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Original Investigation |

Outer Retinal Corrugations in Age-Related Macular Degeneration

Sotaro Ooto, MD1,2; Sritatath Vongkulsiri, MD1,2; Taku Sato, MD1,2; Mihoko Suzuki, MD1,2; Christine A. Curcio, PhD3; Richard F. Spaide, MD1,2
[+] Author Affiliations
1LuEsther T. Mertz Retinal Research Center, New York, New York
2Vitreous Retina Macula Consultants of New York, New York
3EyeSight Foundation of Alabama Vision Research Laboratories, Department of Ophthalmology, University of Alabama School of Medicine, Birmingham
JAMA Ophthalmol. 2014;132(7):806-813. doi:10.1001/jamaophthalmol.2014.1871.
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Importance  Optical coherence tomography (OCT) abnormalities of age-related macular degeneration (AMD) have not been fully characterized because of the complex morphology and a lack of correlative histologic studies. Expansion of our ability to interpret increasing attributes brings us closer to the goal of in vivo histologic analysis of the eye by OCT.

Objective  To describe a new outer retinal finding of AMD using spectral-domain (SD) OCT and suggest histopathologic correlates.

Design, Setting, and Participants  Twenty-five eyes of 16 patients with AMD with severe atrophy due to either choroidal neovascularization (CNV) or geographic atrophy (GA) and 53 donor eyes of 53 patients with late AMD were included. Imaging studies were conducted at a referral retinal practice and histopathology was done at a university research laboratory.

Exposures  Findings in the outer retina were evaluated in SD-OCT images in eyes with atrophy of the retinal pigment epithelium (RPE) and compared with histopathologic findings in eyes with GA or CNV that also showed loss of the RPE.

Main Outcomes and Measures  Spectral-domain OCT and histologic characteristics of the outer retina.

Results  The mean (SD) age of the 16 patients was 82.7 (7.9) years. Twenty eyes had CNV and 5 eyes had GA. The mean best-corrected visual acuity was 0.800 logMAR (interquartile range, 0.350-1.000 logMAR), a Snellen equivalent of 20/126. A curvilinear hyperreflective density was identified above the Bruch membrane line within the atrophic area in the SD-OCT images. At the internal border, the material was contiguous with the outer portion of the RPE band. Below the material was a relatively hyporeflective space. The material was thrown into folds in cases with atrophy following CNV or was seen as a sheet with numerous bumps in eyes with GA. Review of histopathologic findings of eyes with advanced GA and CNV revealed a rippled layer of basal laminar deposits in an area of RPE atrophy that was located in the same level as the curvilinear line seen in the OCT images.

Conclusions and Relevance  We have described a new entity, termed outer retinal corrugations, which may correspond to histological findings of basal laminar deposits, extracellular deposits that persist in eyes with late AMD. Observation of this undulating band does not necessarily mean there is exudation or leakage; as a consequence, these patients do not need treatment based on this solitary finding.

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Figure 1.
Outer Retinal Corrugations Detected by Spectral-Domain Optical Coherence Tomography in an Eye With Choroidal Neovascularization

Images of the left eye of a 71-year-old woman with age-related macular degeneration and best-corrected visual acuity of 20/100. A, Fundus photograph shows severe atrophy due to choroidal neovascularization. B, Serial B-scan images (distance between each scan, 30 μm). B-scan images show a continuous curvilinear hyperreflective material (arrowheads) above the tissue, suggesting fibrovascular scar due to choroidal neovascularization. Below the material there is a hyporeflective space. C, A composite surface volume rendering image shows a sheet of the material thrown into folds.

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Figure 2.
Outer Retinal Corrugations Detected by Spectral-Domain Optical Coherence Tomography in an Eye With Geographic Atrophy

Images of the right eye of a 69-year-old man with age-related macular degeneration and best-corrected visual acuity of 20/200. A, Fundus photograph shows retinal pigment epithelium (RPE) atrophy. B, Serial B-scan images (distance between each scan, 12 μm). B-scan images show a curvilinear hyperreflective material (arrowheads) above the Bruch membrane. At its internal border, the material is contiguous with the outer portion of the retinal pigment epithelium line. There are drusen leading to the hollow drusen appearance; some of the drusen still have content, while others not so much. C, A composite surface volume rendering image shows a sheet with numerous bumps of the material.

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Figure 3.
Histopathology of a Donor Eye of a 90-Year-Old Woman With Geographic Atrophy Associated With Age-Related Macular Degeneration

Ten-µm-thick cryosections were stained with periodic acid–Schiff hematoxylin, as described.8 A, A section through the area with retinal pigment epithelium (RPE) atrophy. The boxes indicate areas shown in panels B and C. The RPE atrophy is seen between the blue arrowheads. The optic nerve head is at the left. B and C, High-magnification images revealing a rippled layer of pink-stained persistent basal laminar deposit (BLamD) (arrowheads) in an area of RPE atrophy. The Bruch membrane is straight and also stained pink. Scale bar applies to B and C. B, Calcific nodules and isolated cells are found external to undulating, persistent BLamD (arrowheads). The outer nuclear layer is absent. C, The yellow arrowhead indicates intact, although dysmorphic, RPE with underlying BLamD. This deposit continues into the area of RPE loss as an undulating layer (black arrowheads). Note that BLamDs persist in spite of the (near) absence of outer nuclear layer, photoreceptor layer, and RPE. A few cone nuclei overlie the deposit near the right black arrowhead.

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Figure 4.
High-Resolution Histology Sections Showing Outer Retinal Corrugation in Advanced Age-Related Macular Degeneration

Tissues were postfixed with osmium tannic acid paraphenylenediamine, and 0.8-µm-thick sections were stained with toluidine blue. A, Neovascular age-related macular degeneration in an 81-year-old woman. Highly corrugated layer of thick, persistent basal laminar deposit (asterisks) between a fibrocellular scar in the subretinal space (above) and sub–retinal pigment epithelium (RPE) space (below). The dotted line separates sublayers of late (above) and early (below) forms of basal laminar deposit. In the corrugation concavities, and overlying fibrovascular scar, are a choroidal neovascularization (CNV) vein with erythrocytes and a multicellular aggregate with spherical melanosomes (red arrowhead). Internal to the corrugations and entombed by scar are RPE cells with green-stained lipofuscin (yellow arrowhead). The Bruch membrane is indicated by black arrowheads. Choriocapillaris endothelium has been replaced by presumed macrophages. B, Neovascular age-related macular degeneration in an 88-year-old man. Highly corrugated and discontinuous layer of thick persistent basal laminar deposit separates scars in the subretinal and sub-RPE compartments. Hyaline and fibrocellular scars are visible. Entombed RPE is present internal to a valley between corrugations. The blue arrowhead indicates a small arteriole.

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Figure 5.
Proposal as to How the Basal Laminar Deposit (BLamD) Can Look Folded and Possible Mechanisms of Formation of the Clinical Picture

A, The left side of the image shows intact retinal pigment epithelium with BLamD (pink material). There is basal linear deposit forming a druse. The right side shows an area where geographic atrophy has developed, with loss of the retinal pigment epithelium and the basal linear deposit. The BLamD remains. B, A case of choroidal neovascularization shows the retinal pigment epithelium and neovascular tissue. C, Some cases of choroidal neovascularization regress, leaving minimal scarring but have extensive tissue loss. The remaining BLamD, which was formed when the choroidal neovascularization formed, has more surface area than the available space. Therefore, the BLamD is thrown into folds.

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