OBJECTIVETo investigate the clinical characteristics and the corneal morphological abnormalities in Fleck corneal dystrophy.

METHODSEighteen eyes with Fleck corneal dystrophy of 9 patients from two unrelated families were examined by slit-lamp biomicroscopy, Cochet-Bonnet esthesiometer and in vivo confocal microscopy. The corneal cells, nerves and stromal deposits were analyzed quantitatively with NAVIS software.

RESULTSlit-lamp biomicroscopy of the Fleck corneal dystrophy revealed that bilateral small grey-white fleck-like or wreath-like opacities were scattered in all layer of the corneal stroma from the center to the periphery, the intervening stroma between the lesions was clear. In vivo confocal microscopy identified that the opacities appeared as doughnut-like or nephroid-like deposits approximately 70.6 μm × 110.3 μm in size, (1.6 ± 0.4)/frame in density and involved about (438.4 ± 22.0) μm of the corneal stroma, with hyper reflective dot-like intracellular particles,measuring 2 to 18 μm in diameter. In two eyes of 1 patient associated with decreased corneal sensation, confocal images showed that abnormal hyper reflective deposits involved the Bowman's layer and the branch and density of the subbasal nerve was reduced. The other eyes with normal corneal sensation appeared normal in the morphology and the density of the corneal nerves.

CONCLUSIONFleck corneal dystrophy has typical clinical characteristics. In vivo confocal microscopy allows to study the morphological changes of the cornea at cellular level, which is valuable for the clinical diagnosis and evaluation of the corneal dystrophy. Decreased corneal sensitivity in Fleck corneal dystrophy is probably caused by corneal stromal deposits involved in the Bowman's layer.