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Simona Degli Esposti, Marketa Cilkova, Rengin Aslihan Kurt, Pearse A Keane, Dawn Sim, Catherine A Egan, Adnan Tufail; Atrophy progression on autofluorescence in patients with Maternally Inherited Diabetes and Deafness (MIDD). Invest. Ophthalmol. Vis. Sci. 2016;57(12):1704.
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© ARVO (1962-2015); The Authors (2016-present)
To evaluate the progression in the area of atrophy in patients with Maternally Inherited Diabetes and Deafness (MIDD).
Retrospective case series data analysis. Eight patients (7 females) with genetically proven MIDD with follow-up of at least two years were identified and images were reviewed retrospectively. Patients were reviewed in clinic yearly and ophthalmological examination was performed and autofluorescence (AF) imaging taken. Images from 3 eyes were not evaluable due to poor quality; therefore only 13 eyes were included in the analysis. AF images were reviewed and areas of atrophy were measured with RegionFinder software (Heidelberg Spectralis, Heidelberg Engineering, Germany).
Mean age of the patient at start of follow-up was 60 years (range 51-76) and mean follow up was 3 years (range 2-5). Patient underwent AF images yearly and they were analysed retrospectively with RegionFinder to evaluate areas of atrophy. Mean area of total atrophy was 18.89mm2 (range 2.02mm2-54.97mm2). The rate of atrophy progression as calculated from the RegionFinder was 1.52mm2 per year (range 0.78mm2-2.62mm2 per year). Rate of progression was similar between the two eyes of the same patient (where data were available to evaluate, variability of less than 0.5mm2 between fellow eyes). Only one single patient had variable progression between the two eyes, 2.67mm2 against 1.81mm2.
Rate of atrophy progression in this pilot study suggests a similar rate as in atropic age-related macular degeneration (AMD). This pilot study supports further analysis atrophy of expansion to aid prognosis for this orphan disease and help design future clinical studies.
This is an abstract that was submitted for the 2016 ARVO Annual Meeting, held in Seattle, Wash., May 1-5, 2016.
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