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DOI: 10.1055/s-0040-1716905
Incidental Pediatric High-Flow Nongalenic Giant Pial Arteriovenous Fistula
Funding Sources This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.A 3-year-old female presented to the emergency department with a severe headache after a fall. She was otherwise without focal neurologic deficit. Computed tomography of the head demonstrated a large hyperdense, well-circumscribed, extra-axial lesion centered along the lateral aspect of the left frontal and anterior temporal lobes with associated mass effect suggestive of vascular pathology ([Fig. 1]).Magnetic resonance imaging demonstrated an avidly enhancing lesion supplied by dilated tortuous vessels ([Fig. 2A, B]). These findings were highly suggestive of a vascular malformation. Subsequent digital subtraction angiography revealed a high-flow, nongalenic pial arteriovenous fistula (NGAVF) with dominant feeders from hypertrophic middle cerebral artery branches and venous outflow into the sphenoparietal sinus with a giant fistulous pouch ([Fig. 3A–D]). The lesion was successfully treated with coil embolization (hydrogels) of the single-hole fistulae ([Figs. 2C], [3E-L]).
NGAVFs are rare, unique vascular malformations with pial arteries feeding directly into a cortical vein without the intervening nidus of arteriovenous malformations.[1] [2] [3] The goal of treatment is occlusion of the fistulous site or the arterial feeders and proximal venous drainage as close to the fistula as able.[1] [2] [3] If left untreated, the natural history of NGAVFs are associated with poor clinical outcomes consequent to venous hypertension, infarction, or hemorrhage.[1] [2] [3]
Special Designations/Previous Presentations
None.
Disclosures
The authors declare that there are no disclosures to state.
Author Contributions
All authors have contributed substantively to the conception, design, analysis, and interpretation of the data, contributed substantively to the drafting of the manuscript and critical reason for important intellectual content, given final approval of the version to be published, and agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Publication History
Received: 25 April 2020
Accepted: 14 July 2020
Article published online:
23 November 2020
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References
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- 2 Hetts SW, Keenan K, Fullerton HJ. et al. Pediatric intracranial nongalenic pial arteriovenous fistulas: clinical features, angioarchitecture, and outcomes. AJNR Am J Neuroradiol 2012; 33 (09) 1710-1719
- 3 Lv X, Li Y, Jiang C, Wu Z. Endovascular treatment of brain arteriovenous fistulas. AJNR Am J Neuroradiol 2009; 30 (04) 851-856