Introduction: Vertebral arterio-venous fistulas (VAVFs) are uncommon lesions that can arise spontaneously or secondarily to iatrogenic or mechanical trauma. Among spontaneous cases, it is most commonly found to be associated with neurofibromatosis type 1 (NF1).
Evidence acquisition: We performed a systematic review of the literature to obtain information regarding demographics, clinical presentation, treatment modalities and outcome of VAVFs associated with NF1. A literature search was performed by using databases PubMed Central, Embase, Cochrane Library, and Ovid MEDLINE. Also, the grey area search was done using the "Google Scholar" search engine. On screening of the original full-text English language articles, a total of 48 cases were considered suitable for inclusion in this review.
Evidence synthesis: VAVFs in NF1 commonly present between 3rd and 6th decade of life affecting females 2.4 times more than males. Left-sided fistulae were more common than the right side and most seen in the upper V2 segment of the vertebral artery. Most VAVFs in NF 1 patients were treated with constructive (occlusion of fistula only) endovascular therapy (N.=26) with a high success rate. Moreover, pooled proportion of the outcome data have shown significant difference between the endovascular constructive and destructive procedure.
Conclusions: The spontaneous VAVF, commonly associated with NF-1, often requires treatment. Awareness of the coexistence between NF1 and VAVF is crucial to avoid diagnostic delays and unnecessary surgical intervention leading to disastrous outcomes. Endovascular treatment is the preferred treatment approach while open surgical treatment is required in some complex fistulae and failure of endovascular techniques.