CLinical Assessment of WEB device in Ruptured aneurYSms (CLARYS): 12-month angiographic results of a multicenter study.

BACKGROUND: The CLinical Assessment of WEB device in Ruptured aneurYSms (CLARYS) study has shown that the endovascular treatment of ruptured bifurcation aneurysms with the Woven EndoBridge (WEB) is safe and effective and provides protection against rebleeding at 1 month and 1 year. The 12-month angiographic follow-up is an important endpoint of the study. METHODS: The CLARYS study is a prospective multicenter study conducted in 13 European centers. The study enrolled 60 patients with 60 ruptured aneurysms of the anterior and posterior circulation. The study was conducted with an independent assessment of safety outcomes and imaging. RESULTS: Sixty patients with 60 ruptured bifurcation aneurysms to be treated with the WEB were included. Fifty-three aneurysms (88.3%) had a broad base with a dome to neck ratio <2 (mean 1.6). Of these, 46 patients were evaluated by an independent core laboratory with follow-up imaging performed at 12 months or before eventual retreatment. At 1 year, 19/46 aneurysms (41.3%) were completely occluded (Raymond-Roy grade I), 21/46 (45.7%) had a residual neck and 6/46 (13.0%) had residual aneurysm filling. Adequate occlusion was reported in 40/46 (87%) aneurysms. Six patients underwent target aneurysm retreatment. CONCLUSIONS: The CLARYS study has previously shown that the use of the WEB in the endovascular treatment of ruptured bifurcation aneurysms provides effective protection against rebleeding with a good safety profile. The angiographic occlusion rates at 1 year reported here are comparable to those already seen in previous multicenter studies which primarily included unruptured aneurysms.

Determinants of cerebral aneurysm occlusion after embolization with the WEB device: a single-institution series of 215 cases with angiographic follow-up.

BACKGROUND: Woven EndoBridge (WEB) devices are becoming a reliable option for the treatment of wide-neck bifurcation aneurysms, but clear predictive factors are still missing to understand the one in five aneurysm remnant rate. OBJECTIVE: To evaluate occlusion outcomes after WEB treatment to identify potential determinants of aneurysm occlusion. METHODS: A single-center database with consecutive aneurysms treated with WEB between July 2012 and October 2021 was reviewed for potential determinants of aneurysm adequate occlusion (defined as a Bicêtre Occlusion Scale Score (BOSS) of 0, 0', 1 or 2), through univariate and multivariable analysis. Patients without angiographic follow-up were excluded. RESULTS: 215 of 247 individual aneurysms were included in the final analysis, of which 59 (27%) were ruptured. Mean age of patients was 56 years (range 23-90 years) and 65% were female. Mean angiographic follow-up was at 18 months (range 3-97 months). Adequate and complete occlusion were achieved in 171/215 (79.5%) and 135/215 (62.8%) of cases, respectively. Aneurysm irregular shape (aOR=0.42, 95% CI 0.20 to 0.88; p=0.02), aneurysm height (aOR=0.79, 95% CI 0.66 to 0.94; p<0.01), and WEB shape modification (aOR=0.98, 95% CI 0.97 to 1.00; p=0.02) were all independent predictors of aneurysm recurrence, whereas the WEB oversizing ratio (WEB width/aneurysm mean width) (aOR=16.4, 95% CI 1.4 to 266.7; p=0.04) was an independent predictor of adequate occlusion. CONCLUSION: In this study we demonstrated that a width oversizing strategy of the WEB device was an independent predictor of aneurysm angiographic occlusion. Conversely, aneurysm height, irregular aneurysm, and WEB shape modification were all independent determinants of angiographic aneurysm remnant. These results may help to select aneurysms suitable for the WEB device and WEB sizing.

Management of aneurysmal recurrence after Woven EndoBridge (WEB) treatment.

BACKGROUND: Around 10% of Woven EndoBridge device (WEB)-treated intracranial aneurysms will need retreatment, and it is generally believed to be more challenging than retreatment after an initial coiling. We aim to report retreatment strategies and outcomes after initial WEB embolizations. METHODS: Databases from four treatment centers, containing consecutive aneurysms treated with a WEB between 2013 and 2022, were reviewed. Demographics, aneurysm characteristics, retreatment strategies and outcomes were collected and analyzed. RESULTS: From a 756 WEB database, 57 aneurysms were included. The global retreatment rate was 7.5% (95% CI 5.6% to 9.4%). The retreatment rate was significantly higher in the ruptured compared with the unruptured population (13% vs 3.9%, respectively, P<0.0001). Aneurysms were retreated on average 21.2 months after the initial WEB treatment (range 4.8-70 months). Surgery was performed in 11% and endovascular treatment in 89% of cases, consisting of flow diversion (48%), stent-assisted coiling (30%), coiling (12%), and second WEB placement (10%). Imaging follow-up was available in 88% of all WEB retreatments (50/57) (average 17 months, 49% digital subtraction angiography), demonstrating complete occlusion in 56% and 'adequate' occlusion in 88%. Morbidity was 5.3% (95% CI 0% to 12.0%) and mortality 0%. No patient experienced rebleeding during the follow-up period. CONCLUSION: The retreatment rate after an initial WEB treatment seems to compare favorably with that of coiling. Endovascular treatment of recurrence following WEB implantation is feasible in most situations; it generally requires the use of a stent and leads to a high rate of satisfactory occlusion.

Recanalization Treatments for Pediatric Acute Ischemic Stroke in France.

Importance: There is to date limited evidence that revascularization strategies are associated with improved functional outcome in children with acute ischemic stroke (AIS). Objectives: To report clinical outcomes and provide estimates of revascularization strategy safety and efficacy profiles of intravenous thrombolysis (IVT) and/or endovascular treatment (EVT) in children with AIS. Design, Setting, and Participants: The KidClot multicenter nationwide cohort study retrospectively collected data of children (neonates excluded) with AIS and recanalization treatment between January 1, 2015, and May 31, 2018. Data analysis was performed from January 1, 2015, to May 31, 2019. Exposure: IVT and/or EVT. Main Outcomes and Measures: Primary outcome was day 90 favorable outcome (modified Rankin Scale [mRs] 0-2, with 0 indicating no symptoms and 6 indicating death). Secondary end points included 1-year favorable outcome (mRs, 0-2), mortality, and symptomatic intracerebral hemorrhage. Other measures included the Pediatric National Institutes of Health Stroke Scale (pedNIHSS), with pedNIHSS 0 indicating no symptoms, 1 to 4 corresponding to a minor stroke, 5 to 15 corresponding to a mild stroke, greater than 15 to 20: severe stroke, and the adult Alberta Stroke Program Early CT Score (ASPECTS), which provides segmental assessment of the vascular territory, with 1 point deducted from the initial score of 10 for every region involved (from 10 [no lesion] to 0 [maximum lesions]). Results: Overall, 68 children were included in 30 centers (IVT [n = 44]; EVT [n = 40]; 44 boys [64.7%]; median [IQR] age, 11 [4-16] years; anterior circulation involvement, 57 [83.8%]). Median (IQR) pedNIHSS score at admission was 13 (7-19), higher in the EVT group at 16 (IQR, 10-20) vs 9 (6-17) in the IVT only group (P < .01). Median time from stroke onset to imaging was higher in the EVT group at 3 hours and 7 minutes (IQR, 2 hours and 3 minutes to 6 hours and 24 minutes) vs 2 hours and 39 minutes (IQR, 1 hour and 51 minutes to 4 hours and 13 minutes) (P = .04). Median admission ASPECTS score was 8 (IQR, 6-9). The main stroke etiologies were cardioembolic (21 [30.9%]) and focal cerebral arteriopathy (17 [25.0%]). Median (IQR) time from stroke onset to IVT was 3 hours and 30 minutes (IQR, 2 hours and 33 minutes to 4 hours and 28 minutes). In the EVT group, the rate of postprocedure successful reperfusion (≥modified Treatment in Cerebral Infarction 2b) was 80.0% (32 of 40). Persistent proximal arterial stenosis was more frequent in focal cerebral arteriopathy (P < .01). Death occurred in 3 patients (4.4%). Median pedNIHSS reduction at 24 hours was 4 (IQR, 0-9) points. Intracerebral hemorrhage occurred in 4 patients and symptomatic intracerebral hemorrhage occurred in 1 patient, all in the EVT group. The median mRS was 2 (IQR, 0-3) at day 90 and 1 (IQR, 0-2) at 1 year, which was not significantly different between EVT and IVT only groups, although different in initial severity. Conclusions and Relevance: The findings of this cohort study suggest that use of EVT and/or IVT is safe in children with AIS.

State of the art of antenatal diagnosis and management of vein of Galen aneurysmal malformations.

Vein of Galen aneurysmal malformation (VGAM) is a severe rare vascular anomaly whose prognosis depends on cerebral and cardiac consequences that can be hard to diagnose, let alone predict in utero. We performed an updated review to summarize current research on the genetics, ultrasound and MRI of VGAM that could help in the diagnosis and management of VGAM. Prenatal diagnosis of VGAM has greatly improved in recent years. Ultrasound allows in utero detection of VGAM in most cases now and is the best exam for prenatal cardiac evaluation. Tricuspid insufficiency is the only cardiac feature associated with poor prognosis. Cardiomegaly may indicate a risk of cardiac failure at birth and should prompt discussion of birth in a specialized facility. Ultrasound can identify constituted cerebral lesions, but MRI diagnoses early signs of cerebral hemodynamic changes, notably through the detection of pseudo-feeders. Genetic exploration should be proposed after VGAM diagnosis. Ultrasound and MRI are essential complementary tools for the diagnosis of VGAM, but also for prognostic evaluation, and provide information for the counseling of parents and optimal management of the pregnancy.

Arteriovenous Cerebral High Flow Shunts in Children: From Genotype to Phenotype.

Objective: To study the genotypes and phenotypes of cerebral arteriovenous fistulas that drain or do not drain through the vein of Galen, and true vein of Galen aneurysmal malformations, in order to determine whether genotyping could help improve classification of these malformations and their management. Methods: We carried out a retrospective review of genetic and phenotypic data in databases of four centers. All children with cerebral arteriovenous fistula or vein of Galen aneurysmal malformations aged below 18 years at onset were included. We recorded the nature of the genetic variant or absence of variant, age at onset, type of malformation, symptoms at onset (hemorrhage, neurological deficit, hydrocephalus, incidental, and heart failure), type of venous drainage and the long-term outcome. Results: One hundred and fifteen children were included. Autosomal dominant variants were identified in 39% of patients. The most frequent variant affected was the RASA1 gene (25%) followed by EPHB4 (8%) and the HHT-associated genes (5%). HHT gene variants were only observed in pial arteriovenous fistula not draining into the vein of Galen; on the contrary, EPHB4 variants were only seen in genuine vein of Galen aneurysmal malformation. RASA1 variants were identified in all types of shunts. Conclusions: EPHB4 variants seem specific to the vein of Galen aneurysmal malformation, RASA1 variants are associated with either pial arteriovenous fistulas or with genuine VGAM and HHT gene variants seem specific to pial arteriovenous fistulas. The genetic data helps to classify these malformations and to guide treatment toward lowest risk of post-operative cerebral ischemic-hemorrhagic complications.

Intracranial Aneurysm in Pediatric Population: A Single-Center Experience.

PURPOSE: Pediatric intracranial aneurysms (PIA) are rarer and more complex when compared to adult aneurysms. In general, the clinical presentation of PIA is due to a mass effect, but the presenting symptoms can be also related to ischemia, subarachnoid hemorrhage (SAH), or in a combination of different symptoms. This paper aimed to report a single-center experience with clinical and angiographic aspects of brain aneurysm in children. METHODS: We retrospectively reviewed our prospectively maintained database for patients with intracranial aneurysms in our institution from July 2015 to February 2021. Among these, all patients under 18 years of age submitted to a diagnostic or therapeutic procedure for an intracranial aneurysm were included. RESULTS: Twelve patients were submitted to diagnostic or therapeutic procedures in our department. Three of them had multiple aneurysms, and in total, 17 intracranial aneurysms were assessed in this study. The most frequent location was in the middle cerebral artery (7 cases/41%). Five out of twelve children (42%) presented SAH due to ruptured aneurysm. Three patients (25%) had symptoms due to the mass effect from large aneurysms, with compression of cranial nerves or brainstem. Aneurysms diameters ranged from 1.5 mm to 34 mm (mean 14.2 mm), with six aneurysms being giant and eight being nonsaccular/fusiform. Twelve aneurysms were submitted to endovascular treatment, with one treatment-related clinical complication and later death. CONCLUSION: PIAs are rare diseases that can arise from a variety of different underlying pathological mechanisms. The management of these conditions requires a detailed understanding of the pathology and a multidisciplinary approach. Despite the availability of new technologies, parent vessel occlusion remains a valid option for aneurysms in the pediatric population.

CLinical Assessment of WEB device in Ruptured aneurYSms (CLARYS): results of 1-month and 1-year assessment of rebleeding protection and clinical safety in a multicenter study.

BACKGROUND: The primary goal of the CLARYS study is to assess the protection against rebleeding when treating ruptured bifurcation aneurysms with the Woven EndoBridge (WEB) device. METHODS: The CLARYS study is a prospective, multicenter study conducted in 13 European centers. Patients with ruptured bifurcation aneurysms were consecutively included between February 2016 and September 2017. The primary endpoint was defined as the rebleeding rate of the target aneurysm treated with the WEB within 30 days postprocedure. Secondary endpoints included periprocedural and postprocedural adverse events, total procedure and fluoroscopy times, and modified Rankin Scale score at 1 month and 1 year. RESULTS: Sixty patients with 60 ruptured bifurcation aneurysms to be treated with the WEB were included. A WEB device was successfully implanted in 93.3%. The rebleeding rate at 1 month and 1 year was 0%. The mean fluoroscopy time was 27.0 min. Twenty-three periprocedural complications were observed in 18 patients and resolved without sequelae in 16 patients. Two of these complications were attributed to the procedure and/or the use of the WEB, leading to a procedure/device-related intraoperative complication rate of 3.3%. Overall mortality at 1 month and 1 year was 1.7% and 3.8%, respectively and overall morbidity at 1 month and 1 year was 15% and 9.6%, respectively. WEB-related 1-month and 1-year morbidity and mortality was 0%. CONCLUSIONS: The interim results of CLARYS show that the endovascular treatment of ruptured bifurcation aneurysms with the WEB is safe and effective and, in particular, provides effective protection against rebleeding. It may induce profound change in the endovascular management of ruptured bifurcation aneurysms.

Woven EndoBridge device shape modification can be mitigated with an appropriate oversizing strategy: a VasoCT based study.

BACKGROUND: The Woven EndoBridge (WEB) shape modification (WShM) during follow-up may be a potential cause of poor angiographic outcomes. WShM predisposing factors have not yet been determined. Our systematic use of rotational cone beam computed tomography (VasoCT) imaging during follow-up allowed us to perform the first quantitative analysis of the shape of WEBs over time. Our goal was to identify possible strategies to reduce the occurrence of this phenomenon. METHODS: All patients treated in our hospital with a WEB device between October 2015 and January 2019 were included. Using VasoCT acquisitions, systematically performed after implantation and during follow-up, we analyzed WEB morphology. WShM was defined as the percentage reduction in the distance between the two WEB markers. RESULTS: Sixty-three aneurysms treated with a WEB device were finally included in this analysis. At the last follow-up (mean 15.5 months), mean WShM was 48%±24. The mean WShM was significantly higher in the aneurysm recurrence group than in the adequate occlusion group (51±6.5% vs 36±3.4%, difference 15% points (95% CI 0.7 to 30); p<0.05). Conversely, the extent of WShM did not directly correlate with occlusion rates. Indeed, 32% of completely occluded aneurysms presented severe WShM (≥50%). Importantly, the absence of WShM guaranteed complete occlusion in our study (n=12). We demonstrated that oversizing the width of the WEB significantly correlated with WShM reduction during follow-up (r=-0.38, p=0.002). CONCLUSION: WShM can be partly overcome by use of an appropriate width oversizing strategy that could lead to improved angiographic results.

WEB device for treatment of posterior communicating artery aneurysms.

BACKGROUND: Woven EndoBridge (WEB) device treatment of wide-neck bifurcation aneurysms has proved to be safe and effective, but the use of these devices in sidewall aneurysms has been reported only in a small number of case series. OBJECTIVE: To report our results in a cohort of consecutive patients in whom a WEB device was used as first-line treatment for posterior communicating artery (PComA) aneurysms. METHODS: We conducted a retrospective analysis of a prospectively maintained database of PComA aneurysms treated with a WEB device in our institution from June 1, 2012 to November 15, 2020. Clinical and radiological findings were evaluated at immediate and last follow-up. RESULTS: A total of 219 aneurysms were treated with a WEB device, including 15 PComA aneurysms in 15 patients, 10 of which were ruptured. Aneurysms were wide necked, with a mean aspect ratio of 1.6 (range 0.7-3.0) and a mean neck size of 4.2 mm (range 2.6-7.4 mm). No intraoperative rupture occurred and only one thromboembolic event was noted. Among the group with at least a 3-month digital subtraction angiography (DSA) follow-up, complete and adequate occlusion were obtained in 54% and 72%, respectively (average follow-up 13 months). Re-treatment was needed for two initially ruptured aneurysms. No procedure-related morbidity or mortality was reported. CONCLUSION: This series suggests the high safety profile of WEB devices even when used in off-label indications. Treatment with these devices seems to be a valuable strategy for ruptured wide-neck PComA aneurysms, avoiding the need for antiplatelet medication. However, occlusion rates should be investigated in further larger studies.

Extending the stroke treatment window beyond DAWN in patients with very slow progressor type collaterals: How far can we go?

Five trials published in 2015 showed the benefit of endovascular thrombectomy (ET) in patients with stroke and large vessel occlusion, extending the treatment window has become an obsession of all physicians. In 2018, the DAWN and DEFUSE-3 trials showed that, with careful selection of patients, the procedure could be carried out up to 24 hours after symptom onset with good outcomes. In addition, there have been cases where the DAWN criteria were met, and treatment occurred >24 hours after symptom onset. We present the case of a 68-year-old female whose groin puncture occurred 52 hours after the time last known well (TLKW), after neurological worsening of the initial situation, with a large mismatch ratio observed on magnetic resonance imaging, achieving TICI (the Thrombolysis in Cerebral Infarction scale) grade 3 recanalization. Five days after the procedure, the patient was discharged with NIHSS (National Institutes of Health Stroke Scale) score of 3. Some types of collateral circulation (slow progressors and "turtle" progressors, our term for very slow progressors) can extend the treatment window beyond 24 hours of the TLKW but can lead to a hyperperfusion-like syndrome immediately after the ET. Further studies are needed to evaluate the reproducibility of this hypothetical syndrome.

Pre-procedural predictive factors of symptomatic intracranial hemorrhage after thrombectomy in stroke.

OBJECTIVE: Symptomatic intracerebral hemorrhage (sICH) is a common complication of acute ischemic stroke (AIS) associated with limited treatments and poor outcomes. We aimed to identify predictive factors of sICH in patients with AIS following mechanical thrombectomy (MT) in a real-world setting. METHODS: Patients with large vessel occlusion of the anterior circulation treated with MT were consecutively included in a prospective monocentric cohort. Clinical, biological, and radiological parameters were collected to identify pre-procedural predictors for sICH. RESULTS: 637 patients were included in our study. Magnetic resonance imaging was performed on most patients (86.7%). sICH occurred in 55 patients (8.6%). 428 patients (67.2%) were treated with intravenous thrombolysis. After multivariate analysis, prior use of antiplatelet therapies (odd ratio (OR) 1.84, 95% confidence interval (CI) 1.01-3.32), high C-reactive protein (OR per standard deviation (SD) increase 1.28, 95% 1.01-1.63), elevated mean arterial blood pressure (OR per 10 mmHg increase 1.22, 95% CI 1.03-1.44), hyperglycemia (OR per one SD-log increase 1.38, 95% CI 1.02-1.87), and low ASPECTS (OR per 1-point decrease 1.42, 95% CI 1.12-1.80) were found to be independent predictive factors of sICH. The pre-procedural predictors did not change when the absence of successful recanalization was considered as a covariate. Patients with strokes of unknown onset time were not especially vulnerable for sICH. CONCLUSION: sICH after MT was associated with several pre-procedural risk factors: prior use of antiplatelet therapies, high C-reactive protein and hyperglycemia at baseline, elevated mean arterial blood pressure, and low ASPECTS.

RASA1 phenotype overlaps with hereditary haemorrhagic telangiectasia: two case reports.

Background We report two cases of RASA1-related capillary malformation-arteriovenous malformation (CM-AVM1) syndrome mimicking hereditary haemorrhagic telangiectasia (HHT).Methods and results A 28-year-old man, previously embolised for cerebral arteriovenous malformations (AVMs), presented with epistaxis and typical nasal telangiectasias of HHT. CT scan revealed a large portocaval shunt. The second patient was a 9-year-old girl presenting with cyanosis and several mucocutaneous telangiectasias, similar to those observed in typical cases of HHT. CT scan revealed a huge and complex pulmonary AVM of the right lower lobe and a hepatic AVM within the left lobe. HHT diagnosis was considered possible according to the Curaçao criteria for the two patients, with at least two criteria for each. Genetic tests did not find any mutation in the three classic genes (Endoglin, Activin receptor-like kinase 1 or Mothers against decapentaplegic homolog 4), but identified in both cases an RASA1 mutation, known to cause CM-AVM1 syndrome.Conclusions Pulmonary AVM and portocaval shunt, usually encountered in HHT, have not yet been described in the CM-AVM1 syndrome. RASA1 screening may be considered in case of HHT suspicion, particularly when mutations are not found in the usually affected genes.

Hyperacute Recanalization Strategies and Childhood Stroke in the Evidence Age.

No controlled pharmacological studies are available in the field of pediatric stroke, except for sickle cell disease. Therefore, while pharmacological and mechanical recanalization treatments have repeatedly shown clinical benefit in adults with arterial ischemic stroke, pediatric strokologists still cannot base their therapeutic management (including hyperacute strategies) on high-level evidence. Once again, pediatricians face the same dichotomic choice: adapting adult procedures now versus waiting-for a long time-for the corresponding pediatric trials. One way out is building a compromise based on observational studies with large, longitudinal, comprehensive, real-life, and multisource dataset. Two recent high-quality observational studies have delivered promising conclusions on recanalization treatments in pediatric arterial ischemic stroke. TIPSTER (Thrombolysis in Pediatric Stroke Extended Results) showed that the risk of severe intracranial hemorrhage after intravenous thrombolysis is low; the Save Childs Study reported encouraging data about pediatric thrombectomy. Beyond the conclusion of a satisfactory global safety profile, a thorough analysis of the methods, populations, results, and therapeutic complications of these studies helps us to refine indications/contraindications and highlights the safeguards we need to rely on when discussing thrombolysis and thrombectomy in children. In conclusion, pediatric strokologists should not refrain from using clot lysis/retrieval tools in selected children with arterial ischemic stroke. But the implementation of hyperacute care is only feasible if the right candidate is identified through the sharing of common adult/pediatric protocols and ward collaboration, formalized well before the child's arrival. These anticipated protocols should never undervalue contraindications from adult guidelines and must involve the necessary pediatric expertise when facing specific causes of stroke, such as focal cerebral arteriopathy of childhood.

Spinal arteriovenous malformation without endovascular arterial access: is a combined neurosurgical approach and direct venous puncture an option?

This case report describes a combined neurosurgical and endovascular approach for the treatment of a conus medullaris arteriovenous malformation resulting in considerable improvement in the patient's neurological condition (modified Rankin Scale score 2).

Republished: Spinal arteriovenous malformation without endovascular arterial access: is a combined neurosurgical approach and direct venous puncture an option?

This case report describes a combined neurosurgical and endovascular approach for the treatment of a conus medullaris arteriovenous malformation resulting in considerable improvement in the patient's neurological condition (modified Rankin Scale score 2).

Higher prevalence of splenic artery aneurysms in hereditary hemorrhagic telangiectasia: Vascular implications and risk factors.

BACKGROUND: Splenic artery aneurysm (SAA) is a rare but potentially fatal condition. Rupture results in 25% mortality up to 75% in pregnant women with 95% fetal mortality. Brief reports suggest an increased risk of developing SAA in patients with HHT. METHODS: We analyzed enhanced multidetector CT data in 186 HHT patients matched (gender and ± 5 year old) with 186 controls. We screened for SAA and recorded diameter of splenic and hepatic arteries and hepatic, pancreatic and splenic parenchymal involvements. We determined by univariate and multivariate analysis, the relationship with age, sex, genetic status, cardiovascular risk factors (CVRF) and visceral involvement. RESULTS: SAA concerned 24.7% of HHT patients and 5.4% of controls, p<0.001. Factors associated with increased risk of SAA in HHT were female gender (p = 0.04, OR = 2.12, IC 95% = 1.03-4.50), age (p = 0.0003, OR = 1.04, 95% CI = 1.02-1.06) and pancreatic parenchymal involvement (p = 0.04, OR = 2.13, 95% CI = 1.01-4.49), but not type of mutation, hepatic or splenic parenchymal involvements, splenic size or splenic artery diameter or CVRF. CONCLUSIONS: We found a 4.57 higher rate of SAA in HHT patients without evidence of splenic high output related disease or increased CVRF. These results suggest the presence of a vascular intrinsic involvement. It should lead to screening all HHT patients for SAA. The vasculopathy hypothesis could require a change in management as screening of all systemic arteries and even the aorta and to further research in the field.

The PHASES score: To treat or not to treat? Retrospective evaluation of the risk of rupture of intracranial aneurysms in patients with aneurysmal subarachnoid hemorrhage.

BACKGROUND AND PURPOSE: The PHASES score was formulated to predict the 5-year risk of rupture for intracranial aneurysms. We retrospectively analyzed all patients treated in our institution for aneurysmal SAH and applied the PHASES score to estimate the probable predicted risk of bleeding in this group of patients. METHODS: Between February 2015 and August 2018, all patients with aneurysmal SAH were retrospectively analyzed and the PHASES score was applied. A total of 155 patients were included with a mean age of 53.8years, including 60 males and 95 females. RESULTS: Of our patients 110 (70.9%) had a PHASES score of below or equal to 5, with a hemorrhagic risk of up to 1.3% over 5years. If we analyze the patients with a risk of below 2% this figure increases to 122 patients (78.7%). Of these 99.3% were European and 0.6% were Japanese (1 patient). In 86 patients (55.4%), the aneurysm was smaller than 5mm and in 10 patients (6.4%) the aneurysm was located in the posterior circulation. CONCLUSION: Of our patients 78.7% had less than a 2% 5-year rupture risk based on their PHASES score, highlighting the discrepancy of the rupture risk calculated with the PHASES score when hypothetically applied to this group of patients. In the hypothetical scenario that our patients had unruptured aneurysms, our retrospective analysis shows that the PHASES score may only provide a weak tool for clinicians to use in the decision-making process as to whether or not to treat these aneurysms.

Long-term outcome of vein of Galen malformation.

AIM: To describe the long-term outcomes of children by the time they reached school age with vein of Galen aneurysmal malformation (VGAM). METHOD: This was a retrospective observational study on a consecutive cohort of patients with VGAM. We included patients with at least one Francophone parent, aged between 6 and 11 years at the time of long-term evaluation. The neurological outcome was assessed with the King's Outcome Scale for Childhood Injury score and eight neurological and behavioural items from the Rivermead Postconcussion Symptoms questionnaire. RESULTS: All 52 patients (17 females, 32 males [data missing for n=3]) with at least one Francophone parent (5 fetuses and 47 children) were included. At the long-term evaluation time-point, 33 patients were alive and 19 patients had died. Risk of postnatal death was associated with severe neonatal cardiac failure (p=0.007) or isosystemic or suprasystemic pulmonary hypertension (p=0.014). Among survivors, 19 had a good outcome with normal schooling and 14 had a poor outcome. Moreover, among the good outcome patients, a large proportion had neurodevelopmental alterations. INTERPRETATION: Long-term outcome of patients with VGAM appears to be less favourable than outcome described at the short- and medium-term, even in the absence of encephalomalacia at birth. Even patients with good outcome often have neuropsychological disorders that may have repercussions on learning and requiring appropriate rehabilitation or medical management. WHAT THIS PAPER ADDS: Long-term outcome appears to be less favourable than described at short- and medium-term follow-up. Even patients with good outcome at these time-points often have minor neuropsychological disorders.

Management and Outcomes of Neonatal Arteriovenous Brain Malformations with Cardiac Failure: A 17 Years' Experience in a Tertiary Referral Center.

OBJECTIVES: To assess the management and outcomes of neonatal arteriovenous brain malformations (mostly vein of Galen malformations) complicated by cardiac failure in the era of prenatal diagnosis and endovascular treatment in a tertiary referral center. STUDY DESIGN: This observational study included 77 living newborn infants with arteriovenous brain malformations with cardiac failure, admitted to our referral center from 2001 to 2017. All infants underwent cardiovascular evaluation including echocardiogram and brain magnetic resonance imaging. Long-term survivors had standard neurocognitive assessments. RESULTS: Infants were admitted to the neonatal intensive care unit at a median of 5 days of age (including 18 inborn patients since 2009). Sixty transarterial shunt embolizations were performed in 46 patients during their first month (at a median age of 7.5 days) or postponed beyond the first month in another 10 long-term survivors. Embolization was not performed in 21 infants, including 19 nonsurvivors with severe brain injury, uncontrolled cardiac failure, or multiple organ failure. Cardiac failure requiring vasopressor infusion occurred in 48 patients (64%) during the hospitalization. Infants who survived the first month underwent a median of 3 embolization sessions. Among the 51 survivors, 21 had a good outcome and 19 had a poor outcome at follow-up (median age, 5.3 years); 11 children were lost to follow-up. CONCLUSIONS: In the era of multidisciplinary prenatal diagnosis, using a standardized care protocol, 47% of liveborn infants with an arteriovenous shunt malformation with cardiac failure experienced a favorable outcome.