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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.

We report transarterial Onyx embolization with flow control using rapid ventricular pacing (RVP) in a middle-aged male patient with tentorial dural arteriovenous fistulas (TDAVFs). The patient completed angiographic obliteration in one session without any complications, and the 6-month postangiographic obliteration follow-up showed no evidence of residual or recurrent dural arteriovenous fistulas. RVP may be a novel treatment option of flow control to facilitate the embolic agent penetrating into the venous side and to achieve complete cure in transarterial embolization of TDAVFs.

Introduction Endovascular therapy has shown to be safe and effective for the treatment of cerebral dural arteriovenous fistulas; however, recurrence after complete occlusion is not uncommon, and the timing of recurrence remains unknown. Methods A retrospective single-center cohort study was conducted from January 2005 to December 2020. Patients with high-grade (≥Borden II–Cognard IIB) dural arteriovenous fistulas treated with endovascular therapy were included in this study. Clinical and angiographic characteristics were collected for hospitalization and at follow-up. Results A total of 51 patients with a median age of 61 years were studied; 57% were female. High-flow symptoms related to the high-flow fistula were the most common presentation (67%), and 24% presented with intracranial hemorrhage. Transverse-sigmoid (26%) and cavernous (26%) sinuses were the most common dural arteriovenous fistula locations. A total of 40 patients (70%) had middle meningeal arterial feeders and 4 (7%) had deep cerebral venous drainage. The mean number of embolization procedures per patient was 1.4. Transarterial access was the most frequent approach (61%). Onyx alone was the most common embolic agent (26%). Complete occlusion rate was achieved in 46 patients (80.1%). Last mean radiographic follow-up time was 26.7 months for all 57 dural arteriovenous fistulas. Dural arteriovenous fistula recurrence after radiographic resolution at last treatment was seen in six cases (6/46, 13.1%). Mean time for recurrence was 15.8 months. Mean time of last clinical follow-up was 46.1 months for the 51 patients (100%). A total of 10 (20%) experienced any procedural complications, among which two (4%) became major thromboembolic events. Conclusion Endovascular therapy is safe and effective for the treatment of high-grade dural arteriovenous fistulas. Given the significant recurrence rate of embolized dural arteriovenous fistulas even after 2 years, long-term angiographic follow-up might be needed.

Ethmoidal dural arteriovenous fistula (DAVF) is a rare type of intracranial DAVF. The aim of this study was to report our experience with a unilateral approach and discuss its effectiveness for ethmoidal DAVF treatment.The study included 19 patients who underwent surgical treatment for ethmoidal DAVF between January 1999 and May 2021.Median age of patients was 59.7 years; 16 (84%) patients were male. Three patients had a ruptured ethmoidal DAVF. Preoperative digital subtraction angiography showed that all ethmoidal DAVFs were supplied by the bilateral external carotid artery branches. In 18 (95%) patients, cortical draining veins were located on the unilateral side. Bilateral lesions were identified in only 1 (5%) patient. The frontobasal approach was performed in 5 patients (26%), the pterional approach was performed in 5 (26%) patients, and the lateral supraorbital approach was performed in 9 (47%) patients; median procedural times were 198 minutes, 172 minutes, and 111 minutes, respectively. Cortical draining vein was successfully disconnected in all 19 patients with 20 ethmoidal DAVFs. Complete obliteration of ethmoidal DAVF was confirmed in all patients, with no postoperative complications. No recurrence or related clinical events were reported in 13 (68%) patients over 12 months of clinical and radiological follow-up.We reconfirmed excellent outcomes of surgical treatment for ethmoidal DAVFs. Three different surgical strategies were attempted, and each had pros and cons. The lateral supraorbital approach is an efficient surgical option for unilateral ethmoidal DAVFs. Careful preoperative examination for the presence of bilateral drainage is essential.

OBJECTIVE There have been no accurate surveillance data regarding the incidence rate of spinal arteriovenous shunts (SAVSs). Here, the authors investigate the epidemiology and clinical characteristics of SAVSs. METHODS The authors conducted multicenter hospital-based surveillance as an inventory survey at 8 core hospitals in Okayama Prefecture between April 1, 2009, and March 31, 2019. Consecutive patients who lived in Okayama and were diagnosed with SAVSs on angiographic studies were enrolled. The clinical characteristics and the incidence rates of each form of SAVS and the differences between SAVSs at different spinal levels were analyzed. RESULTS The authors identified a total of 45 patients with SAVSs, including 2 cases of spinal arteriovenous malformation, 5 cases of perimedullary arteriovenous fistula (AVF), 31 cases of spinal dural AVF (SDAVF), and 7 cases of spinal epidural AVF (SEAVF). The crude incidence rate was 0.234 per 100,000 person-years for all SAVSs including those at the craniocervical junction (CCJ) level. The incidence rate of SDAVF and SEAVF combined increased with advancing age in men only. In a comparative analysis between upper and lower spinal SDAVF/SEAVF, hemorrhage occurred in 7/14 cases (50%) at the CCJ/cervical level and in 0/24 cases (0%) at the thoracolumbar level (p = 0.0003). Venous congestion appeared in 1/14 cases (7%) at the CCJ/cervical level and in 23/24 cases (96%) at the thoracolumbar level (p < 0.0001). CONCLUSIONS The authors reported detailed incidence rates of SAVSs in Japan. There were some differences in clinical characteristics of SAVSs in the upper spinal levels and those in the lower spinal levels.

OBJECTIVE The natural course of dural arteriovenous fistulas (DAVFs) is unfavorable. Transarterial embolization with Onyx is currently the therapeutic method of choice, although the long-term stability of Onyx has been questioned. The literature reports a significant difference in the recurrence rate after complete DAVF occlusion and lacks larger series with long-term follow-up. The authors present the largest series to date with a long-term follow-up to determine the stability of Onyx, prospectively comparing magnetic resonance angiography (MRA) and digital subtraction angiography (DSA) as follow-up diagnostic methods. METHODS Demographics, clinical symptomatology, length of follow-up, diagnostic methods, and angiographic findings of DAVFs were recorded and retrospectively evaluated in 112 patients. A prospective group of 15 patients with more than 5 years of follow-up after complete DAVF occlusion was established. All 15 patients in the prospective group underwent a clinical examination and MRA; 10 of these patients also underwent DSA. The recurrences and the correlation between the two diagnostic methods were evaluated. RESULTS Among the 112 patients, 71 were men and 41 were women, with an average age of 60 years. Intracranial hemorrhage (40%) was the most common clinical presentation of DAVF. At the last follow-up, 73% of the patients experienced clinical improvement, 21% remained unchanged, and 6% worsened. Overall, 87.5% of the DAVFs were occluded entirely with endovascular treatment, and 93% of the DAVFs were classified as cured at the last follow-up (i.e., completely embolized DAVFs and DAVFs that thrombosed spontaneously or after Gamma Knife surgery). Two recurrences of DAVFs were recorded in the entire series. Both were first diagnosed by MRA and confirmed with DSA. The mean follow-up was 27.7 months. In the prospective group, a small asymptomatic recurrence was diagnosed. The mean follow-up of the prospective group was 96 months. CONCLUSIONS Onyx is a stable embolic material, although recurrence of seemingly completely occluded DAVFs may develop because of postembolization hemodynamic changes that accentuate primarily graphically absent residual fistula. These residuals can be diagnosed with MRA at follow-up. The authors’ data suggest that MRA could be sufficient as the follow-up diagnostic method after complete DAVF occlusion with Onyx. However, larger prospective studies on this topic are needed.

Background Spinal dural arteriovenous fistula (SDAVF) is a rare cause of progressive myelopathy in predominantly middle-aged men. Treatment modalities include surgical obliteration and endovascular embolization. In surgically treated cases, failure of obliteration is reported in up to 5%. The aim of this technical note is to present a safe procedure with complete SDAVF occlusion, verified by intraoperative digital subtraction angiography (DSA). Methods We describe four patients with progressive leg weakness who underwent surgical obliteration of SDAVF with spinal intraoperative DSA in the prone position after cannulation of the popliteal artery. All surgeries took place in our hybrid operating room (OR) and were accompanied by electrophysiologic monitoring. Surgeries and cannulation of the popliteal artery were performed in the prone position. Ultrasound was used to guide the popliteal artery puncture. A 5-Fr sheath was inserted and the fistula was displayed using a 5-Fr spinal catheter. Spinal intraoperative DSA was performed prior to and after temporary clipping of the fistula point as well after the final SDAVF occlusion. Results The main feeder of the SDAVF fistula in the first patient arose from the right T11 segmental artery, which also supplied the artery of Adamkiewicz. The second patient initially underwent endovascular treatment and deteriorated 5 months later due to recanalization of the SDAVF via a small branch of the T12 segmental artery. The third and fourth cases were primarily scheduled for surgical occlusion. Access through the popliteal artery for spinal intraoperative DSA proved to be beneficial and safe in the hybrid OR setting, allowing the sheath to be left in place during the procedure. During exposure and after temporary and permanent occlusion of the fistulous point, intraoperative indocyanine green (ICG) video angiography was also performed. In one case, the addition of intraoperative DSA showed failure of fistula occlusion, which was not visible with ICG angiography, leading to repositioning of the clip. Complete fistula occlusion was documented in all cases. Conclusion Spinal intraoperative DSA in the prone position is a feasible and safe intervention for rapid localization and confirmation of surgical SDAVF occlusion.

Background and Purpose: Paravertebral arteriovenous shunts (PVAVSs) are rare. Whether the intradural venous system is involved in drainage may lead to differences in clinical characteristics through specific pathophysiological mechanisms. This study aims to comprehensively evaluate the natural history and clinical outcomes of PVAVSs with or without intradural drainage. Methods: Sixty-four consecutive patients with PVAVSs from 2 institutes were retrospectively reviewed. Lesions were classified as type A (n=28) if the intradural veins were involved in drainage; otherwise, they were classified as type B (n=36). The clinical course from initial presentation to the last follow-up was analyzed. Results: The patients with type A shunts were older at presentation (52.5 versus 35.5 years, P P =0.00006). After presentation, the deterioration rates related to gait and sphincter dysfunction were significantly higher in patients with type A than type B shunts (gait dysfunction: 71.8%/y versus 17.0%/y, P =0.0006; sphincter dysfunction: 63.7%/y versus 11.3%/y, P =0.0002). According to the angiogram at the end of the latest treatment, 79% of type A and 75% of type B PVAVSs were completely obliterated. If the lesions were partially obliterated, a significantly higher clinical deterioration rate was observed in patients with type A shunts than those with type B shunts (69.9%/y versus 3.2%/y, P =0.0253). Conclusions: Type A PVAVSs feature rapid progressive neurological deficits; therefore, early clinical intervention is necessary. For complex lesions that cannot be completely obliterated, surgical disconnection of all refluxed radicular veins is suggested.

A 78-year-old man presented with nephrotic syndrome and new-onset disorientation. Plasma D-dimer level was increased, and a lower leg deep vein thrombosis was identified on ultrasound. Histopathologic analysis of percutaneous renal biopsy samples confirmed the diagnosis of minimal change disease. Treatment with prednisone (20 mg/day), cyclosporine (50 mg/day), and anticoagulant therapy with edoxaban tosylate hydrate led to the complete resolution of nephrotic syndrome after 4 weeks. Despite this, his disorientation persisted. Head CT and MRI have revealed cerebral venous sinus thrombosis and dural arteriovenous fistula, which was considered a possible complication of nephrotic syndrome. Embolization dramatically improved his disorientation. This paper highlights that cerebral venous sinus thrombosis and dural arteriovenous fistula should always be considered in patients with nephrotic syndrome and new-onset disorientation.

A 75-year-old man presented with bilateral frontal hematomas owing to a dural arteriovenous fistula involving an isolated segment of the superior sagittal sinus. After an unsuccessful attempt of transarterial endovascular embolization of the dural arteriovenous fistula, the fistula was occluded using a transvenous approach through recanalization of the occluded superior sagittal sinus.

(1) Background: Dural arteriovenous fistulas (DAVF) represent a rare acquired intracranial vascular malformation, with a variety of clinical signs and symptoms, which make their diagnosis difficult. Intracranial hemorrhage is one of the most serious clinical manifestations. In this paper the authors’ goal was to verify the accuracy and utility of contrast-enhanced brain CT angiography (CTA) for the identification and the characterization of dural arteriovenous fistulas (DAVFs) in patients who presented with brain hemorrhage compared to 3D digital subtraction angiography (3D DSA); (2) a retrospective study of 26 patients with DAVFs who presented with intracranial hemorrhage to our institution was performed. The information reviewed included clinical presentation, location and size of hemorrhage, brain CTA and 3D DSA findings; (3) results: 61% (16/26) of DAVFs were identified by CTA. The vast majority of patients were male (69%, 18/26) and the most common presenting symptom was sudden onset headache. All DAVFs had cortical venous drainage and about one-third were associated with a venous varix. The most common location was tentorial (73%, 19/26); (4) conclusions: CTA can represent a valid alternative diagnostic method to 3D DSA for the study of DAVF in the initial and preliminary diagnostic approach, especially in emergency situations. In fact, it represents a fast, inexpensive, non-invasive and above all, easily accessible and available diagnostic technique, unlike DSA or MRI, allowing to provide information necessary for the identification, classification and treatment planning of DAVFs.

Pial arteriovenous fistulas (AVFs) are rare neurovascular malformations. They differ from arteriovenous malformations (AVMs) in that they involve single or multiple feeding arteries, draining directly into a dilated cortical vein with no intervening nidus. Pial and dural AVFs differ in blood supply, as the first originate from pial or cortical arteries and the latter from outside the dural leaflets. Unlike dural AVFs, most of the pial AVFs are supratentorial. The vast majority are congenital, manifesting during infancy. Acquired pial AVFs are significantly rarer and occur after vasculopathy, head trauma, brain surgery, or cerebral vein thrombosis. We describe a unique case of an acquired pial AVF in a 50-year-old man secondary to a cortical vein thrombosis manifesting as a focal-onset seizure with secondary generalization. A cerebral digital subtraction angiography revealed a low-flow pial AVF fed by a postcentral branch of the left middle cerebral artery draining to the superior sagittal sinus via a cortical vein. It also showed a collateral venous circulation adjacent to the previously thrombosed left parietal vein. There was no evidence of an associated dural AVF or venous varix. Endovascular treatment was scheduled three months later, but the angiogram preceding the embolization showed spontaneous and complete closure of the malformation. To our knowledge, this is the first case illustrating acquired pure pial AVF unaccompanied by a dural component following cortical vein thrombosis, eventually resulting in an unprompted closure.

Background Cognitive decline is a non-hemorrhagic, major complication of intracranial dural arteriovenous fistula (DAVF), thought to be primarily related to venous hypertension. However, imaging features to predict cognitive decline are scanty in the literature. Purpose To evaluate functional connectivity (FC) changes of resting-state networks (RSNs) in DAVF before and after treatment and its relation to cognitive impairment. Study type Prospective. Subjects DAVF subjects were screened for inclusion. Pre-embolization (N = 33, mean age 45.9 years, 29 males), 1 month post-embolization (N = 20, mean age 42.7 years, 19 males), and healthy controls (HC, N = 33, mean age 45.09 years, 27 males). Field strength/sequence 3.0 T, resting-state functional magnetic resonance imaging (MRI), three-dimensional (3D) T1, T2 fast spin echo (FSE), diffusion weighted imaging (DWI), susceptibility weighted imaging (SWI), fluid-attenuated inversion recovery, and time of flight. Assessment Data quality assessment was performed. FC analysis was done using group independent component analysis (ICA) and seed to voxel analysis. Neuropsychology (NP) scores of patients were compared with HC and correlated with FC changes. Statistical tests Voxel-wise parametric T-statistics for F-test was executed in FC analysis (p-FDR corrected Results Both RSNs analysis methods showed reduced FC at the precuneus-posterior cingulate cortex (PC-PCC) of default mode network (DMN), anterior cingulate cortex (ACC) of the salience network (SN), and possible compensatory increased connectivity at the frontoparietal (FPN) and dorsal attention (DAN) networks. DAVF with low NP scores showed reduced FC at DMN and SN and minimal to absent connectivity at FPN and DAN. At post-embolization 1-month follow-up, improvement in FC at PC-PCC of DMN and ACC of SN were noted. Data conclusion RS-fMRI in DAVF displayed FC changes that may be related to cognitive decline and its subsequent reversibility after treatment. FC changes at DMN, SN, FPN, and DAN were linked to cognitive decline and the corresponding NP scores. Level of evidence 2 TECHNICAL EFFICACY: Stage 2.

Background Cranial dural arteriovenous fistulas (dAVFs) are rare lesions managed mainly with endovascular treatment (EVT) and/or surgery. We hypothesize that there may be subtypes of dAVFs responding better to a specific treatment modality in terms of successful obliteration and cessation of symptoms and/or risks. Methods All dAVFs treated during 2011–2018 at our hospital were analyzed retrospectively. Presenting symptoms, radiological variables, treatment modality, complications, and residual symptoms were related to dAVF type using the original Djindjian classification. Results We treated 112 dAVFs in 107 patients (71, 66% males). They presented with hemorrhage (n = 23; 21%), non-hemorrhagic symptoms (n = 75; 70%), or were discovered incidentally (n = 9; 8%). There were 25 (22%) type I, 29 (26%) type II, 26 (23%) type III, and 32 (29%) type IV fistulas. EVT was the primary treatment modality in 72/112 (64%) dAVFs whereas 40/112 (36%) underwent primary surgery with angiographic obliteration rates of 60% and 90%, respectively. Using a secondary treatment modality in 23 dAVFs, we obtained a final obliteration rate of 93%, including all type III/IV and 26/27 (96%) type II dAVFs. Except for headache, residual symptoms were rare and minor. Permanent neurological complications consisted of five cranial nerve deficits. Conclusions We recommend EVT as first treatment modality in types I, II, and in non-hemorrhagic type III/IV dAVFs. We recommend surgery as first treatment choice in acute hemorrhagic dAVFs and as secondary choice in type III/IV dAVFs not successfully occluded by EVT. Combining the two modalities provides obliteration in 9/10 dAVF cases at a low procedural risk.

Background There is no consensus as to whether balloon angioplasty alone or stent placement is effective for sinus occlusion associated with dural arteriovenous fistula (DAVF). Herein, we first report a case of transverse sinus occlusion associated with DAVF in which gradual sinus dilatation was observed after balloon angioplasty with embolization of the affected sinus with shunt flow. Case presentation A 69-year-old man presented with executive dysfunction. Magnetic resonance imaging revealed left transverse sinus–sigmoid sinus DAVF with occlusion of the left jugular vein and right transverse sinus. Before endovascular treatment, the patient had symptomatic epilepsy and subarachnoid hemorrhage. Retrograde leptomeningeal venous drainage disappeared with packing of the left transverse sinus–sigmoid sinus. Subsequently, balloon angioplasty of the right occluded transverse sinus was performed to maintain the normal venous drainage and remaining shunt outflow. Dilatation of the right transverse sinus was poor immediately after surgery. However, angiography after 10 days and 6 months revealed gradual dilatation of the right transverse sinus. Conclusion Sinus occlusion, which is thought to be caused by sinus hypertension associated with DAVF rather than chronic organized thrombosis or thrombophilia, may dilate over time after balloon angioplasty and shunt flow reduction if occluded sinus is necessary for facilitating normal venous drainage.

Contemporary treatments for spinal cord arteriovenous shunts are only based on clinicians’ treatment experiences and expertise due to its rarity. We reviewed the clinical course of the largest multicentred cohort to evaluate the efficacy and deficiency of contemporary interventional treatments for spinal cord arteriovenous shunts. The clinical features, treatment results and clinical outcomes of 463 patients with spinal cord arteriovenous shunts were retrospectively assessed. The main outcome was the neurological deterioration that was evaluated based on the modified Aminoff and Logue scale. According to post-treatment digital subtraction angiography, complete obliteration was defined as disappearance of the intradural lesion, whereas partial obliteration was defined as any residual intradural lesion remaining visible and was further categorized as shunt-reduction obliteration (the nidus or shunt points were reduced) or palliative obliteration (only obliterated aneurysms or feeders). Cure rate was 40.6% for the whole cohort, 58.5% after microsurgery, and 26.4% after embolization. The curative resection was associated with non-metameric lesions, lesions with a maximum diameter Although clinical risks of spinal cord arteriovenous shunts were reduced following clinical interventions, contemporary treatments for spinal cord arteriovenous shunt remains associated with considerable risks and incomplete efficacy. Individualized treatment plans should be adopted according to the angio-architectural features and major clinical risks of specific lesions. There is a higher opportunity for complete obliteration for lesions with simple angio-architecture. However, for most of spinal cord arteriovenous shunts with complex vascular anatomy, partial treatment is the only choice. For these patients, palliative obliteration targeting the aneurysms is recommended for reducing haemorrhagic risk, whereas shunt-reduction obliteration is necessary for non-haemorrhagic myelopathy. Contemporary treatment is ineffective in reducing haemorrhagic risk of incurable metameric spinal cord arteriovenous shunts.

A cavernous sinus (CS) dural arteriovenous fistula (DAVF) is a form of abnormal arteriovenous communication that can be treated with endovascular embolization. Establishing an optimal access route should be based on vascular architecture. We reviewed 64 patients with CS-DAVF who underwent endovascular embolization and report the endovascular treatment approach selection and outcome.Clinical data were obtained from 64 patients with CS-DAVF who had been surgically treated at the authors' hospital between 2009 and 2022. Patients' medical records, imaging data, and follow-up outcomes were retrospectively analyzed.All 64 patients (15 male, 49 female; mean age, 50 years) underwent CS-DAVF embolization. The most common symptoms were exophthalmos (39.1%), chemosis (35.9%), and headache (28.1%). On digital subtraction angiography images, 34.4% of the DAVFs were unilateral, and 82.8% were fed by both the external carotid artery and internal carotid artery. Of the patients' inferior petrosal sinuses (IPSs), 54.7% were nonopacified. The most common intravascular approaches included trans-IPS (37.5%) and trans-artery (28.1%) approaches. More than half of the CS-DAVFs were embolized by both coils and Onyx (62.5%). A total of 85.9% of the fistulas were completely embolized, and the follow-up rate was 76.6%. The modified Rankin Scale score was 0.9 ± 1.0.The vascular architecture of CS-DAVF is closely related to endovascular treatment approach selection and outcome. Combined with the modified IPS recanalization technique, the trans-IPS approach is the safest and most effective approach. Dual microcatheter and balloon assistance techniques ensure the safety and completeness of embolization.

Meningioangiomatosis (MA) is a disease that is extremely rarely reported. Sporadic MA is occasionally combined with meningioma or other lesions (identified as non-pure MA). This retrospective study investigated the difference between pure MA and non-pure MA by exploring clinical manifestations, histopathology characteristics, and outcomes of MA after surgery. We reviewed the medical records of 36 histopathologically confirmed MA patients (18 pure MA and 18 non-pure MA) who received surgery at our institution between 2012 and 2021. We compared differences in demographic, clinical, imaging, pathological features, and surgical outcomes between pure MA and non-pure MA through descriptive statistics. Compared to non-pure MA, pure MA presented with a more prominent male predilection (5:1 vs. 1.57:1, P = 0.264), a higher seizure incidence (83.3% vs 50.0%, P = 0.038), a more seizure type of GTCS (14/15 vs 5/9, P = 0.047), a less prominent enhancement on MRI (27.8% vs 88.9%, P 0.001) and a preference of temporal and frontal lobe (100% vs 44.4%, P 0.001). The differences in clinical characteristics between pure MA and non-pure MA demonstrate their disparate biological natures. Pure MA seems to be a non-neoplastic lesion, while non-pure MA is commonly combined with meningioma, which is a neoplastic lesion. A correct differential diagnosis can be achieved via a triad of the type of seizure, the location of lesion and the radiological presentation. MA is curable and the prognosis is excellent as most patients are free of seizure and recurrence after surgical treatment.

BACKGROUND AND PURPOSE: AVFs of the foramen magnum region, including fistulas of the marginal sinus and condylar veins, have complex arterial supply, venous drainage, symptoms, and risk features that are not well-defined. The purpose of this study was to present the angioarchitectural and clinical phenotypes of a foramen magnum region AVF from a large, single-center experience. MATERIALS AND METHODS: We retrospectively reviewed cases from a 10-year neurointerventional data base. Arterial and venous angioarchitectural features and clinical presentation were extracted from the medical record. Venous drainage patterns were stratified into 4 groups as follows: type 1 = unrestricted sinus drainage, type 2 = sinus reflux (including the inferior petrosal sinus), type 3 = reflux involving sinuses and cortical veins, and type 4 = restricted cortical vein outflow or perimedullary congestion. RESULTS: Twenty-eight patients (mean age, 57.9 years; 57.1% men) had 29 foramen magnum region AVFs. There were 11 (37.9%) type 1, nine (31.0%) type 2, six (20.7%) type 3, and 3 (10.3%) type 4 fistulas. Pulsatile tinnitus was the most frequent symptom (82.1%), followed by orbital symptoms (31.0%), subarachnoid hemorrhage (13.8%), cranial nerve XII palsy (10.3%), and other cranial nerve palsy (6.9%). The most frequent arterial supply was the ipsilateral ascending pharyngeal artery (93.1% ipsilateral, 55.5% contralateral), vertebral artery (89.7%), occipital artery (65.5%), and internal carotid artery branches (48.3%). CONCLUSIONS: We present the largest case series of foramen magnum region AVFs to date and show that clinical features relate to angioarchitecture. Orbital symptoms are frequent when sinus reflux is present. Hemorrhage was only observed in type 3 and 4 fistulas.

Background and importance Dural arteriovenous fistulas (dAVFs) are vascular abnormalities of the central nervous system that can cause a wide array of neurological dysfunction depending on their location, flow, and propensity to rupture. Symptomatic dAVFs at the cranio-cervical junction usually result in hemorrhage or cervical myelopathy. Distantly located dAVFs of the foramen magnum are a rare cause of thoracic intrinsic myelopathy. Clinical presentation An 83-yr-old man presented with progressive lower extremity weakness, numbness, and difficulty walking along with episodes of bowel incontinence. Magnetic resonance imaging of the cervical spine demonstrated multilevel cervical disc disease with stenosis and longitudinal cervical cord signal change extending into the upper thoracic spinal cord. Cerebral and spinal angiography revealed a dAVF in the lateral foramen magnum region. Given the location, feeding vasculature, and morphology of the fistula, endovascular embolization was not attempted. Microsurgical resection with confirmative indocyanine green fluorescent imaging was performed with adequate obliteration of the fistula. The patient's neurological baseline was preserved postoperatively with improvement of lower extremity numbness. Conclusion We present a brief overview of this neuropathologic entity and demonstrate microsurgical resection of a foramen magnum dAVF through operative video. Craniocervical dAVFs should remain on the differential diagnosis of patients presenting with progressive thoracolumbar myelopathy.

International audience; Background and purpose: Spinal arteriovenous fistulas are challenging to cure by endovascular means, with a risk of incomplete occlusion or delayed recurrence. The authors report herein their preliminary experience using the pressure cooker technique for the embolization of spinal arteriovenous fistulas.Materials and methods: Fifteen patients (8 men; mean age, 60.3 years) underwent an endovascular treatment of a spinal arteriovenous fistula (12 dural spinal arteriovenous fistulas and 3 epidural spinal arteriovenous fistulas) in 2 different institutions using the pressure cooker technique. Two microcatheters could be navigated in the segmental artery in all patients using 2 guiding catheters. A proximal plug was achieved with highly concentrated cyanoacrylate ± coils. The liquid embolic agent injected to cure the fistula was diluted cyanoacrylate (n = 11) or ethylene-vinyl alcohol (n = 4). Technical and clinical complications were systematically recorded. Clinical and angiographic outcomes were systematically evaluated at follow-up.Results: One (6.7%) procedure-related complication was recorded, which consisted of a transient radicular deficit, related to nerve root ischemia. Clinical improvement was observed in 10/14 (71%) patients for whom clinical follow-up was available. Complete spinal arteriovenous fistula occlusion on a follow-up angiography was observed in 11/12 patients (91.7%) for whom angiographic follow-up was available. One patient (8.3%) presented with a delayed recurrence at 29 months.Conclusions: The pressure cooker technique is feasible, with either glue or ethylene-vinyl alcohol, for the embolization of spinal arteriovenous fistulas. Our results suggest the safety and effectiveness of this technique.

Background and Purpose Dural arteriovenous fistulae (DAVFs) can develop secondary to cerebral venous thrombosis (CVT). The incidence of DAVF has not yet been investigated prospectively. Methods Between July 2012 and January 2018, combined static and dynamic 4D MR venography (4D-combo-MRV) was performed in 24 consecutive patients at diagnosis of CVT and after 6 months. 3 Tesla magnetic resonance imaging with time of flight and contrast-enhanced magnetization-prepared rapid acquisition with gradient echo were performed at baseline to evaluate the extent of thrombosis and affected vessel segments. Baseline and follow-up 4D-combo-MRV were assessed for signs of DAVF. Interrater reliability of DAVF detection and the extent of recanalization were analyzed with kappa statistics. Results DAVFs were detected in 4/30 CVT patients (13.3%, 95% confidence interval [CI] 3.3–26.7). Two of 24 patients (8.3%, 95% CI: 0–20.8) had coincidental DAVF with CVT on admission. At follow-up, de novo formation of DAVF following CVT was seen in 2/24 patients (8.3%, 95% CI: 0–20.8). Both de novo DAVFs were low grade and benign fistulae (Cognard type 1, 2a), which had developed at previously thrombosed segments. Endovascular treatment was required in two high degree lesions (Cognard 2a + b) detected at baseline and in one de novo DAVF (Cognard 1) because of debilitating headache and tinnitus. Thrombus load, vessel recanalization, and frequency of cerebral lesions (hemorrhage, ischemia) were not associated with DAVF occurrence. Conclusion This exploratory study showed that de novo DAVF formation occurs more frequently than previously described. Although de novo DAVFs were benign, 75% of all detected DAVFs required endovascular treatment. Therefore, screening for DAVF by dynamic MRV, such as 4D-combo-MRV, seems worthwhile in CVT patients.

Background Spinal dural arteriovenous fistulas (SDAVFs) are rare vascular malformations. Digital subtraction angiography is the modality of choice to demonstrate the malformation before endovascular embolization or open surgical repair. Angiographically occult SDAVFs have been previously reported. Surgical considerations in SDAVFs with misleading angiography findings have not yet been assessed. Methods A retrospective evaluation of charts and imaging files of patients operated on for SDAVF in 2018–2019 at a single institution was performed. All patients were referred to surgery following failure of endovascular embolization or owing to clinical and radiographic deterioration in the presence of an angiographically occult lesion. Cases were comprehensively reviewed and evaluated for surgical considerations in these lesions. Results This case series included 4 cases. Two patients underwent embolization before surgical repair but continued to deteriorate neurologically, and 2 patients had a failed embolization attempt owing to a torturous vascular network. In all 4 patients, exploration was successful, yielding either improvement or stabilization of neurological status. Indocyanine green injection for microscopically integrated fluorescent angiography contributed to the identification of the supplying vessels and confirmed the SDAVF closure. Conclusions SDAVFs should be treated promptly after diagnosis. In cases with high suspicion for SDAVF with occult or misleading angiography findings, spinal exploration should be pursued with no delay. Indocyanine green–assisted microscopic angiography may contribute to exploratory spine surgery for SDAVF closure.

Background The principles of endovascular treatment of bilateral cavernous sinus dural arteriovenous fistula (CS-dAVF) are not well established because of the complexity in the number of fistula tracts and their behavior, which seems to be more aggressive. We aimed to determine an efficient technique for endovascular treatment of bilateral CS-dAVF and the associated factors to achieve good clinical and angiographic outcomes. Methods The data were analyzed from 165 consecutive patients diagnosed with CS-dAVF from January 2005 to September 2018. The demographic data included approaching route, embolization times, embolization material, sequence of embolization, number of embolization sessions, and angiographic and clinical outcomes. Interrater agreement of bilateral CS-dAVF diagnosis was performed using the κ coefficient. The factors associated with treatment outcome were analyzed using a Pearson χ2 test. Results Bilateral CS-dAVF was detected in 43 patients (26%). Angiographic presentations that showed evidence of sinus thrombosis, dangerous venous drainage, and higher Satomi classification were more commonly found in bilateral CS-dAVF than in unilateral CS-dAVF. Good clinical outcome and cure from angiography were obtained in 90% and 74%, respectively. Ipsilateral inferior petrosal sinus–intercavernous sinus–contralateral cavernous sinus catheterization was the major approach route of treatment. The factors associated with improved clinical outcome were transvenous approach, shunt closure, coil embolization, and sequencing the embolization (P Conclusions Dangerous venous drainage tends to increase in bilateral CS-dAVF. Retrograde ipsilateral inferior petrosal sinus catheterization using coil embolization and sequencing the embolization are the major concerns for treatment.

Although transvenous embolization (TVE) via the superior ophthalmic vein (SOV) is adopted in treating cavernous sinus dural arteriovenous fistula (CS DAVF), its effect on the coil volume is rarely understood. The purpose of the study was to investigate if there is a difference in the total number of coils used and in patient safety when comparing two access strategies. We retrospectively reviewed charts for patients with CS DAVF treated with TVE between January 2008 and March 2018. The baseline patient characteristics, details of procedure, placed coils, and clinical results were compared. A total of 42 patients with CS DAVF were treated with the inferior petrosal sinus (IPS) (n = 32) or SOV (n = 10) approach. TVE via SOV showed a high success rate of 100% (10/10) by transfemoral access. The total number (23 versus 11; P

Objective Typically, the clinical presentation of a spinal dural arteriovenous fistula (SDAVF) will be insidious, with patients' symptoms regularly attributed to other conditions. Although previous studies have characterized the neurologic outcomes after treatment for SDAVFs, little is known about the pretreatment patient characteristics associated with poor and/or positive patient outcomes. We sought to characterize the pretreatment patient demographics, diagnostic history, and neurologic outcomes of patients treated for SDAVFs and to identify the patient factors predictive of these outcomes. Methods The medical records of patients who had been treated for SDAVFs from 2006 to 2018 across 1 healthcare system were retrospectively analyzed. Neurologic status was assessed both before and after intervention using the Aminoff-Logue scales for gait and micturition disturbances. Results Of 46 total patients, 16 (35%) had a documented misdiagnosis. Patients with a history of misdiagnosis had had a significantly longer symptom duration before treatment compared with those without a misdiagnosis (median, 2.3 vs. 0.9 years; P = 0.018). A shorter symptom duration before intervention was significantly associated with both improved motor function (median, 0.8 vs. 3.1 years; P = 0.001) and improved urinary function (median, 0.8 vs. 2.2 years; P = 0.040) after intervention. Conclusions Misdiagnosis has been relatively common in patients with SDAVFs and contributes to delays in treatment. Delays in diagnosis and treatment of SDAVFs appear to be associated with worse clinical outcomes for patients who, ultimately, receive treatment.

Background Foramen magnum dural arteriovenous fistula (DAVF) is a rare disease, with some reported cases of successful treatment. We achieved complete obliteration of the foramen magnum DAVF through microsurgery after complications of endovascular embolization. We reviewed the treatment modalities and outcomes, focusing on pathologic and anatomic features from the literature. Case Description A 65-year-old man was admitted to our hospital with sudden diplopia. Magnetic resonance imaging revealed a subarachnoid hemorrhage around the right side of the prepontine cistern, and a foramen magnum DAVF was diagnosed by angiography. Subsequent angiography revealed that the fistula was supplied by the right neuromeningeal trunk of the ascending pharyngeal artery and the right posterior meningeal artery of the vertebral artery (VA), and the veins of the pouch via the fistula were retrogradely draining into the intracranial veins. We aimed to treat complete occlusion endovascularly with balloon-augmented transarterial Onyx injection via the posterior meningeal artery, but Onyx was refluxed to the VA through the anastomosis between the VA and posterior meningeal artery. Onyx subsequently migrated to the top of the basilar artery, causing occlusion, so we urgently removed the Onyx with a stent retriever (Trevo). Several weeks later, we performed complete obliteration of the foramen magnum DAVF via a lateral suboccipital approach with a C1 laminectomy. Conclusions Most foramen magnum DAVFs were obliterated completely with only endovascular treatment. Microsurgery is an effective and reliable treatment for incomplete occlusion and complications.

Background Brain dural arteriovenous fistulas(bDAVFs) are anomalous connections between dural arteries and cerebral veins or sinuses. Cerebral venous thrombosis(CVT) often precedes or coincides with bDAVFs and is considered a risk factor for these vascular malformations. Recently, vaccine-induced thrombotic thrombocytopenia causing CVTs has been associated with COVID-19 vaccines. Concurrently with the start of massive vaccination in our region, we have observed a fivefold increase in the average incidence of bDAVFs. Objective To raise awareness of the potential involvement of COVID-19 vaccines in the pathogenesis of bDAVF. Methods A retrospective review of demographic, clinical, radiological, COVID-19 infection and vaccination data of patients diagnosed with bDAVFs between 2011-2021 was conducted. Patients were divided into two cohorts according to their belonging to pre- or post-COVID-19 vaccination times. Cohorts were compared for bDAVFs incidences and demographic and clinical features. Results Twenty-one bDAVFs were diagnosed between 2011- 2021, 7 of which were diagnosed in 2021. The mean age was 57.7 years, and 62% were males. All cases except one were treated; of them, 85% were exclusively managed with surgery. All treated cases were successfully occluded. The incidence in 2021 was significantly higher than that in the prevaccination period (1.72 vs 0.35/100,000/year;p=0.036; 95%Confidence Interval=0.09-2.66). Cohorts were not different in age, sex, hemorrhagic presentation, dural sinus thrombosis or presence of prothrombotic or cardiovascular risk factors. Conclusion The significant increase in the incidence of bDAVF following general vaccination policies against COVID-19 observed in our region suggests a potential correlation between these two facts. Our findings need confirmation from larger cohorts and further pathogenic research.

Middle meningeal artery (MMA) embolization is becoming increasingly studied as a safe, effective treatment for chronic subdural hematoma (cSDH) in adults. Among pediatric patients, however, MMA embolization for cSDH has been rarely described, and the potential benefit of this approach for pediatric patients remains unknown.To systematically review the literature and identify cases of pediatric MMA embolization for cSDH. We also report our experience with pediatric MMA embolization.A systematic review of the literature was performed to identify cases of pediatric MMA embolization for cSDH. Inclusion criteria included English language availability and pediatric age defined as less than 18 years. A pediatric patient treated with MMA embolization was also identified at our institution.Five cases of pediatric MMA embolization for cSDH were identified in the literature. Two were associated with arachnoid cysts, 2 with antiplatelet/anticoagulation therapy, and 1 with abusive head trauma. There were no adverse events, and all patients demonstrated clinical and radiological improvement on follow-up. At our institution, a previously healthy 8-year-old male was found to have a right-sided acute-on-chronic SDH during a headache evaluation. A diagnostic angiogram was performed to rule out a dural arteriovenous fistula, and right-sided MMA embolization was performed concurrently. Rapid clinical and radiological improvement was observed, with complete resolution by 6 months.MMA embolization may represent a treatment option for pediatric patients with cSDH.

Spinal dural arteriovenous fistula (SDAVF) is a rare but curable condition. Microsurgery is a highly effective and readily affordable treatment modality.We present a surgical video of SDAVF to demonstrate the operative nuances involved.A 53-year-old wheelchair-bound man with spastic paraparesis for 1.5 years was found to have a SDAVF at L1/2 level with a single fistula point. During surgery, a L1-L2 laminectomy and durotomy revealed a dilated vein accompanying the nerve root exiting L1/2 foramen that showed early filling on indocyanine green (ICG) video angiography. This vein was occluded, and a segment of this vein was removed during surgery, which led to resumption of normal spinal cord perfusion.The patient showed gradual recovery of lower limb motor power and improved to assisted ambulation after 3 months.Surgery is a simple, effective, and cost-effective treatment option in SDAVF.

Introduction Transcranial color-coded duplex sonography (TCCS) with and without ultrasound contrast agent has been used to diagnose cerebral venous thrombosis, however, no experience in the diagnosis of patients with cerebral venous thrombosis and with dural arteriovenous fistula (dAVF) by contrast-enhanced TCCS has been reported yet. Case report The authors reported a 49-year-old male patient with straight sinus (StS) thrombosis and dAVF. Plain TCCS and contrast-enhanced TCCS demonstrated the direct presentation of the selected feeder and drainage veins of the dAVFs, arterialized venous flow waveform, and an enlarged optic nerve sheath diameter, indicating an increased intracranial pressure, and a filling defect in the StS even after administration of an ultrasound contrast agent. After intravascular thrombolysis and blockage of 2 feeders of the dAVF, TCCS still showed retrograde flow direction in the left basal vein and significantly elevated flow velocity in the StS, reflecting a high venous pressure caused by a high volume of arteriovenous shunt and severe stenosis in the StS. The patient's clinical manifestation did not substantially improve until 3 stents were implanted in the StS, and TCCS unveiled that the stents were well filled with flow signal, and the flow velocity in the StS dramatically decreased. Conclusions TCCS could provide reliable data about the feeder and drainage veins of dAVF in our case. The extent of an increase in venous flow velocity may be closely associated with clinical manifestations, which may influence therapy. Moreover, TCCS possesses a unique advantage in terms of evaluating the patency of the stents compared with other neuroimaging techniques.

Asymptomatic dural arteriovenous fistulas (DAVFs) with cortical venous reflux (CVR) are now more commonly encountered. However, patients with an incidental onset may have a less aggressive clinical course. It is desirable to explore methods and indicators to predict the clinical outcomes. This study investigates whether the relative signal intensity (rSI) of the draining vessels on the time-of-flight magnetic resonance angiography is related to clinical behavior in patients with intracranial DAVFs. We retrospectively reviewed 36 intracranial DAVFs. The patients were categorized as those with either aggressive-presentation or non-aggressive-presentation (n = 16 and 20, respectively). The rSIs of the shunt points, affected sinuses, and veins with CVR were compared between the two groups. The two groups were not significantly different in terms of rSIs of the shunt points and affected sinuses (p = 0.37 and 0.41, respectively). However, a significant positive correlation was observed in the rSI of the veins with CVR between the aggressive and non-aggressive behavior groups (p