Bilateral carotid-cavernous sinus fistula: Case reports and review of the literature

Sachin Chemate; Joy Vargese; Pritam Chatterjee; G Sudhakshina Nathan; M Balamurugan

doi:10.4103/jcvs.jcvs_11_21

CASE REPORT

Year : 2021 | Volume : 9 | Issue : 1 | Page : 32-37

Bilateral carotid-cavernous sinus fistula: Case reports and review of the literature

Sachin Chemate, Joy Vargese, Pritam Chatterjee, G Sudhakshina Nathan, M Balamurugan
Department of Neurosurgery, Apollo Hospitals, Chennai, Tamil Nadu, India

Date of Submission	25-May-2021
Date of Decision	14-Jun-2021
Date of Acceptance	18-Jul-2021
Date of Web Publication	27-Aug-2021

Correspondence Address:
Dr. Sachin Chemate
Department of Neurosurgery, Apollo Hospitals, Chennai, Tamil Nadu
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcvs.jcvs_11_21

Abstract

Carotid-cavernous fistula (CCF) is an abnormal vascular connection between the carotid artery and the cavernous sinus. There are various classifications based on haemodynamic, aetiology or anatomically. Haemodynamic classification is based on whether the fistula is high or low flow. Etiologically, it can be secondary to trauma or can develop spontaneously due to pre-existing aneurysm or medical conditions predisposing to arterial wall defects. Bilateral CCFs are very rare. We present two cases of bilateral CCF – one secondary to trauma and other occurred spontaneously. Both the patients presented with the signs of raised intraocular pressure – decreased vision, chemosis, proptosis and ophthalmoplegia. Magnetic resonance imaging and digital subtraction angiography confirmed a bilateral CCF. Both the patients underwent two settings of endovascular embolisation procedures, and complete embolisation of bilateral CCF was achieved. Available literature is unclear about the aetiology of bilateral CCF, technique of endovascular embolisation and the prognosis of bilateral CCF. In our both the cases, we were able to achieve complete embolisation in two settings.

Keywords: Bilateral treatment, carotid-cavernous fistula, embolisation

How to cite this article:
Chemate S, Vargese J, Chatterjee P, Nathan G S, Balamurugan M. Bilateral carotid-cavernous sinus fistula: Case reports and review of the literature. J Cerebrovasc Sci 2021;9:32-7

How to cite this URL:
Chemate S, Vargese J, Chatterjee P, Nathan G S, Balamurugan M. Bilateral carotid-cavernous sinus fistula: Case reports and review of the literature. J Cerebrovasc Sci [serial online] 2021 [cited 2023 Feb 4];9:32-7. Available from: http://www.jcvs.in/text.asp?2021/9/1/32/324807

Introduction

Trauma is the most common cause of carotid-cavernous fistula (CCF) and accounts for up to 75% of cases. Most common signs of CCF depend on type of haemodynamics – whether it is high or low flow. High-flow CCF presents with features of increased intraocular pressure – chemosis, proptosis, cranial nerve palsy, diplopia and decreased vision. Bilateral CCF can be post-traumatic or spontaneous. Most of bilateral CCF is high flow. Digital subtraction angiography (DSA) is the gold standard diagnostic modality. Endovascular embolisation is the first-line treatment. Embolisation can be achieved with either a metallic coil, endovascular balloon or embolic agent. Endovascular embolisation is a technical challenge.

Case 1

Case history

A 51-year-old female, a known case of diabetes mellitus Type II and systemic hypertension, was admitted from outpatient department with a history of headache, redness, burning and swelling of both eyes with inability to move eyeballs gradually progressing for 2–3 days [Figure 1]. She had a history of road traffic accident with head injury 2 months ago following which there was transient loss of consciousness for 5 min. Computed tomography (CT) brain post-head injury was unremarkable.

Figure 1: Clinical image of patient showing chemosis, proptosis and restrictions of extraocular movements (a) left lateral gaze, (b) right lateral gaze

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Bedside examination

On bedside examination, her visual acuity was counting fingers at 5 feet, colour vision was impaired and visual acuity by Snellen charts was 6/30 in both eyes. Pupils were 1 mm bilaterally reacting to light and accommodation. There was afferent pupillary defect appreciated in the right eye. Extraocular movements were severely restricted in all gaze directions in both the eyes. Bilateral orbital bruit was present.

Ophthalmological examination

Intraocular pressures measured 21 mmHg in the right eye and 25 mmHg in the left eye with Tonopen tonometry. Exophthalmometry showed 4 mm of proptosis on both side. Anterior segment examination showed tortuous and engorged vessels in both eyes with segmental subconjunctival haemorrhage. Posterior segment examination showed a cup-to-disc ratio of 0.2 bilaterally, normal vasculature. Retina was normal.

Initial neuroimaging with CT brain was unremarkable. DSA was done which showed bilateral CCF with a direct high flow communication between internal carotid artery (ICA) and cavernous sinus (Barrow Type A) with reversal of blood flow in bilateral superior ophthalmic vein [Figure 2] which explains the ocular signs due to venous hypertension.

Figure 2: Digital subtraction angiography: Left and right internal carotid artery injections showing bilateral carotid-cavernous fistula (Barrow Type A) with reversal of blood flow in bilateral superior ophthalmic vein

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It was decided to occlude the fistula with endovascular coil embolisation in two settings due to various factors – age of patient, complex high flow haemodynamic fistula and multiple pre-existing comorbidities of the patient. Initially, she underwent transarterial approach through the left ICA and coil embolisation of left CCF was performed and after 1-week right CCF, coil embolisation was done [Figure 3]. Both the procedures were uneventful. Her visual acuity returned to 6/6 in both the eyes using Snellen visual acuity chart. Intraocular pressure returned to a normal range. Her afferent pupillary defect resolved. Extraocular movements improved. Three-month follow-up DSA showed complete obliteration of bilateral CCF and patient had complete resolutions of ocular signs – proptosis, chemosis, extraocular movements [Figure 4].

Figure 3: Coil embolisation and complete obliteration of bilateral carotid-cavernous fistula

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Figure 4: Three-month post-coil embolisation image of patient showing complete resolution of ocular signs (patient had congenital squint)

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Case 2

Case history

A 51-year-old male from Oman with systemic hypertension was referred with complaints of redness, watering and burning of both eyes for 1 year [Figure 5] which was insidious in onset and gradually progressive followed by diplopia and ocular bruit for 9 months. He was evaluated at local hospital with CT brain with contrast and DSA, which showed bilateral CCF. There was no history of trauma.

Figure 5: Clinical image of patient showing chemosis (a), proptosis (b) and right lateral rectus palsy (c)

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Bedside examination

On bedside examination, his visual acuity was counting fingers at 6 feet, colour vision was impaired and visual acuity by Snellen charts was 6/15 in both eyes. Pupils were 2 mm bilaterally reacting to light and accommodation. There was afferent pupillary defect appreciated in the right eye. Extraocular movements showed right lateral gaze palsy [Figure 5]c. Bilateral orbital bruit was audible. There was mild proptosis of the left eye.

Ophthalmological examination

Intraocular pressures measured 22 mmHg in the right eye and 24 mmHg in the left eye with Tonopen tonometry. Exophthalmometry showed 2 mm of proptosis on the left side. Anterior segment examination showed tortuous and engorged vessels in both eyes with segmental subconjunctival haemorrhage. Posterior segment examination showed a cup-to-disc ratio of 0.2 bilaterally, normal vasculature. Retina was normal.

Initial neuroimaging with CT brain with contrast showed bilateral CCF with prominent superior ophthalmic vein bilaterally [Figure 6]a and [Figure 6]b. DSA was done which showed bilateral CCF which was indirect communication between ICA, external carotid artery (ECA) and branches of ECA and cavernous sinus causing a low flow shunting (Barrow Type D) with mild reversal of blood flow in bilateral superior ophthalmic vein [Figure 2] which explains the gradually progressive less dramatic ocular signs due to venous hypertension.

Figure 6: Computed tomography brain with and without contrast showing bilateral carotid-cavernous sinus fistula with engorged superior ophthalmic veins bilaterally

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It was decided to occlude the fistula with endovascular coil embolisation in two settings due to various factors – complex nature of fistula (multiple feeders), age of patient, low flow haemodynamic of fistula. In the first setting, he underwent transarterial approach through right ICA and coil embolisation was performed [Figure 7]. In the second setting, he underwent combined transarterial and transvenous approach through right ECA (and its branch – internal maxillary artery) and left inferior petrosal vein and coil embolisation was done. Both the procedures were uneventful. His visual acuity returned to 6/6 in both the eyes using Snellen visual acuity chart. Intraocular pressure returned to a normal range. His afferent pupillary defect resolved. Extraocular movements improved. Three-month follow-up DSA showed complete obliteration of bilateral CCF and patient had complete resolutions of ocular signs – chemosis, extraocular movements [Figure 8].

Figure 7: Coil embolisation and complete obliteration of bilateral carotid-cavernous fistula

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Figure 8: Three-month post-coil embolisation of carotid-cavernous fistula showing improvement in conjunctival redness, watering and extraocular movements

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Discussion

CCF is an abnormal vascular connection between the carotid artery and the cavernous sinus. There are various classifications based on haemodynamic, aetiology or anatomically. Haemodynamic classification is based on whether the fistula is high or low flow. Etiologically, it can be secondary to trauma or can develop spontaneously due to pre-existing aneurysm or medical conditions predisposing to arterial wall defects. Anatomic classification is based on whether the fistula is arising from the carotid artery (direct) or arising from one of the branches of the carotid artery (indirect). Barrow classifies CCF into 4 types based anatomical variations of shunt between ICA, ECA their branches and cavernous sinus^[1],[2] [Table 1].

Table 1: Barrow's classification of carotid-cavernous fistula

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In 75%–80% cases, CCF is traumatic in origin, which commonly occurs in young males.^[1],[2] It occurs more common with basilar skull fracture.^[3] Spontaneous CCFs classically occur in older age due to ruptured ICA aneurysms or secondary to Ehlers-Danlos, fibromuscular dysplasia and pseudoxanthoma elasticum.^[1]

Most common signs of CCF are dependent on whether the CCF is direct or indirect. Direct will commonly present rapidly with signs of increased intraocular pressure which occurs due to venous hypertension. Common presentations are chemosis, proptosis, increased intraocular pressure, cranial nerve palsy, diplopia and impaired vision, orbital bruits, headache and orbital.^[1],[5] Indirect CCF are relatively low flow fistula; hence, it presents less dramatically with conjunctival injections.

Gold standard diagnostic modality is cerebral angiography. CT contrast or magnetic resonance angiography can also demonstrate the presence of a CCF.^{[1],[2],[3],[4],[5]}

Possible treatment options of CCF are endovascular embolisation, radiosurgery and microsurgery. Earlier case reports show that surgery was mainline treatment but due to advancement in endovascular intervention, first-line treatment at present is endovascular embolisation which can be achieved with metallic coil, endovascular balloon or embolic agent. Complications of endovascular embolisation are cerebral infarction, retroperitoneal haematoma, decreased visual acuity and ophthalmoplegia.^{[1],[2],[3],[4],[5]} Surgical intervention is still a reserved option for cases where endovascular treatment has been unsuccessful or is not possible. Success rates vary between 30% and 80% in the literature.^[1],[2] Radiosurgery is effective in patients with indirect, low flow, CCFs.^[6]

Bilateral CCFs are rare. Review of literature was done and we found a total of 70 cases of bilateral CCF, 44 of which were post-traumatic.^{[1],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21],[22],[23],[24],[25],[26],[27],[28],[29],[30],[31],[32],[33],[34],[35],[36],[37],[38],[39],[40]} Type A was most common [[Table 2] summarises all cases of post-traumatic bilateral CCFs treated with endovascular coiling].

Table 2: Summary of all cases of bilateral carotid-cavernous fistula with coil embolisation

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Bilateral CCF is very rare. They can be traumatic or spontaneous in origin. We present two cases of CCF – one was post-traumatic and other was spontaneous. Direct CCF is high flow in nature which causes rapidly progressive dramatic presentation with signs of raised intraocular pressure due to high venous hypertension – proptosis, severe conjunctival haemorrhage, ophthalmoplegia and deduced vision. The first case had a direct high flow fistula and patient had pre-existing multiple comorbidities; hence, we chose to do endovascular coil embolisation in two setting. Indirect CCF is relatively low flow fistula; hence, it presents less dramatically with conjunctival injections making diagnosis of CCF more confusing. The second case had a low flow fistula but due to complex nature of fistula due to multiple feeders, we performed coil embolisation in two setting. Both the patients had improvement in the ocular signs and follow-up imaging with DSA showed complete obliteration of bilateral CCF.

Conclusion

Bilateral CCF is very rare. Most of them are traumatic in origin. Spontaneous CCF is extremely rare and occurs in the elderly. Clinical features of the patient depend on the haemodynamic and type of fistula. Endovascular coil embolisation is the first line of treatment. It is safe to perform two setting of coil embolisation in case of high flow CCF or complex CCF.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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