@article {Hetts1710, author = {S.W. Hetts and K. Keenan and H.J. Fullerton and W.L. Young and J.D. English and N. Gupta and C.F. Dowd and R.T. Higashida and M.T. Lawton and V.V. Halbach}, title = {Pediatric Intracranial Nongalenic Pial Arteriovenous Fistulas: Clinical Features, Angioarchitecture, and Outcomes}, volume = {33}, number = {9}, pages = {1710--1719}, year = {2012}, doi = {10.3174/ajnr.A3194}, publisher = {American Journal of Neuroradiology}, abstract = {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}, issn = {0195-6108}, URL = {https://www.ajnr.org/content/33/9/1710}, eprint = {https://www.ajnr.org/content/33/9/1710.full.pdf}, journal = {American Journal of Neuroradiology} }