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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.

Activating variants in the platelet-derived growth factor receptor β gene (PDGFRB) have been associated with Kosaki overgrowth syndrome, infantile myofibromatosis, and Penttinen premature aging syndrome. A recently described phenotype with fusiform aneurysm has been associated with mosaic PDGFRB c.1685A > G p.(Tyr562Cys) variant. Few reports however have examined the vascular phenotypes and mosaic effects of PDGFRB variants. We describe clinical characteristics of two patients with a recurrent mosaic PDGFRB p.(Tyr562Cys) variant identified via next-generation sequencing-based genetic testing. We observed intracranial fusiform aneurysm in one patient and found an additional eight patients with aneurysms and phenotypes associated with PDGFRB-activating variants through literature search. The conditions caused by PDGFRB-activating variants share overlapping features including overgrowth, premature aged skin, and vascular malformations including aneurysms. Aneurysms are progressive and can result in morbidities and mortalities in the absence of successful intervention. Germline and/or somatic testing for PDGFRB gene should be obtained when PDGFRB activating variant-related phenotypes are present. Whole-body imaging of the arterial tree and echocardiography are recommended after diagnosis. Repeating the imaging study within a 6- to 12-month period after detection is reasonable. Finally, further evaluation for the effectiveness and safety profile of kinase inhibitors in this patient population is warranted.

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 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 Giant cell arteritis (GCA) is a systemic vasculitis that causes ischemic stroke in 2-7% of patients, typically due to extracranial vessel inflammation. However, in rare instances, GCA can present with fulminant intracranial stenoses which are refractory to medical therapy. In these cases, endovascular treatment (EVT) is a possible rescue strategy to prevent life-threatening ischemic complications, but the safety and efficacy of EVT in this setting are not well-described. Methods A systematic literature review was performed according to PRISMA-IPD guidelines 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 Nine reports comprising 19 treatments (percutaneous transluminal angioplasty, PTA, with or without stenting) in 14 patients (mean age 69.6 ± 6.3 years, 63.4% women) were identified in the literature. 66.7% of patients had >1 pre-existing cardiovascular risk factor. All patients had infarction on MRI while on corticosteroids and 50% progressed despite adjuvant immunosuppressive agents. Treatment was PTA alone in 78.9% of cases and PTA + stenting in 21.1%. Repeat treatments were necessary for 28.6% of patients (100% PTA-only). Non-flow limiting dissection was reported in 10.5% of treatments. The technical details of a single patient with aggressive, FDG-PET avid intracranial GCA who was treated with multiple EVT are discussed. Adequate restoration of cerebral blood flow was achieved in 100% of interventions and complications included one non-flow limiting dissection (12.5%) and two small, delayed, reperfusion hemorrhages (25%). The authors also report the novel use of intra-arterial calcium-channel blocker infusion (verapamil) as an adjuvant to PTA and as monotherapy, which resulted in immediate improvement in cerebral blood flow (figure 1). Conclusions Endovascular treatment, including PTA (with or without stenting) and CCB infusion, may be effective in medically-refractory GCA with intracranial arterial stenosis but complication rates are considerable. The efficacy of CCB monotherapy implicates vascular smooth muscle dysfunction in the pathogenesis of intracranial GCA. Calcium-channel blocker infusion as monotherapy for intracranial giant cell arteritis. Pre-treatment angiography (lateral right internal carotid artery projection) shows severe focal supraclinoid ICA stenosis (curved white arrow, 1A). Post-verapamil infusion (20mg, 15 min delay) angiogram (1B) shows significant improvement in lumen diameter. Four-dimensional digital subtraction angiography (not shown) confirmed normalization of time-to-peak in the petrous ICA from 4.5s to 1.0s after verapamil infusion. Disclosures M. Caton: None. I. Mark: None. A. Baker: None. K. Narsinh: None. V. Halbach: None. S. Hetts: None. D. Cooke: None. R. Higashida: None. C. Dowd: None. W. Smith: None. M. Amans: None.

Background and Purpose Intra-arterial chemotherapy (IAC) infusion for the treatment of intraocular retinoblastoma is an effective and relatively safe therapeutic option.1–3 However, one of the well described systemic complications following IAC is neutropenia.4 The purpose of this retrospective study is to examine the incidence of neutropenia over the course of treatment for each patient, and to further analyze if the incidence is affected by systemic chemotherapy or number of chemotherapeutic agents used for IAC. Methods We retrospectively identified 76 patients from a neurointerventional database spanning 10 years (March 2010 - August 2020) who underwent 214 cycles of IAC. Patient demographics, treatment course, angiographic technique as well as pre and post procedural complete blood counts were extracted from the medical record. Neutropenia, including all grades I-IV, is defined by an absolute neutrophil count (ANC) equal to or less than 1.5 x 10(9)/L. Eleven patients who did not obtain a post-procedural complete blood count during their treatment course were excluded. The presence of postprocedural neutropenia was further subdivided based on whether systemic intravenous chemotherapy was administered as well as according to the number of intra-arterial chemotherapeutic agents. Results The overall incidence of neutropenia was 62% in our total patient cohort. There was no statistically significant difference between the incidence of neutropenia in patients who did and did not receive systemic chemotherapy. The majority of patients received two chemotherapeutic agents (melphalan and topotecan), of which 58% and 54% of patients experienced neutropenia with and without systemic chemotherapy, respectively. The highest incidence of neutropenia was 100%, in five patients who received systemic chemotherapy and a three agent intra-arterial regimen (melphalan, topotecan, carboplatin). Conclusion Neutropenia is a systemic toxicity in more than half of patients who receive IAC infusion for intraocular retinoblastoma, and does not significantly differ in patients who do and do not receive systemic chemotherapy. References Ammanuel S, Alexander MD, Damato B, et al. Improved procedural safety following protocol changes for selective ophthalmic arterial infusion of chemotherapy for treatment of ocular retinoblastoma. Interv Neuroradiol. 2018;24(3):345–350. Yousef YA, Soliman SE, Astudillo PPP, et al. Intra-arterial Chemotherapy for Retinoblastoma: A Systematic Review. JAMA Ophthalmol. 2016;134(5):584–591. Monroy JE, Orbach DB, VanderVeen D. Complications of intra-arterial chemotherapy for retinoblastoma. Semin Ophthalmol. 2014;29(5–6):429–433. Dunkel IJ, Shi W, Salvaggio K, et al. Risk factors for severe neutropenia following intra-arterial chemotherapy for intra-ocular retinoblastoma. PLoS One. 2014;9(10):e108692. Disclosures A. Baker: None. S. Ammanuel: None. M. Caton: None. K. Narsinh: None. A. Ashfar: None. A. Banerjee: None. D. Cooke: None. C. Dowd: None. M. Amans: None. V. Halbach: None. R. Higashida: None. S. Hetts: None.

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.

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.

Background and purpose The purpose of this article is to estimate the distribution of superselective intra-arterial chemotherapy (IAC) delivery to ocular target tissue using quantitative digital subtraction angiography (qDSA). Materials and methods From March 2010 to January 2016, 50 ophthalmic artery contrast DSAs obtained immediately prior to IAC infusions in 22 patients were analyzed. This study was conducted under a retrospective review IRB (no. 10-01862). Parametric color-coded DSAs (iFlow, Siemens Medical) were post-processed (MATLAB, The Mathworks Inc.) using two methods: two box regions of interest (pre-retina and globe) and four custom regions of interest (ROIs—ophthalmic artery, choroid, supraclinoid internal carotid artery (ICA), cavernous ICA). Mean interobserver reliability of custom ROI selection is presented as a 95% confidence interval of interclass correlation, and fractional chemotherapy delivery to selected ROIs as means ± standard deviation in this study. Results The estimated fraction of chemotherapy delivered to the globe with the first method was 79.5%. Percentage regional delivery using the second method was as follows: ophthalmic artery, 85.8%; choroid, 60.5%; supraclinoid ICA, 14.2%. The cavernous ICA ROI (encompassing distal catheter and potential reflux) gave a signal equivalent to 9.3% of total delivery. Conclusion Parametric color-coded qDSA can estimate the fraction of IAC delivered to the retina and other orbital structures in ocular retinoblastoma patients. This information can inform delivery location and dosing strategies on a patient-specific basis.

Purpose Earlier reperfusion of large-vessel occlusion (LVO) stroke improves functional outcomes. We hypothesize that use of a stroke cart in the angiography suite, containing all commonly used procedural equipment in a mechanical thrombectomy, combined with parallel staff workflows, and use of conscious sedation when possible, improve mechanical thrombectomy time metrics. Methods We identified 47 consecutive LVO patients who underwent mechanical thrombectomy at our center, retrospectively and prospectively from implementation of these three workflow changes (19 pre- and 28 post-). For each patient, last known normal, NIHSS, angiography suite in-room time, type of anesthesia, groin puncture time, on-clot time, recanalization time, LVO location, number of passes, device(s) used, mTICI score, and outcome (mRS) were recorded. Between-group comparisons of time metrics and multivariate regression were performed. Results Stroke cart, parallel workflows, and primary use of conscious sedation decreased in-room time to groin puncture (−21.3 min, p Conclusions Use of a stroke cart, parallel workflows by neurointerventionalists, technologists, and nursing staff, and use of conscious sedation may be useful to other institutions in efforts to improve procedural times.

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

A 47-year-old woman presented with a right cervical mass that had been slowly enlarging for 6 years, causing pulsatile tinnitus and dizziness with compression. Computed tomography angiography demonstrated a partially thrombosed 8-cm right internal carotid artery aneurysm with a coexisting arteriovenous fistula. Conventional angiography demonstrated a vertebrojugular fistula (right vertebral artery to right internal jugular vein) and retrograde flow in the right vertebral artery to the fistula. Successful repair was performed in a staged fashion: operative repair of the internal carotid artery aneurysm with interposition bypass, followed by endovascular embolization of the vertebrojugular fistula through a bilateral vertebral artery approach.

OBJECTIVE Intracranial dural arteriovenous fistulas (DAVFs) are rare in children. This study sought to better characterize DAVF presentation, angioarchitecture, and treatment outcomes. METHODS Children with intracranial DAVFs between 1986 and 2013 were retrospectively identified from the neurointerventional database at the authors' institution. Demographics, clinical presentation, lesion angioarchitecture, treatment approaches, angiographic outcomes, and clinical outcomes were assessed. RESULTS DAVFs constituted 5.7% (22/423) of pediatric intracranial arteriovenous shunting lesions. Twelve boys and 10 girls presented between 1 day and 18 years of age; boys presented at a median of 1.3 years and girls presented at a median of 4.9 years. Four of 8 patients ≤ 1 year of age presented with congestive heart failure compared with 0/14 patients > 1 year of age (p = 0.01). Five of 8 patients ≤ 1 year old presented with respiratory distress compared with 0/14 patients > 1 year old (p = 0.0021). Ten of 14 patients > 1 year old presented with focal neurological deficits compared with 0/8 patients ≤ 1 year old (p = 0.0017). At initial angiography, 16 patients harbored a single intracranial DAVF and 6 patients had 2–6 DAVFs. Eight patients (38%) experienced DAVF obliteration by the end of treatment. Good clinical outcome (modified Rankin Scale score 0–2) was documented in 77% of patients > 1 year old at presentation compared with 57% of patients ≤ 1 year old at presentation. Six patients (27%) died. CONCLUSIONS Young children with DAVFs presented predominantly with cardiopulmonary symptoms, while older children presented with focal neurological deficits. Compared with other pediatric vascular shunts, DAVFs had lower rates of angiographic obliteration and poorer clinical outcomes.

The authors describe, for the first time to their knowledge, a case of a congenital macrocystic lymphatic malformation of the orbit with associated venous stasis retinopathy that acutely normalized after drainage and sclerotherapy of the lesion. Prenatal ultrasound revealed prominence of the left orbital soft tissue, and at birth, the patient was noted to have unilateral proptosis, tortuous retinal vessels, and intraretinal hemorrhages in all 4 quadrants in the left eye. MRI demonstrated a primarily intraconal, multiloculated, T2-hyperintense mass consistent with a lymphatic malformation. Ultrasound-guided cyst aspiration and sclerotherapy was performed, with subsequent improvement of the proptosis and resolution of the vessel tortuosity and intraretinal hemorrhages. Although venous stasis retinopathy is usually related to central retinal vein occlusion or carotid artery occlusive disease, any entity that increases orbital venous resistance can generate retinal venous dilation and intraretinal hemorrhages, including an orbital lymphatic malformation.

Background and Purpose Automated computed tomography perfusion (CTP) software is increasingly utilized to aid in treatment decisions in emergent large vessel occlusion (ELVO), particularly in extended window stroke patients. Neurointerventionalists must be cognizant of pitfalls of these software packages in predicting ischemic core and penumbra. This study aims to assess whether RAPID post-processing software underestimates core infarct in patients who have recently received intravenous iodinated contrast, a common scenario in stroke transfer patients who undergo CTA at an outside hospital to confirm ELVO prior to transfer. Methods We retrospectively reviewed 271 consecutive patients at our institution who underwent CTA ± CTP for symptoms of acute ischemic stroke (AIS) from May 2018 through January 2019. Patient exclusion was predominantly on the basis of lack of anterior circulation ELVO on CTA or lack of performance of CTP with RAPID post-processing. Two blinded raters independently assessed CT Alberta Stroke Program Early Computed Tomography Score (ASPECTS) on the non-enhanced head CT (NECT) at the time of CTP and also collected clinical as well imaging data, including time from last known well (LKW) to CTP, collaterals grade, and core infarct (mL) as predicted by RAPID software. Patients were dichotomized into those who received recent intravenous contrast (within 12 hours of CTP) and contrast naive patients. Correlation between ASPECTS and core infarct (mL) on RAPID software were analyzed for the two cohorts. Results A total of 48 patients with a mean age of 74.1 years (SD 16.4) were included. The two cohorts comprised 24 patients each. There were baseline differences in mean ASPECTS (8.5 vs 5.6, p Conclusion Patients that previously received contrast had a much higher likelihood of underestimation of ischemic core using RAPID than contrast naive patients. Those involved in the care of ELVO patients should avoid overreliance on RAPID post-processing for treatment disposition, particularly if the patient has received recent intravenous contrast. Disclosures A. Copelan: None. G. Drocton: None. E. Smith: None. R. Khangura: None. D. Murph: None. C. Dowd: None. V. Halbach: None. R. Higashida: None. S. Hetts: None. D. Cooke: None. M. Amans: None.

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.

Purpose The aim was to evaluate the impact of protocol changes in selective ophthalmic arterial infusion (SOAI) for treatment of retinoblastoma (Rb). Methods A retrospective review was completed of 35 patients with Rb who were treated with SOAI between March 2010 and January 2017. Treatment details were tabulated for each SOAI session. SOAI protocol was changed in June 2015, and differences before and after this change were evaluated using two-tail chi-square tests and independent sample t-tests to note any differences in technical complications, need for enucleation, and other outcome variables Results 125 SOAI sessions occurred. No technical complications occurred during the study. Two complications (1.6%) occurred in the postoperative setting. Both complications occurred prior to the change in protocol. Comparing the complication rates between the two protocols showed no significant difference (2.2% versus 0.0%; p = 0.505); 29 of 43 (67.4%) eyes had their vision preserved overall. Conclusions SOAI is an effective treatment for Rb. The refined protocol described herein was associated with fewer complications.

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.

OBJECT Although the development and prevalence of cerebral vasospasm (CV) has been extensively investigated in adults, little data exist on the development of CV in children. The authors hypothesized that even though children have highly vasoreactive arteries, because of a robust cerebral collateral blood flow, they rarely develop symptomatic CV. METHODS The authors retrospectively reviewed their university hospital's neurointerventional database for children (that is, patients ≤ 18 years) who were examined or treated for aneurysmal or traumatic subarachnoid hemorrhage (SAH) during the period 1990–2013. Images from digital subtraction angiography (DSA) were analyzed for the extent of CV and collateralization of the cerebral circulation. Results from transcranial Doppler (TCD) ultrasonography were correlated with those from DSA. Cerebral vasospasm on TCD ultrasonography was defined according to criteria developed for adults. Clinical outcomes of CV were assessed with the pediatric modified Rankin Scale (mRS). RESULTS Among 37 children (21 boys and 16 girls ranging in age from 8 months to 18 years) showing symptoms of an aneurysmal SAH (comprising 32 aneurysms and 5 traumatic pseudoaneurysms), 17 (46%) had CV confirmed by DSA; CV was mild in 21% of these children, moderate in 50%, and severe in 29%. Only 3 children exhibited symptomatic CV, all of whom had poor collateralization of cerebral vessels. Among the 14 asymptomatic children, 10 (71%) showed some degree of vessel collateralization. Among 16 children for whom TCD data were available that could be correlated with the DSA findings, 13 (81%) had CV according to TCD criteria. The sensitivity and specificity of TCD ultrasonography for diagnosing CV were 95% and 59%, respectively. The time to CV onset detected by TCD ultrasonography was 5 ± 3 days (range 2–10 days). Twenty-five (68%) of the children had good long-term outcomes (that is, had mRS scores of 0–2). CONCLUSIONS Children have a relatively high incidence of angiographically detectable, moderate-to-severe CV. Children rarely develop symptomatic CV and have good long-term outcomes, perhaps due to robust cerebral collateral blood flow. Criteria developed for detecting CV with TCD ultrasonography in adults overestimate the prevalence of CV in children. Larger studies are needed to define TCD ultrasonography–based CV criteria for children.

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.

Introduction/Purpose To achieve aneurysm occlusion, flow diverters (FDs) must be accurately sized to maximize coverage over the neck and induce thrombosis. Catheterization for diagnostic angiography can cause vasospasm that may affect vessel measurements. This study evaluates impacts of intra-arterial infusion of a calcium channel blocker (CCB) on angiographic measurements in patients treated with FDs to determine effects on final diameter of the FD and subsequent occlusion. Materials and methods Pre-treatment measurements were recorded for diameter of the distal and proximal landing zones and maximum and minimum diameters between these segments. Post-treatment measurements of the stent following deployment were recorded at these locations. When CCB was infused, post-infusion pre-treatment measurements were recorded. Rates of occlusion were noted for all patients. T-tests were performed to assess for differences in pre- and post-treatment measurements and rates of occlusion between groups with and without CCB infusion. Results Twenty-eight FDs were deployed to treat 25 aneurysms in 24 patients. CCB infusion was performed prior to deployment of 12 (42.9%) devices. No significant difference was noted between groups for pre- and post-treatment measurement changes. Confirmed aneurysm occlusion was more likely to occur in the CCB infusion group (88.9% vs. 36.4%, p = 0.009). Conclusion Optimization of device sizing is important to increase FD density over the aneurysm neck and promote thrombosis. To improve measurement accuracy, CCB infusion can reduce effects of mild vasospasm. Subsequent aneurysm occlusion was more likely to occur following FD treatment when device size selection was based on measurements performed following CCB infusion.

Venous malformations (VMs) are congenital anomalies of the venous vasculature, but not all are evident at birth. The factors that lead to presentation later in life are not well understood. The objective of this retrospective cohort study of patients with VMs evaluated at the University of California at San Francisco Birthmarks and Vascular Anomalies Center from 2005 to 2009 was to investigate the clinical presentation of VMs and correlate these features with different types of tissues (e.g., skin, subcutis, intramuscular). Main outcomes included the age at which lesions were first noticed, tissue type involved, presenting signs and symptoms, aggravating factors, and morbidities. A total of 115 subjects was included. The mean age when VM was first noted was 6.7 ± 0.9 years. Tissue types involved included skin/subcutaneous (46%); intramuscular (40%); and bone, tendon, or joint (14%). Presenting signs/symptoms included soft tissue swelling (44%), discrete mass (34%), pain (33%), and skin discoloration (26%). When compared with VMs limited to the skin or subcutis, those restricted to the intramuscular compartment were less likely to present at birth (27% vs 53%, p < 0.05) but were more frequently painful (79% vs 60%, p < 0.05) and contained more phleboliths (28% vs 11%, p < 0.05), and were associated with more exercise limitation (35% vs 16%, p < 0.05). VMs differ in age of onset, clinical features, and complications based on differing tissues and sites of involvement, with isolated intramuscular involvement associated with later presentation and greater morbidity.

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.

Object Spinal dural arteriovenous fistulas (SDAVFs) cause myelopathy through arterialization of the perimedullary venous plexus and venous congestion of the spinal cord. The authors hypothesized that the craniocaudal extent of engorgement of intrathecal draining veins between the fistula site and the point of drainage out of the thecal sac correlates with the degree of myelopathy. Methods A retrospective review of the authors' institution's radiology databases identified 31 patients with SDAVFs who had undergone digital subtraction angiography (DSA) and MRI examinations of the spine. The authors counted the number of vertebral body levels of spinal cord enhancement and intrathecal vessel enhancement on T1-weighted postcontrast MRI studies. They also counted the number of levels of cord hyperintensity and intrathecal flow voids on T2-weighted MRI studies. On DSA, the authors identified the number of vertebral body levels of dilated intrathecal draining veins and outflow points from intrathecal veins to epidural veins. Functional status of the patients at the time of diagnosis was assessed using the Aminoff-Logue scale (ALS). Results Enlargement of the intrathecal draining veins averaged 10 ± 7.7 spinal levels on DSA. Patients with enlarged draining veins extending 10 or more spinal levels on DSA had worse ALS scores (mean gait 3.4, mean micturition 1.5) than patients with draining veins extending fewer than 10 levels (mean gait 1.8, mean micturition 0.6; p = 0.009 and 0.02, respectively). The number of vertebral body levels of enlarged draining veins correlated with the ALS score (gait r = 0.42, p = 0.009; and micturition r = 0.55, p = 0.0006). More extensive enlarged draining veins were associated with more spinal cord T2 hyperintensity, T2 intrathecal flow voids, and T1 vessel enhancement but not cord enhancement. Conclusions The craniocaudal extent of enlarged intrathecal veins draining SDAVF correlates with patient functional status, providing further insight into the pathophysiology of venous hypertensive myelopathy.

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.

A 13-year-old boy with meningiomatosis, McCune-Albright syndrome, and gray platelet syndrome presented with an enlarging “lump” on his right forehead. A head CT scan revealed a polyostotic fibrous dysplasia involving the entire skull. A 3.4-cm right frontal osseous cavity and an overlying right forehead subcutaneous soft-tissue mass were seen, measuring 5.2 cm in diameter and 1.6 cm thick. Ultrasound of the cavity and overlying mass showed swirling of blood and an arterialized waveform. MRI revealed an en plaque meningioma underlying the cavity. An intraosseous pseudoaneurysm fed by 3 distal anterior division branches of the right middle meningeal artery (MMA) with contrast extravasation was found on angiography. Two MMA feeders were embolized with Onyx, with anterograde filling of the intraosseous cavity with Onyx. A small pocket of residual intracavity contrast filling postembolization from a smaller third MMA feeder eventually thrombosed and the forehead lump regressed.

© 2015, Springer-Verlag Berlin Heidelberg. Background: The incidence of localized intravascular coagulopathy (LIC) in venous malformations varies with lesion size and location, as well as the presence of palpable phleboliths. The development of LIC can cause pain and hemorrhage and can progress to disseminated intravascular coagulopathy (DIC) and thromboembolic disease resulting in death in some cases. Early recognition of LIC can relieve symptoms and prevent progression to life-threatening complications. Objective: The aim of this work was to identify MRI features of venous malformation associated with LIC. We hypothesized that venous malformations with larger capacitance, slower flow and less physiological compression (greater stasis) were more likely to be associated with LIC. Materials and methods: In this HIPAA-compliant and IRB-approved study, we retrospectively reviewed clinical records and MRI for consecutive patients undergoing evaluation of venous malformations at our multidisciplinary Birthmarks and Vascular Anomalies Center between 2003 and 2013. Inclusion required consensus diagnosis of venous malformation and availability of laboratory data and MRI; patients on anticoagulation or those previously undergoing surgical or endovascular treatment were excluded. LIC was diagnosed when D-dimer exceeded 1,000 ng/mL and/or fibrinogen was less than 200 mg/dL. Two board-certified radiologists assessed the following MRI features for each lesion: morphology (spongiform vs. phlebectatic), presence of phleboliths, size, location (truncal vs. extremity), and tissue type(s) involved (subcutis, muscle, bone and viscera). Univariate logistic regression analyses were used to test associations between LIC and MRI findings, and stepwise regression was applied to assess the significance of the individual imaging predictors. Results: Seventy patients, 37 with LIC, met inclusion criteria during the 10-year study period (age: 14.5 +/- 13.6 years [mean +/- standard deviation]; 30 male, 40 female). Both elevated D-dimer and low fibrinogen were associated with the presence of phleboliths, larger lesion sizes and visceral involvement on MRI (all P < 0.05). In stepwise regressions, lesion size (P < 0.001), the presence of phleboliths (P = 0.005) and lesion morphology (P = 0.006) were all significant predictors of LIC. Conclusion: LIC is associated with larger lesion size, visualized phleboliths, truncal location and spongiform morphology on MRI in venous malformations, suggesting that lesions with larger capacitance, slower flow and less physiological compression are more likely to be associated with coagulopathy.

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.

Arteriovenous malformation (AVM) of the mandible is a rare entity but one that has significant potential for fatality due to massive hemorrhage. Current treatment has involved surgical resection of the mandible in conjunction with adjunctive endovascular embolization to help control hemorrhage. However, jaw resection is deforming and often leaves a significant defect requiring subsequent bone grafting and replacement of lost teeth. We report an endovascular and surgical technique to treat central AVMs of the mandible that permits resection and complete removal of the intraosseous lesion yet prevents facial deformity by preserving the mandibular bone contour and permitting reconstruction of the dentition and restoration of function.

Microcephalic osteodysplastic primordial dwarfism type II (MOPD II) is a rare genetic syndrome characterized by extremely small stature and microcephaly, and is associated in 25% of patients with intracranial aneurysms and moyamoya disease. Although aneurysmal subarachnoid hemorrhage and stroke are leading causes of morbidity and death in these patients, MOPD II is rarely examined in the neurosurgical literature. The authors report their experience with 3 patients who presented with MOPD II, which includes a patient with 8 aneurysms (the most aneurysms reported in the literature), and the first report of a patient with both moyamoya disease and multiple aneurysms. The poor natural history of these lesions indicates aggressive microsurgical and/or endovascular therapy. Microsurgery, whether for aneurysm clip placement or extracranial-intracranial bypass, is challenging due to tight surgical corridors and diminutive arteries in these patients, but is technically feasible and strongly indicated when multiple aneurysms must be treated or cerebral revascularization is needed.

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: 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.

Purpose: Intracranial aneurysm (IA) growth is associated with increased morbidity. We sought to establish a quantitative computational method based on contrast-enhanced MR angiography (CE-MRA) for estimating aneurysmal volume changes over time. Materials and Methods: Computational volume calculations were tested against a distensible phantom. Untreated patients with IA were followed longitudinally with annual MRI. Maximal linear dimensions along the longitudinal axis and two transverse axes were determined by visual review of maximum intensity projection (MIP) data, and aneurysm volume was approximated as (length x width x height)/2. Averages of the visual approximations were compared to the lumenal volume as determined with a computational algorithm using the MRI data. Results: MRI-based measurements accurately represented volume changes in the phantom (R-2 = 0.97, Y = 1.06x + 271 CM3). In the clinical study there were a total of 11 intervals of one-year follow-up in six patients (mean +/- SD age = 53 +/- 20 years). The raw one-year growth using the computational volume was 9% +/- 17%. The corresponding value for the averaged measurement of the reviewers was 8% +/- 14%. Neither the mean values nor the SDs were different (P = .51). Conclusion: MRI-based measurement of aneurysm volume appears feasible for longitudinal studies of aneurysm natural history.

BackgroundThe absence of a discrete mass, surrounding signal abnormality and solid enhancement are imaging features that have traditionally been used to differentiate soft-tissue arteriovenous malformations from vascular tumors on MRI. We have observed that these findings are not uncommon in arteriovenous malformations, which may lead to misdiagnosis or inappropriate treatment.ObjectiveTo estimate the frequency of atypical MRI features in soft-tissue arteriovenous malformations and assess their relationship to lesion size, location, tissue type involved and vascular architecture.Materials and methodsMedical records, MRI and histopathology were reviewed in consecutive patients with soft-tissue arteriovenous malformations in a multidisciplinary vascular anomalies clinic. Arteriovenous malformations were divided into those with and without atypical MRI findings (perilesional T2 signal abnormality, enhancement and/or a soft-tissue mass). Lesion location, size, tissue involved and vascular architecture were also compared between groups. Tissue stains were reviewed in available biopsy or resection specimens to assess relationships between MRI findings and histopathology.ResultsThirty patients with treatment-naïve arteriovenous malformations were included. Fifteen lesions demonstrated atypical MRI. There was no difference in age, gender, lesion size or involved body part between the groups. However, more than half of the atypical lesions demonstrated multicompartmental involvement, and tiny intralesional flow voids were more common in atypical arteriovenous malformations. Histopathology also differed in atypical cases, showing densely packed endothelial cells with connective tissue architectural distortion and edema.ConclusionArteriovenous malformations may exhibit features of a vascular tumor on MRI, particularly when multicompartmental and/or containing tiny internal vessels. These features are important to consider in suspected fast-flow vascular malformations and may have implications with respect to their treatment.

OBJECT Retroclival hematomas are rare, appearing mostly as posttraumatic phenomena in children. Spontaneous retroclival hematoma (SRH) in the absence of trauma also has few descriptions in the literature. None of the reported clinical cases features the combination of an SRH and intraventricular hemorrhage (IVH). Nevertheless, despite extensive cases of idiopathic or angiographically negative subarachnoid hemorrhage (SAH) of the posterior fossa, only a single case report of a patient with a unique spontaneous retroclival hematoma has been identified. In this study, the authors reviewed the presentation, management, and clinical outcome of this rare entity. METHODS The authors performed a retrospective analysis of all patients with diagnosed SRH at their institution over a 3-year period. Collected data included clinical history, laboratory results, treatment, and review of all imaging studies performed. RESULTS Four patients had SRH. All were appropriately evaluated for coagulopathic and/or traumatic etiologies of hemorrhage, though no etiology could be found. Moreover, all of the patients demonstrated SRH that both clearly crossed the basioccipital synchondrosis and was contained within a nondependent configuration along the retroclival dura mater. CONCLUSIONS Spontaneous retroclival hematoma, often associated with IVH, is a rare subtype of intracranial hemorrhage frequently recognized only when MRI demonstrates compartmentalization of the posterior fossa hemorrhage. When angiography fails to reveal an underlying lesion, SRH patients, like patients with traditional angiographically negative SAH, enjoy a remarkably good prognosis.

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