Carotid-Cavernous Fistula (CCF) Clinical Presentation

Updated: May 24, 2019
  • Author: Ingrid U Scott, MD, MPH; Chief Editor: Hampton Roy, Sr, MD  more...
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Presentation

History

Elicit history of trauma, recent childbirth, or surgical manipulation.

Elicit history of atherosclerosis, systemic hypertension, collagen vascular disease, pseudoxanthoma elasticum, connective tissue diseases (eg, Ehlers-Danlos syndrome), or pregnancy.

Patients may present with the following ocular complaints:

  • Bruit (buzzing or swishing sounds)

  • Decreased vision

  • Bulging eye

  • Facial pain in the distribution of the first (and rarely the second) division of the trigeminal nerve

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Physical

Ophthalmologic examination findings consistent with carotid-cavernous sinus fistula include the following:

  • Proptosis

  • Eyelid edema

  • Ocular pulsations (visible and/or palpable)

  • Pulsating exophthalmos

  • Ocular bruit

  • Conjunctival arterialization and chemosis

  • Exposure keratopathy

  • Dilation of retinal veins

  • Optic disc swelling

  • Intraretinal hemorrhage

  • Proliferative retinopathy

  • Central retinal vein occlusion

  • Elevated intraocular pressure

  • Neovascular glaucoma

  • Angle-closure glaucoma (In rare cases, increased orbital venous pressure leads to iris and choroid congestion and forward displacement of the iris-lens diaphragm.)

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Causes

Approximately 25% of carotid-cavernous sinus fistulae occur spontaneously, especially in middle-aged to elderly women, and may be associated with atherosclerosis, systemic hypertension, collagen vascular disease, pregnancy, connective tissue disorders (eg, Ehlers-Danlos syndrome), and minor trauma.

Cerebral trauma accounts for approximately 75% of carotid-cavernous sinus fistulae, with motor vehicle accidents, fights, and falls representing the most common settings. The injuries may be penetrating or nonpenetrating and may be associated with basal or facial skull fracture.

Iatrogenic fistulae have been reported following trans-sphenoidal pituitary surgery, endarterectomy, ethmoidal sinus surgery, and percutaneous gasserian and retro-gasserian procedures.

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