Cerebral Arteriovenous Malformations Research Articles

Current advances in epilepsy among patients with arteriovenous malformations

Epileptic seizures are prevalent in people with brain vascular abnormalities like arteriovenous malformations (AVMs) and cavernous malformations, greatly affecting their quality of life. The connection between intracranial vascular abnormalities and epilepsy is still under debate. Therefore, investigating epilepsy in individuals with AVMs is a crucial and current research area. This review presents a comprehensive examination of recent developments in epilepsy among individuals with brain AVMs. The authors conducted a detailed analysis of the natural progression, epidemiology, diagnostic methods, therapeutic approaches, and post-treatment outcomes for individuals with epilepsy associated with AVMs.

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

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. 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. 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. The clinical and angiographic differences observed between brain AVM groups allow the formulation of profiles according to their location.

A comprehensive analysis of patients with cerebral arteriovenous malformation with headache: assessment of risk factors and treatment effectiveness

BackgroundDue to the high mortality and disability rate of intracranial hemorrhage, headache is not the main focus of research on cerebral arteriovenous malformation (AVM), so research on headaches in AVM is still scarce, and the clinical understanding is shallow. This study aims to delineate the risk factors associated with headaches in AVM and to compare the effectiveness of various intervention treatments versus conservative treatment in alleviating headache symptoms.MethodsThis study conducted a retrospective analysis of AVMs who were treated in our institution from August 2011 to December 2021. Multivariable logistic regression analysis was employed to assess the risk factors for headaches in AVMs with unruptured, non-epileptic. Additionally, the effectiveness of different intervention treatments compared to conservative management in alleviating headaches was evaluated through propensity score matching (PSM).ResultsA total of 946 patients were included in the analysis of risk factors for headaches. Multivariate logistic regression analysis identified that female (OR 1.532, 95% CI 1.173–2.001, p = 0.002), supply artery dilatation (OR 1.423, 95% CI 1.082–1.872, p = 0.012), and occipital lobe (OR 1.785, 95% CI 1.307–2.439, p < 0.001) as independent risk factors for the occurrence of headaches. There were 443 AVMs with headache symptoms. After propensity score matching, the microsurgery group (OR 7.27, 95% CI 2.82–18.7 p < 0.001), stereotactic radiosurgery group(OR 9.46, 95% CI 2.26–39.6, p = 0.002), and multimodality treatment group (OR 8.34 95% CI 2.87–24.3, p < 0.001) demonstrate significant headache relief compared to the conservative group. However, there was no significant difference between the embolization group (OR 2.24 95% CI 0.88–5.69, p = 0.091) and the conservative group.ConclusionsThis study identified potential risk factors for headaches in AVMs and found that microsurgery, stereotactic radiosurgery, and multimodal therapy had significant benefits in headache relief compared to conservative treatment. These findings provide important guidance for clinicians when developing treatment options that can help improve overall treatment outcomes and quality of life for patients.

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 MethodsThis 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. ResultsElective 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. ConclusionsThe 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.

Somatic BrafV600E mutation in the cerebral endothelium induces brain arteriovenous malformations.

Current treatments of brain arteriovenous malformation (BAVM) are associated with considerable risks and at times incomplete efficacy. Therefore, a clinically consistent animal model of BAVM is urgently needed to investigate its underlying biological mechanisms and develop innovative treatment strategies. Notably, existing mouse models have limited utility due to heterogenous and untypical phenotypes of AVM lesions. Here we developed a novel mouse model of sporadic BAVM that is consistent with clinical manifestations in humans. Mice with BrafV600E mutations in brain ECs developed BAVM closely resembled that of human lesions. This strategy successfully induced BAVMs in mice across different age groups and within various brain regions. Pathological features of BAVM were primarily dilated blood vessels with reduced vascular wall stability, accompanied by spontaneous hemorrhage and neuroinflammation. Single-cell sequencing revealed differentially expressed genes that were related to the cytoskeleton, cell motility, and intercellular junctions. Early administration of Dabrafenib was found to be effective in slowing the progression of BAVMs; however, its efficacy in treating established BAVM lesions remained uncertain. Taken together, our proposed approach successfully induced BAVM that closely resembled human BAVM lesions in mice, rendering the model suitable for investigating the pathogenesis of BAVM and assessing potential therapeutic strategies.

Clinical diagnostic reference levels in neuroradiology based on clinical indication.

This study focuses on patient radiation exposure in interventional neuroradiology (INR) procedures, a field that has advanced significantly since its inception in the 1980s. INR employs minimally invasive techniques to treat complex cerebrovascular diseases in the head, neck, and spine. The study establishes diagnostic reference levels (DRLs) for three clinical indications (CIs): stroke (S), brain aneurysms (ANs), and brain arteriovenous malformation (AVM). Data from 209 adult patients were analyzed, and DRLs were determined in terms of various dosimetric and technical quantities. For stroke, the established DRLs median values were found to be 78Gycm2, 378mGy, 118mGy, 12min, 442 images, and 15 runs. Similarly, DRLs for brain AN are 85Gycm2, 611mGy, 95.5mGy, 19.5, 717 images, and 26 runs. For brain AVM, the DRL's are 180Gycm2, 1144mGy, 537mGy, 36min, 1375 images, and 31 runs. Notably, this study is unique in reporting DRLs for specific CIs within INR procedures, providing valuable insights for optimizing patient safety and radiation exposure management.

Lesion-Filling Index from Quantitative DSA Correlates with Hemorrhage of Cerebral AVM.

Rupture is the most life-threatening manifestation of cerebral AVMs. This study aimed to explore the hemodynamic mechanism of AVM rupture. We introduced a new quantitative DSA parameter that can reflect the degree of intranidal blood stasis, called the lesion-filling index. This study examined patients with AVMs who had undergone both DSA and MR imaging between 2013 and 2014. Clinical presentations, angioarchitecture, and hemodynamic parameters generated from quantitative DSA were analyzed using univariate and multivariable logistic regression. The lesion-filling index was defined as the arterial diagnostic window divided by the volume of the AVM. To assess the correlation between the lesion-filling index and rupture, we incorporated the lesion-filling index into 2 published prediction models widely recognized for predicting AVM rupture risk, R2eD and VALE. The DeLong test was used to examine whether the addition of the lesion-filling index improved predictive efficacy. A total of 18 patients with AVMs were included. The mean lesion-filling index values in the ruptured group were higher compared with the unruptured group (390.27 [SD, 919.81] versus 49.40 [SD, 98.25]), P < .001). A higher lesion-filling index was significantly correlated with AVM rupture in 3 different multivariable logistic models, adjusting for angioarchitecture factors (OR = 1.004, P = .02); hemodynamic factors (OR = 1.005, P = .009); and combined factors (OR = 1.004, P = .03). Both R2eD (area under the curve, 0.601 versus 0.624; P = .15) and VALE (area under the curve, 0.603 versus 0.706; P < .001) predictive models showed improved predictive performance after incorporating the lesion-filling index and conducting 10-fold cross-validation. The lesion-filling index showed a strong correlation with AVM rupture, suggesting that overperfusion is the hemodynamic mechanism leading to AVM rupture.

Natural history and clinical manifestation of Pediatric Brain Arteriovenous Malformations.

Brain arteriovenous malformations (bAVMs) are aberrant arteriovenous shunts through a vascular nidus with no intervening capillary beds. They are one of the commonest causes of spontaneous intracranial haemorrhage in children and may be associated with significant morbidity and mortality in cases of rupture. Treatment strategies include microsurgical resection, endovascular embolisation, stereotactic radiosurgery, multimodality treatment with a combination thereof, and particularly in high-grade bAVMs, conservative management. Clinicians involved in treating bAVMs need to have familiarity with the natural history pertaining to bAVMs in terms of risk of rupture, risk factors elevating rupture risk as well as understanding the clinical manifestations of bAVMs. This invited review serves to provide a synthesis on natural history and clinical presentation of bAVMs with particular focus in children to inform decision-making pertaining to management.

Most Promising Approaches to Improve Brain AVM Management: ARISE I Consensus Recommendations.

Brain arteriovenous malformations (bAVMs) are complex, and rare arteriovenous shunts that present with a wide range of signs and symptoms, with intracerebral hemorrhage being the most severe. Despite prior societal position statements, there is no consensus on the management of these lesions. ARISE (Aneurysm/bAVM/cSDH Roundtable Discussion With Industry and Stroke Experts) was convened to discuss evidence-based approaches and enhance our understanding of these complex lesions. ARISE identified the need to develop scales to predict the risk of rupture of bAVMs, and the use of common data elements to perform prospective registries and clinical studies. Additionally, the group underscored the need for comprehensive patient management with specialized centers with expertise in cranial and spinal microsurgery, neurological endovascular surgery, and stereotactic radiosurgery. The collection of prospective multicenter data and gross specimens was deemed essential for improving bAVM characterization, genetic evaluation, and phenotyping. Finally, bAVMs should be managed within a multidisciplinary framework, with clinical studies and research conducted collaboratively across multiple centers, harnessing the collective expertise and centralization of resources.

Angiographic Evolution of Brain Arteriovenous Malformation Angioarchitecture After Partial Endovascular Treatment.

Endovascular embolization of brain arteriovenous malformations (AVMs) is sometimes intentionally partial, in the case of staged treatment for instance. Residual AVMs may be prone to angioarchitectural modification during follow-up. The objective of this work is to evaluate the nature and extent of these modifications. We performed a retrospective monocentric study on a cohort of adult patients treated by incomplete endovascular embolization for ruptured and unruptured AVMs with an available angiographic follow-up, without any intervening confounding event between the 2 angiographic examinations. AVM angioarchitectural modifications (arterial, nidal, and venous) were analyzed. Clinical and radiological data were tested in univariate analyses for association with the occurrence of AVM regression or progression. Eighty-two partial embolization sessions in 57 patients were included in the study. A 40% (33/82) rate of modification was found on follow-up, with 23/82 (28%) controls showing at least one angioarchitectural regression feature and 15/82 (18.3%) showing at least one angioarchitectural progression item. Nidal growth was the most frequent modification occurring after 12/82 (14.6%) embolizations. The only factor associated with nidal volume growth was a longer time interval between embolization and follow-up (median [IQR]: 190 [250] days vs 89.5[133] days in the subgroup without nidal growth; P = .02). Specific modifications of arterial supply, nidal anatomy, and venous drainage were identified and documented. Angioarchitectural modifications (both progression and regression) of brain AVMs are frequent findings after partial embolization. Nidal volume growth is associated with longer time intervals between embolization and follow-up.

Posterior Interhemispheric-Transtentorial Approach for Resection of an Arteriovenous Malformation of the Superior Medullary Velum and Fourth Ventricle: 2-Dimensional Operative Video.

Brain arteriovenous malformations (AVMs) of the fourth ventricle represent a rare subtype associated with an aggressive natural course.1,2 In this case, a woman in her early 50s presented with dizziness. An AVM was diagnosed in the left superior cerebellar peduncle extending into the fourth ventricle. The AVM was supplied by superior cerebellar artery branches and classified as a Spetzler-Martin grade III and a Lawton-Young grade III, with a supplemented grade of 6.3,4 Being a single case report, institutional review board approval was not needed. Patient consent was obtained. The lesion was accessed through a torcular craniotomy and posterior interhemispheric-transtentorial approach, employing gravity to naturally retract the parietooccipital lobe.5-7 Dissection continued into the quadrigeminal and ambient cisterns, where the tentorium was incised parallelling the straight sinus to reach the superior vermis. Partial resection of the lingual and central lobules of the vermis facilitated access to the superior medullary velum. The superior cerebellar artery feeders were divided and followed to the superior cerebellar peduncle and through the superior medullary vellum. A vertical incision in the superior medullary velum facilitated entry into the fourth ventricle, where the AVM nidus was dissected circumferentially and resected en bloc. Intraoperative indocyanine green videoangiography and postoperative digital subtraction angiography confirmed complete obliteration of the AVM. After surgery, the patient experienced mild ataxia, but motor symptoms greatly improved during 3-month follow-up. This video illustrates resection of a complex fourth ventricular AVM through a posterior interhemispheric-transtentorial approach, highlighting pivotal considerations of patient positioning and approach selection to optimize treatment outcome for complex posterior fossa AVM resection.

Implementation of 3D modelling to improve understanding and conceptualisation of arteriovenous malformation (AVM) morphology for the execution of safe microsurgical excision of complex paediatric AVMs.

Brain arteriovenous malformations (bAVMs) present complex challenges in neurosurgery, requiring precise pre-surgical planning. In this context, 3D printing technology has emerged as a promising tool to aid in understanding bAVM morphology and enhance surgical outcomes, particularly in pediatric patients. This study aims to assess the feasibility and effectiveness of using 3D AVM models in pediatric bAVM surgery. The study was conducted at Great Ormond Street Hospital, and cases were selected sequentially between October 2021 and February 2023. Eight pediatric bAVM cases with 3D models were compared to eight cases treated before the introduction of 3D printing models. The 3D modelling fidelity and clinical outcomes were assessed and compared between the two cohorts. The study demonstrated excellent fidelity between 3D models and actual operative anatomy, with a median difference of only 0.31mm. There was no statistically significant difference in angiographic cure rates or complications between the 3D model group and the non-3D model group. Surgical time showed a non-significant increase in cases involving 3D models. Furthermore, the 3D model cohort included higher-grade bAVMs, indicating increased surgical confidence. This study demonstrates the feasibility and efficacy of utilizing 3D AVM models in pediatric bAVM surgery. The high fidelity between the models and actual operative anatomy suggests that 3D modelling can enhance pre-surgical planning and intraoperative guidance without significantly increasing surgical times or complications. Further research with larger cohorts is warranted to confirm and refine the application of 3D modelling in clinical practice.

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

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. 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). 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). 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.

A Case of Cerebral Arteriovenous Malformation and Malformation of the Lower Limbs

A Case of Cerebral Arteriovenous Malformation and Malformation of the Lower Limbs

Phenotypic characterisation of SMAD4 variant carriers

BackgroundBoth hereditary haemorrhagic telangiectasia (HHT) and juvenile polyposis syndrome (JPS) are known to be caused by SMAD4 pathogenic variants, with overlapping symptoms for both disorders in some patients. Additional connective...

400 Brain AVMs-Related MicroRNAs: Machine Learning Algorithm for Expression Profiles of Target Genes

INTRODUCTION: microRNAs (miRNAs) are a class of non-coding RNAs playing a myriad of important roles in regulating gene expression. Of note, recent work demonstrated a critical role of miRNAs in the genesis and progression of brain arteriovenous malformations (bAVMs). METHODS: A PRISMA-based literature review was performed using PubMed/Medline database with the following search terms: "brain arteriovenous malformations", "cerebral arteriovenous malformations", "microRNA", and "miRNA". All preclinical and clinical studies written in English, regardless of date, were selected. For our bioinformatic analyses, miRWalk and miRTarBase machine learning algorithms were employed; the Kyoto Encyclopedia of Genes and Genomes (KEGG) database was quired for associated pathways/functions. RESULTS: 4 studies were ultimately included in the final analyses. Sequencing data consistently revealed the decreased expression of miR-18a in bAVM-endothelial cells, resulting in increased levels of vascular endodermal growth factor (VEGF), Id-1, matrix metalloproteinase, and growth signals. Our analyses also suggest that the downregulation of miR-137 and miR-195* within vascular smooth muscle cells (VSMCs) may foster the activation of inflammation, aberrant angiogenesis, and phenotypic switching. In the peripheral blood, the overexpression of miR-7-5p, miR-629-5p, miR-199a-5p, miR-200b-3p, and let-7b-5p may contribute to endothelial proliferation and nidus development. The machine learning algorithms employed confirmed associations between miRNA-related target networks, vascular rearrangement, and bAVM progression. CONCLUSIONS: miRNAs expression appears to be critical in managing bAVMs' post-transcriptional signals. Targets of microRNAs regulate canonical vascular proliferation and reshaping. Although additional scientific evidence is needed, the identification of bAVM miRNA signatures may facilitate the development of novel prognostic/diagnostic tools and molecular therapies for bAVMs.

160 Gamme Knife Radio Surgery for Cerebral Arteriovenous Malformation (AVM) in Children: A Systematic Review and Meta-analysis of Clinical Outcomes

INTRODUCTION: As radiosurgical devices are becoming more commonly used and applied to treat cerebral arteriovenous malformations (cAVMs), there is a growing concern about whether the outcomes of radiosurgery might differ between children, adolescents, and adults due to the distinct characteristics of cAVMs in these age groups. METHODS: Search: A search for RCT, not RCT, prospective and retrospective cohort studies in PUBMED; SCOPUS; Central Cochrane Registry ofControlled Trials (The Cochrane Library); MEDLINE; EMBASE (Ovid); CINAHL. The search strategy include subject headings (MeSH) and text words connected with Booleans term. Population: Children (younger than 18 years old) with Cerebral Arterio-venous Malformation (AVMs). Intervention: Gamma Knife radiosurgery (GKS). Outcomes: Primary Outcome: Mortality to end of Follow-up and Surgery successfull determined by the percentage of obliterated AVMs. Secondary Outcome: New Neurological deterioration and Post-Radiosurgery intracranial hemorrhage. RESULTS: 21 studies with 2142 children who had Gamma knife surgery for cerebral arteriovenous malformations were found for this systematic review. For children with cerebral AVMs treat with GKS, the death rate was 0.725% (95% CI 0.411%-1.126%; I2 = 0%) while the success rate of gamma knife radiosurgery was 71.74% (95% CI 65.716%-77.211%; I2 = 88.13). The rate of complications, such as the rate of new neurological deficits, was 2.57% (1.43% to 3.89%; I2 = 66.78%), and the postoperative intracranial bleeding rate was 2.463% (1.348% to 3.9%; I2 = 70.14%). CONCLUSIONS: Gamma Knife radiosurgery is a safe and effective treatment option for cerebral arteriovenous malformations, with high surgical success rates, low mortality, and low complication rates. However, further high-quality case-control studies are needed to evaluate its long-term efficacy and safety.

Shifts in Diagnostic Testing for Headache in the Emergency Department, 2015 to 2021

Subarachnoid hemorrhage is typically diagnosed by noncontrast head computed tomography (CT); lumbar puncture is recommended if computed tomography is nondiagnostic, although CT cerebral angiography has been promoted as an alternative to lumbar puncture in this diagnostic pathway. The outcomes of this debate in practice have not been studied. To determine whether CT cerebral angiography use has increased in lieu of lumbar puncture among emergency department (ED) patients with headache, with an increase in unruptured intracranial aneurysm detection. This retrospective cohort study took place in 21 community EDs of an integrated health care system in Northern California between 2015 and 2021. Participants were adult (aged >17 years) health plan members with a chief concern of headache. Exclusions were prior diagnoses of subarachnoid hemorrhage, unruptured intracranial aneurysm, cerebral arteriovenous malformation, or cerebrospinal fluid shunt. Data were analyzed from October to November 2023. CT cerebral angiography and/or lumbar puncture during the ED encounter. Primary and secondary outcomes were 14-day and 90-day unruptured intracranial aneurysm detection, respectively. Safety outcomes were missed diagnoses of subarachnoid hemorrhage or bacterial meningitis. The annual incidence of unruptured intracranial aneurysm detection was normalized to the incidence of subarachnoid hemorrhage (UIA:SAH ratio). Average annualized percentage changes were quantified using joinpoint regression analysis. Among 198 109 included ED encounters, the mean (SD) age was 47.5 (18.4) years; 140 001 patients (70.7%) were female; 29 035 (14.7%) were Black or African American, 59 896 (30.2%) were Hispanic or Latino, and 75 602 (38.2%) were White. Per year, CT cerebral angiography use increased (18.8%; 95% CI, 17.7% to 20.3%) and lumbar punctures decreased (-11.1%; 95% CI, -12.0% to -10.4%), with a corresponding increase in the 14-day UIA:SAH ratio (3.5%; 95% CI, 0.9% to 7.4%). Overall, computed tomography cerebral angiography use increased 6-fold relative to lumbar puncture, with a 33% increase in the detection of UIA. Results were similar at 90 days and robust to sensitivity analyses. Subarachnoid hemorrhage (1004 cases) and bacterial meningitis (118 cases) were misdiagnosed in 5% and 18% of cases, respectively, with no annual trends (P = .34; z1003 = .95 and P = .74; z117 = -.34, respectively). In this cohort study of ED patients with headache, increases in CT cerebral angiography use were associated with fewer lumbar punctures and higher detection of unruptured intracranial aneurysms, with no significant change in missed diagnoses of subarachnoid hemorrhage or bacterial meningitis. While this shift in diagnostic strategy appeared safe in the short-term, the long-term consequences remain unclear.

691 Correlation of Cerebral Arteriovenous Malformation Flow Measured by Quantitative Magnetic Resonance Angiography and MR-detected Arteriovenous Malformation Microhemorrhage

INTRODUCTION: Intracranial hemorrhage (ICH) significantly contributes to morbidity and mortality in cerebral arteriovenous malformations (AVM) patients, with a history of prior hemorrhage being the most significant predictor of a future hemorrhage. Recently, silent microhemorrhage as detected on magnetic resonance imaging (MRI) has been proposed as a potential risk factor for future hemorrhage in AVM patients. Recent data suggests that silent microhemorrhage is associated with higher nidal velocity measured through color-coded DSA, raising the question of correlation between flow and microhemorrhage. METHODS: All unruptured AVM patients with a baseline QMRA with noninvasive optimal vessel analysis (NOVA) and gradient echo or susceptibility-weighted MRI were retrospectively reviewed (2004-2022). AVM flow was calculated from the aggregate flow within primary arterial feeders relative to their contralateral counterparts. A review of the MRI determined the presence of microhemorrhages. Descriptive statistics, the X2 test, and a binomial logistic regression were performed to test the association of demographics, clinical, and AVM features with microhemorrhage. RESULTS: The study consisted of ninety patients, with fifty microhemorrhage-positive and forty microhemorrhage-negative patients. The presence of venous anomaly (varix, ectasia, aneurysm) is independently predictive of microhemorrhage (OR = 2.444, p = 0.050), and a compact AVM nidus trended towards independent negative predictability of microhemorrhage (OR =.462, p = 0.081). Calculated AVM flow trends higher in the microhemorrhage-positive group with the median flow 407 mL/min vs. 238 mL/min, and a trend towards predicting presence of microhemorrhage (OR = 1.375, p = 0.106). CONCLUSIONS: Patients with AVM-associated venous anomalies have an increased likelihood of AVM microhemorrhage. Higher AVM flow and a diffuse AVM nidus trend towards a higher likelihood of AVM microhemorrhage. These findings demonstrate the possible relationship between higher AVM flow and risk of future bleeding.

313 Development and Validation of a Scoring System for Hemorrhage Risk in Brain Arteriovenous Malformations

INTRODUCTION: The dilemma between natural rupture risk and adverse outcomes of intervention is of major concern for patients with unruptured arteriovenous malformations (AVMs). The existing risk score for AVM rupture includes factors that are controversial and lacks prospective validation. METHODS: This prognostic study developed a prediction model derived from a single-center cohort (derivation cohort) and validated in a multicenter external validation cohort and a conservative treatment validation cohort. Patients were recruited from a nationwide multicenter prospective collaboration registry in China. A total of 4135 patients were enrolled. 3962 patients were included (3585 in the derivation cohort and 377 in the multicenter external validation cohort); 1028 patients from the derivation cohort who had time-to-event data and prerupture imaging results were included in the conservative treatment validation cohort. RESULTS: Four risk factors were used to develop the scoring system: ventricular system involvement, venous aneurysm, deep location, and exclusively deep drainage (VALE). The VALE scoring system performed well in all 3 cohorts, with areas under the receiver operating characteristic curve of 0.77 (95% CI, 0.75-0.78) in the derivation cohort, 0.85 (95% CI, 0.81-0.89) in the multicenter external validation cohort, and 0.73 (95% CI, 0.65-0.81) in the conservative treatment validation cohort. The 10-year hemorrhage-free rate was 95.5% (95% CI, 87.1%-100%) in the low-risk group, 92.8% (95% CI, 88.8%-97.0%) in the moderate-risk group, and 75.8% (95% CI, 65.1%-88.3%) in the high-risk group; the model discrimination was significant when comparing these rates between the high-risk group and the low- and moderate-risk groups (p &lt; .001 for both comparisons). CONCLUSIONS: In this prognostic study, the VALE scoring system was developed to distinguish rupture risk among patients with AVMs. The stratification of unruptured AVMs may enable patients with low risk of rupture to avoid unnecessary interventions.