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Background: Even in the modern endovascular era, the treatment of Vein of Galen Malformations (VOGM) is extremely challenging. While their natural history is very poor, endovascular embolization has emerged as the standard of care. These lesions often require multiple treatment sessions to decrease shunting, with each treatment including multiple pedicles. Here we present the first reported use of the Scepter Mini (Microvention, Aliso Viejo, CA) in the treatment of vein of Galen malformations. Clinical presentation: A 7 month old female presented with an enlarging VOGM that was initially identified on prenatal ultrasound. Given the enlarging size of the lesion and failure to meet developmental milestones, the patient underwent planned endovascular embolization of the VOGM. The novel Scepter Mini balloon catheter was used for treatment of this lesion affording easy access to the target pedicle and immediate flow arrest which allowed for immediate cure of the lesion. Conclusion: The novel Scepter Mini Balloon (Microvention, Aliso Viejo, CA) afforded excellent distal access with subsequent immediate flow arrest therefore facilitating endovascular cure. Initially, a staged approach was favored for the treatment of the lesion, but the flow arrest achieved by the Scepter mini facilitated immediate occlusion from a single pedicle. [ABSTRACT FROM AUTHOR]

The roles of the Notch pathway proteins in normal adult vascular physiology and the pathogenesis of brain arteriovenous malformations are not well-understood. Notch 1 and 4 have been detected in human and mutant mice vascular malformations respectively. Although mutations in the human Notch 3 gene caused a genetic form of vascular stroke and dementia, its role in arteriovenous malformations development has been unknown. In this study, we performed immunohistochemistry screening on tissue microarrays containing eight surgically resected human brain arteriovenous malformations and 10 control surgical epilepsy samples. The tissue microarrays were evaluated for Notch 1-4 expression. We have found that compared to normal brain vascular tissue Notch-3 was dramatically increased in brain arteriovenous malformations. Similarly, Notch 4 labelling was also increased in vascular malformations and was confirmed by western blot analysis. Notch 2 was not detectable in any of the human vessels analysed. Using both immunohistochemistry on microarrays and western blot analysis, we have found that Notch-1 expression was detectable in control vessels, and discovered a significant decrease of Notch 1 expression in vascular malformations. We have demonstrated that Notch 3 and 4, and not Notch 1, were highly increased in human arteriovenous malformations. Our findings suggested that Notch 4, and more importantly, Notch 3, may play a role in the development and pathobiology of human arteriovenous malformations. [ABSTRACT FROM AUTHOR]

Endovascular embolization is being increasingly used to treat cerebral arteriovenous malformations. Common complications associated with embolization include intracranial hemorrhage and ischemic stroke; intracranial infections seldomly occur and are even less frequently reported. Although abscess formation after embolization is exceedingly rare, it is a serious condition that warrants immediate attention. This 53-year-old male with a ruptured left temporal-occipital arteriovenous malformation. He underwent uncomplicated 2-stage embolization with Onyx and was discharged. Five weeks after embolization, he returned to the hospital with worsening aphasia and contralateral hemiparesis, and a cerebral abscess was detected at the site of embolization. Bacterial cultures were positive for Escherichia coli , and he was treated successfully with surgical excision of the abscess and Onyx material, followed by long-term antibiotics. Although rare, formation of an intracranial abscess after endovascular embolization is a potential complication in the treatment of arteriovenous malformations. [ABSTRACT FROM AUTHOR]

Resection of deep medial frontal and parietal arteriovenous malformations (AVMs) is often challenging due to a tangential angle of attack and deep, narrow working corridor. Adequate visualization of the AVM and its feeding arteries without brain retraction is of particular importance when operating in or near eloquent cortical areas, where brain manipulation could inadvertently result in neurologic deficits. The aim of this paper is to provide a step-by-step description of surgical approach and report our experience with the contralateral transfalcine approach for resection of deep-seated parasagittal AVMs. Contralateral transfalcine resection of deep frontal, parietal, and cingulate gyrus AVMs was performed with the unaffected hemisphere positioned in a gravity-dependent manner in 5 cases. Surgical procedures were video documented, and an illustrative case is presented. All 5 patients had a modified Rankin Scale score of 0 or 1 at the last follow-up. Complete resection of the AVM was achieved in all 5 cases. No permanent major neurologic deficit was observed postoperatively. This approach allowed a superior visualization of arterial feeders, the parenchymal side of the AVM, and an early control of small parenchymal feeders while minimizing retraction of the brain. The contralateral transfalcine approach is a useful technique in the cerebrovascular surgeon's armamentarium for management of deep-seated medial frontal, parietal, and cingulate gyrus AVMs in or around eloquent brain areas, allowing to minimize normal brain retraction and avoid associated neurologic deficits. [ABSTRACT FROM AUTHOR]

Dural arteriovenous fistulas (dAVFs) are rare vascular malformations of uncertain pathophysiology. If associated with cortical venous reflux, they present a significant risk of hemorrhage. We present a 69-year-old patient who presented with spontaneous right-sided symptomatic chronic subdural hematoma, which was evacuated via burr hole. Due to lack of history of prior trauma, computer tomography angiography was performed, which showed dilated convoluted vessels in the right occipital region and an enlarged right posterior cerebral artery, suspected to be an arteriovenous malformation or dAVF. Subsequent angiography demonstrated occipital tentorial dAVF with primary cortical venous reflux (Cognard III) and reflux into the transverse sinus, fed primarily from the petrosquamous branch of the middle meningeal artery and neuromeningeal trunk. The fistula was initially treated by transarterial endovascular embolization of the occipital artery and neuromeningeal trunk with Onyx, achieving complete obliteration. However, follow-up angiography demonstrated recurrence of the lesion fed from the contralateral middle meningeal artery and pial branches of the enlarged right posterior cerebral artery. Given the recruitment of the contralateral supply from the left middle meningeal artery and ipsilateral posterior cerebral artery, we felt that surgical disconnection of the fistula was the best option for the patient. An occipital craniotomy with disconnection of the fistula without isolation of the transverse sinus was performed (Video 1), as published literature demonstrated lower intraoperative risk with disconnection only. The perioperative course was uneventful. Immediate postoperative and follow-up angiography demonstrated complete occlusion of the fistula. [ABSTRACT FROM AUTHOR]

Brain arteriovenous malformations (BAVMs) are frequently managed by endovascular embolization with a growing number of centers embolizing with intent to cure. Hemorrhage post-embolization is a severe and poorly understood complication. We present a novel imaging finding associated with post-embolization hemorrhage that has significantly impacted the management of patients at our institution.A retrospective review of all patients undergoing embolization of BAVM at a single center was performed. Post-embolization magnetic resonance imaging (MRI) was reviewed for the presence of T2 fluid-attenuated inversion recovery (FLAIR) hyperintense vessels (FHVs). Bivariate analysis was performed to determine associations between patient characteristics and risk of hemorrhage.A total of 50 patients underwent 75 embolization procedures. Forty-six post-embolization MRIs were available for review. There were four hemorrhages and 100% of those presented with FHV. In contrast, only 11.9% of embolization procedures without post-procedural hemorrhage had FHVs on MRI. In total, 18.7% of embolizations led to some morbidity or mortality, with only 6.7% leading to permanent morbidity or mortality. In bivariate analysis, only the presence of FHVs was correlated with the risk of hemorrhage (p < 0.05).This is the first series to describe the finding of hyperintense blood vessels on FLAIR imaging after embolization of BAVMs and correlate it with hemorrhage post embolization. This finding can help guide practitioners and potentially identify patients at risk of delayed hemorrhage post embolization. [ABSTRACT FROM AUTHOR]

Objective Awake craniotomy for removal of intra-axial lesions is a well-established procedure. Few studies, however, have investigated the usefulness of this approach for resection of arteriovenous malformations adjacent to eloquent language areas. We demonstrate our experience by using cortical stimulation mapping and report for the first time on the usefulness of subcortical stimulation with interrogation of language function during resection of arteriovenous malformations (AVMs) located near language zones. Methods Patients undergoing awake craniotomy for AVMs located in language zones and at least 5 mm away from the closest functional magnetic resonance imaging activation were analyzed. During surgery, cortical bipolar stimulation at 50 Hz, with an intensity of 2 mA, increased to a maximum of 10 mA was performed in the region around the AVM before claiming it negative for language function. In positive language site, the area was restimulated 3 times to confirm the functional deficit. The AVM resection was started based on cortical mapping findings. Further subcortical stimulation performed in concert with speech interrogation by the neuropsychologist continued at key points throughout the resection as feasible. The usefulness of cortical and subcortical stimulation in addition to patient outcomes was analyzed. Results Between March 2009 and September 2014, 42 brain AVM resections were performed. Four patients with left-sided language zone AVMs underwent awake craniotomy. The AVM locations were fronto-opercular in 2 patients and posterior temporal in 2. The AVM Spetzler-Martin grades were II (2 patients) and III (2 patients). In 1 patient, complete speech arrest was noticed during mapping of the peri-malformation zone, which was not breached during resection. In a second patient who initially demonstrated negative cortical mapping, a speech deficit was noticed during resection and subcortical stimulation. This guided the approach to protect and avoid the sensitive zone. This patient experienced mild postoperative expressive dysphasia that improved to normal within 6 weeks. Complete resection was achieved in all 4 patients. There were no other complications and no permanent neurological morbidity, resulting in good outcome in all 4 patients. Conclusions Language mapping, both cortical and subcortical during AVM resection, may be valuable in a very select group of AVMs in language zones. Defining safe margins and feedback to the surgeon may provide the highest chances of a surgical cure while minimizing the risk of incurring a language deficit. [ABSTRACT FROM AUTHOR]