De Novo Formation of Pial Arteriovenous Fistulas: Systematic Review of Acquired Lesions and Their Clinical Differences Compared with Primary Lesions

Abstract

Acquired pial arteriovenous fistula (pAVF) is an extremely rare intracranial vascular malformation, with few case reports in the English literature. This study presents a thorough review and analysis of all acquired pAVF cases from the literature in addition to an illustrated case. We report a case with de novo development of intracranial pAVF after craniotomy. A medical literature database search between 1975 and 2018, including the Medline, Ovid, and PubMed databases, was performed to identify all reports with possible acquired lesions. Differences between these acquired lesions and previously reported primary lesions were evaluated. A total of 8 patients with de novo formation of acquired pAVF were included in this series. Most of these pAVFs were fed and drained via a similar arteriovenous pattern, from distal/cortical branches of the middle cerebral artery (6/8, 75%) to the superficial middle cerebral vein (6/8, 75%). Compared with a previously reported primary pAVF series, acquired pAVF tended to be asymptomatic (P < 0.0001) and found essentially in adults (P= 0.0061). Fewer venous varices (P= 0.0049) and associated intracranial mass effect (P= 0.0189) were found in the cases of acquired pAVF. All 4 reported acquired pAVFs that were treated microsurgically resulted in complete angiographic obliteration (4/4, 100%). The overall outcome was good or stable even with observation only (7/8, 87.5%). Acquired pAVF is highly correlated with sentinel neurosurgical procedures or venous occlusion events. These lesions should be regarded as a different disease entity from primary pAVF because of the relatively low-flow shunting and benign clinical course.