Pediatric Brain Arteriovenous Malformations Research Articles

Three-Pillar Expansive Craniotomy in Children with Acute Ruptured Supratentorial Brain Arteriovenous Malformations

Acute rupture and hemorrhage of pediatric brain arteriovenous malformations (AVMs) may lead to cerebral herniation or intractable intracranial hypertension, necessitating emerging surgical interventions to alleviate intracranial pressure. However, there is still controversy regarding the timing of treatment for ruptured AVMs. This study aimed to assess the feasibility of utilizing three-pillar expansive craniotomy (3PEC) at different times during the treatment of pediatric ruptured supratentorial AVMs. A retrospective analysis was conducted on all consecutive cases of acute rupture in supratentorial AVM children who underwent 3PEC at a single institution from 2020 to 2022. General information, clinical characteristics, radiological data, and prognosis were reviewed and analyzed. Thirteen children were included in the analysis. The intracranial pressure of all patients decreased to below 15mmHg within 10days. The expansion volume of the cranial cavity of the patients increased by 18.3cm3 (95% confidence interval, 10.2-26.3; P<0.001) compared to the hematoma volume. None of the patients required decompressive craniectomy due to intractable intracranial hypertension caused by cerebral swelling. The median waiting period for patients with delayed AVMs treatment was 8days, during which no rebleeding occurred. Emergency intervention with 3PEC in children experiencing acutely ruptured supratentorial AVMs appears to be feasible. For children requiring delayed management of the AVMs, 3PEC may diminish the risk of rebleeding during the waiting period and shorten the waiting period.

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.

External validation of the R2eD AVM scoring system to assess rupture risk in pediatric AVM patients.

The assessment of hemorrhagic risk is of central importance in the management of pediatric brain arteriovenous malformations (AVMs). A recently published scoring system, the R2eD AVM (race, exclusive deep location or not, AVM size < 3 or ≥ 3, venous drainage exclusively deep or not, and monoarterial feeding or >1 arterial feeders) considers patient race and AVM location, size, venous drainage, and arterial supply and was successfully externally validated for use in adults to predict the risk of AVM hemorrhagic presentation. In this study the authors sought to externally validate the R2eD AVM score for prediction of hemorrhagic risk in the pediatric AVM population. A pediatric database at Boston Children's Hospital was retrospectively reviewed for all patients with a diagnosis of intracranial AVM. Exclusion criteria included age > 21 years, multiple AVMs, and incomplete clinical/radiographic data. Demographic data and R2eD AVM score calculations were collected for all patients. Univariate binary logistic regression and multivariate stepwise backward elimination models were used to determine factors associated with hemorrhagic presentation. A total of 212 AVMs were identified in 212 patients with vascular anomalies from 1995 to 2021, and 144 patients met the inclusion criteria (74 [51.4%] male, 111 [77.1%] White), of whom 87 (60.4%) patients presented with rupture and 122 (84.7%) underwent resection. The mean age at surgery was 12 years (range 3 months to 20 years). The R2eD AVM scoring system was applied to each patient. The score components of size < 3 cm and exclusive deep venous drainage were significantly associated with rupture risk (p < 0.05). The complete model for the R2eD AVM score had an area under the curve of 0.671 (95% CI 0.586-0.757). This study demonstrated poor external validity of the R2eD AVM score in predicting pediatric AVM rupture risk. The results suggest that future studies are warranted to determine a better scoring method to capture pediatric rupture risk, given the significant differences in clinical presentation in pediatric compared with adult AVM patients.

Validation of the Ruptured Arteriovenous Malformation Grading Scale in a pediatric cohort.

Pediatric brain arteriovenous malformations (AVMs) are the leading cause of spontaneous intracranial hemorrhage (SICH) in children. Although the incidence of SICH is low in pediatric populations, such events cause substantial morbidity. The recently created Ruptured Arteriovenous Malformation Grading Scale (RAGS) is proposed as a reliable and novel grading system to specifically serve as a predictor of clinical outcomes in patients following AVM rupture, similar to the Hunt and Hess (HH) grade for ruptured aneurysms. While these data are promising, pediatric patients were notably absent from the original study validating the RAGS. Therefore, correlation of the RAGS score with clinical outcomes following AVM rupture in individuals younger than 18 years of age using the RAGS score is needed. The objective of this study was to validate the RAGS in a cohort of pediatric patients with AVMs who presented with hemorrhage, thereby demonstrating the score's generalizability, and expanding its external validity. A cohort of children with ruptured AVMs were retrospectively reviewed. Using disability, measured by the modified Rankin Scale (mRS), as the response variable, the area under the receiver operating characteristic curve (AUROC) was calculated for patients based on their RAGS scores for three time periods. The AUROC values were then compared with those generated by two commonly used clinical grading systems, the HH classification and Glasgow Coma Scale. A total of 81 children who presented with ruptured AVMs were included in the study, with a mean follow-up duration of 4 years. The RAGS score outperformed other clinical grading scales in predicting mRS scores, with AUROC values of 0.81, 0.82, and 0.81 at three distinct follow-up periods. The RAGS score correlated well with the clinical outcome after AVM rupture in pediatric patients. Additional validation studies across multiple treatment centers are needed to further demonstrate the generalizability of the scoring system.

Effect of Prior Embolization on Outcomes After Stereotactic Radiosurgery for Pediatric Brain Arteriovenous Malformations: An International Multicenter Study.

Pediatric brain arteriovenous malformations (AVMs) are a significant cause of morbidity but the role of multimodal therapy in the treatment of these lesions is not well understood. To compare the outcomes of stereotactic radiosurgery (SRS) with and without prior embolization for pediatric AVMs. We retrospectively evaluated the International Radiosurgery Research Foundation pediatric AVM database. AVMs were categorized, based on use of pre-embolization (E+SRS)or lack thereof (SRS-only). Outcomes were compared in unadjusted and inverse probability weight (IPW)-adjusted models. Favorable outcome was defined as obliteration without post-SRS hemorrhage or permanent radiation-induced changes (RIC). The E+SRS and SRS-only cohorts comprised 91 and 448 patients, respectively. In unadjusted models, the SRS-only cohort had higher rates of obliteration (68.5%vs 43.3%, <.001) and favorable outcome (61.2%vs 36.3%, P<.001) but a lower rate of symptomatic RIC (9.0%vs 16.7%, P=.031). The IPW-adjusted rates of every outcome were similar between the 2 cohorts. However, cumulative obliteration rates at 3, 5, 8, and 10 yr remained higher in the absence of prior embolization (46.3%, 64.6%, 72.6%, and 77.4% for SRS-only vs 24.4%, 37.2%, 44.1%, and 48.7% for E+SRS cohorts, respectively; SHR=0.449 [0.238-0.846], P=.013). Embolization appears to decrease cumulative obliteration rates after SRS for pediatric AVMs without affecting the risk of post-treatment hemorrhage or adverse radiation effects arguingagainst the routine use of pre-SRS embolization. While endovascular therapy can be considered for occlusion of high-risk angioarchitectural features prior toSRS, future studies are necessary to clarify its role.

Stereotactic Radiosurgery for Unruptured versus Ruptured Pediatric Brain Arteriovenous Malformations

Stereotactic Radiosurgery for Unruptured versus Ruptured Pediatric Brain Arteriovenous Malformations

Radiosurgery for Unruptured Intervention-Naïve Pediatric Brain Arteriovenous Malformations.

Long-term data regarding stereotactic radiosurgery (SRS) as a standalone therapy for unruptured pediatric brain arteriovenous malformations (AVMs) are incompletely defined. To evaluate, in a multicenter, retrospective cohort study, the outcomes after SRS for unruptured, intervention-naïve pediatric AVMs. To retrospectively analyze the International Radiosurgery Research Foundation pediatric AVM database from 1987 to 2018. Pediatric patients with unruptured, previously untreated AVMs who underwent SRS were included. The primary endpoint was a composite of hemorrhagic stroke, death, or permanently symptomatic radiation-induced changes. The study cohort comprised 101 patients (mean follow-up 80.8 mo). The primary endpoint occurred in 14%, comprising hemorrhagic stroke, death, and permanent radiation-induced changes in 6%, 3%, and 8%, respectively. Estimated probabilities of the primary endpoint were 5.2%, 10.8%, and 23.0% at 2, 5, and 10 yr, respectively. Estimated probabilities of AVM obliteration at 5 and 10 yr were 64% and 82%, respectively. Single SRS treatment (P=.007) and higher margin dose (P=.005) were predictors of obliteration. Subgroup analysis of Spetzler-Martin grade I-III AVMs estimated primary endpoint probabilities of 3.7%, 8.4%, and 18.7% at 2, 5, and 10 yr, respectively. Treatment of unruptured, intervention-naïve AVMs in the pediatric population with SRS carries an approximately 2% annual risk of morbidity and mortality, which appears to plateau after 10 yr. The poorly described natural history of pediatric AVMs renders any comparison of SRS vs conservative management imperfect.

Early obliteration of pediatric brain arteriovenous malformations after stereotactic radiosurgery: an international multicenter study.

Stereotactic radiosurgery (SRS) is a treatment option for pediatric brain arteriovenous malformations (AVMs), and early obliteration could encourage SRS utilization for a subset of particularly radiosensitive lesions. The objective of this study was to determine predictors of early obliteration after SRS for pediatric AVMs. The authors performed a retrospective review of the International Radiosurgery Research Foundation AVM database. Obliterated pediatric AVMs were sorted into early (obliteration ≤ 24 months after SRS) and late (obliteration > 24 months after SRS) responders. Predictors of early obliteration were identified, and the outcomes of each group were compared. The overall study cohort was composed of 345 pediatric patients with obliterated AVMs. The early and late obliteration cohorts were made up of 95 (28%) and 250 (72%) patients, respectively. Independent predictors of early obliteration were female sex, a single SRS treatment, a higher margin dose, a higher isodose line, a deep AVM location, and a smaller AVM volume. The crude rate of post-SRS hemorrhage was 50% lower in the early (3.2%) than in the late (6.4%) obliteration cohorts, but this difference was not statistically significant (p = 0.248). The other outcomes of the early versus late obliteration cohorts were similar, with respect to symptomatic radiation-induced changes (RICs), cyst formation, and tumor formation. Approximately one-quarter of pediatric AVMs that become obliterated after SRS will achieve this radiological endpoint within 24 months of initial SRS. The authors identified multiple factors associated with early obliteration, which may aid in prognostication and management. The overall risks of delayed hemorrhage, RICs, cyst formation, and tumor formation were not statistically different in patients with early versus late obliteration.

Abstract 127: Radiosurgery for Unruptured Intervention-Naïve Pediatric Brain Arteriovenous Malformations

Background and Purpose: To evaluate, in a multicenter, retrospective cohort study, the outcomes after stereotactic radiosurgery (SRS) for unruptured, intervention-naïve pediatric brain arteriovenous malformations (AVM). Methods: We retrospectively analyzed the International Radiosurgery Research Foundation pediatric AVM database from 1987-2018. Pediatric patients with unruptured, previously untreated AVMs who underwent SRS were included. The primary endpoint was a composite of hemorrhagic stroke, death, or permanently symptomatic radiation-induced changes (RIC). Results: The study cohort comprised 101 patients (mean follow-up 80.8 months). The primary endpoint occurred in 14%, comprising hemorrhage stroke, death, and permanent RIC in 6%, 3%, and 8%, respectively. Estimated probabilities of the primary endpoint were 5.2%, 10.8%, and 23.0% at 2, 5, and 10 years, respectively (Figure 1). Estimated probabilities of AVM obliteration at 5 and 10 years were 64% and 82%, respectively (Figure 2). Single SRS treatment (p=0.007) and higher margin dose (p=0.005) were predictors of obliteration. Subgroup analysis of Spetzler-Martin grade I-III AVMs estimated primary endpoint probabilities of 3.7%, 8.4%, and 18.7% at 2, 5 and 10 years, respectively. Conclusion: Treatment of unruptured, intervention-naïve AVMs in the pediatric population with SRS carries an approximately 2% annual risk of morbidity and mortality, which appears to plateau after 10 years. The poorly described natural history of pediatric AVMs makes any comparison of SRS versus conservative management imperfect. However, due to the young age at diagnosis and excessive cumulative life hemorrhage risk of an untreated AVM in a child, SRS is likely to have a favorable long-term risk-benefit profile for appropriately selected unruptured pediatric AVMs.

Stereotactic Radiosurgery for Pediatric Brain Arteriovenous Malformations: Long-Term Outcomes

Abstract INTRODUCTION Contrary to the better described obliteration and hemorrhage data after stereotactic radiosurgery (SRS) treatment of brain arteriovenous malformations (AVMs) in pediatric patients, estimates of the rarer complications including cyst and tumor formation are limited in the literature. The aim of the study was to assess the long-term outcomes and risks of SRS for AVMs in pediatric (age &lt; 18 yr) patients. METHODS We retrospectively analyzed the International Radiosurgery Research Foundation pediatric AVM database from 1987 to 2018. AVM obliteration, post-SRS hemorrhage, cyst formation, and tumor formation were assessed. Cumulative probabilities, adjusted for competing risk of death, were calculated. RESULTS The study cohort comprised 539 pediatric AVM patients (mean follow-up: 85.8 mo). AVM obliteration was observed in 64.3% of patients, with cumulative probabilities of 63.6% (95% CI: 58.8%-68.0%), 77.1% (95% CI: 72.1%-81.3%), and 88.1% (95% CI: 82.5%-92.0%) over 5, 10, and 15 yr, respectively. Post-SRS hemorrhage was observed in 8.4% of patients, with cumulative probabilities of 4.9% (95% CI: 3.1%-7.2%), 9.7% (95% CI: 6.4%-13.7%), and 14.5% (95% CI: 9.5%-20.5%) over 5, 10, and 15 yr, respectively. Cyst formation was observed in 2.1% of patients, with cumulative probabilities of 5.5% (95% CI: 2.3%-10.7%) and 6.9% (95% CI: 3.1%-12.9%) over 10 and 15 yr, respectively. Meningioma was observed in 2 patients (0.4%) at 10 and 12 yr after SRS, with cumulative probability of 3.1% (95% CI: 0.6%-9.7%) over 15 yr. CONCLUSION AVM obliteration can be expected after SRS in the majority of the pediatric population, with a relatively low risk of hemorrhage during the latency period. Cyst and benign tumor formation after SRS can be observed in 7% and 3% of patients over 15 yr, respectively. Longitudinal surveillance for delayed neoplasia is prudent despite its low incidence.

Stereotactic Radiosurgery for Unruptured Versus Ruptured Pediatric Brain Arteriovenous Malformations.

Background and Purpose- The effects of prior hemorrhage on stereotactic radiosurgery (SRS) outcomes for pediatric arteriovenous malformations (AVMs) are not well defined. The aim of this multicenter, retrospective cohort study is to compare the SRS outcomes for unruptured versus ruptured pediatric AVMs. Methods- The International Radiosurgery Research Foundation pediatric AVM database from 1987 to 2018 was reviewed retrospectively. Favorable outcome was defined as AVM obliteration, no post-SRS hemorrhage, and no permanently symptomatic radiation-induced changes. Associations between prior hemorrhage and outcomes were adjusted for baseline differences, inverse probability weights, and competing risks. Results- The study cohort comprised 153 unruptured and 386 ruptured AVMs. Favorable outcome was achieved in 48.4% and 60.4% of unruptured and ruptured AVMs, respectively (adjusted odds ratio, 1.353; P=0.190). Cumulative AVM obliteration probabilities were 51.2%, 59.4%, 64.2%, and 70.0% for unruptured and 61.0%, 69.3%, 74.0%, and 79.3% for ruptured AVMs at 4, 6, 8, and 10 years, respectively (subhazard ratio, 1.311; P=0.020). Cumulative post-SRS hemorrhage probabilities were 4.5%, 5.6%, 5.6%, and 9.8% for unruptured and 4.7%, 6.1%, 6.1%, and 10.6% for ruptured AVMs at 4, 6, 8, and 10 years, respectively (subhazard ratio, 1.086; P=0.825). Probabilities of AVM obliteration (adjusted subhazard ratio, 0.968; P=0.850) and post-SRS hemorrhage (adjusted subhazard ratio, 1.663; P=0.251) were comparable between the 2 cohorts after inverse probability weight adjustments. Symptomatic (15.8% versus 8.1%; adjusted odds ratio, 0.400; P=0.008) and permanent (9.2% versus 5.0%; adjusted odds ratio, 0.441; P=0.045) radiation-induced change were more common in unruptured AVMs. Conclusions- The overall outcomes after SRS for unruptured versus ruptured pediatric AVMs are comparable. However, symptomatic and permanent radiation-induced change occur more frequently in pediatric patients with unruptured AVMs.

General Principles for Preoperative Planning and Microsurgical Treatment of Pediatric Brain Arteriovenous Malformations: 2-Dimensional Operative Video.

Children with arteriovenous malformations (AVMs) are at cumulative, life-long risk of debilitating and fatal intracranial hemorrhage, especially with syndromes such as hereditary hemorrhagic telangiectasia. Cerebral angiography is the gold standard for diagnosis and allows simultaneous adjunctive embolization limiting radiation and contrast exposure and intraoperative blood loss, important in pediatric patients with low blood volume. Microsurgical resection of low-grade AVMs offers cure with minimal morbidity. The plasticity of the pediatric brain may allow resection of AVMs near eloquent regions. Multidisciplinary care offers the best outcomes in these cases. Discussion of the operative plan with all team members at the start of surgery is carried out. Confirmation that blood products are present and that the microscope, additional suction, and surgical clips are available is performed. A generous craniotomy is created, adequately exposing the lesion. The dura is carefully opened to avoid injury to draining veins. Circumferential dissection and isolation of the AVM is performed, coagulating small arterial feeders, dissecting to the lesion's apex to coagulate, and divide major deep feeders. Once all feeders have been obliterated, venous drainage is disconnected. Indocyanine green distinguishes arterial feeders from arterialized veins and confirms complete resection. Since 2008, all of our patients undergo perioperative angiography in our dedicated suite, greatly improving resection rates. Strict blood pressure control and close neurological monitoring in the intensive care unit is performed postoperatively. In conclusion, microsurgical resection of AVMs can be performed safely with low rates of morbidity. Protocols for preoperative evaluation and planning appear to improve outcomes. Anonymous video is permitted when done without identifying patient related information.

Long-Term Outcomes for Pediatric Patients with Brain Arteriovenous Malformations Treated with Gamma Knife Radiosurgery, Part 2: The Incidence of Cyst Formation, Encapsulated Hematoma, and Radiation-Induced Tumor

Long-term data about the incidence of late adverse radiation effects (AREs) in pediatric brain arteriovenous malformations (AVMs) treated with Gamma Knife radiosurgery (GKRS) are lacking. This study addresses the incidence of late AREs, including cyst formation (CF), chronic encapsulated hematoma (CEH), and radiation-induced tumor, in pediatric patients with AVM treated with GKRS. This is a single-institutional study involving pediatric patients with AVM who underwent GKRS between 1991 and 2014. Among 201 pediatric patients with AVM (age ≤15 years), 189 who had at least 12 months of follow-up were assessed in this study. The median treatment volume was 2.2 cm3, and the median marginal dose was 20 Gy. The mean follow-up period was 136 months. During the follow-up period, symptomatic radiation-induced perilesional edema was found in 5 patients (3%), CFs in 7 patients (4%), CEHs in 7 patients (4%), and radiation-induced tumors in 2 patients (1%). The cumulative incidences of late AREs including CF, CEH, and radiation-induced tumor were 1.2% at 5 years, 5.2% at 8 years, 6.1% at 10 years, 7.2% at 15 years, and 17.0% at 20 years. In the multivariate analysis, treatment volume alone was a significant factor for late AREs (P < 0.001; hazard ratio, 1.111). GKRS is a reasonable treatment option for pediatric AVMs to prevent future intracranial hemorrhages, particularly in the eloquent regions. However, considerable attention should be paid to late AREs such as CFs, CEHs, and radiation-induced tumors because of longer life expectancy in pediatric patients.

Long-Term Outcomes for Pediatric Patients with Brain Arteriovenous Malformations Treated with Gamma Knife Radiosurgery, Part 1: Analysis of Nidus Obliteration Rates and Related Factors

Little is known about long-term outcomes for pediatric brain arteriovenous malformations (AVMs) treated with Gamma Knife radiosurgery (GKRS). This study investigated annual hemorrhage rates and nidus obliteration rates, and the factors affecting them, in pediatric AVMs treated with GKRS. We examined 189 pediatric AVM patients (age ≤15 years) who underwent GKRS and had at least 12 months of follow-up. The Spetzler-Martin (S-M) grade was I in 29 patients (15%), II in 57 (30%), III in 82 (43%), IV in 16 (9%), and V in 5 (3%). The median treatment volume was 2.2 cm3, and the median marginal dose was 20 Gy. The mean follow-up period was 136 months. During a cumulative latency period to nidus obliteration of 813 years, 23 hemorrhages occurred, resulting in an annual post-GKRS hemorrhage rate of 2.8%. The cumulative hemorrhage rates after GKRS were 3.3%, 8.5%, and 11.9% at 3, 5, and 10 years, respectively. Higher S-M grade was significantly associated with intracranial hemorrhages during the latency period (P < 0.001). The actuarial nidus obliteration rates with repeated GKRS were 64% and 81% at 5 and 10 years, respectively. Absence of pre-GKRS embolization (P= 0.023) and higher marginal dose (P= 0.029) were significant factors predicting nidus obliteration. GKRS is a reasonable treatment option in pediatric AVMs to prevent future hemorrhages. Because higher S-M grade AVMs are more likely to hemorrhage during the latency period, a combined therapy with endovascular embolization should be considered to prevent AVM rupture.

International multicenter cohort study of pediatric brain arteriovenous malformations. Part 2: Outcomes after stereotactic radiosurgery.

OBJECTIVE Pediatric patients (age < 18 years) harboring brain arteriovenous malformations (AVMs) are burdened with a considerably higher cumulative lifetime risk of hemorrhage than adults. Additionally, the pediatric population was excluded from recent prospective comparisons of intervention versus conservative management for unruptured AVMs. The aims of this multicenter, retrospective cohort study are to analyze the outcomes after stereotactic radiosurgery for unruptured and ruptured pediatric AVMs. METHODS We analyzed and pooled AVM radiosurgery data from 7 participating in the International Gamma Knife Research Foundation. Patients younger than 18 years of age who had at least 12 months of follow-up were included in the study cohort. Favorable outcome was defined as AVM obliteration, no post-radiosurgical hemorrhage, and no permanently symptomatic radiation-induced changes (RIC). The post-radiosurgery outcomes of unruptured versus ruptured pediatric AVMs were compared, and statistical analyses were performed to identify predictive factors. RESULTS The overall pediatric AVM cohort comprised 357 patients with a mean age of 12.6 years (range 2.8-17.9 years). AVMs were previously treated with embolization, resection, and fractionated external beam radiation therapy in 22%, 6%, and 13% of patients, respectively. The mean nidus volume was 3.5 cm3, 77% of AVMs were located in eloquent brain areas, and the Spetzler-Martin grade was III or higher in 59%. The mean radiosurgical margin dose was 21 Gy (range 5-35 Gy), and the mean follow-up was 92 months (range 12-266 months). AVM obliteration was achieved in 63%. During a cumulative latency period of 2748 years, the annual post-radiosurgery hemorrhage rate was 1.4%. Symptomatic and permanent radiation-induced changes occurred in 8% and 3%, respectively. Favorable outcome was achieved in 59%. In the multivariate logistic regression analysis, the absence of prior AVM embolization (p = 0.001) and higher margin dose (p < 0.001) were found to be independent predictors of a favorable outcome. The rates of favorable outcome for patients treated with a margin dose ≥ 22 Gy vs < 22 Gy were 78% (110/141 patients) and 47% (101/216 patients), respectively. A margin dose ≥ 22 Gy yielded a significantly higher probability of a favorable outcome (p < 0.001). The unruptured and ruptured pediatric AVM cohorts included 112 and 245 patients, respectively. Ruptured AVMs had significantly higher rates of obliteration (68% vs 53%, p = 0.005) and favorable outcome (63% vs 51%, p = 0.033), with a trend toward a higher incidence of post-radiosurgery hemorrhage (10% vs 4%, p = 0.07). The annual post-radiosurgery hemorrhage rates were 0.8% for unruptured and 1.6% for ruptured AVMs. CONCLUSIONS Radiosurgery is a reasonable treatment option for pediatric AVMs. Obliteration and favorable outcomes are achieved in the majority of patients. The annual rate of latency period hemorrhage after radiosurgery for both ruptured and unruptured pediatric AVM patients conveys a significant risk until the nidus is obliterated.

Periventricular Location as a Risk Factor for Hemorrhage and Severe Clinical Presentation in Pediatric Patients with Untreated Brain Arteriovenous Malformations.

The morphologic features of brain arteriovenous malformations differ between children and adults; therefore, our aim was to analyze various features of brain arteriovenous malformations to assess the risk of hemorrhage in children. We identified all consecutive children admitted to Beijing Tiantan Hospital for brain arteriovenous malformations between July 2009 and April 2014. The effects of demographic characteristics and brain arteriovenous malformation morphology on hemorrhage presentation, annual bleeding rates, postnatal hemorrhage, and immediate posthemorrhagic neurologic outcomes were studied by using univariate and multivariable regression analyses. A total of 108 pediatric brain arteriovenous malformation cases were identified, 66 (61.1%) of which presented with hemorrhage. Of these, 69.7% of ruptured brain arteriovenous malformations were in a periventricular location. Periventricular nidus location (OR, 3.443; 95% CI, 1.328-8.926; P = .011) and nidus size (OR, 0.965; 95% CI, 0.941-0.989; P = .005) were independent predictors of hemorrhagic presentation. The annual hemorrhage rates in children with periventricular brain arteriovenous malformations were higher at 6.88% (OR, 1.965; 95% CI, 1.155-3.341; P < .05). The hemorrhage-free survival rates were also lower for children with periventricular brain arteriovenous malformations (log-rank, P = .01). Periventricular location (hazard ratio, 1.917; 95% CI, 1.131-3.250; P = .016) and nidus size (hazard ratio, 0.983; 95% CI, 0.969-0.997; P = .015) were associated with hemorrhage after birth in pediatric brain arteriovenous malformations. An ordinal analysis showed lower immediate posthemorrhage mRS in patients with periventricular brain arteriovenous malformations (OR for greater disability, 2.71; 95% CI, 1.03-7.11; P = .043). Small periventricular brain arteriovenous malformations were associated with increased hemorrhage risk in pediatric patients. Cautious follow-up of children with untreated periventricular brain arteriovenous malformations is recommended because of a higher hemorrhage risk and potentially more severe neurologic outcomes.

Arterial spin labeling magnetic resonance imaging: toward noninvasive diagnosis and follow-up of pediatric brain arteriovenous malformations.

OBJECT Arterial spin labeling (ASL)-MRI is becoming a routinely used sequence for ischemic strokes, as it quantifies cerebral blood flow (CBF) without the need for contrast injection. As brain arteriovenous malformations (AVMs) are highflow vascular abnormalities, increased CBF can be identified inside the nidus or draining veins. The authors aimed to analyze the relevance of ASL-MRI in the diagnosis and follow-up of children with brain AVM. METHODS The authors performed a retrospective analysis of 21 patients who had undergone digital subtraction angiography (DSA) and pseudo-continuous ASL-MRI for the diagnosis or follow-up of brain AVM after radiosurgery or embolization. They compared the AVM nidus location between ASL-MRI and 3D contrast-enhanced T1 MRI, as well as the CBF values obtained in the nidus (CBFnidus) and the normal cortex (CBFcortex) before and after treatment. RESULTS The ASL-MRI correctly demonstrated the nidus location in all cases. Nidal perfusion (mean CBFnidus 137.7 ml/100 mg/min) was significantly higher than perfusion in the contralateral normal cortex (mean CBFcortex 58.6 ml/100 mg/min; p < 0.0001, Mann-Whitney test). Among 3 patients followed up after embolization, a reduction in both AVM size and CBF values was noted. Among 5 patients followed up after radiosurgery, a reduction in the nidus size was observed, whereas CBFnidus remained higher than CBFcortex. CONCLUSIONS In this study, ASL-MRI revealed nidus location and patency after treatment thanks to its ability to demonstrate focal increased CBF values. Absolute quantification of CBF values could be relevant in the follow-up of pediatric brain AVM after partial treatment, although this must be confirmed in larger prospective trials.

Safety and efficacy using a detachable tip microcatheter in the embolization of pediatric arteriovenous malformations

The treatment of deep-seeded pediatric brain arteriovenous malformations (AVMs) remains a challenging task. We describe our experience using a new detachable tip microcatheter in the embolization of brain arteriovenous malformations, pial arteriovenous fistulas, and vein of Galen malformations. We describe the safety and efficacy using a new detachable tip microcatheter in the treatment of pediatric deep brain arteriovenous malformations, pial malformations, and vein of Galen malformations. During a period of 9 months from March 2013 through January 2014, 11 pediatric patients in 14 procedures with 27 total injections were selected for treatment with a detachable tip under Food and Drug Administration (FDA) compassionate use exemption and were admitted to our department for treatment of their brain AVM using a liquid embolic agent and a detachable tip microcatheter. The ages of the patients ranged from 3 months to 18 years old. Of the 27 total injections done, the tip detached in seven cases. For the 16 n-BCA injections, the tip detached six times (37.5 %), and for the 11 Onyx injections, the tip detached one time (9 %). There were no cases of premature microcatheter detachment during normal vessel navigation. The introduction of these detachable tip microcatheters allows for a safe and relaxed injection that permits a true circumferential occlusion, and may further permit filling a larger amount of angioarchitecture without the risk of distal migration, or vessel damage during the usual rapid removal of non detachable micocatheters.