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Background In 2014, A Randomized Trial of Unruptured Brain Arteriovenous Malformations (ARUBA) concluded that medical management alone for cranial arteriovenous malformations (AVMs) had better clinical outcomes than interventional treatment. The impact of the ARUBA study on changes in the rates of intervention and outcomes is unknown. Thus, we investigated whether the conclusions from ARUBA may have influenced treatment modalities and outcomes of unruptured AVMs. Methods The National Inpatient Sample (NIS) was queried between 2006 and 2018, for adult patients with an AVM who were admitted on an elective basis. Interventions included open, endovascular, and stereotactic surgeries. Join-point regression was used to assess differences in slopes of treatment rate for each modality before and after the time-point. Logistic regression was used to assess the odds of non-routine discharge and hemorrhage between the two time-points for each treatment modality. Linear regression was used to assess the mean length of stay (LOS) for each treatment modality between the two time-points. Results A total of 40,285 elective admissions for AVMs were identified between 2006 and 2018. The rate of intervention was higher pre-ARUBA (n = 15,848; 63.8%) compared to post-ARUBA (n = 6985; 45.2%; difference in slope − 8.24%, p  < 0.001). The rate of open surgery decreased, while endovascular and stereotactic surgeries remained the same, after the ARUBA trial time-point (difference in slopes − 8.24%, p  < 0.001; − 1.74%, p  = 0.055; 0.20%, p  = 0.22, respectively). For admissions involving interventions, the odds of non-routine discharge were higher post-ARUBA (OR 1.24; p  = 0.043); the odds of hemorrhage were lower post-ARUBA (OR 0.69; p  = 0.025). There was no statistical difference in length of stay between the two time-points ( p  = 0.22). Conclusion The rate of intervention decreased, the rate of non-routine discharge increased, and rate of hemorrhage decreased post-ARUBA, suggesting that it may have influenced treatment practices for unruptured AVMs.

Abstract OBJECTIVE Stereotactic radiosurgery makes brain arteriovenous malformations (AVM) more manageable during their microsurgical resection. To better characterize these effects, we compared results of microsurgical resection of radiated (RS+) and nonradiated (RS−) AVMs to demonstrate that previous radiosurgery facilitates surgery and decreases operative morbidity. METHODS From our series of 344 patients who underwent AVM resections at the University of California, San Francisco (1997–2007), 21 RS+ patients were matched with 21 RS+ patients based on pretreatment clinical and AVM characteristics. Matching was blinded to outcomes, which were assessed with the modified Rankin Scale. RESULTS Mean AVM volume was reduced by 78% (P < 0.01), and Spetzler-Martin grades were reduced in 52% of RS+ patients (P < 0.001). Preoperative embolization was used less in RS+ than in RS− patients (P < 0.001). Mean operative time (P < 0.01), blood loss (P < 0.05), and length of hospital stay (P < 0.05) were lower in the RS+ group. Surgical morbidity was 14% higher in RS− patients, and they demonstrated significant worsening in modified Rankin Scale scores after surgery, whereas RS+ patients did not (P < 0.01). RS+ patients deteriorated between AVM diagnosis and surgery owing to hemorrhages during the latency period (P < 0.05). CONCLUSION Previous radiosurgery facilitates AVM microsurgery and decreases operative morbidity. Radiosurgery is recommended for unruptured AVMs that are not favorable for microsurgical resection. Microsurgical resection is recommended for radiated AVMs that are not completely obliterated after the 3-year latency period but are altered favorably for surgery, even in asymptomatic patients. Prompt resection of persistent AVMs should be considered to avoid the risk of postlatency hemorrhage and to optimize patient outcomes.

Abstract BACKGROUND Population-based long-term data on pediatric patients with cerebral arteriovenous malformations (AVMs) are limited. OBJECTIVE To clarify the characteristics and long-term outcome of pediatric patients with AVM. METHODS A retrospective analysis was performed on 805 consecutive brain AVM patients admitted to a single center between 1942 and 2014. The patients were defined as children if they were under 18 yr at admission. Children were compared to an adult cohort. Changing patterns of presentation were also analyzed by decades of admission. RESULTS The patients comprised 127 children with a mean age of 12 yr. The mean follow-up time was 21 yr (range 0-62). Children presented more often with intracerebral hemorrhage (ICH) but less often with epilepsy than adults. Basal ganglia, cerebellar, and posterior paracallosal AVMs were more common in pediatric than in adult patients. Frontal and temporal AVMs, in contrast, were more common in adult than in pediatric patients. As the number of incidentally and epilepsy-diagnosed AVMs increased, ICH rates dropped in both cohorts. In total, 22 (82%) pediatric and 108 (39%) adult deaths were assessed as AVM related. After multivariate analysis, small AVM size and surgical treatment correlated with a favorable long-term outcome. CONCLUSION Hemorrhagic presentation was more common in children than in adults. This was also reflected as lower prevalence of epileptic presentation in the pediatric cohort. Lobar and cortical AVM locations were less frequent, whereas deep and cerebellar AVMs were more common in children. Hemorrhagic presentation correlated negatively with incidentally and epilepsy-diagnosed AVMs. In children, AVM was a major cause of death, but in adults, other factors contributed more commonly to mortality.

Background Vasospasm is a devastating sequelae of ruptured arteriovenous malformations (AVMs) in adults. Comorbidities and presenting factors have been suggested as risks, but only in cross-sectional studies. The objective of this study was to characterize risk factors associated with vasospasm and mortality in ruptured AVM. Methods Adult patients from the TriNetX Research Network were included, based on the ICD-10 codes, over a period of 20 years. Cox proportional hazard models assessed the hazards of vasospasm (I67.84) and mortality separately, adjusting for age, sex, comorbidities, substance use history, presenting factors (e.g., hypernatremia, hypokalemia), criteria of the National Inpatient Sample-Subarachnoid Hemorrhage Severity Score, and location of hemorrhage. Outcomes were assessed in the first 30 days following rupture. Results Among 10,375 patients with ruptured AVMs, 523 (5.3%) died and 297 (3.0%) experienced vasospasm in the first 30 days. After matching for age and sex, vasospasm was associated with increased mortality at three months (11.1% vs. 4.8%, p  = 0.003), six months (12.6% vs. 5.1%, p  = 0.001), and one year (13.5% vs. 6.9%, p  = 0.005). Female sex was protective against vasospasm within 30 days (HR = 0.714, p  = 0.007) while the greatest risk factors present on admission included subarachnoid hemorrhage (6.086, p  < 0.001), hydrocephalus (3.783, p  < 0.001), and leukocytosis (2.0677, p  < 0.001). The greatest risk factors for 30-day mortality were coma (HR = 3.700, p  < 0.001), hydrocephalus (2.698, p  < 0.001), and chronic kidney disease (1.596, p  = 0.003). Conclusions In this retrospective cohort study of 10,375 adult patients with ruptured AVMs, vasospasm occurred in approximately 3%. Risk factors for vasospasm included subarachnoid hemorrhage, male sex, hydrocephalus, and leukocytosis. The presence of vasospasm was associated with a more than twofold increase in mortality at both 30 days and one year.

Background and objectives Natural history of brain arteriovenous malformations (bAVMs) with associated intracranial aneurysms (IAs) reveals a higher rate of hemorrhage. We aimed to identify the prevalence and risk factors for hemorrhage in a subgroup of patients with bAVMs and associated arterial IAs. Methods The authors conducted an international, bicentric retrospective study of patients with ruptured and unruptured bAVMs with associated IAs treated at tertiary centers between January 2013 and December 2022. Sociodemographic data, clinical characteristics, and radiological parameters in patients with bAVM and associated IAs were analyzed. Results Of 944 patients with bAVM, 137 individuals with 191 associated arterial IAs were included in the final analysis. Bleeding presentation was documented in 85 cases (62.0%). The mean size of bAVM-associated IAs was 6.8 (SD = 4.8) mm. Multiple intracranial aneurysms (MIA) were present in 35 patients (25.5%). bAVM-associated IAs were classified as nidal in 19.0%, flow-related in 73.7%, and unrelated in 7.3% of cases. Univariate analysis revealed that arterial hypertension (odds ratio 4.37 [CI 1.52–12.57]; P  =.004), history of smoking (odds ratio 5.77 [CI 1.26–26.53]; P  =.013), and high-grade bAVMs (grades IV/V, odds ratio 0.35 [CI 0.15–0.87]; P  =.02), were associated with a bleeding risk. In the multivariable analysis, only arterial hypertension remained significantly associated with the bleeding event (adjusted odds ratio 3.37 [CI 1.07–10.58]; P  =.038). Conclusions Observational data from our large bicentric cohort of patients with bAVM and associated IAs identified arterial hypertension as a risk factor associated with an increased risk of bleeding. Trial registration The study was approved by the Institutional Review Board (IRB) of the University Clinical Center of Vojvodina and the University of Duisburg-Essen (20-9288-BO).

Objectives To evaluate the safety and efficacy of a new liquid embolic agent in brain arteriovenous malformation (bAVMs) embolisation. Methods A prospective, multicentre series was conducted at 11 interventional centres in Europe to evaluate embolisation of bAVMs with the new liquid embolic agent. Technical conditions, complications, clinical outcome and anatomical results were independently analysed. Results From December 2005 to December 2008, 117 patients (72 male; 45 female, aged 18–75 years) were included. Clinical presentation was mostly haemorrhage (34.2 %) and seizures (28.2 %). Most AVMs were located in the brain hemispheres (85.5 %). AVMs were <3 cm in 52.1 % of patients and ≥3 cm in 47.9 %. Morbidity was observed in 6/117 patients (5.1 %), related to haemorrhagic events in 2 cases and non-haemorrhagic complications in 4 cases. Five patients (4.3 %) died in relation to the treatment (bleeding in 4 patients and extensive venous thrombosis in 1). Complete occlusion of the AVM by embolisation alone was obtained in 23.5 % of patients. Complementary treatment was performed in 82.3 % of patients with partial AVM occlusion, mostly radiosurgery. Conclusions In this prospective, multicentre, European, observational series, the new liquid embolic agent proved to be suitable for BAVM embolisation, with acceptable morbidity and mortality and good efficacy. Key Points • Numerous interventional techniques have been used to embolise brain arteriovenous malformations (AVMs). • This prospective multicentre study demonstrates the suitability of a liquid embolic agent. • The safety of treatment using Onyx is acceptable. • Such embolisation leads to complete AVM occlusion in 23.5 % of patients.

Abstract BACKGROUND Recommendations on the management of brain arteriovenous malformations (bAVM) with respect to pregnancy are based upon conflicting literature. OBJECTIVE To systematically review the reported risk and annualized rate of first intracranial hemorrhage (ICH) from bAVM during pregnancy and puerperium. METHODS MEDLINE, EMBASE, and Scopus databases were searched for relevant articles in English published before April 2018. Studies providing a quantitative risk of ICH in bAVM during pregnancy were eligible. RESULTS From 7 initially eligible studies, 3 studies met the criteria for providing quantitative risk of first ICH bAVM during pregnancy. Data from 47 bAVM ICH during pregnancy across 4 cohorts were extracted for analysis. Due to differences in methodology and definitions of exposure period, it was not appropriate to combine the cases. The annualized risk of first ICH during pregnancy for these 4 cohorts was 3.0% (95% confidence interval [CI]: 1.7-5.2%); 3.5% (95% CI: 2.4-4.5%); 8.6% (95% CI: 1.8-25%); and 30% (95% CI: 18-49%). Only the last result from the last cohort could be considered significantly increased in comparison with the nonpregnant period (relative rate 6.8, 95% CI: 3.6-13). The limited number of eligible studies and variability in results highlighted the need for enhanced rigor of future research. CONCLUSION There is no conclusive evidence of an increased risk of first hemorrhage during pregnancy from bAVM. Because advice to women with bAVM may influence the management of pregnancy or bAVM with significant consequences, we believe that a retrospective multicenter, case crossover study is urgently required.

Long-term follow-up studies in patients with brain arteriovenous malformations (AVM) have yielded contradictory results regarding both risk factors for rupture and annual rupture rate. We performed a long-term follow-up study in an unselected, consecutive patient population with AVMs admitted to the Department of Neurosurgery at Helsinki University Central Hospital between 1942 and 2005. Patients with untreated AVMs were followed from admission until death, occurrence of AVM rupture, initiation of treatment, or until the end of 2005. Patients with at least 1 month of follow-up were included in further analysis. Annual and cumulative incidence rates of AVM rupture as well as several potential risk factors for rupture were analyzed using Kaplan-Meier life table analyses and Cox proportional hazards regression models. We identified 238 patients with a mean follow-up period of 13.5 years (range, 1 month-53.1 years). The average annual risk of hemorrhage from AVMs was 2.4%. The risk was highest during the first 5 years after diagnosis, decreasing thereafter. Risk factors predicting subsequent AVM hemorrhage in univariate analysis were young age, previous rupture, deep and infratentorial locations, and exclusively deep venous drainage. Previous rupture, large AVM size, and infratentorial and deep locations were independent risk factors according to multivariate models. According to this long-term follow-up study, AVMs with previous rupture and large size, as well as with infratentorial and deep locations have the highest risk of subsequent hemorrhage. This risk is highest during the first few years after diagnosis but remains significant for decades.

Seizures occur in 20–45% of patients with cerebral arteriovenous malformations (AVMs) and can potentially influence their quality of life. Some studies have suggested risk factors influencing their development, but the evidence is limited to small cohorts. To analyze seizure presentation and factors influencing seizure development in patients with cerebral AVMs using a multi-institutional consortium. Retrospective multicenter registry from multiple centers in North America and Europe of patients aged 1 to 89 years who had undergone any intervention for brain AVMs between January 2010 and December 2023. Demographics, functional assessment (modified Rankin Scale; mRS), and AVM characteristics, were evaluated to assess for relationship with seizures using a multivariate generalized linear mixed-effects model. 1,005 AVM patients were analyzed; the median age was 42, 73% had a baseline mRS ≤ 2. The median nidus size was 2.2 cm, and most AVMs were Spetzler-Martin grade II (37%). Seizure was the presenting symptom in 237 patients (24%). After adjusting for significant variables, patient-specific factors associated with seizures were younger age (OR 0.99, CI95% 0.98–1), male sex (OR 1.65, CI95% 1.18–2.30), and smoking history (OR 1.69, CI95% 1.17–2.44). AVM-specific factors associated with seizures included rupture status (OR 0.42, CI95% 0.30–0.61); eloquent cortex (OR 1.61, CI95% 1.13–2.29); frontal (OR 1.54, CI95% 1.01–2.35), temporal (OR 1.93, CI95% 1.26–2.96) and parietal (OR 1.71 CI95% 1.08–2.71) location; larger nidal size (OR = 1.23, CI95% 1.08–1.39), and superficial draining vein location (OR 1.86, CI95% 1.15–3.01). In this multicenter consortium, after controlling for significant variables, the incidence of AVM-related seizures was associated with younger age, male sex, smoking history, larger AVMs, eloquent locations, and AVMs in the frontal, temporal, and parietal cortices. Conversely, rupture status, deep venous drainage, and cerebellar locations were negatively associated with seizures.

Background Aneurysms associated with brain arteriovenous malformations (bAVMs) influence the natural history of these lesions and pose important therapeutic challenges. However, the epidemiology, natural history, and appropriate management of the aneurysms associated with bAVMs are not completely understood due to the paucity of large and uniform studies. We performed a systematic review of published series examining the association between aneurysms and bAVMs with the purpose of clarifying the prevalence, risk of hemorrhage, and appropriate management of these lesions. Method PRISMA/MOOSE guidelines were followed. We conducted a comprehensive literature search of three databases (PubMed, Ovid MEDLINE, and Ovid EMBASE) on aneurysms associated with bAVMs. Only studies examining consecutive case series of aneurysms associated with bAVMs were included. From the collected studies, we extracted data regarding prevalence of bAVM-associated aneurysms, risk of aneurysm rupture in relation to bAVM location and aneurysm characteristics, and treatment-related outcomes. Results Our systematic review included 44 articles with a total of 10,093 bAVMs. The proportion of bAVMs with an associated aneurysm was 20.2 % (95 % CI = 19.4–20.9 %). Among ruptured bAVMs with associated aneurysms, the aneurysm was the source of hemorrhage in 49.2 % (95 % CI = 43.7–54.7 %) of cases. Flow-related aneurysms were the most common source of aneurysm rupture (78.5 %, 95 % CI = 70.6–84.9 %). Infratentorial bAVM-associated aneurysms presented a higher risk of rupture (60 %, 95 % CI = 47.4–71.9 %) when compared with supratentorial lesions (29 %, 95 % CI = 21.4–38.5 %). Endovascular treatment of aneurysms associated with bAVMs had a cure rate of 80.0 % (95 % CI = 73.3–85.3 %), complication rate of 8.7 % (95 % CI = 5.5–13.1 %), and a good neurological outcome rate of 78.8 % (95 % CI = 72.5–83.9 %). Conclusions Twenty percent of bAVMs harbored arterial aneurysms. The presence of aneurysm increases the risk of bleeding of the bAVM, especially when flow-related or infratentorially located. Aneurysms associated with bAVMs should be treated promptly. Selective endovascular treatment of bAVM-associated aneurysms appears safe and effective.