Presentation and management of cerebral proliferative angiopathy: a systematic review and meta-analysis on treatment outcomes.

Cerebral Proliferative Angiopathy (CPA) is a rare brain vascular malformation, similar to Arteriovenous Malformations (AVM) but lacking of early venous drainage. Presentation and treatment outcomes were investigated, examining for morbidity, mortality and complications. A meta-analysis was conducted according to PRISMA guidelines. PubMed, Embase and Web Of Science were searched with keywords such as "cerebral proliferative angiopathy" and "management". We pooled and meta-analyzed outcomes on documented CPA cases. 11,079 studies were pooled as a result of manual citation searching, 50 studies were included, adding up to 115 CPA cases. The majority of patients were females (1.38:1), with a mean age of presentation of 26.9 (19.4) years. Headache (46%) and seizures (34%) were the most common presenting symptoms. 37% of patients presented with focal neurologic deficit. Patients managed conservatively from the surgical standpoint (i.e. nonoperative management) did not undergo homogenous treatment strategies, and major complications were at 47% (95% CI: 17%, 76%), with a 1% mortality (95% CI: 0%, 6%). Surgical and embolization interventions presented the highest proportion of major complications, 66% (95% CI: 33%, 99%) and 73% (95% CI: 42%, 100%), respectively. The embolization subgroup led in mortality, with 3% (95% CI: 0%, 10%). No death was documented in patients undergoing surgery. CPA has a similar presentation to brain arteriovenous malformations, but its treatment outcomes are potentially worse. This difference is not attributable to heterogeneity in assigning patient treatment strategies. This highlights the need for more accurate diagnostic methods.

The role of procedural factors on the outcomes of embolization followed by radiosurgery for the treatment of brain arteriovenous malformations: systematic review and proportional meta-analyses.

BACKGROUND: Multimodal therapy for brain arteriovenous malformations (bAVM) with embolization followed by stereotactic radiosurgery (E + SRS) has shown varying outcomes. Its benefits over other treatment modalities have been questioned. The goal of this systematic review was to determine the factors associated with cure and complication rates of this treatment strategy. METHODS: A literature search in Medline and Global Index Medicus, from inception to October 2023, was performed. Studies reporting relevant outcome data from bAVM patients treated with E + SRS were included. Data on several patient, lesion and procedure-related factors were collected. Embolization intent was classified as Targeted (of high-risk features), Devascularizing (feeder embolization/flow reduction) and Occluding (intent-to-cure, nidus embolization). The primary outcome was obliteration rate. Secondary outcomes were post-SRS bleeding (PSB), post-embolization neurological complications (PENC) and post-SRS neurological complications (PSNC). Subgroup analyses included embolic agent, embolization intent and radiosurgery type. Proportional meta-analyses and meta-regressions were performed. RESULTS: Forty-one studies were included in the review. The pooled obliteration rate was 56.45% (95% CI 50.94 to 61.88). Meta-regression analyses showed higher obliteration rates with Copolymers and lower obliteration rates with Devascularizing embolization. The pooled PSB, PENC and PSNC rates were 5.50%, 13.75% and 5.02%, respectively. Meta-regression analyses showed higher rates of PSB, PENC and PSNC with Devascularizing embolization, Liquid & Solid embolic agents and Targeted & Devascularizing intent, respectively. CONCLUSION: Embolic agent and embolization intent were procedural factors associated with treatment outcomes of E + SRS in the management of bAVM patients. The efficacy and safety profiles favor copolymers as embolic agents and disfavor Devascularizing as embolization intent. STUDY REGISTRATION: The protocol of the systematic review was registered in PROSPERO as CRD42023474171.

Long-term complications and outcomes of therapeutic embolization of cerebral arteriovenous malformations: a systematic review.

BACKGROUND: Embolization is a promising treatment strategy for cerebral arteriovenous malformations (AVMs). However, consensus regarding the main complications or long-term outcomes of embolization in AVMs remains lacking. OBJECTIVE: To characterize the most prevalent complications and long-term outcomes in patients with AVM undergoing therapeutic embolization. DESIGN AND SETTING: This systematic review was conducted at the Federal University of Alagoas, Arapiraca, Brazil. METHODS: This systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses criteria. Data were obtained from MEDLINE, PubMed, LILACS, and SciELO databases, which included the epidemiological profile of the population, characteristics of the proposed therapy, complications (hemorrhagic events and neurological deficits), and long-term outcomes (modified Rankin scale pre- and post-treatment, AVM recanalization, complete obliteration, and deaths). RESULTS: Overall, the analysis included 34 articles (2,799 patients). Grade III Spetzler-Martin AVMs were observed in 34.2% of cases. Notably, 39.3% of patients underwent embolization combined with radiosurgery. The most frequently reported long-term complication was hemorrhage, which occurred in 8.7% of patients at a mean follow-up period of 58.6 months. Further, 6.3% of patients exhibited neurological deficits after an average of 34.7 months. Complete obliteration was achieved in 51.4% of the cases after a mean period of 36 months. Recanalization of AVMs was observed in 3.5% of patients. Long-term death occurred in 4.0% of patients. CONCLUSION: Embolization of AVMs is an increasingly safe strategy with low long-term complications and satisfactory outcomes, especially in patients who have undergone combination therapies. SYSTEMATIC REVIEW REGISTRATION: https://www.crd.york.ac.uk/prospero/ Registration number CRD42020204867.

Location-based clinical and angiographic profile of brain arteriovenous malformations - a single-center observational study.

BACKGROUND: The location of brain arteriovenous malformations (bAVM) is one of the most relevant prognostic factors included in surgical, endovascular and radiosurgical scores. However, their characteristics according to location are seldom described. The goal of this study was to describe the clinical and angiographic characteristics of bAVM classified according to their location. METHODS: This retrospective observational study included patients diagnosed with bAVM and attending a national referral hospital during the period 2010-2020. Data regarding clinical and angiographic variables were extracted, including characteristics on nidus, arterial afferents, venous drainage and associated aneurysms. BAVM were classified in 8 groups according to their location: frontal, temporal, parieto-occipital, periventricular, deep, cerebellar, brainstem and mixed. Data distribution for each group was determined and between-group differences were assessed. RESULTS: A total of 269 bAVM (in 258 patients) were included. The most frequent location was parieto-occipital; and the least frequent, brainstem. Statistically significant differences were observed between groups for most studied variables, including: clinical presentation, functional status at admission; nidus size and density, classification according to the Spetzler-Martin, Buffalo and modified Pollock-Flickinger scales; number, diameter, origin and type of afferents; number, diameter, type and direction of venous drainage, retrograde venous flow; and presence and size of flow-related aneurysms. CONCLUSION: The clinical and angiographic differences observed between brain AVM groups allow the formulation of profiles according to their location.

Changes in inpatient brain arteriovenous malformation management in the United States following the ARUBA trial: analysis of an interrupted time series design.

The November 2013 online publication of ARUBA, the first multi-institutional randomized controlled trial for unruptured brain arteriovenous malformations (AVMs), has sparked over 100 publications in protracted debates METHODS: This study sought to examine inpatient management patterns of brain AVMs from 2009 to 2016 and observe if changes in U.S. inpatient management were attributable to the ARUBA publication using interrupted time series of brain AVM studies from the National Inpatient Sample data 2009-2016. Outcomes of interest were use of embolization, surgery, combined embolization and microsurgery, radiotherapy, and observation during that admission. An interrupted time series design compared management trends before and after ARUBA. Segmented linear regression analysis tested for immediate and long-term impacts of ARUBA on management. RESULTS: Elective and asymptomatic patient admissions declined 2009-2016. In keeping with the ARUBA findings, observation for unruptured brain AVMs increased and microsurgery decreased. However, embolization, radiosurgery, and combined embolization and microsurgery also increased. For ruptured brain AVMs, treatment modality trends remained positive with even greater rates of observation, embolization, and combined embolization and microsurgery occurring after ARUBA (data on radiosurgery were scarce). None of the estimates for the change in trends were statistically significant. CONCLUSIONS: The publication of ARUBA was associated with a decrease in microsurgery and increase in observation for unruptured brain AVMs in the US. However, inpatient radiotherapy, embolization, and combined embolization and surgery also increased, suggesting trends moved counter to ARUBA's conclusions. This analysis suggested that ARUBA had a small impact as clinicians rejected ARUBA's findings in managing unruptured brain AVMs.

Brain Arteriovenous Malformation In Vitro Model for Transvenous Embolization Using 3D Printing and Real Patient Data.

BACKGROUND AND PURPOSE: Transvenous embolization has emerged as a novel technique for treating selected brain AVMs with high reported occlusion rates. However, it requires anatomic and technical skills to be successful and to ensure patient safety. Therefore, training and testing are essential for preparing clinicians to perform these procedures. Our aim was to develop and test a novel, patient-specific brain AVM in vitro model for transvenous embolization by using 3D printing technology. MATERIALS AND METHODS: We developed a brain AVM in vitro model based on real patient data by using stereolithography resin 3D printing. We created a closed pulsed circuit with flow passing from the arterial side to the venous side, and we tested the effect of mean arterial pressure on retrograde nidal filling with contrast injections. Transvenous embolization simulations were conducted for each of the 12 identical models divided into 2 groups (2×6). This involved the use of an ethylene-vinyl alcohol liquid embolic agent injected through microcatheters either without or with a coil in the vein (groups 1 and 2, respectively). RESULTS: Retrograde contrast advance to nidus was directly related to lower mean arterial pressure. Transvenous embolization tests with a liquid embolic agent adequately reproduced the usual embolization plug and push technique. We found no differences between the 2 group conditions, and additional venous coil neither increased nidus penetration nor reduced injection time in the model (57.6 versus 61.2% nidus occlusion rate, respectively). CONCLUSIONS: We were able to develop and test a functional in vitro brain AVM model for transvenous embolization by using 3D printing to emulate its conditions and characteristics. Better contrast penetration was achieved with less mean arterial pressure, and no embolization advantage was found by adding coil to the vein in this model.

Enhancing the quality of evidence, comparability, and reproducibility in brain arteriovenous malformations treated with open surgery research: a systematic review and proposal of a reporting guideline for surgical and clinical outcomes.

Brain Arteriovenous Malformations (bAVMs) are rare but high-risk developmental anomalies of the vascular system. Microsurgery through craniotomy is believed to be the mainstay standard treatment for many grades of bAVMs. However, a significant challenge emerges in the existing body of clinical studies on open surgery for bAVMs: the lack of reproducibility and comparability. This study aims to assess the quality of studies reporting clinical and surgical outcomes for bAVMs treated by open surgery and develop a reporting guideline checklist focusing on essential elements to ensure comparability and reproducibility. This is a systematic literature review that followed the PRISMA guidelines with the search in Medline, Embase, and Web of Science databases, for studies published between January 1, 2018, and December 1, 2023. Included studies were scrutinized focusing on seven domains: (1) Assessment of How Studies Reported on the Baseline Characteristics of the Patient Sample; (2) Assessment and reporting on bAVMs grading, anatomical characteristics, and radiological aspects; (3) Angioarchitecture Assessment and Reporting; (4) Reporting on Pivotal Concepts Definitions; (5) Reporting on Neurosurgeon(s) and Staff Characteristics; (6) Reporting on Surgical Details; (7) Assessing and Reporting Clinical and Surgical Outcomes and AEs. A total of 47 studies comprising 5,884 patients were included. The scrutiny of the studies identified that the current literature in bAVM open surgery is deficient in many aspects, ranging from fundamental pieces of information of methodology to baseline characteristics of included patients and data reporting. Included studies demonstrated a lack of reproducibility that hinders building cumulative evidence. A bAVM Open Surgery Reporting Guideline with 65 items distributed across eight domains was developed and is proposed in this study aiming to address these shortcomings. This systematic review identified that the available literature regarding microsurgery for bAVM treatment, particularly in studies reporting clinical and surgical outcomes, lacks rigorous scientific methodology and quality in reporting. The proposed bAVM Open Surgery Reporting Guideline covers all essential aspects and is a potential solution to address these shortcomings and increase transparency, comparability, and reproducibility in this scenario. This proposal aims to advance the level of evidence and enhance knowledge regarding the Open Surgery treatment for bAVMs.

Unruptured Brain Arteriovenous Malformations: A Systematic Review and Meta-Analysis of Mortality and Morbidity in Aruba-Eligible Studies.

BACKGROUND: Treating unruptured brain arteriovenous malformations (bAVMs) represent significant challenges, with numerous uncertainties still in debate. The ARUBA trial induced further investigation into optimal management strategies for these lesions. Here, we present a systematic-review and meta-analysis focusing on ARUBA-eligible studies, aiming to correlate patient data with outcomes and discuss key aspects of these studies. METHODS: Following PRISMA guidelines, we conducted a systematic-review. Variables analyzed included bAVM Spetzler-Martin (SM) grade, treatment modalities, and outcomes such as mortality and neurological deficits. We compared studies with a minimum of 50% cases classified as SM 1-2 lesions and those with less than 50% in this category. Similarly, a comparison between studies with at least 50% microsurgery-cases and those with less than 50% was performed. We examined correlations between mortality incidence, SM distribution, and treatment modalities. RESULTS: Our analysis included 16 studies with 2.417 patients. The frequency of bAVMs SM-grade 1-2 ranged from 44% to 76%, SM-grade 3 from 19% to 48%, and SM 4-5 from 5 to 23%. Notably, studies with more than 50% cases presenting lesions SM-grade 1-2 presented significantly lower mortality rates than those with less than 50% cases of SM 1-2 lesions (P < 0.001). No significant difference in mortality rates or neurological deficits was identified between studies with more than 50% of microsurgery-cases and those with less than 50%. CONCLUSIONS: The analysis revealed that studies with a higher proportion of bAVMs presenting SM 1-2 lesions were associated with lower mortality rates. Mortality did not show a significant association with treatment modalities.

Prevalence and Predictors of Hereditary Hemorrhagic Telangiectasia and Capillary-Malformation Arteriovenous Malformation Syndrome Among Children with Neurovascular Malformations.

OBJECTIVE: To investigate the prevalence and predictors of hereditary hemorrhagic telangiectasia (HHT) and capillary-malformation arteriovenous malformation (CM-AVM) syndrome among children with no prior personal or family history of these diseases who presented with an arteriovenous shunt lesion. STUDY DESIGN: A retrospective chart review was completed on patients aged 0 through 21 years with arteriovenous shunt lesions evaluated at our Cerebrovascular Center. Diagnosis of definite or suspected HHT or CM-AVM was based on clinical features and genetic testing. Associations between final diagnosis and type and number of lesions, epistaxis, telangiectasias, CM, and pulmonary AVMs were assessed. RESULTS: Eighty-nine patients were included. Thirteen (14.6%) had definite HHT, 11 (12.4%) suspected HHT, and 4 (4.5%) definite CM-AVM. Having ≥2 episodes of epistaxis/year and ≥ 2 sites with telangiectasias were each associated with definite HHT (P < .001). Having ≥ 2 CM was associated with definite CM-AVM (P < .001). Pulmonary AVM was associated with increased odds of having definite HHT (OR = 6.3, 95% CI: 1.2-33.4). Multiple lesions (OR = 24.5, 95% CI: 4.5-134.8) and arteriovenous fistulas (OR = 6.2, 95% CI: 1.9-20.3) each increased the likelihood of having definite HHT or CM-AVM. Genetic testing was positive in 31% of patients tested. CONCLUSIONS: We recommend that children with neurovascular shunt lesions be offered genetic testing and undergo further evaluation for HHT and CM-AVM. Awareness and early diagnosis of these conditions is a critical step toward improving long-term outcomes and preventing disease-associated complications.

Comparative Study of Automated Algorithms for Brain Arteriovenous Malformation Nidus Extent Identification Using 3DRA.

PURPOSE: When performing a brain arteriovenous malformation (bAVMs) intervention, computer-assisted analysis of bAVMs can aid clinicians in planning precise therapeutic alternatives. Therefore, we aim to assess currently available methods for bAVMs nidus extent identification over 3DRA. To this end, we establish a unified framework to contrast them over the same dataset, fully automatising the workflows. MATERIALS AND METHODS: We retrospectively collected contrast-enhanced 3DRA scans of patients with bAVMs. A segmentation network was used to automatically acquire the brain vessels segmentation for each case. We applied the nidus extent identification algorithms over each of the segmentations, computing overlap measurements against manual nidus delineations. RESULTS: We evaluated the methods over a private dataset with 22 3DRA scans of individuals with bAVMs. The best-performing alternatives resulted in [Formula: see text] and [Formula: see text] dice coefficient values. CONCLUSIONS: The mathematical morphology-based approach showed higher robustness through inter-case variability. The skeleton-based approach leverages the skeleton topomorphology characteristics, while being highly sensitive to anatomical variations and the skeletonisation method employed. Overall, nidus extent identification algorithms are also limited by the quality of the raw volume, as the consequent imprecise vessel segmentation will hinder their results. Performance of the available alternatives remains subpar. This analysis allows for a better understanding of the current limitations.

Pediatric Interventional Neuroradiology.

Pediatric neurovascular disorders can lead to substantial mortality and morbidity if not diagnosed early and adequately managed. Children with neurovascular diseases cannot be treated as small adults as the vascular and central nervous system anatomy, physiology, and pathologies in children differ greatly from those of adults. In addition, some neurovascular pathologies are seen exclusively in children such as aneurysmal malformation of the vein of Galen, pial fistulas, and dural fistulas in the context of dural sinus disease. In this review, we aim to present an overview of the common pediatric neurovascular diseases along with their endovascular management.

Stereotactic Radiosurgery or Fractionated Stereotactic Radiotherapy for Arteriovenous Malformation.

BACKGROUND: The best management for AVM, particularly high-grade ones and those that have been ruptured before, is still unknown. Data from prospective data lacks support for the best approach. METHODS: We retrospectively review patients with AVM at a single institution that were treated with radiation or a combination of radiation and embolization. These patients were divided into two groups based on radiation fractionation: SRS and fSRS. RESULTS: One-hundred and thirty-five (135) patients were first assessed and 121 met study criteria. Mean age at treatment was 30.5 years, and most patients were male. The groups were otherwise balanced, except for nidus size. SRS group had smaller lesions (P > 0.005). SRS correlates to better chance of nidus occlusion and lesser chance of retreatment. Complications such as radionecrosis (5%) and bleeding after nidus occlusion (1 patient) were rare. CONCLUSIONS: Stereotactic radiosurgery plays an important role on the treatment of AVM. Whenever possible, SRS should be preferred. Data from prospective trials about larger and previously ruptured lesions are needed.

Microsurgical Resection of Tonsillar Arteriovenous Malformation: Operative Video.

Cerebellar arteriovenous malformation (AVM) comprises 10%-15% of intracranial AVMs.1 Rupture often leads to devastating brainstem compression, with mortality reported as high as 67%.2 AVM can be a challenging disease, especially when large in size.3 AVMs can be treated by 1 or a combination of treatment modalities, namely embolization, radiosurgery, or microsurgical resection.4,5 Arterial adhesions to tonsilobulbar and telovelonsilar segments of posterior inferior cerebellar artery (PICA) can be a challenge, increasing bleeding and ischemic risk.6 We present a 2-dimensional video case of a tonsillar AVM. The patient, a previously healthy female in her 20s, presented with a chronic headache. She had no medical history. Initial magnetic resonance imaging revealed a tonsillar AVM classified as Spetzler-Martin grade II. It received its supply from the tonsilobulbar and telovelotonsilar segments of the PICA and drained directly into the precentral vein, transverse sinus, and sigmoid sinus. An angiogram revealed severe venous engorgement-the source of the patient's headache. The AVM was partially embolized 1 month preoperatively. A medial suboccipital telovelar approach was chosen to reduce the working distance and afford a wider corridor to expose the suboccipital surface of the cerebellum.7,8 Complete resection of the AVM was achieved with no additional morbidity. Microsurgery in experienced hands offers the best chance of cure for AVMs. In Video 1, we demonstrate the relationships among the tonsila, biventral lobule, vallecula cerebelli, PICA, and cerebellomedullary fissure as an important anatomic landmark in a safe total resection of a tonsillar AVM.

Posterior-Transcallosal Approach to a Choroidal Fissure Arteriovenous Malformation: Video of Adapting a "Bridging Vein Free" Corridor to This Complex Region.

Choroidal fissure arteriovenous malformations (ChFis-AVMs) are uncommon and challenging to treat due to their deep location and pattern of supply.1 The choroidal fissure lies between the thalamus and fornix, from the foramen of Monroe to the inferior choroidal point.2 AVMs in this location receive their supply from the anterior, lateral posterior choroidal artery and medial posterior choroidal arteries and drain to the deep venous system.3 The anterior-transcallosal corridor to the ChFis is favored due to the ease in opening the taenia fornicis from the foramen Monroe, and it increases in length for lesions located more posteriorly.4-7 We present a case of a posterior ChFis-AVM. The patient, a previously healthy woman in her 20s, presented with a sudden severe headache. She was diagnosed with intraventricular hemorrhage. This was managed conservatively with subsequent magnetic resonance imaging and digital subtraction angiography revealing a ChFis-AVM at the body of the left lateral ventricle, between the fornix and superior layer of the tela choroidae. It received its supply from the left lateral posterior choroidal artery and medial posterior choroidal artery and drained directly into the internal cerebral vein, classified as Spetzler-Martin grade II.8 A posterior-transcallosal approach to the ChFis was chosen to reduce the working distance and afford a wider corridor by avoiding cortical bridging veins (Video 1). Complete resection of the AVM was achieved with no additional morbidity. Microsurgery in experienced hands offers the best chance of cure for AVMs.9 In this case we demonstrate how to adapt the transcallosal corridor to the choroidal fissures for safe AVM surgery in this complex location.

Sphenoidal meningoencephalocele associated with CSF fistula and arteriovenous malformation Spetzler-Martin V: A case report.

Cerebral Arteriovenous malformations (AVMs) are presumed congenital anomalies of the blood vessels, which can increase intracranial pressure by uncertain mechanisms. We report the rare case of a 55-year-old male patient who complained about CSF rhinorrhea. Persisting CSF leakage prompted CT, which evidenced a bone defect in the right middle cranial fossa with protruding brain tissue. The diagnosis of a sphenoidal meningoencephalocele was made. Neuroimaging evidenced an AVM Spetzler Martin V. The lesion was targeted via an endonasal approach with resection of the herniated brain tissue and closure of the bony and dural defects. The postoperative course was uneventful without recurrence of the CSF fistula. Documentation of these cases is essential to come up with standardized therapeutical protocols and follow-up. Nevertheless, conservative management of the AVM and surgical repair of the bone defects is an appropriate approach in the first instance, depending on the morphology and characterization of the AVM.

Importance of Arachnoid Dissection in Arteriovenous Malformation Microsurgery: A Technical Note.

Intracranial arteriovenous malformations (AVMs) are congenital anomalies where arteries and veins connect without a capillary bed. AVMs are the leading cause of nontraumatic intracerebral hemorrhages in people younger than 35 years old.1 The leptomeninges (arachnoid and pia) form from the meninx primitiva.2,3 Endothelial channels produce a vascular plexus in the meninx connected by primitive arachnoid. Remodeling of the plexus in response to changing metabolic demands results in a recognizable pattern of arteries and veins.2,3 Defects at the level of capillaries during arteriovenous specification are most likely responsible for arteriovenous fistula formation.4-6 Interplay between the congenital dysfunction and flow-related maturation in adulthood, when vasculogenesis has stopped, produces the AVM.6,7 The relationship between the primitive arachnoid and aberrant AVM vessels is preserved and forms the basis of microsurgical disconnection discussed in Video 1. Several authors have described dissecting these natural planes to delineate the abnormal AVM vessels, relax the brain, and avoid morbidity during AVM surgery.8-10 We recommend sharp arachnoid dissection with a scalpel or microscissors, occasionally helped by blunt dissection with patties or bipolar forceps. We present a 2-dimensional video of the microsurgical resection of a right parietal AVM. The patient, a healthy 30-year-old female, presented with intermittent headaches and mild impairment of arithmetic and visuospatial ability. Magnetic resonance imaging and digital subtraction angiography showed a compact 3.5-cm supramarginal gyrus AVM supplied by the middle cerebral artery, with superficial drainage. Complete microsurgical resection was performed without morbidity. We demonstrate the principles of arachnoid dissection requisite to disentanglement of the nidus and safe resection of the AVM.

Multiple brain arteriovenous malformations: systematic review and individual patient data meta-analysis.

Multiple brain arteriovenous malformations (bAVM) are rare neurovascular lesions usually related to genetic syndromes. Its management is not well established given its rarity. The objective of this study was to describe the clinical and angiographic features of published cases and to explore their associations with treatment outcomes. We performed a literature search of published cases in Medline and the Regional Index Medici. Additional cases were searched in our single-center registry. Data on the proportions of patients and clinical and angiographic characteristics were extracted. The study outcomes were nidal instability in patients who underwent staged treatment and radiological cure in patients who underwent treatment using any treatment modality. Logistic regression models for the study outcomes were analyzed. Data on the proportions of multiple bAVM patients were summarized with meta-analyses of proportions. We included 118 patients (reported in 68 studies) from the literature and 6 cases identified in our registry. A total of 124 patients harboring 339 bAVM nidi were included in the analyses. Differences between syndromic and non-syndromic cases were observed. The logistic regression analyses showed that angiographically occult untreated bAVM was associated (OR 14.37; 95% CI 2.17 to 95.4) with nidal instability after staged treatment, and deep (OR 5.11; 95% CI 1.51 to 17.27) and eloquent (OR 3.91; 95% CI 1.22 to 12.52) locations were associated with residual disease after treatment. Inconsistent reporting of relevant data throughout the included studies undermined the planned analyses. Some prognostic factors were found to be related to the study outcomes. Study Registration: The protocol of the systematic review was registered in PROSPERO as CRD42021245814.