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V. C. Greenstein, J. N. Carlson, M. Wang, S. H. Tsang, R. T. Smith, R. E. Carr, D. C. Hood; A Comparison of Retinal Structure and Visual Function in Foveal and Preferred Retinal Location Regions of Patients With Stargardt Disease. Invest. Ophthalmol. Vis. Sci. 2009;50(13):6269.
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To assess the relationship between the thicknesses of retinal layers measured with frequency domain optical coherence tomography (fdOCT) and visual function measured with fundus tracking microperimetry (MP-1) in patients with Stargardt disease.
Thirteen patients (19 eyes) with Stargardt disease (ABCA4 related maculopathy) aged 12-65 yrs. were studied. All tested eyes had best corrected visual acuities >= 20/200. The preferred retinal location (PRL) of fixation was evaluated with fundus photography and the Nidek microperimeter (MP-1). Visual fields were measured with the MP-1 (10-2 program, 4-2 protocol). The structure of the foveal and eccentric PRL regions was evaluated with fd-OCT (Zeiss) using horizontal linear scans and macular cube analyses. Fundus autofluorescence (FAF) images were obtained with a confocal scanning laser ophthalmoscope (HRA II). The thicknesses of the total retina (TR) and total receptor (R: Bruch's membrane to OPL) layers through the foveal and eccentric PRL regions were measured using a computer-aided manual technique (1). The values were compared to visual field sensitivities obtained at corresponding retinal locations.
In the central foveal region thicknesses of the TR and R layers were significantly decreased for all eyes compared to normal controls. Visual field sensitivity was decreased or non-recordable in the same locations. The lower sensitivity values corresponded to the thinner TR and R values. Fifteen eyes had developed an eccentric PRL. For 13, the retinal location of the eccentric PRL was superior to the fovea; for the other 2 eyes, it was located either 2° temporal or nasal to the fovea. The 15 eyes had a central scotoma on the MP-1 corresponding to the atrophic lesion. The combined PRL, MP-1 and FAF images showed that retinal areas underlying the eccentric PRL had normal FAF, thicker R and better visual field sensitivity values.
The majority of patients had an eccentric PRL. The thickness and sensitivity values for the central foveal region were significantly decreased, whereas the location of the PRL showed better TR and R thickness values and improved visual field sensitivity. A combination of structural, FAF, and visual function measurements is essential in the evaluation and management of patients with Stargardt disease.1. Hood DC, Lin CE, Lazow MA, Locke KG, Zhang X, Birch DG IOVS 2008 Nov 14.
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