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Retrobulbar spot sign: Ultrasonic evidence of embolic central retinal artery occlusion in a patient with clinical features of temporal arteritis Stephanie M. Aguilar, O.D. A patient presents with sudden painless vision loss from central retinal artery occlusion and clinical features suggestive of temporal arteritis. B scan findings point to embolic disease. The determination of etiology and management are discussed. I. Case history Patient demographics: 72 year old white male of Irish and French descent Chief complaint: Patient reports constant, dark vision OS that began 15 days prior upon awakening in the morning. He reports that when he looks directly at a light he can see some light but the center is completely black. It has not gotten worse or better since symptoms first started. No flashes of light noted. He reports a dull ache around his left eye. No numbness or tingling sensations in extremities. No jaw claudication, scalp tenderness, or temple pain. Ocular history: left homonymous hemianopsia OU, cataract OD, pseudophakia OS, dry eye syndrome OU, refractive error and presbyopia Medical history: DM type II, hypertension, hyperlipidemia, obesity, right occipital CVA in 2005, anemia, gastrointestinal stromal tumor in remission, congestive heart failure, cardiomyopathy, obstructive sleep apnea, smoking cessation in 2008 Medications: Aspirin 81mg, Multivitamin, Allopurinol 300mg, Carvedilol 25mg, Glucose 4gm, Lisinopril 5mg, Torsemide 20mg, Rosuvastatin CA 20mg, Insulin 100unit/ml, Colchicine 0.6mg Salient information: Torsemide 20mg and Carvedilol 25mg dosed at 11:00pm II. Pertinent findings Clinical findings Visual acuity: OD 20/25-2 PH 20/25+2 OS: light perception with projection CVF: OD constricted nasal, OS HM in all quadrants except NLP temporal Pupils: RRL 4+APD OS IOP: OD 20 mmHg, OS 14 mmHg Macula: OD clear, OS cherry red spot Vessels: OD clear, OS attenuated arteries Posterior pole: OD clear, OS superficial whitening of retina with apparent edema Optic nerve: OD 0.55 C/D, OS pale nerve 360 with visible plaque in central retinal artery (photographic evidence reveals plaque was known to be present in November 2012) No NVI/NVA/NVD/NVE upon 4 week follow up Physical findings Temporal arteries: non-tender R/L, pulsatile on right only Laboratory findings ESR: 74 CRP: 8.54 Platelets: 194 eGFR: 18 Creatinine: 3.34 Glucose: 120 mg/dL Triglyceride: 674 mg/dL Radiology studies Carotid Doppler: >90% stenosis of left internal carotid artery B scan: two hyper-reflecting areas that may correspond to two plaques in central retinal artery OS CT head: no acute process SD-OCT: edematous inner retinal layers OS III. Differential Diagnoses Primary: embolic central retinal artery occlusion Secondary: central retinal artery occlusion due to temporal arteritis IV. Diagnosis and discussion The diagnosis is embolic central retinal artery occlusion (CRAO) of the left eye occurring at the beginning of July 2015, two weeks prior to patient presenting to clinic. Given the ultrasound result of greater than 90 percent stenosis of the internal carotid ipsilateral to the symptomatic eye and the absence of systemic symptoms suggestive of giant cell arteritis a diagnosis of embolic central retinal artery occlusion is primary. Follow up of the ESR demonstrated a declining value consistent with non- arteritic stenosis. His risk factors for temporal arteritis include his increased age and race. The ocular pain in the left eye was reportedly present for only thirty minutes and subsided indefinitely. The carotid duplex showed greater than 90 percent stenosis of the left internal carotid artery. Interestingly, in November 2012, a calcific embolus in the left central retinal artery was noted which led to a subclinical branch retinal artery occlusion in the inferior nasal quadrant. B scan ultrasonography showed two hyper-reflective plaques, likely the second more proximal plaque is located where the central retinal artery pierces the dural sheath of the optic nerve, causing the occlusive event. This is known as the “spot sign”. 1 Thus, the CRAO is likely due to a second deep embolus at the lamina cribosa in combination with reduced arterial pressure and severe ipsilateral internal carotid artery stenosis contributing to the perfusion failure. It is worthwhile to note that the patient first became aware of vision loss upon awakening and is dosing antihypertensive medication, Carvedilol and Terosemide, at 11:00pm. It is possible that this dosing pattern exacerbated physiological nocturnal hypotension and in the setting of carotid disease, reduced retinal perfusion to such a degree that infarct resulted. This patient’s functional impairment attributable to the artery occlusion is made worse by the history of right occipital lobe vascular accident in 2005 resulting in a left homonymous hemianopsia. Thus making long-term management of this case more complicated and atherosclerotic risk factor management all the more significant. After the most recent occlusive event of the central retinal artery, the patient has only one-fourth or approximately 90 degrees of his bilateral visual field intact, leaving his visual field severely constricted and his stereopsis nonexistent. V. Treatment and management Acute management of CRAO begins with an attempt to recover vision. It has been shown in an experimental study of CRAO in rhesus monkeys that complete ischemia to the retina of more than four hours resulted in severe irreversible neuronal loss. 2 The conservative method of treatment for CRAO includes ocular massage, hyperbaric oxygen, anterior chamber paracentesis, corticosteroids and topical anti-hypertensive medications among others. Although, none have proven to be more effective than a placebo and may actually be harmful.3 The exception is CRAO due to temporal arteritis, where prompt systemic steroid therapy can improve the visual prognosis. 4 Thrombolysis is currently the most popular therapy, and success has been enthusiastically claimed. However, intra-arterial thrombolytic therapy has only been proven beneficial for treatment of fibrin emboli and within four hours of onset.5 Long term management of CRAO focuses on preventing recurrent vascular events. Management is cause specific; high grade carotid disease should undergo carotid endarterectomy while a cardiac source of emboli should be managed with anti-coagulation medication. 6 This population is at high risk of secondary or tertiary ischemic events, so risk factor modification is prudent. 7 In all geriatric cases, atherosclerosis risk factor modification should be employed. In this particular case, the patient underwent a left carotid endarterectomy. He will be scheduled for mobility training and rehabilitation in the low vision clinic. One month follow up revealed no neovascularization and similar visual acuity to that at first presentation. Comanagement with the patient’s cardiologist and primary care physician are employed to hopefully increase the patient’s life span and quality of life. Bibliography 1. Nedelmann M, Graef M, Weinand F, Wassill KH, Kaps M. Retrobulbar spot sign predicts thrombolytic treatment effects and etiology in central retinal artery occlusion. Stroke. 2015 Aug;46(8):2322-4. Epub 2015 Jun 25. 2. Hayreh SS, Zimmerman MB, Kimura A, Sanon A. Central retinal artery occlusion. Retinal survival time. Exp Eye Res 2004; 78: 723-736. 3. Schrag M, Youn T, Schindler J, Kirshner H, Greer D. Intravenous Fibrinolytic Therapy in Central Retinal Artery Occlusion, A patient level meta- analysis. JAMA Neurol. Published online August 10, 2015. jamaneurol.2015.1578. 4. Azhar SS, Tang RA, Dorotheo EU. Giant cell arteritis: diagnosing and treating inflammatory disease in older adults. Geriatrics. 2005 Aug;60(8):26-30. 5. Hayreh SS. Intra-arterial thrombolysis for central retinal artery occlusion. Br J Ophthalmol 2008 92: 585-587. 6. Kramer M, Goldenberg-Cohen N, Shapira Y, Axer-Siegel R, Shmuely H, Adler Y. Role of transesophageal echocardiography in the evaluation of patients with retinal artery occlusion. Ophthalmology. Volume 108, Issue 8, August 2001, 1461-1464. 7. Varma DD, Cugati S, Lee AW, Chen CS. A review of central retinal artery occlusion: clinical presentation and management. Eye 2013. Published online: 8 March 2013. VI. Conclusion This case elaborates on how atherosclerotic disease and consequently, stroke can change a person’s life, render them legally blind and alter their daily functionality. It is possible the CRAO event may have saved this patient’s life because without it, the high grade carotid artery stenosis may not have been discovered until too late. In this ophthalmic emergency, prompt consultation with the neurology department was key . B scan ultrasonography was supportive of our diagnosis and management. As optometrists, it is our goal to use every tool available to conserve what is left of our patient’s vision and visual field and thus enable them to live a full life.