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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 4  |  Page : 923-925

Ophthalmic artery occlusion in a patient with sickle cell trait and hyperhomocysteinemia


1 Department of Ophthalmology, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India
2 Shri Bhagwan Mahavir Vitreoretinal Services, Medical Research Foundation, Sankara Nethralaya, Chennai, Tamil Nadu, India

Date of Submission23-Jun-2022
Date of Acceptance10-Aug-2022
Date of Web Publication11-Oct-2022

Correspondence Address:
Dr. Himanshu Kashyap
Shri Bhagwan Mahavir Vitreoretinal Services, Medical Research Foundation, Sankara Nethralaya, 18 College Road, Chennai - 600 006, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_1476_22

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  Abstract 


Ophthalmic artery occlusion is a rare, blinding condition seen in patients with disorders like hypertension, hyperlipidemia, and hypercoagulable state. We report a unique case of ophthalmic artery occlusion in a patient with sickle cell trait and hyperhomocysteinemia. A 23-year-old male presented with a complaint of loss of vision in the right eye for the last two days. There is a history of right-sided hemiparesis due to a thromboembolic event. He is a carrier of sickle cell trait. Fundus showed a pale disc, attenuated artery and vein. OCT revealed foveal thinning and thick posterior hyaloid. ERG showed an extinguished scotopic and photopic response.

Keywords: Hyperhomocysteinemia, ophthalmic artery occlusion, sickle cell trait


How to cite this article:
Sahu V, Kashyap H. Ophthalmic artery occlusion in a patient with sickle cell trait and hyperhomocysteinemia. Indian J Ophthalmol Case Rep 2022;2:923-5

How to cite this URL:
Sahu V, Kashyap H. Ophthalmic artery occlusion in a patient with sickle cell trait and hyperhomocysteinemia. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Nov 30];2:923-5. Available from: https://www.ijoreports.in/text.asp?2022/2/4/923/358154



An ophthalmic artery occlusion (OAO) is a partial or complete obstruction of the ophthalmic artery. OAO is usually proximal to the branch point of the posterior ciliary arteries (choroidal supply) and central retinal artery (retinal supply).[1] A similar entity with identical pathophysiology and clinical features is central retinal artery occlusion (CRAO). In a patient with an OAO, vision is profoundly reduced compared to CRAO. Arteritic retinal arterial occlusion (RAO) is caused by giant cell arteritis, whereas most non-arteritic RAO is commonly embolic in origin. Embolic etiologies include auto-immune disorders, malignancies, and blood dyscrasias such as hyperhomocysteinemia and leukemia.[2] Such occlusions may represent an important clinical indicator of events that may require a systemic medical evaluation. The incidence of concurrent stroke is 20–24% cases.[3],[4]

Sickle cell trait (SCT) has been considered a benign condition by ophthalmologists.[5] Complications associated with sickle cell disease (SCD) are usually characterized by proliferative retinopathy.[6] Similarly, hyperhomocysteinemia is an independent risk factor for cardiovascular, cerebrovascular, and retinal vascular occlusions. Herein, we report a rare case of ophthalmic artery occlusion in a young patient with sickle cell trait and hyperhomocysteinemia.


  Case Report Top


A 23-year-old male presented with a complaint of sudden onset and painless vision loss in the right eye (RE) for 2 days. He is a carrier of sickle cell trait. There was a history of left side hemiparesis with facial nerve palsy 1 month back. Previous carotid ultra-sonography (USG) and vertebral Doppler imaging showed a thrombus in the right common carotid artery bulb with extension into the internal carotid artery. Magnetic resonance imaging (MRI) suggested an infarct in the right basal ganglia and internal capsule. He was managed with oral Aspirin (150 mg) and Nicoumalone (2 mg) and subcutaneous low-molecular weight heparin (LMWH) 0.4 ml and was observed systemically for the past 1 month. On ophthalmic evaluation, his best-corrected visual acuity in the left eye (LE) was 6/6, but he had no perception of light in the RE. Anterior segment examination showed grade 3 RAPD in the right eye with no other significant finding. Fundus evaluation of the RE showed a pale disc and severely attenuated retinal blood vessels; the retina was pale all over, but pallor was noted more in the posterior pole. Taut posterior hyaloid at the posterior pole with loss of fovea reflex could be appreciated, whereas the LE had no significant abnormality [Figure 1]a and [Figure 1]b. Optical coherence tomography (OCT) findings of the RE revealed foveal thinning, thick posterior hyaloid, loss of the full-thickness inner retina at multiple places with moth-eaten appearance, and loss of the ellipsoid zone. OCT of the LE was normal [Figure 2]a. Fundus fluorescein angiography (FFA) of the RE revealed an increased arm to retina time, arteriolar narrowing, delayed venous filling, an increased A-V transit time, and the persistent absence of fluorescence at macula because of obliteration of vessels [Figure 1]c. FFA of the LE showed a normal study [Figure 1]d. Although the patient had a 2-day vision loss history, the OCT and FFA findings indicated chronic arterial occlusion. The electro-retinogram (ERG) showed flat waves on photopic, scotopic, oscillatory responses and a 30 Hz flicker response, indicating inner and outer retinal loss occurring chiefly in ophthalmic artery occlusion [Figure 2]b.
Figure 1: (a) Fundus photo of the RE with a pale disc and severely attenuated retinal blood vessels and taut posterior hyaloid at the posterior pole with loss of fovea reflex. (b) Fundus photo of the LE with no abnormality. (c) Fundus fluorescein angiography (FFA) of the RE showing delayed filling, arteriolar narrowing, beginning of the laminar flow, and the persistent absence of fluorescence at the macula. (d) FFA of the LE showing a normal study

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Figure 2: (a) Enface image and section through the macula and disc of the RE showing foveal thinning, thick posterior hyaloid, and loss of the full-thickness inner retina at multiple places with moth-eaten appearance, with loss of the ellipsoid zone. (b) ERG of the RE showing flat waves on photopic, scotopic, oscillatory responses and 30 Hz flicker response

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Hemoglobin electrophoresis showed SCT: HBA of 64%, HBA2 of 4% (range: 1.5–3.5), HBS of 30%, and PTT of 40.2 s (range 29.45–38.74 s), suggestive of sickle cell trait (heterozygous). He had an abnormally high serum homocysteine level of 65 mcmol/L (range 5–15 mcmol/L) and normal Protein C, S, anti-phospholipid, and anti-cardiolipin antibody levels.

Other markers of inflammation and auto-immunity, such as C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and rheumatic antibodies (ANA, dsDNA, ENA, ANCA), were all within normal limits.


  Discussion Top


A sickle cell trait occurs when a person has one gene for sickle hemoglobin and one for normal hemoglobin. It is an established hypothesis that vascular occlusion in SCD is primarily because of micro-vascular obstruction, that is, “log jamming” effect by sickle erythrocytes at the capillary level. It has been proven that SCD can cause large vessel obstruction also, for example, in cases of cerebral stroke. The likely mechanism is an intimal injury caused by adherence to SS RBC.[7] Thrombo-embolic events affect small vessels in retinal circulation, causing CRAO. The ophthalmic artery, although a large vessel, is prone to get involved in such events. In our case, the concurrent occurrence of hyperhomocysteinemia, an independent known risk factor for vascular occlusive disease, could have had an additive effect on the already risk-prone vasculature. There are various mechanisms reported in hyperhomocysteinemia leading to endothelial dysfunction, such as decreased nitric oxide bioavailability, mitogenic effects on smooth muscle cells, and expression of acute stress-related genes.[8],[9]

Treatment options are limited in vascular occlusions in SCD. Major vaso-occlusive complications, such as stroke or severe acute chest syndrome, require immediate manual or automated exchange transfusion to decrease HbS and sickling.[10] Whether maintaining a low HbS and a low homocysteine level prevents recurrence or affects prognosis warrants study.


  Conclusion Top


To conclude, ophthalmic artery occlusion is a rare complication of SCD and hyperhomocysteinemia with a devastating visual outcome. We recommend that patients with fluctuating vision and acute visual changes with a prior history of vaso-occlusive events should be monitored closely for the potential development of ophthalmic artery occlusion and CRAO. Particular emphasis should be given to patients under constant threat of conditions that promote de-hydration, hypoxemia, and intra-vascular sickling. Regular ophthalmic evaluation and ERG monitoring should be contemplated to catch the catastrophic event in the incipient stage and prevent irreversible damage from complete occlusion. In cases where a triggered vaso-occlusive event has been caught early, an immediate physician's consultation is to be advised to avoid life-threatening sequelae.

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.
Olsen TW, Pulido JS, Folk JC, Hyman L, Flaxel CJ, Adelman RA. Retinal and ophthalmic artery occlusions preferred practice pattern®. Ophthalmology 2017;124:P120-43.  Back to cited text no. 1
    
2.
Rachitskaya AV, Lee RK, Dubovy SR, Schiff ER. Combined central retinal vein and central retinal artery occlusions and neovascular glaucoma associated with interferon treatment. Eur J Ophthalmol 2012;22:284-7.  Back to cited text no. 2
    
3.
Flaxel CJ, Adelman RA, Bailey ST, Fawzi A, Lim JI, Vemulakonda GA, et al. Retinal and ophthalmic artery occlusions preferred practice pattern®. Ophthalmology 2020;127:P259-87.  Back to cited text no. 3
    
4.
Lee J, Kim SW, Lee SC, Kwon OW, Kim YD, Byeon SH. Co-occurrence of acute retinal artery occlusion and acute ischemic stroke: Diffusion-weighted magnetic resonance imaging study. Am J Ophthalmol 2014;157:1231-8.  Back to cited text no. 4
    
5.
Jackson H, Bentley C, Hingorani M, Atkinson P, Aclimandos W, Thompson G. Sickle retinopathy in patients with sickle trait. Eye 1995;9:589-93.  Back to cited text no. 5
    
6.
Downes SM, Hambleton IR, Chuang EL, Lois N, Serjeant GR, Bird AC. Incidence and natural history of proliferative sickle cell retinopathy: Observations from a cohort study. Ophthalmology 2005;112:1869-75.  Back to cited text no. 6
    
7.
Francis R. Large-vessel occlusion in sickle cell disease: Pathogenesis, clinical consequences, and therapeutic implications. Med Hypotheses 1991;35:88-95.  Back to cited text no. 7
    
8.
Postea O, Krotz F, Henger A, Keller C, Weiss N. Stereospecific and redox-sensitive increase in monocyte adhesion to endothelial cells by homocysteine. Arterioscler Thromb Vasc Biol 2006;26:508-13.  Back to cited text no. 8
    
9.
Weiss N. Mechanisms of increased vascular oxidant stress in hyperhomocysteinemia and its impact on endothelial function. Curr Drug Metab 2005;6:27-36.  Back to cited text no. 9
    
10.
Liem RI, Calamaras DM, Chhabra MS, Files B, Minniti CP, Thompson AA. Sudden onset blindness in sickle cell disease due to retinal artery occlusion. Pediatr Blood Cancer 2008;50:624-7.  Back to cited text no. 10
    


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