[Expert consensus on multidisciplinary diagnosis and treatment of brain arteriovenous malformation].

Brain arteriovenous malformation (BAVM) is a cerebral vasculature disorder caused by gene mutation. Current available treatment measures include surgical resection, interventional embolization and stereotactic radiosurgery. The three therapeutic methods have their own advantages for different vascular structures.However, due to the complex vascular architecture of the lesion and its close anatomical relationship with brain tissue, any single treatment can not safely and effectively treat all BAVM cases. Therefore, in order to better regulate and guide the clinical diagnosis and treatment of BAVM patients in China, the National Medical School for Neurological Diseases, the Professional Committee of Neurointervention of the Chinese Medical Doctor Association and the radio-neurosurgery Expert Committee of the World Chinese Neurosurgeons Association jointly discussed and formulated this expert consensus. After in-depth analysis of the evidence of evidence-based medicine at home and abroad, the expert group combined with the specific situation of China, and gave 33 recommendations on specific clinical diagnosis and treatment issues such as predictive factors of cerebral arteriovenous malformation hemorrhage, clinical risks during pregnancy, imaging diagnosis measures, and clinical treatment strategies, in order to provide guidance for the diagnosis and treatment of BAVM nationwide.

CNS resident macrophages enhance dysfunctional angiogenesis and circulating monocytes infiltration in brain arteriovenous malformation.

Myeloid immune cells are abundant in both ruptured and unruptured brain arteriovenous malformations (bAVMs). The role of central nervous system (CNS) resident and circulating monocyte-derived macrophages in bAVM pathogenesis has not been fully understood. We hypothesize that CNS resident macrophages enhance bAVM development and hemorrhage. RNA sequencing using cultured endothelial cells (ECs) and mouse bAVM samples revealed that downregulation of two bAVM causative genes, activin-like kinase 1 (ALK1) or endoglin, increased inflammation and innate immune signaling. To understand the role of CNS resident macrophages in bAVM development and hemorrhage, we administrated a colony-stimulating factor 1 receptor inhibitor to bAVM mice with brain focal Alk1 deletion. Transient depletion of CNS resident macrophages at an early stage of bAVM development mitigated the phenotype severity of bAVM, including a prolonged inhibition of angiogenesis, dysplastic vasculature formation, and infiltration of CNS resident and circulating monocyte-derived macrophages during bAVM development. Transient depletion of CNS resident macrophages increased EC tight junction protein expression, reduced the number of dysplasia vessels and severe hemorrhage in established bAVMs. Thus, EC AVM causative gene mutation can activate CNS resident macrophages promoting bAVM progression. CNS resident macrophage could be a therapeutic target to mitigate the development and severity of bAVMs.

Effect of Cobalt-60 Treatment Dose Rate on Arteriovenous Malformation Obliteration After Stereotactic Radiosurgery With Gamma Knife.

BACKGROUND AND OBJECTIVES: Stereotactic radiosurgery (SRS) marginal dose is associated with successful obliteration of cerebral arteriovenous malformations (AVM). SRS dose rate-how old the cobalt-60 sources are-is known to influence outcomes for some neurological conditions and benign tumors. The objective of this study was to determine the association between cobalt-60 treatment dose rate and cerebral AVM obliteration in patients treated with SRS. METHODS: We performed a retrospective cohort study of 361 patients undergoing 411 AVM-directed SRS treatments between 2005 and 2019 at a single institution. Lesion characteristics, SRS details, obliteration dates, and post-treatment toxicities were recorded. Univariate and multivariate regression analyses of AVM outcomes regarding SRS dose rate (range 1.3-3.7 Gy, mean = 2.4 Gy, median = 2.5 Gy) were performed. RESULTS: At 10 years post-SRS, 68% of AVMs were obliterated on follow-up imaging. Dose rates >2.9 Gy/min were found to be significantly associated with AVM obliteration compared with those <2.1 Gy/min ( P = .034). AVM size, biologically effective dose, and SRS marginal dose were also associated with obliteration, with obliteration more likely for smaller lesions, higher biologically effective dose, and higher marginal dose. Higher dose rates were not associated with the development of post-SRS radiological or symptomatic edema, although larger AVM volume was associated with both types of edema. CONCLUSION: Patients with cerebral AVMs treated with higher SRS dose rates (from newer cobalt-60 sources) experience higher incidences of obliteration without a significant change in the risk of post-treatment edema.

Evolution of clinical and translational advances in the management of pediatric arteriovenous malformations.

Arteriovenous malformations (AVMs) represent one of the most challenging diagnoses in pediatric neurosurgery. Until recently, the majority of AVMs was only identified after hemorrhage and primarily treated with surgery. However, recent advances in a wide range of fields-imaging, surgery, interventional radiology, radiation therapy, and molecular biology-have profoundly advanced the understanding and therapy of these complex lesions. Here we review the progress made in pediatric AVMs with a specific focus on innovations relevant to clinical care.

Key biomarkers in cerebral arteriovenous malformations: Updated review.

The formation of vascular networks consisting of arteries, capillaries, and veins is vital in embryogenesis. It is also crucial in adulthood for the formation of a functional vasculature. Cerebral arteriovenous malformations (CAVMs) are linked with a remarkable risk of intracerebral hemorrhage because arterial blood is directly shunted into the veins before the arterial blood pressure is dissipated. The underlying mechanisms responsible for arteriovenous malformation (AVM) growth, progression, and rupture are not fully known, yet the critical role of inflammation in AVM pathogenesis has been noted. The proinflammatory cytokines are upregulated in CAVM, which stimulates overexpression of cell adhesion molecules in endothelial cells (ECs), leading to improved leukocyte recruitment. It is well-known that metalloproteinase-9 secretion by leukocytes disrupts CAVM walls resulting in rupture. Moreover, inflammation alters the angioarchitecture of CAVMs by upregulating angiogenic factors impacting the apoptosis, migration, and proliferation of ECs. A better understanding of the molecular signature of CAVM might allow us to identify biomarkers predicting this complication, acting as a goal for further investigations that may be potentially targeted in gene therapy. The present review is focused on the numerous studies conducted on the molecular signature of CAVM and the associated hemorrhage. The association of numerous molecular signatures with a higher risk of CAVM rupture is shown through inducing proinflammatory mediators, as well as growth factors signaling, Ras-mitogen-activated protein kinase-extracellular signal-regulated kinase, and NOTCH pathways, which are accompanied by cellular level inflammation and endothelial alterations resulting in vascular wall instability. According to the studies, it is assumed that matrix metalloproteinase, interleukin-6, and vascular endothelial growth factor are the biomarkers most associated with CAVM and the rate of hemorrhage, as well as diagnostic methods, with respect to enhancing the patient-specific risk estimation and improving treatment choices.

Radiological Parameters for Gamma Knife Radiosurgery.

Accurate lesion targeting is the essence of stereotactic radiosurgery. With the currently available imaging modalities, scanning has become quick and robust providing a high degree of spatial resolution resulting in optimal contrast between normal and abnormal tissues. Magnetic resonance imaging (MRI) forms the backbone of Leksell radiosurgery. It produces images with excellent soft tissue details highlighting the target and surrounding "at-risk" structures conspicuously. However, one must be aware of the MRI distortions that may arise during treatment. Computed tomography (CT) has quick acquisition times giving excellent bony information but inferior soft tissue details. To avail benefits of both these modalities and overcome their individual fallacies and shortcomings, they are often co-registered/fused for stereotactic guidance. Vascular lesions like an arteriovenous malformation (AVM) are best planned with cerebral digital subtraction angiography (DSA) in conjunction with MRI. In specific cases, specialized imaging methods like magnetic resonance (MR) spectroscopy, positron emission tomography (PET), magneto-encephalography (MEG), etc., may be added to the treatment planning for stereotactic radiosurgery (SRS).

Segmentation techniques of brain arteriovenous malformations for 3D visualization: a systematic review.

BACKGROUND: Visualization, analysis and characterization of the angioarchitecture of a brain arteriovenous malformation (bAVM) present crucial steps for understanding and management of these complex lesions. Three-dimensional (3D) segmentation and 3D visualization of bAVMs play hereby a significant role. We performed a systematic review regarding currently available 3D segmentation and visualization techniques for bAVMs. METHODS: PubMed, Embase and Google Scholar were searched to identify studies reporting 3D segmentation techniques applied to bAVM characterization. Category of input scan, segmentation (automatic, semiautomatic, manual), time needed for segmentation and 3D visualization techniques were noted. RESULTS: Thirty-three studies were included. Thirteen (39%) used MRI as baseline imaging modality, 9 used DSA (27%), and 7 used CT (21%). Segmentation through automatic algorithms was used in 20 (61%), semiautomatic segmentation in 6 (18%), and manual segmentation in 7 (21%) studies. Median automatic segmentation time was 10 min (IQR 33), semiautomatic 25 min (IQR 73). Manual segmentation time was reported in only one study, with the mean of 5-10 min. Thirty-two (97%) studies used screens to visualize the 3D segmentations outcomes and 1 (3%) study utilized a heads-up display (HUD). Integration with mixed reality was used in 4 studies (12%). CONCLUSIONS: A golden standard for 3D visualization of bAVMs does not exist. This review describes a tendency over time to base segmentation on algorithms trained with machine learning. Unsupervised fuzzy-based algorithms thereby stand out as potential preferred strategy. Continued efforts will be necessary to improve algorithms, integrate complete hemodynamic assessment and find innovative tools for tridimensional visualization.

Plasma Matrix Metalloproeteinase-9 Is Associated with Seizure and Angioarchitecture Changes in Brain Arteriovenous Malformations.

Both angiogenesis and inflammation contribute to activation of matrix metalloproeteinase-9 (MMP-9), which dissolves the extracellular matrix, disrupts the blood-brain barrier, and plays an important role in the pathogenesis of brain arteriovenous malformations (BAVMs). The key common cytokine in both angiogenesis and inflammation is interleukin 6 (IL-6). Previous studies have shown elevated systemic MMP-9 and decreased systemic vascular endothelial growth factor (VEGF) in BAVM patients. However, the clinical utility of plasma cytokines is unclear. The purpose of this study is to explore the relationship between plasma cytokines and the clinical presentations of BAVMs. Prospectively, we recruited naive BAVM patients without hemorrhage as the experimental group and unruptured intracranial aneurysm (UIA) patients as the control group. All patients received digital subtraction angiography, and plasma cytokines were collected from the lesional common carotid artery. Plasma cytokine levels were determined using a commercially available, monoclonal antibody-based enzyme-linked immunosorbent assay. Subgroup analysis based on hemorrhagic presentation and angiograchitecture was done for the BAVM group. Pearson correlations were calculated for the covariates. Means and differences for continuous and categorical variables were compared using Student's t and χ2 tests respectively. Plasma MMP-9 levels were significantly higher in the BAVM group (42,945 ± 29,991 pg/mL) than in the UIA group (28,270 ± 17,119 pg/mL) (p < 0.001). Plasma MMP-9 levels in epileptic BAVMs (57,065 ± 35,732; n = 9) were higher than in non-epileptic BAVMs (35,032 ± 28,301; n = 19) (p = 0.049). Lower plasma MMP-9 levels were found in cases of BAVM with angiogenesis and with peudophlebitis. Plasma MMP-9 is a good biomarker reflecting ongoing vascular remodeling in BAVMs. Angiogenesis and pseudophlebitis are two angioarchitectural signs that reflect MMP-9 activities and can potentially serve as imaging biomarkers for epileptic BAVMs.

Long-term outcomes of Spetzler-Martin grade IV and V arteriovenous malformations: a single-center experience.

OBJECTIVE: This study aimed to explore whether intervention can benefit Spetzler-Martin (SM) grade IV-V arteriovenous malformations (AVMs). METHODS: Eighty-two patients with SM grade IV-V AVMs were retrospectively reviewed from 2015 to 2018. Patients were divided into two groups: those who received conservative management (22 cases [26.8%]) and intervention (60 cases [73.2%], including 21 cases of microsurgery, 19 embolization, and 20 hybrid surgery). Neurofunctional outcomes were assessed with the modified Rankin Scale (mRS). The primary outcome was long-term neurofunctional status, and the secondary outcomes were short-term neurofunctional status, long-term obliteration rate, seizure control, and risk of subsequent hemorrhage. RESULTS: Regarding the primary outcome, after an average of 4.7 years of clinical follow-up, long-term neurofunctional outcomes were similar after conservative management or intervention (absolute difference -0.4 [95% CI -1.5 to 0.7], OR 0.709 [95% CI 0.461-1.090], p = 0.106), whereas intervention had an advantage over conservative management for avoidance of severe disability (defined as mRS score > 3) (1.7% vs 18.2%, absolute difference 16.5% [95% CI -23.6% to 56.6%], OR 0.076 [95% CI 0.008-0.727], p = 0.025). Regarding the secondary outcomes, intervention was conducive to better seizure control (Engel class I-II) (70.0% vs 0.0%, absolute difference 70.0% [95% CI 8.6%-131.4%], p = 0.010) and avoidance of subsequent hemorrhage (1.4% vs 6.0%, absolute difference 4.6% [95% CI -0.4% to 9.6%], p = 0.030). In the subgroup analysis based on different intervention modalities, microsurgery and hybrid surgery achieved higher complete obliteration rates than embolization (p < 0.001), and hybrid surgery resulted in significantly less intraoperative blood loss than microsurgery (p = 0.041). CONCLUSIONS: Intervention is reasonable for properly indicated SM grade IV-V AVMs because it provides satisfactory seizure control with decreased risks of severe disability and subsequent hemorrhage than conservative management. Clinical trial registration no.: NCT04572568 (ClinicalTrials.gov).

Resection of Brainstem Arteriovenous Malformations: Pearls and Pitfalls for Minimizing Complications.

BACKGROUND: The decision-making process surrounding resection of arteriovenous malformations (AVMs) in proximity to vital brainstem structures is a complex topic. Intricate vasculature in the setting of exquisite brainstem eloquence creates a high-risk operative landscape with the potential for devastating complications. Effective resections are driven by mastery of the relevant operative anatomy, preservation of pertinent vasculature, and technical experience and acumen. METHODS: This article provides a narrative literature review on the resection of brainstem AVMs. RESULTS: Operative anatomy and approaches to AVMs of the midbrain (anterior/posterior), pons (anterior/lateral), and medulla (anterior/lateral) are discussed herein, with a focus on pearls and pitfalls for minimizing complications during resection. CONCLUSIONS: Careful consideration of the patient's clinical background, the natural history of the lesion, and expertise of the treating surgeon are paramount for improving the natural course of brainstem AVMs.

Exome-wide Analysis of De Novo and Rare Genetic Variants in Patients With Brain Arteriovenous Malformation.

BACKGROUND AND OBJECTIVES: Brain arteriovenous malformation (bAVM) is a congenital disorder and a leading cause of hemorrhagic stroke. Germline genetic variants play an essential role in the pathogenesis of bAVM. However, the biological relevance of disease-associated genes identified in previous studies is elusive. In this study, we aim to systematically investigate the contribution of germline variants to bAVM and explore the critical molecular pathways underlying the pathogenesis of bAVM. METHODS: Probands with sporadic bAVM were consecutively recruited into this study from November 2015 to November 2018 and underwent exome sequencing. The controls were aggregated from individuals who were not known to have vascular malformation and underwent exome sequencing for clinical or research purposes. The retained control dataset included 4,609 individuals, including 251 individuals with parental samples sequenced. We first analyzed de novo variants in cases and controls and performed a pathway enrichment analysis. A gene-based rare variant association analysis was then performed to identify genes whose variants were significantly enriched in cases. RESULTS: We collected an exome-sequenced bAVM cohort consisting of 152 trios and 40 singletons. By first focusing on de novo variants, we observed a significant mutational burden of likely gene-disrupting variants in cases vs controls. By performing a pathway enrichment analysis of all nonsynonymous de novo variants identified in cases, we found the angiopoietin-like protein 8 (ANGPTL8) regulatory pathway to be significantly enriched in patients with bAVM. Through an exome-wide rare variant association analysis utilizing 4,394 in-house exome data as controls, we identified SLC19A3 as a disease-associated gene for bAVM. In addition, we found that the SLC19A3 variants in cases are preferably located at the N' side of the SLC19A3 protein. These findings implicate a phenotypic expansion of SLC19A3-related disorders with a domain-specific effect. DISCUSSION: This study provides insights into the biological basis of bAVM by identifying novel molecular pathways and candidate genes.

Endoluminal Biopsy for Molecular Profiling of Human Brain Vascular Malformations.

BACKGROUND AND OBJECTIVES: Ras-mitogen-activated protein kinase (MAPK) signaling abnormalities occur in most brain arteriovenous malformations (bAVMs). No means exist to molecularly profile bAVMs without open surgery, limiting precision medicine approaches to treatment. Here, we report use of endoluminal biopsy of the vessel lumen of bAVMs to characterize gene expression and blood flow-mediated transcriptional changes in living patients. METHODS: Endoluminal biopsy and computational fluid dynamic modeling (CFD) were performed in adults with unruptured AVMs with cerebral angiography. Each patient underwent surgical resection and cell sampling from a contiguous arterial segment. Fluorescence-assisted cell sorting enriched endothelial cells, which were sequenced on an Illumina HiSeq 4000 sequencer. Gene expression was quantified with RNA sequencing (RNAseq). Differential gene expression, ontology, and correlative analyses were performed. Results were validated with quantitative reverse transcription PCR (RT-qPCR). RESULTS: Endoluminal biopsy was successful in 4 patients without complication. Endoluminal biopsy yielded 269.0 ± 79.9 cells per biopsy (control 309.2 ± 86.6 cells, bAVM 228.8 ± 133.4 cells). RNAseq identified 106 differentially expressed genes (DEGs) in bAVMs (false discovery rate ≤0.05). DEGs were enriched for bAVM pathogenic cascades, including Ras-MAPK signaling (p < 0.05), and confirmed with RT-qPCR and a panel predictive of MAPK/extracellular signal-regulated kinase inhibitor response. Compared to patient-matched surgically excised tissues, endoluminal biopsy detected 83.3% of genes, and genome-wide expression strongly correlated (Pearson r = 0.77). Wall shear stress measured by CFD correlated with inflammatory pathway upregulation. Comparison of pre-embolization and postembolization samples confirmed flow-mediated gene expression changes. DISCUSSION: Endoluminal biopsy allows molecular profiling of bAVMs in living patients. Gene expression profiles are similar to those of tissues acquired with open surgery and identify potentially targetable Ras-MAPK signaling abnormalities in bAVMs. Integration with CFD allows determination of flow-mediated transcriptomic alterations. Endoluminal biopsy may help facilitate trials of precision medicine approaches to bAVMs in humans.

Somatic mosaicism in the MAPK pathway in sporadic brain arteriovenous malformation and association with phenotype.

OBJECTIVE: Sporadic brain arteriovenous malformation (BAVM) is a tangled vascular lesion characterized by direct artery-to-vein connections that can cause life-threatening intracerebral hemorrhage (ICH). Recently, somatic mutations in KRAS have been reported in sporadic BAVM, and mutations in other mitogen-activated protein kinase (MAPK) signaling pathway genes have been identified in other vascular malformations. The objectives of this study were to systematically evaluate somatic mutations in MAPK pathway genes in patients with sporadic BAVM lesions and to evaluate the association of somatic mutations with phenotypes of sporadic BAVM severity. METHODS: The authors performed whole-exome sequencing on paired lesion and blood DNA samples from 14 patients with sporadic BAVM, and 295 genes in the MAPK signaling pathway were evaluated to identify genes with somatic mutations in multiple patients with BAVM. Digital droplet polymerase chain reaction was used to validate KRAS G12V and G12D mutations and to assay an additional 56 BAVM samples. RESULTS: The authors identified a total of 24 candidate BAVM-associated somatic variants in 11 MAPK pathway genes. The previously identified KRAS G12V and G12D mutations were the only recurrent mutations. Overall, somatic KRAS G12V was present in 14.5% of BAVM lesions and G12D was present in 31.9%. The authors did not detect a significant association between the presence or allelic burden of KRAS mutation and three BAVM phenotypes: lesion size (maximum diameter), age at diagnosis, and age at ICH. CONCLUSIONS: The authors confirmed the high prevalence of somatic KRAS mutations in sporadic BAVM lesions and identified several candidate somatic variants in other MAPK pathway genes. These somatic variants may contribute to understanding of the etiology of sporadic BAVM and the clinical characteristics of patients with this condition.

Clinical Characteristics, Angioarchitecture and Management of Tectum Mesencephali Arteriovenous Malformations : A Retrospective Case Series.

PURPOSE: Tectum mesencephali arteriovenous malformations (TM-AVMs) are rare lesions deeply located close to eloquent structures making them challenging to treat. We aimed to present clinical presentation, angiographic features and treatment strategies of TM-AVMs through a single center retrospective case series. METHODS: A TM-AVMs is defined as a nidus located in the parenchyma or on the pia mater of the posterior midbrain. Records of consecutive patients admitted with TM-AVMs over a 21-year period were retrospectively analyzed. Vascular anatomy of the region is also reviewed. RESULTS: In this study 13 patients (1.63% of the complete cohort; 10 males), mean age 48 years, were included. All patients presented with intracranial hemorrhage and two patients (15%) died after an early recurrent bleeding. Mean size of the TM-AVMs was 10.1 ± 5 mm. Multiple arterial feeders were noted in every cases. Of the patients 11 underwent an exclusion treatment, 8 via embolization (6 via arterial access and 2 via venous access) and 4 via stereotactic radiosurgery (SRS) (1 patient received both). Overall success treatment rate was 7/11 patients (64% overall; 63% in the embolization group, 25% in the SRS group). Two hemorrhagic events led to a worsened outcome, one during embolization and one several years after SRS. All other patients remained clinically stable or improved. CONCLUSION: The TM-AVMs are rare but stereotypic lesions found in a hemorrhagic context. Multiple arterial feeders are always present. Endovascular treatment seems to be an effective technique with relatively low morbidity; SRS had a low success rate but was only use in a limited number of patients.

Molecular Biomarkers and Drug Targets in Brain Arteriovenous and Cavernous Malformations: Where Are We?

Vascular malformations of the brain (VMB) comprise abnormal development of blood vessels. A small fraction of VMBs causes hemorrhages with neurological morbidity and risk of mortality in patients. Most often, they are symptomatically silent and are detected at advanced stages of disease progression. The most common forms of VMBs are arteriovenous and cavernous malformations in the brain. Radiopathological features of these diseases are complex with high phenotypic variability. Early detection of these malformations followed by preclusion of severe neurological deficits such as hemorrhage and stroke is crucial in the clinical management of patients with VMBs. The technological advances in high-throughput omics platforms have currently infused a zest in translational research in VMBs. Besides finding novel biomarkers and therapeutic targets, these studies have withal contributed significantly to the understanding of the etiopathogenesis of VMBs. Here we discuss the recent advances in predictive and prognostic biomarker research in sporadic and familial arteriovenous malformations as well as cerebral cavernous malformations. Furthermore, we analyze the clinical applicability of protein and noncoding RNA-based molecular-targeted therapies which may have a potentially key role in disease management.

Somatic PIK3CA Mutations in Sporadic Cerebral Cavernous Malformations.

BACKGROUND: Cerebral cavernous malformations (CCMs) are common sporadic and inherited vascular malformations of the central nervous system. Although familial CCMs are linked to loss-of-function mutations in KRIT1 (CCM1), CCM2, or PDCD10 (CCM3), the genetic cause of sporadic CCMs, representing 80% of cases, remains incompletely understood. METHODS: We developed two mouse models harboring mutations identified in human meningiomas with the use of the prostaglandin D2 synthase (PGDS) promoter. We performed targeted DNA sequencing of surgically resected CCMs from patients and confirmed our findings by droplet digital polymerase-chain-reaction analysis. RESULTS: We found that in mice expressing one of two common genetic drivers of meningioma - Pik3ca H1047R or AKT1 E17K - in PGDS-positive cells, a spectrum of typical CCMs develops (in 22% and 11% of the mice, respectively) instead of meningiomas, which prompted us to analyze tissue samples from sporadic CCMs from 88 patients. We detected somatic activating PIK3CA and AKT1 mutations in 39% and 1%, respectively, of lesion tissue from the patients. Only 10% of lesions harbored mutations in the CCM genes. We analyzed lesions induced by the activating mutations Pik3ca H1074R and AKT1 E17K in mice and identified the PGDS-expressing pericyte as the probable cell of origin. CONCLUSIONS: In tissue samples from sporadic CCMs, mutations in PIK3CA were represented to a greater extent than mutations in any other gene. The contribution of somatic mutations in the genes that cause familial CCMs was comparatively small. (Funded by the Fondation ARC pour la Recherche contre le Cancer and others.).

An analysis of lesions associated with developmental venous anomalies.

OBJECTIVE: The aim of this study was to describe different lesions and features associated with developmental venous anomalies (DVAs). METHODS: The records and magnetic resonance imaging (MRI) images of 1,722 patients who underwent cranial MRI between 2010 and 2017 were retrospectively reviewed. It was found that 124 (7.2%) patients had DVAs, and 48 of these patients (38.7%) had additional anomalies accompanying DVAs. Of the patients with DVAs, 25 were female and 23 were male, with a mean age of 39.3 years (range, 3-77 years). MRI was performed in all the patients. RESULTS: In addition to DVAs, cavernomas were present in 30 patients (62.5%), haematomas in 7 (14.5%), gliosis in 6 (12.5%), demyelinating plaques in 4 (8.3%) and a glioblastoma in 1 (2.2%). The mean diameter of the DVAs was 1.1mm and the mean diameter of the lesions was 17.4mm. The susceptibility weighted imaging (SWI) sequence was also applied to 12 patients with cavernomas. The relevant sequence in all of these patients contributed to the diagnosis. CONCLUSION: Our study shows that DVAs can accompany a wide spectrum of lesions, especially cavernomas. Although their pathophysiology has not yet been clearly established, these lesions may have a common aetiology.

Clinical Features and Treatment Outcomes of Cerebral Arteriovenous Malformation Associated With Moyamoya Disease.

BACKGROUND: Cerebral arteriovenous malformation (AVM) can rarely occur in conjunction with moyamoya disease (MMD). There is still no consensus on how to treat AVM when accompanied by MMD. In this study, we assessed the clinical features and suggested appropriate management when AVM was combined with MMD. METHODS: From August 1994 to December 2020, 7 out of 4004 patients with MMD were found to have AVM. The Karnofsky Performance Scale (KPS) was used to evaluate the clinical outcomes of AVM and MMD. KPS greater than 80 was classified as a good outcome. In addition, the radiologic outcomes of the patients were evaluated. RESULTS: The incidence of AVM with MMD was 1.7 per 1000 persons. Five patients underwent bypass surgery for MMD, and 5 patients underwent Gamma Knife surgery (GKS) for concurrent AVM. Postoperative perfusion magnetic resonance imaging and brain single photon emission computerized tomography showed improved cerebral hemodynamics in 4 out of 7 territories. Postoperative cerebral angiography showed good revascularization in 4 out of 8 territories. After GKS, 4 patients showed complete obliteration, and 1 patient showed a significantly decreased AVM size. Six patients showed favorable clinical outcomes (KPS 80-100), and 1 patient with delayed GKS for AVM had a poor outcome (KPS 20) due to AVM rupture. CONCLUSIONS: In this study, AVM tended to occur where the angiographic stage of MMD was higher. When AVM is combined with MMD, MMD bypass surgery is recommended based on symptoms and cerebral perfusion status. For AVM, less invasive but effective treatments, such as GKS, should be implemented as soon as possible.

Vermian subtentorial arteriovenous malformation supplied by the artery of Wollschlaeger and Wollschlaeger.

BACKGROUND: The artery of Wollschlaeger and Wollschlaeger is a tentorial branch of the superior cerebellar artery: due to its small diameter, it is not usually seen in normal angiograms except when enlarged in the setting of a dural AVF or tentorial meningioma. Its presence has been rarely described in the Literature. CASE REPORT: herein we describe the first ever reported case of a vermian subtentorial arteriovenous malformation supplied by the artery of Wollschlaeger and Wollschlaeger in 70 year old female patient. CONCLUSION: vermian subtentorial AVMs supplied by the artery of Wollschlaeger and Wollschlaeger are extremely rare vascular malformations. The presence of the artery of Wollschlaeger and Wollschlaeger must be carefully evaluated during preoperative surgical planning due to its key role in the supply of vascular malformation and to decrease the risk of intra operative bleeding during surgery.

The Thalamic Arteriovenous Malformations Have Better Prognosis than Basal Ganglia Malformations Regarding Obliteration: Prognostic Factors Analysis.

BACKGROUND: Arteriovenous malformations (AVMs) are rare vascular congenital lesions that affect mainly patients during their productive years of life. In order to obtain a better quality of life for patients with this disease, a multidisciplinary approach is recommended. Radiosurgery is one of the treatment modalities available for AVMs, but many factors may influence the effectiveness of this strategy. Classically, it has been said that deep-seated lesions have a particular behavior compared with AVMs in other regions, but a differentiation between thalamic lesions and those located in the basal ganglia has not been made. METHODS: Institutional records for central core AVMs treated with radiosurgery between January 2004 and January 2014 were retrospectively analyzed. Brainstem lesions were excluded from the analysis. RESULTS: Forty-nine patients with deep-seated AVMs were included. Forty-three (87.8%) were located in the thalamus and 6 (12.2%) in the area of basal ganglia. The nidus mean volume was 4.1 cm3 (SD: 4.1), the maximum diameter mean was of 19.5 mm (SD: 8.0). The prescription dose was 18.2 Gy (SD: 2.1), and the follow-up time was 75.8 months (SD: 32.5). There was a greater obliteration rate in thalamic AVMs compared with those located in the basal ganglia: 81.4% versus 33.3% (P = 0.026), respectively. There was no association between categorical variables and obliteration rate. CONCLUSIONS: Stereotactic radiosurgery is a good option for patients with thalamic and basal ganglia AVMs, but a multidisciplinary approach to decision-making is mandatory in order to achieve the best results.