Stargardt Disease

Published on 10/05/2015 by admin

Filed under Opthalmology

Last modified 22/04/2025

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16.2

Stargardt Disease

OCT Features:

OCT confirms the RPE as the location of the abnormal deposits and shows associated outer retinal atrophy, which may be present parafoveally (Fig. 16.2.2) or involve the fovea (Fig. 16.2.3). In more advanced stages of the disease, there is more widespread outer retinal atrophy that can lead to geographic atrophy (Fig. 16.2.4).

Ancillary Testing:

Fluorescein angiography and fundus autofluorescence (FAF) can help in confirming the diagnosis. FA can show a characteristic dark choroid (Fig. 16.2.5), present in about 70% of cases. FAF highlights the abnormal RPE deposits and best demonstrates the peripapillary sparing (Figs 16.2.6 and 16.2.7).

image

Figure 16.2.6 FAF corresponding to Figures 16.2.1 and 16.2.2 shows areas of both hyperautofluorescence and hypo-autofluorescence corresponding to pisciform flecks. The characteristic peripapillary sparing is well demonstrated.

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Figure 16.2.7 FAF corresponding to Figure 16.2.3 shows areas of both hyperautofluorescence and hypo-autofluorescence corresponding to pisciform flecks. There is a large area of profound central hypo-autofluorescence corresponding to the geographic atrophy. Again, there is characteristic peripapillary sparing.