Abstract

A minority of intracranial dural arteriovenous fistulas progress with time. We sought to determine features that predict progression and define outcomes of patients with progressive dural arteriovenous fistulas. We performed a retrospective imaging and clinical record review of patients with intracranial dural arteriovenous fistula evaluated at our hospital. Of 579 patients with intracranial dural arteriovenous fistulas, 545 had 1 fistula (mean age, 45 ± 23 years) and 34 (5.9%) had enlarging, de novo, multiple, or recurrent fistulas (mean age, 53 ± 20 years; P = .11). Among these 34 patients, 19 had progressive dural arteriovenous fistulas with de novo fistulas or fistula enlargement with time (mean age, 36 ± 25 years; progressive group) and 15 had multiple or recurrent but nonprogressive fistulas (mean age, 57 ± 13 years; P = .0059, nonprogressive group). Whereas all 6 children had fistula progression, only 13/28 adults (P = .020) progressed. Angioarchitectural correlates to chronically elevated intracranial venous pressures, including venous sinus dilation (41% versus 7%, P = .045) and pseudophlebitic cortical venous pattern (P = .048), were more common in patients with progressive disease than in those without progression. Patients with progressive disease received more treatments than those without progression (median, 5 versus 3; P = .0068), but as a group, they did not demonstrate worse clinical outcomes (median mRS, 1 and 1; P = .39). However, 3 young patients died from intracranial venous hypertension and intracranial hemorrhage related to progression of their fistulas despite extensive endovascular, surgical, and radiosurgical treatments. Few patients with dural arteriovenous fistulas follow an aggressive, progressive clinical course despite treatment. Younger age at initial presentation and angioarchitectural correlates to venous hypertension may help identify these patients prospectively.

Highlights

  • BACKGROUND AND PURPOSEA minority of intracranial dural arteriovenous fistulas progress with time

  • In the early days of cerebral angiography, dural arteriovenous fistulas (DAVFs) were considered a subset of AVMs: Newton and Cronqvist[2] classified AVMs by arterial supply as pure dural, mixed pial-dural, or pure pial malformations

  • 1912 Hetts Oct 2015 www.ajnr.org thought to be acquired, and DAVFs lack a nidus of vessels in the brain parenchyma

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Summary

Methods

We performed a retrospective imaging and clinical record review of patients with intracranial dural arteriovenous fistula evaluated at our hospital. Subjects In this institutional review board–approved study, a neurointerfeeders, or worsening of the venous drainage pattern of an existing fistula during treatment or on follow-up. When we combined these descriptions for analysis, the progressive fistula group inventional radiology data base was used to identify 579 subjects cluded patients with enlarging and/or de novo fistulas and the diagnosed with or treated for intracranial DAVFs at the University nonprogressive group included patients with unchanging multiof California, San Francisco between 1986 and 2013 (Fig 1). Outcomes at last follow-up Mean follow-up duration Median (range) follow-up duration Death Median outcome (normal ϭ 0, deficits/residual fistula ϭ 1, death ϭ 2) Median last mRS (25%, 75%) Range last mRS Good outcome (last mRS 0–2) Median change in mRS (25%, 75%) Range change mRS

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