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Late-Stage Sjögren-Larsson Syndrome Maculopathy Imaged With OCT Angiography
Journal article   Peer reviewed

Late-Stage Sjögren-Larsson Syndrome Maculopathy Imaged With OCT Angiography

Jaclyn L Kovach
Ophthalmic surgery, lasers & imaging retina, Vol.49(9), pp.e78-e82
2018-09-01
DOI: https://doi.org/10.3928/23258160-20180907-11
PMID: 30222823

Abstract

Retinal Diseases - diagnosis Capillaries - pathology Follow-Up Studies Fluorescein Angiography - methods Humans Sjogren-Larsson Syndrome - diagnosis Male Tomography, Optical Coherence - methods Retinal Vessels - pathology Sjogren-Larsson Syndrome - complications Retinal Diseases - etiology Adult Female Fundus Oculi Macula Lutea - pathology Siblings
Three adult siblings with Sjögren-Larsson syndrome (SLS) demonstrated signs of late-stage SLS maculopathy, including intraretinal crystals, atrophic changes, and lipofuscin deposition. This first report of SLS maculopathy imaged with optical coherence tomography angiography revealed decreased retinal capillary density, vessel dilation, and increased flow voids in the superficial and deep capillary plexuses. [Ophthalmic Surg Lasers Imaging Retina. 2018;49:e78-e82.].

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Citation topics
1 Clinical & Life Sciences
1.239 Tissue Barriers
1.239.1146 Intermediate Filaments
Web Of Science research areas
Ophthalmology
Surgery
ESI research areas
Clinical Medicine

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