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© 2013 Neurocritical Care Society Practice Update

CAVERNOUS CAROTID AND DURAL ARTERIOVENOUS FISTULAS

Owen Samuels, MD Emory University Atlanta, GA

Adam Webb, MD Emory University Atlanta, GA

OVERVIEW AND EPIDEMIOLOGY

Arteriovenous fistulas are abnormal shunts between the arterial and venous circulation. Dural arteriovenous fistulas (DAVFs) and cavernous carotid fistulas (CCFs) are forms of arteriovenous fistulas in the central nervous system. The incidence in the population is unknown but they are thought to make up 5-20% of intracranial vascular malformations [1].

DAVFs are direct connections between meningeal arteries and draining veins contained within the dura. They can be spinal or intracranial. Though the majority of DAVFs are thought to be acquired lesions, most are idiopathic, while some result from cranial surgery, trauma, or venous sinus thrombosis. Venous outflow obstruction can precede formation of DAVFs [2].

DAVFs are classified predominantly by their venous drainage. The Borden classification system divides DAVFs into 3 types depending on if they drain into dural venous sinuses or into subarachnoid veins (See Table 1) [3].

CCFs are divided into direct and indirect forms. Direct CCFs are high flow shunts usually resulting from trauma or aneurysm rupture causing direct communication between the cavernous portion of the internal carotid artery and the cavernous sinus. Indirect CCFs are more akin to DAVFs with indirect communication between the carotid circulation (either internal or external or both) and the cavernous sinus through dural arteries and veins. The cause of indirect CCFs is unclear and likely shares a similar pathophysiological mechanism with DAVFs. CCFs are classified as Type A-D depending on the flow rate and arterial supply (See Table 2) [4].

Spontaneous CCFs are more common in middle-aged and elderly women. Other risk factors include pregnancy, systemic hypertension, connective tissue diseases and trauma [5].

PATHOPHYSIOLOGY

Patients with DAVFs may present with hemorrhage or neurological symptoms secondary to venous congestion. Hemorrhages may be intracerebral, subarachnoid or subdural. A recent

© 2013 Neurocritical Care Society Practice Update

study described 50% of ruptured DAVFs presenting with pure intracerebral hemorrhage and 50% presenting with a combination of intracerebral and subarachnoid or subdural hemorrhage [6]. Neurological symptoms are related to the anatomical location of the fistula and venous drainage.

Borden type II and III DAVFs are more likely to present with clinical symptoms and or hemorrhage. The risk of hemorrhage is about 2% per year [7].

Factors associated with an increased risk of hemorrhage include cortical drainage, retrograde venous drainage, venous varix, and drainage into the vein of Galen [8].

CCFs allow high-pressure arterial blood flow to be transmitted to the cavernous sinus creating venous hypertension. This predominantly affects the ophthalmic venous system.

CLINICAL FEATURES

Symptoms and Exam Findings DAVFs with venous drainage into the transverse or sigmoid sinus may present with pulsatile tinnitus. Those involving the cavernous sinus may present with ophthalmoplegia, chemosis, proptosis, and retroorbital or facial pain. Patients may present with seizures, impairment of consciousness or cognition as well as with symptoms of intracranial hypertension. Patients with spinal DAVFs may present with myelopathy. Symptoms may be acute, especially with hemorrhage, or may present in a subacute progressive or even remitting/relapsing manner.

CCFs present with a combination of a cavernous sinus and orbital syndrome with variable ophthalmoplegia, proptosis, chemosis, and orbital pain. Vision loss may develop with rising orbital and intraocular pressure. An orbital bruit may be heard. Direct CCFs secondary to trauma or aneurysm rupture demonstrate an abrupt onset of symptoms while indirect CCFs follow a more indolent course.

DIAGNOSIS

Non-contrast head CT is insufficient to detect a DAVF in the absence of hemorrhage. A high level of suspicion should be maintained in patients who present with intracranial hemorrhage without significant risk factors (age, trauma, hypertension, anticoagulation, etc.) and in those with an atypical pattern of hemorrhage, such as a combination of intracerebral hemorrhage and subarachnoid or subdural hemorrhage. This should prompt follow up imaging with MRI and MR angiography/venography or catheter angiography.

Additionally, patients who present with subarachnoid hemorrhage in a non-aneurysmal pattern should raise concern for cerebral or cervical spinal DAVF.

In those patients who present with progressive neurological symptoms without hemorrhage, subtle venous dilation and or parenchymal signal change may be seen.

© 2013 Neurocritical Care Society Practice Update

CT findings in CCFs include proptosis, enlargement of the superior ophthalmic vein as well as prominent extraocular muscles. MRI can demonstrate orbital edema and an abnormal flow void within the cavernous sinus [9].

Catheter cerebral and/or spinal angiography is the gold standard for diagnosis of DAVFs and is necessary for any patient with a suspected or confirmed DAVF or CCF for classification and treatment planning.

TREATMENT

DAVF presenting with hemorrhage or progressive neurological deficits require evaluation for either surgical or endovascular treatment. Surgical treatment involves the disconnection of arterialized veins with preservation of the sinus if it is patent or excision of the sinus and surrounding dura if it is occluded. Endovascular treatment involves transarterial or transvenous coil or liquid chemical embolization. Stereotactic radiosurgery as a primary or adjunct therapy is also safe and can be effective.

Recurrence of DAVFs after treatment has been described.

Many CCFs will resolve spontaneously. Direct CCFs and those with intractable symptoms or those that threaten vision mandate treatment.

The goal of treatment of direct CCFs is the elimination of the communication between the ICA and the cavernous sinus. This may be accomplished via a transarterial or transvenous route by embolization of the fistula with a detachable balloon, detachable coils or liquid embolic agent. Additionally, stents may be deployed in the ICA to assist closure. Occasionally, the only treatment option is sacrifice of the ICA.

The goal of treatment of indirect CCFs is to diminish the shunt between the arterial and venous circulation and decrease the venous pressure in the cavernous sinus. This can be achieved by transarterial embolization of the arterial feeders or transvenous embolization of the cavernous sinus [9].

REFERENCES

1. Wecht D, Awad I. Carotid Cavernous and Other Dural Arteriovenous Fistulas in Primer on Cerebrovascular Diseases 1997.

2. Brown R, Flemming K, Meyer F, Cloft H, Pollock B, Link M. Natural history, evaluation, and management of intracranial vascular malformations. Mayo Clinic Proc 2005;80:269-281.

3. Borden JA, Wu JK, Shucart WA. A proposed classification for spinal and cranial dural arteriovenous fistulous malformations and implications for treatment. J Neurosurg

1995;82:166-179.

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4. Barrow DL, Spector RH, Braun IF, Landman JA, Tindall SC, Tindall GT. Classification and treatment of spontaneous carotid-cavernous sinus fistulas. J Neurosurg 1985;62:248-256.

5. Miller N. Diagnosis and management of dural carotid-cavernous sinus fistulas. Neurosurg focus 2007;23:E13.

6. Cordonnier C, Al-Shahi Salman R, Bhattacharya JJ, Counsell CE, Papanastassiou V, Ritchie V, Roberts RC, Sellar RJ, Warlow C. Differences between intracranial vascular malformation types in the characteristics of their presenting haemorrhages: prospective, population- based study. J Neurol Neurosurg Psychiatr 2008;79:47-51.

7. Brown R, Wiebers D, Nichols D. Intracranial dural arteriovenous malformations: a clinical, radiologic, and long-term followup study [abstract]. Stroke 1992;23:157. Abstract 85.

8. Awad I, Little J, Akrawi W, Ahl J. Intracranial dural arteriovenous malformations: factors predisposing to an aggressive neurological course. J Neurosurg 1990;72:839-850.

9. Gemmete JJ, Ansari SA, Gandhi DM. Endovascular techniques for treatment of carotid- cavernous fistula. J Neuro-Ophthalmol 2009;29:62-71.

10. Wilson M, Enevoldson P, Menezes B. Intracranial dural arterio-venous fistula. Pract Neurol

2008;8:362-369.

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Table 1. Borden Classification of DAVFs

 

Venous Drainage

Clinical Course

Type I

Directly into dural venous sinuses or meningeal veins

Often benign, high rate of spontaneous remission

Type II

Directly into dural venous sinuses or meningeal veins but also have retrograde drainage into subarachnoid veins

Progressive neurological deficit or hemorrhage

Type III

Directly into subarachnoid veins without dural sinus or meningeal venous drainage

Progressive neurological deficit or hemorrhage

Table 2. Barrow Classification of CCFs

Type A: Direct high-flow shunts between the internal carotid artery and the cavernous sinus.

Type B:

Dural shunts between the meningeal branches of the ICA and the cavernous

Type C:

sinus. Dural shunts between the meningeal branches of the ECA and the cavernous

sinus. Type D: Dural shunts between the meningeal branches of both the ICA and ECA and the cavernous sinus.