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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.

Background For patients with acute ischemic stroke undergoing endovascular mechanical thrombectomy with x-ray angiography, the use of adjuncts to maintain vessel patency, such as stents or antiplatelet medications, can increase risk of periprocedural complications. Criteria for using these adjuncts are not well defined. Purpose To evaluate use of MRI to guide critical decision making by using a combined biplane x-ray neuroangiography 3.0-T MRI suite during acute ischemic stroke intervention. Materials and Methods This retrospective observational study evaluated consecutive patients undergoing endovascular intervention for acute ischemic stroke between July 2019 and May 2020 who underwent either angiography with MRI or angiography alone. Cerebral tissue viability was assessed by using MRI as the reference standard. For statistical analysis, Fisher exact test and Student t test were used to compare groups. Results Of 47 patients undergoing acute stroke intervention, 12 patients (median age, 69 years; interquartile range, 60-77 years; nine men) underwent x-ray angiography with MRI whereas the remaining 35 patients (median age, 80 years; interquartile range, 68-86 years; 22 men) underwent angiography alone. MRI results influenced clinical decision making in one of three ways: whether or not to perform initial or additional mechanical thrombectomy, whether or not to place an intracranial stent, and administration of antithrombotic or blood pressure medications. In this initial experience, decision making during endovascular acute stroke intervention in the combined angiography-MRI suite was better informed at MRI, such that therapy was guided in real time by the viability of the at-risk cerebral tissue. Conclusion Integrating intraprocedural 3.0-T MRI into acute ischemic stroke treatment was feasible and guided decisions of whether or not to continue thrombectomy, to place stents, or to administer antithrombotic medication or provide blood pressure medications. © RSNA, 2021 Online supplemental material is available for this article. See also the editorial by Lev and Leslie-Mazwi in this issue.

Background Spinal epidural arteriovenous fistulas (seAVF) are a rare subset of vascular lesions that are most commonly found in the cervical levels. Unlike spinal dural AVF, seAVF are typically supplied by multiple arteries, including direct branches from the vertebral artery, which increases the risk of nontarget embolization using a transarterial endovascular approach. In these cases, transvenous embolization may be a preferable option, but accessing the cervical epidural venous space, also termed the internal vertebral venous plexus, can be challenging and requires advanced foreknowledge of cervical venous channels. Methods The authors review salient neurovascular anatomy and present 2 techniques for endovascular access of the cervical epidural space to treat seAVF. Results The physiology and structure of the cervical internal vertebral venous plexus is briefly reviewed. Next, the authors describe 2 complementary methods for transvenous access to the cervical internal vertebral venous plexus via the jugular vein (cranial‐caudal) and the vertebral vein (caudal‐cranial). The first approach involves retrograde microcatheterization via the jugular bulb and condylar veins. The second approach involves direct antegrade approach via the vertebral vein, arising from the brachiocephalic vein. Both approaches enable stable catheter positioning for coil embolization at remote cervical levels to treat a wide spectrum of seAVF. Conclusions Accessing the cervical epidural venous space is technically demanding and requires effective planning and knowledge of relevant spinal venous anatomy. These techniques are important tools for safe and effective endovascular treatment of seAVF.

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.

The use of intracranial stents in neurointerventional surgery has been practiced for decades. However, the spectrum of treatable pathology, available devices, and clinical adoption of intracranial stents has exploded in recent years. Diagnostic neuroradiologists play a critical role in the evaluation of these devices after deployment, yet may not be familiar with state-of-the-art intracranial stent devices and indications. This review provides an overview of intracranial stents for cerebrovascular disease, with 3 chief learning objectives: 1) to understand the basic principles of stent design, biomechanics, and deployment, and the resulting influence on cerebrovascular hemodynamics; 2) to be familiar with the spectrum of intracranial pathology amenable to endovascular stent placement; and 3) to recognize the radiographic appearance of successful intracranial stent deployment and intracranial stent‐related complications.Learning Objective: Recognize the key principles of design (form), current indications (function), and potential complications of intracranial stents used in neurointerventional surgery

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.

Background and Purpose Pediatric neurointerventional radiology is an evolving subspecialty with growing indications and technological advancement such as miniaturization of devices and decreased radiation dose. The ability to perform these procedures is continuously balanced with necessity given the inherently higher risks of radiation and cerebrovascular injury in infants. The purpose of this study is to review our institution's neurointerventional experience in infants less than one year of age to elucidate trends in this patient population. Methods We retrospectively identified 132 patients from a neurointerventional database spanning 25 years (1997–2022) who underwent 226 procedures. Treatment type, indication, and location as well as patient demographics were extracted from the medical record. Results Neurointerventional procedures were performed as early as day of life 0 in a patient with an arteriovenous shunting malformation. Average age of intervention in the first year of life is 5.9 months. Thirty-eight of 226 procedures were completed in neonates. Intra-arterial chemotherapy (IAC) for the treatment of retinoblastoma comprised 36% of neurointerventional procedures completed in infants less than one year of age followed by low flow vascular malformations (21.2%), vein of Galen malformations (11.5%), and dural arteriovenous fistulas (AVF) (9.3%). Less frequent indications include non-Galenic pial AVF (4.4%) and tumor embolization (3.0%). The total number of interventions has increased secondary to the onset of retinoblastoma treatment in 2010 at our institution. Conclusion The introduction of IAC for the treatment of retinoblastoma in the last decade is the primary driver for the increased trend in neurointerventional procedures completed in infants from 1997 to 2022.

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.

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.

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

Appropriate segregation within manufacturing facilities is required by regulators and utilized by manufacturers to ensure that the final product has not been contaminated with 1) adventitious viruses, 2) another pre-/ post-viral clearance fraction of the same product, or 3) with another product processed in the same facility. However, there is not consensus on what constitutes appropriate facility segregation to minimize these risks. In part, this is due to the fact that a wide variety of manufacturing facilities and operational practices exist, including single and multi-product manufacturing, using traditional segregation strategies with separate rooms for specific operations that may use stainless steel or disposable equipment to more modern ballroom style operation that use mostly disposable equipment (i.e. pre- and post-viral clearance manufacturing operations are not physically segregated by walls). Further, there is a lack of consensus around basic definitions and approaches related to facility segregation. For example, given that several unit operations provide assurance of virus clearance during downstream processing, how does one define pre- and post-viral clearance and at which point(s) should a viral segregation barrier be introduced? What is a “functionally closed” system? How can interventions be conducted so that the system remains “functionally closed”? How can “functionally closed” systems be used to adequately isolate a product stream and ensure its safety? To address these issues, the member companies of the Consortium on Adventitious Agent Contamination in Biomanufacturing (CAACB) have conducted a facility segregation project with the following goals: define “pre- and post-viral clearance zones” and “pre- and post-viral clearance materials”; define “functionally closed” manufacturing systems; and identify an array of facility segregation approaches that are used for the safe and effective production of recombinant biologics as well as plasma products. This article reflects the current thinking from this collaborative endeavor.

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.

As a refresher course on current techniques and practice of cerebral angiography, this chapter outlines the steps in effective angiography, incorporating modern quality and safety considerations. The format is step-by-step from preprocedure to intraprocedure to postprocedure for ease of reference. The chapter will serve as a framework for the fledgling angiographer, augmenting the important teaching provided by a seasoned angiographer.

Vascular anomalies are broadly classified into two major categories: vascular tumors and vascular malformations. Most vascular anomalies are caused by sporadic mosaic gene mutations, and recent genetic studies have advanced our understanding of the molecular pathways involved in their pathogenesis. These findings have suggested new therapeutic approaches to vascular anomalies, focusing on their pathogenetic mechanism. This chapter seeks to integrate an improved molecular understanding within the updated classification system of the International Society for Study of Vascular Anomalies. We emphasize the genetic, radiologic, and interventional aspects of diagnosis and management in hopes of allowing improved multidisciplinary collaboration surrounding these complex and interesting anomalies.

As a refresher course on current techniques and practice of cerebral angiography, this chapter outlines the steps in effective angiography, incorporating modern quality and safety considerations. The format is step-by-step from preprocedure to intraprocedure to postprocedure for ease of reference. The chapter will serve as a framework for the fledgling angiographer, augmenting the important teaching provided by a seasoned angiographer.

Vascular anomalies are broadly classified into two major categories: vascular tumors and vascular malformations. Most vascular anomalies are caused by sporadic mosaic gene mutations, and recent genetic studies have advanced our understanding of the molecular pathways involved in their pathogenesis. These findings have suggested new therapeutic approaches to vascular anomalies, focusing on their pathogenetic mechanism. This chapter seeks to integrate an improved molecular understanding within the updated classification system of the International Society for Study of Vascular Anomalies. We emphasize the genetic, radiologic, and interventional aspects of diagnosis and management in hopes of allowing improved multidisciplinary collaboration surrounding these complex and interesting anomalies.

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.

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.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.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.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: Patient registries are organized systems that use observational methods to collect uniform data on specified outcomes in a population defined by a particular disease, condition, or exposure. Data collected in registries often coincide with data that could support clinical trials. Integrating clinical trials within registries to create registry-embedded clinical trials offers opportunities to reduce duplicative data collection, identify and recruit patients more efficiently, decrease time to database lock, accelerate time to regulatory decision-making, and reduce clinical trial costs. This article describes a project of the Clinical Trials Transformation Initiative (CTTI) intended to help clinical trials researchers determine when a registry could potentially serve as the platform for the conduct of a clinical trial. METHODS: Through a review of registry-embedded clinical trials and commentaries, semi-structured interviews with experts, and a multi-stakeholder expert meeting, the project team addressed how to identify and describe essential registry characteristics, practices, and processes required to for conducting embedded clinical trials intended for regulatory submissions in the United States. RESULTS: Recommendations, suggested practices, and decision trees that facilitate the assessment of whether a registry is suitable for embedding clinical trials were developed, as well as considerations for the design of new registries. Essential registry characteristics include relevancy, robustness, reliability, and assurance of patient protections. CONCLUSIONS: The project identifies a clear role for registries in creating a sustainable and reusable infrastructure to conduct clinical trials. Adoption of these recommendations will facilitate the ability to perform high-quality and efficient prospective registry-based clinical trials. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1007/s43441-020-00185-5) contains supplementary material, which is available to authorized users.

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.

The Cystic Fibrosis Foundation Patient Registry (CFFPR) is an ongoing patient registry study that collects longitudinal demographic, clinical, and treatment information about persons with cystic fibrosis (CF) in the United States. CF is a life-shortening genetic disorder that occurs in approximately 1 in 3,500 births in the United States. High-quality observational data is important for clinical research, quality improvement, and clinical management.To describe the data collection, patient population, and key limitations of the CFFPR.Inclusion criteria for the CFFPR include diagnosis with CF or a CFTR-associated disorder, care at an accredited care center program, and provision of informed consent. Data from clinic visits and hospitalizations are collected through a secure website. Loss to follow-up and generalizability were examined using several methods. The accuracy of CFFPR data was evaluated with an audit of 2012 CFFPR data compared to the medical record.Since 1986, the CFFPR contains the records of 48,463 individuals with CF. Participation among individuals seen at accredited care centers is high, and loss to follow-up is low. An audit of 2012 CFFPR data suggests that the CFFPR contains 95% of clinic visits and 90% of hospitalizations found in the medical record for these patients, and nearly all of the audited fields were highly accurate.Registries such as the CFFPR are important tools for research, clinical care, and tracking incidence, mortality and population trends.

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.

OBJECTIVEPreoperative embolization of brain arteriovenous malformations (AVMs) is performed to facilitate resection, although its impact on surgical performance has not been clearly defined. The authors tested for associations between embolization and surgical performance metrics.METHODSThe authors analyzed AVM cases resected by one neurosurgeon from 2006 to 2017. They tested whether cases with and without embolization differed from one another with respect to patient and AVM characteristics using t-tests for continuous variables and Fisher’s exact tests for categorical variables. They used simple and multivariable regression models to test whether surgical outcomes (blood loss, resection time, surgical clip usage, and modified Rankin Scale [mRS] score) were associated with embolization. Additional regression analyses integrated the peak arterial afferent contrast normalized for the size of the region of interest (Cmax/ROI) into models as an additional predictor.RESULTSThe authors included 319 patients, of whom 151 (47%) had preoperative embolization. Embolized AVMs tended to be larger (38% with diameter > 3 cm vs 19%, p = 0.001), less likely to have hemorrhaged (48% vs 63%, p = 0.013), or be diffuse (19% vs 29%, p = 0.045). Embolized AVMs were more likely to have both superficial and deep venous drainage and less likely to have exclusively deep drainage (32% vs 17% and 12% vs 23%, respectively; p = 0.002). In multivariable analysis, embolization was not a significant predictor of blood loss or mRS score changes, but did predict longer operating times (+29 minutes, 95% CI 2–56 minutes; p = 0.034) and increased clip usage (OR 2.61, 95% CI 1.45–4.71; p = 0.001). Cmax/ROI was not a significant predictor, although cases with large Cmax/ROI tended to have longer procedure times (+25 minutes per doubling of Cmax/ROI, 95% CI 0–50 minutes; p = 0.051).CONCLUSIONSIn this series, preoperative embolization was associated with longer median resection times and had no association with intraoperative blood loss or mRS score changes.

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.