Cavernous dural arteriovenous fistula with cortical venous reflux

Case contributed by Peter Mitchell
Diagnosis certain

Presentation

Recurrent right eye chemosis, proptosis, diplopia.

Patient Data

Age: 70 years
Gender: Female

Right superior ophthalmic vein engorged, prominent veins in Sylvian fissure. T2 and FLAIR signal in the frontal operculum, all consistent with right cavernous dural arteriovanous fistula (dAVF) and cortical vein drainage with venous hypertension.

Initial angiograms showed right cavernous dAVF with slow filling of cavernous sinus from ECA and ICA meningeal branches, with cortical venous opacification to Sylvian vein.

Further improvement in appearance, hence no treatment offered as now asymptomatic. MR follow-up confirmed resolution of all high-risk features.

The area of venous infarction/congestion shown on presenting MRI has resolved, as have the MR features of the cavernous dAVF

Case Discussion

Patient presents with features suspicious for cavernous sinus dAVF (also known as caroticocavernous fistula (CCF)). Close inspection identifies the enlarged and asymmetric ipsilateral superior ophthalmic vein, but note should be made of the unusual FLAIR signal of the right frontal lobe adjacent to the Sylvian fissure. This is not typical of small vessel ischemia or old infarction as would be suspected from patient age and intracranial atherosclerosis.  It should prompt consideration of other pathologies - venous hypertension or infarction in this case with ipsilateral cavernous dAVF; tumor in other circumstances.

Close inspection of the DSA confirms retrograde opacification of a cortical vein in the Sylvian fissure, confirming venous "infarction" or hypertension as the etiology, confirmed by resolution of the changes following spontaneous thrombosis of the fistulas on follow-up imaging.

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