Pediatric intracranial nongalenic pial arteriovenous fistulas: Clinical features, angioarchitecture, and outcomes

Authors

Steven W. Hetts, University of California, San Francisco
K. Keenan, University of California, San Francisco
H. J. Fullerton, University of California, San Francisco
W. L. Young, University of California, San Francisco
J. D. English, University of California, San Francisco
N. Gupta, University of California, San Francisco
C. F. Dowd, University of California, San Francisco
R. T. Higashida, University of California, San Francisco
M. T. Lawton, University of California, San FranciscoFollow
V. V. Halbach, University of California, San Francisco

Document Type

Article

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.

Publication Date

10-1-2012

Publication Title

American Journal of Neuroradiology

ISSN

01956108

E-ISSN

1936959X

Volume

33

Issue

9

First Page

1710

Last Page

1719

PubMed ID

22766672

Digital Object Identifier (DOI)

10.3174/ajnr.A3194

Recommended Citation

Hetts, Steven W.; Keenan, K.; Fullerton, H. J.; Young, W. L.; English, J. D.; Gupta, N.; Dowd, C. F.; Higashida, R. T.; Lawton, M. T.; and Halbach, V. V., "Pediatric intracranial nongalenic pial arteriovenous fistulas: Clinical features, angioarchitecture, and outcomes" (2012). Neurosurgery. 1041.
https://scholar.barrowneuro.org/neurosurgery/1041