A carotid cavernous sinus fistula, abbreviated as CCF, is a pathological channel which leads to the communication of the carotid artery and the cavernous sinus in the skull.
Presentation
The first step towards a successful diagnosis is a thorough medical history, including recent traumatization to the head, recent surgical intervention, pregnancy, and labor, as well as comorbidities that may increase the risk for the development of a fistula, such as hypertension, collagen vascular disease, atherosclerosis, etc.
Patients usually initially consult an ophthalmologist, since ocular symptoms are the only predominant symptoms caused by a carotid cavernous sinus fistula. Individuals affected by the condition commonly present with a red eye, attributed to the edema of the conjunctiva due to the blockage of the blood flow and the high blood pressure [9]. They report ocular bruit, namely distinct vibrations caused by the turbulence in intracranial blood vessels; bruit is experienced by the patient as a buzz or a swishing sound. Exophthalmos is common, as well as vision impairment; diplopia or a recent deterioration in the ability to see may be reported by the patient. Lastly, such fistulae may lead to palsies of the cranial nerves and affected individuals frequently complain of pain on the face, which follows the path of the 1st division of the trigeminal nerve.
An examination will help to reveal further signs which are compatible with the existence of a carotid cavernous sinus fistula. Intraocular pressure is often augmented and the development of angle-closure glaucoma may be attributed to the elevated pressure in the veins of the episclera or to retinal ischemia, which leads to a secondary neovascularization [10]. Vitreous or intraretinal hemorrhagic phenomena may be observed, alongside edema of the optic disc and a dilatation of the retinal veins [11]. Ophthalmoplegia is also often exhibited, which may imitate the manifestations of Graves' ophthalmopathy [12]. Eye pulsations may be felt or seen with a bare eye and proptosis, accompanied by exposure keratopathy, are also common manifestations.
Entire Body System
- Pain
A 61-year-old woman presented with painful ophthalmoplegia, Tolosa-Hunt syndrome. The patient had undergone a placement of dental implant 5 months before the presentation and had a local maxillary sinusitis 1 month later. [ncbi.nlm.nih.gov]
Gastrointestinal
- Nausea
Initial symptoms were diplopia in 3 cases, severe orbital pain or headache with nausea and vomiting in 3 cases, dull pain around the orbit in 3 cases, red eye in 2 cases and tinnitus in 2 cases. [jstage.jst.go.jp]
[…] both openings on the skin. perilymph fistula rupture of the round window with leakage of perilymph into the inner ear, so that changes in middle ear pressure directly affect the inner ear, causing sensorineural deafness as well as dizziness, vertigo, nausea [medical-dictionary.thefreedictionary.com]
Case Report A 73-year-old right-handed woman suffered from tinnitus, occipital headache, nausea, and anorexia since March 2009. [karger.com]
[…] neoplasm (=悪性腫瘍) 【悪性組織球症】*malignant histiocytosis (略 MH) 【悪性中皮腫】*malignant mesothelioma 【悪性貧血】*pernicious anemia 【悪性黒子】*lentigo maligna[L] 【悪性黒子黒色腫】*lentigo maligna melanoma (略 LMM) 【悪性傍神経節腫】*malignant paraganglioma 【悪性リンパ腫】*malignant lymphoma 【悪心】*nausea [medo.jp]
- Dyschezia
肺胞内肺炎】*intra-alveolar pneumonia 【肺癆】*phthisis pulmonum[L]〈肺結核のこと〉 (敗) 【敗血症】*sepsis *septicemia 【敗血性子宮炎】*septimetritis 【敗血症性ショック】*septic shock (胚) 【胚細胞腫】*germ cell tumor *germinoma (排) 【排尿緩慢】*bradyuria 【排尿困難】*dysuria 【排尿痛】*urodynia *uralgia 【排便困難】【排便障害】*dyschezia [medo.jp]
Cardiovascular
- Hypertension
These findings suggest that the absence of orbito-ocular signs in dural CCF with an anterior venous drainage could be attributed to the relief of venous hypertension of the SOV. [ncbi.nlm.nih.gov]
Clinical signs often include VISION DISORDERS and INTRACRANIAL HYPERTENSION. [definitions.net]
Often in the setting of hypertension / atherosclerotic disease, these congenital shunts proliferate into a carotid cavernous fistula. [eyewiki.org]
[…] conjunctival congestion and hemorrhage, with potential bleeding in the retina and vitreous humor Increase in intraocular pressure Optic disc swelling If not treated swiftly, carotid-cavernous fistulas may result in cerebral hemorrhage/ infarction, intracranial hypertension [amboss.com]
- Vascular Disease
However, these fistulas may be congenital arteriovenous connections that open spontaneously in the settings of collagen vascular disease, atherosclerosis, hypertension, or may develop in females during peripartum period. [ncbi.nlm.nih.gov]
Etiology Direct CCF may result from trauma, rupture of a cavernous carotid aneurysms, or from a tear in the wall of a congenitally weak cavernous ICA secondary to collagen vascular disease. Indirect CCF are usually spontaneous and idiopathic. [neurosurgery.pitt.edu]
Dural cavernous sinus fistulas (DCSFs) are benign vascular diseases consisting in an arteriovenous shunt at the cavernous sinus that if misdiagnosed can lead to potentially serious ophthalmologic complications. [books.google.com]
- Carotid Bruit
Other systemic examinations were unremarkable except for bilateral carotid bruits. [intjem.springeropen.com]
Skin
- Flushing
These patients receive many needle sticks to flush their blood through dialysis machines and for routine blood analysis testing. The veins used may scar and become difficult to use. [medical-dictionary.thefreedictionary.com]
Eyes
- Chemosis
After transcatheter intra-arterial embolisation, the ptosis and chemosis improved. [ncbi.nlm.nih.gov]
The patient demonstrated substantial clinical improvement with resolution of her chemosis and protruding eye. Citation: Yao CA, Tsou HK, Sheehan J and Pan HC. [austinpublishinggroup.com]
- Diplopia
Patients experiencing diplopia can be treated with prism or occlusion therapy and IOP-lowering medications can be started if IOP is substantially elevated. [eyewiki.org]
On following up 4 months after coiling, cranial nerve VI palsy and related symptoms-diplopia and limit of ocular movement-were improved significantly. [ncbi.nlm.nih.gov]
The patient first noticed binocular horizontal diplopia two months prior to her visit. She described diplopia in primary position that worsened in right gaze and she had resorted to wearing an occlusive patch in order to control her symptoms. [webeye.ophth.uiowa.edu]
- Pulsating Exophthalmos
The orbital or frontal bruit and pulsating exophthalmos are common only to the carotid-cavernous fistula. [ncbi.nlm.nih.gov]
- Unilateral Blindness
German, Triad of unilateral blindness, orbital fractures and massive epistaxis after head injury. J. Neurosurg. 18 (1961), 837–840. PubMed Google Scholar McCormick, W. F., and J. D. [link.springer.com]
Ears
- Tinnitus
After the procedure, tinnitus, bruit, and headache were reduced immediately. On following up 4 months after coiling, cranial nerve VI palsy and related symptoms-diplopia and limit of ocular movement-were improved significantly. [ncbi.nlm.nih.gov]
Furthermore, in 10 cases with posterior venous drainage via the inferior petrosal sinus, 8 cases complainted of tinnitus, but other 3 cases without showing posterior drainage, did not complain of tinnitus. [jstage.jst.go.jp]
Other common symptoms include pulsatile tinnitus, exophthalmos, and headache. Diagnosis is established based on typical findings on CT / MRI or angiography (e.g., enlarged cavernous sinus ). [amboss.com]
Cranial nerve palsies, epitaxis, headache, bruit, and tinnitus may be present with either anterior or posterior drainage. Rare cases with subarachnoid hemorrhage. [neurosurgery.pitt.edu]
- Pulsatile Tinnitus
Other common symptoms include pulsatile tinnitus, exophthalmos, and headache. Diagnosis is established based on typical findings on CT / MRI or angiography (e.g., enlarged cavernous sinus ). [amboss.com]
tinnitus") Refer non-urgently to ophthalmologist for confirmation of diagnosis CT or MRI often shows dilated superior ophthalmic vein, but... [kellogg.umich.edu]
Patients with a direct Type A fistula generally present with varied complaints, including unilateral visual loss, proptosis, lid swelling, pulsatile tinnitus and/or diplopia. [webeye.ophth.uiowa.edu]
Other conditions that predispose to increased risk include: Ehlers-Danlos syndrome fibromuscular dysplasia pulsatile exophthalmos : ~75% 3 chemosis and subconjunctival hemorrhage proptosis progressive visual loss: 25-32% 3 pulsatile tinnitus (usually [radiopaedia.org]
Notice also bilateral transverse/sigmoid sinus stenoses in this patient with pulsatile tinnitus and intracranial hypertension. [neuroangio.org]
Face, Head & Neck
- Facial Pain
Advanced dural fistulas, particularly those with significant orbital or cavernous sinus venous occlusion, may cause periorbital or retrobulbar discomfort and possibly facial pain. Rising episcleral venous pressure can increase intraocular pressure. [aao.org]
pain, usually unilateral and shock-like, located in the somatosensory distribution of the trigeminal nerve. [surgicalneurologyint.com]
Neurologic
- Headache
Here, we present the case of a young male patient with right-sided CCF, who presented with right-side headache and partial third nerve palsy with pupillary involvement. [ncbi.nlm.nih.gov]
- Paresis
A 58-year-old female presented with right conjunctival chemosis and right abducens nerve paresis. Cerebral angiography demonstrated a right carotid-cavernous sinus fistula associated with persistent primitive trigeminal artery. [ncbi.nlm.nih.gov]
[…] to patients with improved cranial nerve VI paresis (243 mm 3 ) and individuals with unchanged cranial nerve VI paresis (324 mm 3 ). [ajnr.org]
Posterior drainage usually produces no ocular symptoms, although some patients may experience a cranial neuropathy, such as facial paresis or ocular motor nerve paresis. [aao.org]
- Seizure
He was followed and at the first seizure (third month), a CT angiography was performed, which showed a partial thrombosis and an increase of the mass effects ( Figure 2 ). [scielo.br]
Combined surgical and transarterial approach A 66-year-old woman presented with intermittent paraparesis and generalized tonic-clonic seizure. [jkns.or.kr]
【全身性紅斑性狼瘡】*systemic lupus erythematosus (略 SLE) ^=全身性エリテマトーデス 【全身性進行性硬化症】*progressive systemic sclerosis 【全身性癲癇】*generalized epilepsy 【全身性皮膚硬化症】*systemic scleroderma (=全身性強皮症) 【全身性ヘモジデリン沈着症】【全身性ヘモジデリン症】*generalized hemosiderosis 【全身性発作】*generalized seizure [medo.jp]
- Vertigo
In 3 patients with vertigo an orthostatic origin was underlying. One patient reported with vertigo since the traumatic fistula. A total of 9 patients reported nonpulsating tinnitus, while 8 described a nonpermanent noise in the ear. [ajnr.org]
[…] tract with both openings on the skin. perilymph fistula rupture of the round window with leakage of perilymph into the inner ear, so that changes in middle ear pressure directly affect the inner ear, causing sensorineural deafness as well as dizziness, vertigo [medical-dictionary.thefreedictionary.com]
】【無気力】*asthenia 【無腕症】*abrachia (メ) 【メラニン尿】*melanuria =黒色尿 (明) 【明細胞汗腺腫】*clear cell hidradenoma 【明細胞癌】*clear cell carcinoma 【明細胞性棘細胞腫】*clear cell acanthoma (迷) 【迷走神経炎】*vagitis 【迷入膵】*aberrant pancreas 【迷入唾液腺】*aberrant salivary gland (眩) 【眩暈】*dizziness *vertigo [medo.jp]
- Dizziness
[…] semicircular tract with both openings on the skin. perilymph fistula rupture of the round window with leakage of perilymph into the inner ear, so that changes in middle ear pressure directly affect the inner ear, causing sensorineural deafness as well as dizziness [medical-dictionary.thefreedictionary.com]
(=尿閉) 【無力症】【無気力】*asthenia 【無腕症】*abrachia (メ) 【メラニン尿】*melanuria =黒色尿 (明) 【明細胞汗腺腫】*clear cell hidradenoma 【明細胞癌】*clear cell carcinoma 【明細胞性棘細胞腫】*clear cell acanthoma (迷) 【迷走神経炎】*vagitis 【迷入膵】*aberrant pancreas 【迷入唾液腺】*aberrant salivary gland (眩) 【眩暈】*dizziness [medo.jp]
Workup
Both direct and indirect carotid cavernous sinus fistulae can be diagnosed via a computerized tomography scan (CT), a magnetic resonance imaging scan (MRI) and an orbital echogram. These procedures can outline the existence of a congested cavernous sinus and superior ophthalmic veins [13].
A cerebral arteriography can help to establish a definitive diagnosis since it can illustrate the course of the vessels and reveal the existence of a pathological channel of communication with great precision.
Treatment
Patients diagnosed with carotid cavernous sinus fistulae receive symptomatic treatment, to relieve them of the ocular complications caused by the cavernous congestion and surgical treatment to repair the fistula. As far as supportive treatment is concerned, eye lubricants or a tarsorrhaphy may be necessary in order to treat exposure keratopathy. Patients presenting with glaucoma may require hyperosmotic medication or aqueous suppressants.
As far as surgical options are concerned, the aim is to achieve the closure of the fistula, either via embolization or via electrothrombosis or balloon occlusion [14] [15]. Some indirect fistula may resolve on their own; the rest require surgical intervention to prevent long-lasting and severe complications. Under some circumstances, fistulae may find an alternative route and reappear after surgery.
Prognosis
Prognosis depends on the type of fistula, the time that has passed from its formation until treatment and the symptoms a patient presents with.
Individuals that are diagnosed with dural, indirect fistulae generally present with milder symptomatology. Most of the ocular symptoms, such as eye pulsations, bruit, and thrill are resolved instantaneously following a surgery, whereas other symptoms like a conjunctival hematoma, disc swelling or glaucoma pay persist for up to half a year until they are resolved. Patients that are diagnosed with a high-pressure direct fistula may recover completely after surgical intervention, but some may be left with residual defects such as partial vision impairment or ophthalmoplegia.
Etiology
Most cases of carotid cavernous sinus fistulae occur as a result of traumas sustained to the head, following various types of accidents or falls. Fistulae exhibit an increased frequency amongst patients who have sustained a fracture of the basal or facial skull. On the other hand, postmenopausal women usually present with fistulae that arise of their own accord, without any prior traumatization. Risk factors for the development of a spontaneous carotid cavernous sinus fistula are:
- Hypertension
- Pregnancy
- Atherosclerotic lesions
- Connective tissue disorders
Under some still unclear circumstances, the low-pressure fistulae that develop between branches of the carotid arteries and the cavernous sinus can resolve without intervention, possibly due to increased clotting [5] [6].
Epidemiology
A carotid cavernous sinus fistula is an uncommon occurrence. The existence of high-pressure, direct fistulae can damage vision severely if it is left undiagnosed or untreated because it can lead to vision impairment or loss, as well as glaucoma. The fistula may also lead to intracerebral hemorrhage, hemorrhage in the subarachnoid space and increased mortality/ morbidity in some cases.
Low pressure, spontaneous fistulae are usually diagnosed amongst female patients over 50 years of age, whereas direct canals that are a result of trauma are observed amongst younger, more active individuals. In general, the condition seems to exhibit a predilection for women in their middle age.
Pathophysiology
Carotid cavernous sinus fistulae are abnormal connections between the internal or external carotid artery or its branches and the cavernous sinus, which belongs to the venous system [7]. As a result, high-pressure arterial blood is directed into the cavernous sinus, leading to a congested sinus and ocular symptoms, due to the direct connection of the cavernous sinus to the superior ophthalmic vein.
Generally, direct fistulae comprise up to 9 out of 10 fistulae that are diagnosed. The pathological connection is between the carotid artery itself and the cavernous sinus. A great amount of arterial blood is exchanged via this fistula. They are commonly induced by a severe traumatization that damaged the walls of the carotid artery. On the other hand, dural fistulae are those that are formed between the cavernous sinus and meningeal branches of the carotid artery. They do not develop a traumatic background but arise spontaneously.
In a nutshell, carotid cavernous sinus fistulae can be categorized broadly into two categories: direct fistulae, between the part of the internal carotid artery that lies within the cavernous sinus, and the indirect, that develop between branches of the internal or external carotid artery and the cavernous sinus. More specifically:
- Type A fistula represents a direct connection between the cavernous sinus and the segment of the internal carotid artery that is found within the sinus.
- Type B is an indirect, dural channel between branches of the internal carotid artery that lie within the sinus and the sinus itself.
- Type C is also an indirect shunt between meningeal branches of the external carotid artery and the cavernous sinus
- Type D describes type B + C.
Fistulae belonging to the C group are the most common [8].
Prevention
There are not specific suggestions that could prevent the development of such a fistula. After a traumatic injury to the head, all patients, regardless of age, should undergo extensive examinations and evaluation, especially if a fracture of the basal or facial skull is suspected. The only way to prevent permanent damage and severe complications is to diagnose the condition in time and treat it appropriately.
Summary
The cavernous sinus is a venous sinus located in the skull, specifically on the sides of the pituitary fossa. Under various circumstances, channels between the cavernous sinus and the external or internal carotid artery can form, which lead to the redirection of vascular blood flow. The augmented carotid arterial pressure leads to blood entering the cavernous sinus through these abnormal channels (fistulae).
The cavernous sinus is a venous structure and differs from its arterial counterparts, as far as the amount of blood it can receive is concerned and also with regard to the pressure it can maintain. The entrance of high-pressure blood from the arteries leads to a blockage of the flow or a reversed flow within the walls of the cavernous sinus. Given the fact that the structure is connected to the superior ophthalmic veins, and either drains to or from them, this change of flow balance leads to an excessive amount of blood being redirected into the ophthalmic veins; as a result, ocular symptoms emerge, such as diplopia, orbital pain, blindness, ophthalmoplegia and other symptoms.
A carotid cavernous sinus fistula (CCF) can either arise after a specific incident, such as a head traumatization which leads to damage to the internal carotid artery or in a spontaneous way [1]. Spontaneous fistulae do not usually form between the carotid artery itself and the cavernous sinus, but rather between the sinus and smaller arterial branches. These fistulae contain blood that flows with lower pressures, in comparison to fistulae between the principal artery and the sinus, which are high-pressure canals. Tortuous conjunctival vessels along with retinal hemorrhages can also present with CCF [2].
It seems that the majority of the CCF cases that lead to the appearance of ocular symptoms are reported from industrialized countries of the West [3] [4].
Patient Information
A fistula is an abnormal channel that allows for the communication between two structures that normally do not communicate. A carotid cavernous sinus fistula is such a channel, that is formed between the carotid artery, one of the major arteries of the body, and the cavernous sinus, a venous sinus that lies within the head. It may be a result of trauma to the head, or it may develop spontaneously, particularly in middle-aged women.
The fistula allows high-pressure blood from the artery to enter the cavernous sinus and thus, the latter is congested. This congestion leads to eye-related symptoms because the cavernous sinus is directly connected to the superior ophthalmic veins. Patients with such fistulae often present with a red, bulging and pulsating eye, report buzzing sounds and have trouble to move their eyes in all normal directions. They may also complain that their vision has deteriorated and the ophthalmologist may diagnose increased pressure within the eye.
Treatment comprises measures to alleviate the symptoms and surgical treatment in order to close the fistula and stop the abnormal communications between the carotid artery and the cavernous sinus.
References
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- 2Bhatti MT, Peters KR. A red eye and then a really red eye. Surv Ophthalmol. 2003;48:224–9.
- Goldberg RA, Goldley SH, Duckwiler G, Vinuela F. Management of cavernous sinus-dural fistulas. Arch Ophthalmol. 1996;114:707–14.
- Rucker JC, Biousse V, Newman NJ. Magnetic resonance angiography source images in carotid cavernous fistulas. Br J Ophthalmol. 2004;88:311.
- Newton TH , Hoyt WF. Dural arteriovenous shunts in the region of the cavernous sinus . Neuroradiology 1970; 1:71-81.
- Brismar G, Brismar J . Spontaneous carotid-cavernous fistulas . Phlebographic appearance and relation to thrombosis. Acta Radio/ [Diagn] (Stockh) 1976; 17: 180-192.
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- Halbach VV, Hieshima GB, Higashida RT et-al. Carotid cavernous fistulae: indications for urgent treatment. AJR Am J Roentgenol. 1987;149 (3): 587-93.
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- Barke RM, Yoshizumi MO, Hepler RS, Krauss HR, Jabour BA. Spontaneous dural carotid-cavernous fistula with central retinal vein occlusion and iris neovascularization. Ann Ophthalmol. 1991;23:11–7.
- Choi HY, Newman NJ, Biousse V, Hill DC, Costarides AP. Serous retinal detachment following carotid-cavernous fistula. Br J Ophthalmol. 2006;90:1440.
- Oestreicher JH, Frueh BR. Carotid-cavernous fistula mimicking Graves' eye disease. Opthal Plast Reconstr Surg. 1995;11:2.
- 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). 2008 Aug 20. 121(16):1558-62.
- Wang C, Xie X, You C, et al. Placement of Covered Stents for the Treatment of Direct Carotid Cavernous Fistulas. AJNR Am J Neuroradiol. 2009 Aug;30(7):1342-6.
- Bing F, Albrieux M, Vinh Moreau-Gaudry V, Vasdev A. Cavernous sinus fistula treated through the transvenous approach: Report of four cases. J Neuroradiol. 2009 Dec;36(5):265-9.