1383 Clinical Severity of Cerebral Proliferative Angiopathy: A Meta-Analysis Highlighting its Significance Beyond Arteriovenous Malformations

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INTRODUCTION: Cerebral Proliferative Angiopathy (CPA) is a rare subtype of brain arteriovenous malformations (AVM), mainly characterized by intermingled brain parenchyma with vascular network. Current evidence remains insufficient to decide on the best treatment principles and rationale for this somewhat newly categorized disease. Today, endovascular, revascularization surgery, radiotherapy and conservative strategies have been proposed. Moreover, a gap in morbimortality exists, in comparison with approaches for brain AVM management. METHODS: A meta-analysis was conducted according to PRISMA guidelines. For this, we elected PubMed, Embase and Web Of Science as databases. Keywords, such as “cerebral proliferative angiopathy” and “management” were utilized. We pooled and meta-analyzed outcomes on documented CPA cases. RESULTS: 11079 studies were pooled, 50 studies were included, adding up to 115 CPA cases. The majority of patients were females (1.38:1), with a mean age of presentation of 27 (19) years. Headache (46%) were the most common presenting symptom. 37% of patients presented with focal neurologic deficit (FND), and 34% with seizures. Patients managed conservatively experienced major complications in 47% of cases (95% CI: 17%, 76%), with a 1% mortality (95% CI: 0%, 6%). Surgical and embolization interventions presented the highest proportion of major complications: 66% (95% CI: 33%, 99%) and 73% (95% CI: 42%, 100%), respectively. The embolization subgroup led in mortality, with 3% (95% CI: 0%, 10%). No death was documented in patients undergoing revascularization surgery. CONCLUSIONS: CPA clinical presentation can be compatible with brain arteriovenous malformations, with headaches, seizures and FND. However, our meta-analysis revealed treatment outcomes are potentially worse. This gap is not entirely explained by differences in patient profiles undergoing different treatment strategies, rendering special attention to diagnostic methods.

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