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

BMC Ophthalmology

Research Article

Andreas Ebneter¹  Martin S. Zinkernagel²  Helena Giannakaki-Zimmermann²  Marion R. Munk³  Vittorio Capuano⁴  Giuseppe Querques⁵  Inger Christine Munch⁶  Xuejing Chen⁷  David Sarraf⁸  Charu Gupta⁹  Daraius Shroff⁹  Murilo W. Rodrigues^1,10  Eduardo Cunha-Souza^1,11  Sarah Mrejen^1,12 

[1] Department of Ophthalmology and Department of Clinical Research, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland;Department of Ophthalmology and Department of Clinical Research, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland;Bern Photographic Reading Center, Inselspital, University Clinic Bern, Bern, Switzerland;Department of Ophthalmology and Department of Clinical Research, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland;Bern Photographic Reading Center, Inselspital, University Clinic Bern, Bern, Switzerland;Department of Ophthalmology, Northwestern University, Feinberg School of Medicine, Chicago, IL, USA;Department of Ophthalmology, University Vita-Salute, IRCCS Ospedale San Raffaele, Milan, Italy;Department of Ophthalmology, University Vita-Salute, IRCCS Ospedale San Raffaele, Milan, Italy;Department of Ophthalmology, University Paris Est, CHI, Creteil, France;Department of Ophthalmology, Zealand University Hospital, University of Copenhagen, Copenhagen, Denmark;Jules Stein Eye Institute, UCLA, Los Angeles, CA, USA;Jules Stein Eye Institute, UCLA, Los Angeles, CA, USA;Greater LA VA Healthcare Center, Los Angeles, CA, USA;Shroff Eye Center, New Delhi, India;University of Sao Paulo, Sao Paulo, Brazil;University of Sao Paulo, Sao Paulo, Brazil;Faculdade de Medicina de Ribeirão Preto, Ribeirão Preto, Brazil;Vitreous Retina Macula Consultants of New York, New York, USA;

关键词: RPE tear;    Geographic atrophy;    Age-related macular degeneration;    RPE-aperture;    Photoreceptor;   

DOI  :  10.1186/s12886-017-0452-0

 received in 2017-02-23, accepted in 2017-04-22,  发布年份 2017

来源: Springer

【 摘 要 】

BackgroundTo 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.MethodsMultimodal 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).ResultsTen 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.ConclusionsBeside 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.

【 授权许可】

CC BY   
© The Author(s). 2017