PMC2947750_01
Natural history of fundus changes in eyes with drusenoid pigment epithelial detachments (DPEDs) not progressing to advanced forms of age-related macular degeneration (AMD) by 5 years (n = 163). Prevalences of the presence of each fundus finding at year 0, 1, 2, 3, 4, 5 following baseline are plotted. A. Percentage of eyes with hyperpigmentary changes. B. Percentage of eyes with hypopigmentary changes. C. Percentage of eyes with calcified drusen. D. Percentage of eyes with geographic atrophy (GA) not involving the center of the fovea (i.e. non-central GA). E. The total drusen area in each eye was estimated and classified in one of the following categories: smaller than area of grading circle O2 (circle diameter = 660 microns), from area of circle O2 to 1/2 disk area (DA), from 1/2 DA to 1 DA, and greater than 1 DA. The proportions of eyes in each area category are shown for each year of follow-up. The total numbers of eyes for each time point are as follow: 163 eyes at year 0, 87 eyes at year 1, 156 eyes at year 2, 155 eyes at year 3, 149 eyes at year 4, 152 eyes at year 5).
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Although the overall prevalence of large drusen and hyperpigmentary changes are maintained throughout the period of observation (Fig. 2A, B), the exact location and configuration of these features in this example are seen to vary dynamically over time.
Case 1: Fundus changes occurring in the left eye of a 73 year-old woman with a drusenoid pigment epithelial detachment (DPED) at baseline. A. Fundus at year 0 (baseline): A centrally located DPED is seen with scattered large drusen in the inferior macula. B. At year 4, new calcified, refractile drusen emerge in the temporal macula (black arrow). Central DPED is decreased in height and area. C. At year 7, new hyperpigmentary changes appear at the fovea (black arrowhead). Large drusen in the inferior macula enlarge in size and coalesce to form a new DPED (white arrows). D. At year 8, DPEDs in both the central and inferior macula have disappeared. Calcified drusen are increased in number and an area of hypopigmentation has appeared in the nasal macula (white arrowhead). Central hyperpigmentary changes have also decreased as areas of hypopigmentation have increased. This natural history illustrates the dynamic changes in fundus features over a 8-year period, involving the appearance and disappearance of DPEDs, hyper- and hypopigmentary changes and calcified drusen, without overt progression to advanced forms of age-related macular degeneration.
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As seen in this example (Fig. 3), eyes containing DPEDs, even in the absence of progression, can demonstrate marked changes in the size and distribution of large drusen and DPEDs.
Case 2: Fundus changes occurring in the left eye of a 73-year old man with a drusenoid pigment epithelial detachment (DPED) that progressed to central geographic atrophy (CGA) during the course of the study. A. Fundus at year 0: A centrally located DPED is present with hyperpigmentary changes. B. At year 2, most of the DPED has disappeared and a new area of hypopigmentation has emerged (black arrow). C. At year 4, an additional area of hypopigmentation has arisen (black arrows). In the meantime, the hyperpigmentary changes seen earlier have decreased. D. At year 7, areas of geographic atrophy have emerged from earlier patches of hypopigmentation that have enlarged and coalesced.
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Case 2 is an example of an eye with DPED that progressed to CGA during follow-up (Fig. 4).
Case 3: Fundus changes occurring in the left eye of a 72 year-old woman with a drusenoid pigment epithelial detachment (DPED) that progressed to neovascular age-related macular degeneration (NV AMD) during follow-up. A. Fundus at year 0: A DPED with associated hyperpigmentary changes is located in the temporal perifovea (black arrowhead). B. At year 4, additional hyperpigmentary changes have emerged (black arrow) overlying the DPED. C. At year 9, new onset retinal edema, subretinal lipid exudate emerged in the region of the DPED. D. Three months after the onset of retinal edema, subretinal hemorrhage, indicative of NV AMD is seen in the same location.
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