title: Pediatric intracranial nongalenic pial arteriovenous fistulas: clinical features, angioarchitecture, and outcomes.
abstract: OBJECTIVE
  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.
  
  
  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.
authors: Department of Radiology, University of California-San Francisco, San Francisco, California, USA. steven.hetts@ucsf.edu
authors: S W
authors: SW
authors: Hetts
authors: K
authors: K
authors: Keenan
authors: H J
authors: HJ
authors: Fullerton
authors: W L
authors: WL
authors: Young
authors: J D
authors: JD
authors: English
authors: N
authors: N
authors: Gupta
authors: C F
authors: CF
authors: Dowd
authors: R T
authors: RT
authors: Higashida
authors: M T
authors: MT
authors: Lawton
authors: V V
authors: VV
authors: Halbach
mesh_terms: D000293:Adolescent; D002140:California / Q000453:epidemiology; D002533:Cerebral Angiography / Q000706:statistics & numerical data*; D002648:Child; D002675:Child, Preschool; D006801:Humans; D015994:Incidence; D007223:Infant; D002538:Intracranial Arteriovenous Malformations / Q000000981:diagnostic imaging* / Q000401:mortality* / Q000628:therapy; D008297:Male; D010841:Pia Mater / Q000000981:diagnostic imaging; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D015996:Survival Rate; D016896:Treatment Outcome