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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 1  |  Issue : 1  |  Page : 11

A continuous red eye: Case report about a carotid cavernous fistula


Department of Ophthalmology, Hospital Garcia de Orta, Almada, Portugal

Date of Submission26-Jun-2019
Date of Acceptance27-Jun-2019
Date of Web Publication16-Oct-2019

Correspondence Address:
Dr. Sonia Cristina Raposo Parreira
Av. Torrado da Silva, 2805-267 Almada
Portugal
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/PAJO.PAJO_7_19

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  Abstract 


The purpose of the study is to present a case of indirect carotid cavernous fistula (CCF) successfully treated with endovascular approach. A 46-year-old male presented with a progressive, painless visual loss and conjunctival injection of the right eye with 1 month of evolution. On ophthalmology examination, the patient had best-corrected visual acuity of 20/30 in the right eye and an intraocular pressure (IOP) of 20 mmHg. The patient still had pulsating proptosis and an orbital bruit on the auscultation. Slit-lamp examination revealed corkscrew episcleral vessels. The patient was submitted a magnetic resonance angiography that confirmed the presence of an indirect carotid-cavernous sinus fistula. The patient was submitted to endovascular embolization by the transarterial approach to close the fistula with complete clinical and imaging resolution. Two months after the procedure, the visual acuity was 20/20, and the IOP was 8 mmHg. CCF is an uncommon disease and may be misdiagnosed when there are few signs and symptoms on presentation. Different subtypes of CCF cause different clinical signs, with varying speeds of onset and severity. The diagnosis is based on clinical findings and by imaging studies. However, the definite diagnosis is only possible with cerebral angiography. It is important to consider risks and benefits in relation to endovascular treatment, mainly in indirect CCF.

Keywords: Carotid cavernous fistula, endovascular treatment, red eye


How to cite this article:
Parreira SC, Barros S, Lopes D, Silva J, Campos N. A continuous red eye: Case report about a carotid cavernous fistula. Pan Am J Ophthalmol 2019;1:11

How to cite this URL:
Parreira SC, Barros S, Lopes D, Silva J, Campos N. A continuous red eye: Case report about a carotid cavernous fistula. Pan Am J Ophthalmol [serial online] 2019 [cited 2019 Dec 9];1:11. Available from: http://www.thepajo.org/text.asp?2019/1/1/11/269299




  Introduction Top


Carotid cavernous fistula (CCF) is an abnormal communication between the cavernous sinus (CS) and the carotid arterial system. Anatomically, these fistulas can be divided into two types: direct and indirect or dural CCF. Direct CCF corresponding to 70%–80% of all cases and they are characterized by a direct connection between the intracavernous segment of the internal carotid artery (ICA) and the CS. These fistulae usually have high rates of arterial blood flow and most commonly are caused by a single traumatic tear in the arterial wall. Dural CCF is characterized by a communication between the CS and one or more meningeal branches of the ICA, external carotid artery (ECA), or both. These fistulae usually have low rates of arterial blood flow and almost always produce symptoms and signs spontaneously, without any antecedent trauma or manipulation. The lesions may represent congenital arteriovenous malformations, which develop spontaneously or in association with atherosclerosis, systemic hypertension, collagen vascular disease, pregnancy, and during or after childbirth.[1],[2] The diagnosis is based on patient's signs and symptoms and imaging studies such as computed tomography scan (CT scan) or magnetic resonance imaging (MRI) of the orbits where the most specific sign is the dilation of the superior ophthalmic vein (SOV). However, definite diagnosis, fistula characterization, and planning of endovascular treatment are only possible with cerebral angiography.[2],[3] Most CCFs are not life-threatening, but the involved eye is at risk. Spontaneous closure from thrombosis of CS is unlikely (especially those that occur after trauma or in high-flow fistulas). Dural fistulas may undergo spontaneous closure, especially after diagnostic angiography. Main indications for treatment include glaucoma, diplopia, intolerable bruit or headache, and severe proptosis causing exposure keratopathy.[2],[4]


  Case Report Top


A 46-year-old male with a past medical history of chronic liver disease with portal hypertension, presented with a progressive, painless visual loss and conjunctival injection of the right eye with 1 month of evolution. He was treated 1 month ago with antibiotic drops for conjunctivitis without improvements of symptoms. On ophthalmology examination, the patient had best-corrected visual acuity of 20/30 in the right eye and 20/20 in the left eye and an intraocular pressure (IOP) of 20 mmHg and 10 mmHg, respectively. The patient still had pulsating proptosis and an orbital bruit on the auscultation. There was no restriction on eye movements or diplopia. Slit-lamp examination of the right eye revealed a superior swollen eyelid without pain, inferior chemosis, and corkscrew episcleral vessels [Figure 1]. Fundoscopy showed slight venous tortuosity. Slit-lamp examination and fundoscopy of the left eye was unremarkable. For clinical suspicion of CCF, the patient was submitted a magnetic resonance angiography that confirmed the presence of an indirect carotid-CS fistula supplied by the right accessory meningeal artery and draining to the SOV. This examination showed the asymmetry between the CSs [Figure 2], the orbital muscles, and the proptosis of the right eye [Figure 3]. The examination demonstrates dilation of the right SOV [Figure 4]. Antihypertensive eye drops were initiated in the right eye, and due to the symptoms presentation, we decided to refer the patient for diagnostic and therapeutic cerebral angiography. The patient was submitted to endovascular embolization [Figure 5] and [Figure 6] by the transarterial approach to close the fistula with complete clinical and imaging resolution. Two months after the procedure, the visual acuity was 20/20, the IOP was 8 mmHg, and the slit-lamp examination was unremarkable [Figure 7].
Figure 1: Right red eye demonstrate corkscrew episcleral vessels

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Figure 2: Magnetic resonance imaging: Asymmetry between cavernous sinuses

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Figure 3: Magnetic resonance imaging: Proptosis and enlargement of the extrinsic muscles

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Figure 4: Magnetic resonance imaging: Suoerior Ophthalmic Vein (SOV) dilated

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Figure 5: Cerebral angiography: VOS before embolization with cyanoacrylate

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Figure 6: Cerebral angiography: VOS after embolization with cyanoacrylate

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Figure 7: Right eye 2 months after embolization

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  Discussion Top


CCF is an uncommon disease and may be misdiagnosed when there are few signs and symptoms on presentation. Several classifications of CCF exist depending on anatomy, etiology, and pathophysiology. The most used classification has been proposed by Barrow et al.[5] and is based on the arterial supply of these communications. Type A CCF results from direct communication between ICA and CS, Type B results from a communication between dural ICA branches and CS, Type C results from communication between dural ECA branch and CS, and Type D results from a communication from dural branches of ICA and ECA branches to CS. CCF Type A is the only type with direct communication between the ICA and the CS; therefore, high flow is usually present. A compilation of different series shows that brain trauma is responsible for up to 80% of cases, the others occurring spontaneously such as aneurysm rupture or weakness of the vascular wall in cases of collagen deficiency. While acute forms occur more often in young men, spontaneous forms tend to occur in middle-aged women.[5],[6],[7]

Different subtypes of CCF cause different clinical signs, with varying speeds of onset and severity, which is usually higher in direct forms. Pathogenesis involves CS dilation and retrograde drainage (cortex and orbit), with increased vascular volume and enlarged orbital muscles, increased capillary transudation, elevated episcleral venous pressure, and impairment of the optic nerve and retina due to stasis. In direct CCF, these events lead to a more acute presentation with more abundant signs and symptoms.[8] Indirect forms, however, have a milder or minimal presentation. This is possibly due to the fact that in many cases, drainage of dural shunts often occurs to the basal and petrosal sinus, and only in the presence of thrombosis, there is flow back into the orbit.[2],[5]

Clinical signs range from mild paresis and dysfunctions (nerves III, V, and VI) without orbital congestion to pulsatile proptosis (milder in indirect forms). Characteristic symptoms include pulsatile exophthalmos, murmur, and venular dilation with chemosis (Dandy's triad).[9],[10]

Diagnostic suspicion is stronger in cases with a variety of characteristic signs. However, the gold standard for any type of CCF is cerebral angiography. It identifies the type, location, and size of the connection, examines the vascular surroundings and coexisting alterations, especially ischemic effects on the cortex. Initial evaluation is done with CT scan or MRI. However, normal results in these tests do not exclude the diagnosis. Images can assess proptosis, CS and SOV dilation, and enlargement of the extrinsic muscles.[11]

Emergency endovascular treatment of CCF is indicated in the presence of pseudoaneurysm, large CS varix, venous drainage toward the cortex, thrombosis extending beyond the fistula, increased intracranial pressure, altered mental status, proptosis and/or progressive loss visual acuity, brain hemorrhage, and ischemic stroke.[2],[4],[12] In other situations, the severity of the condition versus the possibility of spontaneous resolution should be taken into account, especially in dural forms.[2] The endovascular approach is the current procedure of choice, either through an arterial or venous access. Different natural or synthetic occlusive materials can be used. Proptosis, chemosis, and keratopathy should be managed according to severity. Lubricants and topical antibiotics could be prescribed, when necessary. For glaucoma, drugs that reduce the production of aqueous humor are indicated (beta-blockers, alpha-adrenergic agonists, and carbonic anhydrase inhibitors), while drugs promoting drainage are ineffective (pilocarpine and prostaglandin analogs).[11]

This case illustrates an indirect CCF that was treated with the endovascular approach. We decided to treat because the patient presented with proptosis and progressive loss visual acuity. This reinforces the importance of considering the literature and the signs and symptoms of our patients in order to obtain the best results.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
de Keizer R. Carotid-cavernous and orbital arteriovenous fistulas: Ocular features, diagnostic and hemodynamic considerations in relation to visual impairment and morbidity. Orbit 2003;22:121-42.  Back to cited text no. 1
    
2.
Miller NR. Dural carotid-cavernous fistulas: Epidemiology, clinical presentation, and management. Neurosurg Clin N Am 2012;23:179-92.  Back to cited text no. 2
    
3.
Théaudin M, Saint-Maurice JP, Chapot R, Vahedi K, Mazighi M, Vignal C, et al. Diagnosis and treatment of dural carotid-cavernous fistulas: A consecutive series of 27 patients. J Neurol Neurosurg Psychiatry 2007;78:174-9.  Back to cited text no. 3
    
4.
Bhatti MT, Peters KR. A red eye and then a really red eye. Surv Ophthalmol 2003;48:224-9.  Back to cited text no. 4
    
5.
Barrow DL, Spector RH, Braun IF, Landman JA, Tindall SC, Tindall GT. Classification and treatment of spontaneous carotid-cavernous sinus fistulas. J Neurosurg 1985;62:248-56.  Back to cited text no. 5
    
6.
Levin LA. Neuro-ophthalmologic diagnosis and therapy of central nervous system trauma. Ophthalmol Clin North Am 2004;17:455-64, vii.  Back to cited text no. 6
    
7.
Naesens R, Mestdagh C, Breemersch M, Defreyne L. Direct carotid-cavernous fistula: A case report and review of the literature. Bull Soc Belge Ophtalmol. 2006;(299):43-5.  Back to cited text no. 7
    
8.
Barry RC, Wilkinson M, Ahmed RM, Lim CS, Parker GD, McCluskey PJ, et al. Interventional treatment of carotid cavernous fistula. J Clin Neurosci 2011;18:1072-9.  Back to cited text no. 8
    
9.
Ringer AJ, Salud L, Tomsick TA. Carotid cavernous fistulas: Anatomy, classification, and treatment. Neurosurg Clin N Am 2005;16:279-95, viii.  Back to cited text no. 9
    
10.
Biousse V, Mendicino ME, Simon DJ, Newman NJ. The ophthalmology of intracranial vascular abnormalities. Am J Ophthalmol 1998;125:527-44.  Back to cited text no. 10
    
11.
Vilela MP. Carotid-cavernous fistula. Rev Bras Oftalmol 2013;72:70-5.  Back to cited text no. 11
    
12.
Gemmete JJ, Ansari SA, Gandhi D. Endovascular treatment of carotid cavernous fistulas. Neuroimaging Clin N Am 2009;19:241-55.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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