8% and correctly lateralized the lesion in 89.7%, while CVS correctly lateralized in 52.2-65.2% (depending on location of sampling). Among patients with both studies, dMRI and CVS correctly lateralized in 76.9 and 61.5-69.2%, respectively. Accuracy of CVS improved if only patients with symmetric venous drainage were considered. In this mixed population of Cushing's disease patients, dMRI was more accurate than CVS at localizing adenomas, supporting the use of advance MRI techniques in the work-up of Cushing's disease. CVS, however, remains an important tool in the workup of Cushing's syndrome.

Background and purpose Retinoblastoma (RB) is a rare malignancy affecting the pediatric population. Intravenous chemotherapy is the longstanding delivery method, although intra-arterial (IA) chemotherapy is gaining popularity given the reduced side effects compared with systemic chemotherapy administration. Given the sensitivity of the target organ, patient age, and secondary tumor susceptibility, a premium has been placed on minimizing procedural related radiation exposure. Materials and methods To reduce patient x-ray dose during the IA infusion procedure, customized surgical methods and fluoroscopic techniques were employed. The routine fluoroscopic settings were changed from the standard 7.5 pulses/s and dose level to the detector of 36 nGy/pulse, to a pulse rate of 4 pulses/s and detector dose to 23 nGy/pulse. The angiographic dose indicators (reference point air kerma (K a ) and fluoroscopy time) for a cohort of 10 consecutive patients (12 eyes, 30 infusions) were analyzed. An additional four cases (five eyes, five infusions) were analyzed using dosimeters placed at anatomic locations to reflect scalp, eye, and thyroid dose. Results The mean K a per treated eye was 20.1±11.9 mGy with a mean fluoroscopic time of 8.5±4.6 min. Dosimetric measurements demonstrated minimal dose to the lens (0.18±0.10 mGy). Measured entrance skin doses varied from 0.7 to 7.0 mGy and were 73.4±19.7% less than the indicated Ka value. Conclusions Ophthalmic arterial melphalan infusion is a safe and effective means to treat RB. Modification to contemporary fluoroscopic systems combined with parsimonious fluoroscopy can minimize radiation exposure.

Segmental arterial mediolysis (SAM) is a rare, non-inflammatory, non-atherosclerotic vasculopathy typically affecting the abdominal arteries although it may also affect the great vessels and cerebral vasculature. Diseased vessels manifest with aneurysms and/or dissections, often presenting clinically with catastrophic thromboembolic injury and less frequently with subarachnoid hemorrhage (SAH). The etiology of SAM remains indeterminate although there is evidence it may be an endogenous pathological response to vasospasm. The SAM literature is reviewed and a case of SAH related to a ruptured dissecting-type vertebral artery aneurysm is described. In addition to furthering awareness of SAM, this unique case offers insight into the acute phase of the disease and the potential role of vasospastic induction.

Background and Purpose— We investigated whether brain arteriovenous malformation silent intralesional microhemorrhage, that is, asymptomatic bleeding in the nidal compartment, might serve as a marker for increased risk of symptomatic intracranial hemorrhage (ICH). We evaluated 2 markers to assess the occurrence of silent intralesional microhemorrhage: neuroradiological assessment of evidence of old hemorrhage—imaging evidence of bleeding before the outcome events–and hemosiderin positivity in hematoxylin and eosin-stained paraffin block sections. Methods— We identified cases from our brain arteriovenous malformation database with recorded neuroradiological data or available surgical paraffin blocks. Using 2 end points, index ICH or new ICH after diagnosis (censored at treatment, loss to follow-up, or death), we performed logistic or Cox regression to assess evidence of old hemorrhage and hemosiderin positivity adjusting for age, sex, deep-only venous drainage, maximal brain arteriovenous malformation size, deep location, and associated arterial aneurysms. Results— Evidence of old hemorrhage was present in 6.5% (n=975) of patients and highly predictive of index ICH ( P P =0.010). Hemosiderin positivity was found in 36.2% (29.6% in unruptured; 47.8% in ruptured; P =0.04) and associated with index ICH in univariate (OR, 2.18; 95% CI, 1.03–4.61; P =0.042; n=127) and multivariable models (OR, 3.64; 95% CI, 1.11–12.00; P =0.034; n=79). Conclusions— The prevalence of silent intralesional microhemorrhage is high and there is evidence for an association with both index and subsequent ICH. Further development of means to detect silent intralesional microhemorrhage during brain arteriovenous malformation evaluation may present an opportunity to improve risk stratification, especially for unruptured brain arteriovenous malformations.

BACKGROUND AND PURPOSE: Children with brain aneurysms may be at higher risk than adults to develop new or enlarging aneurysms in a relatively short time. We sought to identify comorbidities and angiographic features in children that predict new aneurysm formation or enlargement of untreated aneurysms. MATERIALS AND METHODS: Retrospective analysis of the University of California–San Francisco Pediatric Aneurysm Cohort data base including medical records and imaging studies was performed. RESULTS: Of 83 patients harboring 114 intracranial aneurysms not associated with brain arteriovenous malformations or intracranial arteriovenous fistulas, 9 (8.4%) developed new or enlarging brain aneurysms an average of 4.2 years after initial presentation. Comorbidities that may be related to aneurysm formation were significantly higher in patients who developed new aneurysms (89%) as opposed to patients who did not develop new or enlarging aneurysms (41%; RR, 9.5; 95% CI, 1.9%–48%; P = .0099). Patients with multiple aneurysms at initial presentation were more likely than patients with a single aneurysm at presentation to develop a new or enlarging aneurysm (RR, 6.2; 95% CI, 2.1%–185; P = .0058). Patients who initially presented with at least 1 fusiform aneurysm were more likely to develop a new or enlarging aneurysm than patients who did not present with a fusiform aneurysm (RR, 22; 95% CI, 3.6%–68%; P = .00050). Index aneurysm treatment with parent artery occlusion also was associated with higher risk of new aneurysm formation (RR, 4.2; 95% CI, 1.3%–13%; P = .024). New aneurysms did not necessarily arise near index aneurysms. The only fatality in the series was due to subarachnoid hemorrhage from a new posterior circulation aneurysm arising 20 months after index anterior circulation aneurysm treatment in an immunosuppressed patient. CONCLUSIONS: Patients who presented with a fusiform aneurysm had a significantly greater incidence of developing a new aneurysm or enlargement of an index aneurysm than did those who presented with a saccular aneurysm. In our patient cohort, 8 of the 9 children who eventually developed new or enlarging brain aneurysms initially presented with fusiform aneurysm morphology. Other comorbidities or multiple aneurysms were also common in these patients at initial presentation.

BACKGROUND AND PURPOSE: The natural history of PMAVFs, also known as type IV spinal cord AVFs, is incompletely understood. Both open surgical and endovascular approaches have been described as treatment modalities for this disease. The goal of this study was to evaluate the long-term outcome of patients with PMAVFs treated at a single tertiary care institution. MATERIALS AND METHODS: We conducted a retrospective study of 32 patients with PMAVFs, evaluated between 1983 and 2009. Data were gathered by reviewing outpatient clinic notes, operative and radiologic reports, and spinal angiograms. The PMAVFs were categorized into 1 of 3 types based on the angiographic imaging criteria. Pretreatment and posttreatment ambulation and micturition symptoms were quantified by using the ALS. RESULTS: Thirty patients underwent corrective procedures, 4 by embolization alone, 11 by surgery alone, and 15 with a combination of the 2. Twenty-eight patients underwent follow-up spinal angiography, with residual shunt noted in 6 patients. The mean follow-up period was 54 months (range, 1–228 months). Analysis of the ALS scores revealed that treatment of PMAVFs, independent of technique, resulted in significant improvement in ambulation but inconsistent changes in micturition. In addition, residual fistula at the time of the follow-up angiogram was associated with worsened neurologic status or lack of improvement. Outcome analysis based on fistula type showed dramatic improvement in ALS ambulation scores (62%) for type 3 fistulas, compared with types 1 and 2 (26% and 27%, respectively). CONCLUSIONS: Significant improvement in ambulation but in not micturition was observed following treatment. Residual fistula on follow-up angiography was associated with progressive worsening or lack of improvement in neurologic function. Patients with type 3 fistulas were shown to benefit most from treatment, with marked improvement in posttreatment ambulation scores. As endovascular and surgical techniques continue to evolve, further studies are warranted.

BACKGROUND AND PURPOSE: CV following aneurysmal SAH is a significant cause of morbidity and mortality. We review our experiences using PTA and IA verapamil infusion for treating medically refractory cases. MATERIALS AND METHODS: We performed a retrospective review of patients with SAH admitted from July 2003 to January 2008. RESULTS: Of 546 patients admitted within 72 hours of symptom onset, 231 patients (42%) developed symptomatic CV and 189 patients (35%) required endovascular therapy. A total of 346 endovascular sessions were performed consisting of 1 single angioplasty, 286 IA verapamil infusions, and 59 combined treatments. PTA was performed on 151 vessel segments, and IA verapamil was infused in 720 vessel segments. IA verapamil doses ranged from 2.0 to 30.0 mg per vessel segment and from 3.0 to 55.0 mg per treatment session. Repeat treatments were necessary in 102 patients (54%) for persistent, recurrent, or worsening CV. There were 6 treatment-related complications, of which 2 resulted in clinical worsening. No deaths were attributable to endovascular therapy. At follow-up, 115 patients (61%) had a good outcome and 55 patients (29%) had a poor outcome. Sixteen patients died from causes related to SAH, while 3 died from other medical complications. CONCLUSIONS: Endovascular treatments are an integral part of managing patients with medically refractory CV. In our experience, PTA and IA verapamil are safe, with a low complication rate, but further studies are required to determine appropriate patient selection and treatment efficacy.

A man aged 61 years with a history of a ruptured basilar fenestration aneurysm underwent unassisted coiling in 1997 and repeat intervention for a recurrence at the aneurysm mouth in 2011. At repeat intervention, the decision was made to intentionally leave some filling at the base to preserve the parent vessels. Stent-assisted coil embolization, although technically feasible, was not pursued given the relative risks of the procedure. In 2017, the patient returned for repeat surveillance and further coil compaction was found at the aneurysm base. With the advent of more compliant woven stents deliverable through 0.017 microcatheters, stent-assisted coiling was possible. This case demonstrates hereto unseen agility afforded by novel low-profile stents allowing a circumferential approach to a basilar artery fenestration aneurysm and resultant limb-to-limb stent-assisted coiling. Techniques described here may be extended to more common anatomic variants that require stent-assisted coiling.

BACKGROUND AND PURPOSE: Pediatric aneurysms are rare and, thus, relatively poorly understood as compared to those in adults. Our aim was to characterize the clinical, imaging, treatment, and outcome data of patients younger than 19 years diagnosed with intracranial aneurysms at a tertiary care institution. MATERIALS AND METHODS: We performed a retrospective medical record review of pediatric patients examined at our university hospital between 1981 and 2008. RESULTS: We evaluated 77 patients (mean age, 12 years; 40 female, 37 male) with 103 intracranial aneurysms. Patients presented with headache (45%), cranial neuropathies (16%), nausea/vomiting (15%), vision changes (13%), trauma (13%), seizure (4%), or sensory changes (3%). Subarachnoid hemorrhage occurred in 25 patients. Thirty-one fusiform aneurysms occurred in 25 patients. Forty-seven saccular aneurysms occurred in 35 patients. Twelve infectious aneurysms occurred in 6 patients. Fifteen traumatic aneurysms occurred in 12 patients. Fifty-nine patients underwent treatment of their aneurysms; 18 patients’ conditions were managed conservatively. Nineteen patients underwent primary endovascular coiling, 1 patient had endovascular stent-assisted coiling, 11 patients underwent endovascular parent artery occlusion, 19 patients underwent surgical clipping, and 10 patients had aneurysms trapped and bypassed. Mortality was 1.3%. Morbidity included 8% infarction and 4% new-onset seizures. Six patients developed new aneurysms or had enlargement of untreated aneurysms. CONCLUSIONS: In our experience, intracranial aneurysms of childhood show a female predilection and predominantly saccular morphology. In neurovascular centers where microneurosurgical and endovascular options are available, most children with intracranial aneurysms can be successfully treated with low morbidity and mortality. Fusiform aneurysms require a combined microneurosurgical and endovascular approach more often than saccular aneurysms. The development of new aneurysms in pediatric patients during limited follow-up warrants further investigation.

OBJECTIVE: Brain arteriovenous malformations (AVM) have high matrix metalloproteinase-9, interleukin-6, and myeloperoxidase (MPO) expression, and polymorphic variations in inflammatory genes are associated with an increased risk of hemorrhage. In this study, we characterized the presence of inflammatory cells in AVM lesional tissue specimens. METHODS: Immunohistochemistry was used to identify and localize neutrophils (MPO as marker), macrophages/microglia (CD68 as marker), T lymphocytes (CD3 as marker), and B lymphocytes (CD20 as marker). Endothelial cell (EC) marker CD31 was used as an index to assess vascular mass (EC mass). Surgical specimens from 20 unruptured, nonembolized AVMs were examined; seven cortical samples from temporal lobectomy were used as controls. Positive signals for inflammatory cell markers were counted and analyzed by normalizing to the area of the tissue section and the amount of endothelial cells (cells/mm 2 /EC mass pixels). Levels of MPO and matrix metalloproteinase 9 were determined by enzyme-linked immunosorbent assay. RESULTS: Neutrophils and macrophages are all frequently identified in the vascular wall of AVM tissue. In contrast, T and B lymphocytes are rarely observed in AVM tissue. AVM tissue displayed more neutrophil and macrophage/microglia markers than epilepsy control tissue (MPO: 434 ± 333 versus 5 ± 4, P = 0.0001; CD68: 454 ± 404 versus 4 ± 2, P = 0.0001; cells/mm 2 /EC mass pixels). In ex vivo studies, neutrophil quantity, MPO, and matrix metalloproteinase-9 levels were all colinear (R 2 = 0.98-0.99). CONCLUSION: Our study demonstrates that inflammatory cells are present in AVM tissue. Taken together with previous genetic and cytokine studies, these data are consistent with a novel view that inflammation is associated with AVM disease progression and rupture.

OBJECTIVE: The goals of this study were to characterize the clinical, radiological, treatment, and outcome data associated with patients diagnosed with spinal dural arteriovenous fistulae (SDAVFs) at a single tertiary care institution over a 20-year period. METHODS: A 20-year retrospective study was undertaken at our university hospital. Patients with mixed intracranial and spinal dural fistulas were excluded. A literature review of articles reporting endovascular or combined treatment of SDAVFs was performed. RESULTS: Between 1984 and 2005, our institution diagnosed and treated 63 patients (mean age, 62; 13 women, 50 men) with SDAVFs. The presenting symptoms were consistent with progressive myelopathy, and included lower extremity weakness (33 patients, 52%), parasthesias (19 patients, 30%), back pain (15 patients, 24%), and urinary symptoms (four patients, 6%). Thirty-nine patients underwent an initial endovascular embolization with 27 requiring only this first procedure for complete obliteration. On the other hand, 24 patients underwent an initial surgical procedure with 20 of them treated successfully with a single operation. Endovascular patients presented at mean age 62.3 years (standard deviation [SD], 10.6), were hospitalized for an average of 3.1 days (SD, 2.6), and were followed-up for 39 months (SD, 33). Surgical patients presented at mean age of 65.8 years (SD, 10.3), were hospitalized for 9.8 days (SD, 2.7), and were followed-up for 35 months (SD, 44). A significant improvement in Aminoff-Logue scores was found in both the endovascular and surgery groups (gait, P < 0.001; micturition, P = 0.005). The endovascular group had reduced hospitalization (P = 0.0001). No differences were found in the magnitude of clinical response to treatment. CONCLUSION: SDAVFs most commonly present with progressive myelopathy, yet often remain undiagnosed for months or years. Endovascular therapies and surgical therapies are associated with significantly improved symptoms once the definitive diagnosis of SDAVF is made.

OBJECTIVE: Diffuse arteriovenous malformations (AVM) have non-compact niduses, irregular margins, and intervening brain parenchyma. Deep perforating arteries often contribute to the ragged border of these diffuse AVMs. We hypothesized that diffuseness and deep perforator supply increase the difficulties and risks associated with microsurgical AVM resection. METHODS: Diffuseness was quantified using computer-generated outlines of AVMs on angiograms, contour plots with varying image intensities, and calculations of nidus area-intensity profiles. Diffuse AVMs had nonlinear area-intensity profiles with high transition intensities ([I*] greater than 0.5). A consecutive series of 304 patients who were treated with microsurgical AVM resection over a period of 7.8 years was analyzed, along with quantification of diffuseness in a subset of 103 consecutive patients. Neurological outcomes were assessed by using the Modified Rankin Scale, and logistic regression analysis was used to identify predictors of deterioration and poor outcome at late follow-up evaluation. RESULTS: Diffuse niduses were observed in 25% of patients, and 18% of patients had deep perforating artery supply. Patients with compact AVMs were more likely to have good outcomes or overall improvement (88 and 87%, respectively) than patients with diffuse AVMs (65 and 54%, respectively) (P = 0.008 and P< 0.001 / respectively), Similarly, absence of deep perforator supply was associated with good outcomes or improvement in 85 and 78% of patients, respectively, compared with 63 and 64% of patients, respectively; in patients with deep perforator supply (P< 0.001 and P= 0.028, respectively). By logistic regression analysis, diffuseness and deep perforator supply were both associated with significant increases in surgical risk. CONCLUSION: Diffuseness and deep perforating artery supply are subtle features of an AVM that predict worse outcomes after microsurgical resection. Diffuseness makes surgical planes more difficult to determine and follow, whereas deep perforators are friable, poorly visualized, and located in eloquent white matter tracts. The Spetzler-Martin grading scale does not directly account for these two features; however, they should be considered carefully when making treatment recommendations to patients with AVMs.

BackgroundVein of Galen malformations (VOGMs) are rare and complex congenital arteriovenous fistulas. The clinical and radiological features of VOGMs and their relation to clinical outcomes are not fully characterized.ObjectiveTo examine the clinical and radiological features of VOGMs and the predictors of outcome in patients.MethodsWe retrospectively reviewed the available imaging and medical records of all patients with VOGMs treated at the University of California, San Francisco between 1986 and 2013. Radiological and clinical features were identified. We applied the modified Rankin Scale to determine functional outcome by chart review. Predictors of outcome were assessed by χ(2) analyses.ResultsForty-one cases were confirmed as VOGM. Most patients (78%) had been diagnosed with VOGM in the first year of life. Age at treatment was bimodally distributed, with predominantly urgent embolization at

Background and purposeThe proper role of endovascular treatment of cervicocerebral atherosclerosis is unclear. Posterior circulation disease has not been investigated as extensively as disease in the anterior circulation. In this study, we characterized the rates of technical success, transient ischemic attack, stroke, and death or disability, for both acute and elective endovascular treatment of atherosclerosis in the vertebrobasilar system.MethodsWe identified patients with atherosclerosis of the vertebrobasilar circulation who underwent endovascular intervention at our hospital through retrospective medical record review, and evaluated the association between lesion and treatment features and subsequent stroke, death, or disability at 30 days and 1 year.ResultsWe identified 136 lesions in 122 patients, including 13 interventions for acute strokes. Technical success was achieved in 123 of 136 cases (90.4%). Elective procedures had higher rates of technical success (6.5% vs 15.4%, p=0.21) and better clinical outcomes. In multivariate analysis, intracranial lesions were associated with more disability (modified Rankin Scale score >2) at 30 days (OR 7.1, p=0.01) and 1 year (OR 10, p=0.03). Patients with non-hypoperfusion related symptoms had fewer strokes at follow-up at 1 year when treated after an asymptomatic interval of >10 days compared with those treated within 10 days of the presenting symptoms (OR 0.2, p=0.03). Statin treatment prior to intervention was associated with favorable outcomes across several examined endpoints. Preoperative antiplatelet treatment was associated with lower rates of disability at 30 days and 1 year (OR 0.1, pConclusionsEndovascular treatment of symptomatic vertebrobasilar atherosclerosis can be performed safely and with good outcomes. Technical results were better for those with extracranial disease while clinical outcomes were more favorable in those patients with non-progressive symptoms in the subacute period and those receiving statin therapy.

ACTA2 mutations have recently been shown to cause a multisystem smooth muscle dysfunction syndrome that may result in pediatric stroke. We report a case of ACTA2 mutation in a 3-year-old girl presenting with acute ischemic stroke and provide high resolution imaging of the cerebral arteries demonstrating novel findings of multiple tiny aneurysms (particularly in the posterior circulation), as well as the more characteristic imaging phenotype of straightened and narrowed proximal intracranial vessels, dilated cervical vessels and occlusion of the M1 MCA segment without lenticulostriate collateral formation. This newly identified disease should be added to the differential diagnosis of pediatric stroke and cerebral vasculopathy. Neuroradiologists, interventionalists, surgeons and neurologists should become familiar with this rare disease and its clinical sequelae.

OBJECTIVE Despite its quantitative definitions, the Spetzler-Martin grading scale for brain arteriovenous malformations (AVMs) is subject to interobserver variability, particularly when observers differ in their subspecialties. Interobserver variability between neuroradiologist and neurosurgeon grading was analyzed in a large AVM series to determine its extent, causes, and clinical implications. METHODS In a consecutive surgical series of 224 AVM patients, Spetzler-Martin grades were assigned independently by a neuroradiologist and a neurosurgeon. Interobserver agreement was measured with a Cohen kappa analysis and the Wilcoxon signed-rank test. RESULTS Disagreement in grades occurred in 62 (27.7%) patients. By kappa analysis, agreement was highest for venous drainage pattern (kappa = 0.90), intermediate for eloquence (kappa = 0.71), and lowest for size (kappa = 0.67), with substantial agreement on overall grade (kappa = 0.61). By Wilcoxon signed-rank test, size scores and AVM grades were significantly different. Causes of interobserver variability in grading included diffuse nidus, differences in angiographic versus surgical borders, paradoxical venous drainage, and the ambiguity of eloquence. Interobserver variability affected outcome data but did not diminish the predictive value of the Spetzler-Martin scale. CONCLUSION The Spetzler-Martin grading system can be applied reliably to most AVMs with good agreement between observers, but some unusual AVMs expose the system's imprecision and subjectivity. Interobserver variability can affect reporting of results, surgical risk assessment, and patient selection. Undergrading may encourage borderline surgical candidates to choose surgery and obtain results below their expectations.

OBJECTIVE: We hypothesized that coexisting extranidal arterial aneurysms (EAs) would be associated with an increased risk of incident intracranial hemorrhage (ICH) from brain arteriovenous malformation (BAVM) rupture. METHODS: To determine the presence of EAs and compare the sources and locations of ICH, we retrospectively reviewed the computed tomographic, magnetic resonance imaging, and angiographic studies of patients who presented between 1990 and 1999. EAs were defined as saccular luminal dilations of the parent feeding vessels that were proximally flow-related (i.e., at the circle of Willis), distally flow-related (i.e., distal to the circle of Willis), and unrelated (i.e., in circulation distant from the BAVM). RESULTS: Of 314 BAVM patients, 138 (44%) presented with ICH. In the ICH group, 22 patients (16%) had aneurysmal ICH, 100 (72%) had BAVM ICH, and 16 (12%) had ICH from an indeterminate source. There were 61 patients with 1 or more EAs (29 patients with 42 flow-related proximal aneurysms, 39 patients with 48 flow-related distal aneurysms, and 10 patients with 20 unrelated aneurysms). Multivariate regression analysis revealed that ICH patients were more likely than non-ICH patients to have a coexisting EA (35 versus 13%; odds ratio = 3.9; 95% confidence interval, 2.1-7.5; P < 0.001), but this effect was not present when only BAVM-related ICH was considered (odds ratio = 0,3; 95% confidence interval, 0.1-1.0; P = 0.052). Other independent predictors of ICH included small AVM size (

We report a case of fatal subarachnoid hemorrhage from nontraumatic rupture of an aneurysm at the basilar terminus in which both computed tomography angiography and conventional angiography showed evidence of active bleeding. The time period from initial ictus to CT angiography was 30-50 minutes and to conventional angiography was 120-140 minutes. This case illustrates that aneurysmal bleeding is not necessarily as brief as a few seconds and can last up to 30 to 50 minutes and perhaps longer. Continued bleeding from an intracranial aneurysm is a rare event that can be recognized using computed tomography angiography and likely indicates a poor prognosis.

Introduction/purpose To best achieve complete occlusion of aneurysms treated with flow diverters (FDs), devices must be accurately sized to precisely match the parent vessel diameter. Appropriately sized FDs will have maximal coverage over the aneurysm neck and will be more likely to induce thrombosis. Catheterization for diagnostic angiography can cause vasospasm that may affect vessel measurements. This study evaluates the effects of intra-arterial infusion of a calcium channel blocker (CCB) on angiographic measurements in patients treated with FDs, investigating the impact on the final diameter of the expanded stent following deployment and subsequent occlusion. Materials and methods Retrospective analysis was performed of prospectively maintained procedure records to identify patients with aneurysms treated with FDs. In all patients, pre-treatment measurements were recorded for vessel diameter of the distal and proximal landing zones, as well as maximum and minimum diameters between these segments. Post-treatment measurements of the stent itself following deployment were recorded at the distal and proximal ends in addition to maximum and minimum diameters. When CCB was infused prior to treatment, post-infusion pre-treatment measurements were recorded. All measurements were performed after calibrating to a catheter included on the image. Rates of occlusion and time to confirmed occlusion were noted for all patients. Independent-sample 2 tailed T-tests were performed to assess for differences in pre- and post-treatment measurements, rates of occlusion, and time to confirmed occlusion between groups with and without CCB infusion. Results 21 FDs were deployed to treat 19 aneurysms in 18 patients. CCB infusion was performed prior to deployment of 9 devices. The change in measurements noted after CCB infusion are summarized in Table 1. No significant difference was noted between groups for pre- and post-treatment measurement changes. Subsequent aneurysm occlusion was more likely to occur in the CCB infusion group (100% vs. 44.4%, p Conclusion Optimization of device sizing is important to increase FD density over the aneurysm neck and promote thrombosis. To improve accuracy of measurements of parent vessels prior to device selection, CCB infusion can reduce the effects of mild vasospasm. In this study, subsequent aneurysm occlusion was more likely to occur following FD treatment when device size selection was based on measurements performed following CCB infusion. Disclosures M. Alexander: None. R. Darflinger: None. F. Settecase: None. A. Nicholson: None. D. Cooke: None. M. Amans: None. S. Hetts: None. C. Dowd: None. R. Higashida: None. V. Halbach: None.

OBJECTIVE The most common location for dural arteriovenous fistulae (AVFs) is the transverse/sigmoid sinus. We describe our prospective analysis of data for 10 patients with recipient fistulae parallel to the transverse/sigmoid sinus. Recognition of this entity allows embolization of the fistula with preservation of the parent sinus. This report reviews the presentation and angiographic characteristics of the “parallel venous channel” and the treatment results for this series of patients. METHODS Between 1995 and June 2002, at the medical center of the University of California, San Francisco, we identified 10 patients with a parallel venous channel as the recipient pouch for all arterial input into a transverse/sigmoid sinus AVF. The clinical presentations, angiographic features, endovascular treatments, and outcomes are described. Angiographic follow-up monitoring was performed for 1 to 6 years for all patients with cortical venous drainage (5 of 10 patients). Clinical follow-up periods ranged from 1 to 7 years. RESULTS All patients presented with pulsatile tinnitus disruptive to sleep. Other symptoms included severe headaches, papilledema and visual disturbances, hemiparesis, and mastoid pain. All 10 parallel venous channels communicated with the transverse or sigmoid sinus. Cortical venous drainage was present in 50% of cases. Endovascular ablative procedures, using either coils or ethanol, were performed for all patients. The parallel venous channel was successfully embolized, with preservation of the transverse/sigmoid sinus, for all 10 patients. There were no major complications. All patients experienced resolution of their symptoms, with no recurrence. CONCLUSION The existence of a parallel venous channel as the recipient pouch for all arterial inflow in a series of 10 transverse/sigmoid sinus AVFs is described. Endovascular obliteration of the parallel channel, with preservation of the parent sinus, was successfully performed for all 10 patients. Recognition of the parallel venous channel is clinically important for the treatment of transverse/sigmoid AVFs.

OBJECTIVE To determine the usefulness of endovascular embolization for treatment of dural arteriovenous fistulae of the superior petrosal sinus. METHODS We performed a retrospective review of 18 patients treated during a 16-year period. Transarterial and/or transvenous embolizations were performed as a preoperative adjunct or definitive therapy. Clinical follow-up status was supplemented by telephone interviews to determine Glasgow Outcome Scale scores. RESULTS Fourteen patients (78%) were treated with a combination of endovascular therapy and open surgery, and 4 were treated by embolization alone (22%). Angiographic cure was achieved in all patients (100%). Thirty-day morbidity and mortality were 11 and 0%, respectively. The mean follow-up period was 5.4 years. At the latest follow-up examination, all patients had returned to independent clinical status (Glasgow Outcome Scale scores of 1 or 2). CONCLUSION Endovascular treatment of dural arteriovenous fistulae of the superior petrosal sinus can result in cure when access to the site of the fistula can be achieved. Preoperative embolization is a safe and effective adjunct to minimize bleeding during open neurosurgery.