The management for craniocervical junction dural arteriovenous fistulas (CCJ-DAVFs) remains controversial and clinically challenging. We systemically summarized the clinical and angiographic outcomes of microsurgery, embolization, and conservative management. Relevant articles were retrieved from PubMed, Scopus, Web of Science, and Cochrane, following PRISMA guidelines. A systematic review and meta-analysis were conducted on the clinical characteristics, management approaches, and clinical and angiographic outcomes. We included 13 articles (166 patients). The weighted mean age was 58.9 years (95%CI: 53.2-64.5), 58.8 years (95%CI: 48.4-69.2), and 63.8 years (95%CI: 60.1-67.5), in microsurgery, embolization, and conservative groups respectively, with an overall male sex predominance (microsurgery [n = 51/77, 66.2%], embolization [n = 44/56, 78.6%], and conservative management [n = 6/8, 75.0%]). Patients were managed with microsurgery (n = 80/172, 46.5%), embolization (n = 79/172, 45.9%), and conservative treatment (n = 13/172, 7.6%). Foramen magnum was the most common location (microsurgery [n = 34/77, 44.2%], embolization [n = 31/56, 55.4%], and conservative treatment [n = 3/8, 37.5%]). Vertebral artery was the primary feeder (microsurgery [n = 58/84, 69.1%], embolization [n = 41/86, 47.6%], and conservative treatment [n = 4/7, 57.1%]). Complete fistula obliteration rates were 74.1% (95%CI:52.3-88.2%) in the microsurgery group and 54.9% (95%CI:30.7-77.0%) in the embolization group. Complications rates were 16.2% (95%CI:6.7-34.5%) in the embolization group, 11.6% (95%CI:3.8-30.4%) in the microsurgery group, and 7.7% (95%CI:1.1-39.1%) in the conservative group. Different rates of good clinical outcomes were observed [microsurgery: 66.4% (95%CI:48.1-80.8%), embolization: 51.9% (95%CI:30.8-72.4%), and conservative: 11.6% (95%CI:4.4-27.4%)]. In patients with CCJ-DAVFs, each management approach has its own merits based on the fistula and patient characteristics.
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