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A.C. Sodi, S. Cappelli, F. Menchini, U. Menchini; Optical Coherence Tomography Findings in Hereditary Retinal Degenerations . Invest. Ophthalmol. Vis. Sci. 2003;44(13):526.
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Purpose: Aim of the work was the analysis of the macular region using optical coherence tomography (OCT) in patients affected by inherited retinal degenerations . Methods: In a retrospective study we reviewed the OCT records of 4 patients ( 8 eyes ) with retinitis pigmentosa and cystoid macular edema before and after treatment with systemic acetazolamide ; we considered also 6 patients ( 12 eyes ) with Best disease and 9 patients ( 18 eyes ) with Stargardt maculopathy.For every patient 4 mm vertical and horizontal sections passing through the fovea were obtained. Foveal thickness was measured manually five times at the center of the macular depression . Results: In all the 4 patients with retinitis pigmentosa and cystoid macular edema the OCT scan showed after acetazolamide treatment a significant reduction of foveal thickness parallely to visual acuity increase.In all the 6 patients with Best disease OCT showed a significant thickening in the reflective band representing the retinal pigment epithelium. Finally in the Stargardt disease patients it was possible to remark the reduction of foveal thickness , mainly due to partial photoreceptors disappearance. The macular thinning was related to visual acuity decrease , even if the relation was not statistically significant. Conclusions: OCT technology was able to remark "in vivo" the main structural retinal abnormalities in patients suffering from hereditary retinal degenerations.This result might be particularly interesting for uncommon diseases in which histopathologic samples are not easiliy availables . Besides that , OCT proved to be particularly useful in monitoring the efficacy of acetazolamide in reducing macular edema in retinitis pigmentosa and the progressive decrease of central visual function in Stargardt disease.
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