Progressive versus Nonprogressive Intracranial Dural Arteriovenous Fistulas: Characteristics and Outcomes

Abstract

Background and purpose: 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.

Materials and methods: We performed a retrospective imaging and clinical record review of patients with intracranial dural arteriovenous fistula evaluated at our hospital.

Results: 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.

Conclusions: 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.

Fig 1.

Cohort selection.

Fig 2.

Age of diagnosis for patients with progressive-versus-nonprogressive fistulas.

Fig 3.

Multiple and progressive DAVFs. A 25-year-old female patient with large skull base DAVFs refractory to multiple endovascular treatments. She was initially diagnosed at 18 years of age and died of intracranial hemorrhage 6 years later in the setting of intractable intracranial venous hypertension. CTA images demonstrate an extensive skull base vascular abnormality with dilated cortical veins suggestive of intracranial venous hypertension.

Fig 4.

Multiple and progressive DAVFs. Same patient as in Fig 3. DSA images before treatment at our institution but 4 years after initial proximal coil embolization of external carotid artery feeders below the skull base at an outside institution demonstrate multiple skull base fistulas associated with venous hypertension, cortical venous reflux, venous sinus dilation, and a jugular bulb outflow stenosis (between white arrows). (Upper row: right vertebral artery lateral, right vertebral artery lateral, left vertebral artery anteroposterior; lower row: left ICA anteroposterior, left external carotid artery anteroposterior, left external carotid artery lateral). A middle meningeal artery to the torcular fistula indicated by the white arrows is shown in greater detail in Fig 5.

Fig 5.

Multiple and progressive DAVFs. Same patient as in Figs 3 and 4. DSA demonstrates progression of a left middle meningeal artery to a torcular DAVF with interval enlargement of a feeding artery (arrow in upper left panel versus arrow in upper right panel) during 7 months. Lateral CT scanograms obtained at the same time as DSA provide an overview of treatment with interval deposition of embolic coils and n-BCA glue (lower left versus lower right panel).