In Vivo Confocal Microscopy in Patients With Central Cloudy Dystrophyof François

Abstract

To report in vivo corneal confocal microscopic findings of patients with central cloudy dystrophy of François. Two unrelated patients, a 78-year-old man and a 75-year-old woman, with central cloudy dystrophy of François were examined using routine slitlamp biomicroscopy and confocal microscopy. In both cases, slitlamp biomicroscopy showed bilateral polygonal opacities separated by clear spaces. The corneal opacities were most prominent centrally and were located in the deeper stromal layer immediately anterior to the Descemet membrane. By confocal microscopy, normal superficial and basal epithelial layers, midstromal layers, and endothelial layers were noted in both cases. However, small highly refractile granules and deposits were observed in the anterior stromal layer in both cases. Also, multiple dark striae among the extracellular matrix with increased intensities were observed in the posterior stroma adjacent to the corneal endothelial layer in both cases. Abnormal stromal deposits and multiple dark striae were observed in central cloudy dystrophy of François using in vivo corneal confocal microscopy. Use of confocal microscopy to investigate these abnormal stromal opacities may be helpful in differentiating various corneal stromal pathologic features.