Central Retinal Artery Occlusion With Subsequent Central Retinal Vein Occlusion in Biopsy-Proven Giant Cell Arteritis

We performed bilateral optic nerve sheath fenestrations on a patient with the syndrome of acquired hyperopia and choroidal folds. We are unaware of previous reports of this procedure being performed in this clinical setting. Despite the incomplete resolution of his posterior segment findings postoperatively, the results of the procedure, along with an understanding of the relevant anatomy, may help to shed light on the pathogenesis of this rare entity.

Photo Essay Section Editor: Timothy J. McCulley, MD Central Retinal Artery Occlusion With Subsequent Central Retinal Vein Occlusion in Biopsy-Proven Giant Cell Arteritis Zoë R. Williams, MD, Xiaofei Wang, MD, PhD, David A. DiLoreto Jr, MD, PhD FIG. 1. The left fundus reveals diffuse preretinal and intraretinal hemorrhages, venular dilation and tortuosity, arteriolar attenuation, optic disc edema, and a cherry-red spot in the macula. Inset, temporal artery biopsy specimen shows lymphocytic infiltrate, a giant cell, and disruption of the internal elastic lamina (hematoxylin and eosin, ·400). Abstract: Central retinal artery occlusion with subsequent central retinal vein occlusion in the same eye is a rare entity. We present a 72-year-old man with biopsy-proven giant cell arteritis who developed bilateral arteritic anterior ischemic optic neuropathy and a left central retinal artery occlusion. Subsequently, he developed a left central retinal vein occlusion within 2 weeks of his initial vision loss. His vision did not improve with corticosteroids. Journal of Neuro-Ophthalmology 2016;36:290-291 doi: 10.1097/WNO.0000000000000385 © 2016 by North American Neuro-Ophthalmology Society A 72-year-old man with well-controlled hypertension awoke with vision loss in his left eye. The previous Flaum Eye Institute (ZRW, DAD), University of Rochester Medical Center, Rochester, New York; Department of Ophthalmology (XW), Rochester General Hospital, Rochester, New York. Supported by an unrestricted grant from Research to Prevent Blindness. The authors report no conflicts of interest. Address correspondence to Zoë R. Williams, MD, Flaum Eye Institute, University of Rochester Medical Center, 601 Elmwood Avenue, Box 659, Rochester, NY 14642; E-mail: [e-mail] 290 evening, he noticed transient obscurations of vision in his left eye, lasting minutes. He reported new onset global headaches, jaw claudication, and scalp tenderness 3 weeks before his vision loss and an unintentional weight loss of 8 pounds. He had no complaints regarding vision in his right eye. On examination, visual acuity was 20/20 in the right eye and light perception in the left eye, with a left relative afferent pupillary defect. There was pallid optic disc edema, left . right, and a left central retinal artery occlusion. His erythrocyte sedimentation rate was 85 mm/h and C-reactive protein (CRP) was 70.5 mg/L (normal: 1.0- 10.0 mg/L). Complete blood count revealed normal hemoglobin and hematocrit and thrombocytosis of 466,000 platelets/mL (normal: 150,000-450,000 platelets/mL). He was immediately treated for presumed giant-cell arteritis (GCA) with intravenous methylprednisolone 250 mg QID for 5 days, followed by prednisone 1 mg/kg daily, and aspirin 81 mg daily. Left temporal artery biopsy was consistent with GCA (Fig. 1). The patient's vision remained stable, but his headaches, jaw claudication, and scalp tenderness resolved. Two weeks later, the patient was referred for neuroophthalmic evaluation. Visual acuity was 20/40 in the Williams et al: J Neuro-Ophthalmol 2016; 36: 290-291 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay right eye and no light perception in the left eye. He could only recognize 7 of 14 color plates (Hardy-Rand-Rittler). Automated perimetry revealed superior arcuate and nasal field defects in the right eye. The right fundus showed pallid optic disc edema with arteriolar attenuation and scattered cotton-wool spots. In the left eye, there was disc edema with confluent peripapillary hemorrhage, 4 quadrants of dot-blot hemorrhages, venous engorgement, and retinal whitening with a cherry-red spot in the macula (Fig. 1). Repeat erythrocyte sedimentation was 2 mm/h, and CRP was 2.0 mg/L. The patient's examination and inflammatory markers remained stable with slow steroid taper until 3 months after his initial vision loss when the patient developed neovascular glaucoma in his left eye with an intraocular pressure of 38 mm Hg. He was treated with intravitreal anti- vascular endothelial growth factor injections and diode laser therapy with excellent intraocular pressure response and regression of iris neovascularization. Four months after his vision loss, there was an optic disc pallor without cupping in the right eye with arteriolar attenuation. In the left eye, there was profound optic atrophy with sclerotic vessels, fibrovascular tissue from chronic macular edema, and residual peripapillary hemorrhage and diffuse intraretinal hemorrhages. Fluorescein angiography showed neovascularization of the left optic disc and normal choroidal filling. The rare presentation of ipsilateral central retinal artery and vein occlusion typically is found in patients with cardiovascular disease, hypertension, coagulation disorders, autoimmune disease, and malignancy (1). Chu and Chen (2) reported a patient with a concurrent central retinal artery occlusion and contralateral branch retinal vein occlusions in a patient with hypertension, peripheral vascular disease, and biopsy-proven GCA. Wagener and Hollenhorst (3) described a single case of combined central retinal artery and vein occlusion in a patient with a clinical diagnosis of GCA (no biopsy performed) in their series of 122 patients. To our knowledge, this is the first report of arteritic anterior ischemic optic neuropathy and central retinal artery occlusion with subsequent central retinal vein occlusion in the same eye secondary to biopsyproven GCA. Our patient had well-controlled hypertension and a remote history of smoking (7 pack-year history) but otherwise no cardiovascular risk factors including diabetes mellitus or hyperlipidemia. Acute vascular inflammation from GCA may cause endothelial damage and luminal thickening in an artery with pre-existing arteriosclerosis, thus promoting venular compression and thrombosis due to the shared adventitial sheath of the central retinal artery and vein at the lamina cribrosa (2,4-7). Alternatively, reduced flow, induced by central retinal artery occlusion, may promote thrombosis in the central retinal vein (7-9). In 1956, Klien and Olwin Williams et al: J Neuro-Ophthalmol 2016; 36: 290-291 (7) described a third category in the pathophysiology of central retinal vein occlusion: primary degenerative or inflammatory venous disease. In their 2 patients found to have histologic evidence of inflammatory venous disease causing central retinal vein occlusion, there was fibrosis of the venular walls of the retrolaminar central retinal vein with lymphocytic infiltration, causing progressive narrowing of the lumen. Combined retinal artery and vein occlusions have been reported in patients with other vasculitides, including systemic lupus erythematosus and Behçet disease, and may occur by a similar mechanism. However, the study by Schmidt (1) did not include information regarding whether the retinal arterial occlusion preceded or followed the retinal vein occlusion. The rarity of this presentation suggests a multifactorial etiology, but in the appropriate clinical setting, GCA should be considered in the differential diagnosis for central retinal artery occlusion with subsequent ipsilateral central retinal vein occlusion. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: D. A. DiLoreto, Jr and Z. R. Williams; b. Acquisition of data: D. A. DiLoreto, Jr, Xiaofei Wang, and Z. R. Williams; c. Analysis and interpretation of data: D. A. DiLoreto, Jr and Z. R. Williams. Category 2: a. Drafting the manuscript: D. A. DiLoreto, Jr, Xiaofei Wang, and Z. R. Williams; b. Revising manuscript for intellectual content: D. A. DiLoreto, Jr and Z. R. Williams. Category 3: a. Final approval of the completed manuscript: D. A. DiLoreto, Jr, Xiaofei Wang, and Z. R. Williams. Acknowledgments The authors thank Joel Shapiro, MD, of the Department of Pathology, Rochester General Hospital. REFERENCES 1. Schmidt D. Comorbidities in combined retinal artery and vein occlusions. Eur J Med Res. 2013;18:27. 2. Chu ER, Chen CS. Concurrent central retinal artery occlusion and branch retinal vein occlusion in giant cell arteritis. Clin Ophthalmol. 2010;4:565-567. 3. Wagener HP, Hollenhorst RW. The ocular lesions of temporal arteritis. Trans Am Ophthalmol Soc. 1957;55:249-273. 4. 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