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

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.

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  • Publisher Full Text
  • Authors

    Hetts SW, Keenan K, Fullerton HJ, Young WL, English JD, Gupta N, Dowd CF, Higashida RT, Lawton MT, Halbach VV

    Institution

    Department of Radiology, University of California-San Francisco, San Francisco, California, USA. steven.hetts@ucsf.edu

    Source

    AJNR. American journal of neuroradiology 33:9 2012 Oct pg 1710-9

    MeSH

    Adolescent
    California
    Cerebral Angiography
    Child
    Child, Preschool
    Humans
    Incidence
    Infant
    Intracranial Arteriovenous Malformations
    Male
    Pia Mater
    Retrospective Studies
    Risk Factors
    Survival Analysis
    Survival Rate
    Treatment Outcome

    Pub Type(s)

    Journal Article
    Research Support, N.I.H., Extramural
    Research Support, Non-U.S. Gov't

    Language

    eng

    PubMed ID

    22766672