BACKGROUND AND OBJECTIVES:Despite many publications about cavernous malformations (CMs), controversy remains regarding diagnostic and management strategies. To update evidence-based guidelines for the clinical management of brain and spinal cord CMs.

METHODS: The Alliance to Cure CMs, the patient support group in the United States advocating on behalf of patients and research in CM, convened a multidisciplinary writing group comprising expert CM clinicians to help summarize the existing literature related to the clinical care of CM, focusing on 5 topics: (1) epidemiology and natural history, (2) genetic testing and counseling, (3) diagnostic criteria and imaging standards, (4) neurosurgical considerations, and (5) neurological considerations. Building on prior evidence-based recommendations reflecting literature review through October 2014, the group conducted a systematic review of the more recent literature, identified references for mandatory citation, rated evidence, developed recommendations, and established consensus according to a prespecified protocol. Finally, the writing group outlined remaining knowledge gaps and controversies to guide future research.

RESULTS: From 2672 publications published between October 1, 2014, and March 15, 2023, and meeting key word criteria, 234 were selected based on prearticulated criteria for mandatory consideration in evidence-based recommendations. Topic authors used these and other supporting references to summarize current knowledge and arrive at 53 management recommendations, with unanimous consensus based on a Delphi process. These were rated by class (strength of recommendation) and level (quality of evidence) per the American Heart Association/American Stroke Association criteria. Eighteen recommendations were class 1 (34%), class 2 in 31 (58%), and class 3 in 4 (8%). Three were level A (6%), 19 (36%) were level B, and 31 (58%) were level C.

CONCLUSION: Current evidence supports prior and new recommendations for the management of CMs, but many reflect moderate classes and low levels, mandating further research to better inform clinical practice.

背景与目的：尽管关于海绵状血管畸形（CMs）的研究已发表众多，但在诊断与管理策略上仍存在争议。本研究旨在更新脑和脊髓CMs临床管理的循证指南。

方法：美国治愈CMs联盟（Alliance to Cure CMs）作为代表患者并推动CM研究的支持组织，召集了由CM临床专家组成的多学科写作小组，围绕以下5个主题总结CM临床管理的现有文献：（1）流行病学与自然病史；（2）基因检测与遗传咨询；（3）诊断标准与影像学规范；（4）神经外科考量；（5）神经科考量。该小组在2014年10月前文献综述形成的既往循证推荐基础上，对近年文献进行系统回顾，筛选必须引用的参考文献，评估证据等级，制定推荐意见，并按预设方案达成共识。最后，写作小组梳理了现存知识空白与争议，以指导未来研究。

结果：在2014年10月1日至2023年3月15日期间发表的2672篇符合关键词标准的文献中，根据预先制定的纳入标准筛选出234篇作为循证推荐的必须参考文献。各主题作者基于这些文献及其他支持性参考文献，总结当前认知并形成53条管理建议，通过德尔菲法达成一致共识。推荐意见依据美国心脏协会/美国卒中协会标准，按类别（推荐强度）和等级（证据质量）分级：18条为Ⅰ类推荐（34%），31条为Ⅱ类（58%），4条为Ⅲ类（8%）；证据等级中A级3条（6%），B级19条（36%），C级31条（58%）。

结论：现有证据支持CMs管理的既往及新增推荐意见，但多数推荐强度为中等、证据等级较低，亟需进一步研究以优化临床实践指导。