Photo Essay Bilateral Anterior Ischemic Optic Neuropathy Accompanied with Unilateral Central Retinal Artery Occlusion in a Biopsy-proven Case of Giant Cell Arteritis Kaveh Abri Aghdam1, MD, PhD; Ali Aghajani1,2, MD; Mehdi Khakpour1, MD; Mostafa Soltan Sanjari1, MD 1Eye Research Center, Eye Department, The Five Senses Health Institute, School of Medicine, Iran University of Medical Sciences, Tehran, Iran 2Isfahan Eye Research center, Department of Ophthalmology, Isfahan University of Medical Sciences, Isfahan, Iran ORCID: Kaveh Abri Aghdam: https://orcid.org/0000-0001-7568-6455 Ali Aghajani: https://orcid.org/0000-0002-7790-6033 J Ophthalmic Vis Res 2022; 17 (3): 443–446 PRESENTATION An 80-year-old male patient was referred to the neuro-ophthalmology clinic with a history of sudden vision loss in the right eye one week earlier and vision loss in the left eye four days after that. The vision assessment was no light perception in both eyes at the time of the initial examination. Both eyes were pseudophakic and anterior segment examination was unremarkable. Funduscopic examination revealed a swollen optic disc in the right eye and a diffuse retinal whitening with a cherry-red spot, arterial attenuation, and a chalky-white blurred- margin optic disc in the left eye (Figures 1A & 1B). Fluorescein angiography (FA) disclosed a diffuse choroidal filling delay with leakage from the optic Correspondence to: Ali Aghajani, MD. Department of Ophthalmology, Eye Research Center, The Five Senses Institute, Rassoul Akram Hospital, Niayesh Avenue, Sattarkhan St., Tehran 1445613131, Iran. E-mail: aliaghajani_y@yahoo.com Received: 13-06-2021 Accepted: 22-09-2021 Access this article online Website: https://knepublishing.com/index.php/JOVR DOI: 10.18502/jovr.v17i3.11585 nerve head in the venous phase in the right eye and nasal choroidal filling delay and diffuse delay in the retinal arterial filling in the left eye [Figure 2]. The erythrocyte sedimentation rate was 98 mm/hr, and the level of C-reactive protein was 76 mg/L. The patient was admitted and pulse corticosteroid therapy was commenced with the diagnosis of giant cell arteritis (GCA). The diagnosis was later confirmed with a histopathologic evaluation of the left side temporal artery biopsy specimen [Figure 1C]. Unfortunately, even though treatment was started immediately, it failed to improve the patient’s vision. DISCUSSION Giant cell arteritis (GCA) is a granulomatous vasculitis that involves large- and medium-sized vessels. Anterior ischemic optic neuropathy (AION), This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. How to cite this article: Abri Aghdam K, Aghajani A, Khakpour M, Sanjari MS. Bilateral Anterior Ischemic Optic Neuropathy Accompanied with Unilateral Central Retinal Artery Occlusion in a Biopsy-proven Case of Giant Cell Arteritis. J Ophthalmic Vis Res 2022;17:443–446. © 2022 Abri Aghdam et al. THIS IS AN OPEN ACCESS ARTICLE DISTRIBUTED UNDER THE CREATIVE COMMONS ATTRIBUTION LICENSE | PUBLISHED BY KNOWLEDGE E 443 http://crossmark.crossref.org/dialog/?doi=10.18502/jovr.v17i3.11585&domain=pdf&date_stamp=2019-07-17 https://knepublishing.com/index.php/JOVR Photo Essay; Abri Aghdam et al Figure 1. Color fundus photograph of the right eye showing a blurred-margin optic disc; note the paleness of the swollen optic disc (A). Color fundus photograph of the left eye showing diffuse opaque retina, severe arterial attenuation, box-carring, and chalky white blurred-margin optic disc with multiple superficial hemorrhages (B). Photomicrograph of the biopsy specimen from the left temporal artery reveals fibrinoid necrosis (arrowheads), endothelial destruction, and transmural infiltration of lymphocytes, polymorphonuclear neutrophils (black asterix), histiocytes, and giant cells (black arrow). Organized thrombi in the lumen are seen (white asterix). The overall findings are compatible with the diagnosis of giant cell arteritis (hematoxylin and eosin staining, 40X magnification) (C). central retinal artery occlusion (CRAO), and posterior ischemic optic neuropathy (PION) are the most reported ophthalmic presentations of GCA.[1] The main goal of corticosteroid therapy in GCA is to prevent the occurrence or progression of visual loss. Bilateral ocular involvement is another concern in these patients. Hayreh et al[2] reported that in all GCA patients with bilateral vision impairment, older changes could be found in one eye, suggesting that the patients were unaware of vision loss in one eye until the second eye was affected giving the erroneous interpretation of simultaneous bilateral ocular involvement. Thus, the estimations of the prevalence of simultaneous bilateral GCA ocular involvement suffer from the inaccuracy in the timing of the examination and perhaps were mostly imprecise and possibly preventable. CRAO is a rare but well-known disaster that has been reported in 4% of GCA patients.[3] 444 JOURNAL OF OPHTHALMIC AND VISION RESEARCH Volume 17, Issue 3, July-September 2022 Photo Essay; Abri Aghdam et al Figure 2. Fluorescein angiography in the early arteriovenous phase (30 sec) reveals a diffuse choroidal filling delay in the right eye (A), and a choroidal filling defect in the nasal retina as well as arterial filling delay in the left eye (B). Fluorescein angiography in the venous phase (1 min), patchy choroidal filling defects accompanied with optic disc leakage in the right eye (C), and a sectoral choroidal filling defect in the nasal fundus as well as arterial filling delay in the left eye (D). Fluorescein angiography in the late phase (5 min) shows patchy choroidal filling defect and optic nerve head leakage in the right eye (E), and patchy choroidal filling defect and arterial filling delay in the left eye (F). JOURNAL OF OPHTHALMIC AND VISION RESEARCH Volume 17, Issue 3, July-September 2022 445 Photo Essay; Abri Aghdam et al CRAO in these patients is almost invariably associated with AION, and posterior ciliary artery occlusion (revealed by FA study in patients with CRAO) is highly suggestive of the arteritic nature of the occlusion.[4] There has been only one report of a similar presentation of GCA in literature.[5] Although we cannot confirm the similarity of the extent of bilateral involvement between these two cases due to the lack of sufficient angiographic data, both reports have one thing in common, which is neither of these patients has enjoyed vision improvement after pulse corticosteroid therapy. Overall, this report presents an extreme manifestation of GCA with detailed illustrations to emphasize the importance of timely diagnosis and urgent treatment in these patients. Consent to Participate Informed consent including publication of photographs in medical journals was obtained from the participant of this study. Financial Support and Sponsorship The authors did not receive any financial support or funding for this work. Conflicts of Interest The authors have no conflicts of interest to declare. REFERENCES 1. Fein AS, Ko MW. Neuro-ophthalmologic complications of giant cell arteritis: Diagnosis and treatment. Semin Neurol 2019;39:673–681. 2. Hayreh SS. Giant cell arteritis: Its ophthalmic manifestations. Indian J Ophthalmol 2021;69:227–235. 3. Singh AG, Kermani TA, Crowson CS, Weyand CM, Matteson EL, Warrington KJ. Visual manifestations in giant cell arteritis: Trend over 5 decades in a population-based cohort. J Rheumatol 2015;42:309–315. 4. Hayreh SS, Zimmerman MB. Central retinal artery occlusion: Visual outcome. Am J Ophthalmol 2005;140:376.e1–376.e. 5. Joseph ADD, Pradeepan JA, Kumanan T, Malaravan M. Combined left central retinal artery occlusion and bilateral anterior ischemic optic neuritis: A rare presentation of giant cell arteritis. Case Rep Rheumatol 2019;2019:3236821. 446 JOURNAL OF OPHTHALMIC AND VISION RESEARCH Volume 17, Issue 3, July-September 2022