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SUPPLEMENT TO APRIL 2012 www.revoptom.com The Clinical Utility of Fundus Autofluorescence Imaging By Jeffry D. Gerson, O.D., F.A.A.O. The Role of the Vitreous in Retinal Disease By Carolyn E. Majcher, O.D., Andrew S. Gurwood, O.D., F.A.A.O., Dipl., and Julie K. Hutchinson, O.D. Earn 1 CE Credit: A Case of Late-Onset Leber’s Hereditary Optic Neuropathy By Carol Aune, B.S., O.D., and James Walters Ph.D., O.D. A Case of Suspected Retinal Melanocytoma By W. Lee Ball, O.D., F.A.A.O. 001_ro0412 RetinaGuide.indd 1 4/12/12 9:41 AM FUNDUS AUTOFLUORESCENCE The Clinical Utility of Fundus Autofluorescence Imaging By Jeffry D. Gerson, O.D., F.A.A.O. THE HISTORIC STANDARD for the doc- umentation of posterior segment findings has been fundus photog- raphy. Generally, the need for fun- dus photography is substantiated by the detection of a clinical find- ing that warrants documentation for future reference. However, we are now learning that there is more to be seen than what conventional fundus photography can capture. Although there have been refer- ences to fundus autofluorescence (FAF) in the medical literature for more than 35 years, this approach is only now becoming a more mainstream imaging technique.1 Not only can FAF be an ideal tool for documentation, but also it will Normal fundus autofluorescence (FAF) image of a patient’s posterior pole. often serve as a diagnostic modal- ity because the device can elucidate hypo-FAF secondary to xantho- dystrophies and Stargardt’s many conditions that are not vis- phyll pigment absorbing the signal. disease, to age-related macular ible through traditional means of Areas of hyper-autofluorescence degeneration and other condi- examination or other technologies. or hypo-autofluorescence may be tions that involve impaired RPE Additionally, FAF can be used to indicative of an abnormality in cell function. Lipofuscin builds detect structural abnormalities and RPE activity that is either active up through incomplete degrada- predict functional deficits.2,3 These or has already occurred. These tion of photoreceptor outer seg- abilities go beyond what optical changes in fluorescence are visu- ments and incomplete release of coherence tomography (OCT), ally reminiscent of those found in degraded material.6 fluorescein angiography (FA), elec- FA (although they are caused by FAF, however, is not an “all- trophysiology and other imaging different fundamentals). More spe- telling” diagnostic modality. In technologies can provide. cifically, while FA reveals vascular some cases of blinding pathol- leakage, FAF actually shows meta- ogy, the FAF appearance may How does FAF Work? bolic activity. be perfectly normal—even if the FAF provides an objective, non- From a technical standpoint, RPE-photoreceptor complex is invasive measurement of the reti- FAF permits topographic mapping unaffected. nal pigment epithelium’s (RPE’s) of lipofuscin distribution in the metabolic activity. A measurement RPE as well as other fluorophores Clinical Applications of FAF of FAF is decreased by a loss of that occur in the outer retina and There are several areas of prac- photoreceptor cell integrity and is the sub-neurosensory space.4 This tical interest where FAF has the increased by abnormal RPE behav- is important, because accumulation potential to change how we diag- ior and/or function. of lipofuscin is a significant marker nose and/or treat patients: Autofluorescence of the retina for aging of metabolically active • Early macular disease detec- and macula typically will appear cells, particularly those comprising tion. Understanding the extent of a uniform gray color, with darker the RPE.5 RPE defects of the posterior pole vasculature due to blood absorp- Excess lipofuscin has been as well as other macular diseases tion and a darker optic nerve head indicted in a variety of retinal may be accomplished via periph- due to a lack of RPE. In the fovea, disorders—from inherited dis- eral FAF imaging, as evidenced there is reduced FAF, or mild eases, such as macular pattern in multiple clinical trials.7 In fact, APRIL 2012 REVIEW OF OPTOMETRY 3 001_ro0412 RetinaGuide.indd 3 4/12/12 9:41 AM FUNDUS AUTOFLUORESCENCE This patient presented with Stargardt’s macular dystrophy (FAF left, color fundus right). The FAF image illustrates the extent of macular disease more clearly. peripheral changes may pre-date an early diagnosis, but rather the it may help educate a patient on macular changes in AMD, central detection of an already advanced the chronic and diffuse nature of serous chorioretinopathy (CSCR) disease state.9 this condition. and many other diseases thought We also know that macular tox- • AMD and GA. The extent and to be specific to the macula. Using icity is more common than once progression of AMD can be closely peripheral FAF as a screening believed, partially due to previous monitored with FAF. Further, FAF tool for such macular conditions techniques not being accurate may potentially indicate which may help to predict their future enough to detect mild pathologic patients are at an increased risk onset and permit early initiation of changes. The updated guidelines for the development of geographic disease-modifying treatments. For call for objective testing, which is to atrophy (GA).12 FAF also may help instance, if peripheral changes are include FAF, spectral domain OCT evaluate the potential effectiveness indicative of early macular degen- and/or multi-focal electroretino- of various treatment modalities— eration, then lifestyle modification gram testing.8 This updated screen- especially if the patient has GA. can be instituted to prevent visu- ing protocol is particularly signifi- Currently, several pharmaceuti- ally significant changes associated cant because of the sheer number of cal companies are using FAF to with AMD. patients who use Plaquenil. evaluate the treatment efficacy of • Plaquenil retinopathy. In • CSCR. When we see patients several developmental drugs for 2011, the screening guidelines for with CSCR, we often wonder if GA (oral, injectable and topical Plaquenil (hydroxychloroquine, it is a first occurrence or a recur- medications). This is an important Sanofi-Aventis) toxicity were rence, how much global RPE dam- consideration, because several updated in an article published age there is, and what their visual studies have shown that changes in Ophthalmology.8 Screening for outcome will be. FAF can help us documented on FAF imaging often Plaquenil retinopathy is more answer these questions, and even precede GA or its expansion.13,14 accurately performed through FAF address the concern of whether Areas of new GA expansion initial- than conventional funduscopic the patient has CSCR.10 If the ly may appear bright on FAF imag- observation.9 Historically, clinical individual has CSCR, FAF may be ing, and then subsequently exhibit observation and visual fields were used to help guide our follow-up a dark or “dead” appearance on thought to be adequate for timely schedule and/or referral for treat- various areas of the RPE. diagnosis of macular toxicity sec- ment. Studies have shown that FAF Although we believe AMD to be ondary to Plaquenil use. However, appearance can be a predictor of a macular disease that only occurs we now know that these screen- visual outcome and function in in the posterior pole, results from ing techniques will not facilitate patients with CSCR.11 Additionally, the Reykjavik Eye Study show that 4 REVIEW OF OPTOMETRY APRIL 2012 001_ro0412 RetinaGuide.indd 4 4/12/12 9:41 AM peripheral and posterior pole pathologies regularly coexist and are often signs of each other.15 Additionally, there are advantages to having FAF documentation for patients with neovascular AMD, because the technology may help predict visual outcome—especially in patients who have developed choroidal neovascularization with- in six months or less of diagnosis.16 We diagnosed this patient with retinitis pigmentosa (O.D. left, O.S. right). A Glance at Current FAF Devices Fortunately, it is now possible widefield imaging platform in a on a referral basis for colleagues. He is (and practical) to include this tabletop design that offer unprec- a fellow of both the American Academy technology in your practice. The edented views of peripheral chang- of Optometry and the Optometric two primary companies that read- es.17 Additionally, this device exhib- Retina Society. ily offer devices with FAF imag- its strong sensitivity in the detec- 1. Miller SA. Fluorescence in Best’s Viteliform dystrophy, lipofuscin ing capabilities are Heidelberg tion of macular-specific diseases, and fundus flaimaculatus. Br J Ophthalmol. 1978 Apr;62(4):256- Engineering and Optos. providing results that are in close 60. 2. Querques L, Querques G, Forte R, Souied EH. Microperimetric • Spectralis (Heidelberg agreement to those obtained via Correlations of Autofluorescence and Optical Coherence 17 Tomography Imaging in Dry Age-Related Macular Degeneration. Engineering) offers BluePeak blue traditional fundus photography. Am J Ophthalmol. 2012 Feb 7. [Epub ahead of print] laser autofluorescence technology 3. Seidensticker F, Neubauer AS, Wasfy T, et al. Wide-field fundus autofluorescence corresponds to visual fields in chorioretinitis that is being used in several clinical Whether your practice currently patients. Clin Ophthalmol. 2011;5:1667-71. trials. In particular, the company has access to FAF imaging, this tech- 4. Schmitz-Valckenberg S, Holz FG, Bird AC, Spaide RF. Fundus autofluorescence imaging: review and perspectives. Retina. 2008 is using BluePeak to evaluate the nology soon will become even more Mar;28(3):385-409. 5. Terman A, Brunk UT. Oxidative stress, accumulation of bio- potential efficacy of drugs that pre- essential to the proper care and logical garbage, and aging. Antioxid Redox Signal. 2006 Jan- vent the onset or enlargement of will be able to provide our patients Feb;8(1-2):197-204 6. Wing GL, Blanchard GC, Weiter JJ.