Search

OBJECTIVE Patients with unruptured brain arteriovenous malformations (AVMs) may present with headaches, seizures, and/or neurological deficits. A smaller number of cases may be discovered incidentally. These lesions remain incompletely understood due to their sparse reporting. Herein, the authors describe the largest series to date comparing the presentation, angioarchitecture, and management of incidental versus symptomatic unruptured AVMs in children. METHODS The authors performed a retrospective analysis of patients who presented with brain AVMs from 1998 to 2022 at the University of California, San Francisco. Inclusion criteria were age ≤ 18 years at the time of presentation and an angiographically proven unruptured AVM that had been diagnosed postnatally. RESULTS Of 76 children with unruptured AVMs, 66 (86.8%) presented with headaches, seizures, and/or neurological deficit. Ten AVMs (13.1%) were incidentally discovered through unrelated disease workup (50%), cranial trauma (40%), or research study participation (10%). Compared with patients with symptomatic unruptured AVMs, patients with incidental unruptured AVMs had a smaller mean ± SD maximum nidus diameter (2.82 ± 1.1 vs 3.98 ± 1.52 cm, p = 0.025) and fewer had deep venous drainage (20% of patients vs 61%, p = 0.036). They also presented at an earlier age (10 ± 5.2 vs 13.5 ± 4 years, p = 0.043) and with longer duration to first treatment (541 ± 922 vs 196 ± 448 days, p = 0.005). During the observation period, 1 patient developed recurring headaches and demonstrated AVM nidus growth. Four AVMs greater than 3 cm in size or in a deep location were treated with radiosurgery. Six other AVMs were treated with resection, with 2 receiving preoperative embolization. Eight AVMs (80%) were obliterated on last follow-up. Postprocedural complications included 2 transient neurological deficits after resection and 1 case of delayed seizure development after radiosurgery. The mean follow-up period was 5.7 ± 5.7 years without any hemorrhage episodes. CONCLUSIONS A substantial proportion of pediatric patients with unruptured AVMs are discovered incidentally. With earlier presentation and more elementary angioarchitecture than symptomatic unruptured AVMs, these incidental lesions provide a snapshot into the natural history of AVM before symptom development or rupture.

Dural arteriovenous fistulas (dAVFs) located at craniocervical junction are extremely rare (1%-2% of intracranial/spinal dAVFs). Their angio-architectural complexity renders endovascular embolization to be challenging given multiple small feeders with risk of embolysate reflux into vertebral artery and limited transvenous access. The available literature discussing microsurgery for these lesions is limited to few case reports.To report a multicenter experience assessing microsurgery safety/efficacy.Prospectively maintained registries at 13 North American centers were queried to identify craniocervical junction dAVFs treated with microsurgery (2006-2021).Thirty-eight patients (median age 59.5 years, 44.7% female patients) were included. The most common presentation was subarachnoid/intracranial hemorrhage (47.4%) and myelopathy (36.8%) (92.1% of lesions Cognard type III-V). Direct meningeal branches from V3/4 vertebral artery segments supplied 84.2% of lesions. All lesions failed (n = 5, 13.2%) or were deemed inaccessible/unsafe to endovascular treatment. Far lateral craniotomy was the most used approach (94.7%). Intraoperative angiogram was performed in 39.5% of the cases, with angiographic cure in 94.7% of cases (median imaging follow-up of 9.2 months) and retreatment rate of 5.3%. Favorable last follow-up modified Rankin Scale of 0 to 2 was recorded in 81.6% of the patients with procedural complications of 2.6%.Craniocervical dAVFs represent rare entity of lesions presenting most commonly with hemorrhage or myelopathy because of venous congestion. Microsurgery using a far lateral approach provides robust exposure and visualization for these lesions and allows obliteration of the arterialized draining vein intradurally as close as possible to the fistula point. This approach was associated with a high rate of angiographic cure and favorable clinical outcomes.

Dural arteriovenous fistulas (DAVFs) of the sphenoparietal sinus or sphenoid wing region are uncommon lesions with unique and interesting angioarchitecture. Understanding appropriate anatomy and recognizing patterns provide important treatment implications.To describe a single surgeon's experience with open surgical treatment of sphenoparietal sinus DAVFs, the surgical indications for this uncommon lesion, and the microsurgical techniques related to its treatment and to review the literature on its surgical treatment.Consecutive cases of sphenoparietal sinus DAVF treatment conducted by a single surgeon over 24 years (1997-2020) were retrospectively reviewed. Published reports of similar cases were reviewed.Of 202 surgically treated DAVFs, 10 lesions in 10 patients were sphenoparietal sinus DAVFs. Four patients presented with intracranial hemorrhage, 3 with headache, and 2 with pulsatile tinnitus; 1 patient was incidentally identified as having a DAVF during treatment for a ruptured aneurysm. Most patients (7 of 10) had undergone endovascular embolization previously. Nine patients had Borden type III DAVFs and one had a Borden type II fistula. Surgery in all 10 patients resulted in angiographically confirmed fistula obliteration. Clinical outcomes at the last follow-up, measured by a modified Rankin Scale (mRS) score, were excellent in 6 patients (mRS ≤ 2) and poor in 1 patient (mRS ≥ 3); late outcomes were not available for 3 patients.Sphenoparietal sinus DAVFs are an uncommon anatomic subtype. Careful attention to angiographic detail leads to identification of the site of venous interruption and results in a high rate of surgical cure with excellent clinical outcomes.

OBJECTIVE 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. METHODS 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. RESULTS 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. CONCLUSIONS 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.

Introduction: The benefits of performing open and endovascular procedures in a hybrid neuroangiography surgical suite include confirmation of treatment results and reduction in number of procedures, leading to improved efficiency of care. Combined procedural suites are infrequently used in pediatric facilities due to technical and logistical limitations. We report the safety, utility, and lessons learned from a single-institution experience using a hybrid suite equipped with biplane rotational digital subtraction angiography and pan-surgical capabilities. Methods: We conducted a retrospective review of consecutive cases performed at our institution that utilized the hybrid neuroangiography surgical suite from February 2020 to August 2021. Demographics, surgical metrics, and imaging results were collected from the electronic medical record. Outcomes, interventions, and nuances for optimizing preoperative/intraoperative setup and postoperative care were presented. Results: Eighteen procedures were performed in 17 patients (mean age 13.4 years, range 6–19). Cases included 14 arteriovenous malformations (AVM; 85.7% ruptured), one dural arteriovenous fistula, one mycotic aneurysm, and one hemangioblastoma. The average operative time was 416 min (range 321–745). There were no intraoperative or postoperative complications. All patients were alive at follow-up (range 0.1–14.7 months). Five patients had anticipated postoperative deficits arising from their hemorrhage, and 12 returned to baseline neurological status. Four illustrative cases demonstrating specific, unique applications of the hybrid angiography suite are presented. Conclusion: The hybrid neuroangiography surgical suite is a safe option for pediatric cerebrovascular pathologies requiring combined surgical and endovascular intervention. Hybrid cases can be completed within the same anesthesia session and reduce the need for return to the operating room for resection or surveillance angiography. High-quality intraoperative angiography enables diagnostic confirmation under a single procedure, mitigating risk of morbidity and accelerating recovery. Effective multidisciplinary planning enables preoperative angiograms to be completed to inform the operative plan immediately prior to definitive resection.

BackgroundFamilial cerebral cavernous alformation (CCM) is an autosomal dominant disease caused by mutations inKRIT1,CCM2, orPDCD10. Cases typically present with multiple lesions, strong family history, and neurological symptoms, including seizures, headaches, or other deficits. Intracranial hemorrhage (ICH) is a severe manifestation of CCM, which can lead to death or long‐term neurological deficits. Few studies have reported ICH rates and risk factors in familial CCM. We report ICH rates and assess whether CCM lesion burden, a disease severity marker, is associated with risk of symptomatic ICH during follow‐up in a well‐characterized cohort of familial CCM cases.Methods and ResultsWe studied 386 patients with familial CCM with follow‐up data enrolled in the Brain Vascular Malformation Consortium CCM Project. We estimated symptomatic ICH rates overall and stratified by history of ICH before enrollment. CCM lesion burden (total lesion count and large lesion size) assessed at baseline enrollment was tested for association with increased risk of subsequent ICH during follow‐up using Cox regression models adjusted for history of ICH before enrollment, age, sex, and family structure and stratified on recruitment site. The symptomatic ICH rate for familial CCM cases was 2.8 per 100 patient‐years (95% CI, 1.9–4.1). Those with ICH before enrollment had a follow‐up ICH rate of 4.5 per 100 patient‐years (95% CI, 2.6–8.1) compared with 2.0 per 100 patient‐years (95% CI, 1.3–3.5) in those without (P=0.042). Total lesion count was associated with increased risk of ICH during follow‐up (hazard ratio [HR], 1.37 per doubling of total lesion count [95% CI, 1.10–1.71],P=0.006).The symptomatic ICH rate for familial CCM cases was 2.8 per 100 patient‐years (95% CI, 1.9–4.1). Those with ICH before enrollment had a follow‐up ICH rate of 4.5 per 100 patient‐years (95% CI, 2.6–8.1) compared with 2.0 per 100 patient‐years (95% CI, 1.3–3.5) in those without (P=0.042). Total lesion count was associated with increased risk of ICH during follow‐up (hazard ratio [HR], 1.37 per doubling of total lesion count [95% CI, 1.10–1.71],P=0.006).ConclusionsPatients with familial CCM with prior history of an ICH event are at higher risk for rehemorrhage during follow‐up. In addition, total CCM lesion burden is significantly associated with increased risk of subsequent symptomatic ICH; hence lesion burden may be an important predictor of patient outcome and aid patient risk stratification.

BACKGROUND The horizontal fissure approach is a workhorse for brainstem lesions in the central and dorsolateral pons and middle cerebellar peduncle (MCP). The cerebellopontine fissure is a V-shaped fissure with a superior and inferior limb between the cerebellum, pons, and MCP. The horizontal or petrosal fissure is at the apex of the cerebellopontine fissure and extends laterally to divide the petrosal surface of the cerebellum into superior and inferior parts. Splitting this fissure exposes the posterolateral aspect of the MCP without excessive retraction or transgression of the cerebellum. METHOD We demonstrate and describe the horizontal fissure operative approach to the middle cerebellar peduncle for resection of a pontine cavernoma with illustrative figures and operative video. CONCLUSION Splitting the horizontal (petrosal) fissure of the cerebellum brings the middle cerebellar peduncle into view behind the root entry zone of the trigeminal nerve, providing an expanded, safe corridor to the central and dorsolateral pons.

Vertebrobasilar junction and basilar trunk aneurysms are extremely difficult to treat, with significant morbidity associated with bypass-trapping procedures. As a result, endovascular techniques including flow diversion and stent-assisted coil embolization have become the mainstay of treatment.1-3 Traditional flow diverters pose risk of occlusion of basilar perforator arteries while traditional stents used as adjuncts for coil-embolization often have high porosity and cannot function as flow diverters. On the other hand, the LVIS stent uniquely provides more porosity than other flow diverters,4,5 allowing patency of perforators while also being amenable to stent-assisted coiling. We present a case of a mixed saccular and fusiform basilar trunk aneurysm that underwent LVIS stent-assisted coil embolization. Using bifemoral access with triaxial systems, one microcatheter was placed in the aneurysm sac and another was used to deploy a stent from the basilar apex into the right vertebral artery. A durable angiographic outcome was observed at 9 months.

SCA aneurysms are rare, making data on their management sparse compared to other posterior circulation aneurysms. While saccular aneurysms at the SCA origin can be treated with primary coiling or surgical clipping, fusiform and distal SCA aneurysms often require endovascular techniques such as stent-assisted coil embolization or parent vessel occlusion. We present a case of a mixed saccular/fusiform SCA aneurysm that underwent staged stent-assisted coil embolization. A right SCA stent was placed in the first stage. Given the acute angle between the BA and the SCA, microcatheter access and stent placement proved challenging. The decision was made to allow the stent to endothelialize and return in a staged fashion to perform a trans-radial, transcirculatory (trans-left PCoA, trans-left PCA) coil embolization. This provided a straight course between the left P1 segment and the right SCA origin, allowing easy microcatheter navigation through the stent and into the aneurysm for coil embolization.

Objective The extradural anterior petrosectomy is a widely used skull base approach to the upper petroclival region, basilar trunk, and ventral pons. However, there is significant procedure-related morbidity and a complete petrosectomy is required, as the intradural structures are not in view at the time of drilling. We describe an intradural anterior petrosectomy for aneurysms of the basilar trunk and anterior inferior cerebellar artery with intraoperative photographs and artwork to illustrate the approach. Methods A temporal craniotomy is made at the root of the zygoma and middle fossa floor. After opening dura, the trochlear nerve is identified at the tentorial edge. The tentorium is incised posterior to the trochlear nerve and the incision is carried forward across the cisternal segment of the trigeminal nerve toward V3 and the superior petrosal sinus. The petrous apex is identified and drilled lateral and posterior to V3 with a diamond bur. Intraoperative navigation is useful to confirm its location. Once posterior fossa dura is identified, the superior petrosal sinus may be safely cauterized and divided, connecting the tentorial incision with an incision in the posterior fossa dura, and exposing the upper basilar trunk and its branches. Additional bone is removed only as needed. Results Two patients underwent successful treatment of a basilar trunk perforator and anterior inferior cerebellar artery aneurysm with a subtemporal approach and tailored intradural petrosectomy. Conclusions The intradural anterior petrosectomy allows limited drilling of the petrous apex and provides sufficient exposure of upper basilar artery aneurysms.

Activating variants in the platelet-derived growth factor receptor β gene (PDGFRB) have been associated with Kosaki overgrowth syndrome, infantile myofibromatosis, and Penttinen premature aging syndrome. A recently described phenotype with fusiform aneurysm has been associated with mosaic PDGFRB c.1685A > G p.(Tyr562Cys) variant. Few reports however have examined the vascular phenotypes and mosaic effects of PDGFRB variants. We describe clinical characteristics of two patients with a recurrent mosaic PDGFRB p.(Tyr562Cys) variant identified via next-generation sequencing-based genetic testing. We observed intracranial fusiform aneurysm in one patient and found an additional eight patients with aneurysms and phenotypes associated with PDGFRB-activating variants through literature search. The conditions caused by PDGFRB-activating variants share overlapping features including overgrowth, premature aged skin, and vascular malformations including aneurysms. Aneurysms are progressive and can result in morbidities and mortalities in the absence of successful intervention. Germline and/or somatic testing for PDGFRB gene should be obtained when PDGFRB activating variant-related phenotypes are present. Whole-body imaging of the arterial tree and echocardiography are recommended after diagnosis. Repeating the imaging study within a 6- to 12-month period after detection is reasonable. Finally, further evaluation for the effectiveness and safety profile of kinase inhibitors in this patient population is warranted.

Background Currently, most basilar artery aneurysms (BAAs) are treated endovascularly. Surgery remains an appropriate therapy for a subset of all intracranial aneurysms. Whether open microsurgery would be required or utilized, and to what extent, for BAAs treated by a surgeon who performs both endovascular and open procedures has not been reported. Methods Retrospective analysis of prospectively maintained, single-surgeon series of BAAs treated with endovascular or open surgery from the first 5 years of practice. Results Forty-two procedures were performed in 34 patients to treat BAAs—including aneurysms arising from basilar artery apex, trunk, and perforators. Unruptured BAAs accounted for 35/42 cases (83.3%), and the mean aneurysm diameter was 8.4 ± 5.4 mm. Endovascular coiling—including stent-assisted coiling—accounted for 26/42 (61.9%) treatments and led to complete obliteration in 76.9% of cases. Four patients in the endovascular cohort required re-treatment. Surgical clip reconstruction accounted for 16/42 (38.1%) treatments and led to complete obliteration in 88.5% of cases. Good neurologic outcome (mRS ≤ 2) was achieved in 88.5% and 75.0% of patients in endovascular and open surgical cohorts, respectively (p = 0.40). Univariate logistic regression analysis demonstrated that advanced age (OR 1.11[95% CI 1.01–1.23]) or peri-procedural adverse event (OR 85.0 [95% CI 6.5–118.9]), but not treatment modality (OR 0.39[95% CI 0.08–2.04]), was the predictor of poor neurologic outcome. Conclusions Complementary implementation of both endovascular and open surgery facilitates individualized treatment planning of BAAs. By leveraging strengths of both techniques, equivalent clinical outcomes and technical proficiency may be achieved with both modalities.

The use of intracranial stents in neurointerventional surgery has been practiced for decades. However, the spectrum of treatable pathology, available devices, and clinical adoption of intracranial stents has exploded in recent years. Diagnostic neuroradiologists play a critical role in the evaluation of these devices after deployment, yet may not be familiar with state-of-the-art intracranial stent devices and indications. This review provides an overview of intracranial stents for cerebrovascular disease, with 3 chief learning objectives: 1) to understand the basic principles of stent design, biomechanics, and deployment, and the resulting influence on cerebrovascular hemodynamics; 2) to be familiar with the spectrum of intracranial pathology amenable to endovascular stent placement; and 3) to recognize the radiographic appearance of successful intracranial stent deployment and intracranial stent‐related complications.Learning Objective: Recognize the key principles of design (form), current indications (function), and potential complications of intracranial stents used in neurointerventional surgery

BackgroundTools predicting intracranial dural arteriovenous fistulas (dAVFs) treatment outcomes remain scarce. This study aimed to use a multicenter database comprising more than 1000 dAVFs to develop a practical scoring system that predicts treatment outcomes.MethodsPatients with angiographically confirmed dAVFs who underwent treatment within the Consortium for Dural Arteriovenous Fistula Outcomes Research-participating institutions were retrospectively reviewed. A subset comprising 80% of patients was randomly selected as training dataset, and the remaining 20% was used for validation. Univariable predictors of complete dAVF obliteration were entered into a stepwise multivariable regression model. The components of the proposed score (VEBAS) were weighted based on their ORs. Model performance was assessed using receiver operating curves (ROC) and areas under the ROC.ResultsA total of 880 dAVF patients were included. Venous stenosis (presence vs absence), elderly age (ConclusionThe VEBAS score is a practical grading system that can guide patient counseling when considering dAVF intervention by predicting the likelihood of treatment success, with higher scores portending a greater likelihood of complete obliteration.

BackgroundPediatric spinal arteriovenous shunts (SAVS) are rare lesions with heterogeneous pathogenesis and clinical manifestations.ObjectiveTo evaluate the clinical characteristics, angioarchitecture, and technical/clinical outcomes in SAVS through a large single-center cohort analysis and meta-analysis of individual patient data.MethodsA retrospective institutional database identified children (aged 0–21 years) who underwent digital subtraction spinal angiography (DSA) for SAVS between January 1996 and July 2021. Clinical data were recorded to evaluate angioarchitecture, generate modified Aminoff-Logue gait disturbance scores (AL) and McCormick grades (MC), and assess outcomes. We then performed a systematic literature review following PRISMA-IPD (Preferred Reporting Items for Systematic Reviews and Meta-Analyses for individual patient data) guidelines, extracting similar data on individual patients for meta-analysis.ResultsThe cohort consisted of 28 children (M:F=11:17) with 32 SAVS lesions, with a mean age of 12.8±1.1 years at diagnosis. At presentation, SAVS were most highly concentrated in the cervical region (40.6%). Children had a median AL=2 and MC=2, with thoracolumbar AVS carrying the greatest disability. Among treated cases, complete obliteration was achieved in 48% of cases and median AL scores and MC grades both improved by one point. Systematic literature review identified 161 children (M:F=96:65) with 166 SAVS lesions with a mean age of 8.7±0.4 years. Among studies describing symptom chronicity, 37/51 (72.5%) of children presented acutely. At presentation, children had a median AL=4 and MC=3, with thoracolumbar AVS carrying the highest MC grades. After intervention, median AL and MC both improved by one point.ConclusionsThis study provides epidemiologic information on the location, onset, and presentation of the full spectrum of pediatric SAVS, highlighting the role of targeted treatment of high-risk features.

Background Brainstem cavernous malformations with symptomatic hemorrhage have a poor natural history. Those without a pial or ependymal presentation are often observed given the morbidity of resection. Surgical removal is considered only in patients with accessible lesions that have repeated symptomatic hemorrhagic. Objective To describe a novel supracerebellar infratentorial infratrochlear trans-quadrangular lobule approach to safely resect lesions in the upper pons. Methods We use a hybrid paramedian/lateral suboccipital craniotomy in the gravity-dependent supine position. Opening the cerebellomesencephalic fissure over the tentorial surface of the cerebellum brings the trochlear nerve, branches of the superior cerebellar artery, and the quadrangular lobule of the cerebellum into view. Removal of small a portion of the quadrangular lobule defines an entry point on the superomedial aspect of the middle cerebellar peduncle, and a surgical trajectory aimed superior to inferior. Results A total of 6 patients underwent this approach. All presented with symptomatic hemorrhage and all cavernous malformations were completely resected. Five patients were improved or unchanged with modified Rankin scale scores of 1 or 2. Conclusion The trans-quadrangular lobule approach allows safe resection of upper pontine cavernous malformations along a superior to inferior trajectory.

Introduction: Tumor-associated intracranial aneurysms are rare and not well understood. Case Presentation: We describe a 4-year-old female with multiple intracranial aneurysms intimately associated with a suprasellar germ cell tumor (GCT). We provide the clinical history, medical, and surgical treatment course, as well as a comprehensive and concise synthesis of the literature on tumor-associated aneurysms. Discussion: We discuss mechanisms for aneurysm formation with relevance to the current case, including cellular and paracrine signaling pertinent to suprasellar GCTs and possible molecular pathways involved. We review the complex multidisciplinary treatment required for complex tumor and cerebrovascular interactions.

Introduction: Pial arteriovenous fistulas are characterized by an abnormal connection between an intracranial artery and vein without an intervening nidus. Their predominant symptoms largely arise from mass effect, shunting, or hemorrhage. Most conservatively managed cases progress to death, but endovascular and/or surgical intervention is often successful. Case Presentation: We present the unique case of a 15-year-old girl with spontaneous intracranial hemorrhage from a single-vessel arteriovenous fistula. Although preoperative imaging failed to show a distinct nidus, intraoperative indocyanine green angiography performed after successful clipping of the primary fistulous site revealed residual shunting from a peri-lesional arteriovenous malformation. Discussion/Conclusion: This case demonstrates the importance of intraoperative imaging and meticulous circumferential inspection of these lesions to detect residual vascular shunting.

The lateral supraorbital (LSO) approach is a minimally invasive modification of the standard pterional approach to anterior circulation aneurysms. This study aimed to describe a dual-trained cerebrovascular neurosurgeon's first 18-month experience with the LSO approach, including decision-making criteria and lessons learned.This retrospective case series analyzed 50 consecutive patients treated with LSO craniotomy for aneurysm clipping by a single surgeon. Aneurysms were separated into 3 categories by location: internal carotid artery, anterior communicating artery, and middle cerebral artery. Surgical characteristics were evaluated for differences by location and rupture status.Aneurysm clipping via LSO was performed on 57 aneurysms in 50 patients. Fixed retraction was employed less often in patients with internal carotid artery aneurysms than in patients with anterior communicating artery, middle cerebral artery, or multiple aneurysms (10% vs. 68.2%, 45.5%, and 42.9, P = 0.02). Of patients, 26 (52%) presented with subarachnoid hemorrhage; the majority of patients (92.3%) had Hunt and Hess grade I-III. No differences were noted in intraoperative rupture rates, fixed retractor use, operative duration, or estimated blood loss by rupture status. Adverse events included permanent frontalis nerve palsy in 1 patient (2%), temporalis atrophy in 1 patient, and transient aphasia in 1 patient. No postoperative hematomas or strokes were observed.The LSO approach can safely and effectively treat anterior circulation aneurysms and should be considered a viable minimally invasive option for aneurysm clipping. Further studies comparing the LSO approach with other cranial approaches are needed.

Intraoperative angiography (IOA) has been shown to be a useful adjunct in surgical treatment of cerebral aneurysms. However, its use can be limited by hybrid operating room availability. On the other hand, the use of C-arm fluoroscopy can add challenges to IOA during navigation of the aortic arch and selection of the great vessels. We aimed to describe a simple method of IOA that can be applied during surgery of paraclinoid aneurysms and can be performed in a normal operating room without the need to navigate the aortic arch.In patients undergoing surgery for paraclinoid aneurysms with need for cervical carotid artery exposure, IOA was performed using a single plane C-arm fluoroscopy unit after direct puncture of the carotid artery.Five patients were included: 2 with subarachnoid hemorrhage, 2 with unruptured aneurysm and history of subarachnoid hemorrhage, and 1 with unruptured aneurysm. There were 2 internal carotid blister aneurysms, 2 ophthalmic artery aneurysms, and 1 superior hypophyseal artery aneurysm. IOA was performed using direct carotid puncture through the neck incision required for proximal control. In all cases, intraoperative images were of sufficient quality to determine the completeness of aneurysm occlusion as well as parent and branching vessel patency. There were no postoperative infarctions and no complications related to IOA.IOA using direct carotid puncture can be performed in a standard operating room with the use of a C-arm, eliminating the need to catheterize the great vessels of the aortic arch.

Cerebrovascular diseases are a leading cause of death and neurologic disability. Further understanding of disease mechanisms and therapeutic strategies requires a deeper knowledge of cerebrovascular cells in humans. Here, we profiled transcriptomes of 181,388 single-cells from the adult cerebrovasculature. Our results define a cell atlas of the human cerebrovasculature, including endothelial cell molecular signatures with arteriovenous segmentation and expanded perivascular cell diversity. By leveraging this reference, we investigated cellular and molecular perturbations in brain arteriovenous malformations, a leading cause of stroke in young people, and identified pathologic endothelial transformations with abnormal vascular patterning and the ontology of vascular-derived inflammation. Herein, we illustrated an interplay between vascular and immune cells contributory to brain hemorrhage and cataloged opportunities for targeting angiogenic and inflammatory programs in vascular malformations.

Background and purposeAutomated CTP software is increasingly used for extended window emergent large-vessel occlusion to quantify core infarct. We aimed to assess whether RAPID software underestimates core infarct in patients with an extended window recently receiving IV iodinated contrast.Materials and methodsWe reviewed a prospective, single-center data base of 271 consecutive patients who underwent CTA ± CTP for acute ischemic stroke from May 2018 through January 2019. Patients with emergent large-vessel occlusion confirmed by CTA in the extended window (>6 hours since last known well) and CTP with RAPID postprocessing were included. Two blinded raters independently assessed CT ASPECTS on NCCT performed at the time of CTP. RAPID software used relative cerebral blood flow of

BackgroundThe transcirculation approach (TCA) for stent-assisted coiling (SAC) of intracranial aneurysms may be useful for certain wide-neck bifurcation aneurysms as well as those with acute-angle efferent branches.ObjectiveTo describe a multicenter experience using the TCA for SAC.MethodsA multicenter, retrospective study (2016–2020) of aneurysm treatment using SAC via the TCA. Angiographic outcome was scored using the Raymond Scale (adequate occlusion 1 and 2), and clinical outcome was scored using a modified Rankin Scale (good outcome 0–2)ResultsTwenty-nine patients with 29 aneurysms were included (62.1% female; average age 61; 89.7% unruptured; 13.8% previously treated; average dome size 6.4 mm; average neck 4.4 mm). Aneurysm locations included internal carotid artery–fetal posterior cerebral artery (n=4), internal carotid artery terminus (n=4), anterior communicating artery (n=8), vertebral artery–posterior inferior cerebellar artery (n=2), and basilar tip (n=11). The TCA used communicating arteries (93.1%; average 1.6 mm), intermediate catheters (51.7%), jailing technique (62.1%), and staged procedures (10.3%). The most common stent was the Neuroform Atlas (Stryker; 69%). Immediate adequate occlusion was obtained in 75.9%, and five patients with inadequate occlusion progressed to adequate occlusion at follow-up. One (3.4%) procedural complication occurred: a watershed stroke in the setting of baseline four-vessel extracranial disease. Two patients had a poor outcome unrelated to the TCA. The majority of patients (86.4%) had a good clinical outcome. One case of in-stent stenosis due to non-compliance with medication was seen, which resolved with medication resumption.ConclusionsThe TCA for SAC can be performed for a variety of aneurysms with a low complication rate and good clinical outcomes.

Cerebral revascularization utilizing a variety of bypass techniques can provide either flow augmentation or flow replacement in the treatment of a range of intracranial pathologies, including moyamoya disease, intracranial atherosclerotic disease, and complex aneurysms that are not amenable to endovascular or simple surgical techniques. Though once routine, the publication of high-quality prospective evidence, along with the development of flow-diverting stents, has limited the indications for extracranial-to-intracranial (EC-IC) bypass. Nevertheless, advances in imaging, assessment of cerebral hemodynamics, and surgical technique have changed the risk-benefit calculus for EC-IC bypass. New variations of revascularization surgery involving multiple anastomoses, flow preserving solutions, IC-IC constructs, and posterior circulation bypasses have been pioneered for otherwise difficult to treat pathology including giant aneurysms, dolichoectasia, and medically refractory intracranial atherosclerosis. This review provides a practical update on recent advances in adult intracranial bypass surgery.

Moyamoya is a progressive cerebrovascular arteriopathy that affects children of any age. The goal of this study was to determine imaging and clinical outcomes as well as complication rates in a pediatric cohort undergoing either a combined direct/indirect or indirect-only revascularization approach. Patients with moyamoya disease or syndrome≤18years of age at the time of initial surgery were identified, and clinical data were collected retrospectively. Over a 12-year period, 26 patients underwent revascularization procedures on 49 hemispheres with a median follow-up of 2.6years from surgery. Median age at surgery was 7.3years (range 1.4-18.0years). Thirty-three hemispheres (67.3%) underwent combined revascularization with a direct bypass and encephalomyosynangiosis, and sixteen hemispheres (32.7%) underwent indirect-only revascularization. The rate of 30-day perioperative complication was 10.2%, and the rate of postoperative clinical stroke by end of follow-up was 10.2% by hemisphere. There was a 5.7% rate of intraoperative bypass failure requiring conversion to an indirect revascularization approach. On follow-up imaging, 96.9% of direct bypasses remained patent. On multivariate analysis, higher preoperative Pediatric Stroke Outcome Measure (PSOM) scores were associated with lower rates of good clinical outcome on follow-up (unit OR 0.03; p=0.03). Patients with age&nbsp

Background and purpose Extracranial-to-intracranial (EC-IC) surgical bypass improves cerebral blood flow (CBF) and cerebrovascular vasoreactivity (CVR) for patients with carotid occlusion. Bypass graft patency and contribution of the graft to the postoperative increase in CVR are challenging to assess. To assess the effectiveness of 4D flow magnetic resonance imaging (MRI) to evaluate bypass graft patency and flow augmentation through the superficial temporal artery (STA) before and after EC-IC bypass. Methods Three consecutive patients undergoing EC-IC bypass for carotid occlusion were evaluated pre- and postoperatively using CVR testing with pre- and poststimulus 4D flow-MRI for assessment of the bypass graft and intracranial vasculature. Results Preoperatively, 2 patients (patients 1 and 3) did not augment flow through either native STA. The third, who had evidence of extensive native EC-IC collateralization on digital subtraction angiography (DSA), did augment flow through the STA preoperatively (CVR = 1). Postoperatively, all patients demonstrated CVR > 1 on the side of bypass. The patient who had CVR > 1 preoperatively demonstrated the greatest increase in resting postoperative graft flow (from 40 to 130 mL/minute), but the smallest CVR, with a poststimulus graft flow of 160 mL/minute (CVR = 1.2). The 2 patients who did not demonstrate augmentation of graft flow preoperatively augmented postoperatively from 10 to 20 mL/minute (CVR = 2.0) and 10-80 mL/minute (CVR = 8.0), respectively. Intracranial flow was simultaneously interrogated. Two patients demonstrated mild reductions in resting flow velocities in all interrogated vessels immediately following bypass. The patient who underwent CVR testing on postoperative day 48 demonstrated a stable or increased flow rate in most intracranial vessels. Conclusion Four-dimensional flow MRI allows for noninvasive, simultaneous interrogation of the intra- and extracranial arterial vasculature during CVR testing, and reveals unique paradigms in cerebrovascular physiology. Observing these flow patterns may aid in improved patient selection and more detailed postoperative evaluation for patients undergoing EC-IC bypass.

Background Management of small unruptured aneurysms is controversial. Small aneurysms and those with low PHASES scores are often observed. The primary aim of this study was to assess whether the PHASES score classified the patients with subarachnoid hemorrhage as high risk for rupture. Methods We retrospectively reviewed a consecutive series of 628 aneurysmal subarachnoid hemorrhage neurosurgical cases over a 10-year period between 2008 and 2018. We collected patient and aneurysm characteristics and calculated PHASES scores. Results The median aneurysm size was 5.3 mm (interquartile range, 3.5–7). Of the aneurysms, 75% (473/628) were less than 7 mm in size. Nearly half of the aneurysms were less than 5 mm (48%, 302/628). The median PHASES score was 5 (interquartile range, 4–6), corresponding to a 5-year risk of rupture of only 1.3%. Most ruptured aneurysms in our series were small with low PHASES scores, suggesting a low risk of rupture. Many of these patients would have been conservatively managed. Conclusions PHASES is inadequate in management of unruptured aneurysms because it fails to identify many patients at risk for subarachnoid hemorrhage. A more nuanced assessment of rupture risk should be undertaken.

The superficial temporal artery-middle cerebral artery (STA-MCA) bypass augments blood flow in patients with cerebral ischemia or replaces flow in patients with complex aneurysms or skull base tumors requiring vessel sacrifice. We provide a description of the STA-MCA bypass with figures and video to illustrate the procedure. The STA-MCA end-to-side anastomosis is a foundational skill for the cerebrovascular surgeon and a building block for more complex bypasses.

BACKGROUND AND PURPOSE: The Neuroform Atlas is a new microstent to assist coil embolization of intracranial aneurysms that recently gained FDA approval. We present a postmarket multicenter analysis of the Neuroform Atlas stent. MATERIALS AND METHODS: On the basis of retrospective chart review from 11 academic centers, we analyzed patients treated with the Neuroform Atlas after FDA exemption from January 2018 to June 2019. Clinical and radiologic parameters included patient demographics, aneurysm characteristics, stent parameters, complications, and outcomes at discharge and last follow-up. RESULTS: Overall, 128 aneurysms in 128 patients (median age, 62 years) were treated with 138 stents. Risk factors included smoking (59.4%), multiple aneurysms (27.3%), and family history of aneurysms (16.4%). Most patients were treated electively (93.7%), and 8 (6.3%) underwent treatment within 2 weeks of subarachnoid hemorrhage. Previous aneurysm treatment failure was present in 21% of cases. Wide-neck aneurysms (80.5%), small aneurysm size (

Despite a variety of treatment options for brain arteriovenous malformations (bAVMs), many lesions remain challenging to treat and present significant ongoing risk for hemorrhage. In Vitro investigations have recently led to a greater understanding of the formation, growth, and rupture of bAVMs. This has, in turn, led to the development of therapeutic targets for medications for bAVMs, some of which have begun testing in clinical trials in humans. These include bevacizumab, targeting the vascular endothelial growth factor driven angiogenic pathway; thalidomide or lenalidomide, targeting blood-brain barrier impairment; and doxycycline, targeting matrix metalloproteinase overexpression. A variety of other medications appear promising but either requires adaptation from other disease states or development from early bench studies into the clinical realm. This review aims to provide an overview of the current state of development of medications targeting bAVMs and to highlight their likely applications in the future.

Background The extent of obliteration of ruptured intracranial aneurysms treated with coil embolization has been correlated with the risk of rerupture. However, many practitioners consider that a small neck remnant is unlikely to result in significant risk after coiling. Objective To report our recent experience with ruptured anterior cerebral artery aneurysms treated with endovascular coiling, which recurred or reruptured, requiring microsurgical clipping for subsequent treatment. Methods Retrospective review of patients with intracranial aneurysms treated at our institution since August 2018. Patient and aneurysm characteristics, initial and subsequent treatment approaches, and outcomes were reviewed. Results Six patients were included. Out of those 6 patients, 5 patients had anterior communicating artery aneurysms, and 1 patient had a pericallosal aneurysm. All initially presented with subarachnoid hemorrhage (SAH) and were treated with coiling. Recurrence occurred at a median of 7.5 mo. In 2 cases, retreatment was initially performed with repeat endovascular coiling, but further recurrence was observed. Rerupture from the residual or recurrent aneurysm occurred in 3 cases. In 2 cases, the aneurysm dome recurred; in 1 case, rerupture occurred from the neck. All 6 patients underwent treatment with microsurgical clipping. Follow-up catheter angiography demonstrated a complete occlusion of the aneurysm in all cases with the preservation of the parent vessel. Conclusion Anterior cerebral artery aneurysms may recur after endovascular treatment, and even small neck remnants present a risk of rerupture after an initial SAH. Complete treatment requires a complete exclusion of the aneurysm from the circulation. Even in cases that have been previously coiled, microsurgical clipping can represent a safe and effective treatment option.

Background Subarachnoid hemorrhage resulting from spontaneous perforation of a small intracranial vessel, with resultant pseudoaneurysm formation, has not been widely reported in the literature. Case Description We present the case of a 71-year-old patient with rupture of a small aneurysm of a duplicated left anterior choroidal artery causing an acute third nerve palsy. The aneurysm was not able to be treated endovascularly without sacrifice of the parent vessel. At surgery, a pseudoaneurysm was seen completely separate from the parent vessel, which was actively bleeding through a hole in the vessel. The pseudoaneurysm was indenting the oculomotor nerve. After confirmation of adequate collateral flow, the abnormal segment of vessel was trapped and the pseudoaneurysm removed with surrounding clot. The patient's cranial nerve palsy resolved. Conclusions This case illustrates an unusual sequela of subarachnoid hemorrhage presenting a unique challenge in surgical management.

The far-lateral (FL) approach is a classic technique for skull base surgeries involving the lower clivus (LC). Recently, a modified suboccipital midline approach known as the subtonsillar (ST) approach, along with the endoscope-assisted subtonsillar (EST) approach, has been described as a minimally invasive technique to treat LC lesions. However, there is no quantitative study on comparing these approaches together for reaching LC. We aimed to compare surgical exposure and freedom provided by ST, EST, and FL approaches for various targets at LC. These approaches were performed on each side of five cadaveric specimens (total 10 sides), and relevant parameters were quantified and compared using a repeated measures ANOVA test. FL approach yielded the greatest surgical area (237.8 ± 56.0 mm2) and exposure, including lengths of glossopharyngeal nerve (16.2 ± 1.9 mm), hypoglossal nerve (11.4 ± 2.4 mm), vertebral artery (23.9 ± 3.3 mm), followed by EST and ST approaches. For surgical freedom, FL approach provided the greatest angle of attack (90.0 ± 14.0° at jugular foramen, 95.1 ± 15.8° at hypoglossal canal, 83.4 ± 31.4° at bifurcation point of posterior inferior cerebellar artery and vertebral artery). Our systematic comparison suggests that EST approach, compared to ST approach, can significantly increase surgical exposure to the medial side of LC, but FL approach still provides the greatest surgical exposure and freedom at LC. Despite the limitations of a cadaveric study, our quantitative data can update the literature on currently available surgical techniques for reaching LC and better inform preoperative planning in this area. Further studies should be performed to evaluate these approaches in clinical practice.

Background Despite a variety of technologies that are available for treatment of complex intracranial aneurysms, certain anatomic configurations remain challenging to address endovascularly. Case Description A patient was found to have an incidental 12 mm × 11 mm × 10 mm, wide-necked right posterior communicating artery aneurysm with a fetal origin of the posterior cerebral artery arising directly from the aneurysm dome. After multidisciplinary discussion, a staged endovascular treatment approach was undertaken in 2 stages. First, a Y-stent construct using 2 overlapping Neuroform Atlas stents was placed into the M1 and fetal posterior cerebral artery segments. Two months later, after endothelialization of the stent construct, coil embolization of the aneurysm was performed. The patient tolerated both stages of the procedure well and was discharged the following day in each case. She remained neurologically intact, and at follow-up 5 months later had no evidence of residual or recurrent aneurysm. Conclusions This case illustrates a number of important considerations in the management approach for wide-necked intracranial aneurysms.

Introduction Distal ophthalmic artery (OpA) aneurysms are a rare subset of vascular lesions with lack of optimal treatment. The management of these aneurysms may require complete occlusion of the parent vessel, carrying a risk of permanent visual impairment due to individual variations of extracranial collateral flow to the intraorbital ophthalmic artery (iOpA). Objective To test the feasibility of a superficial temporal artery (STA) to iOpA bypass to prevent acute ischemic retinal injury. Two different transorbital corridors (superomedial and posterolateral approaches) for this bypass were evaluated. Methods Each approach was carried out in 10 specimens each (n = 20). The corridors were compared to achieve the optimal exposure of the iOpA until the central retinal artery origin was visualized. An end-to-end anastomosis was performed from STA-to-iOpA. The arterial caliber and length at the anastomotic sites, required donor artery length, and intraorbital surgical area were measured. Results STA-iOpA bypasses were performed in all specimens. For the posterolateral transorbital approach, the mean caliber of STA was 1.8 ± 0.2 mm, and that of iOpA was 1.7 ± 0.5 mm. The required STA graft length was 78.3 ± 1 mm with lateral iOpA transposition of 8.2 ± 1.1 mm. For the superomedial approach, the average STA length required for an intraorbital bypass was 130.8 ± 14.0 mm. The mean calibers of iOpA and STA were 1.5 ± 0.1 mm and 1.5 ± 0.1 mm, respectively. Conclusions This study demonstrates the feasibility of a novel revascularization technique of the iOpA using 2 different transorbital approaches. These techniques can be used in the management of intraorbital lesions such as OpA aneurysms, tumoral infiltrations, or intraoperative injuries.

The pharyngo-tympano-stapedial middle meningeal artery (PTS-MMA) variant has been described in one case report and never in the setting of arterial supply to a dural arteriovenous fistula, to our knowledge. We report the case of a middle-aged patient with severe, daily headache who presented to our institution for angiography and treatment. CT angiography and MRI demonstrated an enlarged left middle meningeal artery coursing to a large venous varix in the falcotentorial region. Dural arteriovenous fistula was confirmed by subsequent cerebral angiography. Endovascular treatment was performed but without complete obliteration of the fistula. Follow-up angiography demonstrated parasitized arterial supply from a right middle meningeal artery arising from the proximal cervical internal carotid artery coursing through the middle ear consistent with a PTS-MMA variant. The fistula was then treated surgically without recurrence at the 6-month follow-up.

OBJECTIVE Sporadic brain arteriovenous malformation (BAVM) is a tangled vascular lesion characterized by direct artery-to-vein connections that can cause life-threatening intracerebral hemorrhage (ICH). Recently, somatic mutations in KRAS have been reported in sporadic BAVM, and mutations in other mitogen-activated protein kinase (MAPK) signaling pathway genes have been identified in other vascular malformations. The objectives of this study were to systematically evaluate somatic mutations in MAPK pathway genes in patients with sporadic BAVM lesions and to evaluate the association of somatic mutations with phenotypes of sporadic BAVM severity. METHODS The authors performed whole-exome sequencing on paired lesion and blood DNA samples from 14 patients with sporadic BAVM, and 295 genes in the MAPK signaling pathway were evaluated to identify genes with somatic mutations in multiple patients with BAVM. Digital droplet polymerase chain reaction was used to validate KRAS G12V and G12D mutations and to assay an additional 56 BAVM samples. RESULTS The authors identified a total of 24 candidate BAVM-associated somatic variants in 11 MAPK pathway genes. The previously identified KRAS G12V and G12D mutations were the only recurrent mutations. Overall, somatic KRAS G12V was present in 14.5% of BAVM lesions and G12D was present in 31.9%. The authors did not detect a significant association between the presence or allelic burden of KRAS mutation and three BAVM phenotypes: lesion size (maximum diameter), age at diagnosis, and age at ICH. CONCLUSIONS The authors confirmed the high prevalence of somatic KRAS mutations in sporadic BAVM lesions and identified several candidate somatic variants in other MAPK pathway genes. These somatic variants may contribute to understanding of the etiology of sporadic BAVM and the clinical characteristics of patients with this condition.

Brain arteriovenous malformations (bAVM) are an important cause of intracranial hemorrhage (ICH), especially in younger patients. The pathogenesis of bAVM are largely unknown. Current understanding of bAVM etiology is based on studying genetic syndromes, animal models, and surgically resected specimens from patients. The identification of activating somatic mutations in the Kirsten rat sarcoma viral oncogene homologue (KRAS) gene and other mitogen-activated protein kinase ( MAPK) pathway genes has opened up new avenues for bAVM study, leading to a paradigm shift to search for somatic, de novo mutations in sporadic bAVMs instead of focusing on inherited genetic mutations. Through the development of new models and understanding of pathways involved in maintaining normal vascular structure and functions, promising therapeutic targets have been identified and safety and efficacy studies are underway in animal models and in patients. The goal of this paper is to provide a thorough review or current diagnostic and treatment tools, known genes and key pathways involved in bAVM pathogenesis to summarize current treatment options and potential therapeutic targets uncovered by recent discoveries.