Reis-Bücklers corneal dystrophy: a reappraisal using in vivo and ex vivo imaging techniques.

[]

To characterize the phenotype of Reis-B ücklers corneal dystrophy (RBCD ) using in vivo and ex vivo imaging techniques .Five RBCD patients with penetrating keratoplasty (PK ) were enrolled . Before surgery , all patients underwent a complete ophthalmological examination including slitlamp biomicroscopy , in vivo confocal microscopy (IVCM ) and anterior segment (AS ) optical coherence tomography (OCT ). After PK , corneal buttons were examined by light and transmission electron microscopy (TEM ). Correlations between in vivo and ex vivo images were analyzed .In all cases , irregular geographic-like subepithelial gray-white opacities were observed in the central and mid-peripheral cornea . AS-OCT images of the cornea of all patients revealed hyperreflective homogeneous and continuous deposits concentrated at the level of Bowman 's layer and anterior stroma . Using IVCM , a highly reflective irregular amorphous material was observed from intermediate epithelial cells to the anterior stroma . Sparse deposits of highly reflective material were also detected in the posterior stroma . TEM showed in all specimens basal epithelial cells containing small vesicles with rod-shaped dense material .IVCM and AS-OCT may be a useful adjunct to biomicroscopy for the diagnosis and management of RBCD . The correlations between the different in vivo and ex vivo imaging techniques emphasize the hypothesis of an epithelial origin for RBCD .