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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