INTRACEREBRAL HEMORRHAGE / VASCULAR MALFORMATIONS

Carotid-cavernous fistula (CCF)

Created 21/04/2021, last revision 23/01/2023

  • carotid-cavernous fistula is a specific variant of arteriovenous fistula (→ DAVF) – it is defined as a pathological communication between the cavernous sinus (CS) and 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, the superior ophthalmic vein (SOV), the inferior ophthalmic vein (IOV), the superior petrosal sinus (SPS), the inferior petrosal sinus (IPS), and the 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 a 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
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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 the heartbeat and disappearing after compression of the carotid artery 
  • painful ophthalmoparesis with diplopia (symptoms may fluctuate) [Li, 2019]
  • ipsilateral amaurosis
  • conjunctival chemosis ipsi- or bilaterally
  • dilated subcutaneous periorbital veins
  • papilledema
  • hemorrhagic complications (2%) – SAH, ICH, epistaxis
Ocular manifestations of CCF

Diagnostic evaluation

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Differential diagnosis

  • tumors compressing the cavernous sinus (e.g., pituitary adenomas, craniopharyngiomas)
  • endocrine orbitopathies Endocrine orbitopathy on MRI
    • no significant chemosis, no increased filling of  vessels on the fundus
  • cavernous sinus thrombosis
  • retrobulbar orbital expansion
  • cluster headache

Management

  • endovascular (transarterial or transvenous) embolization techniques are the preferred treatment modality for the management of CCFs
  • tinnitus disappears immediately after successful procedure
  • eye symptoms resolve gradually (within weeks-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 previous 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
Embolization of feeding artery (CCF Barrow type C)

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Carotid-cavernous fistula (CCF)
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