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K. I. Cubukcu, R. Khaireddin, M. Klemm, G. Richard, U. Bartsch, S. Linke; Systematic in vivo Confocal Microscopy and Histological Analysis of a Two Generation Family With Schnyder Corneal Dystrophy. Invest. Ophthalmol. Vis. Sci. 2007;48(13):5877.
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Schnyder crystalline corneal dystrophy (SCCD) is clinically characterized by bilateral central corneal opacities. It is a rare autosomal dominant disease and might be associated with Arcus lipoides, genu valgum, and systemic abnormalities of lipoprotein metabolism. Here, we describe our results of a systematic analysis of eight members of a two generation family with Schnyder crystalline corneal dystrophy.
Using a confocal scanning laser ophthalmoscope with a diode laser of 670 nm wavelength (HRT II / Rostock Cornea Module - Heidelberg Engineering) we acquired two-dimensional confocal images of the different corneal layers. Each image consists of 384 x 384 picture elements (pixels) covering a corneal area of 400 µm x 400 µm. Eight affected family members with SCCD were analyzed. The confocal findings are shown in direct comparison to biomicroscopic examinations. Selected cases were analyzed in more detail using various histological techniques and electron microscopy.
In vivo confocal microscopy revealed pathological alterations of the normal corneal anatomy in patients with SCCD, such as highly reflective crystalline structures in the anterior- and mid-stroma. These confocal findings and biomicroscopic examination results are mostly parallel each other. However, we also found these crystalline corneal structures in those family members, who were clinically not yet affected by SCCD.
In vivo confocal microscopy is a useful tool to evaluate corneal dystrophies because it allows the early detection of subtle changes of the corneal morphology. The ability to visualize small, highly reflective deposits might enable the clinician to reveal earliest stages of corneal dystrophies such as Schnyder’s crystalline corneal dystrophy.
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