|Year : 2020 | Volume
| Issue : 3 | Page : 209-211
Bilateral central retinal artery occlusion as a premonitory presentation in giant cell arteritis
Stephanie Sebastian Koilparampil, Krishnagopal Srikanth, Muthukrishnan Vallinayagam
Department of Ophthalmology, Mahatma Gandhi Medical College and Research Institute, Sri Balaji Vidyapeeth, Puducherry, India
|Date of Submission||19-Mar-2020|
|Date of Decision||21-Apr-2020|
|Date of Acceptance||06-May-2020|
|Date of Web Publication||14-Sep-2020|
Dr. Krishnagopal Srikanth
Department of Ophthalmology, Mahatma Gandhi Medical College and Research Institute, Sri Balaji Vidyapeeth, Puducherry
Source of Support: None, Conflict of Interest: None
Central retinal artery occlusion (CRAO) is an ophthalmologic emergency. Carotid artery atherosclerosis, cardiogenic emboli, hypertension, smoking, diabetes, and giant cell arteritis (GCA) are the contributing risk factors for CRAO. It is imperative to rule out GCA in patients aged above 50 years as it can cause severe visual loss involving both eyes, if not treated promptly, making diagnosis critical. Elevated C-reactive protein and erythrocyte sedimentation rate with a raised platelet count favor GCA as a probable diagnosis. Typical granulomatous inflammation on temporal artery biopsy confirms the diagnosis. The vision loss is permanent, but early steroid therapy is effective in preventing further visual loss.
Keywords: Bilateral central retinal artery occlusion, giant cell arteritis, sudden vision loss
|How to cite this article:|
Koilparampil SS, Srikanth K, Vallinayagam M. Bilateral central retinal artery occlusion as a premonitory presentation in giant cell arteritis. TNOA J Ophthalmic Sci Res 2020;58:209-11
|How to cite this URL:|
Koilparampil SS, Srikanth K, Vallinayagam M. Bilateral central retinal artery occlusion as a premonitory presentation in giant cell arteritis. TNOA J Ophthalmic Sci Res [serial online] 2020 [cited 2021 Mar 3];58:209-11. Available from: https://www.tnoajosr.com/text.asp?2020/58/3/209/294979
| Introduction|| |
Central retinal artery occlusion (CRAO) is an ophthalmologic emergency with poor prognosis. CRAO is classically unilateral and presents with sudden, painless, profound loss of vision. Carotid atherosclerosis, cardiogenic emboli, hypertension, and giant cell arteritis (GCA) are known risk factors. It is imperative to rule out GCA as it can result in severe visual loss involving both eyes. We report this case distinguished by its rarity of a 68-year-old hypertensive male with a history of cerebrovascular accident (CVA) who presented with sequential bilateral CRAO over a period of 48 h and was diagnosed with GCA.
| Case Report|| |
A 68-year-old hypertensive male presented with sudden, painless loss of vision in both eyes. The vision loss had first established itself in the right eye, followed by the left eye 2 days later. General examination revealed prominent temporal arteries in an ill-built febrile patient. The visual acuity was hand movements in both eyes with both the pupils sluggishly reacting to light. The extraocular muscle movements were globally restricted. There was jaw claudication. There was a history of CVA 1 year back, with no residual neurological deficits.
Fundus examination disclosed bilateral pale edematous retina with cherry red spot at the macula [Figure 1]. Significant arteriolar attenuation and prominent veins were noted along with the pathognomic “cattle trucking” of the vessels [Figure 2]. Magnetic resonance imaging brain corresponded to the features suggestive of small-vessel ischemic changes in the past, and carotid Doppler ruled out flow-limiting plaques or stenosis. Fluorescein angiography revealed delayed arterial filling in both eyes, suggestive of bilateral CRAO [Figure 3].
|Figure 1: Right eye showing disc edema, with cherry red spot (black arrow)|
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|Figure 2: Left eye showing disc edema with cherry red spot and pale edematous retina (black arrow)|
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|Figure 3: Fundus fluorescein angiography showing delayed arterial filling|
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Inflammatory markers such as C-reactive protein (CRP, 96 mg/dl) and erythrocyte sedimentation rate (ESR, 120 mg/dl) were highly elevated, and platelets (5.3 lakh/cm3) were significantly raised. The patient underwent temporal artery biopsy, and histopathology demonstrated features of chronic inflammatory infiltration with foci of calcification [Figure 4]. The patient was treated with 1 g of intravenous (IV) methyl prednisolone daily for 3 days, after which it was converted to oral steroids which was tapered gradually over the next 6 months. The visual acuity remained unchanged in the follow-up visits.
|Figure 4: Histopathological section showing chronic inflammatory cells (green arrow), elastic degeneration (red arrow), and calcification (black arrow)|
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| Discussion|| |
GCA or temporal arteritis is a systemic autoimmune disease affecting primarily the elderly. It is characterized by granulomatous inflammation of the large- and medium-sized arteries and is known to cause sudden, severe, and sequential visual loss. A tense, tender, inflamed, nodular, and nonpulsatile temporal artery is the characteristic clinical sign of GCA. Headache at the temples is the most common symptom. Jaw claudication is the most specific. Other symptoms include scalp tenderness, neck pain, malaise, weight loss, and low-grade fever. Visual acuity is usually less than 20/200 in majority of the patients. The fellow eye is known to get involved within days to weeks, which makes early diagnosis critical. Elevated CRP and ESR exceeding 50 mm/h and typical granulomatous inflammation on temporal artery biopsy help in confirming the diagnosis. Permanent visual loss has been reported to occur in up to 15%–20% of the affected patients.
GCA has a calamitous association with CRAO. CRAO, alternately termed the “stroke of the eye,” presents with sudden, profound, painless, monocular loss of vision and is an ophthalmic emergency. Retinal damage is extensive and vision loss is irreversible when the blood flow is interrupted for more than 4 h. Vision can drastically decline to hand movements within minutes. Typically unilateral, its incidence is around 1 in 10,000–100,000. This case report of sequentially developed bilateral CRAO is set apart by its uniqueness and rarity. To the best of our knowledge, there is only one such case that boasts of bilateral CRAO in association with GCA. Carotid artery atherosclerosis, cardiogenic emboli, hypertension, CVAs, smoking, diabetes, and GCA are known risk factors for CRAO. Spontaneous visual recovery after CRAO is extremely unusual.
Raised ESR and CRP indicate systemic inflammation. Hayreh et al. reported that the combination of ESR and CRP is 97% specific for the diagnosis of GCA. This case had remarkably high ESR, and platelets were unmistakably raised. Thrombocytosis is a sign of active inflammation. Thrombocytosis and CRP can be more sensitive inflammatory markers than ESR for GCA.
A temporal artery biopsy is considered the gold standard in diagnosis. Occasional false-negative results are obtained due to “skip lesions.” The specificity is considered to be 100% when a giant cell is identified. Histopathologically, two patterns are regarded diagnostic for GCA – (1) active arteritis which includes thickening of the arterial wall with a preponderance of lymphocytes, macrophages, and giant cells and (2) healed arteritis which presents with fibrosis of the vessel wall and disruption of the internal elastic lamina. Our patient presented with signs of both active and healed arteritis on histopathological examination.
Corticosteroids acting at the cellular level may be important in the acute phase of injury. Dramatic recovery of vision has been reported on conventional steroid doses and even when steroids were not used. For the most part, visual loss is permanent. However, steroid therapy is usually effective in preventing further visual loss. This patient, treated with methyl prednisolone, showed no improvement in visual acuity on further visits.
| Conclusion|| |
This case illustrates that bilateral CRAO should be considered as a premonitory presentation in GCA. In patients with concurrent CRAO and GCA, prompt recognition and early treatment are crucial to prevent life-threatening systemic consequences, such as stroke and visual loss in the fellow eye.
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.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]