Abstract

SA.09.08

Morphological and functional findings in patients with Stargardt’s disease

Berisha F.^1,2, Feke G. T.¹, Hirai K.¹, Pfeiffer N.², Hirose T.^1
1Schepens Retina Associates, Harvard Medical School, ²Universitäts-Augenklinik Mainz

Objective: To investigate whether optical coherence tomography (Stratus OCT) and scanning laser ophthalmoscope (SLO) microperimetry provide diagnostic information in patients with Stargardt’s disease (STGD), and to evaluate the correlation between foveal thickness and best-corrected visual acuity (VA) in these patients.
Methods: Fifteen patients with STGD (mean age 41 years, range 14 to 71 years) and 15 age-matched healthy control subjects were included in the study. OCT imaging was performed by an experienced operator (F.B.) using six radial line scans manually centered on the fovea. Foveal thickness was defined as the average thickness in the central 1 mm diameter area. SLO microperimetry was used to assess central scotoma and fixation behavior in patients with STGD.
Results: Mean VA was 20/80, range 20/25 to 20/300 (log MAR 0.6, range 0.1 to 1.2) in the STGD group and 20/20 (log MAR 0.0) in the control group. Foveal thickness was significantly reduced in patients with STGD (114.2±25.8 µm) compared to controls (206.5±21.6 µm, P<0.0001). Linear regression analysis showed a significant inverse correlation between foveal thickness and best-corrected VA within the STGD group (R²=0.41, P<0.01). OCT images showed photoreceptor loss, identified as a discontinuation of the hyperreflective band (junction between photoreceptor inner and outer segments) in the macular area in all STGD patients. A dense central scotoma corresponding to the photoreceptor loss was observed in all patients with STGD. The fixation point was dependent on the severity of the disease. Central fixation was present in patients with mild damage of the photoreceptor layer in the fovea, whereas patients with more severe central atrophy showed eccentric fixation typically located at the top edge of the scotoma.
Conclusions: OCT findings, particularly reduced foveal thickness and photoreceptor loss in the macular area may be useful in the diagnosis of STGD. Furthermore, a strong correlation between foveal thickness and visual function was observed in our patients. Assessment of central visual function using SLO microperimetry provides additional useful information, important in the management of STGD.

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