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Background and Objectives: Natural history of hemorrhage in brain arteriovenous malformations (bAVM) is reported at 2%-4% per year. Published studies using survival analysis fail to account for recurrent hemorrhagic events. In this study, we present a large, single institution series to elucidate the natural history of bAVM using multivariable Poisson regression., Methods: This is a retrospective cohort study. All patients with bAVM seen at our institution from 1990 to 2021 were included. Hemorrhages after detection of bAVM during the untreated interval were recorded. Natural history of hemorrhage was calculated by dividing number of hemorrhages by untreated interval. The frequency of hemorrhages followed a Poisson distribution. Multivariable Poisson regression with an offset variable of untreated interval in patient-years was constructed. Model selection was through a stepwise Akaike information criterion method. Stratified hemorrhagic rate was presented using different combinations of significant factors., Results: A total of 1066 patients with nonhereditary hemorrhagic telangiectasia harboring a single bAVM were included. Ninety (8.44%) patients had 101 hemorrhages during an untreated interval of 3596.3344 patient-years, translating to an overall hemorrhagic rate of 2.81% per year. Significant factors increasing hemorrhage risk included ruptured presentation ( P < .001), increasing age ( P < .001), female sex ( P = .043), and deep location ( P = .040). Adult male patients with ruptured presentation and deep bAVMs sustained the highest annual risk at 10.81%, whereas no hemorrhages occurred in unruptured pediatric male patients or ruptured pediatric (younger than 18 years) male patients with superficial bAVMs., Conclusion: Hemorrhage after bAVM detection occurs in 8.41% of all patients, and the rate averages 2.81% per year. However, this risk varies from 0.00% to 10.81% per year depending on various risk factor combinations. Efforts should be made to stratify bAVM hemorrhage rate by risk factors for more precise estimation of bleeding risk if left untreated., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

Background: Previous reports indicated an association between hemorrhagic presentation and flow-related aneurysms in arteriovenous malformation (AVM) patients. However, it remains unclear whether these flow-related aneurysms result in the hemorrhage of AVM., Objective: To characterize this hemorrhage risk using our institutional experience over 25 yr., Methods: We retrospectively reviewed records of patients at our institution diagnosed with AVM from 1990 to 2015. Patients without associated aneurysms (AVM only) and those with flow-related aneurysms (AVM-FA) were compared. Those with intranidal or unrelated aneurysms were excluded. Annual risk of AVM-related hemorrhage was calculated using the birth-to-treatment approach and compared using Poisson rate ratio test., Results: Among 526 patients, there were 457 AVM only patients and 69 with flow-related aneurysms. AVM-FA patients were older (P = .005). AVMs with flow-related aneurysms were more likely located in the cerebellar vermis and hemispheres (P = .023 and .001, respectively). Presence of flow-related aneurysms increased the risk of presentation with subarachnoid hemorrhage (P < .001). Interestingly, no significant differences in presenting hemorrhage due to AVM rupture were found (P > .356). The majority of aneurysms were untreated (69.5%), and only 8 (9.8%) had ruptured presentation. At follow-up (mean = 5.3 yr), patients with flow-related aneurysms were less likely to develop seizures (P = .004). The annual risk of AVM hemorrhage was 1.33% and 1.05% for AVM only patients and AVM-FA patients, respectively (P = .248)., Conclusion: Despite increased risk of subarachnoid hemorrhage at presentation, there was no increased likelihood of rupture in AVMs with flow-related aneurysms. More studies are warranted, as clarifying the competing risks of AVM vs aneurysm rupture may be critical in determining optimal treatment strategy., (Copyright © 2018 by the Congress of Neurological Surgeons.)

Background: Embolization has been discussed as a feasible single modality treatment for intracranial arteriovenous malformations (AVMs)., Objective: To compare hemorrhagic risk between embolization and conservative management in a multivariate survival analysis., Methods: We retrospectively reviewed records of patients with intracranial AVMs evaluated at our institution from 1990 to 2013. We included patients recommended to undergo embolization without other treatment modalities and patients managed conservatively. Multivariate Cox regression analysis of hemorrhage-free survival was performed, with the survival interval right-censored to date of either last follow-up or salvage treatment., Results: We identified 205 patients matching our inclusion criteria, with 160 patients in the noninterventional group and 45 in the embolization group. The average age of all patients was 40.2 ± 19.5 yr, with younger patients undergoing embolization more often (P = .026). Fifty-one (31.9%) conservatively managed patients and 13 (28.9%) patients treated by embolization (P = .703) presented with hemorrhage. Other baseline characteristics were similar between the 2 management groups. During an average follow-up period of 7.7 yr, 30 patients (14.6%) experienced hemorrhage recurrence. Multivariate Cox regression revealed older age (P = .031) and hemorrhagic presentation (P < .001) to be statistically associated with follow-up hemorrhage. In a subset analysis of unruptured AVMs, embolization was associated with a 4-fold hazard ratio of hemorrhage compared to conservative management (P = .044)., Conclusion: Older age and initial presentation with hemorrhage were associated with increased risk of hemorrhage during follow-up. Treatment of AVMs with embolization as the sole modality may increase hemorrhagic risk compared with conservative management, especially in unruptured AVMs.

Background: The Spetzler-Martin grading system for brain arteriovenous malformations (AVMs) is based on size (S), eloquence (E), and deep venous drainage (V). However, variation exists due to subgroup heterogeneity. While previous studies have demonstrated variations in outcomes within grade III AVMs, no studies have focused on grade II AVM subtypes., Objective: We aim to delineate how functional outcomes differ among patients with subtypes of grade II AVMs., Methods: We retrospectively reviewed patients with AVMs evaluated at our institution from 1990 to 2013. Grade II AVMs were divided into 3 subtypes: group 1 (S2V0E0), group 2 (S1V0E1), and group 3 (S1V1E0). Baseline characteristics were compared, and functional status was assessed using the modified Rankin Scale (mRS) at pretreatment and last follow-up. Differences in mRS between the 2 time points were compared., Results: A total of 208 grade II patients (34.0%) were retrieved from 611 graded patients. After accounting for missing data, our cohort consisted of 137 patients. Mean age of all patients was 37.2 years, with 74 females (54.0%). No significant difference was observed across subgroups for pretreatment mRS ( P = .096), treatment modalities ( P = .943), follow-up durations ( P = .125), and mRS at last follow-up ( P = .716). In a subgroup analysis between group 1 and group 3, more patients with worsened mRS were observed in group 3 ( P = .039). This distinction was further confirmed in surgically treated patients ( P = .049), but not in patients treated with radiosurgery ( P = .863)., Conclusion: Subtypes of grade II AVMs portend different posttreatment gains in functional outcome. Group 1 (S2V0E0) patients had the best functional outcome gain from treatment, while group 3 (S1V1E0) patients fared less well, particularly with surgical treatment., (Copyright © 2017 by the Congress of Neurological Surgeons)

Background: Seizure risk has not been fully characterized in pediatric patients with arteriovenous malformations (AVMs)., Objective: To describe the progression and risk factors of post-treatment seizure in children with AVMs., Methods: We retrospectively reviewed pediatric patients diagnosed with intracranial AVMs at our institution between 1990 and 2013. Clinical and angiographic variables were included in univariate and multivariate Cox proportional hazard models to explore risk factors associated with time-related seizure outcomes. The outcome event is defined as first seizure occurrence after initial treatment. Kaplan-Meier survival curve is depicted for each significant variable, and survival differences were confirmed by Log-rank test., Results: We included 90 pediatric patients with complete data in our study cohort. Average age is 13.3 ± 3.8 years with 43.3% male patients. Thirty-seven patients had a hemorrhagic presentation, and 39 patients presented with seizure. Post-treatment seizure occurred in 33 patients (36.7%) over an average follow-up period of 8.1 ± 10.6 years; average time to onset is 5.3 ± 8.2 years, with partial seizures as the most common presentation. Multivariate Cox proportional hazard regression revealed seizure presentation ( P = .005), male gender ( P = .026), and nonconservative treatment modality to be significantly associated with earlier onset of post-treatment seizure after adjusting for AVM location and deep venous drainage. Overall annualized seizure risk is 7.4% for patients with pretreatment seizure, and 1.4% for those without., Conclusion: Risk of seizure persists after treatment in pediatric AVM patients. Seizure presentation, male gender, and nonconservative management are associated with increased risk of post-treatment seizure. Early identification of these risk factors provides important information for determining seizure management strategies., (Copyright © 2017 by the Congress of Neurological Surgeons)

Background: Giant intracranial arteriovenous malformations (AVMs) are rare cerebrovascular lesions that pose management challenges., Objective: To further clarify outcomes in patients with giant cerebral AVMs managed with conservative or interventional therapies., Methods: We performed a retrospective review of all patients diagnosed with AVMs evaluated at our institution from 1990 to 2013. Patients with a single intracranial AVM >6 cm were included. Patients were divided into 2 groups: conservative management or intervention (microsurgery, radiosurgery, or embolization). Functional outcome was assessed with the modified Rankin Scale (mRS) and compared between the 2 groups., Results: A total of 55 patients with giant AVMs were included, and 35 patients (63.6%) had clinical follow-up with a mean of 11.8 years. Spetzler-Martin grades were as follows: grade III, n = 2 (3.6%); grade IV, n = 15 (27.3%); and grade V, n = 38 (69.1%). Twenty-four patients (43.6%) were conservatively managed. The patients in the conservatively managed group had larger AVMs (P < .05) with more frequent involvement of the temporal lobe (P = .02). Five patients (26.3%) in the conservatively managed group and 5 (31.3%) in the intervention group experienced hemorrhage during follow-up, translating to an annualized risk of 2.7% and 4.1%, respectively. No significant difference in risk of first subsequent hemorrhage was observed (P = .78). Despite comparable mRS scores at presentation, we observed a trend toward better outcomes (mRS < 2) in patients undergoing conservative management (P = .06) compared with the intervention group at last follow-up., Conclusion: This study suggests that interventions for giant AVMs should be considered cautiously because hemorrhagic risk is similar regardless of management strategy and functional outcome is likely to be same or better in the conservatively managed population., Abbreviations: AVM, arteriovenous malformationmRS, modified Rankin Scale.

Background: Patients diagnosed with hereditary hemorrhagic telangiectasia (HHT) are at risk of developing intracranial arteriovenous malformations (AVM). However, the clinical manifestations and natural history of HHT-related AVMs remain unclear due to the rarity of these lesions., Objective: To clarify the clinical characteristics and hemorrhagic risk in HHT-related AVMs., Methods: We performed a retrospective review of all patients diagnosed with both HHT and intracranial AVMs who were evaluated at our institution from 1990 to 2013. Patients with missing data or lost to follow-up were excluded. Baseline characteristics and subsequent hemorrhagic risk were evaluated., Results: In an AVM database of 531 patients with 542 AVMs, a total of 12 HHT patients (2.3%) with 23 AVMs were found. Mean age at diagnosis was 36.5 years, with 41.7% male. Compared to patients with sporadic AVMs, patients with HHT were less likely to present with ruptured AVM (P = .04), headaches (P = .02), and seizures (P = .02), and presented with better modified Rankin scores (P < .01). HHT-related AVMs were smaller in size (P < .01), of lower Spetzler-Martin grade (P = .01), and had less temporal lobe involvement (P = .02) compared to sporadic AVMs. Six HHT patients (50.0%) were found with multiple intracranial AVMs. One hemorrhage was found during an observation period of 149.6 patient-years and 297.5 lesion-years, translating to 1.3% per patient per year or 0.7% per AVM per year., Conclusion: HHT-related AVMs are smaller in size with lower Spetzler-Martin grade and less temporal lobe involvement than sporadic AVMs. Patients with HHT frequently present with multiple intracranial AVMs. Conservative management is generally recommended due to lesion multiplicity and relatively low hemorrhagic risk.