Department of Neurological Surgery, School of Medicine, Universidade de Sao Paulo, Rua Dr Eneas de Carvalho Aguiar, 255 - Pinheiros, São Paulo, SP, Brazil.
Department of Neurosugery, Hospital Ophir Loyola, Belem, PA, Brazil.
Acta Neurochir (Wien). 2020 Jul;162(7):1727-1733. doi: 10.1007/s00701-020-04289-7. Epub 2020 Mar 23.
Cerebral proliferative angiopathy (CPA) is a rare subset of arteriovenous malformations (AVM). It has unique clinical presentation, angiographic characteristics, and pathophysiology which often brings challenges for the treatment. We aimed to define its epidemiology, pathophysiology are unknown, and best management strategies.
A systematic review was conducted according to the PRISMA guidelines. MEDLINE was searched for articles regarding CPA. Extracted data included epidemiological, clinical, and angiographical characteristics, treatment, and outcomes. Treatment was classified as conservative, radiosurgery, endovascular, decompression, and indirect vascularization. A meta-analytical approach was employed for description of the data as study-size adjusted percentages or weighted means, as appropriate.
Thirty-three studies were analyzed, rendering a total 95 cases-half of which came from a single study. Patients were predominantly young (mean 23 years old) and female (60.0%) presenting with headaches (44.9%), seizures (37.1%), or transient ischemic attacks (33.7%). Hemorrhage was present in 18.0%, but rebleeding rates were as high as 67%. The majority of nidus were > 6 cm (52.5%) with hemispheric extension (73.0%). Capillary angioectatic appearance (85.7%), transdural supply (62.5%), and deep venous drainage (73.0%) were also frequent features. Most patients were treated conservatively (54.4%), followed by endovascular (34.2%). Indirect vascularization and radiosurgery were attempted in five and two patients, respectively. Mean follow-up was 110.8 patient-years. Neurological status improved in 50.7%, remained stable in 40.2%, and worsened in 9.0%.
Conservative and endovascular treatments seem adequate interventions, despite limited evidence. Complementary techniques can be used in patients throughout disease history, according to symptom-based, individualized approach. More studies are required for choosing interventions based on reliable long-term results.
脑增殖性血管病变（CPA）是动静脉畸形（AVM）的一个罕见亚型。它具有独特的临床表现、血管造影特征和病理生理学，这常常给治疗带来挑战。我们旨在定义其流行病学、发病机制尚不清楚以及最佳管理策略。
根据 PRISMA 指南进行系统评价。对有关 CPA 的文章进行 MEDLINE 检索。提取的数据包括流行病学、临床和血管造影特征、治疗和结局。治疗分为保守治疗、放射外科治疗、血管内治疗、减压治疗和间接血管化治疗。采用荟萃分析方法对数据进行描述，适当采用研究大小调整后的百分比或加权平均值。
分析了 33 项研究，共涉及 95 例患者-其中一半来自一项研究。患者主要为年轻（平均 23 岁）和女性（60.0%），表现为头痛（44.9%）、癫痫发作（37.1%）或短暂性脑缺血发作（33.7%）。18.0%的患者存在出血，但再出血率高达 67%。大多数病灶>6cm（52.5%），并延伸至半球（73.0%）。毛细血管血管扩张样外观（85.7%）、硬膜外供血（62.5%）和深静脉引流（73.0%）也是常见特征。大多数患者接受保守治疗（54.4%），其次是血管内治疗（34.2%）。分别有 5 例和 2 例患者尝试间接血管化和放射外科治疗。平均随访时间为 110.8 患者年。神经功能状态改善 50.7%，稳定 40.2%，恶化 9.0%。
尽管证据有限，但保守治疗和血管内治疗似乎是合适的干预措施。根据基于症状的个体化方法，可以在疾病的整个过程中对患者使用补充技术。需要更多的研究来根据可靠的长期结果选择干预措施。
