Dural Arteriovenous Fistulas of the Foramen Magnum Region: Clinical Features and Angioarchitectural Phenotypes.

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.

Recent Administration of Iodinated Contrast Renders Core Infarct Estimation Inaccurate Using RAPID Software.

BACKGROUND AND PURPOSE: Automated 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 METHODS: We 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 <30% as a surrogate for irreversible core infarct. Patients were dichotomized on the basis of receiving recent IV iodinated contrast (<8 hours before CTP) for a separate imaging study. RESULTS: The recent IV contrast and contrast-naïve cohorts comprised 23 and 15 patients, respectively. Multivariate linear regression analysis demonstrated that recent IV contrast administration was independently associated with a decrease in the RAPID core infarct estimate (proportional increase = 0.34; 95% CI, 0.12-0.96; P = .04). CONCLUSIONS: Patients who received IV iodinated contrast in proximity (<8 hours) to CTA/CTP as part of a separate imaging study had a much higher likelihood of core infarct underestimation with RAPID compared with contrast-naïve patients. Over-reliance on RAPID postprocessing for treatment disposition of patients with extended window emergent large-vessel occlusion should be avoided, particularly with recent IV contrast administration.

Interrater Reliability in the Measurement of Flow Characteristics on Color-Coded Quantitative DSA of Brain AVMs.

BACKGROUND AND PURPOSE: Hemodynamic features of brain AVMs may portend increased hemorrhage risk. Previous studies have suggested that MTT is shorter in ruptured AVMs as assessed on quantitative color-coded parametric DSA. This study assesses the interrater reliability of MTT measurements obtained using quantitative color-coded DSA. MATERIALS AND METHODS: Thirty-five color-coded parametric DSA images of 34 brain AVMs were analyzed by 4 neuroradiologists with experience in interventional neuroradiology. Hemodynamic features assessed included MTT of the AVM and TTP of the dominant feeding artery and draining vein. Agreement among the 4 raters was assessed using the intraclass correlation coefficient. RESULTS: The interrater reliability among the 4 raters was poor (intraclass correlation coefficient = 0.218; 95% CI, 0.062-0.414; P value = .002) as it related to MTT assessment. When the analysis was limited to cases in which the raters selected the same image to analyze and selected the same primary feeding artery and the same primary draining vein, interrater reliability improved to fair (intraclass correlation coefficient = 0.564; 95% CI, 0.367-0.717; P < .001). CONCLUSIONS: Interrater reliability in deriving color-coded parametric DSA measurements such as MTT is poor so minor differences among raters may result in a large variance in MTT and TTP results, partly due to the sensitivity and 2D nature of the technique. Reliability can be improved by defining a standard projection, feeding artery, and draining vein for analysis.

Impact of Aortic Arch Anatomy on Technical Performance and Clinical Outcomes in Patients with Acute Ischemic Stroke.

BACKGROUND AND PURPOSE: Arterial access is a technical consideration of mechanical thrombectomy that may affect procedural time, but few studies exist detailing the relationship of anatomy to procedural times and patient outcomes. We sought to investigate the respective impact of aortic arch and carotid artery anatomy on endovascular procedural times in patients with large-vessel occlusion. MATERIALS AND METHODS: We retrospectively reviewed imaging and medical records of 207 patients from 2 academic institutions who underwent mechanical thrombectomy for anterior circulation large-vessel occlusion from January 2015 to July 2018. Preintervention CTAs were assessed to measure features of the aortic arch and ipsilateral great vessel anatomy. These included the cranial-to-caudal distance from the origin of the innominate artery to the top of the aortic arch and the takeoff angle of the respective great vessel from the arch. mRS scores were calculated from rehabilitation and other outpatient documentation. We performed bootstrap, stepwise regressions to model groin puncture to reperfusion time and binary mRS outcomes (good outcome, mRS ≤ 2). RESULTS: From our linear regression for groin puncture to reperfusion time, we found a significant association of the great vessel takeoff angle (P = .002) and caudal distance from the origin of the innominate artery to the top of the aortic arch (P = .05). Regression analysis for the binary mRS revealed a significant association with groin puncture to reperfusion time (P < .001). CONCLUSIONS: These results demonstrate that patients with larger takeoff angles and extreme aortic arches have an association with longer procedural times as approached from transfemoral access routes.

Revisiting classic MRI findings of venous malformations: Changes in protocols may lead to potential misdiagnosis.

Introduction Magnetic resonance imaging (MRI) is most sensitive and specific for characterizing venous malformations (VMs). VMs typically demonstrate central enhancement on delayed-contrast imaging. Fluid-fluid levels (FFLs) are uncommon in VMs and common in lymphatic malformations (LMs). Technology has advanced since the initial description of these findings. Rates of detection of these MRI findings in VMs may have changed as MRI technology and techniques have evolved. Methods and methods A prospectively maintained database from a multidisciplinary vascular anomalies clinic was reviewed to identify patients with final diagnosis of VM or LM. Patients with reviewable contrast-enhanced MRIs were selected, reviewing the oldest MRI studies in the database against the newest MRI studies to identify equal numbers of patients from the temporal extremes. Imaging was reviewed to assess for presence of FFLs. Enhancement was quantified by measuring signal in the same location of the lesion both on pre- and postcontrast sequences Results Forty patients were identified for analysis. Twenty studies with sufficient archived imaging for review were performed between 1995 and 2006; 20 such studies were performed between 2011 and 2012. The new imaging cohort had higher rates of FFL visualization ( p = 0.001). Correlation was found between time to imaging following contrast and degree of enhancement ( p < 0.001). Inverse correlation was found between scan date and time to contrast ( p = 0.001) and scan date and enhancement ( p = 0.021). Conclusion FFLs should no longer be considered exclusionary for the diagnosis of VMs. Timing following contrast administration should be maximized to increase degree of enhancement to confirm the diagnosis of VMs.

Pial Artery Supply as an Anatomic Risk Factor for Ischemic Stroke in the Treatment of Intracranial Dural Arteriovenous Fistulas.

BACKGROUND AND PURPOSE: Although intracranial dural arteriovenous fistulas are principally supplied by dural branches of the external carotid, internal carotid, and vertebral arteries, they can also be fed by pial arteries that supply the brain. We sought to determine the frequency of neurologic deficits following treatment of intracranial dural arteriovenous fistulas with and without pial artery supply. MATERIALS AND METHODS: One hundred twenty-two consecutive patients who underwent treatment for intracranial dural arteriovenous fistulas at our hospital from 2008 to 2015 were retrospectively reviewed. Patient data were examined for posttreatment neurologic deficits; patients with such deficits were evaluated for imaging evidence of cerebral infarction. Data were analyzed with multivariable logistic regression. RESULTS: Of 122 treated patients, 29 (23.8%) had dural arteriovenous fistulas with pial artery supply and 93 (76.2%) had dural arteriovenous fistulas without pial arterial supply. Of patients with pial artery supply, 4 (13.8%) had posttreatment neurologic deficits, compared with 2 patients (2.2%) without pial artery supply (P = .04). Imaging confirmed that 3 patients with pial artery supply (10.3%) had cerebral infarcts, compared with only 1 patient without pial artery supply (1.1%, P = .03). Increasing patient age was also positively associated with pial supply and treatment-related complications. CONCLUSIONS: Patients with dural arteriovenous fistulas supplied by the pial arteries were more likely to experience posttreatment complications, including ischemic strokes, than patients with no pial artery supply. The approach to dural arteriovenous fistula treatment should be made on a case-by-case basis so that the risk of complications can be minimized.

Effects on vessel measurement accuracy and subsequent occlusion after calcium channel blocker infusion during treatment of cerebral aneurysms with the Pipeline embolization device.

Introduction/Purpose To achieve aneurysm occlusion, flow diverters (FDs) must be accurately sized to maximize coverage over the neck and induce thrombosis. Catheterization for diagnostic angiography can cause vasospasm that may affect vessel measurements. This study evaluates impacts of intra-arterial infusion of a calcium channel blocker (CCB) on angiographic measurements in patients treated with FDs to determine effects on final diameter of the FD and subsequent occlusion. Materials and methods Pre-treatment measurements were recorded for diameter of the distal and proximal landing zones and maximum and minimum diameters between these segments. Post-treatment measurements of the stent following deployment were recorded at these locations. When CCB was infused, post-infusion pre-treatment measurements were recorded. Rates of occlusion were noted for all patients. T-tests were performed to assess for differences in pre- and post-treatment measurements and rates of occlusion between groups with and without CCB infusion. Results Twenty-eight FDs were deployed to treat 25 aneurysms in 24 patients. CCB infusion was performed prior to deployment of 12 (42.9%) devices. No significant difference was noted between groups for pre- and post-treatment measurement changes. Confirmed aneurysm occlusion was more likely to occur in the CCB infusion group (88.9% vs. 36.4%, p = 0.009). Conclusion Optimization of device sizing is important to increase FD density over the aneurysm neck and promote thrombosis. To improve measurement accuracy, CCB infusion can reduce effects of mild vasospasm. Subsequent aneurysm occlusion was more likely to occur following FD treatment when device size selection was based on measurements performed following CCB infusion.

Superselective Intra-Arterial Ethanol Sclerotherapy of Feeding Artery and Nidal Aneurysms in Ruptured Cerebral Arteriovenous Malformations.

In the endovascular treatment of cerebral arteriovenous malformations, ethanol sclerotherapy is seldom used due to safety concerns. However, when limited reflux of an embolic agent is permissible or when there is a long distance to the target, ethanol may be preferable. We reviewed 10 patients with 14 cerebral AVM feeding artery aneurysms or intranidal aneurysms treated with intra-arterial ethanol sclerotherapy at our institution between 2005 and 2014. All patients presented with acute intracranial hemorrhage. Thirteen of 14 aneurysms were treated primarily with 60%-80% ethanol into the feeding artery. Complete target feeding artery and aneurysm occlusion was seen in all cases; 8/13 (62%) were occluded by using ethanol alone. No retreatments or recurrences were seen. One permanent neurologic deficit (1/13, 7.7%) and no deaths occurred. In a subset of ruptured cerebral AVMs, ethanol sclerotherapy of feeding artery aneurysms and intranidal aneurysms can be performed with a high degree of technical success and a low rate of complication.

Lesion stability characteristics outperform degree of stenosis in predicting outcomes following stenting for symptomatic intracranial atherosclerosis.

BACKGROUND: Intracranial atherosclerotic disease (ICAD) causes substantial morbidity and mortality. Treatment decisions have most commonly been driven by the degree of luminal stenosis. This study compares ICAD lesion stability features with percentage stenosis for associations with adverse outcomes following treatment with stents. MATERIALS AND METHODS: Retrospective analysis was performed of prospectively maintained procedure logs. Lesions were classified by symptom type as hypoperfusion, non-hypoperfusion, or indeterminate, and pretreatment asymptomatic intervals were noted. Hypoperfusion lesions and indeterminate or non-hypoperfusion lesions with ≥14 days of asymptomatic interval were classified as stable. Percentage stenosis was calculated and compared against these other symptom features for value in predicting technical complication, ischemic stroke, disability, or death at 90 days and 2 years using univariate and multivariate analysis. RESULTS: 130 lesions were treated in 124 patients. The only statistically significant percent stenosis finding was lesions with 60-99% stenosis were less likely to have technical complications. In univariate analysis, stroke at 2 years was less common with hypoperfusion and stable lesions. In multivariate analysis, only hypoperfusion status was associated with lower stroke rates at 2 years. CONCLUSIONS: Lesion stability features, particularly non-hypoperfusion symptomatology, outperform percentage stenosis in predicting outcomes following treatment of ICAD with stents. Further examination is needed to better classify the natural history of ICAD and more precisely classify lesion stability.

Progressive versus Nonprogressive Intracranial Dural Arteriovenous Fistulas: Characteristics and Outcomes.

BACKGROUND AND PURPOSE: A minority of intracranial dural arteriovenous fistulas progress with time. We sought to determine features that predict progression and define outcomes of patients with progressive dural arteriovenous fistulas. MATERIALS AND METHODS: We performed a retrospective imaging and clinical record review of patients with intracranial dural arteriovenous fistula evaluated at our hospital. RESULTS: Of 579 patients with intracranial dural arteriovenous fistulas, 545 had 1 fistula (mean age, 45 ± 23 years) and 34 (5.9%) had enlarging, de novo, multiple, or recurrent fistulas (mean age, 53 ± 20 years; P = .11). Among these 34 patients, 19 had progressive dural arteriovenous fistulas with de novo fistulas or fistula enlargement with time (mean age, 36 ± 25 years; progressive group) and 15 had multiple or recurrent but nonprogressive fistulas (mean age, 57 ± 13 years; P = .0059, nonprogressive group). Whereas all 6 children had fistula progression, only 13/28 adults (P = .020) progressed. Angioarchitectural correlates to chronically elevated intracranial venous pressures, including venous sinus dilation (41% versus 7%, P = .045) and pseudophlebitic cortical venous pattern (P = .048), were more common in patients with progressive disease than in those without progression. Patients with progressive disease received more treatments than those without progression (median, 5 versus 3; P = .0068), but as a group, they did not demonstrate worse clinical outcomes (median mRS, 1 and 1; P = .39). However, 3 young patients died from intracranial venous hypertension and intracranial hemorrhage related to progression of their fistulas despite extensive endovascular, surgical, and radiosurgical treatments. CONCLUSIONS: Few patients with dural arteriovenous fistulas follow an aggressive, progressive clinical course despite treatment. Younger age at initial presentation and angioarchitectural correlates to venous hypertension may help identify these patients prospectively.

Association between Venous Angioarchitectural Features of Sporadic Brain Arteriovenous Malformations and Intracranial Hemorrhage.

BACKGROUND AND PURPOSE: Intracranial hemorrhage is the most serious outcome for brain arteriovenous malformations. This study examines associations between venous characteristics of these lesions and intracranial hemorrhage. MATERIALS AND METHODS: Statistical analysis was performed on a prospectively maintained data base of brain AVMs evaluated at an academic medical center. DSA, CT, and MR imaging studies were evaluated to classify lesion side, drainage pattern, venous stenosis, number of draining veins, venous ectasia, and venous reflux. Logistic regression analyses were performed to identify the association of these angiographic features with intracranial hemorrhage of any age at initial presentation. RESULTS: Exclusively deep drainage (OR, 3.42; 95% CI, 1.87-6.26; P < .001) and a single draining vein (OR, 1.98; 95% CI, 1.26-3.08; P = .002) were associated with hemorrhage, whereas venous ectasia (OR, 0.52; 95% CI, 0.34-0.78; P = .002) was inversely associated with hemorrhage. CONCLUSIONS: Analysis of venous characteristics of brain AVMs may help determine their prognosis and thereby identify lesions most appropriate for treatment.

Influence of patient age on angioarchitecture of brain arteriovenous malformations.

BACKGROUND AND PURPOSE: The imaging characteristics and modes of presentation of brain AVMs may vary with patient age. Our aim was to determine whether clinical and angioarchitectural features of brain AVMs differ between children and adults. MATERIALS AND METHODS: A prospectively collected institutional data base of all patients diagnosed with brain AVMs since 2001 was queried. Demographic, clinical, and angioarchitecture information was summarized and analyzed with univariable and multivariable models. RESULTS: Results often differed when age was treated as a continuous variable as opposed to dividing subjects into children (18 years or younger; n = 203) versus adults (older than 18 years; n = 630). Children were more likely to present with AVM hemorrhage than adults (59% versus 41%, P < .001). Although AVMs with a larger nidus presented at younger ages (mean of 26.8 years for >6 cm compared with 37.1 years for <3 cm), this feature was not significantly different between children and adults (P = .069). Exclusively deep venous drainage was more common in younger subjects when age was treated continuously (P = .04) or dichotomized (P < .001). Venous ectasia was more common with increasing age (mean, 39.4 years with ectasia compared with 31.1 years without ectasia) and when adults were compared with children (52% versus 35%, P < .001). Patients with feeding artery aneurysms presented at a later average age (44.1 years) than those without such aneurysms (31.6 years); this observation persisted when comparing children with adults (13% versus 29%, P < .001). CONCLUSIONS: Although children with brain AVMs were more likely to come to clinical attention due to hemorrhage than adults, venous ectasia and feeding artery aneurysms were under-represented in children, suggesting that these particular high-risk features take time to develop.

Symptom differences and pretreatment asymptomatic interval affect outcomes of stenting for intracranial atherosclerotic disease.

BACKGROUND AND PURPOSE: Different types of symptomatic intracranial stenosis may respond differently to interventional therapy. We investigated symptomatic and pathophysiologic factors that may influence clinical outcomes of patients with intracranial atherosclerotic disease who were treated with stents. MATERIALS AND METHODS: A retrospective analysis was performed of patients treated with stents for intracranial atherosclerosis at 4 centers. Patient demographics and comorbidities, lesion features, treatment features, and preprocedural and postprocedural functional status were noted. χ(2) univariate and multivariate logistic regression analysis was performed to assess technical results and clinical outcomes. RESULTS: One hundred forty-two lesions in 131 patients were analyzed. Lesions causing hypoperfusion ischemic symptoms were associated with fewer strokes by last contact [χ(2) (1, n = 63) = 5.41, P = .019]. Nonhypoperfusion lesions causing symptoms during the 14 days before treatment had more strokes by last contact [χ(2) (1, n = 136), 4.21, P = .047]. Patients treated with stents designed for intracranial deployment were more likely to have had a stroke by last contact (OR, 4.63; P = .032), and patients treated with percutaneous balloon angioplasty in addition to deployment of a self-expanding stent were less likely to be stroke free at point of last contact (OR, 0.60; P = .034). CONCLUSIONS: More favorable outcomes may occur after stent placement for lesions causing hypoperfusion symptoms and when delaying stent placement 7-14 days after most recent symptoms for lesions suspected to cause embolic disease or perforator ischemia. Angioplasty performed in addition to self-expanding stent deployment may lead to worse outcomes, as may use of self-expanding stents rather than balloon-mounted stents.

Bioactive versus bare platinum coils in the treatment of intracranial aneurysms: the MAPS (Matrix and Platinum Science) trial.

BACKGROUND AND PURPOSE: The ability of polymer-modified coils to promote stable aneurysm occlusion after endovascular treatment is not well-documented. Angiographic aneurysm recurrence is widely used as a surrogate for treatment failure, but studies documenting the correlation of angiographic recurrence with clinical failure are limited. This trial compares the effectiveness of Matrix(2) polyglycolic/polylactic acid biopolymer-modified coils with bare metal coils and correlates the angiographic findings with clinical failure (ie, target aneurysm recurrence), a composite end point that includes any incident of posttreatment aneurysm rupture, retreatment, or unexplained death. MATERIALS AND METHODS: This was a multicenter randomized noninferiority trial with blinded end point adjudication. We enrolled 626 patients, divided between Matrix(2) and bare metal coil groups. The primary outcome was target aneurysm recurrence at 12 ± 3 months. RESULTS: At 455 days, at least 1 target aneurysm recurrence event had occurred in 14.6% of patients treated with bare metal coils and 13.3% of Matrix(2) (P = .76, log-rank test) patients; 92.8% of target aneurysm recurrence events were re-interventions for aneurysms that had not bled after treatment, and 5.8% of target aneurysm recurrence events resulted from hemorrhage or rehemorrhage, with or without retreatment. Symptomatic re-intervention occurred in only 4 (0.6%) patients. At 455 days, 95.8% of patients with unruptured aneurysms and 90.4% of those with ruptured aneurysms were independent (mRS ≤ 2). Target aneurysm recurrence was associated with incomplete initial angiographic aneurysm obliteration, presentation with rupture, and a larger aneurysmal dome and neck size. CONCLUSIONS: Tested Matrix(2) coils were not inferior to bare metal coils. Endovascular coiling of intracranial aneurysms was safe, and the rate of technical success was high. Target aneurysm recurrence is a promising clinical outcome measure that correlates well with established angiographic measurements.

Stent-assisted coiling versus coiling alone in unruptured intracranial aneurysms in the matrix and platinum science trial: safety, efficacy, and mid-term outcomes.

BACKGROUND AND PURPOSE: Stent-assisted coiling may result in less aneurysm recanalization but more complications than coiling alone. We evaluated outcomes of coiling with and without stents in the multicenter Matrix and Platinum Science Trial. MATERIALS AND METHODS: All patients in the Matrix and Platinum Science Trial with unruptured intracranial aneurysms treated per protocol were included. Baseline patient and aneurysm characteristics, procedural details, neurologic outcomes, angiographic outcomes, and safety data were analyzed. RESULTS: Overall, 137 of 361 (38%) patients were treated with a stent. Stent-coiled aneurysms had wider necks (≥4 mm in 62% with stents versus 33% without, P < .0001) and lower dome-to-neck ratios (1.3 versus 1.8, P < .0001). Periprocedural serious adverse events occurred infrequently in those treated with and without stents (6.6% versus 4.5%, P = .39). At 1 year, total significant adverse events, mortality, and worsening of mRS were similar in treatment groups, but ischemic strokes were more common in stent-coiled patients than in coiled patients (8.8% versus 2.2%, P = .005). However, multivariate analysis confirmed that at 2 years after treatment, prior cerebrovascular accident (OR, 4.7; P = .0089) and aneurysm neck width ≥4 mm (OR, 4.5; P = .02) were the only independent predictors of ischemic stroke. Stent use was not an independent predictor of ischemic stroke at 2 years (OR, 1.1; P = .94). Stent use did not predict target aneurysm recurrence at 2 years, but aneurysm dome size ≥10 mm (OR, 9.94; P < .0001) did predict target aneurysm recurrence. CONCLUSIONS: Stent-coiling had similar outcomes as coiling despite stented aneurysms having more difficult morphology than coiled aneurysms. Increased ischemic events in stent-coiled aneurysms were attributable to baseline risk factors and aneurysm morphology.

Pediatric intracranial nongalenic pial arteriovenous fistulas: clinical features, angioarchitecture, and outcomes.

BACKGROUND AND PURPOSE: NGAVFs are rare vascular malformations usually presenting in infancy or childhood. We sought to identify clinical and angiographic predictors of clinical outcome for these lesions. MATERIALS AND METHODS: Retrospective review of a neurointerventional data base identified 386 pediatric patients with intracranial AVFs and AVMs, from which a cohort of 25 patients with NGAVF were selected for medical record and imaging analysis. RESULTS: NGAVFs constituted 7.3% of pediatric intracranial vascular lesions with a nondural arteriovenous shunt. Seven of 8 patients who presented in the first month of life had CHF and harbored large, complex fistulas with multiple sites of arteriovenous shunting. Single-hole fistulas predominated later in childhood and more frequently presented with seizures, hemorrhage, or focal neurologic deficits. More treatment procedures were performed in subjects presenting at ≤ 2 years of age compared with older children (median = 3 versus 2, P = .041), and in those harboring a multi-hole fistula versus those with a single-hole fistula (median = 3 versus 2, P = .003). Eighteen patients (72%) had complete posttreatment elimination of NGAVF shunting. Compared with patients presenting at >2 years of age, patients presenting in the first 2 years of life were more likely to have a multi-hole fistula (100% versus 25%, P = .0001) and to have a poor clinical outcome (54% versus 0%, P = .0052), defined as a pediatric mRS of ≥ 3. CONCLUSIONS: The morbidity of NGAVF appears higher than previously reported despite a somewhat higher rate of angiographic cure. Poor clinical outcome occurred primarily in patients with multi-hole NGAVFs presenting at ≤ 2 years of age.

Pediatric intracranial aneurysms: new and enlarging aneurysms after index aneurysm treatment or observation.

BACKGROUND AND PURPOSE: Children with brain aneurysms may be at higher risk than adults to develop new or enlarging aneurysms in a relatively short time. We sought to identify comorbidities and angiographic features in children that predict new aneurysm formation or enlargement of untreated aneurysms. MATERIALS AND METHODS: Retrospective analysis of the University of California-San Francisco Pediatric Aneurysm Cohort data base including medical records and imaging studies was performed. RESULTS: Of 83 patients harboring 114 intracranial aneurysms not associated with brain arteriovenous malformations or intracranial arteriovenous fistulas, 9 (8.4%) developed new or enlarging brain aneurysms an average of 4.2 years after initial presentation. Comorbidities that may be related to aneurysm formation were significantly higher in patients who developed new aneurysms (89%) as opposed to patients who did not develop new or enlarging aneurysms (41%; RR, 9.5; 95% CI, 1.9%-48%; P = .0099). Patients with multiple aneurysms at initial presentation were more likely than patients with a single aneurysm at presentation to develop a new or enlarging aneurysm (RR, 6.2; 95% CI, 2.1%-185; P = .0058). Patients who initially presented with at least 1 fusiform aneurysm were more likely to develop a new or enlarging aneurysm than patients who did not present with a fusiform aneurysm (RR, 22; 95% CI, 3.6%-68%; P = .00050). Index aneurysm treatment with parent artery occlusion also was associated with higher risk of new aneurysm formation (RR, 4.2; 95% CI, 1.3%-13%; P = .024). New aneurysms did not necessarily arise near index aneurysms. The only fatality in the series was due to subarachnoid hemorrhage from a new posterior circulation aneurysm arising 20 months after index anterior circulation aneurysm treatment in an immunosuppressed patient. CONCLUSIONS: Patients who presented with a fusiform aneurysm had a significantly greater incidence of developing a new aneurysm or enlargement of an index aneurysm than did those who presented with a saccular aneurysm. In our patient cohort, 8 of the 9 children who eventually developed new or enlarging brain aneurysms initially presented with fusiform aneurysm morphology. Other comorbidities or multiple aneurysms were also common in these patients at initial presentation.

Intraosseous CSF fistula in a patient with Gorham disease resulting in intracranial hypotension.

A 7-year-old girl with a history of headaches and Gorham disease was surgically treated in infancy for Chiari I malformation. Subsequent investigation revealed that her cerebellar tonsillar ectopia was due to a long-standing spinal CSF-lymphatic fistula causing intracranial hypotension. Percutaneous fistula closure was performed several times, resulting in transient symptomatic improvement.

MR imaging of partially thrombosed cerebral aneurysms: characteristics and evolution.

BACKGROUND AND PURPOSE: A comprehensive evaluation of aneurysmal morphometry requires appreciation of both the vascular lumen and the intraluminal thrombus. MR imaging methods can both evaluate the lumen and directly image the vessel wall. We investigated the ability of T1-weighted, T2-weighted, and steady-state MR imaging techniques to delineate thrombus morphology and reveal changes with time. MATERIALS AND METHODS: Nine patients with fusiform basilar or intracranial vertebral artery aneurysms that contained intraluminal thrombus were studied with MR imaging. All patients underwent at least 2 imaging sessions, which were separated by 4-22 months. Analysis of signal intensity to determine the mean signal intensity from thrombus, blood, CSF, and brain in matched regions was performed. Aneurysm maximal diameter and cross-sectional area were determined with and without thrombus. RESULTS: Thrombus was identified on all image sequences, and its general appearance was consistent between imaging sessions. Thrombus produced the highest and most consistent signal intensities with T1-weighted and steady-state techniques, though the latter showed superior contrast between luminal blood and thrombus. Heterogeneity within clot was evident in 4/9 of patients, with peripheral hyperintensity being a common feature. CONCLUSIONS: Steady-state imaging was found to be superior to T1- and T2-weighted imaging for delineating and characterizing intraluminal thrombus within aneurysms. The imaging characteristics of intraluminal thrombus proved to be very consistent for long periods. Assessment of overall aneurysm size, including thrombosed portions, permits more accurate evaluation of aneurysm growth and concomitantly may permit more informed clinical decision-making with regard to the timing and need for aneurysm treatment.