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R.C. Caruso, G.L. Short, P. Lopez, L.M. Reuter; Macular Structure and Function in Stargardt Disease . Invest. Ophthalmol. Vis. Sci. 2006;47(13):1066.
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In juvenile macular dystrophies (including Stargardt disease and fundus flavimaculatus), macular structure and function are compromised by the disease process. The objective of this study was to explore the association between measurements of macular structure, assessed with optical coherence tomography (OCT), and macular function, assessed with multifocal electroretinography (mfERG), in patients with Stargardt disease.
To date, 41 patients with Stargardt disease and fundus flavimaculatus (30 women, 11 men, with an age range of 10 to 68 years) have been included in this study. Their DNA samples were screened for mutations in the ABCR gene. OCT was performed with a Zeiss OCT 3000 instrument. Six 6 mm–long radial scans were used to compute macular thickness and volume. The mfERG was recorded with an EDI VERIS 4 instrument. Responses elicited by the central hexagons (10° radius) of a 61–hexagon stimulus were used to estimate macular function. The association between variables was measured using correlation methods.
All patients had considerable thinning of the foveal area (central 1 mm diameter), while the parafoveal area (1 – 6 mm diameter) showed a variable degree of decrease in volume (5.36 ± 0.53 mm3) (mean ± std dev). Amplitude density of the first kernel of the mfERG was considerably reduced in the fovea and in the parafovea (central 10° radius) (13.6 ± 6.5 nV/deg2). Response density ranged from normal to markedly abnormal at larger eccentricities (10° – 20° radius). A statistically significant linear relationship between OCT macular volume and log mfERG amplitude density was found. In the whole parafoveal area the Pearson correlation coefficient r was 0.70 (p < 0.001). Quadrant–by quadrant analysis yielded similar results; the values of r were 0.71, 0.51, 0.67, and 0.56, for the temporal, nasal, superior, and inferior quadrants, respectively (p < 0.001 in all cases, after Bonferroni correction).
Photoreceptor loss caused by Stargardt disease led to abnormalities in macular structure and function in all our patients. Abnormalities were most severe in patients with fundus flavimaculatus with extensive macular atrophy. The decline in mfERG amplitude density and the reduction in OCT macular volume showed a significant correlation, as expected. However, the variation in mfERG amplitude density could not be accounted for by macular volume alone. A specific OCT measurement of the photoreceptor and RPE layers may increase the strength of this association.
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