malformation

畸形
  • 文章类型: Journal Article
    背景:发情期很少见,但众所周知,脑动脉的血管变异。它们大多是偶然的,无症状的血管造影结果,可能会导致血管病变,如AVM,动脉瘤扩张,甚至缺血症状.然而,动脉开窗术与脑动脉瘤之间的关联尚未明确。目的:评估动脉开窗的发生率是否与脑动脉瘤的发展有关,并研究人脑动脉开窗的患病率和最常见的定位。设计:病例对照研究。设置:所有在大学医院行CT血管造影检查的患者。4从2009年到2019年在卢布林。患者:病例组包括每位显示至少一个脑动脉瘤的患者,对照组包括每位CT血管造影无脑动脉瘤的患者。测量:CT血管造影检查使用放射科第一部门使用的标准协议进行,卢布林医科大学,波兰。使用Statistica软件(ver。13.3,TibcoSoftwareInc.,帕洛阿尔托,CA,美国)。结果:本研究共纳入6545例CTA检查。大多数动脉瘤位于MCA:629(38.59%),ICA:466(28.59%)和AComA:192(11.78%)。在整个研究人群中,脑动脉开窗术显示脑动脉瘤的风险无统计学意义(OR:1.157;95%CI:0.826-1.621;p=0.39)。在6545项头颅CTA检查中,其中49人发现了脑血管开窗,占0.75%。最常见的血管开窗是位于ACA中的开窗(30.61%),BA(30.61%)和AComA(22.45%),而其他开窗很少发生。血管开窗术患者的年龄差异无统计学意义(p>0.05)。VA开窗术在男性(16.67%)比女性(5.41%)更为常见。然而,这些差异没有统计学意义(p=0.216).局限性:我们的研究有几个局限性,包括关于被检查人群的选择偏差。第二,我们假设,与其他放射学方法相比,由于CT方法的局限性,我们的研究中检测到的开窗总数被低估了.结论:脑动脉开窗是一种罕见的血管畸形。ACA是最常见的开窗定位,其次是BA和AComA。脑动脉的开窗没有显着增加脑动脉瘤形成的风险。需要进一步的前瞻性研究才能使这种关联更加精确。
    Background: Fenestrations are rare, but well-known, vascular variations of the cerebral arteries. They are mostly incidental, asymptomatic angiographic findings and might precipitate vascular lesions such as AVM, aneurysmal dilatation, or even ischemic symptoms. However, association between arterial fenestration and brain aneurysms has not been clearly established. Objective: To evaluate whether incidence of arterial fenestrations are associated with brain aneurysm development and investigate the prevalence and most-common localizations of arterial fenestrations of the human brain. Design: Case−control study. Setting: All patients examined by CT angiography in University Hospital No. 4 in Lublin from 2009 to 2019. Patients: Each patient showing at least one cerebral aneurysm was included in the case group and each patient without cerebral aneurysm on CT angiography was included in the control group. Measurements: CT angiography examinations were conducted using the standard protocol used in the 1st Department of Radiology, Medical University of Lublin, Poland. The database and statistical research were conducted by use of the Statistica software (ver. 13.3, Tibco Software Inc., Palo Alto, CA, USA). Results: A total of 6545 CTA examinations were included in the study. Most of the aneurysms were located on the MCA: 629 (38.59%), ICA: 466 (28.59%) and AComA: 192 (11.78%). Cerebral arterial fenestration showed a non-statistically significant elevated risk for brain aneurysms in the entire study population (OR: 1.157; 95% CI: 0.826−1.621; p = 0.39). Among 6545 cranial CTA examinations, cerebral vessel fenestration was found in 49 of them, which constituted 0.75%. The most common vascular fenestrations were those located in the ACA (30.61%), BA (30.61%) and AComA (22.45%), while other fenestrations occurred infrequently. There were no significant differences in the age of patients in the individuals with vascular fenestration (p > 0.05). VA fenestration was slightly more common in men (16.67%) than in women (5.41%). However, these differences were not statistically significant (p = 0.216). Limitations: Our study has several limitations, including selection bias regarding examined population. Second, we assume that the total number of fenestrations detected in our study was underestimated due to the limitations of the CT method in comparison to other radiologic modalities. Conclusions: Cerebral arterial fenestrations are rare vascular malformations. The ACA is the most common localization of fenestrations, followed by BA and AComA. Fenestrations of cerebral arteries insignificantly increase the risk of cerebral aneurysm formation. Further prospective studies are necessary to make this association more precise.
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  • 文章类型: Journal Article
    背景:这篇综述的目的是总结血管造影和栓塞在脑动静脉畸形的综合多学科管理中的作用的可用数据(AVM方法:我们进行了结构化文献综述,以研究检查适应症,功效,以及在脑AVM管理背景下接受血管内治疗的患者的结局。我们对证据的质量进行了评级。建议是通过作者的共识会议达成的,然后由神经介入外科学会(SNIS)标准和指南委员会和SNIS董事会提供额外的投入。
    结果:脑AVM的多学科评估和治疗不断发展。建议包括:(1)数字减影导管脑血管造影(DSA)-包括2D,3D,并在适当时重新格式化横截面视图-建议在治疗前评估大脑AVM。(I,B-NR)。(2)建议在完整的多学科治疗计划的背景下进行脑动静脉畸形的血管内栓塞,以消除AVM并治愈。(I,B-NR)。(3)手术切除前脑AVM栓塞可以有用削减术中出血量,发病率,和手术复杂性。(IIa,B-NR)。(4)脑动静脉畸形的原发性治愈性栓塞的作用尚不确定,特别是与有或没有辅助栓塞的显微外科和放射外科相比。需要进一步的研究,特别是关于AVM复发的风险。(III模棱两可,C-LD)。(5)针对脑动静脉畸形破裂的高危特征进行靶向栓塞治疗可降低再出血的风险。(IIb,C-LD)。(6)姑息性栓塞可能对治疗有症状的AVM有用,否则无法进行治愈性治疗。(IIb,B-NR)。(7)AVM栓塞作为放射外科辅助手段的作用尚未明确。需要进一步的研究。(III模棱两可,C-LD)。(8)建议在脑AVM明显治愈后进行影像学随访以评估复发。尽管非侵入性成像可用于纵向随访,在有影像学和/或临床表现的患者中,DSA仍然是残留或复发性AVM检测的金标准。(I,C-LD)。(9)改进了所有年龄的脑动静脉畸形患者的国家和国际报告,他们的治疗,治疗的副作用,他们的长期结果将增强进行临床试验的能力,并提高对这种罕见疾病的研究的严谨性。(I,C-EO)。
    结论:尽管证据质量低于接受多项随机对照试验的更常见条件,血管内治疗在脑动静脉畸形的管理中具有重要作用,需要进行前瞻性研究以加强支持这些建议的数据.
    BACKGROUND: The purpose of this review is to summarize the data available for the role of angiography and embolization in the comprehensive multidisciplinary management of brain arteriovenous malformations (AVMs METHODS: We performed a structured literature review for studies examining the indications, efficacy, and outcomes for patients undergoing endovascular therapy in the context of brain AVM management. We graded the quality of the evidence. Recommendations were arrived at through a consensus conference of the authors, then with additional input from the full Society of NeuroInterventional Surgery (SNIS) Standards and Guidelines Committee and the SNIS Board of Directors.
    RESULTS: The multidisciplinary evaluation and treatment of brain AVMs continues to evolve. Recommendations include: (1) Digital subtraction catheter cerebral angiography (DSA)-including 2D, 3D, and reformatted cross-sectional views when appropriate-is recommended in the pre-treatment assessment of cerebral AVMs. (I, B-NR) . (2) It is recommended that endovascular embolization of cerebral arteriovenous malformations be performed in the context of a complete multidisciplinary treatment plan aiming for obliteration of the AVM and cure. (I, B-NR) . (3) Embolization of brain AVMs before surgical resection can be useful to reduce intraoperative blood loss, morbidity, and surgical complexity. (IIa, B-NR) . (4) The role of primary curative embolization of cerebral arteriovenous malformations is uncertain, particularly as compared with microsurgery and radiosurgery with or without adjunctive embolization. Further research is needed, particularly with regard to risk for AVM recurrence. (III equivocal, C-LD) . (5) Targeted embolization of high-risk features of ruptured brain AVMs may be considered to reduce the risk for recurrent hemorrhage. (IIb, C-LD) . (6) Palliative embolization may be useful to treat symptomatic AVMs in which curative therapy is otherwise not possible. (IIb, B-NR) . (7) The role of AVM embolization as an adjunct to radiosurgery is not well-established. Further research is needed. (III equivocal, C-LD) . (8) Imaging follow-up after apparent cure of brain AVMs is recommended to assess for recurrence. Although non-invasive imaging may be used for longitudinal follow-up, DSA remains the gold standard for residual or recurrent AVM detection in patients with concerning imaging and/or clinical findings. (I, C-LD) . (9) Improved national and international reporting of patients of all ages with brain AVMs, their treatments, side effects from treatment, and their long-term outcomes would enhance the ability to perform clinical trials and improve the rigor of research into this rare condition. (I, C-EO) .
    CONCLUSIONS: Although the quality of evidence is lower than for more common conditions subjected to multiple randomized controlled trials, endovascular therapy has an important role in the management of brain AVMs. Prospective studies are needed to strengthen the data supporting these recommendations.
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  • 文章类型: Journal Article
    The zebrafish as an alternative animal model for developmental toxicity testing has been extensively investigated, but its assay protocol was not harmonized yet. This study has validated and optimized the zebrafish developmental toxicity assay previously reported by multiple inter-laboratory studies in the United States and Europe. In this study, using this classical protocol, of 31 ICH-positive compounds, 23 compounds (74.2%) were teratogenic in zebrafish, five had false-negative results, and three were neither teratogenic nor non-teratogenic according to the protocol standard; of 14 ICH-negative compounds, 12 compounds (85.7%) were non-teratogenic in zebrafish and two had false-positive results. After we added an additional TI value in the zebrafish treated with testing compounds at 2 dpf along with the original 5 dpf, proposed a new category as the uncategorized compounds for those TI values smaller than the cutoff both at 2 dpf and 5 dpf but inducing toxic phenotypes, refined the testing concentration ranges, and optimized the TI cut-off value from ≥ 10 to ≥ 3 for compounds with refined testing concentrations, this optimized zebrafish developmental assay reached 90.3% sensitivity (28/31 positive compounds were teratogenic in zebrafish) and 88.9% (40/45) overall predictability. Our results from this study strongly support the use of zebrafish as an alternative in vivo method for screening and assessing the teratogenicity of candidate drugs for regulatory acceptance.
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  • 文章类型: Journal Article
    背景:尽管有许多关于脑海绵状畸形(CCMs)的出版物,关于诊断和管理策略仍存在争议。
    目的:制定CCM管理指南。
    方法:血管瘤联盟(www.angioma.org),美国的患者支持小组代表患者和CCM研究,召集了一个由专家CCM临床医生组成的多学科写作小组,以帮助总结与CCM临床护理相关的现有文献,重点关注5个主题:(1)流行病学和自然史,(2)基因检测和咨询,(3)诊断标准和放射学标准,(4)神经外科方面的考虑,(5)神经方面的考虑。该小组审查了文献,评级证据,提出的建议,并建立了共识,争议,和知识差距,根据预先指定的协议。
    结果:在1983年1月1日至2014年9月31日之间发表的1270份出版物中,我们根据方法学标准选择了98份,并确定了另外38种最新或相关出版物。主题作者使用这些出版物来总结当前知识,并得出23个共识管理建议,我们根据美国心脏协会/美国中风协会的标准,按等级(影响大小)和等级(确定性估计)进行评级。没有建议是A级(因为没有随机对照试验),11(48%)为B级,12(52%)为C级。8(35%)的建议为I级,10人中的II类(43%),和III类5(22%)。
    结论:当前证据支持CCM管理的建议,但他们的水平和类别普遍较低,需要进一步研究,以更好地为临床实践提供信息并更新这些建议。完整的建议文件,包括选择参考引文的标准,对各自建议的更详细的理由,以及争议和知识差距的总结,进行了类似的同行评审,并可在线www。angioma.org/CCMGuidelines.
    BACKGROUND: Despite many publications about cerebral cavernous malformations (CCMs), controversy remains regarding diagnostic and management strategies.
    OBJECTIVE: To develop guidelines for CCM management.
    METHODS: The Angioma Alliance ( www.angioma.org ), the patient support group in the United States advocating on behalf of patients and research in CCM, convened a multidisciplinary writing group comprising expert CCM clinicians to help summarize the existing literature related to the clinical care of CCM, focusing on 5 topics: (1) epidemiology and natural history, (2) genetic testing and counseling, (3) diagnostic criteria and radiology standards, (4) neurosurgical considerations, and (5) neurological considerations. The group reviewed literature, rated evidence, developed recommendations, and established consensus, controversies, and knowledge gaps according to a prespecified protocol.
    RESULTS: Of 1270 publications published between January 1, 1983 and September 31, 2014, we selected 98 based on methodological criteria, and identified 38 additional recent or relevant publications. Topic authors used these publications to summarize current knowledge and arrive at 23 consensus management recommendations, which we rated by class (size of effect) and level (estimate of certainty) according to the American Heart Association/American Stroke Association criteria. No recommendation was level A (because of the absence of randomized controlled trials), 11 (48%) were level B, and 12 (52%) were level C. Recommendations were class I in 8 (35%), class II in 10 (43%), and class III in 5 (22%).
    CONCLUSIONS: Current evidence supports recommendations for the management of CCM, but their generally low levels and classes mandate further research to better inform clinical practice and update these recommendations. The complete recommendations document, including the criteria for selecting reference citations, a more detailed justification of the respective recommendations, and a summary of controversies and knowledge gaps, was similarly peer reviewed and is available on line www.angioma.org/CCMGuidelines .
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  • 文章类型: Journal Article
    Epilepsy surgery is an effective treatment in many patients with drug-resistant focal epilepsies. An early decision for surgical therapy is facilitated by a magnetic resonance imaging (MRI)-visible brain lesion congruent with the electrophysiologically abnormal brain region. Recent advances in the pathologic diagnosis and classification of epileptogenic brain lesions are helpful for clinical correlation, outcome stratification, and patient management. However, application of international consensus classification systems to common epileptic pathologies (e.g., focal cortical dysplasia [FCD] and hippocampal sclerosis [HS]) necessitates standardized protocols for neuropathologic workup of epilepsy surgery specimens. To this end, the Task Force of Neuropathology from the International League Against Epilepsy (ILAE) Commission on Diagnostic Methods developed a consensus standard operational procedure for tissue inspection, distribution, and processing. The aims are to provide a systematic framework for histopathologic workup, meeting minimal standards and maximizing current and future opportunities for morphofunctional correlations and molecular studies for both clinical care and research. Whenever feasible, anatomically intact surgical specimens are desirable to enable systematic analysis in selective hippocampectomies, temporal lobe resections, and lesional or nonlesional neocortical samples. Correct orientation of sample and the sample\'s relation to neurophysiologically aberrant sites requires good communication between pathology and neurosurgical teams. Systematic tissue sampling of 5-mm slabs along a defined anatomic axis and application of a limited immunohistochemical panel will ensure a reliable differential diagnosis of main pathologies encountered in epilepsy surgery.
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