Purchase this article with an account.
Drew H Scoles, Mara R Goldberg, Christopher S Langlo, Yusufu N B Sulai, Kimberly E Stepien, David V Weinberg, Judy E Kim, Alfredo Dubra, Joseph Carroll, Barbara A Blodi; Non-invasive evaluation of microscopic retinal pathology in macular telangiectasia type 2. Invest. Ophthalmol. Vis. Sci. 2014;55(13):5951.
Download citation file:
© ARVO (1962-2015); The Authors (2016-present)
To characterize photoreceptor, retinal pigment epithelium (RPE) and vascular changes in macular telangiectasia (MacTel) type 2.
Seven subjects, average age 63 years, diagnosed with MacTel type 2 at various stages were imaged in one eye using confocal adaptive optics scanning light ophthalmoscopy (AOSLO) and spectral domain optical coherence tomography (SD-OCT). In addition, split-detection AOSLO was used in four subjects to visualize the photoreceptor inner segments, vasculature, and inner retina, while dark-field AOSLO was used to image the RPE mosaic. Vascular perfusion maps were generated as the standard deviation of split-detection AOSLO image sequences. The area of ellipsoid zone (EZ) lesion was measured with en face SD-OCT.
SD-OCT revealed disruptions of the EZ in 6 of 7 eyes (average 0.074mm2), with corresponding dark-regions of reduced or complete loss of photoreceptor reflectivity in confocal AOSLO imaging. RPE cells were seen with dark-field AOSLO in the center of two of the EZ lesions, where the photoreceptors appear to have degenerated. Split-detection revealed swollen cone inner segments on the edges of these lesions in 3 of 4 imaged patients, as well as microcysts ranging in size from 2 to 100 µm in all subjects, which were not always visible in SD-OCT. Fibrotic-like structures on the inner retinal surface as well as sharply demarcated cavitations were present in all subjects. Vascular perfusion maps revealed abnormally enlarged and tortuous vessels in all subjects and microaneurysms in two out of four subjects over the central 3° around fixation.
The combination of multiple AOSLO imaging techniques with SD-OCT is useful for non-invasive study of the micro-pathology in MacTel. Specifically, dark-field AOSLO disambiguates the reflectivity inside and surrounding EZ lesions identifying reflective structures as RPE cell granules, while split-detection AOSLO reveals photoreceptor swelling and microaneurysms, which could be useful as biomarkers of disease activity and progression.
This PDF is available to Subscribers Only