Arteriovenous shunts of the cervical spine: patient demographics, presentation, patterns of high-risk venous drainage, and updated classification.

BACKGROUND: Intracranial dural arteriovenous (AV) fistula classifications focus on presence/absence of retrograde flow in the cortical veins of the brain as this angiographic finding portends a worse prognosis. However, prior categorization systems of AV shunts in the spine do not incorporate these features. We propose an updated classification for spinal shunting lesions that terms any shunting lesion with retrograde flow in any cortical vein of the brain or spinal cord medullary vein as "high risk". To present this classification, we analyzed our center's most recent experience with cervical spine shunting lesions. METHODS: The electronic medical record at our institution was reviewed to identify shunting lesions of the cervical spine and patient demographics/presentation. Comprehensive craniospinal digital subtraction angiograms were evaluated to classify shunt location, type (arteriovenous malformation (AVM) vs arteriovenous fistula (AVF)), and presence of high-risk venous drainage. RESULTS: Some 52 lesions were identified and categorized as pial/dural/epidural/paravertebral AVFs and intramedullary/extraspinal AVMs. Lesions were classified as high risk or not depending on the presence of retrograde flow into at least one vein that directly drains the spinal cord or brain. All patients who presented with either hemorrhage or infarct had underlying high-risk lesions. Additionally, 50% (17/34) of symptomatic patients with high-risk lesions presented with neurological extremity symptoms (OR=10.0, p=0.037) most of which fit a myelopathic pattern. CONCLUSION: We present an updated classification system for shunting lesions of the spine that focuses on high-risk retrograde flow to the brain or spine in addition to anatomical location in order to better inform patient management.

Endovascular vs surgical treatment of sigmoid sinus diverticulum causing pulsatile tinnitus: A systematic review.

INTRODUCTION: Sigmoid sinus diverticulum (SSD) has been increasingly reported as a cause of pulsatile tinnitus (PT). While both endovascular and surgical treatments have been used, there is a lack of consensus on the treatment modality to treat SSD. We conducted a systematic review of the available literature to compare the clinical outcomes and safety of endovascular versus surgical approaches for treating SSD. METHODS: A systematic review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses to identify studies encompassing the management of SSD. Studies reporting the clinical outcomes and safety of endovascular or surgical treatments for SSD between January 2000 and January 2023 were included. Results were characterized using descriptive statistics. RESULTS: Endovascular treatment (EVT) was reported by 17 articles, yielding 26 patients with 27 diverticula. Surgical treatment was reported by 20 articles, yielding 105 patients with 107 diverticula. EVT led to complete or near-complete resolution in all patients with SSD and PT. Complications occurred in 3.7% (1/27) with a return to baseline after 2 months. There were no permanent complications from EVT. Surgical treatment resulted in complete resolution in 77.6% (83/107) of cases, incomplete resolution in 11.2% (12/107), and no resolution in 11.2% (12/107). Significant complications occurred in 9.3% (10/107) of the surgical-treated patients. CONCLUSION: EVT in patients with PT and venous diverticulum appears more effective and safer than surgical treatment, but large studies are lacking. Studies directly comparing endovascular and surgical treatment are needed.

Angulation of the dural venous sinuses of the posterior cranial fossa: Anatomical study with clinical and surgical applications.

Cerebral vein and dural venous sinus thromboses (CVST) account for 0.5%-1% of all strokes. Some structural factors associated with a potentially higher risk for developing CVST have been described. However, angulation of the dural venous sinuses (DVS) has yet to be studied as a structural factor. The current study was performed because this variable could be related to alterations in venous flow, thus predisposing to a greater risk of CVST development. Additionally, such information could help shed light on venous sinus stenosis (VSS) at or near the transverse-sigmoid junction. The angulations formed in the different segments of the grooves of the transverse (TS), sigmoid (SS), and superior sagittal sinuses (SSS) were measured in 52 skulls (104 sides). The overall angulation of the TS groove was measured using two reference points. Other variables were examined, such as the communication pattern at the sinuses' confluence and the sinus grooves' lengths and widths. The patterns of communication between sides were compared statistically. The most typical communication pattern at the sinuses' confluence was a right-dominant TS groove (82.98%). The mean angulations of the entire left TS groove at two different points (A and B) were 46° and 43°. Those of the right TS groove were 44° and 45°. The median angulations of the left and right SSS-transverse sinus junction grooves were 127° and 124°. The mean angulations of the left and right TS-SSJsv grooves were 111° (range 82°-152°) and 103° (range 79°-130°). Differentiating normal and abnormal angulations of the DVSs of the posterior cranial fossa can help to explain why some patients are more susceptible to pathologies affecting the DVSs, such as CVST and VSS. Future application of these findings to patients with such pathologies is now necessary to extrapolate our results.

Endovascular therapy for anterior circulation emergent large vessel occlusion stroke in patients with large ischemic cores: a report of the SNIS Standards and Guidelines Committee.

BACKGROUND: Early clinical trials validating endovascular therapy (EVT) for emergent large vessel occlusion (ELVO) ischemic stroke in the anterior circulation initially focused on patients with small or absent completed infarctions (ischemic cores) to maximize the probability of detecting a clinically meaningful and statistically significant benefit of EVT. Subsequently, real-world experience suggested that patients with large core ischemic strokes (LCS) at presentation may also benefit from EVT. Several large, retrospective, and prospective randomized clinical trials have recently been published that further validate this approach. These guidelines aim to provide an update for endovascular treatment of LCS. METHODS: A structured literature review of LCS studies available since 2019 and grading the strength and quality of the evidence was performed. Recommendations were made based on these new data by consensus of the authors, with additional input from the full SNIS Standards and Guidelines Committee and the SNIS Board of Directors. RESULTS: The management of ELVO strokes with large ischemic cores continues to evolve. The expert panel agreed on several recommendations: Recommendation 1: In patients with anterior circulation ELVO who present within 24 hours of last known normal with large infarct core (70-149 mL or ASPECTS 3-5) and meet other criteria of RESCUE-Japan LIMIT, SELECT2, ANGEL-ASPECT, TESLA, TENSION, or LASTE trials, thrombectomy is indicated (Class I, Level A). Recommendations 2-7 flow directly from recommendation 1. Recommendation 2: EVT in patients with LCS aged 18-85 years is beneficial (Class I, Level A). Recommendation 3: EVT in patients with LCS >85 years of age may be beneficial (Class I, Level B-R). Recommendation 4: Patients with LCS and NIHSS score 6-30 benefit from EVT in LCS (Class I, Level A). Recommendation 5: Patients with LCS and NIHSS score <6 and >30 may benefit from EVT in LCS (Class IIa, Level A). Recommendation 6: Patients with LCS and low baseline mRS (0-1) benefit from EVT (Class I, Level A). Recommendation 7: Patients with LCS and time of last known well 0-24 hours benefit from EVT (Class I, Level A). Recommendation 8: It is recommended that patients with ELVO LCS who also meet the criteria for on-label or guideline-directed use of IV thrombolysis receive IV thrombolysis, irrespective of whether endovascular treatments are being considered (Class I, Level B-NR). CONCLUSIONS: The indications for endovascular treatment of ELVO strokes continue to expand and now include patients with large ischemic cores on presentation. Further prospective randomized studies, including follow-up to assess the population-based efficacy of treating patients with LCS, are warranted.

Factors Affecting Selection of TraineE for Neurointervention (FASTEN).

BACKGROUND AND IMPORTANCE: Neurointervention is a very competitive specialty in the United States due to the limited number of training spots and the larger pool of applicants. The training standards are continuously updated to ensure solid training experiences. Factors affecting candidate(s) selection have not been fully established yet. Our study aims to investigate the factors influencing the selection process. METHODS: A 52-question survey was distributed to 93 program directors (PDs). The survey consisted of six categories: (a) Program characteristics, (b) Candidate demographics, (c) Educational credentials, (d) Personal traits, (e) Research and extracurricular activities, and (f) Overall final set of characteristics. The response rate was 59.1%. As per the programs' characteristics, neurosurgery was the most involved specialty in running the training programs (69%). Regarding demographics, the need for visa sponsorship held the greatest prominence with a mean score of 5.9 [standard deviation (SD) 2.9]. For the educational credentials, being a graduate from a neurosurgical residency and the institution where the candidate's residency training is/was scored the highest [5.4 (SD = 2.9), 5.4 (SD = 2.5), respectively]. Regarding the personal traits, assessment by faculty members achieved the highest score [8.9 (SD = 1)]. In terms of research/extracurricular activities, fluency in English had the highest score [7.2 (SD = 1.9)] followed by peer-reviewed/PubMed-indexed publications [6.4 (SD = 2.2)]. CONCLUSION: Our survey investigated the factors influencing the final decision when choosing the future neurointerventional trainee, including demographic, educational, research, and extracurricular activities, which might serve as valuable guidance for both applicants and programs to refine the selection process.

Dural sinus septum as an underlying cause of intrinsic venous sinus stenosis: Anatomical, clinical, and stent placement considerations.

Recently, an interesting study regarding "Dural sinus septum: an underlying cause of cerebral venous sinus stenting failure and complications." was published, to our knowledge, being the launching point of the clinical/interventional applications of this intraluminal variation. Herein, we wish to highlight paramount anatomical, clinical, and stent placement considerations related to DSS located in the dural venous sinus at the posterior cranial fossa and the interventional complications caused by the presence of this variation during stenting procedures.

Transvenous coil embolization of a Cognard V transverse-sigmoid sinus dural arteriovenous fistula.

Dural arteriovenous fistulas with drainage into the spinal veins, classified as Cognard type 5, can be challenging to diagnose and treat. Brainstem and cervical spinal cord signal abnormalities on magnetic resonance imaging result from venous congestion, and can mimic tumor, infection, or inflammation.1-3 Transarterial and transvenous embolization techniques can be used to treat dural arteriovenous fistulas endovascularly. Efficacious transvenous treatment relies on the ability to safely catheterize the draining vein at the dural arteriovenous fistula site. Transvenous access options may seem limited in the setting of occluded venous sinuses. This case highlights the technical aspects of the transvenous approach to embolization of a transverse-sigmoid sinus dural arteriovenous fistula within an isolated sinus,4,5 demonstrating traversal of the occluded venous sinus from a contralateral approach.6,7[Media: see text].

Venous Pathologies and Interventions of the Head.

Intracranial venous pathologies are a historically underrecognized group of disorders that can have a devastating impact on patients. Despite advancements in peripheral venous disorders and arterial neurointerventions, intracranial venous pathologies have received comparatively little attention. Understanding the anatomy, physiology, clinical relevance, and treatment options of intracranial venous pathologies is fundamental to evolving therapies and research priorities. This article provides an overview of major intracranial venous pathologies, the respective pathophysiologies, and treatment options.

Clinical evaluation of pulsatile tinnitus: history and physical examination techniques to predict vascular etiology.

BACKGROUND: Pulsatile tinnitus (PT) may be due to a spectrum of cerebrovascular etiologies, ranging from benign venous turbulence to life threatening dural arteriovenous fistulas. A focused clinical history and physical examination provide clues to the ultimate diagnosis; however, the predictive accuracy of these features in determining PT etiology remains uncertain. METHODS: Patients with clinical PT evaluation and DSA were included. The final etiology of PT after DSA was categorized as shunting, venous, arterial, or non-vascular. Clinical variables were compared between etiologies using multivariate logistic regression, and performance at predicting PT etiology was determined by area under the receiver operating curve (AUROC). RESULTS: 164 patients were included. On multivariate analysis, patient reported high pitch PT (relative risk (RR) 33.81; 95% CI 3.81 to 882.80) compared with exclusively low pitch PT and presence of a bruit on physical examination (9.95; 2.04 to 62.08; P=0.007) were associated with shunting PT. Hearing loss was associated with a lower risk of shunting PT (0.16; 0.03 to 0.79; P=0.029). Alleviation of PT with ipsilateral lateral neck pressure was associated with a higher risk of venous PT (5.24; 1.62 to 21.01; P=0.010). An AUROC of 0.882 was achieved for predicting the presence or absence of a shunt and 0.751 for venous PT. CONCLUSION: In patients with PT, clinical history and physical examination can achieve high performance at detecting a shunting lesion. Potentially treatable venous etiologies may also be suggested by relief with neck compression.

Cerebral venous disorders: Diagnosis and endovascular management.

The role of the venous circulation in neurological diseases has been underestimated. In this review, we present an overview of the intracranial venous anatomy, venous disorders of the central nervous system, and options for endovascular management. We discuss the role the venous circulation plays in various neurological diseases including cerebrospinal fluid (CSF) disorders (intracranial hypertension and intracranial hypotension), arteriovenous diseases, and pulsatile tinnitus. We also shed light on emergent cerebral venous interventions including transvenous brain-computer interface implantation, transvenous treatment of communicating hydrocephalus, and the endovascular treatment of CSF-venous disorders.

More than just noise: Association of pulsatile tinnitus with anxiety, depression, and reduction of quality of life.

BACKGROUND: While many of the causes of pulsatile tinnitus (PT) are treatable with endovascular approaches, the risks of treatment must be balanced with the risks of the underlying cause and the psychological impact of symptoms on patients. While many physicians have anecdotal experience, the comorbid relationship of depression and anxiety with PT is unknown. The objectives of this study are to quantify the prevalence of depression and anxiety, and, to identify the demographic risk factors for impactful depression and anxiety in patients with PT. METHODS: Subjects recruited from online PT groups filled out secure online questionnaires that included demographic questions, validated Tinnitus Functional Index (TFI) as well as PHQ-9 and GAD-7 questionnaires to assess the prevalence of concurrent depression and anxiety, respectively. RESULTS: Sample included 515 surveys (84% female, 65% unemployed, mean(sd) age = 46.4 years (14.2)). Median symptom duration was 1.9 years. Data showed 46% and 37% of patients with moderate to severe depression and anxiety, respectively. Higher TFI scores were associated with moderate to severe depression (OR 1.07; 95% CI 1.06-1.09, p < 0.001) and anxiety (OR 1.05, 95% CI 1.04-1.06, p < 0.001), with TFI subscores also independently being associated in a univariate analysis. CONCLUSIONS: The prevalence of moderate to severe depression and anxiety in the PT population, which was previously unknown, is estimated in our study to be 46% and 37%, respectively. Significant association of TFI score with increased depression and anxiety scales adds further evidence of the impact of PT on the psychological health of these patients.

Clarifying the clinical landscape of pediatric spinal arteriovenous shunts: an institutional experience and individual patient-data meta-analysis.

BACKGROUND: Pediatric spinal arteriovenous shunts (SAVS) are rare lesions with heterogeneous pathogenesis and clinical manifestations. OBJECTIVE: To 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. METHODS: A 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. RESULTS: The 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. CONCLUSIONS: This 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.

Evolving indications for pediatric neurointerventional radiology: A single institutional 25-year experience in infants less than one year of age and a brief historical review.

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.

A national survey of venous sinus stenting practices for idiopathic intracranial hypertension.

BACKGROUND: Little is currently known about physician opinions and preferences on venous sinus stenting (VSS) for idiopathic intracranial hypertension (IIH), practice patterns, or clinical volumes. METHODS: A 19 question online survey was designed and distributed to physician members of the Society of Neurointerventional Surgery (SNIS). RESULTS: A total of 107 individual survey responses were obtained (14% of SNIS members). The majority of respondents (85%) indicated that they had performed at least one VSS procedure independently during their careers. Mean (SD) and median (range) career case volumes were 20.9 (33.8) and 10.0 (0.0-200.0), respectively. On a 1-10 scale, most respondents reported a high level of interest in treating IIH patients with VSS (median 8), a high level of comfort/expertise in treating IIH patients with VSS (median 9), and that VSS was effective in the long term reduction of symptoms and papilledema in IIH patients (median 8). Fifty-nine per cent of respondents reported increasing VSS volumes compared with previous years. A major complication during a VSS procedure, including two deaths, was reported by 11% of respondents. CONCLUSIONS: This is the first study designed to understand the opinions and practices of neurointerventionists regarding VSS for IIH. Overall physician opinion on VSS was quite positive, supported by increasing procedural volumes reported by most over the past few years. However, only a small percentage of respondents had substantial experience with VSS and major complications were not rare.

Cerebrovascular pulsatile tinnitus: causes, treatments, and outcomes in 164 patients with neuroangiographic correlation.

BACKGROUND: Pulsatile tinnitus (PT) can cause significant detriment to quality of life and may herald a life-threatening condition. Endovascular evaluation is the gold standard for the definitive diagnosis of PT and facilitates treatment. However, no large study has determined the distribution of causes and treatment outcomes of PT evaluated endovascularly. METHODS: Consecutive patients evaluated at a multidisciplinary PT clinic from a single academic center were retrospectively reviewed. Patients with a suspected cerebrovascular etiology of PT based on clinical and/or non-invasive imaging, who were evaluated by endovascular techniques (arteriography, venography, manometry, and/or balloon test occlusion), were included in analysis. Baseline clinical features and treatment results by final etiology of PT were compared. RESULTS: Of 552 patients referred for PT evaluation, 164 patients (29.7%) who underwent endovascular evaluation of PT were included. Mean (±SD) age at first clinical evaluation was 54.3±14.1 years (range 25-89 years); 111 patients (67.7%) were female. PT causes were 75.6% vascular and 24.4% non-vascular. Arteriovenous shunting lesions caused 20.7% of cases, venous etiologies 48.2%, and arterial etiologies 6.7%. Of patients with a shunting lesion treated with endovascular embolization, 96.9% had lasting significant improvement or resolution in PT. Endovascular stenting for venous sinus stenosis gave 84.6% of patients lasting improvement or resolution in PT. Arterial and non-vascular PT had fewer patients treated endovascularly and less improvement in PT symptoms. CONCLUSION: PT with a suspected vascular cause is most often attributable to venous etiologies. PT caused by arteriovenous shunting or venous sinus stenosis may be effectively treated endovascularly.

Percutaneous sclerotherapy for head and neck lymphatic malformations in neonates and infants ≤12 months of age.

BACKGROUND: Percutaneous 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. METHODS: We 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. RESULTS: 22 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 (<25% improvement). At last follow-up, 71.4% of children were developmentally normal and asymptomatic, 23.8% had recurring symptoms, and 4.8% required permanent tracheostomy. Patients with ongoing symptoms or limited response to percutaneous sclerotherapy (33.3%) were treated with long-term sirolimus. CONCLUSIONS: Percutaneous 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.

Angiographic Evaluation of Cranial Venous Outflow Patterns in Patients With and Without Idiopathic Intracranial Hypertension.

BACKGROUND: Several collateral venous pathways exist to assist in cranial venous drainage in addition to the internal jugular veins. The important extrajugular networks (EJN) are often readily identified on diagnostic cerebral angiography. However, the angiographic pattern of venous drainage through collateral EJN has not been previously compared among patients with and without idiopathic intracranial hypertension (IIH). OBJECTIVE: To quantify EJN on cerebral angiography among patients both with and without IIH and to determine whether there is a different EJN venous drainage pattern in patients with IIH. METHODS: Retrospective imaging review of 100 cerebral angiograms (50 IIH and 50 non-IIH patients) and medical records from a single academic medical center was performed by 2 independent experienced neuroendovascular surgeons. Points were assigned to EJN flow from 0 to 6 using an increasing scale (with each patient's dominant internal jugular vein standardized to 5 points to serve as the internal reference). Angiography of each patient included 11 separately graded extrajugular networks for internal carotid and vertebral artery injections. RESULTS: Patients in the IIH group had statistically significant greater flow in several of the extrajugular networks. Therefore, they preferentially drained through EJN compared with the non-IIH group. Right transverse-sigmoid system was most often dominant in both groups, yet there was a significantly greater prevalence of codominant sinus pattern on posterior circulation angiograms. CONCLUSION: Patients with IIH have greater utilization of EJN compared with patients without IIH. Whether this is merely an epiphenomenon or possesses actual cause-effect relationships needs to be determined with further studies.

Pharyngo-tympano-stapedial middle meningeal artery variant supply to a falcotentorial dural arteriovenous fistula.

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.

Endovascular Therapy for Intracranial Giant Cell Arteritis : Systematic Review, Technical Considerations and the Effect of Intra-arterial Calcium Channel Blockers.

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.