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.

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.

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.

Brain herniation (encephalocele) into arachnoid granulations: prevalence and association with pulsatile tinnitus and idiopathic intracranial hypertension.

PURPOSE: Brain herniation into arachnoid granulations (BHAG) of the dural venous sinuses is a recently described finding of uncertain etiology. The purpose of this study was to investigate the prevalence of BHAG in a cohort of patients with pulsatile tinnitus (PT) and to clarify the physiologic and clinical implications of these lesions. METHODS: The imaging and charts of consecutive PT patients were retrospectively reviewed. All patients were examined with MRI including pre- and post-contrast T1- and T2-weighted sequences. Images were reviewed separately by three blinded neuroradiologists to identify the presence of BHAG. Their location, signal intensity, size, presence of arachnoid granulation, and associated dural venous sinus stenosis were documented. Clinical records were further reviewed for idiopathic intracranial hypertension, history of prior lumbar puncture, and opening pressure. RESULTS: Two hundred sixty-two consecutive PT patients over a 4-year period met inclusion criteria. PT patients with BHAG were significantly more likely to have idiopathic intracranial hypertension than PT patients without BHAG (OR 4.2, CI 1.5-12, p = 0.006). Sixteen out of 262 (6%) patients were found to have 18 BHAG. Eleven out of 16 (69%) patients had unilateral temporal or occipital lobe herniations located in the transverse sinus or the transverse-sigmoid junction. Three out of 16 (19%) patients had unilateral cerebellar herniations and 2/16 (13%) patients had bilateral BHAG. CONCLUSION: In patients with PT, BHAG is a prevalent MRI finding that is strongly associated with the clinical diagnosis of IIH. The pathogenesis of BHAG remains uncertain, but recognition should prompt comprehensive evaluation for IIH.

Eptifibatide bridging therapy for staged carotid artery stenting and cardiac surgery: Safety and feasibility.

BACKGROUND: Prophylactic carotid artery stenting (CAS) is an effective strategy to reduce perioperative stroke in patients with severe carotid stenosis who require cardiothoracic surgery (CTS). Staging both procedures (CAS-CTS) during a single hospitalization presents conflicting demands for antiplatelet therapy and the optimal pharmacologic strategy between procedures is not established. The purpose of this study is to present our initial experience with a "bridging" protocol for staged CAS-CTS. METHODS: A retrospective review of staged CAS-CTS procedures at a single referral center was performed. All patients had multivessel coronary and/or valvular disease and severe carotid stenosis (>70%). Patients not previously on aspirin were also started on aspirin prior to surgery, followed by eptifibatide during CAS (intraprocedural bolus followed by post-procedural infusion which was continued until the morning of surgery). Pre- and perioperative (30 days) neurologic morbidity and mortality was the primary endpoint. RESULTS: 11 CAS procedures were performed in 10 patients using the protocol. The median duration of eptifibatide bridge therapy was 36 h (range 24-288 h). There was one minor bleeding complication (1/11, 9.1%) and no major bleeding complications during the bridging and post-operative period. There was one post-operative, non-neurologic death and zero perioperative ischemic strokes. CONCLUSIONS: For patients undergoing staged CAS-CTS, Eptifibatide bridging therapy is a viable temporary antiplatelet strategy with a favorable safety profile. This strategy enables a flexible range of time-intervals between procedures.

Combined Use of X-ray Angiography and Intraprocedural MRI Enables Tissue-based Decision Making Regarding Revascularization during Acute Ischemic Stroke Intervention.

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.

Jugular Venous Reflux Can Mimic Posterior Fossa Dural Arteriovenous Fistulas on MRI-MRA.

Dural arteriovenous fistulas (DAVFs) are high-flow acquired shunts that can carry high risk of intracranial hemorrhage. Because DAVFs can often be managed by endovascular means, early and accurate diagnosis can markedly improve patient morbidity. Time-of-flight and arterial spin-labeling MRA have increased the diagnostic utility of MRI for DAVF by showing hemodynamic rather than anatomic evidence of shunting. The purpose of this article is to describe the cases of seven patients who had co-localization of arterial spin-labeling signal intensity and time-of-flight flow-related enhancement in the left skull base, resulting in a misdiagnosis of DAVF and a recommendation for catheter angiography by the interpreting radiologist. Benign jugular venous reflux is identified as a common mechanism in each case, and the physiology behind this imaging pitfall is described. An algorithmic diagnostic approach to differentiating physiologic venous reflux from true posterior skull base DAVFs is presented.

Occult Brain Arteriovenous Malformation Superimposed on a Pial Arteriovenous Fistula: Case Report.

INTRODUCTION: Pial arteriovenous fistulas are characterized by an abnormal connection between an intracranial artery and vein without an intervening nidus. Their predominant symptoms largely arise from mass effect, shunting, or hemorrhage. Most conservatively managed cases progress to death, but endovascular and/or surgical intervention is often successful. CASE PRESENTATION: We present the unique case of a 15-year-old girl with spontaneous intracranial hemorrhage from a single-vessel arteriovenous fistula. Although preoperative imaging failed to show a distinct nidus, intraoperative indocyanine green angiography performed after successful clipping of the primary fistulous site revealed residual shunting from a peri-lesional arteriovenous malformation. DISCUSSION/CONCLUSION: This case demonstrates the importance of intraoperative imaging and meticulous circumferential inspection of these lesions to detect residual vascular shunting.

Brain Arteriovenous Malformation Recurrence After Apparent Microsurgical Cure: Increased Risk in Children Who Present With Arteriovenous Malformation Rupture.

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.

Reversible cerebral vasoconstriction syndrome is a rare cause of stroke after carotid endarterectomy.

Neurologic events after carotid endarterectomy (CEA) require prompt diagnosis and management to avoid potentially catastrophic sequelae. This report describes a 69-year-old gentleman who underwent a left CEA for a high-grade asymptomatic carotid stenosis with concomitant contralateral carotid occlusion. He had transient and crescendo neurologic events in the first 3 postoperative weeks that culminated in right hand weakness and paresthesia, despite dual antiplatelet therapy, maximal anticoagulation, and undergoing stenting of the endarterectomy site. Neurologic events recurred despite these measures and subsequent angiography showed reversible cerebral vasoconstriction syndrome that was successfully managed without further events. Reversible cerebral vasoconstriction syndrome is an unusual but important cause of neurologic events after CEA that requires aggressive and directed medical therapy.

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.

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.

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.

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].

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.

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.

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.

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.

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.