Development of bilateral dural arteriovenous fistulae following pial synangiosis for moyamoya syndrome: case report

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Moyamoya syndrome predisposes patients to ischemic or hemorrhagic stroke due to progressive narrowing of intracranial vessels with subsequent small-vessel collateralization. Dural arteriovenous fistulae (DAVFs) are most commonly noted after venous sinus or cortical vein thrombosis and are believed to be primarily due to venous hypertension and elevated sinus pressures, although there is no known association with moyamoya syndrome, or with surgical treatment for moyamoya disease (MMD). The authors present the case of a 14-year-old girl with Down syndrome treated using pial synangiosis for MMD who subsequently was noted to have bilateral DAVFs. This case provides a new perspective on the origins and underlying pathophysiology of both moyamoya syndrome and DAVFs, and also serves to highlight the importance of monitoring the moyamoya population closely for de novo cerebrovascular changes after revascularization procedures.

ABBREVIATIONS ACA = anterior cerebral artery; bFGF = basic fibroblast growth factor; DAVF = dural arteriovenous fistula; MCA = middle cerebral artery; MMD = moyamoya disease; MRA = MR angiography; SDH = subdural hematoma; VEGF = vascular endothelial growth factor.

Article Information

Correspondence Cormac O. Maher: University of Michigan, Ann Arbor, MI. cmaher@med.umich.edu.

INCLUDE WHEN CITING Published online April 12, 2019; DOI: 10.3171/2019.2.PEDS18603.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.

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    Axial diffusion-weighted MR images showing multifocal areas of hyperintensity in the cortex and white matter of the left cerebral hemisphere above (A) and at the level of the lateral ventricles (B).

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    Anterior-posterior cerebral angiogram demonstrating complete occlusion of the left MCA (arrow) with formation of small collateral vessels. Figure is available in color online only.

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    A: Lateral angiogram of the right external carotid arteries demonstrating a DAVF involving the sigmoid sinus and right internal jugular vein with arterial supply from petrous branches of the right middle meningeal artery and posterior auricular artery (arrow). B: Lateral angiogram of the left external carotid arteries demonstrating a DAVF involving the sigmoid sinus and left internal jugular vein with arterial supply (arrow) from the petrous branch of the middle meningeal artery, the occipital artery, and the posterior division of the ascending pharyngeal artery. Patent pial synangiosis is noted via a hypertrophied superficial temporal artery (star). Figure is available in color online only.

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    A: Twelve-month follow-up time-of-flight MRA showing a persistent DAVF on the left side with early filling and arterial vascularity surrounding the jugular bulb (arrow). B: Left-sided early filling and arterial vascularity at the transverse sigmoid junction (arrow). Figure is available in color online only.

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