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.
2013 Neurocritical Care Society Practice Update

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.


2013 Neurocritical Care Society Practice Update

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
sinus.
Type C: 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.