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Table of Contents    
Year : 2015  |  Volume : 63  |  Issue : 1  |  Page : 116-118

Central retinal artery occlusion with ophthalmoparesis in spontaneous carotid artery dissection

1 Department of Neurology, MS Ramaiah Medical College and Hospital, Bangalore, Karnataka, India
2 Department of Ophthalmology, MS Ramaiah Medical College and Hospital, Bangalore, Karnataka, India

Date of Web Publication4-Mar-2015

Correspondence Address:
Rohan Mahale
Department of Neurology, MS Ramaiah Medical College and Hospital, Bangalore, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.152685

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How to cite this article:
Mahale R, Mehta A, Sharma S, Javali M, Malavika K, Srinivasa R. Central retinal artery occlusion with ophthalmoparesis in spontaneous carotid artery dissection. Neurol India 2015;63:116-8

How to cite this URL:
Mahale R, Mehta A, Sharma S, Javali M, Malavika K, Srinivasa R. Central retinal artery occlusion with ophthalmoparesis in spontaneous carotid artery dissection. Neurol India [serial online] 2015 [cited 2021 Jul 25];63:116-8. Available from:


Spontaneous carotid arterial dissection is a nontraumatic tear or disruption in the wall of the internal carotid artery (ICA) without a clear etiology. It represents a major cause of stroke in younger patients, comprising about 10-25% of ischemic cerebral events. Patients can present with a range of symptoms from being completely asymptomatic to having facial/neck pain, headaches, Horner's syndrome, amaurosis fugax, or transient ischemic attacks (TIAs) and stroke. Isolated central retinal artery occlusion (CRAO) as a presenting manifestation of spontaneous carotid artery dissection is rare. Here, we report a middle-aged male patient who presented with acute spontaneous right carotid artery dissection with monocular visual loss of the right eye due to CRAO.

A 45-year-old male patient presented with a history of acute loss of vision in the right eye in a single day. There was preceding history of severe, throbbing pain on the right side of the neck radiating to the right hemicranial region associated with two episodes of vomiting. A day prior to the onset of acute visual loss, he had similar symptoms lasting for a few seconds that improved with eye massage. There was no history of head or neck trauma. He was conscious and had severe pain with tenderness in the right lateral aspect of the neck. There was no perception of light and a relative afferent pupillary defect (RAPD) in the right eye. The right pupil was dilated with an absent direct and consensual light reflex in the right eye. The fundus examination revealed opacification of the right retina, attenuated retinal arteries, 'boxcar' appearance of retinal arteries, and disc edema [Figure 1]. There was mild ptosis, chemosis, and restriction of right eye abduction and elevation. Magnetic resonance angiography (MRA) of neck vessels and circle of Willis showed smooth tapering of the right ICA (flame sign) soon after the bifurcation of common carotid artery with a thin stream of blood flow distally (string sign). There was filling of the right middle cerebral artery (MCA) from the left anterior cerebral artery (ACA) and ICA via the anterior communicating artery [Figure 2] and [Figure 3]. Fluid-attenuated inversion recovery (FLAIR) image showed hyperintensity in right ACA/MCA border zone, with restriction on diffusion-weighted imaging (DWI) [Figure 4]. The complete hemogram, renal, hepatic, and thyroid function tests were normal. The lipid profile and serum homocysteine levels were normal. The patient initially received heparin as an anticoagulant therapy and was subsequently shifted to warfarin therapy. Intravenous dexamethasone (4 mg thrice a day) was also given for 3 days along with acetazolamide to reduce intraocular pressure. The pain subsided, but the sudden visual loss showed no improvement.
Figure 1: Fundus examination of the right eye (a and b) shows opacification of retina, boxcar appearance of retinal arteries, and disc edema. (c) Fundus examination of the left eye shows normal disc, retinal arteries, and glow

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Figure 2: Magnetic resonance angiography (MRA) of neck vessels and circle of Willis (a and b) showing 'string' sign (red arrow) in distal common carotid artery (CCA) and internal carotid artery (ICA), 'flame' sign in ICA (white arrow); (c) nonvisualization of ICA and filling of middle cerebral artery (MCA) from anterior cerebral artery (ACA) (red arrow)

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Figure 3: MRA of neck vessels (a) normal both CCA; (b) decrease in right ICA caliber (red arrow); (c and d) thin stream of flow eccentrically: 'Crescent' sign in right ICA (red arrow); and (e and f) nonvisualization of right ICA

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Figure 4: Magnetic resonance imaging (MRI) brain (a and b) DWI shows right frontal (ACA/MCA border-zone) hyperintensity suggesting restriction (red arrow); (c and d) apparent diffusion coefficient (ADC) shows corresponding hypointensity suggesting an acute infarct

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Spontaneous dissections of the ICA have been well documented in the literature. Cervical ICA dissections commonly occur 2-3 cm distal to the carotid bulb. The dissection leads to blood accumulation within the layers of the artery resulting in an intramural hematoma that can spread along the vessel both proximally and distally. This intramural hematoma can cause stenosis or occlusion of the vessel lumen. The endothelial disruption predisposes to local thrombus formation that may occlude the artery and result in distal embolization that may precipitate brain infarction or ocular ischemia. Carotid dissections typically cause ipsilateral frontotemporal headaches with neck pain in 26% of ICA dissections. Ocular strokes due to carotid artery dissection are rare. Newman et al., reported two patients with permanent ocular vasoocclusion consequent to dissection of the ipsilateral ICA. [1] Galetta et al., reported a patient with bilateral traumatic carotid dissections who had acute monocular visual loss due to ophthalmic artery occlusion along with proptosis, ophthalmoparesis, and chemosis. [2]

There are two previously reported cases of CRAO as an initial manifestation of carotid dissection. Rao et al., described a patient with traumatic ICA dissection who developed an ipsilateral isolated CRAO. [3] Similarly, Lubin et al., described a patient with spontaneous common carotid artery dissection manifesting as CRAO. [4] Central retinal artery is a branch of the ophthalmic artery and is an end-artery. The probable mechanism of CRAO in our patient was distal embolization from the thrombus at the site of dissection. Ophthalmoparesis and chemosis may have been due to the involvement of the intracavernous portion of the ICA causing oculomotor, and abducens nerve paresis consequent to reactive inflammation

Ocular strokes are a rare but serious complication of carotid artery dissection. Spontaneous carotid artery dissection should be included in the differential diagnosis of CRAO.

  References Top

Newman NJ, Kline LB, Leifer D, Lessell S. Ocular stroke and carotid artery dissection. Neurology 1989;39:1462-4.  Back to cited text no. 1
Galetta SL, Leahey A, Nichols CW, Raps EC. Orbital ischemia, ophthalmoparesis, and carotid dissection. J Clin Neuro Ophthalmol 1991;11:284-7.  Back to cited text no. 2
Rao TH, Schneider LB, Patel M, Libman RB. Central retinal artery occlusion from carotid dissection diagnosed by cervical computed tomography. Stroke 1994;25:1271-2.  Back to cited text no. 3
Lubin J, Capparella J, Vecchione M. Acute monocular blindness associated with spontaneous common carotid artery dissection. Ann Emerg Med 2001;38:332-5.  Back to cited text no. 4


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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