RT Journal Article SR Electronic T1 Pediatric Intracranial Nongalenic Pial Arteriovenous Fistulas: Clinical Features, Angioarchitecture, and Outcomes JF American Journal of Neuroradiology JO Am. J. Neuroradiol. FD American Society of Neuroradiology SP 1710 OP 1719 DO 10.3174/ajnr.A3194 VO 33 IS 9 A1 Hetts, S.W. A1 Keenan, K. A1 Fullerton, H.J. A1 Young, W.L. A1 English, J.D. A1 Gupta, N. A1 Dowd, C.F. A1 Higashida, R.T. A1 Lawton, M.T. A1 Halbach, V.V. YR 2012 UL http://www.ajnr.org/content/33/9/1710.abstract AB BACKGROUND AND PURPOSE: NGAVFs are rare vascular malformations usually presenting in infancy or childhood. We sought to identify clinical and angiographic predictors of clinical outcome for these lesions. MATERIALS AND METHODS: Retrospective review of a neurointerventional data base identified 386 pediatric patients with intracranial AVFs and AVMs, from which a cohort of 25 patients with NGAVF were selected for medical record and imaging analysis. RESULTS: NGAVFs constituted 7.3% of pediatric intracranial vascular lesions with a nondural arteriovenous shunt. Seven of 8 patients who presented in the first month of life had CHF and harbored large, complex fistulas with multiple sites of arteriovenous shunting. Single-hole fistulas predominated later in childhood and more frequently presented with seizures, hemorrhage, or focal neurologic deficits. More treatment procedures were performed in subjects presenting at ≤2 years of age compared with older children (median = 3 versus 2, P = .041), and in those harboring a multi-hole fistula versus those with a single-hole fistula (median = 3 versus 2, P = .003). Eighteen patients (72%) had complete posttreatment elimination of NGAVF shunting. Compared with patients presenting at >2 years of age, patients presenting in the first 2 years of life were more likely to have a multi-hole fistula (100% versus 25%, P = .0001) and to have a poor clinical outcome (54% versus 0%, P = .0052), defined as a pediatric mRS of ≥3. CONCLUSIONS: The morbidity of NGAVF appears higher than previously reported despite a somewhat higher rate of angiographic cure. Poor clinical outcome occurred primarily in patients with multi-hole NGAVFs presenting at ≤2 years of age. AVFarteriovenous fistulaCHFcongestive heart failureDAVFdural arteriovenous fistulaHHThereditary hemorrhagic telangiectasia syndromemRSmodified Rankin Scalen-BCAn-butyl cyanocrylateNGAVFnongalenic arteriovenous fistulaVOGMvein of Galen malformation