Carotid Cavernous Fistula Clinical Presentation

  • Author: Ingrid U Scott, MD, MPH; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Mar 29, 2012
 

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:
    • Red eye
    • Diplopia
    • 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
    • Vitreous 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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Contributor Information and Disclosures
Author

Ingrid U Scott, MD, MPH  Professor, Department of Ophthalmology and Public Health Sciences, Pennsylvania State University College of Medicine

Ingrid U Scott, MD, MPH is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Cataract and Refractive Surgery, American Society of Retina Specialists, Association for Research in Vision and Ophthalmology, Macula Society, Phi Beta Kappa, and Retina Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Stephen D Plager, MD, FACS  Chief, Department of Ophthalmology, Dominican Hospital; Assistant Clinical Professor, Department of Ophthalmology, Stanford University Hospital

Stephen D Plager, MD, FACS is a member of the following medical societies: American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, and California Medical Association

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Mark T Duffy, MD, PhD  Consulting Staff, Division of Oculoplastic, Orbito-facial, Lacrimal and Reconstructive Surgery, Green Bay Eye Clinic, BayCare Clinic; Medical Director, Advanced Cosmetic Solutions, A BayCare Clinic

Mark T Duffy, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Sigma Xi, and Society for Neuroscience

Disclosure: Allergan - Botox Cosmetic Honoraria Speaking and teaching

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

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  2. Yu JS, Lei T, Chen JC, He Y, Chen J, Li L. Diagnosis and endovascular treatment of spontaneous direct carotid-cavernous fistula. Chin Med J (Engl). Aug 20 2008;121(16):1558-62. [Medline].

  3. Kirsch M, Henkes H, Liebig T, et al. Endovascular management of dural carotid-cavernous sinus fistulas in 141 patients. Neuroradiology. Jul 2006;48(7):486-90. [Medline].

  4. Yoon WK, Kim YW, Kim SR, Park IS, Kim SD, Baik MW. Transarterial coil embolization of a carotid-cavernous fistula which occurred during stent angioplasty. Acta Neurochir (Wien). May 5 2009;[Medline].

  5. Wang C, Xie X, You C, Zhang C, Cheng M, He M, et al. Placement of Covered Stents for the Treatment of Direct Carotid Cavernous Fistulas. AJNR Am J Neuroradiol. Apr 2 2009;[Medline].

  6. Bing F, Albrieux M, Vinh Moreau-Gaudry V, Vasdev A. Cavernous sinus fistula treated through the transvenous approach: Report of four cases. J Neuroradiol. Feb 27 2009;[Medline].

  7. de Keizer R. Carotid-cavernous and orbital arteriovenous fistulas: ocular features, diagnostic and hemodynamic considerations in relation to visual impairment and morbidity. Orbit. Jun 2003;22(2):121-42. [Medline].

  8. Debrun GM, Vinuela F, Fox AJ, et al. Indications for treatment and classification of 132 carotid-cavernous fistulas. Neurosurgery. Feb 1988;22(2):285-9. [Medline].

  9. Higginbotham EJ. Glaucoma associated with increased episcleral venous pressure. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology. 2nd ed. 2000: 2781-92.

  10. Ishijima K, Kashiwagi K, Nakano K, et al. Ocular manifestations and prognosis of secondary glaucoma in patients with carotid-cavernous fistula. Jpn J Ophthalmol. Nov-Dec 2003;47(6):603-8. [Medline].

  11. Keltner JL, Satterfield D, Dublin AB, Lee BC. Dural and carotid cavernous sinus fistulas. Diagnosis, management, and complications. Ophthalmology. Dec 1987;94(12):1585-600. [Medline].

  12. Miller NR. Carotid-cavernous sinus fistulas. In: Miller NR, ed. Walsh and Hoyt's Clinical Neuro-Ophthalmology. 4th ed. Baltimore, Md: Williams;1991: 2165-209.

  13. Rai AT, Sivak-Callcott JA, Larzo C, Marano GD. Direct carotid cavernous fistula in infancy: presentation and treatment. AJNR Am J Neuroradiol. Jun-Jul 2004;25(6):1083-5. [Medline].

  14. Troost BT, Glaser JS, Morris PP. Aneurysms, arteriovenous communications, and related vascular malformations. In: Glaser, ed. Neuro-ophthalmology. 3rd ed. Philadelphia, Pa: Lippincott Williams & Wilkins;1999: 589-628.

 
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