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Objective: Cerebral arteriovenous malformation during pregnancy is rare but lethal disease that usually present with new-onset seizures and headaches mimicking eclampsia. We report a rare case of cerebral arteriovenous malformation with abrupt seizures in the third trimester. Case Report: A 28-year-old primipara was brought to our emergency department at 32 6/7 weeks of gestation with new-onset acute seizures and hypertension. Owing to neurological deterioration, the patient underwent emergency cesarean delivery. However, 24 h after cesarean delivery and eclampsia treatment, the seizures worsened. Computed tomography and magnetic resonance imaging showed unruptured arteriovenous malformation of the right frontal lobe. Subsequently, intraarterial embolization was performed. The patient was discharged 5 days after surgery without neurological sequelae or obstetric complications. Conclusion: This case report highlights the differential diagnoses of sudden new-onset seizures in late pregnancy for obstetricians and emergency medicine physicians. Lethal cerebral diseases, apart from eclampsia, should be considered during pregnancy.

A 13-year-old boy, with a history of intermittent headache and transient diplopia, was found to have non-hemorrhagic cerebral arteriovenous malformation in the midbrain tegmental region associated with hydrocephalus. Gamma knife radiosurgery was performed at 16 Gy with 75% marginal dose. Posttreatment course was uneventful. Follow-up MR imaging at one year after the treatment revealed complete disappearance of the abnormal vascular flow voids. The size of each ventricle at the treatment and at one year after treatment were as follows; 60.2 cc and 20.9 cc in the lateral ventricles, 3.7 cc and 2.7 cc in the third ventricle. The hydrocephalus might be caused by obstructive mechanism but mostly by high venous pressure due to the shunt blood flow. The goal of treatment for hydrocephalus should be nidus obstruction and normalizing the vascular flow.

Objective: One of the pressing constraints in the treatment of arteriovenous malformations (AVM) is the potential development of new neurologic deficits, mainly when the AVM is in an eloquent area. The risk of ischemia when an en passage arterial supply is present is not negligible. In this regard, awake surgery holds promise in increasing the safety of low-grade AVM resection., Methods: We conducted a pilot trial on 3 patients with low-grade AVMs affecting speech areas to evaluate the safety of awake craniotomy using Conscious Sedation. Each feeder was temporarily clipped before the section. Also, we performed a systematic review to analyze the existing data about the impact of awake surgery in eloquent AVM resection., Results: None of the 3 patients presented with neurologic deficits after the procedure. Awake craniotomy was useful in 1 case, as it allowed the detection of speech arrest during the temporal clipping of 1 of the feeders. This vessel was identified as an en passage vessel, closer to the nidus. The second attempt revealed the feeder of the AVM, which was sectioned. Systematic review yielded 7 studies meeting our inclusion criteria. Twenty-six of 33 patients included in these studies presented with AVM affecting speech area. Only 2 studies included the motor evoked potentials. Six studies used direct cortical and subcortical stimulation. In all studies the asleep-awake-asleep technique was used., Conclusions: Awake craniotomies are safe procedures and may be helpful in avoiding ischemic complications in low-grade AVMs, either affecting eloquent areas and/or when en passage feeders are present., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Spontaneous regression of arteriovenous malformations (AVMs) is usually associated with bleeding, but sometimes asymptomatic regression occurs. Case description: We describe a case of spontaneous regression of AVM without evidence of hemorrhagic event. A 60-year-old otherwise healthy woman came to our hospital with complaints of headache. Digital subtraction angiography (DSA) showed an AVM with a 1.4 × 1.0 × 0.9 cm nidus in the right temporal lobe. The patient decided on observation without treatment rather than stereotactic radiation therapy or direct surgery. Seven years later, DSA revealed partial regression of the AVM. Venous ectasia and the nidus had decreased in size, and the nidus was obscure, especially around the feeding artery. No obvious features of hemorrhage were detected. Conclusions: Spontaneous regression of AVM is the result of multiple interacting factors. Structural changes to the AVM and histopathological changes may be important.

Vein of Galen malformations (VoGMs) are rare vascular malformations resulting from persistent shunting of primitive choroidal vessels into the median prosencephalic vein of Markowski. VoGMs are associated with poor clinical outcome with a reported 76.7% mortality if left untreated. We present an exceedingly rare case of a giant, untreated VoGM measuring 7.8 × 5.5 × 7 cm in a 42-year-old man. The embryologic origin, classification, clinical manifestations, and treatment options of VoGMs are discussed with a review of pertinent literature.

Objective: Complex cerebral arteriovenous malformations (AVMs) require a combined therapy of endovascular embolization and microsurgical resection to eliminate the lesion and maximize neurological protection, while a deliberate time interval might contribute to optimal clinical outcomes. The present study aimed to explore the feasibility of this paradigm., Methods: All patients who underwent deliberately planned presurgery embolization and microsurgery resection between 2015 and 2023 were reviewed, with baseline data, postoperative complications, and follow-up outcomes recorded. The modified Rankin scale (mRS) was used to evaluate clinical outcomes, with mRS 0-2 defined as good., Results: A total of 30 patients were included in the study (15 were ruptured AVMs). The median Spetzler-Martin grade of baseline AVMs was 3 (interquartile range: 2-3). The median interval between the last embolization and microsurgery was 5 days (interquartile range: 2.25-7). The complete removal rate was 100%, and the overall permanent complication rate was 16.67%. At the last follow-up, 26 patients achieved mRS 0-2, while 28 had improved or unaltered mRS. The last follow-up mRS significantly improved from baseline and discharge (P = 0.0006 and P = 0.006). The last follow-up mRS decreased by 0.65 for each additional day of time interval before the 4.4-day inflection point (β = -0.65, P = 0.02) in the AVM ruptured cohort., Conclusions: The deliberately staged combined procedure of embolization and microsurgery might be a safe and efficacious strategy for Spetzler-Martin grade 2-5 AVMs, 4-5 days might be an appropriate staged time interval for ruptured AVMs, although further studies are needed to substantiate these findings., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction: The current treatment paradigm for intracranial arteriovenous malformations (AVMs) focuses on reducing the risk of intracranial hemorrhage using various therapeutic means including embolization, stereotactic radiosurgery (SRS), and microsurgical resection. To improve AVM obliteration rates with SRS, pre-radiosurgical embolization has been trialed in a number of studies to reduce the volume of the AVM nidus prior to radiosurgery. This study aimed to review the efficacy of pre-radiosurgical embolization in the pre-Onyx era compared to the current Onyx era., Methods: A systematic review was performed using PubMed to identify studies with 20 or more AVM patients, embolization material, and obliteration rates for both embolization + stereotactic radiosurgery (E+SRS) and SRS-only groups., Results: Seventeen articles consisting of 1133 eligible patients were included in this study. A total of 914 (80.7%) patients underwent embolization prior to SRS. Onyx was used as the embolysate in 340 (37.2%) patients in the E+SRS cohorts. Mean obliteration rate for the embolized cohort was 46.9% versus 46.5% in the SRS-only cohort. When comparing obliteration rates based on embolysate material, obliteration rate was 42.1% with Onyx+SRS and 50.0% in the non-Onyx embolysate + SRS cohort., Conclusions: Onyx (ethylene vinyl-alcohol copolymer dissolved in dimethyl sulfoxide and suspended in micronized tantalum powder) has been increasingly used for the embolization of intracranial AVMs with increased success regarding its ease of use from a technical standpoint and performs similarly to other embolysate materials., (Copyright © 2022 The Author(s). Published by Elsevier Inc. All rights reserved.)

Objective: An understanding of the complex morphology of an arteriovenous malformation (AVM) is important for successful resection. We have previously reported the utility of intra-arterial indocyanine green (ICG) videoangiography for this purpose, but that method cannot detect the angioarchitecture covered by brain tissue. 3-dimensional (3D) multimodal fusion imaging is reportedly useful for this same purpose, but cannot always visualize the exact angioarchitecture due to poor source images and processing techniques. This study examined the results of utilizing both techniques in patients with AVMs., Methods: Both techniques were applied in 12 patients with AVMs. Both images were compared with surgical views and evaluated by surgeons., Results: Although evaluations for identifying superficial feeders by ICG videoangiography were high in all cases, the more complicated the AVM, the lower the evaluation by 3D multimodal fusion imaging. Conversely, evaluation of the estimated range of the nidus was high in all cases by 3D multimodal fusion imaging, but low in all but one case by ICG videoangiography. Nidus flow reduction was recognized by Flow 800 analysis obtained after ICG videoangiography., Conclusions: These results showed that utilizing both techniques together was more useful than each modality alone in AVM surgery. This was particularly effective in identifying superficial feeders and estimating the range of the nidus. This technique is expected to offer an optimal tool for AVM surgery., (Copyright © 2022 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Cerebral arteriovenous malformations (cAVMs) represent tangles of abnormal vasculature without intervening capillaries. High-pressure vascular channels due to abnormal arterial and venous shunts can lead to rupture. Multiple pathways are involved in the pathobiology of cAVMs including inflammation and genetic factors such as KRAS mutations. Neutrophil release of nuclear chromatin, known as neutrophil extracellular traps (NETs), plays a multifunctional role in infection, inflammation, thrombosis, intracranial aneurysms, and tumor progression. However, the relationship between NETs and the pathobiology of cAVMs remains unknown. We tested whether NETs play a role in the pathobiology of cAVMs., Methods: We analyzed samples from patients who had undergone surgery for cAVM and immunohistochemically investigated expression of citrullinated histone H3 (CitH3) as a marker of NETs. CitH3 expression was compared among samples from cAVM patients, epilepsy patients, and normal human brain tissue. Expressions of thrombotic and inflammatory markers were also examined immunohistochemically in samples from cAVM patients., Results: Expression of CitH3 derived from neutrophils was observed intravascularly in all cAVM samples but not other samples. Nidi of AVMs showed migration of many Iba-I-positive cells adjacent to the endothelium and endothelial COX2 expression, accompanied by expression of IL-6 and IL-8 in the endothelium and intravascular neutrophils. Unexpectedly, expression of CitH3 was not necessarily localized to the vascular wall and thrombus., Conclusions: Our results offer the first evidence of intravascular expression of NETs, which might be associated with vascular inflammation in cAVMs., (Copyright © 2021 The Author(s). Published by Elsevier Inc. All rights reserved.)

Objective: Acute hemorrhage caused by cerebral arteriovenous malformation (cAVM) during pregnancy is uncommon but life-threatening for both mother and fetus and presents a great challenge to clinical management. However, there is still no consensus on the treatment strategy and the treatment timing of acute hemorrhage from cAVM during pregnancy. The aim of this study was to amalgamate reported case series and our cases regarding the clinical management of pregnant patients under this special condition., Methods: We report a case series of 3 pregnant patients with acute hemorrhage caused by cAVM in our hospital. A systematic PubMed search of the English-language literature published between 1970 and 2020 was carried out. Clinical information including patients' age, gestational age, imaging studies, treatment strategy, treatment timing, delivery mode, and outcomes were collected and analyzed., Results: The rebleed rate is about 7.1% and the mortality from rebleeding is up to 25%. Treatment modalities included radical surgery, endovascular embolization, radiosurgery/stereotactic radiosurgery, palliative surgery, and conservative treatment. There were no maternal deaths in either the intrapartum intervention group and the postpartum intervention subgroup of gestational age <34 weeks., Conclusions: A high rebleed rate and high mortality from rebleeding indicate that the intervention of ruptured cAVM should not be delayed. Intervention of ruptured cAVM within 2 weeks after initial hemorrhage is advisable in patients at gestational age <34 weeks, whereas termination of pregnancy as soon as possible followed by timely intervention of ruptured cAVM is practicable in patients at gestational age ≥34 weeks., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objective: Cerebral arteriovenous malformation (AVM) is characterised by an abnormal tangle of arteries and veins, the rupture of which is a significant portion of the morbidity and mortality cases, especially in young populations. However, the exact risk factors and pathophysiologic mechanisms of AVM remain poorly understood. RNF213 variants have been identified as obvious susceptible factors of several cerebrovascular disorders, such as Moyamoya disease and intracranial aneurysms. Thus, this study aimed to determine whether there is an association between RNF213 rare variants and AVM., Methods: The AVM group included 22 patients with AVM. The control group included 1007 samples from the GeneSky in-house database and 208 samples from the 1000 Genome Project of Chinese Han Population. Genomic DNA samples were extracted from the peripheral blood of the AVM patients, and targeted exome sequencing of RNF213 was performed to assess the existence of low-frequency or rare variants. Sanger sequencing was performed to validate the identified variants. Logistic regression analysis was performed to calculate the odds ratios (ORs) and 95 % confidence intervals (CIs) of the candidate variants and risk of AVM. Statistical analyses were performed using SPSS version 21.0., Results: The RNF213 c.10997T>C variant (amino acid mutation p.M3666T, NM&#95;001256071) was observed in two AVM patients after filtration. It was significantly associated with AVM in the Chinese population (ORs, 10.30 and 25.08; 95 %; CIs, 1.38-77.10 and 4.34-144.90 compared with 1000 Genome Project of Chinese Han Population and GeneSky in-house database, respectively)., Conclusion: Rare variants of RNF213 are associated with AVM in the Chinese population, suggesting the important role of RNF213 in AVM. Further studies are needed to verify these findings., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Background: Although carcinoma metastasis to primary intracranial neoplasms has occasionally been reported, metastasis to a cerebral arteriovenous malformation (AVM) has been exceedingly rare, with only 5 cases reported to date. In the present study, we have reported a case of lung carcinoma that had metastasized to a cerebral AVM. To the best of our knowledge, the present report is the first case in which the pathological examination detected the bleeding mechanism of this rare condition, showing destruction of the feeders by the metastatic tumor., Case Description: A 61-year-old man who had had a tumor shadow in the right middle lung field identified at a medical examination 5 weeks previously had suddenly experienced a disturbance of consciousness. Head computed tomography and computed tomography angiography revealed a right occipital subcortical hemorrhage with abnormal vessels, suggesting a ruptured AVM. Magnetic resonance imaging with gadolinium-based contrast agents did not show any other lesions. Cerebral angiography revealed a Spetzler-Martin grade III AVM in the right occipital lobe. Endovascular feeder embolization and subsequent removal of the AVM were performed. Histopathological examination of the resected mass showed a small cell carcinoma that had metastasized to the AVM. The tumor cells had infiltrated to the vessel walls of the feeders, which might have elicited the bleeding., Conclusion: Although rare, clinicians should recognize that undifferentiated carcinomas can metastasize to AVMs and cause bleeding. Because the preoperative diagnosis can be difficult, even using the latest imaging modalities, careful examination of the resected specimen is required to reveal such pathological conditions., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Endovascular microcatheter adherence and retention is an uncommon complication during brain arteriovenous malformation (AVM) embolization with glue or ethylene-vinyl alcohol copolymer that has previously reported, although there are sparse reports of symptomatic complications thereafter., Case Description: We present a unique complication 6 years after initial embolization of a cerebral AVM. The patient presented with acute lower limb insufficiency with computed tomography angiogram revealing fragmentation of the microcatheter and associated popliteal aneurysm. The patient underwent an emergency grafting and removal of the retained fragment and recovered without deficit postoperatively., Conclusions: Fragmentation over retained microcatheters remains a concern that may be worth monitoring in the long term, although there are no recommendations for timing., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objectives: Cerebral arteriovenous malformations (AVMs) are congenital malformations, and right-sided dominance of the language cortex is not a rare phenomenon for patients with AVM involving language area. We tried to use voxel-based lesion-symptom mapping (VLSM) method to depict the location of AVM nidus and to demonstrate the relationship between AVM location and the pattern of language cortex reorganization., Patients and Methods: The authors retrospectively reviewed clinical and imaging data of 70 adult patients with unruptured cerebral AVMs who underwent blood oxygen level-dependent (BOLD) functional magnetic resonance imaging (fMRI) of language task. All patients were right handed, and all lesions were located in the left cerebral hemisphere. Lateralization indexes (LI) of the BOLD signals were calculated for Broca and Wernicke areas separately and were used to reflect the degree of right-sided dominance of the two language areas. VLSM method was applied to study the relationship between AVM location and LI of language task activations., Results: Statistical analysis revealed that the change of LI of Broca area was significantly associated with lesions located in the inferior frontal gyrus, pre- and post-central gyrus, supramarginal gyrus and middle frontal gyrus. The change of LI of Wernicke area was significantly associated with lesions located in the left superior, middle, inferior and transverse temporal gyrus., Conclusion: These findings provide new evidence that the language cortex reorganization patterns in AVM patients have anatomic specificity., Competing Interests: Declaration of Competing Interest The authors declare that they have no conflict of interest., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Background: Endovascular embolization is a minimally invasive interventional method for the treatment of neurovascular pathologies such as aneurysms, arterial stenosis or arteriovenous malformations (AVMs). In this context, neuroradiologists need efficient tools for interventional planning and microcatheter embolization procedures optimization. Thus, the development of helpful methods is necessary to solve this challenging issue., Methods: A complete pipeline aiming to assist neuroradiologists in the visualization, interpretation and exploitation of three-dimensional rotational angiographic (3DRA) images for interventions planning in case of AVM is proposed. The developed method consists of two steps. First, an automated 3D region-based segmentation of the cerebral vessels which feed and drain the AVM is performed. From this, a graph-like tree representation of these connected vessels is then built. This symbolic representation provides a vascular network modelization with hierarchical and geometrical features that helps in the understanding of the complex angioarchitecture of the AVM., Results: The developed workflow achieves the segmentation of the vessels and of the malformation. It improves the 3D visualization of this complex network and highlights its three main components that are the arteries, the veins and the nidus. The symbolic representation then brings a better comprehension of the vessels angioarchitecture. It provides decomposition into topologically related vessels, offering the possibility to reduce the complexity due to the malformed vessels and also determine the optimal paths for AVM embolization during interventions planning., Conclusions: A relevant vascular network modelization has been developed that constitutes a breakthrough in the assistance of neuroradiologists for AVM endovascular embolization planning., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Background: Cerebral arteriovenous malformations (CAVMs) are characterized by altered hemodynamics and associated with angioanatomic changes, such as aneurysms. We encountered a patient with a CAVM-associated dissection of the medial trunk of the posterior inferior cerebellar artery (PICA) instead of an aneurysm., Case Description: We report the case of a 56-year-old male patient with spontaneous subarachnoid hemorrhage within the cisterna magna and fourth ventricle. Digital subtraction angiography revealed a cerebellar arteriovenous malformation located within the inferior semilunar lobule that did not anatomically match the bleeding pattern. The left PICA, serving as a primary feeding artery, showed a dissection of the proximal portion of the medial trunk with a precise anatomic association with the blood in the telovelotonsillar space. CAVM-induced hyperdynamic flow through the feeding vessel is the most plausible pathophysiologic explanation for the dissection. Complete microsurgical resection of the CAVM was performed, and 3-month follow-up digital subtraction angiography showed complete regression of the dissection, disappearance of irregularities, and significant caliber reduction of the medial PICA trunk., Conclusions: This is the first report of a hyperdynamic flow-related dissection of a CAVM-associated feeding vessel. Microsurgical resection of the CAVM allowed for spontaneous resolution of the dissected area by restoration of normal rheodynamics., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Whether the risk of rupture of a cerebral arteriovenous malformation (AVM) increases during pregnancy remains controversial. Moreover, it is unclear whether the number of pregnancies correlates with AVM rupture risk. We report a case of ruptured AVM during the fifth pregnancy., Case Description: A 34-year-old woman presenting sudden headache and vomiting was admitted to our hospital. Neuroimaging revealed a subarachnoid hemorrhage due to a ruptured AVM in the right temporal lobe. She was pregnant (9 weeks and 1 day), and she had previously experienced 4 normal vaginal deliveries (gravidity and parity G 4 P 4 ) without complications. Elective treatment including neuroendovascular and direct surgery was performed, and the AVM was safely resected (at 15 weeks, 6 days of pregnancy). She underwent elective caesarean section without complications 151 days after the AVM resection (at 37 weeks, 1 day of pregnancy), and the baby was healthy., Conclusions: This report suggests that the risk of AVM rupture persists even after multiple deliveries. Intracranial hemorrhage should be suspected in pregnant patients who underwent multiple deliveries, and a rapid diagnosis and appropriate treatment are necessary., (Copyright © 2018. Published by Elsevier Inc.)

Background: Successful resection of arteriovenous malformation (AVM) depends on preoperative assessment of the detailed morphology of the AVM. Simultaneous detailed three-dimensional visualization of the feeding arteries, draining veins, and surrounding structures is needed. The aim of this study was to evaluate the usefulness of high-resolution three-dimensional multifusion medical imaging (HR-3DMMI) for preoperative planning of AVM resection., Methods: HR-3DMMI combined magnetic resonance imaging, magnetic resonance angiography, thin-slice computed tomography, and three-dimensional rotational angiography. Surface rendering was mainly used for creation of HR-3DMMI using multiple thresholds to create three-dimensional models. HR-3DMMI technique was used in 8 patients for preoperative planning, and imaging findings were compared with operative findings., Results: All feeding arteries and draining veins were found intraoperatively at the same position as estimated preoperatively and were occluded as planned preoperatively., Conclusions: HR-3DMMI technique demonstrated the precise locations of feeding arteries, draining veins, and surrounding important tissues, such as corticospinal tract and arcuate fiber, preoperatively and estimated the appropriate route for resection of the AVM. HR-3DMMI is expected to be a very useful support tool for surgery of AVM., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Radical treatment of brain arteriovenous malformations (AVMs) remains a challenge despite recent advances in endovascular, microsurgical, and stereotactic radiosurgery treatments. The radical removal of AVMs with prevention of hemorrhage can be achieved through either single or combined modalities. Our retrospective study summarizes the safety and efficacy of combined endovascular embolization and microsurgical removal of AVMs in 49 consecutive patients in the hybrid operating room (hOR)., Methods: Patients underwent nonhybrid (i.e., endovascular, stereotactic radiosurgery, observation, combination) or hybrid (i.e., endovascular with microsurgery in hOR) treatment. The variables analyzed included age, sex, hospitalization time, Spetzler-Martin/Buffalo grades, clinical symptoms, previous treatment, intraoperative digital angiographic (iDSA) findings, postsurgical treatment, operation duration, complications, obliteration rate, and remnants., Results: Of 18 patients treated in the hOR, 15 (83%) presented with Spetzler-Martin grades I and II and were younger than those treated with other modalities (mean age, 41 vs. 46 years). Thirteen (87%) patients were admitted with signs of hemorrhage after AVM rupture. The initial surgical plan was modified in 3 (17%) of 18 cases based on iDSA findings. All patients who eventually underwent microsurgical removal of AVMs showed neither postinterventional remnant nor new neurologic signs and symptoms. Complications included 1 postoperative epidural hematoma and 1 vasospasm during endovascular treatment., Conclusions: Treatment of AVMs in the hOR offers the possibility to adapt surgical approaches based on iDSA findings after intraoperative embolization, thus achieving better clinical and radiologic results. Radical treatment of AVMs with low Spetzler-Martin grades by combining endovascular and microsurgical techniques in the hOR was technically feasible, effective, and safe., (Copyright © 2018 Elsevier Inc. All rights reserved.)

An elevated end-diastolic (ED) ratio of the common carotid artery (CCA) is an indicator of occlusive lesions of the distal portion of the internal carotid artery. We report 2 cases of cerebral arteriovenous malformation (AVM) showing an elevated ED ratio of the CCA, which decreased after surgery. Case 1 was a 28-year-old man with chronic recurrent headache with aura, and case 2 was a 29-year-old woman with sudden-onset headache and intracerebral hemorrhage without neurologic abnormality. In both cases, digital subtraction angiography revealed a Spetzler-Martin Grade IV AVM, which was mainly fed by branches of the left middle cerebral artery with venous drainage into superficial and deep cerebral veins. Preoperative carotid ultrasonography showed an elevated CCA ED ratio (1.38 in case 1 and 1.47 in case 2; left > right) without atherosclerotic lesions. Patients' AVMs were successfully resected. In both cases, the ED ratio was decreased after surgery (to 1.05 in case 1 and 1.20 in case 2). A decrease in vascular resistance on 1 side caused by cerebral AVM can result in an increase in the CCA ED ratio comparable to that of carotid axis occlusion.

Objective: The goal of this study is to report our experience in the surgical treatment of cerebral arteriovenous malformations (cAVMs) related permanent symptomatic adverse radiation effects (PSAREs), to clarify an appropriate surgical management and to identify the risk factors related to their development., Methods: We evaluated 549 patients treated with Gamma Knife radiosurgery (GKRS) for cAVMs with a follow-up of at least 8 years. Univariate and multivariate analyses were used to test different risk factors related to the development of PSARE. We retrospectively reviewed the records of these patients to analyze the clinical outcome., Results: Fourteen patients (2.5%) developed PSARE and were submitted to surgery. Higher average treated volume represents a significant risk factors for the development of PSARE (P < 0.05); on the other hand, older age and higher average dose reduce the risk of PSARE (P < 0.05). A favorable clinical outcome was achieved in 13 patients (93%) after surgery; in 1 patient, the unfavorable outcome was due to hemorrhage that occurred months after GKRS. Serial MRI scans following either surgical removal of the nodule or Ommaya reservoir positioning showed progressive reduction of brain edema in all cases., Conclusions: The management of PSARE is controversial, especially for cAVMs treated with SRS. Surgical removal is rarely needed, but-if unavoidable-it can be a valuable option in experienced hands. A careful preoperative planning is always necessary to detect pathologic blood flow through the PSARE., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: An arteriovenous malformation (AVM) consists of a pathologic arteriovenous shunt formed from a tangle of vessels lacking a capillary bed. AVMs were previously accepted as congenital in nature; however, an increase in the number of reported de novo cerebral AVMs challenges the assertion that all AVMs develop in utero hence, the possibility of these lesions presenting postnatally cannot be excluded. A review of literature revealed 31 published cases of de novo AVM formation between 1996 and 2017., Case Description: We present the case of a 7-year-old boy with a history of an intracranial aneurysm that was found to have an asymptomatic de novo AVM formation on a routine follow-up angiography. He was being followed since the age of 8 months when the aneurysm was incidentally found during work-up for mild hemiparesis. At that time, magnetic resonance imaging revealed a 4-mm right posterior communicating artery aneurysm along with a right hemispheric stroke and cortical dysplasia. No arteriovenous malformation was noted., Conclusions: The exact pathophysiology of de novo AVMs is not completely understood, but a combination of genetic, molecular, and environmental factors may play a role. Our case report only adds to the growing body of clinical evidence supporting the notion that these lesions may be acquired. It would be appropriate to consider an AVM as an abnormal vascular response to an injury rather than a static congenital lesion., (Copyright © 2017. Published by Elsevier Inc.)

Objective: To review outcomes after linear accelerator stereotactic radiosurgery (SRS) and hypofractionated stereotactic radiotherapy (hfSRT) of arteriovenous malformations (AVMs) from a consecutive and pooled series of 2 Novalis centers and to analyze the influence of AVM size, Spetzler-Martin (SM) grade, pretreatment, and hemorrhagic versus nonhemorrhagic presentation. A subgroup analysis of A Randomized Trial of Unruptured Brain Arteriovenous Malformations (ARUBA)-eligible patients also was performed., Methods: Prospectively collected treatment and outcome data were supplemented by retrospectively collected follow-up data for 93.8% of all patients. A total of 129 patients with AVM had SRS or hfSRT between 2000 and 2014 with the same linear accelerator system in 2 centers. Data analysis included initial presentation, SM grade, occlusion rates assessed by magnetic resonance and/or digital subtraction angiography, neurologic and therapeutic complications, and pretreatments. Statistical analysis was performed for patient demographic data and for factors potentially influencing outcome., Results: Initial presentation was hemorrhage in 43.8% or seizures/neurologic deficits in 46.2%. The series included 6 SM grade I (5%), 26 SM II (21.5%), 55 SM III (45.5%), 28 SM IV (23%), and 6 SM V cases (5%). Pre-embolization was used in 36 patients (29.8%), 8 patients had previous surgery (6.6%), and 6 patients were irradiated before elsewhere (5%); 5 patients (4.2%) received multimodal pretreatment. Mean follow-up was 43 months. The occlusion rate for the total series was 71.1%, for SM I/II cases 80.6%, and 67.4% for the SM ≥ subgroup. The occlusion rate was 75.0% for the small volume (<4 cc) and 55.6% for the large volume (>10 cc) subgroup. There was no statistical difference between the occlusion rate of patients with or without pretreatment if taken all modalities together (72.7% and 69.7%, respectively). There was only a trend of a belated occlusion of pre-embolized AVMs. The occlusion rate for hemorrhagic AVM was with 77.4% better than for nonhemorrhagic (66.2%) or ARUBA-eligible AVMs (64.8%) but without reaching statistical significance. Neurologic deterioration was seen in 13.2% of the patients. There were 2 re-bleedings within 17-18 months (1.7%), 1 of them without a new neurologic deficit and total occlusion after re-SRS. One patient with pre-existing epilepsy died a sudden unexpected death (mortality rate: 0.8%)., Conclusions: Overall SRS and hfSRT are valuable therapy options, especially in symptomatic patients with AVM, with a low rate of morbidity and mortality and an acceptable overall complete occlusion rate of >70% and >80% for SM I/II AVMs., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Bleeding secondary to recurrences of spontaneously obliterated arteriovenous malformations (AVMs) is an extremely rare occurrence., Case Description: We report a 25-year-old man with cerebellar hemorrhage secondary to a recurrent AVM. His current admission with bleeding from a ruptured cerebellar AVM followed a previous presentation 15 years earlier with the similar clinical picture of AVM rupture within the same vascular territory. At that time, he was managed conservatively with follow-up digital subtraction angiography (DSA) 2 years later, confirming no residuum of the AVM. At the current presentation, he had DSA confirming AVM recurrence. He was managed by complete excision of the AVM via a suboccipital craniotomy., Conclusion: This case illustrates the need for long-term imaging follow-up to exclude recanalization even many years after AVM obliteration., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: The relationship between preoperative functional findings, derived from functional magnetic resonance imaging and diffusion tensor imaging, and surgical outcomes in patients with motor cortical arteriovenous malformations (AVMs) has not been determined. The aim of this study was to determine the relationship between preoperative functional findings and surgical outcomes in patients with motor cortical AVM., Methods: Fifteen patients with AVM involving the precentral knob and/or paracentral lobule were reviewed. Each motor function involved was examined as an independent object. Preoperative functional findings included cortical reorganization, activation around the nidus, corticospinal tract (CST) reorganization, nidus involving the CST, and the level of the CST involved. The relationship between preoperative functional findings and surgical outcomes was analyzed statistically. A muscle strength of grade 3 or less was defined as a poor outcome after surgery, and a muscle strength of grade 4 or greater was defined as a good outcome., Results: Eighteen objects were created and analyzed. Cortical reorganization (P = 1.000) was not correlated with surgical outcomes. However, nidus involving the CST was significantly associated with poor short-term outcomes (1 week after surgery) (P = 0.028) and permanent deficit in muscle strength (P = 0.042). Most of the objects had good outcomes (16/18, 88.9%) at 6 months after surgery (long-term outcomes). Only 1 patient with CST ruptured at the corona radiata level and 1 patient who had postoperative hemorrhage obtained poor long-term outcomes., Conclusions: Nidus involving the CST and the level of the CST involved, rather than cortical reorganization, may be associated with surgical outcomes in patients with motor cortical AVM., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: We aimed to evaluate microsurgical outcomes after classifying Grade III arteriovenous malformations (AVMs) according to Lawton's modified Spetzler-Martin grading system., Methods: Of 131 patients with Grade III AVMs, 55 had undergone microsurgery between 1995 and 2010. The 55 AVMs were classified as follows: Grade III-/S1E1V1, Grade III/S2E0V1, Grade III+/S2E1V0, or Grade III*/S3E0V0. The surgical obliteration rate, morbidity rate, and functional outcomes for each subtype were compared before surgery and after follow-up. Additionally, factors related with morbidity were investigated from demographic and morphological characteristics., Results: We observed 18 Grade III-, 16 Grade III, 20 Grade III+, and 1 Grade III* AVMs. Complete resection was achieved in 49 patients (obliteration rate, 89.1%). Incomplete resection rates were higher for Grade III (12.5%) and III+ (15.0%) AVMs than that for Grade III- (5.6%) AVMs. Seven patients (12.7%) presented postoperative deficits, of which 3 (5.4%) experienced disabilities. Patients with Grade III+ (25.0%) had higher morbidity rates than those with other subtypes. Modified Rankin scale scores at the last follow-up indicated unfavorable outcomes for Grades III (18.8%) and III+ (25.0%) AVMs. AVM size (≥3 cm) and non-hemorrhagic type were associated with the occurrence of postoperative deficits (p<0.05)., Conclusion: The modified classification of Grade III AVMs was useful to predict surgical morbidity and clinical outcomes. We recommend that microsurgery should be used to treat Grade III- AVMs, but should be considered carefully for the treatment of Grades III and III+., (Copyright © 2014 Elsevier B.V. All rights reserved.)

Objective: We present the case of a professional jazz guitarist with temporal lobe epilepsy secondary to an arteriovenous cerebral malformation., Case Description: The patient underwent a left temporal lobectomy in 1980. After surgery, he presented with severe retrograde amnesia and complete loss of musical interest and capabilities. The patient's musical abilities recovered over time, and he regained his previous virtuoso status. In 2007, his medical history, neuropsychologic functions, and structural magnetic resonance imaging study were examined and revealed a remarkable degree of recovery of memory and musical abilities in the context of extensive temporal lobe resection. The neuropsychologic findings and neuroanatomic features of the magnetic resonance imaging study were analyzed to try to understand the high degree of recovery of both long-term memory and musical processing abilities in this musician., Conclusions: This case reveals the possibility of an unusual degree of cerebral plasticity and reorganization. Additionally, it emphasizes the question of musical virtuosity. This report shows that the musical capabilities of professional musicians, in specific cases, can completely recover even when much of the left temporal lobe has been removed., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Diffuse pulmonary arteriovenous malformations (AVMs) are associated with a poor prognosis and the therapeutic strategy remains controversial. We describe a pediatric patient with diffuse pulmonary AVMs associated with hereditary hemorrhagic telangiectasia (HHT), who presented with two cerebral AVMs in the parietal and occipital lobes as well. Of note, successful bilateral lung transplantation not only improved the hypoxemia but also resulted in size reduction of the cerebral AVMs. Although it is essential to consider involvements other than pulmonary AVMs, especially brain AVMs, to decide the indication, lung transplantation can be a viable therapeutic option for patients with diffuse pulmonary AVMs and HHT., (© Copyright 2013 The American Society of Transplantation and the American Society of Transplant Surgeons.)