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Austin Roorda, Brandon Lujan, Kavitha Ratnam, Vincent Liu, Johnny Tam, Steven Schwartz, Andrew Kaines, Paul Bernstein, Yuhua Zhang, Jacque Duncan; Microscopic Retinal Structure in Macular Telangiectasia. Invest. Ophthalmol. Vis. Sci. 2013;54(15):3606.
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© ARVO (1962-2015); The Authors (2016-present)
To assess the retinal structure of patients with macular telangectasia (MacTel)
28 eyes from 17 patients diagnosed with MacTel were imaged with AOSLO. 14 of the 17 were at a relatively early stage. Semi-contiguous patches of cones were manually labeled in 26 of the eyes at 1545 locations with an average of 51 (+/- 15) cones per location. Cone spacing was estimated by analyzing a histogram of all intercone spacing within each dataset. Effort was made to measure cone spacing at locations as evenly distributed across the image as possible. To avoid making erroneous conclusions about cone structure, we only reported on regions where cones were clear and unambiguous, avoiding regions where we suspected their visibility might have been obscured by inner retinal pathology. Registered SD-OCT data was used to confirm retinal anatomy in all questionable regions that were analyzed. Cone spacing was compared against a normative data base generated from 27 healthy eyes. Vascular perfusion images were generated in 9 of the patients using AOSLO motion contrast imaging. 6 of the patients had at least one follow-up visit.
The most common features in patients at a relatively early stage of the disease (n=14) were focal areas of cone loss (12/14 Pts). With the exception of regions close to the focal areas of loss, remaining cones generally appeared healthy, having contiguously packed arrays with normal density. Inner retinal microcysts, which were always bilateral when present, were found in 7 of 14 early stage patients. These microcysts were spherical in shape with sizes ranging from 30 to 100 microns. In patients where we analyzed perfusion the vascular abnormalities were characterized by multiple branching, corkscrew vessels, large-caliber tortuous vessels, some of which resided in the outer retina in the larger regions of focal cone loss. In eyes tracked over time, we observed expansion of focal regions of cone loss, development of new focal regions of loss, reductions in cone density in some regions, and vascular remodeling.
The microscopic view of living eyes offered by adaptive optics is improving our understanding of MacTel, a rare disease with no animal model and for which eyes from only three human donors have been secured for study.
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