An 85-year-old female with a past history of hypertension presents with acute right-eye pain, redness, and proptosis/bulging for the past two months that has been worsening over the past two days. She endorses blurry vision that began two days prior. She does not use contacts or glasses. No trauma, headache, or loss of consciousness are reported. She reports a “whooshing” sound in her right ear for two to three months.
Vitals:
- HR 87
- BP 167/83
- RR 16
- SpO2 99% on room air
HEENT:
- Pupils are equal, round, and reactive to light and accommodation bilaterally.
- The right eye has chemosis and subconjunctival hemorrhage.
- There is ptosis, proptosis, and pulsatility of the right eye.
- Extraocular movements are intact in the left eye. In the right eye, they are limited in all directions, although less when doing adduction.
- Visual Acuity: the right eye is 20/70, the left eye is 20/80.
- There is an ocular bruit heard over the right side.
Neurological: Her remaining cranial nerves are intact with her face symmetric and tongue midline; there is no pronator drift, muscle strength is 5/5 in upper and lower extremities, and there are no sensory deficits.
None
The diagnosis is a carotid-cavernous fistula.
The most common clinical signs are proptosis, dilated conjunctival vessels, and the presence of an orbital bruit.
Computed tomography (CT) angiography is the noninvasive imaging modality of choice for diagnosing carotid-cavernous fistula.
Ultimate management most commonly includes endovascular therapy for closure.
A carotid-cavernous fistula (CCF) is an abnormal communication between the venous cavernous sinus and the carotid artery [1]. The fistula may occur spontaneously, but most commonly occurs following head trauma. In one retrospective study, the time to presentation following injury ranged from one day to as late as two years after injury.
There are two distinct types of carotid-cavernous fistulas:
- A direct fistula is a high-flow fistula between the cavernous internal carotid artery and the cavernous sinus. It is the most common CCF following head trauma and is thought to form from a traumatic tear in the wall of the cavernous internal carotid artery or following the rupture of an aneurysm [2]. Thus, high-pressure arterial blood gains rapid access to the venous system and leads to venous hypertension, causing the presence of an orbital bruit, exophthalmos, proptosis, dilated conjunctival vessels, and cranial nerve dysfunction as seen in this patient.
- Indirect fistulas are low-flow and typically occur between branches of the external or internal carotid artery and the cavernous sinus. The etiology of this type of fistula is unclear, but it has been associated with pregnancy, sinusitis, age, and trauma. These low-flow fistulas generally resolve without treatment [2].
CCFs typically require endovascular therapy. This may be transarterial (most commonly in the case of direct CCF) or transvenous (most commonly in indirect CCF).
Take-Home Points
- There are two distinct types of carotid-cavernous fistulas: indirect and direct.
- Clinical findings include venous congestion of the eyelids, palsies of cranial nerves 3, 4, or 6, progressive visual loss, proptosis, and an ocular bruit.
- Once a CCF is diagnosed, it is important to consult the appropriate treating specialist, either an interventional neurologist or neurosurgeon.
- Chaudhry IA, Elkhamry SM, Al-Rashed W, et al. Carotid Cavernous Fistula: Ophthalmological Implications. Middle East Afr J Ophthalmol 2009;16:57–63. doi:10/d854ss. PMCID: PMC2813585
- Miller NR. Dural carotid-cavernous fistulas: epidemiology, clinical presentation, and management. Neurosurg Clin N Am 2012;23:179–92. doi:10/csvj77. PMID: 22107868
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