title: Pediatric intracranial dural arteriovenous fistulas: age-related differences in clinical features, angioarchitecture, and treatment outcomes.
abstract: OBJECTIVE Intracranial dural arteriovenous fistulas (DAVFs) are rare in children. This study sought to better characterize DAVF presentation, angioarchitecture, and treatment outcomes. METHODS Children with intracranial DAVFs between 1986 and 2013 were retrospectively identified from the neurointerventional database at the authors' institution. Demographics, clinical presentation, lesion angioarchitecture, treatment approaches, angiographic outcomes, and clinical outcomes were assessed. RESULTS DAVFs constituted 5.7% (22/423) of pediatric intracranial arteriovenous shunting lesions. Twelve boys and 10 girls presented between 1 day and 18 years of age; boys presented at a median of 1.3 years and girls presented at a median of 4.9 years. Four of 8 patients ≤ 1 year of age presented with congestive heart failure compared with 0/14 patients > 1 year of age (p = 0.01). Five of 8 patients ≤ 1 year old presented with respiratory distress compared with 0/14 patients > 1 year old (p = 0.0021). Ten of 14 patients > 1 year old presented with focal neurological deficits compared with 0/8 patients ≤ 1 year old (p = 0.0017). At initial angiography, 16 patients harbored a single intracranial DAVF and 6 patients had 2-6 DAVFs. Eight patients (38%) experienced DAVF obliteration by the end of treatment. Good clinical outcome (modified Rankin Scale score 0-2) was documented in 77% of patients > 1 year old at presentation compared with 57% of patients ≤ 1 year old at presentation. Six patients (27%) died. CONCLUSIONS Young children with DAVFs presented predominantly with cardiopulmonary symptoms, while older children presented with focal neurological deficits. Compared with other pediatric vascular shunts, DAVFs had lower rates of angiographic obliteration and poorer clinical outcomes.
authors.affiliation: Departments of 1 Radiology and Biomedical Imaging.
authors.forename: Steven W
authors.initials: SW
authors.lastname: Hetts
authors.affiliation: Departments of 1 Radiology and Biomedical Imaging.
authors.forename: Parham
authors.initials: P
authors.lastname: Moftakhar
authors.affiliation: Department of Neurology, University of California, Los Angeles, California.
authors.forename: Neil
authors.initials: N
authors.lastname: Maluste
authors.affiliation: Neurology.
authors.forename: Heather J
authors.initials: HJ
authors.lastname: Fullerton
authors.affiliation: Departments of 1 Radiology and Biomedical Imaging.
authors.forename: Daniel L
authors.initials: DL
authors.lastname: Cooke
authors.affiliation: Departments of 1 Radiology and Biomedical Imaging.
authors.forename: Matthew R
authors.initials: MR
authors.lastname: Amans
authors.affiliation: Departments of 1 Radiology and Biomedical Imaging.|Neurology.|Neurological Surgery, and.|Anesthesia and Perioperative Care, University of California, San Francisco; and.
authors.forename: Christopher F
authors.initials: CF
authors.lastname: Dowd
authors.affiliation: Departments of 1 Radiology and Biomedical Imaging.|Neurology.|Neurological Surgery, and.|Anesthesia and Perioperative Care, University of California, San Francisco; and.
authors.forename: Randall T
authors.initials: RT
authors.lastname: Higashida
authors.affiliation: Departments of 1 Radiology and Biomedical Imaging.|Neurology.|Neurological Surgery, and.|Anesthesia and Perioperative Care, University of California, San Francisco; and.
authors.forename: Van V
authors.initials: VV
authors.lastname: Halbach
mesh_terms: D000293:Adolescent; D000367:Age Factors; D020785:Central Nervous System Vascular Malformations / Q000000981:diagnostic imaging* / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: AVM = arteriovenous malformation; CCA = common carotid artery; CHF = congestive heart failure; CI = confidence interval; DAVF; DAVF = dural arteriovenous fistula; DSA = digital subtraction angiogram; DSM = dural sinus malformation; NGAVF = non-Galenic pial arteriovenous fistula; OR = odds ratio; SSS = superior sagittal sinus; VOGM = vein of Galen malformation; cardiopulmonary; dural arteriovenous fistula; mRS = modified Rankin Scale; neurological deficit; vascular disorders