|
SYMPTOMS
|
Orbital pain/pressure | diplopia | swishing sound | headaches | rarely vision loss
|
|
SIGNS
|
Pulsatile proptosis | Horner’s syndrome | EOM restrictions | dilated (corkscrew) conjunctival and episcleral vessels | chemosis | elevated IOP | tortuous retinal veins | vein occlusions | papilledema | retinal hemorrhages
|
|
DIRECT carotid-cavernous fistulas (usually high flow and caused by trauma) are more likely to present with signs and symptoms than INDIRECT carotid-cavernous fistulas (usually low flow and occur spontaneously)
|
|
WORK-UP
|
Thorough history | Pupils | EOMs | Slit lamp examination | Dilated retinal examination | Exophthalmometry | Check for orbital bruit (Ocular auscultation)
|
|
TREATMENT
|
Refer to a neuro-ophthalmologist STAT for additional testing and treatment. Treatment is usually conservative but surgery is an option: surgical resection, radiation therapy, or embolization
|
|
FOLLOW-UP
|
Depends on severity. A multi-disciplinary approach is warranted (neurology, oculoplastics, etc.). After seeing neuro-ophthalmogist, patients should be followed every 6-12 months.
|
|
ADDITIONAL LAB | TESTS
|
CT | CTA | MRI of the brain and orbits with and without contrast | MRA | Digital Subtraction Angiography (DSA) is the gold standard.
|
|
ETIOLOGY
|
Abnormal connections between the carotid artery and venous channels of the cavernous sinus. Can occur spontaneously or secondary to trauma or surgery.
|
|
DIFFERENTIAL DX
|
Thyroid eye disease | idiopathic orbital inflammatory syndrome | cavernous sinus thrombosis | superior orbital fissure syndrome | retrobulbar hemorrhage | orbital apex syndrome.
|
|
NOTES
|
Improvement in ocular signs and symptoms typically occurs shortly after treatment.
|
