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 Table of Contents  
Year : 2023  |  Volume : 3  |  Issue : 2  |  Page : 458-462

Optic disk pallor—Catch the masqueraders. Role of carotid angiography: A case report and review

1 Institute of Ophthalmology, Jawaharlal Nehru Medical College and Hospital, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
2 Department of Radiology, All India Institute of Medical Sciences, Delhi, India

Date of Submission10-Nov-2022
Date of Acceptance19-Jan-2023
Date of Web Publication28-Apr-2023

Correspondence Address:
Naheed Akhtar
Assistant Professor, Paediatric Ophthalmologist and Strabismologist, Institute of Ophthalmology, Jawaharlal Nehru Medical College and Hospital, Aligarh Muslim University, Aligarh, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJO.IJO_2979_22

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NA-AION is the most common ischemic optic neuropathy, with an incidence rate of 2.5–11.8 per 1,00,000 in the population older than 50 years. Patients at high risk of complications due to carotid atherosclerosis may only present with ophthalmic symptoms initially. We report here a case of a 52-year-old male with disk pallor, provisionally diagnosed as NAAION. On examination, his BP was raised, and had raised ESR and hypercholesterolemia on his blood work. CT Carotid angiogram revealed attenuated internal carotids on the side of involvement with a small eccentric hypodense plaque in the common carotid. This case report of frequently diagnosed NA-AION has been shared to lay emphasis on the importance of a thorough systemic workup in these patients, as their association with systemic illnesses like stroke and obstructive sleep apnea is well known. It will help in not only saving the other eye but also their lives from such life-altering diseases.

Keywords: Arteritic ischemic optic neuropathy, carotid angiography, non-arteritic ischemic optic neuropathy, optic neuropathy, stroke

How to cite this article:
Gounder S, Ahuja P, Akhtar N, Waris A, Narang M. Optic disk pallor—Catch the masqueraders. Role of carotid angiography: A case report and review. Indian J Ophthalmol Case Rep 2023;3:458-62

How to cite this URL:
Gounder S, Ahuja P, Akhtar N, Waris A, Narang M. Optic disk pallor—Catch the masqueraders. Role of carotid angiography: A case report and review. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 4];3:458-62. Available from: https://www.ijoreports.in/text.asp?2023/3/2/458/375009

AION is the most common cause of acute optic nerve ischemia-related vision loss and the second most common cause of optic nerve-related permanent vision loss in adults after glaucoma.[1],[2] NA-AION is the most common ischemic optic neuropathy, with an incidence rate of 2.5–11.8 per 1,00,000 cases in men older than 50 years.[2]

Risk factors associated with NA-AION include nocturnal hypotension, hypertension, diabetes mellitus, smoking, hyper-homocysteinemia, and prothrombotic risk factors like lupus anticoagulant, anticardiolipin antibodies, factor V Leiden mutation, Protein C and S mutations.[1] It is characterized by an acute painless monocular or rarely binocular loss of vision that may progress over time.

Patients with symptoms in the ipsilateral eye often herald the presence of an asymptomatic atherosclerotic carotid disease.[3] Due to its known association with insufficiency and infarction of cerebral blood vessels, patients are at high risk of stroke with risk ranging between 2 to 2.8% and reaching up to 8.4% in ipsilateral carotid stenosis.[4] It is important for an ophthalmologist to be aware of this significant association and help pick these at-risk patients in time, for timely intervention.

We highlight here a case where ophthalmic symptoms were the first presenting feature of a systemic pathology and how it was worked up.

  Case Report Top

We describe the case of a 52-year-old farmer who presented with painless, gradual onset, progressive diminution of vision in the left eye for the past year. His blood pressure was 136/74 mm Hg. The vision was log Mar 0.1 in the right eye and 0.4 in the left eye. IOP by AT was 14 mm Hg in the right eye and 15 mm Hg in the left eye. The patient had a normal color vision with reduced contrast sensitivity in the right eye. Ocular movements were full in all gazes with symmetrical pupils in both eyes. Ocular examination of the right eye was normal, while the left eye had a pre-existing nebulo-macular corneal opacity at 5'o clock measuring 3 × 0.5 mm with a pale oval disk of a cup disk ratio of 0.6 with intact margins following ISNT rule on fundus examination [Figure 1].
Figure 1: (a) Slit-lamp image of the right eye is within normal limits. (b) Slit-lamp image of the left eye shows a pre-existing nebulo-macular corneal opacity inferiorly measuring 3 × 0.5 mm. (c) shows the fundus photograph of the right eye whose parameters were within normal limits. (d) Shows a fundus photograph of the left eye showing a pale disk with raised CD ratio of about 0.6 with intact margins following the ISNT rule

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The perimetry (30-2) in the right eye was normal, while the left eye showed marked depression in the superior visual field. OCT of the right eye showed decreased RNFL thickness in inferotemporal and superonasal quadrants in the left eye with loss of RNFL symmetry with diffuse NRR thinning. Ganglion cell analysis with IPL layer thickness at the macula showed marked thinning in the left eye compared to the right eye [Figure 2].
Figure 2: (a) Perimetry of the right eye was within normal limits. Perimetry of the left eye shows marked depression of light sensitivity in the superior quadrant. There is diffuse loss of superotemporal and superonasal visual field in the left eye. (b) Optical coherence tomography of the right eye showed decreased RNFL thickness in inferotemporal and superonasal quadrants in the left eye with loss of RNFL symmetry. The nasal quadrant was relatively spared in RNFL. The NRR was also diffusely thinned out in the left eye. Ganglion cell analysis with IPL layer thickness at the macula showed marked thinning in the left eye as compared to the right eye

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Though the left eye disk and field changes suggested NTG, the pale neuroretinal rim hinted at a camouflaged neurological etiology. VEP showed prolonged bilateral p100 latency. These findings were suggestive of bilateral optic nerve pathology, with the right eye affected more than the left [Figure 3]. His blood work showed raised ESR and hyperlipidemia [Table 1].
Figure 3: VEP-VEP was done with pattern stimulation using montages Oz-Cz-Fz bilaterally, showed prolonged p100 latency bilaterally, right side p100 was 156 ms, and left was 163 ms. These features suggest bilateral optic nerve pathology, with the right optic nerve affected more than the left

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Table 1: Biochemical investigations of the patient

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CT angiography of the carotid artery showed a relatively attenuated lumen along the entire course of ICA, ACA, MCA, and ophthalmic artery on the left side with no evidence of any intraluminal thrombus. A small, eccentric hypodense plaque was seen in the left distal CCA's left lateral wall and left carotid bulb [Figure 4].
Figure 4: CT angiogram showed a relatively attenuated lumen of the left internal carotid artery (ICA) throughout it's course. 1 - Tiny plaque at the bulb of ICA, 2, 3 - attenuated cervical ICA in coronal and axial sections respectively, 4,5,6,7 - attenuated petrous (4), cavernous (5,6) and lacerum (7) portionof the ICA, 8-attenuated left middle cerebral artery, 9- Maximum Intensity Projection (MIP) CT angiography reconstruction. There was no evidence of any thrombus. A small eccentric atherosclerotic plaque was seen in lateral wall of left distal common carotid artery and left carotid bulb

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Echocardiogram showed an ejection fraction of 65% with no valvular or ventricular abnormalities. He was then prescribed strict blood pressure control with diet modification, Tab Amlodipine 10 mg OD, Tab Atenolol 20 mg OD, and Tab Atorvastatin 40 mg after a cardiology opinion.

  Discussion Top

Asymptomatic extracranial carotid artery disease with more than 50% stenosis is seen in 1.5 to 9% population, having a higher incidence in the elderly. The risk of carotid artery disease may reach up to 8.4% from 2% in patients with high grades of ipsilateral carotid stenosis.[4],[5]

Several authors have found that patients with NAAION have a higher risk of subsequent cerebrovascular and cardiovascular events and an increased mortality from vascular events than the matched population. Symptoms in ipsilateral eye herald the presence of asymptomatic atherosclerotic carotid artery disease and predispose the patients to a high risk of stroke.[6] It has been proven that patients with NA-AION in one eye have a 15–19% risk of developing a similar event in the opposite eye over the subsequent five years.[2] It is important for ophthalmologists to be familiar with these symptoms and signs so that high-risk patients for ischemic stroke can be picked up at their first medical contact and referred for early intervention.[3],[7] Marked asymmetry of findings more marked on the side of the occluded carotid can suggest changes due to carotid narrowing.[8]

Up to 20% of non-glaucomatous cupping, especially when associated with normal intraocular pressure, has been misdiagnosed and treated as normal-tension glaucoma.[9] Young age, disk pallor, loss of visual acuity and color vision, and a poor correlation between the optic nerve and visual field findings are suggestive of a condition other than glaucoma.[4],[10]

Whether it is glaucoma, an intracranial problem, or a carotid artery aneurysm, these cases can be a complex challenge for even the most experienced observer.[5],[6] Scant data are available for neuro-ophthalmic diseases resembling glaucoma, especially when it can be misdiagnosed as NTG. Information about these will help us work up such cases adequately and thus prevent any misdiagnosis.[5]

  Conclusion Top

Carotid angiography is thus recommended in a symptomatic glaucomatous disk, especially with marked disk rim pallor and normal IOP. As the prognosis of vision in the other eye is critically important for the treating clinician and the patient, we wish to re-emphasize on the importance of close follow-ups, full systemic workups, and prompt physician reference in such patients to help prevent vision loss.

Author's contribution

AW and NA conceptualized and designed the article and gave the final approval for this version of the article to be published. SG was responsible for data collection. PA was the major contributor in writing this manuscript. AW and NA critically revised the article. SG and PA edited the illustrations for this article. MN helped with the radiological interpretation of data.


We would like to acknowledge our Head of Department (Institute of Ophthalmology, JNMCH, AMU, Aligarh), Prof Yogesh Gupta sir was always providing us with a guiding hand.

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


Conflicts of interest

There are no conflicts of interest.

  References Top

Patil M, Ganger A, Saxena R. Non-arteritic anterior ischemic optic neuropathy (NAION)—A brief review. Open J Ophthalmol 2016;6:158–63.  Back to cited text no. 1
Miller NR, Arnold AC. Current concepts in the diagnosis, pathogenesis and management of nonarteritic anterior ischaemic optic neuropathy. Eye 2014;29:65–79.  Back to cited text no. 2
Hayreh SS, Joos KM, Podhajsky PA, Long CR. Systemic diseases associated with nonarteritic anterior ischemic optic neuropathy. Am J Ophthalmol 1994;118:766–80.  Back to cited text no. 3
Lee YC, Wang JH, Huang TL, Tsai RK. Increased risk of stroke in patients with nonarteritic anterior ischemic optic neuropathy: A nationwide retrospective cohort study. Am J Ophthalmol 2016;170:183–9.  Back to cited text no. 4
Bruno A, Jones WL, Austin JK, Carter S, Qualls C. Vascular outcome in men with asymptomatic retinal cholesterol emboli: A cohort study. Ann Intern Med 1995;122:249–53.  Back to cited text no. 5
Donders RCJM, Algra A, Rappelle LJ, Van Gijn J, Koudstaal PJ. Clinical features of transient monocular blindness and the likelihood of atherosclerotic lesions of the internal carotid artery. J Neurol Neurosurg Psychiatry 2001;71:247–9.  Back to cited text no. 6
James CB, Sawle G V., Ross Russell RW. The natural history of non-arteritic anterior ischaemic optic neuropathy. J Neurol Neurosurg Psychiatry 1990;53:830–3.  Back to cited text no. 7
American Academy of Ophthalmology. When It's Not Glaucoma. Available from: https://www.aao.org/eyenet/article/when-its-not-glaucoma. [Last accessed on 2022 Apr 29].  Back to cited text no. 8
Choudhari NS, Neog A, Fudnawala V, George R. Cupped disc with normal intraocular pressure: The long road to avoid misdiagnosis. Indian J Ophthalmol 2011;59:491–7.  Back to cited text no. 9
[PUBMED]  [Full text]  
Dias DT, Ushida M, Battistella R, Dorairaj S, Prata TS. Neurophthalmological conditions mimicking glaucomatous optic neuropathy: Analysis of the most common causes of misdiagnosis. BMC Ophthalmol 2017;17:2. doi: 10.1186/s12886-016-0395-x.  Back to cited text no. 10


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

  [Table 1]


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