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Home , Optic neuropathy

Case Communications IMAJ • VOL 17 • june 2015

Amaurosis Fugax, Anterior Ischemic and Cilioretinal Artery Occlusion Secondary to Oriel Spierer MD and Michael Waisbourd MD

Department of , Tel Aviv Sourasky Medical Center affiliated with Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel

Laboratory tests revealed erythrocyte Key words: giant cell arteritis (GCA), amaurosis PATIENT DESCRIPTION sedimentation rate of 40 mm/hr and C- fugax, anterior ischemic optic A 70 year old woman presented with sud- reactive protein level of 42 mg/L. Com- neuropathy, cilioretinal artery den visual loss in her left eye. Visual acuity plete blood count including platelets was occlusion, visual loss was no light and there was a within normal limits. Treatment with 60 mg IMAJ 2015; 17: 392–393 3+ relative afferent pupillary defect in the (1 mg/kg/day) oral prednisone was imme- left eye. Funduscopy revealed a pale and diately started. edematous optic disk [Figure A, arrow] and An emergent biopsy of her left tempo- ischemia of the papillomacular bundle area ral artery demonstrated a thickened-wall [Figure A, asterisk], which was also noticed artery with an inflammatory response that ermanent visual loss due to giant cell on fluorescein angiography [Figure B, included giant cells [Figures C and D, P arteritis (GCA) may be prevented by asterisk]. She reported having experienced arrow]. This confirmed the diagnosis of early diagnosis and treatment. Transient several episodes of transient loss of vision in arteritic anterior ischemic optic neuropathy visual loss in one eye () her left eye during the previous few weeks combined with cilioretinal artery occlusion may be the first sign of GCA [1]. We report which resolved spontaneously. According secondary to GCA. In the following weeks the rare case of a patient who presented to the patient she was examined by an the left and retinal edema slowly with the combination of anterior ischemic ophthalmologist who concluded her eye resolved but the optic nerve was pale and optic neuropathy and cilioretinal artery examination as normal. Review of systems visual acuity was no light perception. occlusion resulting in permanent visual revealed that she had had temporal head- Follow-up was continued in conjunction loss in her left eye. The patient reported aches and jaw claudication for a few weeks. with a rheumatologist and during the next having had several episodes of transient Left scalp tenderness with nodular and few months the corticosteroid dosage was visual loss prior to that. Temporal artery pulseless temporal artery was noted. She gradually tapered by 10 mg every month. biopsy confirmed the diagnosis of GCA. did not show weight loss or systemic fever. Six months after her initial presentation

[A] Edematous optic disk (arrow) and ischemia of the papillomacular bundle area (asterisk). [B] Fluorescein angiography shows papillomacular bundle ischemia (asterisk). [C] Thickened-wall temporal artery with an inflammatory response. [D] Giant cell (arrow)

A B C

D

392 IMAJ • VOL 17 • june 2015 Case Communications

the patient was on 10 mg prednisone. The with systemic corticosteroids might have eye. Other acceptable approaches would corticosteroids were then tapered down by prevented the additional ischemic events have been starting a single intravenous 2.5 mg decrements each year. The patient resulting in no light perception in the steroid dose followed by oral treatment was left on a prednisone maintenance dose affected eye. The combination of anterior or intravenous methylprednisolone for 3 of 2.5 mg. Additional ocular or systemic ischemic optic neuropathy and cilioretinal days followed by oral prednisone. There is manifestations of GCA did not occur dur- artery occlusion is a rare clinical mani- no consensus on the optimal way to taper ing 4 years of follow-up. festation of giant cell arteritis. It results the corticosteroid; however, it should be from concomitant thrombotic occlusion done slowly over a period of at least 1 year. of the short posterior ciliary arteries and Patients may stop treatment by 2 years but COMMENT the cilioretinal artery. The recurrent epi- some will continue treatment further on. Prompt diagnosis of GCA is critical to sodes of amaurosis fugax in the affected GCA in the setting of amaurosis fugax prevent further vision loss in the affected eye constitute a rare case of three ocular can be easily missed by both ophthalmolo- eye or any involvement of the second eye. manifestations of GCA in one patient. gists and general practitioners. In their This can be achieved by early treatment There is universal agreement among recent article, Hassan et al. [2] reviewed with systemic corticosteroids, given intra- clinicians that corticosteroids are the main- the symptoms and signs of GCA, includ- venously or orally. GCA occurs in 2.2 per stay of treatment for GCA and should be ing jaw claudication, temporal headache, 10,000 patient-years and a general prac- initiated immediately, even if the diagnosis , abnormality on palpation of the titioner may expect to see one new case is not yet confirmed [5]. However, there temporal artery, elevated erythrocyte sedi- every 1–2 years [2]. The reported incidence has been controversy about whether intra- mentation rate, and anemia. This article was of visual loss and ocular involvement in venous or oral corticosteroid therapy is criticized because amaurosis fugax was not giant cell arteritis varies greatly, between required to treat in GCA. mentioned as one of the symptoms [1]. 6% and 70% [3]. Some physicians claim that intravenous GCA should always be sought in a patient The most common ophthalmic compli- corticosteroid therapy is no more effective presenting with amaurosis fugax, as delay in cation is anterior ischemic optic neuropathy than oral therapy in preventing visual dete- recognition may lead to irreversible loss of followed by central retinal artery occlusion, rioration in GCA [5]. Others will always vision. The occurrence of amaurosis fugax, cilioretinal artery occlusion, and posterior recommend intravenous corticosteroids anterior ischemic optic neuropathy and ischemic optic neuropathy [3]. Other ocu- (methylprednisolone at a dose of 500–1000 cilioretinal artery occlusion in one patient lar symptoms are diplopia and non-specific mg per day for 3 days) followed by 1 mg/ is a rare complication of GCA. . Amaurosis fugax, which is believed kg/day of oral prednisone and admis- to be due to optic nerve head ischemia, is sion of patients with acute visual loss and Correspondence Dr. O. Spierer a presenting symptom in 7–36% of cases presumed GCA [5]. Hayreh and Biousse Dept. of Ophthalmology, Tel Aviv Sourasky Medical [4]. It often precedes permanent visual [5] advised oral prednisone treatment, Center, Tel Aviv 64239, Israel loss and may be the key to early diagnosis except in the following three situations: i) Phone: (972-3) 697-3408 Fax: (972-3) 697-3870 and prevention in many cases [1]. The dif- a history of amaurosis fugax but no visual email: [email protected] ferential diagnosis of ameurosis fugax or loss (amaurosis fugax is an ominous sign painless transient monocular visual loss of impending visual loss), ii) complete or References includes ocular pathology such as tear film marked loss of vision in one eye, and iii) 1. Krishnan R, Farnworth D, Kirwan J. Don’t forget abnormality or transient elevation of intra- early signs of involvement of the second amaurosis fugax. BMJ 2011; 342: d3686. 2. Hassan N, Dasgupta B, Barraclough K. Giant cell ocular pressure, optic nerve disorders such eye. In these conditions, he suggested arteritis. BMJ 2011; 342: d3019. as compressive lesion or demyelinating one dose of intravenous corticosteroid 3. Hayreh SS, Podhajsky PA, Zimmerman B. Ocular disease, vascular disease such as carotid or (as outpatients), followed by the oral cor- manifestations of giant cell arteritis. Am J Ophthal- cardiac emboli, migraine or vasculitis and ticosteroid regimen, starting with 80 mg mol 1998; 125: 509-20. 4. Hayreh SS, Zimmerman MB. Amaurosis fugax in non-organic visual loss. prednisone daily [5]. In the current case, as ocular vascular occlusive disorders: prevalence The episodes of transient loss of vision the patient presented 2 days after she had a and pathogeneses. 2014; 34: 115-22. for several weeks should have alerted the complete vision loss (no light perception), 5. Hayreh SS, Biousse V. Treatment of acute visual loss in giant cell arteritis: should we prescribe patient’s local ophthalmologist to the she was treated with oral steroids only, in high-dose intravenous steroids or just oral possibility of GCA. An earlier treatment order to prevent involvement of the second steroids? J Neuroophthalmol 2012; 32: 278-87.

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