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MEDICAL RETINA FEATURE STORY Hollenhorst Plaques These may be just the tip of the iceberg. By Charles C. Wykoff, MD, PhD; and Daniel E. Croft, BA “The good physician treats the disease; the great physician treats the patient who has the disease.” –William Osler, MD, one of the Founding Professors of Johns Hopkins Hospital Many of our patients are affected by retinal pathologies that are merely a manifestation of a systemic disease. A s retina specialists, we have the privilege of caring for patients with blinding diseases every day. Many of these patients have isolated retinal pathologies such as age-related macular degeneration or rhegmatogenous retinal detachment. Many of our patients, however, are affected by retinal pathologies that are merely a manifestation of a systemic disease, such as diabetic retinopathy. Indeed, because of the high metabolic demand of the retina and because the retinal and choroidal circulations receive proportionally higher blood flow volumes per unit area than many other tissues, these vascular beds are often affected by systemic diseases earlier than other parts of the body. his left eye (Figure 1) with normal filling in his right eye. Following a discussion of management options, the patient chose to be evaluated immediately in a local emergency room. There, the patient was found to have >90% proximal, left internal carotid artery narrowing (Figure 2). The patient underwent urgent vascular surgery with left internal carotid endarterectomy (CEA) and implantation of a xenograft patch. The patient recovered rapidly and has remained asymptomatic. Four months following CEA, FA revealed significantly normalized vascular filling of his left retinal circulation (Figure 3). Case Presentation and Results An asymptomatic 55-year-old man with 20/20 visual acuity in both eyes was referred after presenting with a Hollenhorst plaque in the left eye during a routine ophthalmic examination. Fluorescein angiography (FA) revealed significantly delayed vascular filling in A Discussion Hollenhorst plaques were first described in 1961 by Robert Hollenhorst, MD, who aptly inferred their intraarterial location as indicative of embolic disease, classically B Figure 1. Fundus photograph left eye: Hollenhorst plaque (arrow) involving the inferior major retinal arteriole within the optic nerve head (A). Fluorescein angiograph showing delayed vascular filling of the retinal circulation (1 minute 51 seconds; B). 74 RETINA Today November/December 2013 MEDICAL RETINA FEATURE STORY A B C D Figure 2. CT angiogram: Right common, internal, and external carotid circulations are normal (A). Proximal left internal carotid artery has >90% lumen narrowing (arrow; B). Carotid ultrasound: Right common, internal, and external carotid arteries with normal vascular flow (C). Left internal carotid artery with significantly reduced vascular flow (D). A B C Figure 3. External photograph showing scar from carotid endarterectomy (A). Fundus photograph left eye: Stable Hollenhorst plaque (arrow; B). Fluorescein angiograph showing improved vascular filling of the retinal circulation with complete filling at 52 seconds (C). related to carotid arterial disease.1,2 Does the presence of a Hollenhorst plaque, whether symptomatic or asymptomatic, necessitate emergent evaluation for an embolic source? Certainly the answer depends on the specific circumstances of each patient. In the case of a symptomatic Hollenhorst plaque, urgent embolic evaluation including carotid ultrasound analysis is probably indicated, as approximately 25% may have substantial carotid artery stenosis.3 In patients with moderate to severe carotid artery stenosis, CEA can substantially reduce the risk of subsequent hemispheric cere- bral vascular accident (CVA). For example, in the North American Symptomatic Carotid Endarterectomy Trial (NASCET), patients with transient monocular visual loss (TMVL), transient ischemic attack (TIA), or nondisabling stroke and severe carotid stenosis (70% to 99%) were randomized to CEA or medical management; CEA led to a 2-year ipsilateral stroke rate of 9% vs 26% for patients undergoing medical management alone (P = .001).4 Approximately 75% of Hollenhorst plaques seen in ophthalmic practice are asymptomatic.3 Many studies have considered the relationship between asymptomatic November/December 2013 RETINA Today 75 MEDICAL RETINA FEATURE STORY plaques and the presence of significant carotid artery stenosis; significant ipsilateral carotid stenosis can be identified in 5.6% to 9% of such patients.3,5,6 According to a meta-analysis of 3 randomized controlled trials involving 5223 patients with asymptomatic moderate to severe stenosis, intervention with CEA may be indicated if the perioperative risk is low, as risk of subsequent stroke or mortality can be reduced substantially (relative risk = 0.69, favoring CEA).7 In the presence of a Hollenhorst plaque, one may consider auscultation of the ipsilateral carotid artery with a stethoscope. The presence of a carotid bruit may signify a higher risk of significant carotid artery stenosis and may help predict CVA, myocardial infarction, and death.3,8 Although some studies have identified value in prognostication, others have suggested that the results of carotid artery auscultation have neither high specificity nor sensitivity, particularly in asymptomatic patients.9 Evidence suggests that both symptomatic and asymptomatic Hollenhorst plaques may be markers for significant carotid artery disease, and their presence indicates risk factor analysis and carotid ultrasonography. If a patient chooses to defer evaluation until a later time, one may consider documenting that urgent referral was offered. Just as the tip of an iceberg is only a small part of the whole underneath the ocean’s surface, a Hollenhorst plaque may be but a marker for a more serious systemic situation. n Charles C. Wykoff, MD, PhD, is a member of the Retina Consultants of Houston and a Clinical Assistant Professor of Ophthalmology at Weill Cornell Medical College, Methodist Hospital, Houston, TX. He is a member of the Retina Today Editorial Board. Dr. Wykoff states that he has no financial interest in any products mentioned in this article. He can be reached at [email protected]. Daniel E. Croft, BA, is a senior research assistant with the Retina Consultants of Houston. 1. Hollenhorst RW. Ocular manifestations of insufficiency or thrombosis of the internal carotid artery. Trans Am Ophthalmol Soc. 1958;56:474-506. 2. Hollenhorst RW. Significance of bright plaques in the retinal arterioles. Trans Am Ophthalmol Soc.1961;59:252-273. 3. Bakri SJ, Luqman A, Pathik B, et al. Is carotid ultrasound necessary in the clinical evaluation of the asymptomatic hollenhorst plaque? (An american ophthalmological society thesis). Trans Am Ophthalmol Soc. 2013;111:17-23. 4. North American Symptomatic Carotid Endarterectomy Trial Collaborators. Beneficial effect of carotid endarterectomy in symptomatic patients with high-grade carotid stenosis. N Engl J Med. 1991;325(7):445-453. 5. Bull DA, Fante RG, Hunter GC, et al. Correlation of ophthalmic findings with carotid artery stenosis. J Cardiovasc Surg (Torino). 1992;33(4):401-406. 6. Wakefield MC, O’Donnell SD, Goff JM, Jr. Re-evaluation of carotid duplex for visual complaints: who really needs to be studied? Ann Vasc Surg. 2003;17(6):635-640. 7. Chambers BR, Donnan GA. Carotid endarterectomy for asymptomatic carotid stenosis. Cochrane Database Syst Rev. 2005(4):CD001923. 8. Pickett CA, Jackson JL, Hemann BA, et al. Carotid bruits as a prognostic indicator of cardiovascular death and myocardial infarction: a meta-analysis. Lancet. 2008;371(9624):1587-1594. 9. Cournot M, Boccalon H, Cambou JP, et al. Accuracy of the screening physical examination to identify subclinical atherosclerosis and peripheral arterial disease in asymptomatic subjects. J Vasc Surg. 2007;46(6):1215-1221.