FUNDUS AUTOFLUORESCENCE IN PATIENTS WITH STARGARDT'S DISEASE/ FUNDUS FLAVIMACULATUS

97th DOG Annual Meeting 1999

P463
FUNDUS AUTOFLUORESCENCE IN PATIENTS WITH STARGARDT'S DISEASE/ FUNDUS FLAVIMACULATUS

C.Gerth¹, H. El Toukhy², M. Andrassi¹, B. Lorenz¹

The fundus-autofluorescence (AF) allows an in-vivo study of the fundus lipofuscin distribution (1,2). The intracellular lipofuscin accumulation is a sign of aging retinal pigment epithelium (RPE) (3). In Stargardt's disease lipofuscin granules have been shown not only in RPE cells but also in the photoreceptor layer (4).

Patients and Methods: AF was studied in both eyes of 18 affected patients aged 10-45 yrs. using a confocal laser ophthalmoscope (Heidelberger Retina Angiograph; excitation wavelength 488 nm, irradiance 0,2 mW/cm², blocking filter at 500nm, scanning field 30º, image averaging of 16 single images, image resolution 256x256 pixels, image digitizing with 256 gray scale levels). The diagnosis was confirmed by clinical, electrophysiological (Ganzfeld- and multifocal ERG) and psychophysical testings (scotopic and photopic perimetry, dark adaption).

Results: In all patients AF was different from that in normal age-matched probants. Generally, there was a central hypofluorescence but of different extent (<1 disc diameter (dd) n=14, >1dd n=4). In the perimacular area there was either a circular hyperfluorescence (n=4) or dotlike areas of hyperfluorescence and hypofluorescence (n=12). There was no correlation of the AF and the electrophysiological and psychophysical results, patient age or duration of the disease. The hyperfluorescent areas did not always correspond to fundus flavimaculatus dots seen on biomicroscopy.

Conclusion: AF shows different distribution pattern in patients with Stargardt's disease/ Fd. flavimaculatus. At the moment no conclusions appear possible as to the functional significance of AF. Additional measurments are necessary to evaluate the dynamics of the AF, and thus of the lipofuscine distribution in M. Stargardt/ Fd. flavimaculatus. Possibly AF will become useful in the detection of early changes and to evaluate theapeutical trials.

1. F.C. Delori et al.: Invest Ophthalmol Vis Sci. 36 (1995) 718-729.
2. A. v.Rückmann, F.W. Fitzke, A.C.Bird: Br.J.Ophthalmol 79 (1995) 407-412.
3. L. Feeney-Burns, G.E. Eldred: Trans Ophthalmol Soc UK 103 (1984) 416-421.
4. C.D. Birnbach et al.: Ophthalmology 101 (1994) 1211-1219.

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