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BACKGROUND: Von Hippel-Lindau (VHL) disease is a rare autosomal dominant disorder that predisposes patients to develop multiple cysts and tumors, such as hemangioblastomas (HBs) and clear cell renal cell carcinoma (ccRCC), due to mutations in the VHL tumor suppressor gene. While treatment of HBs varies based on their characteristics and has improved patient survival, it still involves high morbidity and mortality, leading to ongoing debates and studies to refine therapy strategies. Recent developments include the emergence of Belzutifan, a novel inhibitor targeting hypoxia-inducible factor 2α (HIF-2α), which has shown promising results in ongoing trials, particularly for patients not immediately requiring surgery. METHODS: This systematic review and meta-analysis aimed to comprehensively evaluate the efficacy and safety of Belzutifan for treating HBs associated with VHL disease. Search was conducted across Medline, Embase, Cochrane, and Web of Science databases. Statistical Analysis was performed, with proportions and 95 % confidence intervals. Statistical analyses were carried out using R Studio. RESULTS: Ten studies were selected, comprising 553 patients. The population mean age was 40 (24-65), and 50 % of the population was formed by males. In terms of proportion, 6 analyses were performed: Disease Stability of 31 % [95 %CI:14 %-47 %; I2 = 2 %]; Disease Progression of 2 %[95 %CI:0 %-9 %; I2 = 0 %]; Partial Response of 75 % [95 %CI:54 %-96 %; I2 = 58 %]. Complete response of 1 % [95 %CI:0 %-7 %; I2 = 0 %];and Side effects, anemia 81 % rate [95 % CI:54 %-100 %; I2 = 94 %], and fatigue rate of 79 % [95 % CI:54 %-100 %;I2 = 94 %]. CONCLUSION: Results indicate that Belzutifan effectively stabilizes disease, reduces tumor progression, and achieves significant therapeutic responses, although side effects like anemia and fatigue were noted.

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OBJECTIVE: Unruptured middle cerebral artery aneurysm (uMCAA) has traditionally been treated with open surgical clipping (SC). Endovascular treatments (EVTs) were designed to reduce surgical risks in these cases. Nevertheless, despite its potential benefits, many surgeons favor SC for uMCAA. This updated meta-analysis aimed to compare the safety, efficacy, and clinical outcomes of SC and EVT for uMCAA. METHODS: The authors searched the Medline, Embase, and Cochrane Library databases according to the Cochrane and PRISMA guidelines. Eligible studies included those with ≥ 4 patients with uMCAA reporting comparative data of SC and EVT. The endpoints were the complete occlusion rate (Raymond class I and II), good clinical outcomes (modified Rankin Scale score ≤ 2 or Glasgow Outcome Scale score ≥ 4), procedure-related complications (further divided into major and minor), and mortality. The authors pooled OR with 95% CI values with a random-effects model. I2 statistics were used to assess heterogeneity, and sensitivity analysis was conducted to address high heterogeneity. Publication bias was assessed with funnel plot analysis and the Egger's test. RESULTS: The analysis included data from 10 studies. Regarding the complete occlusion assessment, the comparative analysis revealed OR 0.17 (95% CI 0.08-0.40, p < 0.01), favoring SC. In terms of achieving good clinical outcomes, OR 0.44 (95% CI 0.20-0.97, p < 0.05) was determined, favoring SC. No differences regarding total procedure-related complications, major complications, or mortality were identified. However, a higher likelihood of minor complications was identified for EVT, with OR 4.68 (95% CI 2.01-10.92, p < 0.01). CONCLUSIONS: This systematic review and meta-analysis identified a lower likelihood of complete occlusion at last follow-up and lower likelihood of good clinical outcomes in patients treated with EVT when compared with SC. Furthermore, a higher likelihood of minor complications was identified in patients who underwent EVT when compared with SC. The findings reinforce that, based on the currently available data, SC should be considered the primary approach for treating uMCAA. However, EVT is an evolving approach, and this study's findings represent a synthesis of observational studies. Randomized trials are warranted to elucidate which approach should be the mainstay for uMCAA and to identify the nuances that determine whether SC or EVT is more or less indicated for addressing uMCAA with consideration of the individuality of each patient and aneurysm.

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INTRODUCTION: Conventionally, one branch of the superficial temporal artery (STA) is utilized to revascularize the middle cerebral artery (MCA). However, there is the possibility of utilizing both branches of the STA when performing the bypass, characterizing the double-barrel (DB) STA-MCA bypass. Notably, a lack of studies evaluating this technique led the authors to conduct a systematic review and single-arm meta-analysis. METHODS: PubMed, Embase and Web of Science were searched systematically for publications of DB-STA-MCA bypass on November 1st, 2023. The findings were reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Case reports were not included for statistical analysis purposes. RESULTS: The review included 408 patients and 534 bypasses from 34 studies. The main etiology was Moyamoya disease (64.6%), followed by cerebral ischemia (22.2%) and aneurysms (12.5%). The median of the mean follow-ups of each study was 12.8 months (range 1.5-87.9). The postoperative patency was 100%. The follow-up patency was 98% (95% CI: 96%-100%; I2 = 0%). The procedure-related mortality was 0% (95% CI: 0%-1%; I2 = 0%). Aneurysms obtained 87% (95% CI: 72%-100%; I2 = 4%) of good clinical outcomes, while Moyamoya disease yielded a rate of 70% (95% CI: 10%-100%; I2 = 97%). Ischemic complications occurred at a rate of 6% (95% CI: 2%-11%; I2 = 36%), while hemorrhagic occurred at 6% (95% CI: 1%-11%; I2 = 56%). Hyperperfusion syndrome rate was calculated as 18% (7%-30%; I2 = 55%) for Moyamoya disease. CONCLUSIONS: The procedure appears to be safe, with excellent patency rates. The clinical efficacy for ischemic and Moyamoya diseases warrants further standardized robust investigation with a broader number of patients, and aneurysm studies are required to enhance sample sizes. The main complication for the Moyamoya subgroup is hyperperfusion syndrome.

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Extramedullary plasmacytoma (EMP) is an uncommon disorder characterized by the development of abnormal plasma cell tumors outside the bone marrow. These tumors are typically observed in various locations, including the upper respiratory tract, gastrointestinal tract, and other soft tissues. Among the less explored manifestations of EMP is intracranial EMP, which remains poorly understood due to the limited literature available on the subject. The objective was to comprehend the population characteristics, localization, type, treatment, and outcomes of intracranial EMP. A systematic review of the literature for EMPs was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. The strategy "extramedullary plasmacytoma AND multiple myeloma" was used for the search. The search terms were queried using PubMed, Embase, Scopus, Cochrane, and Web of Science databases. We included only those studies that presented clinical studies with patients diagnosed with intracranial plasmacytomas. In this study, a total of 84 patients from 25 studies were analyzed. The average age of diagnosis was 57.25 years, with a slightly higher proportion of females (57%) compared to male patients (43%). The most common locations of intracranial plasmacytomas were the clivus (29.7%), frontal lobe (18.9%), parietal lobe (8.1%), occipital lobe (6.7%), temporal lobe (6.7%), and sphenoid (4%). Chordoma and meningioma were the most common differential diagnoses encountered during clinical investigations. Treatment modalities included radiotherapy (RT), chemotherapy (QT), surgical resection (SR), and conservative approaches. The most frequent treatment combinations were SR + RT (19%) and RT only (17.8%). Mortality was reported in 48% of the cases, with complete resolution observed in 10 cases and partial resolution in 3 cases. The average follow-up duration was 37.5 months. The clivus is the most frequently reported site of extramedullary intracranial plasmacytoma (EMIP) occurrence, representing 29.7% of cases. Chordomas were commonly observed alongside EMIPs and emerged as the primary differential diagnosis. RT was the predominant treatment modality, with SR considered when feasible. RT alone demonstrated the highest effectiveness in managing EMIPs (30%), while QT as a sole intervention showed lower efficacy. However, a combination of dexamethasone, lenalidomide, and targeted RT displayed promising results, offering improved tumor response and increased safety.

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Osteoporotic vertebral fractures frequently result in pain and decreased quality of life (QoL). The management of these fractures remains a topic of debate. Following Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines, we analyzed randomized controlled trials comparing percutaneous vertebroplasty (PV) with non-operative treatment (non-OT). The outcomes of interest included pain, QoL, cement leakage, and new osteoporotic vertebral fractures after 1 year. Compared to non-OT regarding pain relief, PV yielded significant improvement at 1-2 weeks, 1 month, 6 months (standard mean difference [SMD] = -0.67 (6/14; 95% confidence interval [CI]: -1.29 to -0.06; I2 = 92%, random effects) and 1 year (mean difference = -1.07 (4/14; 95% CI: -1.97 to -0.18; I2 = 97%, random effects). For QoL, notable improvements were observed at 1 week (standard mean difference = -2.10 (5/14; 95% CI: -3.77 to -0.42; I2 = 98%, random effects) and 3 months (mean difference = -1.58 (4/14; 95% CI: -3.07 to -0.09; I2 = 96%, random effects), with 1 month, 6 months and 1 year being inconclusive. A cement leakage rate of 42% (10/14; 95% CI: 25% to 59%; I2 = 99%, random effects) was found. Further, PV did not significantly heighten the risk of new fractures within a year (odds ratio = 1.26 (6/14; 95% CI: 0.63 to 2.53; I2 = 74%, random effects). PV emerges as a promising intervention for specific time intervals regarding pain relief, especially in the extended-term analysis, and QoL, especially in the short-term analysis, compared to non-OT. However, clinicians must consider cement leakage risks. Heterogeneity among studies underscores careful patient selection.

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INTRODUCTION: Small and very small anterior communicating artery (ACoA) aneurysms pose a complex challenge in neurosurgery and interventional neuroradiology due to their critical location and potential for severe consequences upon rupture. Surgical clipping has been a traditional approach, but it presents challenges requiring precision and expertise. Endovascular treatment has emerged as an alternative, offering minimally invasive techniques with potential advantages. This study aims to comprehensively compare outcomes and efficacy between surgical clipping and endovascular treatment for small or very small ACoA aneurysms. OBJECTIVES: We aimed to perform a meta-analysis of small or very small anterior communicating artery aneurysms comparing surgical clipping and endovascular treatments. METHODS: A systematic review and meta-analysis were conducted, including studies reporting on both treatment modalities. Eligible studies were identified through PubMed, Cochrane Library, and Embase databases. Pooled analyses with 95% confidence intervals were used to compare treatment effects, and statistical analysis followed PRISMA guidelines. RESULTS: Thirteen studies with 637 patients were included. Endovascular treatment, predominantly coiling, was performed in 60.3% of patients, while 39.7% underwent surgical management. Endovascular treatment exhibited an 18% retreatment rate, contrasting with 0% in the surgery group. Mortality rates were 3% and 6% for endovascular and surgical treatments, respectively. Overall complications occurred in 1.8% of patients, with intraoperative rupture and cerebral infarction being the most common. CONCLUSION: In summary, our study indicates a comparable outcome between surgical clipping and endovascular treatment for small ACoA aneurysms, with the former showing a lower retreatment rate. Decision factors include surgeon expertise, healthcare context, and patient age. Further research is needed to refine treatment strategies, considering variations in aneurysm status and evolving techniques.

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Background: Open cell stents (OC) and closed cell stents (CC) each offer unique advantages and potential drawbacks in the context of idiopathic intracranial hypertension (IIH) treatment. We aim to investigate the safety and efficacy of using OC and CC for IIH.Methods: We conducted a systematic review in PubMed, Embase, and Cochrane Library databases following the PRISMA guidelines. Eligible studies included ≥4 patients with IIH treated by OC or CC. Primary outcomes were headache, visual acuity, and papilledema status before and after the procedure. Additionally, failure rate, minor complications, major complications, and total complications were assessed. Pooled analysis of the OC group and CC group were done separately and then compared.Results: Twenty-four studies were included. Of these, 20 reported on OC and 6 reported on CC. Pooled analysis of failure rate was 8% (4%-12%) in OC and 5% (0%-11%) in CC. For headache improvement rate: 78% (70%-86%) in OC and 81% (66%-69%) in CC. For visual acuity improvement: 78% (65%-92%) in OC and 76% (29%-100%) in CC. For papilledema improvement: 88% (77%-98%) in OC and 82% (67%-98%) in CC. For minor complications: 0% (0%-1%) in OC and 0% (0%-2%) in CC. For major complications: 0% (0%-1%) in OC and 2% (0%-6%) in CC. Total complications: 0% (0%-1%) in OC and 2% (0%-6%) in CC.Conclusion: Low failure and complication rates were found in both OC and CC, with no significant difference between them in effectiveness. The CC showed a slight but significant increase in major and total complications compared to the OC. Additionally, a subtle yet significantly lower failure rate was identified in the CC.

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Neuroendoscopy (NE) surgery emerged as a promising technique for the treatment of spontaneous intracerebral hemorrhage (ICH). A previous meta-analysis of randomized controlled trials (RCTs) analyzed the efficacy and safety of NE compared to craniotomy, but NE did not present a significant improvement in functional outcomes. However, a new study provided an opportunity to update the current knowledge. We searched PubMed, Embase, and Cochrane Central Register of Controlled Trials for RCTs reporting NE evacuation of spontaneous supratentorial ICH compared to craniotomy. The efficacy outcomes of interest were favorable functional outcome, functional disability, hematoma evacuation rate, and residual hematoma volume. The safety outcomes of interest were rebleeding, infection, and mortality. Seven RCTs were included containing 879 patients. The NE approach presented a significantly higher rate of favorable functional outcome compared with craniotomy (RR: 1.42; 95% CI 1.17, 1.73; p < 0.001). The evacuation rate was higher in patients who underwent the NE approach (MD: -8.36; 95% CI -12.66, -4.07; p < 0.001). NE did not show a benefit in improving the mortality rate (RR: 0.81, 95% CI 0.54, 1.22; p = 0.32). NE was associated with more favorable functional outcomes and lower rates of functional disabilities compared to craniotomy. Also, NE was superior regarding evacuation rate, while presenting a reduction in residual hematoma volume. NE might be associated with lower infection rates. Mortality was not improved by NE surgery. Larger, higher-quality randomized studies are needed to adequately evaluate the efficacy and safety of NE compared to craniotomy.

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Dural Arteriovenous Fistulas (dAVFs) of the anterior cranial fossa (ACF) are uncommon but carry a high risk of hemorrhage and pose substantial treatment challenges. Recent advancements in endovascular treatment (EVT), including the introduction of novel liquid embolic agents, have markedly bolstered EVT's role in managing ACF-dAVFs, with notable series published in the last five years. We aimed to assess the feasibility, safety, and efficacy of EVT for ACF-dAVFs. We searched Medline, Scopus, Web of Science, and Cochrane Library databases following PRISMA guidelines. Eligible studies included those with ≥ 5 patients undergoing embolization of ACF-dAVFs, detailing both angiographic and clinical outcomes. We used single proportion analysis with 95% confidence intervals under a random-effects model, I2 to assess heterogeneity, and Baujat and sensitivity analysis to address high heterogeneity. Publication bias was assessed by funnel-plot analysis and Egger's test. Outcomes included complete occlusion following embolization, unsuccessful endovascular embolization attempts, incomplete occlusion following embolization, symptom resolution or clinical improvement following embolization, recurrence; procedure-related complications, morbidity, and mortality. Additionally, a subanalysis for studies exclusively utilizing Onyx™ embolic system was done. Eighteen studies comprising 231 ACF-dAVF were included. Unsuccessful endovascular embolization attempts rate was 2%. Complete occlusion rate was 85%, with 4% of complications. Incomplete occlusion rate was 10%. Successfully embolized patients experienced either symptom resolution or clinical improvement in 94% of cases. Morbidity and mortality rates were 1% and 0%, respectively. Onyx subanalyses showed an overall rate of 0% for unsuccessful attempts, 95% for complete occlusion, and 5% for incomplete occlusion. Symptom resolution or clinical improvement was 98% and recurrence rate was 0%. EVT for ACF-dAVF is highly feasible, effective, and safe, with a low rate of complications, morbidity, and mortality. The subanalyses focusing on Onyx embolizations revealed superior efficacy and safety outcomes compared to the findings of the primary analyses involving all included studies.

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