Reconstruction of the sphenoid sinus erosion or dehiscence after treatment of unruptured intracavernous aneurysms with flow diverter stents.

BACKGROUND: Intracavernous carotid aneurysms (ICCAs) are rare, frequently asymptomatic, with a low rupture risk, which, however, can lead to life-threatening epistaxis. The aim of this study was to assess the effect of the treatment of asymptomatic ICCAs with flow diverters (FD) on sphenoid bone erosion or dehiscence in a selected cohort of patients. METHODS: We retrospectively reviewed all asymptomatic ICCAs with sphenoid bone erosion or dehiscence detected on cone beam CT (CBCT) and treated with FD between December 2018 and December 2022. Patients were followed-up with CBCT and bone reconstruction was blindly evaluated by two interventional neuroradiologists and classified as unchanged, partial, or complete. RESULTS: A total of 10 patients (women: 90%, mean age 58 years) treated with an FD for an asymptomatic ICCA with associated sphenoid bone erosion or dehiscence were included in this cohort. Sphenoid bone erosion was present in seven patients and dehiscence was observed in the remaining three. After treatment with FD, complete reconstruction of the sphenoid sinus wall occurred in seven cases, and partial reconstruction in two cases. Sphenoid bone erosion remained unchanged after treatment in only one patient. CONCLUSIONS: The decision to treat asymptomatic and unruptured ICCAs remains challenging due to their benign natural history and low hemorrhagic risk. The presence of sphenoid sinus erosion or dehiscence should not be overlooked since it could be considered as an indication for prophylactic treatment of life-threatening epistaxis. The mechanisms of bone erosion by the aneurysm and of reconstruction after treatment are still to be fully elucidated.

Angiographic collateral venous phase: a novel landmark for leptomeningeal collaterals evaluation in acute ischemic stroke.

BACKGROUND: Although recanalization rates constantly increase (>80%), a favorable clinical outcome is achieved in only 45-55% of patients undergoing mechanical thrombectomy (MT) for anterior circulation stroke. Collateral circulation seems to play a major role in determining this discrepancy. The aim of the study was to investigate a novel angiographic landmark assessing the collateral venous phase (CVP) compared with the American Society of Interventional and Therapeutic Neuroradiology/Society of Interventional Radiology (ASITN/SIR) score, based on the arterial collateral assessment. METHODS: Two hundred patients with anterior circulation stroke treated by MT between 2016 and 2021 were included. The ASITN/SIR score and the presence of CVP were blindly evaluated by expert neuroradiologists. Three subanalyses were performed comparing patients with good versus poor collaterals, CVP presence versus absence, and a composite analysis including both ASITN/SIR and CVP grading results. RESULTS: Good collateral circulation (ASITN >2) was observed in 113 patients (56.5%) whereas CVP was present in 90 patients (45%) and mostly in patients with good collaterals. Favorable clinical and neuroradiological outcomes were more likely observed in patients with both good collaterals and the presence of CVP than in those with good collaterals and absence of CVP (modified Rankin Scale score 0-2: 77.3% vs 7.9%, p<0.0001; mortality: 9.3% vs 26.3%, p=0.02; 24-hour Alberta Stroke Program Early CT Score: 8 vs 6, p<0.0001), while ASITN/SIR score alone was not significantly associated with clinical outcomes. CONCLUSIONS: The presence of CVP improves the angiographic assessment of collateral circulation. CVP could be proposed as a new imaging landmark to better understand the functionality of collaterals.

Rupture of a detachable-tip microcatheter away from the detachment zone during Onyx 18 embolization of an arteriovenous malformation and management of the complication.

Leakage of liquid embolic material is a rare but dreaded complication during embolization of an arteriovenous malformation (AVM). We describe the rupture of a microcatheter away from the detachment site and the successful removal of the cast of Onyx 18 in the carotid artery by aspiration. A young adult patient diagnosed with a right temporal unruptured AVM underwent endovascular embolization using Onyx 18. The AVM was approached using an Apollo microcatheter. After 4 min of Onyx 18 injection, leakage of Onyx 18 in the petrous segment of the internal carotid artery was noticed while the distal tip of the microcatheter remained patent. The plug of Onyx was successfully removed using an aspiration technique via the Envoy DA catheter. Analysis of the microcatheter confirmed that the rupture occurred away from the detachment site. However, no definite cause for the rupture has been identified.

Natural history and management of spinal cord arteriovenous shunts in pregnancy: A monocentric series of 10 consecutive cases with emphasis on endovascular treatment.

PURPOSE: Intradural spinal cord arteriovenous shunts (IDSCAVS) are rare and constitute a challenging situation if symptoms occur during pregnancy. We present a series of ten such cases referred to our center: five cervical, four thoracic and one lumbar. METHODS: We retrospectively reviewed our global series of 215 IDSCAVSs between 2002 and March 2020 and found ten patients who had presented during pregnancy. Clinical, radiological and therapeutic data were studied. RESULTS: Seven shunts were AVM type niduses and three were micro AV-fistulae. All were associated with pial venous reflux and six hemorrhagic cases had pseudo aneurysms. Symptoms occurred mainly during the third trimester, 80% of patients presented with hemorrhage and spinal cord dysfunction. We embolized seven patients and proposed surgery in one, always after delivery: all recovered well. One woman declined treatment; one other was operated in emergency but did not improve. Mean follow-up was 3.9 years (0.5...19 years). CONCLUSIONS: Despite this small group of patients, our initial experience of IDSCAVSs diagnosed during pregnancy indicates that embolization is an effective management strategy if performed after delivery and a recovery period. Results indicate that IDSCAVSs seem to have a low risk of early rebleedings after the ictal event and may be closely followed up until delivery. The results obtained show good clinical outcome without long-term rebleeds. Women with known IDSCAVSs should not be discouraged from becoming pregnant, however it seems wise to embolize them before pregnancy in order to offer protection against risks during pregnancy.

Mechanical Thrombectomy in Distal Residual Occlusions of the Middle Cerebral Artery after Large Vessel Recanalization in Acute Stroke: 2b or not 2b? A Pragmatic Approach in Real-Life Scenarios.

BACKGROUND /OBJECTIVE: Recent studies have suggested that a recanalization grade of modified Thrombolysis in Cerebral Infarction (mTICI) score ≥2c is strongly related with good clinical outcome rather than the current therapeutic angiography target ≥2b. To achieve better recanalization, additional further maneuvers on distal residual vessel occlusion (RVO) may be required. The aim of this study was to evaluate the safety and efficacy of rescue treatment in RVOs after recanalization of large vessel occlusions in the anterior circulation. METHODS: A single-center retrospective review of a prospectively maintained stroke databank was performed. Patients presenting with RVOs after mechanical thrombectomy on the M1/internal carotid artery terminus were included and further divided into treated and untreated groups: the former underwent additional maneuvers on RVOs, whereas the latter did not. Baseline and posttreatment clinical, radiologic, and angiographic data were compared between the 2 groups. End points included good functional outcome (modified Rankin Scale [mRS] score ≤2) rates of hemorrhagic transformations, neurologic deterioration and mortality. RESULTS: RVOs were observed in 183/488 patients (37.5%). 74/183 (40.4%) underwent rescue treatment, showing a better outcome in terms of median 24 hours National Institutes of Health Stroke Scale score (13 vs. 18; P < 0001), 24 hours Alberta Stroke Programme Early CT Score (6 vs. 5; P < 0.001) and 3 months mRS score 0-2 (47.3% vs. 33.1%; P = 0.06). Recanalization of the superior (frontal) branch of the middle cerebral artery was particularly critical in terms of outcome. Hemorrhagic transformation was higher in the untreated group (53.6% vs. 66.6%; P = 0.1) as well as symptomatic intracranial hemorrhage (13.1% vs. 29.4%; P = 0.01). Neurologic deterioration occurred more often among untreated patients (16.2% vs. 25.7%; P = 0.1). Three complications (1.3%) occurred during rescue treatment. CONCLUSIONS: When feasible, improving mTICI score 2a-2b recanalization to mTICI 2c/3 is safe and associated with a better clinical outcome, particularly for residual occlusions involving the superior branch of bifurcation.

Immediate post-operative aneurysm occlusion after endovascular treatment of intracranial aneurysms with coiling or balloon-assisted coiling in a prospective multicenter cohort of 1189 patients: Analysis of Recanalization after Endovascular Treatment of intracranial Aneurysm (ARETA) Study.

BACKGROUND: Coiling, including balloon-assisted coiling (BAC), is the first-line therapy for ruptured and unruptured aneurysms. Its efficacy can be clinically evaluated by bleeding/rebleeding rate after coiling, and anatomically evaluated by aneurysm occlusion post-procedure and during follow-up. We aimed to analyze immediate post-coiling aneurysm occlusion and associated factors within the Analysis of Recanalization after Endovascular Treatment of intracranial Aneurysm (ARETA) population. METHODS: Between December 2013 and May 2015, 16 neurointerventional departments prospectively enrolled participants treated for ruptured and unruptured aneurysms (ClinicalTrials.gov: NCT01942512). Participant demographics, aneurysm characteristics, and endovascular techniques were recorded. In patients with aneurysms treated by coiling or BAC, immediate post-operative aneurysm occlusion was independently evaluated by a core lab using a 3-grade scale: complete occlusion, neck remnant, and aneurysm remnant. RESULTS: Of 1135 participants (age 53.8±12.8 years, 754 women (66.4%)), 1189 aneurysms were analyzed. Treatment modality was standard coiling in 645/1189 aneurysms (54.2%) and BAC in 544/1189 (45.8%). Immediate post-operative aneurysm occlusion was complete occlusion in 57.8%, neck remnant in 34.4%, and aneurysm remnant in 7.8%. Adequate occlusion (complete occlusion or neck remnant) was significantly more frequent in aneurysms with size <10 mm (93.1% vs 86.3%; OR 1.8, 95% CI 1.1 to 3.2; p=0.02) and in aneurysms with a narrow neck (95.8% vs 89.6%; OR 2.5, 95% CI 1.5 to 4.1; p=0.0004). Patients aged <70 years had significantly more adequate occlusion (92.7% vs 87.2%; OR 1.9, 95% CI 1.1 to 3.4; p=0.04). CONCLUSIONS: Immediately after aneurysm coiling, including BAC, adequate aneurysm occlusion was obtained in 92.2%. Age <70 years, aneurysm size <10 mm, and narrow neck were factors associated with adequate occlusion. TRIAL REGISTRATION NUMBER: NCT01942512, http://www.clinicaltrials.gov.

Delayed thromboembolic events after coiling of unruptured intracranial aneurysms in a prospective cohort of 335 patients.

BACKGROUND: Coiling is the first-line treatment for the management of unruptured intracranial aneurysms (UIAs), but delayed thromboembolic events (TEEs) can occur after such treatment. ARETA (Analysis of Recanalization after Endovascular Treatment of Intracranial Aneurysm) is a prospective multicenter study conducted to analyze aneurysm recanalization. We analyzed delayed TEEs in the UIA subgroup. METHODS: Sixteen neurointerventional departments prospectively enrolled patients treated for ruptured and unruptured aneurysms between December 2013 and May 2015. Participant demographics, aneurysm characteristics, and endovascular techniques were recorded. Data were analyzed from participants with UIA treated by coiling or balloon-assisted coiling. We assessed the rates, timing, management, clinical outcomes, and risk factors for delayed TEEs using univariable and multivariable analyses. RESULTS: The rate of delayed TEEs was 2.4% (95% CI 1.0% to 4.6%) in patients with unruptured aneurysms, with all events occurring in the week following the procedure. In multivariate analysis, two factors were associated with delayed TEEs: autosomal dominant polycystic kidney disease (ADPKD): 20.0% in patients with ADPKD vs 1.9% in patients without ADPKD (OR 27.3 (95% CI 3.9 to 190.2), p=0.0008) and post-procedure aneurysm remnant: 9.4% in patients with post-procedure aneurysm remnant vs 1.6% in patients with adequate occlusion (OR 9.9 (95% CI 1.0 to 51.3), p=0.006). We describe modalities of management as well as clinical outcomes. CONCLUSIONS: Delayed TEE is a relatively rare complication after coiling of UIAs. In this series, all occurred in the week following the initial procedure. Two factors were associated with delayed TEE: ADPKD and aneurysm remnant at procedure completion. CLINICAL TRIAL REGISTRATION: NCT01942512.

De novo arteriovenous shunts after endovascular cure of cerebrospinal macro arteriovenous fistulas. A role for the vasa vasorum?

BACKGROUND AND PURPOSE: Macro-arteriovenous fistulas (MAVFs) are arteriovenous shunts draining into a giant venous ectasia. They can be treated by surgery or embolisation. Angiographic controls are usually performed rapidly after treatment in order to prove the cure of the lesion but no long term angiographies are generally scheduled. We wanted to control the stabilities of such lesions at follow-up. METHOD: Clinical history and imaging of ninety-five patients with high flow shunts draining into venous ectasias (MAVFs, Vein of Galen malformations and dilatations) were reviewed. RESULTS: De novo arteriovenous shunts related to angiogenesis involving vasa vasorum developed in three patients with MAVFs at various intervals. Genetic underlying conditions as HHT or RASA 1 mutations were suspected in each patient. CONCLUSIONS: Neo-angiogenesis can occur after cure of MAVFs. Long term imaging follow-ups should be considered as the natural history of such recurrent shunts is currently unknown.

Endovascular management of torcular dural sinus malformations in children: the role of straight sinus occlusion.

BACKGROUND: Torcular dural sinus malformations (tDSMs) with arteriovenous shunts are rare congenital intracranial vascular malformations that carry a high rate of neurologic impairment and death in the neonatal, infant and young pediatric population. Their impact on brain venous drainage, especially the deep venous system, is one of the key factors in the clinical prognosis and natural history of the disease. We describe our therapeutic strategy for tDSMs, disconnecting the reflux into the deep venous system by performing an endovascular straight sinus occlusion. METHODS: Among all children with dural sinus malformations seen between 2002 and 2020, we retrospectively reviewed those with tDSM in whom straight sinus occlusion had been performed. RESULTS: Our databank included nine patients with tDSM that were embolized. Mean age at the clinical onset was 8.9±9.6 months (min-max=0-31). Five patients presented a significant reflux in the straight sinus on digital subtraction angiography. Those patients were initially clinically worse (mean modified Rankin Scale (mRS) 3.8) than those who did not present with reflux (mean mRS 2.25), this reflux being responsible for intraventricular hemorrhage in three patients. The reflux was suppressed by transarterial embolization in one patient and by transvenous straight sinus occlusion in four patients. Staged endovascular treatment resulted in a complete cure in six patients without complications, and clinical improvement in all patients. CONCLUSION: Straight sinus occlusion is a feasible technique that needs to be considered in the treatment strategy for tDSM with deep venous reflux in order to avoid or minimize brain damage.

Rebleeding and bleeding in the year following intracranial aneurysm coiling: analysis of a large prospective multicenter cohort of 1140 patients-Analysis of Recanalization after Endovascular Treatment of Intracranial Aneurysm (ARETA) Study.

BACKGROUND: Endovascular treatment is the first line therapy for the management of ruptured and unruptured intracranial aneurysms, but delayed aneurysm rupture leading to bleeding/rebleeding can occur subsequently. ARETA (Analysis of Recanalization after Endovascular Treatment of intracranial Aneurysm) is a prospective, multicenter study conducted to analyze aneurysm recanalization. We analyzed delayed bleeding and rebleeding in this large cohort. METHODS: 16 neurointerventional departments prospectively enrolled patients treated for ruptured and unruptured aneurysms between December 2013 and May 2015 (ClinicalTrials.gov: NCT01942512). Participant demographics, aneurysm characteristics and endovascular techniques were recorded. Data were analyzed from participants with ruptured or unruptured aneurysms treated by coiling or balloon-assisted coiling. Rates of bleeding and rebleeding were analyzed and associated factors were studied using univariable and multivariable analyses. RESULTS: The bleeding rate was 0.0% in patients with unruptured aneurysms and 1.0% (95% CI 0.3% to 1.7%) in patients with ruptured aneurysms. In multivariate analysis, two factors were associated with rebleeding occurrence: incomplete aneurysm occlusion after initial treatment (2.0% in incomplete aneurysm occlusion vs 0.2% in complete aneurysm occlusion, OR 10.2, 95% CI 1.2 to 83.3; p=0.03) and dome-to-neck ratio (1.5±0.5 with rebleeding vs 2.2±0.9 without rebleeding, OR 0.2, 95% CI 0.04 to 0.8; p=0.03). Modalities of management of aneurysm rebleeding as well as clinical outcomes are described. CONCLUSIONS: Aneurysm coiling affords good protection against bleeding (for unruptured aneurysms) and rebleeding (for ruptured aneurysms) at 1 year with rates of 0.0% and 1.0%, respectively. Aneurysm occlusion and dome-to-neck ratio are the two factors that appear to play a role in the occurrence of rebleeding.

Combined use of contact aspiration and the stent retriever technique versus stent retriever alone for recanalization in acute cerebral infarction: the randomized ASTER 2 study protocol.

RATIONALE: Mechanical thrombectomy (MT) using a stent retriever (SR) device is currently the recommended treatment in ischemic stroke due to anterior circulation large vessel occlusion. Combining contact aspiration (CA) with SR is a promising new treatment, although it was not found to be superior to SR alone as first-line treatment for achieving successful reperfusion. AIM: To determine whether endovascular treatment combining first-line use of CA and SR is more efficient than SR alone. METHODS: The ASTER 2 clinical trial is a prospective, randomized, multicenter, open-label trial with a blinded endpoint. We included patients admitted with suspected anterior circulation ischemic stroke secondary to large vessel occlusion <8 hours from symptom onset. They were randomly allocated in a 1:1 ratio to one of two treatment groups (combined CA and SR or SR alone). In the case of failure of the assigned technique after three attempts, other adjunctive techniques were applied. STUDY OUTCOME: The primary outcome is the rate of successful/complete reperfusion (modified Thrombolysis In Cerebral Infarction (mTICI) score 2c/3) after the entire endovascular procedure. Secondary outcomes include reperfusion rates after the assigned first-line intervention alone and at the end of the procedure, procedural times, change in NIH Stroke Scale score at 24 hours, intracerebral hemorrhage at 24 hours, procedure-related serious adverse events, the modified Rankin Scale score, and all-cause mortality at 90 days and 1 year. The cost effectiveness of the two procedures will also be analyzed. DISCUSSION: This is the first head-to-head randomized trial to directly compare the efficacy of the combined use of CA and SR versus SR alone. This prospective trial aims to demonstrate the synergistic effects of CA and SR devices in first-line endovascular treatment.

Multimodal Management of a Ruptured Right Medial Parietal Arteriovenous Malformation: 2-Dimensional Operative Video.

Arteriovenous malformations (AVM) of the medial surface of the cerebral hemispheres are challenging because of the limited access to the interhemispheric fissure, the presence of the bridging veins, and the difficult control of arterial feeders and deep venous drainage. We present a 20-yr-old patient with a grade 3 Spetzler Martin ruptured right medial parietal AVM revealed by headaches, left hemiparesis, and ataxia. We highlight the importance of a detailed and selective study of AVM angioarchitecture with new sequences as XperCT (Philips Medical) viewing which permits a better understanding of the anatomy and pathology and a better therapeutical planning. Selective embolization of arterial feeders with Glubran2 (GEM) allows a better control of selected sectors of the AVM which may be difficult to access at surgery. This type of planning is especially important when the AVM is in close relationship with an important sulcus, as was the case of this patient whose AVM was adherent to the postcentral sulcus. We present the surgical nuances concerning patient positioning, craniotomy, AVM dissection, and resection. Early identification and interruption of the main arterial feeders facilitate further dissection. We discuss the timing of deep venous drainage interruption in AVMs with mixed superficial and deep venous drainage. The postoperative course was favorable and the postoperative angiogram showed complete resection of the AVM. At the last follow-up, the patient had only slight left ataxia. Multimodal management with planned selective embolization may facilitate microsurgical resection of AVMs. Given the retrospective nature of this report, informed consent was not required.

Mutations in Chromatin Modifier and Ephrin Signaling Genes in Vein of Galen Malformation.

Normal vascular development includes the formation and specification of arteries, veins, and intervening capillaries. Vein of Galen malformations (VOGMs) are among the most common and severe neonatal brain arterio-venous malformations, shunting arterial blood into the brain's deep venous system through aberrant direct connections. Exome sequencing of 55 VOGM probands, including 52 parent-offspring trios, revealed enrichment of rare damaging de novo mutations in chromatin modifier genes that play essential roles in brain and vascular development. Other VOGM probands harbored rare inherited damaging mutations in Ephrin signaling genes, including a genome-wide significant mutation burden in EPHB4. Inherited mutations showed incomplete penetrance and variable expressivity, with mutation carriers often exhibiting cutaneous vascular abnormalities, suggesting a two-hit mechanism. The identified mutations collectively account for ∼30% of studied VOGM cases. These findings provide insight into disease biology and may have clinical implications for risk assessment.

Human genetics and molecular mechanisms of vein of Galen malformation.

Vein of Galen malformations (VOGMs) are rare developmental cerebrovascular lesions characterized by fistulas between the choroidal circulation and the median prosencephalic vein. Although the treatment of VOGMs has greatly benefited from advances in endovascular therapy, including technical innovation in interventional neuroradiology, many patients are recalcitrant to procedural intervention or lack accessibility to specialized care centers, highlighting the need for improved screening, diagnostics, and therapeutics. A fundamental obstacle to identifying novel targets is the limited understanding of VOGM molecular pathophysiology, including its human genetics, and the lack of an adequate VOGM animal model. Herein, the known human mutations associated with VOGMs are reviewed to provide a framework for future gene discovery. Gene mutations have been identified in 2 Mendelian syndromes of which VOGM is an infrequent but associated phenotype: capillary malformation-arteriovenous malformation syndrome ( RASA1) and hereditary hemorrhagic telangiectasia ( ENG and ACVRL1). However, these mutations probably represent only a small fraction of all VOGM cases. Traditional genetic approaches have been limited in their ability to identify additional causative genes for VOGM because kindreds are rare, limited in patient number, and/or seem to have sporadic inheritance patterns, attributable in part to incomplete penetrance and phenotypic variability. The authors hypothesize that the apparent sporadic occurrence of VOGM may frequently be attributable to de novo mutation or incomplete penetrance of rare transmitted variants. Collaboration among treating physicians, patients' families, and investigators using next-generation sequencing could lead to the discovery of novel genes for VOGM. This could improve the understanding of normal vascular biology, elucidate the pathogenesis of VOGM and possibly other more common arteriovenous malformation subtypes, and pave the way for advances in the diagnosis and treatment of patients with VOGM.

Concomitant conus medullaris arteriovenous shunts and sacral dural arteriovenous fistulas: pathophysiological links related to the venous drainage of the lesions in a series of five cases.

BACKGROUND: Spinal cord arteriovenous shunts (scAVSs) are a group of lesions located in the spinal cord itself or in the surrounding structures. The most common scAVSs are spinal dural arteriovenous fistulas (sDAVFs), which are acquired lesions. The pathogenesis of sDAVFs involves thrombosis and venous hypertension as trigger factors. Intradural scAVSs such as spinal cord arteriovenous nidus type malformations (AVMs) and pial arteriovenous fistulas are less common than sDAVFs and are considered to have a so-called 'congenital' origin. The association between different concomitant scAVSs is very rare and the association of sDAVFs with intradural scAVSs has been described in only a few case reports. METHODS: We describe a case series of five patients presenting with a conus medullaris AVS associated with a lower lumbar or sacral DAVF. RESULTS: Three of our patients were <30 years old at presentation. In four of these five cases the intradural scAVS drained caudally, engorging the epidural plexus in the same location as the sDAVF. In only one case, who presented with thrombosis of the drainage of the main compartment of a conus medullaris pial AVF, was the location of the DAVF opposite to the location of the residual drainage. CONCLUSION: We discuss the pathophysiological link between scAVS and sDAVF on the basis of the rarity of the DAVF, the uncommon association between scAVS and sDAVF, the presence of sDAVF in young patients, and the venous hypertension created by the venous drainage towards the sacral area responsible for angiogenesis creating the dural shunt.

Endovascular treatment of posterior condylar canal dural arteriovenous fistula.

Posterior condylar canal dural arteriovenous fistulas (PCC DAVFs) are rare lesions that may present with pulse-synchronous bruit. In cases with venous reflux there is a risk of haemorrhage or even dementia. Diagnosis and endovascular treatment require a profound knowledge of the vascular anatomy of the craniocervical junction and comprehensive neurovascular imaging. We describe the clinical presentation, angiographic imaging and endovascular treatment of a PCC DAVF in a female patient with pulse-synchronous bruit as the presenting symptom. The fistula drained almost exclusively into the sigmoid sinus and internal jugular vein. There was no intracranial reflux. The PCC DAVF was treated with transvenous coil occlusion of the fistulous pouch in the condylar canal. Symptoms resolved immediately after intervention and the patient recovered quickly without any neurological deficits. MR angiography confirmed occlusion of the DAVF. The dural sinus was patent with normal blood flow.

A spectrum of intracranial vascular high-flow arteriovenous shunts in RASA1 mutations.

PURPOSE AND BACKGROUND: We describe three paediatric cases with different intracranial fast-flow shunts presenting early in life, all with capillary malformation-arteriovenous malformation syndrome and RASA1 verified mutations. Intracranial arteriovenous fast-flow shunts are rare vascular malformations typically presenting early in life and have been associated with cutaneous capillary malformations, characterized as capillary malformation-arteriovenous malformation syndrome. Heterozygous RASA1 gene mutations have been found to be disease causing with high penetrance for the typical cutaneous findings, but only some individuals with the syndrome have intracranial lesions. CASES: One infant presented with a vein of Galen malformation responsible for hydrodynamic disorders, one neonate suffered from severe cardiac insufficiency related to a superior sagittal sinus dural malformation with high-flow fistulas, and one baby was treated at infant age of a choroidal arteriovenous fistula discovered antenatally. RESULTS AND CONCLUSIONS: We report the follow-up of these three cases with RASA1 gene mutation and comment on the possible role of evaluation for vascular lesions and capillary malformation-arteriovenous malformation syndrome in patients and their families, with intracranial fast-flow shunts.

Imaging diagnosis and the role of endovascular embolization treatment for vascular intraspinal tumors.

Intraspinal tumors comprise a large spectrum of neoplasms, including hemangioblastomas, paragangliomas, and meningiomas. These tumors have several common characteristic imaging features, such as highly vascular mass appearance in angiography, hypointense rim and serpentine flow voids in MRI, and intense enhancement after intravenous contrast administration. Due to their rich vascularity, these tumors represent a special challenge for surgical treatment. More recently, the surgical treatment of intraspinal vascular tumors has benefited from the combination of endovascular techniques used to better delineate these lesions and to promote preoperative reduction of volume and tissue blood flow. Endovascular embolization has been proven to be a safe procedure that facilitates the resection of these tumors; hence, it has been proposed as part of the standard of care in their management.

Therapeutic management of intracranial dural arteriovenous shunts with leptomeningeal venous drainage: report of 53 consecutive patients with emphasis on transarterial embolization with acrylic glue.

OBJECT: There is a strong correlation between the venous drainage pattern of intracranial dural arteriovenous shunts (ICDAVSs) and the affected patients' clinical presentation. The ICDAVSs that have cortical venous reflux (CVR) (retrograde leptomeningeal drainage: Borden Type 2 and 3 lesions) are very aggressive and have a poor natural history. Although the necessity of treatment remains debatable in ICDAVSs that drain exclusively into a sinus (Borden Type 1), lesions with CVR must be treated because of the negative effects of the retrograde venous drainage. Surgery, radiosurgery, and embolization have been proposed for management of these lesions, but endovascular therapy is considered the most appropriate therapeutic strategy in ICDAVSs. New embolic materials, such as Onyx, have been recently developed and are considered to represent a kind of "gold standard" for embolization of these lesions. The purpose of this study is to emphasize the importance of transarterial embolization using acrylic glue in the therapeutic management of ICDAVSs with CVR, and to compare the results the authors obtained using this treatment with those reported in the literature for Onyx treatment of the same type of dural shunts. METHODS: The clinical and radiological records of 53 consecutive patients suffering from ICDAVSs with CVR (Borden Types 2 or 3) were reviewed. All cases were managed with the same angiographic and therapeutic protocol. Localization of the lesions, their clinical symptoms, their angioarchitecture, their therapeutic management, and the results were analyzed. RESULTS: Fourteen ICDAVSs were located at the superior sagittal sinus and/or convexity veins, 13 at the transverse and sigmoid sinuses, 10 at the tentorium, 5 in the anterior cranial fossa, 4 at the foramen magnum, 3 at the torcula, 2 at the straight sinus, and 1 at the vein of Galen. One patient presented with an infantile form of ICDAVS with multiple shunts. Hemorrhage had occurred in 36% of cases. Forty-three patients underwent transarterial embolization (42 with acrylic glue). Complete closure of the fistula was obtained in 34 patients. Suppression of the CVR with partial occlusion of the main shunt was achieved in all other cases. No mortality or permanent morbidity was observed in this series. CONCLUSIONS: Intracranial dural arteriovenous shunts can be safely managed by transarterial embolization, which can be considered in most instances as an effective first-intention treatment. Acrylic glue still allows a cheap, fast, and effective treatment with high rates of cures that compare favorably to those obtained with new embolic materials.

Management strategies for anterior cranial fossa (ethmoidal) dural arteriovenous fistulas with an emphasis on endovascular treatment.

OBJECT: Dural arteriovenous fistulas (DAVFs) of the anterior cranial fossa are rare lesions that can cause intracranial hemorrhage. Authors of previous reports mostly have described open surgical treatment for this fistula type. The authors' purpose in the present study was to describe their experience with anterior cranial fossa DAVFs, including their endovascular treatment. METHODS: All patients with anterior cranial fossa DAVFs diagnosed and treated in 3 separate institutions during the last 23 years were retrospectively identified. Clinical charts, imaging studies, and procedural notes were evaluated. RESULTS: Twenty-four patients (22 males and 2 females), ranging in age from 3 to 77 years, harbored 24 DAVFs in the anterior cranial fossa. Eleven patients were primarily treated with surgical disconnection and 2 with radiosurgery. Eleven patients were treated endovascularly; 7 of these patients (63.6%) were cured. In 4 cases of failed embolization, final disconnection was achieved through surgery. In fact, surgery was effective in disconnecting the fistula in 100% of cases. All endovascular procedures consisted of transarterial injections of diluted glue (N-butyl cyanoacrylate [NBCA]), and there were no complications. Brain edema developed around the venous pouch and confusion was apparent after venous disconnection in 1 surgically treated patient. No patient suffered a hemorrhage during the follow-up period. CONCLUSIONS: Disconnection of an anterior cranial fossa DAVF by using transarterial catheterization through the ophthalmic artery and subsequent injection of NBCA is possible with a reasonable success rate and low risk for complications. In patients with good vascular access this procedure could be the treatment of choice, to be followed by open surgery in cases of embolization failure.