UCL Discovery
UCL home » Library Services » Electronic resources » UCL Discovery

Fundus-Wide Subretinal And Pigment Epithelial Abnormalities In Macular Telangiectasia Type 2

Powner, MB; Woods, SM; Zhu, M; Gillies, MC; Bernstein, PS; Hageman, GS; Comer, GM; ... Fruttiger, M; + view all (2018) Fundus-Wide Subretinal And Pigment Epithelial Abnormalities In Macular Telangiectasia Type 2. Retina , 38 (Supp. 1) S105-S113. 10.1097/IAE.0000000000001860. Green open access

[thumbnail of FUNDUS_WIDE_SUBRETINAL_AND_PIGMENT_EPITHELIAL.96742.pdf]
Preview
Text
FUNDUS_WIDE_SUBRETINAL_AND_PIGMENT_EPITHELIAL.96742.pdf - Published Version

Download (1MB) | Preview

Abstract

PURPOSE: Macular telangiectasia Type 2 (MacTel) causes glial and photoreceptor cell death in a small, oval patch in the central retina. Beyond this oval area, no disease manifestations have been described so far. Here, we describe a novel pathological aspect of MacTel in the retinal pigment epithelium (RPE) that is not restricted to the clinically affected area but covers the entire retina. METHODS: We have studied postmortem eyes from four patients with MacTel by immunohistochemistry and electron microscopy. RESULTS: We found cellular debris in the subretinal space (between photoreceptor outer segments and RPE), consisting mainly of outer segments and RPE components. In healthy eyes, the RPE normally phagocytoses the tips of the continuously growing outer segments, a process considered to be essential for photoreceptor survival. However, in the patients with MacTel, we found no evidence of ongoing outer segment phagocytosis, and the apical surface of the RPE appeared abnormal throughout most of the retina. CONCLUSION: Reduced outer segment phagocytosis may explain the accumulating debris in the subretinal space but is a surprising finding because visual function in the peripheral retina is normal in patients with MacTel. Nevertheless, the subclinical pathology might induce a specific stress to which the central area is uniquely susceptible.This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial License 4.0 (CCBY-NC), where it is permissible to download, share, remix, transform, and buildup the work provided it is properly cited. The work cannot be used commercially without permission from the journal.

Type: Article
Title: Fundus-Wide Subretinal And Pigment Epithelial Abnormalities In Macular Telangiectasia Type 2
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1097/IAE.0000000000001860
Publisher version: http://dx.doi.org/10.1097/IAE.0000000000001860
Language: English
Additional information: This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial License 4.0 (CCBY-NC), where it is permissible to download, share, remix, transform, and buildup the work provided it is properly cited. The work cannot be used commercially without permission from the journal.
Keywords: MacTel, photoreceptors, outer segment phagocytosis, RPE.
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > Institute of Ophthalmology
URI: https://discovery.ucl.ac.uk/id/eprint/10026100
Downloads since deposit
0Downloads
Download activity - last month
Download activity - last 12 months
Downloads by country - last 12 months

Archive Staff Only

View Item View Item