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Caroticocavernous Fistula Treatment & Management

  • Author: Michael G Nosko, MD, PhD; Chief Editor: Brian H Kopell, MD  more...
 
Updated: Oct 28, 2015
 

Medical Therapy

In the acute setting of vision loss and/or paralysis of cranial nerves, glucocorticosteroids (eg, dexamethasone) may be used while waiting for definitive diagnostic studies and treatments.

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Surgical Therapy

The definitive management of a caroticocavernous fistula is obliteration of the fistulous connection with restoration of normal arterial and venous flow. This is achieved most often through an endovascular approach. After complete delineation of the fistulous tract, an approach can be planned to close the fistula.

Type-A fistulas usually are approached through the internal carotid artery. A detachable balloon then can be positioned to occlude the fistula while maintaining patency of the internal carotid artery. Venous approaches through the internal jugular vein and the petrosal sinus may allow access to the fistula from the venous side. Guglielmi detachable coils also may be used and are becoming increasingly popular.

Type B, C, and D fistulas have smaller fistulous connections and usually are not amenable to the aforementioned treatment approaches. Carotid self-compression for 20-30 seconds 4 times per hour may lead to thrombosis of the fistula. Patients are instructed to compress the carotid artery on the side of the lesion using their contralateral hand. Should the patient develop cerebral ischemia during the compression, the contralateral hand likely will be affected, releasing the compression.

If compression is not effective or if a more rapid intervention is indicated, selective endovascular embolization of the fistula through the external carotid artery usually is effective. Several choices of embolic material are available, although polyvinyl alcohol usually is preferred.

Occasionally a fistula may require an endovascular approach through the superior ophthalmic vein. This requires surgical exposure of the vein to allow placement of the catheter.

Direct surgical exposure and obliteration of the fistula has been described. This rarely is indicated because endovascular approaches have been developed.

Severely refractory fistulas can be treated by surgical or endovascular sacrifice of the internal carotid artery. This too, rarely is indicated.

In a study of 38 patients with dural carotid-cavernous fistulas, medical treatment was performed in 16% of patients, external ocular compression in 8%, transarterial embolisation in 13%, transvenous embolisation in 60%, and radiosurgery in 3%. Clinical cure was achieved in 58% of patients and improvement in 24%; anatomical cure was demonstrated in 68%; and transient worsening or new onset of ocular symptoms was observed in 29%.[6]

In a study of clinical and neuroradiologic results in 13 patients with carotid-cavernous fistulas treated by coiling of the cavernous sinus, there was complete occlusion of the fistula in 7 patients (7/13, 54%) and a resolution of symptoms in 8 patients (8/12, 67%). Coiling was performed with a semicompliant nondetachable balloon inflated in the internal carotid artery. The authors noted that balloon-assisted coiling permitted a clear visualization of the fistula, facilitated coil positioning, and protected the patency of the artery.[7]

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Follow-up

Patients usually require a follow-up angiogram to ensure that the fistula has not recurred or that alternate fistulous pathways have not developed.

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Complications

Complications in untreated lesions usually are limited to visual loss, cranial nerves paralysis, and the cosmetic concerns of proptosis.

Complications of treatment include the standard complications of cerebral angiography. Arterial and venous compromise also may occur, yielding cerebral or retinal ischemia and resultant infarction.

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Outcome and Prognosis

Patients with caroticocavernous fistulas generally have a good prognosis. These fistulas are associated with a high incidence of spontaneous resolution. Persistent lesions respond well to intervention. The risk of nonophthalmologic neurological complications is not significant; however, persistent untreated lesions may cause significant visual complications.

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Future and Controversies

As techniques for endovascular stenting are developed further, internal carotid artery stenting across the fistula may have a role. This may provide a safer treatment option with respect to maintaining patency of the internal carotid artery while obliterating the fistula.

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Contributor Information and Disclosures
Author

Michael G Nosko, MD, PhD Associate Professor of Surgery, Chief, Division of Neurosurgery, Medical Director, Neuroscience Unit, Medical Director, Neurosurgical Intensive Care Unit, Director, Neurovascular Surgery, Rutgers Robert Wood Johnson Medical School

Michael G Nosko, MD, PhD is a member of the following medical societies: Academy of Medicine of New Jersey, Congress of Neurological Surgeons, Canadian Neurological Sciences Federation, Alpha Omega Alpha, American Association of Neurological Surgeons, American College of Surgeons, American Heart Association, American Medical Association, New York Academy of Sciences, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Ryszard M Pluta, MD, PhD Associate Professor, Neurosurgical Department Medical Research Center, Polish Academy of Sciences, Poland; Clinical Staff Scientist, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health (NIH); Fishbein Fellow, JAMA

Ryszard M Pluta, MD, PhD is a member of the following medical societies: Polish Society of Neurosurgeons, Congress of Neurological Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Brian H Kopell, MD Associate Professor, Department of Neurosurgery, Icahn School of Medicine at Mount Sinai

Brian H Kopell, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, International Parkinson and Movement Disorder Society, Congress of Neurological Surgeons, American Society for Stereotactic and Functional Neurosurgery, North American Neuromodulation Society

Disclosure: Received consulting fee from Medtronic for consulting; Received consulting fee from St Jude Neuromodulation for consulting; Received consulting fee from MRI Interventions for consulting.

Additional Contributors

Duc Hoang Duong, MD Professor, Chief Physician, Departments of Neurological Surgery and Neuroscience, Epilepsy Center, Charles Drew University of Medicine and Science

Duc Hoang Duong, MD is a member of the following medical societies: American Neurological Association, Congress of Neurological Surgeons, North American Skull Base Society

Disclosure: Nothing to disclose.

References
  1. Barrow DL, Spector RH, Braun IF. Classification and treatment of spontaneous carotid-cavernous sinus fistulas. J Neurosurg. 1985 Feb. 62(2):248-56. [Medline].

  2. Karaman E, Isildak H, Haciyev Y, Kaytaz A, Enver O. Carotid-cavernous fistula after functional endoscopic sinus surgery. J Craniofac Surg. 2009 Mar. 20(2):556-8. [Medline].

  3. Hieshima GB, Cahan LD, Mehringer CM. Spontaneous arteriovenous fistulas of cerebral vessels in association with fibromuscular dysplasia. Neurosurgery. 1986 Apr. 18(4):454-8. [Medline].

  4. Bacon KT, Duchesneau PM, Weinstein MA. Demonstration of the superior ophthalmic vein by high resolution computed tomography. Radiology. 1977 Jul. 124(1):129-31. [Medline].

  5. Seeger A, Kramer U, Bischof F, Schuettauf F, Ebner F, Danz S, et al. Feasibility of Noninvasive Diagnosis and Treatment Planning in a Case Series with Carotid-Cavernous Fistula using High-Resolution Time-Resolved MR-Angiography with Stochastic Trajectories (TWIST) and Extended Parallel Acquisition Technique (ePAT 6) at 3 T. Clin Neuroradiol. 2015 Sep. 25 (3):241-7. [Medline].

  6. Rodrigues T, Willinsky R, Agid R, TerBrugge K, Krings T. Management of dural carotid cavernous fistulas: a single-centre experience. Eur Radiol. 2014 Dec. 24 (12):3051-8. [Medline].

  7. De Renzis A, Nappini S, Consoli A, Renieri L, Limbucci N, Rosi A, et al. Balloon-assisted coiling of the cavernous sinus to treat direct carotid cavernous fistula. A single center experience of 13 consecutive patients. Interv Neuroradiol. 2013 Sep. 19 (3):344-52. [Medline].

  8. Dandy WE, Follis RH Jr. On the pathology of carotid-cavernous aneurysms (pulsating exophthalmos). Am J Ophthalmol. 1941. 24:365-385.

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

  10. Hamby WB. Carotid-cavernous fistula. Springfield, Ill: Charles C Thomas. 1966.

  11. Newton TH, Hoyt WF. Dural arteriovenous shunts in the region of the cavernous sinus. Neuroradiology. 1970. 1:71-81.

  12. Pan HC, Sun MH, Sheehan J, Sheu ML, Chen CC, Lee HT. Radiosurgery for dural carotid-cavernous sinus fistulas: Gamma Knife compared with XKnife radiosurgery. J Neurosurg. 2010 Dec. 113 Suppl:9-20. [Medline].

  13. Serbinenko FA. Balloon catheterization and occlusion of major cerebral vessels. J Neurosurg. 1974 Aug. 41(2):125-45. [Medline].

  14. Walker AE, Allegre GE. Carotid-cavernous fistulas. Surgery. 1956. 39:411-422.

 
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Type-D caroticocavernous fistula: the eye demonstrates proptosis, chemosis, and scleral edema. The patient is unable to close the eye, exposing the cornea to dehydration and potential trauma.
Panel A is an angiogram of caroticocavernous fistula showing filling of the cavernous and circular sinuses. Panel B shows a post-Guglielmi detachable coil, ie, coiling of the fistula. The red arrow points to coils within the cavernous and circular sinuses after obliteration of the fistula.
This is a diagrammatic representation of the 4 types of caroticocavernous fistulas. ICA is the internal carotid artery; ECA is the external carotid artery.
 
 
 
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