The present essay is aimed at didactically presenting the classification and imaging findings of cavernous sinus arteriovenous fistulae. [ncbi.nlm.nih.gov]

The presentation can be varied and nonspecific; however, patients with CCFs commonly present with ophthalmic manifestations due to venous drainage of the orbit from the cavernous sinus. [eyewiki.aao.org]

• Hypertension

  Associations with hypertension, female gender, and older age have been reported.[14] An increased incidence is seen in pregnancy and is postulated to be related to a hormonally induced hypercoagulable state.[19] Indirect CCFs can also be seen in patients [eyewiki.aao.org]

• Tinnitus

  Post-traumatic pulsatile tinnitus: the hallmark of a direct carotico-cavernous fistula. J Laryngol Otol. 2007;121:1103–1107. ↑ 24.0 24.1 24.2 24.3 Williams ZR. Carotid-Cavernous Fistulae. [eyewiki.aao.org]

• Diplopia

  Symptoms may include decreased vision, diplopia, intractable headache or bruit, or worsening proptosis with exposure keratitis can. [eyewiki.aao.org]

• Visual Impairment

  Carotid-cavernous and orbital arteriovenous fistulas: ocular features, diagnostic and hemodynamic considerations in relation to visual impairment and morbidity. Orbit. 2003;22(2):121-142. ↑ 8.0 8.1 Chuman H, Trobe JD, Petty EM, et al. [eyewiki.aao.org]

• Epistaxis

  Traumatic carotid-cavernous sinus fistula with delayed epistaxis. Ear Nose Throat J. 1994;73:408–411. [PubMed] [Google Scholar] 8. Debrun G, Lacour P, Vinuela F, et al. Treatment of 54 traumatic carotid-cavernous fistulas. [ncbi.nlm.nih.gov]

• Headache

  The main signs and symptoms include proptosis; conjunctival hyperemia; chemosis; palsy of the II, IV, V and VI cranial nerves; ptosis; glaucoma; visual acuity reduction and headache(1). [ncbi.nlm.nih.gov]

  Symptoms may include decreased vision, diplopia, intractable headache or bruit, or worsening proptosis with exposure keratitis can. [eyewiki.aao.org]

• Intracranial Hemorrhage

  Intracranial hemorrhage may occur as a complication of an AVF in the CS region, and CT is the method of choice for such an evaluation(11). [ncbi.nlm.nih.gov]

This workup includes identifying the arterial supply, venous drainage, presence of intracranial or ophthalmic artery collaterals, and assessment of the carotid bifurcation before initiating treatment.[28] Most patients initially undergo noninvasive imaging [eyewiki.aao.org]

Neurovascular emergencies: imaging diagnosis and neurointerventional treatment. Emerg Radiol. 2017;24:183–193. ↑ Gemmete JJ, Chaudhary N, Pandey A, Ansari S. Treatment of Carotid Cavernous Fistulas. [eyewiki.aao.org]

Despite the similarity of symptoms between both types, a precise diagnosis is essential since the treatment is specific for each type of fistula. [ncbi.nlm.nih.gov]

Clinical and angiographic factors related to the prognosis of cavernous sinus dural arteriovenous fistula. Neuroradiology. 2010;53(12):983-992. [eyewiki.aao.org]

CCFs are classified based on the arterial system involved, hemodynamics, and etiology. [eyewiki.aao.org]

The AVF of the CS are classified according to anatomy, etiology and hemodynamics, into dural cavernous sinus fistulae (DCSF) and direct carotid-cavernous fistulae (CCF). [ncbi.nlm.nih.gov]

Epidemiology and etiology of dural arteriovenous fistula. Brain Nerve. 2008;60:883–886. [PubMed] [Google Scholar] 5. Woo HH, Masaryk TJ, Rasmussen PA. Treatment of dural arteriovenous malformations and fistulae. Neurosurg Clin N Am. 2005;16:381–393. [ncbi.nlm.nih.gov]

Disease ICD-10-CM I77.0 Arteriovenous fistula, acquired Epidemiology CCFs are a rare entity that can occur spontaneously or secondary to trauma. Traumatic CCFs account for the majority of CCFs. [eyewiki.aao.org]

The venous drainage patterns of the cavernous sinus are pertinent for the pathophysiology of CCFs. The cavernous segment of the ICA courses through the cavernous sinus (Figure 1). [eyewiki.aao.org]