Atypical retinal pigment epithelial defects with retained photoreceptor layers: a so far disregarded finding in age related macular degeneration

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Abstract

Background

To report patients with age-related macular degeneration and atypical central retinal pigment epithelium (RPE) defects not attributable to geographic atrophy (GA) or RPE-tears with overlying preserved photoreceptor layers.

Methods

Multimodal imaging case-series evaluating the course of atypical RPE- defects in patients with AMD using Color fundus images, Optical coherence tomography (OCT), OCT-Angiography, fundus autofluorescence (FAF) and fluorescein-angiography (FA).

Results

Ten patients were identified. Three patients had a prior RPE-rip and were excluded. Seven patients with a mean follow-up period of 47 ± 38 months after the occurrence of the RPE-defect were included (age range 71–87 years). Mean distance Best corrected visual acuity (BCVA) at initial presentation was 0.36 ± 0.29logMAR and at last follow-up visit 0.51 ± 0.43logMAR. Patients presented with clinically apparent GA on funduscopy and FAF, but preserved photoreceptor layers on optical coherence tomography (OCT). On FA there was early hyperfluorescence and late pooling visible. Over time, migration of RPE/drusenoid material right above the Bruch’s membrane with concomitant decrease of hypoautofluorescence was detectable in 4 cases. An enlargement of the RPE-defect was apparent in the remaining 3 cases. The majority ( n = 4) showed a drusenoid pigment epithelium detachment (PED) preceding the lesion.

Conclusions

Beside GA and characteristic RPE-tears, another atypical form of RPE-defect with overlying preserved photoreceptor layers are found in AMD. This so far disregarded subgroup of patients present with reasonable visual function and long-term survival of photoreceptors layers. Repair mechanisms such as ingrowth of RPE/drusenoid material and persistent subretinal fluid (SRF), but also a RPE-independent visual cycle for cone photopigment within the neurosensory retina may contribute to their favorable course.

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Most cited references 29

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Photoreceptor loss in age-related macular degeneration.

N Medeiros, David C Millican, B. Curcio (1996)

The authors showed previously that parafoveal rods, but not cones, decrease during the course of adulthood in donor eyes that were screened to exclude the grossly visible macular drusen and pigmentary disturbances typical of age-related macular degeneration (AMD). Because AMD begins in the parafovea, this selective loss of rods actually may be subclinical AMD not yet visible in the fundus. If so, AMD must have a predilection for rods over cones. The authors tested this hypothesis by determining the relative numbers of cones and rods in donor eyes with mid-to late-stage AMD and in age-matched controls. Thirteen eyes (from seven donors) with grossly visible macular drusen and pigmentary disturbances were either wholemounted for photoreceptor counts or sectioned through the fovea for histopathology and carbonic anhydrase histochemistry to label red-green cones. Eyes were assigned to AMD or control groups on the basis of histopathology and clinical history. Five nonexudative AMD (NE-AMD) eyes from three donors showed sparing of foveal cones and loss of rods and cones in the parafovea. In two donors, rod loss exceeded cone loss at most parafoveal locations, and in one donor, rod density was normal and cone density was reduced. In eight exudative AMD (EX-AMD) eyes from five donors, photoreceptors surviving along the margins of and overlying disciform scars were largely cones. Photoreceptors are lost in NE-AMD as well as in the more severe exudative form, consistent with functional and clinical studies. The authors propose that rods die in older eyes without evidence of overt retinal pigment epithelial disease. In persons susceptible to AMD, the retinal pigment epithelium becomes dysfunctional. Secondarily, rod loss continues and cones begin to degenerate. Eventually, only degenerate cones remain; ultimately, all photoreceptors may disappear.

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Aging of the human photoreceptor mosaic: evidence for selective vulnerability of rods in central retina.

C A Curcio, C L Millican, K A Allen … (1993)

Because previous studies suggested degeneration and loss of photoreceptors in aged human retina, the spatial density of cones and rods subserving the central 43 degrees of vision as a function of age was determined. Cones and rods were counted in 27 whole mounted retinas from donors aged 27 to 90 years with macroscopically normal fundi. Photoreceptor topography was analyzed with new graphic and statistical techniques. Changes in cone density throughout this age span showed no consistent relationship to age or retinal location, and the total number of foveal cones was remarkably stable. In contrast, rod density decreased by 30%, beginning inferior to the fovea in midlife and culminating in an annulus of deepest loss at 0.5 to 3 mm eccentricity by the ninth decade. Space vacated by dying rods was filled in by larger rod inner segments, resulting in a similar rod coverage at all ages. At the temporal equator, cone density declined by 23%, but rods were stable throughout adulthood. The stability of both rod coverage and rhodopsin content despite decreasing cell number suggests plasticity of the adult rod system and that age-related declines in scotopic sensitivity may be due to postreceptoral factors. There is no evidence for the massive loss of foveal cones required to explain even modest decrements in acuity, consistent with evidence that visual deficits at high photopic levels may be largely due to optical factors. Why the rods of central retina, which share a common support system and light exposure with the neighboring cones, are preferentially vulnerable to aging remains to be determined.

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Photoreceptor degeneration and dysfunction in aging and age-related maculopathy.

Christine A. Curcio, Cynthia Owsley, Catherine Jackson (2002)

The relative rate of rod and cone degeneration is a fundamental characteristic of any disorder affecting photoreceptors, including aging and age-related maculopathy (ARM). The human macula consists of a small cone-dominated fovea surrounded by a rod-dominated parafovea. In aging and early ARM, rods degenerate before cones, a decline in scotopic (rod-mediated) sensitivity is more prominent than a decline in photopic (cone-mediated) sensitivity, and the time course of dark adaptation of rods slows dramatically. The topography of rod dysfunction and loss in early ARM matches the location of pathology in the retinal pigment epithelium (RPE)/Bruch's membrane complex visible in the ocular fundus. Rod dysfunction and loss in aging and ARM may be due to retinoid deficiency at the level of the photoreceptors cause by impaired retinoid translocation across the RPE/Bruch's membrane complex, a hypothesis deserving of further investigation.

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Author and article information

Contributors

Helena Giannakaki-Zimmermann: helena.zimmermann@insel.ch

Giuseppe Querques: giuseppe.querques@hotmail.it

Inger Christine Munch: imu@regionsjaelland.dk

Daraius Shroff: daraiuss@outlook.com

David Sarraf: dsarraf@ucla.edu

Xuejing Chen: xuejing@gmail.com

Eduardo Cunha-Souza: ecsoftalmo@gmail.com

Sarah Mrejen: sarahmrejen.uretsky@gmail.com

Vittorio Capuano: vittorio.capuano@gmail.com

Murilo W. Rodrigues: murilowrj@yahoo.com.br

Charu Gupta: charumittalgupta@gmail.com

Andreas Ebneter: andreas.ebneter@insel.ch

Martin S. Zinkernagel: martin.zinkernagel@insel.ch

Marion R. Munk: +41 31 632 25 01 , marion_munk@hotmail.com

Journal

Journal ID (nlm-ta): BMC Ophthalmol

Journal ID (iso-abbrev): BMC Ophthalmol

Title: BMC Ophthalmology

Publisher: BioMed Central (London )

ISSN (Electronic): 1471-2415

Publication date (Electronic): 15 May 2017

Publication date PMC-release: 15 May 2017

Publication date Collection: 2017

Volume: 17

Electronic Location Identifier: 67

Affiliations

[1 ]Department of Ophthalmology and Department of Clinical Research, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland

[2 ]Bern Photographic Reading Center, Inselspital, University Clinic Bern, Bern, Switzerland

[3 ]Department of Ophthalmology, University Vita-Salute, IRCCS Ospedale San Raffaele, Milan, Italy

[4 ]Department of Ophthalmology, University Paris Est, CHI, Creteil, France

[5 ]ISNI 0000 0001 0674 042X, GRID grid.5254.6, Department of Ophthalmology, Zealand University Hospital, , University of Copenhagen, ; Copenhagen, Denmark

[6 ]Shroff Eye Center, New Delhi, India

[7 ]ISNI 0000 0000 9632 6718, GRID grid.19006.3e, Jules Stein Eye Institute, , UCLA, ; Los Angeles, CA USA

[8 ]Greater LA VA Healthcare Center, Los Angeles, CA USA

[9 ]ISNI 0000 0004 1937 0722, GRID grid.11899.38, , University of Sao Paulo, ; Sao Paulo, Brazil

[10 ]ISNI 0000 0004 1937 0722, GRID grid.11899.38, , Faculdade de Medicina de Ribeirão Preto, ; Ribeirão Preto, Brazil

[11 ]Vitreous Retina Macula Consultants of New York, New York, USA

[12 ]ISNI 0000 0001 2299 3507, GRID grid.16753.36, Department of Ophthalmology, , Northwestern University, Feinberg School of Medicine, ; Chicago, IL USA

Article

Publisher ID: 452

DOI: 10.1186/s12886-017-0452-0

PMC ID: 5433076

PubMed ID: 28506260

SO-VID: fd9f1b1c-a177-48ef-aade-7976579a879f

License:

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

History

Date received : 23 February 2017

Date accepted : 22 April 2017

Custom metadata

ScienceOpen disciplines: Ophthalmology & Optometry

Keywords: rpe tear,geographic atrophy,age-related macular degeneration,rpe-aperture,photoreceptor

Data availability:

ScienceOpen disciplines: Ophthalmology & Optometry

Keywords: rpe tear, geographic atrophy, age-related macular degeneration, rpe-aperture, photoreceptor

Atypical retinal pigment epithelial defects with retained photoreceptor layers: a so far disregarded finding in age related macular degeneration

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Abstract

Background

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Conclusions

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Karger: Ophthalmology

Most cited references 29

Photoreceptor loss in age-related macular degeneration.

Aging of the human photoreceptor mosaic: evidence for selective vulnerability of rods in central retina.

Photoreceptor degeneration and dysfunction in aging and age-related maculopathy.

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