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

Autor: Marion R. Munk, Eduardo Cunha-Souza, Vittorio Capuano, Giuseppe Querques, Inger Christine Munch, Sarah Mrejen, Xuejing Chen, Murilo Wendeborn Rodrigues, Martin S. Zinkernagel, David Sarraf, Andreas Ebneter, Helena Giannakaki-Zimmermann, Daraius Shroff, Charu Gupta
Přispěvatelé: Giannakaki-Zimmermann, H., Querques, G., Munch, I. C., Shroff, D., Sarraf, D., Chen, X., Cunha-Souza, E., Mrejen, S., Capuano, V., Rodrigues, M. W., Gupta, C., Ebneter, A., Zinkernagel, M. S., Munk, M. R.
Jazyk: angličtina
Rok vydání: 2017
Předmět:
Male
Visual acuity
genetic structures
Visual Acuity
Retinal Pigment Epithelium/pathology
Retinal Pigment Epithelium
Fundus (eye)
Fluorescein Angiography/methods
Severity of Illness Index
Tomography
Optical Coherence/methods

Macular Degeneration
chemistry.chemical_compound
0302 clinical medicine
lcsh:Ophthalmology
Macular Degeneration/diagnosis
Photopigment
Fluorescein Angiography
610 Medicine & health
RPE tear
Aged
80 and over

General Medicine
medicine.anatomical_structure
Female
RPE-aperture
Geographic atrophy
medicine.symptom
Tomography
Optical Coherence

Research Article
Photoreceptor Cells
Vertebrate

Visual phototransduction
medicine.medical_specialty
Fundus Oculi
03 medical and health sciences
Ophthalmology
medicine
Humans
Aged
Retrospective Studies
Retina
Retinal pigment epithelium
Photoreceptor Cells
Vertebrate/pathology

Photoreceptor
business.industry
Age-related macular degeneration
Retinal
Macular degeneration
medicine.disease
eye diseases
chemistry
lcsh:RE1-994
030221 ophthalmology & optometry
sense organs
business
030217 neurology & neurosurgery
Follow-Up Studies
Zdroj: Giannakaki-Zimmermann, Helena; Querques, Giuseppe; Munch, Inger Christine; Shroff, Daraius; Sarraf, David; Chen, Xuejing; Cunha-Souza, Eduardo; Mrejen, Sarah; Capuano, Vittorio; Rodrigues, Murilo W; Gupta, Charu; Ebneter, Andreas; Zinkernagel, Martin; Munk, Marion (2017). Atypical retinal pigment epithelial defects with retained photoreceptor layers: a so far disregarded finding in age related macular degeneration. BMC ophthalmology, 17(1), p. 67. BioMed Central 10.1186/s12886-017-0452-0
BMC Ophthalmology, Vol 17, Iss 1, Pp 1-8 (2017)
Giannakaki-Zimmermann, H, Querques, G, Munch, I C, Shroff, D, Sarraf, D, Chen, X, Cunha-Souza, E, Mrejen, S, Capuano, V, Rodrigues, M W, Gupta, C, Ebneter, A, Zinkernagel, M S & Munk, M R 2017, ' Atypical retinal pigment epithelial defects with retained photoreceptor layers : a so far disregarded finding in age related macular degeneration ', B M C Ophthalmology, vol. 17, 67 . https://doi.org/10.1186/s12886-017-0452-0
BMC Ophthalmology
DOI: 10.1186/s12886-017-0452-0
Popis: 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.
Databáze: OpenAIRE