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GA Case Compendium: Image Findings Suggesting Risk of Geographic Atrophy Progression
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Progression of geographic atrophy (GA) is variable from patient to patient. However, a great deal of research has been performed to identify imaging biomarkers that can be used in the clinic to aid in evaluation, assessment, and decision making. Herein I present two cases that highlight the role of various imaging modalities in assessing an eye with suspected GA secondary to age-related macular degeneration (AMD).
Case Presentation
Case 1
A 77-year-old Hispanic woman presents for a comprehensive eye examination. She reports that the center of her vision is “bad,” and it is hard for her to recognize faces, so she gets lost and can’t find her daughter at the store. She enjoys reading and watching TV and is having difficulty with these tasks. She is also struggling with getting her housework done. She states that she has lived in South Florida her entire life and loves to sunbathe. She also admits to cigarette smoking for more than 20 years.
The patient’s ocular history is unremarkable, although her medical history is positive for hypertension, obesity, and hypercholesterolemia. Her best corrected visual acuity measured 20/320 OD and 20/125 OS. Amsler grid testing showed bilateral metamorphopsia and missing lines OD (Figure 1). Slit-lamp biomicroscopy revealed age-appropriate lenticular changes (not shown).
The retinal exam showed advanced, nonexudative AMD bilaterally with subfoveal GA OD>OS (Figure 2). Fundus autofluorescence (FAF) showed hypoautofluorescence in the areas of GA with hyperautofluorescence at the junctional zone (Figure 3).
Assessing fluorescence at the junctional zone (area between normal and abnormal retina) is a valuable factor in determining the rate of GA progression. Lesion characteristics and junctional zone patterns fall into categories (Figure 4).1 The slowest growth is seen in eyes with no hyperfluorescence or a focal pattern of hyperfluorescence; fastest growth rates are seen with banded and diffuse patterns. The FAF pattern presented in this case is most consistent with banded hyperfluorescence, which is described as increased autofluorescence characterized by a continuous stippled band of increased FAF surrounding the entire atrophic area. A timely retina referral would be recommended in this case due to the likelihood of rapid progression in this patient. The hope would be to spare the fovea of the left eye from becoming more significantly involved.
Clinical Pearls
1. Patients with extrafoveal GA involvement are ideal candidates for therapeutic intervention, as the goal is to delay the progression of the lesion to the foveal center.
2. Use FAF to determine which patients are more likely to progress rapidly and use this when deciding whom to refer for intervention and when.
Case Presentation
Case 2
An 82-year-old White woman presents with complaints of decreased vision, specifically in situations with low light. She reports that her vision is gradually declining overall. She states that she is generally in good health, just some minor aches and pains from arthritis that is relieved with Advil. Her ocular history is positive for cataract extraction 5 years prior. She does not know her family history but thinks someone in her family went blind from a retinal disease.
Her BCVA measured 20/30 OD and OS, but the patient struggled throughout visual acuity testing. Fundus examination revealed large, confluent drusen throughout the vascular arcades and in the macular region bilaterally (Figure 5). There was also evidence of pigmentary changes in the macula OD. OCT and FAF were performed at this visit. FAF showed mottled hyperfluorescence of the drusenoid deposits (Figure 6).
Careful evaluation of the patient’s OCT highlight certain biomarkers that are directly correlated with advanced progression of the disease (Figures 7 and 8). Focal RPE pigmentary abnormalities and large drusenoid pigment epithelial detachments have been identified as risk factors for advanced progression of AMD. In advanced nonexudative AMD, these drusen collapse and lead to the development of GA. Careful assessment and follow-up of patients with identifiable risk factors is critical in the overall prognosis of the condition.
Clinical Pearls
- Use multimodal imaging to categorize the stage of AMD.
- Be familiar with biomarkers on multimodal imaging that are linked to rapid progression of the disease.
Conclusion
In most cases, it is possible to visualize nonexudative AMD clinically, as drusen, pigmentary abnormalities, and areas of hypopigmentation indicating loss of RPE (RPE atrophy) are evident on retinal examination or via color fundus photography. To fully assess these features and the severity of the disease, multimodal imaging including OCT and FAF are recommended. OCT is the gold standard for GA detection, and multimodal imaging, including near infrared imaging and FAF, may reveal subtle disease features, thus aiding in early detection and adding value in decision-making. Certain OCT findings indicate a high risk for GA development, notably subretinal drusenoid deposits, large soft drusen collapse, hyporeflective wedges, loss of the ellipsoid zone, and subsidence (sinking) of the inner nuclear layer and outer plexiform layer. If GA is suspected, hallmark OCT signs, including demarcated zones of RPE and photoreceptor loss with associated choroidal hypertransmission, will help support the diagnosis. On FAF, GA will appear as hypoautofluorescent areas, while any areas of hyperautofluorescence indicate cells at risk for death—likely to progress to GA.
1. Fleckenstein M, Mitchell P, Freund KB, et al. The progression of geographic atrophy secondary to age-related macular degeneration. Ophthalmology. 2018;125(3):369-390.
ABOUT THIS SERIES
Newly available treatment options for geographic atrophy (GA) have the potential to change the prognosis for long-term eye health. However, their newness also raises important practical questions, including about who should be referred and when. The Geographic Atrophy Clinical Case Compendium was developed, with guidance from Carolyn E. Majcher, OD, FAAO, FORS, and Julie Rodman, OD, MSc, FAAO, to demonstrate real-world patient encounters and the impact of treatment on the clinical course.
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