In Vivo Confocal Microscopy in Presumed Central Cloudy Dystrophy of François.

Abstract

A 70-years-old woman with no visual symptoms was incidentally diagnosed with presumed central cloudy dystrophy of François (CCDF) on regular ophthalmic examination. Bilateral polygonal opacities separated by clear spaces were seen in corneal biomicroscopic examination. The opacities were prominent in the central cornea and occupied full-thickness corneal stroma. Confocal microscopy revealed small highly refractile granules and deposits in the anterior and deeper stromal layers. Multiple dark acellular striae among extra-cellular matrices with increased intensities were seen in the posterior stroma. No stromal layer of keratocytes was observed. In the right eye, the corneal endothelial cell density was 3,319 cells/mm(2), polymegathism was 52.9%, and pleomorphism was 34.3%. In the left eye, endothelial cell counts could not be performed because of high stromal reflectance intensity. Full-thickness involvement of the corneal stroma with hyperreflective granules and microstriae and absence of keratocytes may represent a more advanced stage of the dystrophy.