PT  - JOURNAL ARTICLE
AU  - S.W. Hetts
AU  - K. Keenan
AU  - H.J. Fullerton
AU  - W.L. Young
AU  - J.D. English
AU  - N. Gupta
AU  - C.F. Dowd
AU  - R.T. Higashida
AU  - M.T. Lawton
AU  - V.V. Halbach
TI  - Pediatric Intracranial Nongalenic Pial Arteriovenous Fistulas: Clinical Features, Angioarchitecture, and Outcomes
AID  - 10.3174/ajnr.A3194
DP  - 2012 Oct 01
TA  - American Journal of Neuroradiology
PG  - 1710--1719
VI  - 33
IP  - 9
4099  - http://www.ajnr.org/content/33/9/1710.short
4100  - http://www.ajnr.org/content/33/9/1710.full
SO  - Am. J. Neuroradiol.2012 Oct 01; 33
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