Intracranial dural arteriovenous malformations: factors predisposing to an aggressive neurological course

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✓ The natural history of cranial dural arteriovenous malformations (AVM's) is highly variable. The authors present their clinical experience with 17 dural AVM's in adults, including 10 cases with an aggressive neurological course (strictly defined as hemorrhage or progressive focal neurological deficit other than ophthalmoplegia). Two of these 10 patients died prior to surgical intervention and a third was severely disabled by intracerebral hemorrhage. Six patients underwent surgical resection of their dural AVM, with preparatory embolization in two cases. One patient received embolization and radiation therapy without surgery. Six of the seven cases without an aggressive neurological course were treated conservatively, and the seventh patient underwent embolization of a cavernous sinus dural AVM because of worsening ophthalmoplegia. In order to clarify features associated with aggressive behavior, a comprehensive meta-analysis was performed on 360 additional dural AVM's reported in the literature with sufficiently detailed clinical and angiographic information. The location and angiographic features of 100 aggressive cases were compared to those of 277 benign cases. No location of dural AVM's was immune from aggressive neurological behavior; however, an aggressive neurological course was least often associated with cases involving the transverse-sigmoid sinuses and cavernous sinus and most often associated with cases at the tentorial incisura. Contralateral contribution to arterial supply and rate of shunting (high vs. low flow) did not correlate with aggressive neurological behavior as defined. Leptomeningeal venous drainage, variceal or aneurysmal venous dilations, and galenic drainage correlated significantly (p < 0.05) with aggressive neurological presentation. The latter three angiographic features often coexisted in the same dural AVM. It is concluded that these features significantly increase the natural risk of dural AVM's, and warrant a more vigilant therapeutic strategy.

Article Information

Address reprint requests to: Issam A. Awad, M.D., Department of Neurological Surgery, S80, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, Ohio 44195–5275.

© AANS, except where prohibited by US copyright law.

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Figures

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    Case 2. Tentorial and transverse sinus dural arteriovenous malformation and acute cerebellar and ventricular hemorrhage. Upper: Computerized tomography scans. Lower: Right common carotid arteriogram, anteroposterior view. Arterial feeders include the occipital arteries and tentorial branches of the internal carotid arteries. Drainage is via the transverse sinus and multiple retrograde variceal leptomeningeal veins including a giant venous aneurysm. The patient died despite supportive measures.

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    Case 6. Tentorial incisural dural arteriovenous malformation (AVM) and acute thalamic and ventricular hemorrhage. A: Computerized tomography scan revealing hemorrhage into the pulvinar of the right thalamus and intraventricular spillage. B: Right internal carotid arteriogram, lateral view, revealing a premature venous blush at the torcular and a venous aneurysm at the tentorial incisura. C: Same injection, anteroposterior view, during the early venous phase revealing the aneurysm and the galenic venous system and transverse sinus. D: Selective external carotid arteriogram, lateral view, visualizing the same venous aneurysm filling via middle meningeal branches. The aneurysm presents a small daughter sac superiorly, which was the site of hemorrhage. The tentorial leaflet and enclosed dural AVM nidus were resected via a right subtemporal approach.

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    Case 8. Right transverse sinus dural arteriovenous malformation (AVM) and acute temporal lobe hemorrhage. A: Computerized tomography (CT) scan showing intracerebral hematoma. B: Contrast-enhanced CT scan showing serpentine enhancement of vascular channels above and below the tentorial incisura. C: Right external carotid arteriogram, oblique view, showing high-flow shunting into the ipsilateral sigmoid-jugular system. D: Right external carotid arteriogram, lateral view, after embolization of several feeders; the site of shunting of the dural AVM nidus is now clearly localized to the middle third of the right transverse sinus. After multiple embolizations, the right transverse sinus and adjacent dura and tentorium were resected en bloc. E: Postoperative panangiography revealed no residual AVM (right external carotid injection shown).

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    Artist's illustration of three possible stages in the natural history of a dural arteriovenous malformation (AVM). A: Sinus thrombosis and engorged dural venous collaterals with opening of embryonic arteriovenous communications. B: Arteriovenous shunting favors recruitment of arterial feeders into the nidus (sump effect) with secondary venous hypertension. C: Venous hypertension favors leptomeningeal retrograde venous drainage and predisposes such channels to become varicose and aneurysmal. These three stages can be clearly documented in dural AVM's from our experience and from the literature.

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    Diagram of various stages of evolution in the natural history of dural arteriovenous malformations (AVM's) and of pathophysiological consequences and clinical manifestations of each stage. * = Probably accentuated by venous outflow obstruction; † = possibly triggered by extension of sinus thrombosis; ‡ = symptoms due to flow or local arterial or venous congestion.

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