INTRACEREBRAL HEMORRHAGE / VASCULAR MALFORMATIONS
Carotid-cavernous fistula (CCF)
Created 21/04/2021, last revision 26/06/2023
- carotid-cavernous fistula is a specific variant of arteriovenous fistula (→ DAVF) – it is defined as a pathological communication between the cavernous sinus (CS) and the internal carotid artery (ICA) or its branches (from either ICA or ECA or both)
- CCF can occur spontaneously or as a result of trauma
- the cavernous sinus is a venous plexus that receives drainage from the sphenoparietal sinus, superior ophthalmic vein (SOV), inferior ophthalmic vein (IOV), superior petrosal sinus (SPS), inferior petrosal sinus (IPS), and basilar venous plexus → anatomy of cerebral veins and dural sinuses
Classification
- the Barrow classification is the most widely used system to categorize CCFs
- type A is usually of traumatic etiology with the classic triad of clinical symptoms (tinnitus, pulsatile exophthalmos, and conjunctival chemosis)
- indirect CCFs (C-D) are usually of spontaneous origin with milder clinical presentation
Etiology
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Hemodynamic classification
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Anatomy (type A-D) – the Barrow classification
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Clinical presentation
These symptoms are usually fully expressed in type A, whereas the findings are more subtle in indirect types (often only retrobulbar pain, diffuse headache, or conjunctival hyperemia)
- pulsatile or persistent exophthalmos (proptosis)
- ipsilateral (up to 75%)
- bilateral (up to 1/3 of cases)
- contralateral to CCF
- pulsatile tinnitus (mostly synchronous with the heartbeat) + murmur audible in the forehead, synchronous with a heartbeat and disappearing after compression of the carotid artery
- painful ophthalmoparesis with diplopia (symptoms may fluctuate) [Li, 2019]
- ipsilateral amaurosis
- ipsilateral or bilateral conjunctival chemosis
- dilated subcutaneous periorbital veins
- papilledema
- hemorrhagic complications (2%) – SAH, ICH, epistaxis
Diagnostic evaluation
Management
- endovascular (transarterial or transvenous) embolization techniques are the preferred treatment modality for the management of CCFs
- tinnitus disappears immediately after a successful procedure
- eye symptoms resolve gradually (within weeks to months)
Direct fistula (type A)
Endovascular treatment
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Neurosurgery
- open surgery or radiosurgery are used as second-line or adjuvant therapeutic options (typically after the prior failure of endovascular procedures)
Indirect fistula (type B-D)
- it is recommended to monitor the patient with low-flow fistulas and mild symptoms. Spontaneous resolution of symptoms due to fistula thrombosis is not uncommon
- the effect of repeated external carotid compression under ultrasound guidance has been reported [Higashida, 1986] [Goldemund, 2006)
- endovascular treatment is suggested in more severe cases and those with clinical progression (visual deterioration, malignant exophthalmos, and chemosis)
- coiling of the feeding artery
- transvenous cavernous sinus coiling