Diagnostic Value of Vessel Wall Imaging to Determine the Timing of Extracranial‒Intracranial Bypass for Moyamoya Syndrome Associated with Active Sjögren's Syndrome: A Case Report.

BACKGROUND: Sjögren's syndrome is a chronic autoimmune disorder that predominantly affects exocrine organs. It is characterized by an organ-specific infiltration of lymphocytes. The involvement of the major cerebral arteries in Sjögren's syndrome has rarely been reported. A recent study reported a case of successful extracranial-intracranial (EC-IC) bypass without complications, even in the active inflammatory state, although the optimal timing of such a bypass remains unclear. CASE DESCRIPTION: We here report the case of a 43-year-old woman presenting with acute ischemic stroke due to progressive middle cerebral artery (MCA) occlusion and signs of primary Sjögren's syndrome. During intensive immunosuppressive therapy for active Sjögren's syndrome, the patient was monitored using contrast-enhanced magnetic resonance vessel wall imaging (MR-VWI). A couple of intravenous cyclophosphamide injections combined with a methylprednisolone pulse and antiplatelet therapy resulted in clear resolution of vessel wall enhancement, which suggested remission of inflammatory vasculitis. Nevertheless, she still experienced a transient ischemic attack (TIA) due to decreased regional cerebral blood flow by MCA occlusion, as demonstrated by the conventional time-of-flight MR angiography and single-photon emission computed tomography. Considering the increased risk of further stroke, the decision was made to perform an EC-IC bypass as a treatment for medically uncontrollable hemodynamic impairment. Her postoperative course was uneventful without further repeated TIAs, and continued immunosuppressive therapy for Sjögren's syndrome provided effective management. CONCLUSIONS: Our findings emphasize the diagnostic value of contrast-enhanced MR-VWI in monitoring the effect of immunosuppressive therapy for the major cerebral artery vasculitis and in determining the timing of EC-IC bypass as a "rescue" treatment for moyamoya syndrome associated with active Sjögren's syndrome.

Extracranial-Intracranial Bypass and Risk of Stroke and Death in Patients With Symptomatic Artery Occlusion: The CMOSS Randomized Clinical Trial.

Importance: Prior trials of extracranial-intracranial (EC-IC) bypass surgery showed no benefit for stroke prevention in patients with atherosclerotic occlusion of the internal carotid artery (ICA) or middle cerebral artery (MCA), but there have been subsequent improvements in surgical techniques and patient selection. Objective: To evaluate EC-IC bypass surgery in symptomatic patients with atherosclerotic occlusion of the ICA or MCA, using refined patient and operator selection. Design, Setting, and Participants: This was a randomized, open-label, outcome assessor-blinded trial conducted at 13 centers in China. A total of 324 patients with ICA or MCA occlusion with transient ischemic attack or nondisabling ischemic stroke attributed to hemodynamic insufficiency based on computed tomography perfusion imaging were recruited between June 2013 and March 2018 (final follow-up: March 18, 2020). Interventions: EC-IC bypass surgery plus medical therapy (surgical group; n = 161) or medical therapy alone (medical group; n = 163). Medical therapy included antiplatelet therapy and stroke risk factor control. Main Outcomes and Measures: The primary outcome was a composite of stroke or death within 30 days or ipsilateral ischemic stroke beyond 30 days through 2 years after randomization. There were 9 secondary outcomes, including any stroke or death within 2 years and fatal stroke within 2 years. Results: Among 330 patients who were enrolled, 324 patients were confirmed eligible (median age, 52.7 years; 257 men [79.3%]) and 309 (95.4%) completed the trial. For the surgical group vs medical group, no significant difference was found for the composite primary outcome (8.6% [13/151] vs 12.3% [19/155]; incidence difference, -3.6% [95% CI, -10.1% to 2.9%]; hazard ratio [HR], 0.71 [95% CI, 0.33-1.54]; P = .39). The 30-day risk of stroke or death was 6.2% (10/161) in the surgical group and 1.8% (3/163) in the medical group, and the risk of ipsilateral ischemic stroke beyond 30 days through 2 years was 2.0% (3/151) and 10.3% (16/155), respectively. Of the 9 prespecified secondary end points, none showed a significant difference including any stroke or death within 2 years (9.9% [15/152] vs 15.3% [24/157]; incidence difference, -5.4% [95% CI, -12.5% to 1.7%]; HR, 0.69 [95% CI, 0.34-1.39]; P = .30) and fatal stroke within 2 years (2.0% [3/150] vs 0% [0/153]; incidence difference, 1.9% [95% CI, -0.2% to 4.0%]; P = .08). Conclusions and Relevance: Among patients with symptomatic ICA or MCA occlusion and hemodynamic insufficiency, the addition of bypass surgery to medical therapy did not significantly change the risk of the composite outcome of stroke or death within 30 days or ipsilateral ischemic stroke beyond 30 days through 2 years. Trial Registration: ClinicalTrials.gov Identifier: NCT01758614.

Diagnosis and management of acute conditions of the extracranial carotid artery.

Symptomatic carotid stenosis and carotid dissection are acute conditions of extracranial cerebrovascular vessels determining transient ischemic attack or stroke. Medical, surgical, or endovascular management are different options to treat these pathologies. This narrative review focused on the management, from symptoms to treatment, of the acute conditions of extracranial cerebrovascular vessels, including post-carotid revascularization stroke. Symptomatic carotid stenosis (> 50% according to North American Symptomatic Carotid Endarterectomy Trial criteria) with transient ischemic attack or stroke benefits from carotid revascularization-primarily with carotid endarterectomy associated with medical therapy-within 2 weeks from symptom onset to reduce the risk of stroke recurrence. Different from acute extracranial carotid dissection, medical management with antiplatelet or anticoagulant therapy can prevent new neurologic ischemic events, considering stenting only in case of symptom recurrence. Stroke after carotid revascularization can be associated with the following etiologies: carotid manipulation, plaque fragmentation, or clamping ischemia. Medical or surgical management is therefore influenced by the cause and timing of the neurologic events after carotid revascularization. Acute conditions of the extracranial cerebrovascular vessels include a heterogeneous group of pathologies and correct management can reduce symptom recurrence substantially.

[Indications for Extracranial-Intracranial Bypass Surgery].

Previous randomized clinical trials have so far failed to establish the efficacy of extracranial-intracranial(EC-IC)bypass in the prevention of secondary ischemic strokes. For patients with a recent transient ischemic attack or ischemic stroke ipsilateral to a stenosis or occlusion of the middle cerebral or carotid artery, EC-IC bypass is not recommended as per the American heart association/American stroke association guidelines(Class III, Level of Evidence A). However, patients with severe hemodynamic impairment(misery perfusion)are at high risk of developing stroke, and EC-IC bypass is recommended as per the Japan stroke guideline 2021, provided that the patients show cerebral blood flow less than 80% from baseline and cerebral vascular reserve less than 10%. Perioperative management is also important in preventing ischemic complications and hyperperfusion. Some adjunctive drugs, including minocycline and edaravone, have been reported to be effective against brain damage from hyperperfusion. Regarding Moyamoya disease, EC-IC bypass has been established as a recommended strategy for ischemic presentation, hemorrhagic presentation, hemodynamic impairment, and choroidal anastomosis. EC-IC bypass is also necessary for specific types of aneurysms, including fusiform and thrombotic, as well as in the dissection of aneurysms that are difficult to clip.

Comparison of long-term outcomes and risk factors of aortic stenosis treatment in patients undergoing transcatheter aortic valve implantation and surgical aortic valve replacement.

BACKGROUND: Aortic stenosis (AS) is the most common valvular heart disease and untreated has a bleak prognosis. The only effective method of treatment is valve replacement, surgical (SAVR), or transcatheter (TAVI). AIMS: We decided to analyze outcomes and predictors of long-term mortality in patients undergoing TAVI and SAVR. METHODS: A retrospective analysis of 1229 patients with advanced AS, comprising TAVI (n = 211), SAVR (n = 556), SAVR, and additional procedures (n = 462), operated on from 2014 to 2018, was performed. RESULTS: No significant differences between SAVR and TAVI were found for 24-month mortality in groups of consecutive patients. Postoperative stroke or transient ischemic attack (TIA), chronic obstructive pulmonary disease (COPD), and transfusion of red blood cells (RBCs) were independent predictors of 1-year mortality after SAVR. The above-mentioned factors regarding the increased estimated surgery risk in the EuroSCORE II ( > 4%) were predictors of 2-years mortality after SAVR. Risk factors for 6- and 12-month mortality after TAVI were EuroSCORE II, new onset of atrial fibrillation (NOAF), and the increased RBC distribution width (RDW). Postoperative respiratory failure was an independent risk factor for 6-, 12- and 24-month mortality in both groups of patients. CONCLUSIONS: There were no significant differences regarding prognosis after TAVI and SAVR at the 24-month follow-up in the propensity score matching model. Independent predictive factors of late mortality after both procedures were EuroScore II and respiratory failure. Independent predictive factors of late mortality specific for TAVI were NOAF, increased RDW, and for SAVR: TIA, stroke, COPD, and RBC transfusion.

No Evidence for Long Term Blood Pressure Differences Between Eversion and Conventional Carotid Endarterectomy in Two Independent Study Cohorts.

OBJECTIVE: Blood pressure (BP) management is a vital aspect of stroke prevention and post-stroke care. Different surgical carotid endarterectomy (CEA) techniques may impact on BP control post-operatively. Specifically, the carotid sinus nerve, which innervates the carotid baroreceptors and carotid body, is commonly left intact during conventional CEA but is routinely transected as part of eversion CEA. The aim of this study was to assess long term BP control after eversion and conventional CEA. METHODS: Patients from the International Carotid Stenting Study (ICSS cohort) and a personal series of patients from the Stroke Clinical Trials Unit at University College London (UCL cohort) were separately analysed and divided into eversion and conventional CEA groups. Mixed effect linear models were fitted and adjusted for baseline demographic data and antihypertensive treatment to test for changes in BP from baseline over a three year follow up period after the respective procedures. RESULTS: There were no differences in changes in baseline BP readings and follow up readings between eversion and conventional CEA in the ICSS or UCL cohorts. In the ICSS cohort a mild but significant systolic (-8.6 mmHg; 95% confidence interval [CI] -10.6 - -6.6) and diastolic (-4.9 mmHg; 95% CI -6.0 - -3.8) BP lowering effect was evident at discharge in the conventional group but not in the eversion CEA group. BP monitoring during follow up did not reveal any consistent BP changes with either conventional or eversion CEA vs. baseline levels. CONCLUSION: Neither conventional nor eversion CEA seem to result in clinically significant long term BP changes. Potential concerns related to either short or long term alterations in BP levels with transection of the carotid sinus nerve during eversion CEA could not be substantiated.

Patients Receiving Extracranial to Intracranial Bypass for Atherosclerotic Vessel Occlusion Today Differ Significantly From the COSS Population.

Background and Purpose: Despite the findings reported in the COSS (Carotid Occlusion Surgery Study), patients with atherosclerotic cerebrovascular disease continue to be referred for superficial temporal artery to middle cerebral artery bypass surgery. Here, we determined how today's patients differ from the population reported in COSS. Methods: We retrospectively analyzed all patients that were referred to our Department for superficial temporal artery to middle cerebral artery bypass surgery of atherosclerotic cerebrovascular disease following the publication of COSS. Results: Between 2012 and 2019, 179 patients were referred for 186 bypass surgeries. Ninety-one (51%) patients suffered atherosclerotic, unilateral internal carotid occlusion and 88 (49%) atherosclerotic multivessel disease. All patients had received intensive medical management. A single transitory ischemic attack or ischemic stroke within the last 120 days according to the inclusion criteria of COSS occurred in only 36 out of 179 (20%) patients, whereas 27 out of 179 (15%) suffered >1 transitory ischemic attack within 120 days, 109 out of 179 (61%) had recurrent minor ischemic stroke, and 7 out of 179 (4%) were hemodynamically unstable and required blood pressure maintenance. The distribution of symptoms did not differ between atherosclerotic unilateral internal carotid artery occlusion and atherosclerotic multivessel disease (P=0.376) but hemodynamic impairment was significantly greater in atherosclerotic multivessel disease (P<0.001 for atherosclerotic multivessel disease versus atherosclerotic unilateral internal carotid artery occlusion). The overall perioperative stroke rate was 4.3%. Conclusions: Patients referred for flow augmentation surgery today appear to suffer more severe symptoms and vessel occlusion patterns than patients reported in COSS. A new, carefully designed randomized controlled trial appears warranted, considering the still poor prognosis of severe atherosclerotic cerebrovascular disease.

Additional Revascularization Using Multiple Burr Holes for PCA Involvement in Moyamoya Disease.

In specific cases of moyamoya disease (MMD), posterior cerebral artery (PCA) stenosis can develop after treatment of the anterior circulation and require additional revascularization. Here, we report two cases that underwent additional posterior indirect revascularization with multiple burr holes for PCA involvement after bilateral revascularization treatment of the anterior circulation. They presented with transient ischemic attack even after bilateral superficial temporal artery-middle cerebral artery bypass, and magnetic resonance angiography (MRA) showed that PCA stenosis had worsened. Indirect revascularization with multiple burr holes using Benz-marked skin incisions was performed. After surgery, the symptoms improved without perioperative complications, and cerebral angiography showed collateral circulation via the burr hole. Indirect revascularization for MMD is often combined with direct revascularization, and there are only a few reports on the use of multiple burr hole surgery alone. In addition, there are few reports of posterior circulation, despite the emphasis on the importance of PCA involvement in MMD. Indirect revascularization with multiple burr holes alone can be performed in multiple areas and applied to patients who cannot undergo direct revascularization using the occipital artery. The procedure is simple and less invasive than traditional direct revascularization procedures. Therefore, it can be effective, especially in pediatric cases of MMD with PCA involvement.

Impact of the COVID-19 Pandemic on Emergency Department Use: Focus on Patients Requiring Urgent Revascularization.

BACKGROUND: The novel coronavirus (2019-nCOV) appeared in China and precipitously extended across the globe. As always, natural disasters or infectious disease outbreaks have the potential to cause emergency department (ED) volume changes. OBJECTIVE: We aimed to assess the influence of the Coronavirus Disease 2019 (COVID-19) pandemic on ED visits and the impact on the handling of patients requiring urgent revascularization. METHODS: We reviewed the charts of all patients presenting to the ED of Hospital Sainte Anne (Toulon, France) from March 23 to April 5, 2020 and compared them with those of the same period in 2019. Then we analyzed complementary data on acute coronary syndrome (ST-elevation myocardial infarction [STEMI] and non-ST-elevation myocardial infarction [NSTEMI]) and neurovascular emergencies (strokes and transient ischemic attacks). RESULTS: The total number of visits decreased by 47%. The number of people assessed as triage level 2 was 8% lower in 2020. There were five fewer cases of NSTEMI in 2020, but the same number of STEMI. The number of neurovascular emergencies increased (27 cases in 2019 compared with 30 in 2020). We observed a reduction in the delay between arrival at the ED and the beginning of coronary angiography for STEMI cases (27 min in 2019 and 22 min in 2020). In 2020, 7 more stroke patients were admitted. CONCLUSION: The COVID-19 pandemic probably dissuaded "non-critical" patients from coming to the hospital, whereas the same number of patients with a critical illness attended the ED as attended prior to the pandemic. There does not seem to have been any effect of the pandemic on patients requiring reperfusion therapy (STEMI and stroke).

Late (5-20 years) outcomes after STA-MCA anastomosis and encephalo-duro-myo-arterio-pericranial synangiosis in patients with moyamoya disease.

OBJECTIVE: Surgical revascularization is known to reduce the incidence of further ischemic and hemorrhagic events in patients with moyamoya disease, but the majority of previous studies report only short-term (< 5 years) outcomes. Therefore, in this study the authors aimed to evaluate late (5-20 years) outcomes of moyamoya patients after superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis and indirect bypass (encephalo-duro-myo-arterio-pericranial synangiosis [EDMAPS]). METHODS: Cumulative incidences of late morbidity/mortality and disease progression were evaluated among 93 patients who underwent STA-MCA anastomosis and EDMAPS. All of the patients were prospectively followed up for longer than 5 years postsurgery (10.5 ± 4.4 years). There were 35 pediatric and 58 adult patients. Initial presentation included transient ischemic attack/ischemic stroke in 80 patients and hemorrhagic stroke in 10 patients, and 3 patients were asymptomatic. Surgery was performed in a total of 141 hemispheres. Follow-up MRI/MRA was performed within a 6- or 12-month interval during the follow-up periods. RESULTS: During the follow-up periods, 92/93 patients were free from any stroke or death, but 1 patient had a recurrence of hemorrhagic stroke (0.10% per patient-year). Disease progression occurred in the territory of the contralateral carotid or posterior cerebral artery (PCA) in 19 hemispheres of 15 patients (1.5% per patient-year). The interval between initial surgery and disease progression varied widely, from 0.5 to 15 years. Repeat bypass surgery for the anterior and posterior circulation resolved ischemic attacks in all 10 patients. CONCLUSIONS: The study results indicate that STA-MCA anastomosis and EDMAPS would be the best choice to prevent further ischemic and hemorrhagic stroke for longer than 10 years on the basis of the demonstrated widespread improvement in cerebral hemodynamics in both the MCA and ACA territories in the study patients. However, after 10 years postsurgery regular follow-up is essential to detect disease progression in the territory of the contralateral carotid artery and PCA and prevent late cerebrovascular events.

Cerebral Revascularization for Moyamoya Syndrome Associated with Sickle Cell Disease: A Systematic Review of the Literature on the Role of Extracranial-Intracranial Bypass in Treating Neurologic Manifestations of Pediatric Patients with Sickle Cell Disease.

Moyamoya syndrome (MMS) in patients with sickle cell disease (SCD) accentuates the risk of recurrent strokes. Chronic transfusion therapy (CTT) is an excellent option for preventing recurrent strokes in most patients with SCD. In SCD with MMS, CTT may fail as a long-term solution. Cerebral revascularization, in the form of extracranial-intracranial bypass, has been shown to prevent recurrent strokes in this cohort. We review the evolution of this paradigm shift in the management of SCD-associated MMS. A systematic review, adhering to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses protocol, was conducted. Our primary objectives were 1) to study the evolution of cerebral revascularization techniques in management of MMS in SCD and 2) to analyze the impact of neurosurgical intervention in this high-risk population. Four patients with SCD-associated MMS, who underwent indirect cerebral revascularization at our institute were retrospectively reviewed. A summary of 13 articles chronicling the advent and subsequent evolution of cerebral revascularization as a viable treatment strategy for stroke prevention in SCD-associated MMS is presented. The literature review suggests that early detection and surgical intervention (in addition to CTT) could significantly reduce stroke recurrence and improve neurocognitive outcome. Our short series of 4 patients also had a good outcome and no recurrence of strokes postoperatively. The literature emphasizes the use of a traditional standardized protocol for early identification (transcranial Dopplers, selective magnetic resonance angiography, and CTT). Early treatment and screening that involves early magnetic resonance angiography and referral to neurosurgery for revascularization may be considered for this high-risk population.

Dual versus Single Antiplatelet Therapy in Carotid Artery Endarterectomy: Direct Comparison of Complications Related to Antiplatelet Therapy.

BACKGROUND: Dual and single antiplatelet therapies are routinely used in carotid artery endarterectomy (CEA). However, the efficacy and safety of these therapies are controversial. The present study aimed to comprehensively compare the clinical outcomes between dual and single antiplatelet therapies in CEA. METHODS: This study retrieved available academic studies evaluating the complications related to antiplatelet therapy between dual and single antiplatelet therapies in CEA from the databases of ScienceDirect, the Cochrane Library, EMBASE, and PubMed. References to previous reviews and related clinical trials were manually checked to retrieve potential literature that was not included in our electronic search results. RESULTS: A total of 10 articles (1 randomized controlled trial, 9 non-randomized controlled trials) were included in the study. The overall number of patients in the dual antiplatelet group was 14,280, and the number of patients in the single antiplatelet group was 125,850. The results revealed that the single antiplatelet group had a lower incidence of 30-day death (rate difference [RD] 0.002; 95% confidence interval [CI] 0.000-0.003; P = 0.014), neck hematoma (odds ratio [OR] 2.120; 95% CI 1.431-3.142; P < 0.001), myocardial infarction (RD 0.004; 95% CI 0.001-0.007; P = 0.003), and major bleeding (RD 0.005; 95% CI 0.002-0.008; P < 0.001). Meanwhile, the single antiplatelet group was associated with a shorter operation time (weighted mean difference 4.000; 95% CI= 2.564-5.436; P < 0.001). However, there was no significant difference in the rate of postoperative transient ischemic attack (P = 0.215), stroke (P = 0.130), or length of stay (P = 0.563). CONCLUSIONS: Based on current evidence, using single antiplatelet therapy in CEA may reduce operation time and the incidences of 30-day death, neck hematoma, major bleeding, and myocardial infarction without increasing the risks of transient ischemic attack, stroke, or a longer operation time.

Cerebral glucose metabolism changes in chronic ischemia patients following subcranial-intracranial bypass.

The use of the internal maxillary artery (IMA) in intracranial artery bypass or subcranial-intracranial (SC-IC) bypass has recently been described as an alternative to traditional bypass. This study explores cerebral glucose metabolism characteristics of SC-IC bypass. Ten crescendo transient ischemic attack (TIA) patients with chronic occlusion of the middle cerebral artery (MCA) received bypass surgery of IMA with the radial artery graft (RAG) to the branch of MCA. The graft's flow volume (FV) was measured by operative intraoperative duplex ultrasonography. Positron emission tomography (PET)/computed tomography (CT) was used to calculate the preoperational and postoperational average of the standard uptake value (SUVavg) of the 18-fluoro-2-deoxy-D-glucose (18F-FDG) in the region of interest (ROI). The asymmetric index (AI) is recommended to reflect the SUVavg changes, and subsequently, cerebral glucose metabolism changes are supposedly clarified. Patent IMA-RAG-MCA bypass in ten chronic ischemia patients was confirmed by angiography after surgery. The intraoperative FV measurement value was 65.64 ± 10.52 (58.11-73.17) ml/min. Before the operation, the SUVavg of the ROI in the ischemic hemisphere (4.76 ± 2.35 (3.08-6.04)) clearly decreased compared to the one (5.99 ± 2.63 (4.11-7.87)) in the contralateral mirror region (P = 0.003). The result of AI of preoperation minus AI of postoperation was more than 10% (P = 0.031), which indicated suspicious significant changes in cerebral metabolism. All symptoms of study patients having crescendo ischemia were resolved in 1 month after the operation. In the cerebral hypoperfusion territory, uptake of 18F-FDG deceased. Improving the flow volume via SC-IC bypass makes available an elevated uptake of 18F-FDG.

Submaximal angioplasty in the treatment of patients with symptomatic ICAD: a systematic review and meta-analysis.

INTRODUCTION: Management approaches for intracranial atherosclerosis include medical, surgical, or endovascular treatment. Among endovascular treatments, recent studies have stated that submaximal angioplasty (SA) may offer considerable benefits and be a promising alternative to aggressive medical therapyand/or stenting in the treatment of intracranial atherosclerotic disease (ICAD). OBJECTIVE: To investigate the rates of periprocedural and long-term cerebrovascular accidents, mortality, and restenosis in patients with symptomatic ICAD who were treated with SA. METHODS: An electronic database search was performed for relevant studies that reported clinical outcomes of patients with ICAD following SA. Outcomes of interest were incidence of transient ischemic attack, intracerebral hemorrhage, stroke, and mortality in the periprocedural period and at 1 year. The periprocedural period was defined as the time from SA until 30 days after the procedure. Technical success and restenosis rates after the procedure were also analyzed. RESULTS: A total of 19 studies with 777 patients were identified. The technical success rate was 93% (95% CI 85% to 98%). The incidence of 30-day and 1-year stroke (all types) was 3% (95% CI 1% to 5%) and 5% (95% CI 4% to 8%), respectively. Thirty-day and 1-year mortality was found to be 1% (95% CI 0% to 2%) and 2% (95% CI 0% to 4%), respectively. The combined incidence of stroke or death was 5% (95% CI 3% to 8%) at 30 days, and 9% (95% CI 7% to 12%) at 1 year. CONCLUSION: The findings suggest that SA might be a promising alternative treatment in the treatment of symptomatic ICAD due to its favorable technical profile, periprocedural safety, and long-term efficacy. A randomized clinical trial is warranted to compare the safety and efficacy of SA with 'gold standard' medical treatment.

Procedural success with radial access for carotid artery stenting: systematic review and meta-analysis.

BACKGROUND: Femoral access is the traditional approach for endovascular carotid artery stenting. Radial access is increasingly used as an alternative approach due to its known anatomical advantages in patients with unfavorable aortic arch morphology via the femoral approach and its excellent access site safety profile. Our objective was to analyze procedural success using radial access for carotid artery stenting as reported in the literature. METHODS: Three online databases were systematically searched following PRISMA guidelines for studies (n ≥20) using radial artery access for carotid artery stenting (1999-2018). Random-effects meta-analysis was used to pool the procedural success (successful stent placement with no requirement for crossover to femoral access), mortality, and complication rates associated with radial access. RESULTS: Seven eligible studies reported procedural success outcomes with a pooled meta-analysis rate of 90.8% (657/723; 95% CI 86.7% to 94.2%; I2=53.1%). Asymptomatic radial artery occlusion occurred in 5.9% (95% CI 4.1% to 8.0%; I2=0%) and forearm hematoma in 1.4% (95% CI 0.4% to 2.9%; I2=0%). Risk of minor stroke/transient ischemic attack was 1.9% (95% CI 0.6% to 3.8%; I2=42.3%) and major stroke was 1.0% (95% CI 0.4% to 1.8%; I2=0%). There were three deaths across the seven studies (0.6%; 95% CI 0.2% to 1.3%; I2=0%). The meta-analysis was limited by statistically significant heterogeneity for the primary outcome of procedural success. CONCLUSION: Radial access for carotid artery stenting has a high procedural success rate with low rates of mortality, access site complications, and cerebrovascular complications. The potential benefits of this approach in patients with unfavorable aortic arch access should be explored in a prospective randomized trial.

Carotid Endarterectomy and Carotid Artery Stenting for Patients With Crescendo Transient Ischemic Attacks: A Systematic Review.

Importance: Thromboembolic stroke attributable to an ipsilateral carotid artery plaque is a leading cause of disability in the United States and a major source of morbidity. Randomized clinical trials have demonstrated the efficacy of carotid endarterectomy and carotid stenting at minimizing stroke risk in patients with minor stroke and transient ischemic attack. However, there is no consensus on guidelines for medical management and the timing of revascularization in patients with multiple recurrent episodes of transient ischemic attack over hours or days, an acute neurological event known as crescendo transient ischemic attack. Objective: To review the management of and timing of intervention in patients presenting with crescendo transient ischemic attack. Evidence Review: This systematic review included all English-language articles published from January 1, 1985, to January 1, 2019, available from PubMed (MEDLINE) and Google Scholar. Articles were excluded if they did not include analysis of patients with symptoms, did not report the timing of intervention after crescendo transient ischemic attack, or mixed analysis of patients with stroke in evolution with patients with crescendo transient ischemic attack. The quality of the evidence was assessed with the modified rating from the Oxford Centre for Evidence-based Medicine. Observations: Patients with crescendo transient ischemic attack were found to have a higher risk of stroke or death after carotid endarterectomy compared with patients with a single transient ischemic attack or stable stroke. With medical therapy alone, a considerable number of patients with crescendo transient ischemic attack experience a completed stroke within several months and have a poor prognosis without intervention. Urgent carotid endarterectomy, typically performed within 48 hours of initial presentation, is beneficial in carefully selected patients. There have been several reports of operative treatment within the first 24 hours of presentation; however, review of these reports does not show any additional benefit from emergency treatment. Carotid artery stenting is reserved only for selected patients with prohibitive surgical risk for endarterectomy. The literature does not clearly support any additional benefit of intravenous heparin therapy over mono or dual antiplatelet therapy prior to carotid endarterectomy. Conclusions and Relevance: Crescendo transient ischemic attack is best managed with optimal medical management as well as urgent carotid endarterectomy within 2 days of presentation. Surgical endarterectomy appears to be preferred because of the increased embolic potential of bifurcation plaque, whereas stenting is an option for patients with contraindications for surgery. With ongoing advances in cerebrovascular imaging and medical treatment of stroke, there is a need for better evidence to determine the optimal timing and preoperative medical management of patients with crescendo transient ischemic attack.

Multiple Burr-Hole Surgery for the Treatment of Moyamoya Disease and Quasi-Moyamoya Disease in Children: Preliminary Surgical and Imaging Results.

OBJECTIVE: Moyamoya disease (MMD) is characterized by a progressive spontaneous occlusion of distal internal carotid arteries and its main branches. It is necessary to promptly diagnose and treat this condition, especially in children, because of the high risk of stroke and consequent severe disability. In this study, we examine the role of multiple burr-hole surgery (MBHS) in the treatment of pediatric patients with MMD and quasi-MMD and the role of perfusion magnetic resonance imaging (MRI) in the surgical indication and during follow-up. METHODS: We reviewed preoperative, early postoperative, and late postoperative MRI perfusion and digital subtraction angiography images together with clinical and surgical outcomes in 10 children with MMD treated by MBHS. RESULTS: Fourteen MBHS procedures (6 unilateral, 2 bilateral, and 2 single-setting bilateral) were performed in 10 children. Clinical and radiologic follow-up for all patients ranges from 16 months to 7 years. No ischemic events (transient ischemic attacks or stroke) occurred during the follow-up period. Postoperative digital subtraction angiography showed good revascularization around the burr-hole sites in all patients. The functional efficacy of the surgery was confirmed by dynamic susceptibility contrast perfusion MRI studies in 8 patients. CONCLUSIONS: Our data underline the good rate of success of MBHS in pediatric MMD and quasi-MMD. This technique results in good collateral revascularization, improved cerebral perfusion and excellent short and long-term symptoms control, with low perioperative risk. Postoperative changes of perfusion parameters and ivy sign MRI finding seem to show a positive correlation and mainly occur in posterior middle cerebral artery territory.

Genotype-Phenotype Correlation in Long-Term Cohort of Japanese Patients with Moyamoya Disease.

BACKGROUND: Homozygosity of this p.R4810K founder variant of RNF213moyamoya disease (MMD) susceptibility gene is known to influence the severity of the clinical disease phenotype at disease onset. However, the association between this genotype and long-term clinical manifestations has remained unclear. OBJECTIVES: The principal goal of this study was to investigate whether and how the p.R4810K variant of RNF213influences the long-term phenotype in Japanese patients with MMD. METHOD: This retrospective cohort study included 94 Japanese patients with MMD who underwent direct or combined bypass for revascularization with the p.R4810K genotype determined in our hospital. The following phenotypic parameters were analyzed at disease onset and over a long-term period: age and initial presentation at onset, recurrent stroke after initial revascularization, and final modified Rankin Scale. RESULTS: The p.R4810K genotype was significantly associated with the phenotype at onset, especially in younger patients. Over a median follow-up period of 100 months, recurrent stroke occurred in 6 out of 94 patients: none out of 5 patients with the homozygous variant, 5 out of 64 with the heterozygous variant, and 1 out of 25 in the wild-type group. There were no significant differences among the genotypes. In particular, recurrent cerebral hemorrhage occurred in 5 patients, all possessing the heterozygous variant. The log-rank test showed no difference between the genotypes in the stroke-free survival rate. Furthermore, the p.R4810K genotype was not associated with a poor functional condition. CONCLUSIONS: The p.R4810K founder variant of RNF213 affects the phenotype at disease onset. However, the optimal revascularization may be effective, regardless of the genotype, even for the homozygous variant, which has been thought to be the most pathogenic. This genotype may not strongly influence the long-term clinical manifestations or poor prognosis in MMD.

Endovascular Recanalization of Symptomatic Intracranial Arterial Stenosis Despite Aggressive Medical Management.

BACKGROUND: The optimal management of intracranial arterial stenosis is unclear, particularly in patients who have failed medical management. We report a multicenter real-world experience of endovascular recanalization of intracranial atherosclerotic stenosis refractory to aggressive medical therapy. METHODS: Retrospective multicenter case series of consecutive endovascularly treated patients presenting with symptomatic (transient ischemic attack [TIA] or stroke) intracranial stenosis who had failed medical therapy. Patients were divided into 2 groups: patients with recurrent TIA or stroke despite medical management (group 1) versus patients presenting with a stroke and worsening symptoms (progressive or crescendo stroke) despite medical management (group 2). RESULTS: A total of 101 patients were treated in 8 stroke centers from August 2009 to May 2017. Sixty-nine presented with recurrent TIA or stroke and 32 with stroke and worsening symptoms. Successful recanalization was achieved in 84% of patients. Periprocedural stroke occurred in 3 patients and 2 had a recurrent ischemic stroke at the 90-day follow-up. Symptomatic intraparenchymal hemorrhage secondary to reperfusion injury occurred in 3 patients and 1 had a hemorrhagic stroke after discharge. There were 2 periprocedural perforations that resulted in death. At 90 days, 86% of patients (64/74) did not have a recurrence of stroke and the 90-day cumulative ischemic stroke rate was 6.7% with 90-day mortality of 11.2%. The 90-day favorable outcome (modified Rankin Scale score, ≤2) rate was 77.5%. CONCLUSIONS: Endovascular recanalization of unstable intracranial atherosclerotic stenosis in patients who have failed medical therapy is feasible. Future randomized trials need to determine if recanalization is of any value for this population.