Association of reticular pseudodrusen and early onset drusen

Abstract

Purpose. To report an association between reticular pseudodrusen, located above the retinal pigment epithelium (RPE), and Early Onset Drusen (EOD) as described using Spectral-Domain Optical Coherence Tomography (SD-OCT). Methods. Eight patients (16 eyes) with EOD were examined. EOD were classified into three entities called Large Colloid Drusen (LCD), Malattia Leventinese (ML), and Cuticular Drusen (CD). Best-corrected visual acuity, fundus examination, color fundus photographs, fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), and SD-OCT were performed in all study patients. Results. Four patients had LCD, 2 had ML, and 2 had CD. Reticular pseudodrusen were observed with SD-OCT in all study patients; all these patients had hyperreflective lesions above and below the RPE. Conclusion. Early Onset Drusen appear to be associated with reticular pseudodrusen. SD-OCT is helpful in distinguishing the location of the deposits that are above and below the RPE in EOD. Further studies are needed to understand the role of reticular pseudodrusen in the pathophysiology of EOD.

Figure 1

A forty-year-old woman affected by Large ColloId Drusen in both eyes. (a) Multicolor images with large yellowish drusen in the macular area. (b) The larger drusen are hyperautofluorescent in FAF. (c) In the late phase of ICG angiography, LCD are hypofluorescent with a dark centre surrounded by a more fluorescent halo, bordered by a thin hypofluorescent ring (“donut effect”). (d) The colloid drusen are located under the RPE on B-scan OCT.

Figure 2

A thirty-five-year-old woman affected by Malattia Leventinese in both eyes. (a) Color fundus photographs with large and small drusen associated with pigmentary changes. The larger drusen are confluent in the macular area while the smaller drusen are located temporally. (b) The larger drusen are hyperautofluorescent. (c) Large drusen are hyperfluorescent with a hypofluorescent halo in the late phase of ICG angiography. (d) The large drusen are located under the RPE on B-scan OCT.

Figure 3

A forty-year-old woman affected by Cuticular Drusen in both eyes. (a) Color fundus photographs with small drusen randomly scattered in the macula and in the middle periphery of the retina. (b), (c) In the late phase of FA and ICG, the drusen are hyperfluorescent with a typical “stars-in-the-sky” appearance. (d) The small drusen are located under the RPE on B-scan OCT.

Figure 4

Patient affected by Large Colloid Drusen: SD-OCT shows two different types of deposits. Colloid drusen are convex and observed under the RPE, like soft drusen, with variable reflectivity. Bruch's membrane is visible (white arrowhead). The second type of deposit which is smaller, triangular, and hyperreflective is located above the RPE, like reticular pseudodrusen (white arrow). An abrupt interruption of both the inner band of RPE and IS/OS interface appears at the border of the hyperreflective lesion. Hyporeflective cysts are observed in the inner retina without choroidal neovascularization (black arrow).

Figure 5

Patient with Large Colloid Drusen in both eyes. (a), (c) Color fundus photographs show large drusen in the macular area and in the periphery of the retina. (b), (d) SD-OCT shows hyperreflective deposits located above the RPE (white arrows). An abrupt interruption of both the inner band of RPE and IS/OS interface appears at the border of these hyperreflective lesions. Hyporeflective cysts are observed in the inner retina along with an epiretinal membrane (black arrow).

Figure 6

Patient affected by LCD in both eyes. (a), (c) Multicolor images show large drusen located in the macular area and the periphery of the retina. (b), (d) SD-OCT shows hyperreflective deposits with a black center above the RPE and with interruption of both the inner band of the RPE and the IS/OS interface (white arrows).

Figure 7

Patient affected by Malattia Leventinese in both eyes. (a), (b) Color fundus photographs show drusen associated with pigmentary changes. (c), (d) SD-OCT shows the larger drusen as diffuse deposition of hyperreflective material under the RPE (white arrowhead). Above the RPE elevation, a hyperreflective haze is observed in the external nuclear layer (black arrow). The smaller drusen are seen above the RPE (white arrows).

Figure 8

Patient affected by Malattia Leventinese in both eyes. (a), (b) SD-OCT shows the larger drusen as focal deposition of hyperreflective material under the RPE (white arrowhead). Another kind of deposit is seen above the RPE (white arrow). A hyperreflective haze is also seen located in the external nuclear layer close to plexiform layer (black arrow).

Figure 9

Patient affected by Cuticular Drusen in both eyes. SD-OCT shows some Cuticular Drusen as small RPE elevations (white arrowhead) and others deposits as “sawtooth” RPE elevations above the RPE (white arrow).