Incidental Pediatric High-Flow Nongalenic Giant Pial Arteriovenous FistulaFunding 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.   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.   If left untreated, the natural history of NGAVFs are associated with poor clinical outcomes consequent to venous hypertension, infarction, or hemorrhage.  
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Received: 25 April 2020
Accepted: 14 July 2020
23 November 2020 (online)
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