Background: The purpose of this infrastructure is to provide to the Network researchers a database and diverse related tools for the anatomical and functional analysis of the normal, pathological and surgical cornea.
Methods: This database is composed of normal and pathological individuals, totaling more than 36,000 patients. It includes anatomical and functional imaging data, physiological optics data, psychometric and clinical data (medical history, surgical parameters, acuteness, etc.). Various corneal topography tools were added, giving the database a unique character: tools for analyzing individual maps, average map tools for the study and comparison of populations, 3D modeling and visualization tools, statistical tools, etc. There are also screening tools for detecting various corneal conditions (LASIK, PRK, RK, keratoconus) and for secure data exchange between colleagues.
Results: Several studies were made in recent years thanks to this common infrastructure. For example, this database has provided important information regarding the evolution of the 3D shape of the normal cornea with age and ametropia and has confirmed the mirror symmetry of corneas for the right and the left eyes (enantiomorphism). The different stages of Fuchs’ dystrophy were also characterized to provide essential knowledge for surgery of the posterior layer of the cornea. Our database also allowed studying the anatomy of the wounds and the shape of the cornea before and after a transfixing transplant or an endothelial transplant (DSAEK and DSEK). The data on the characterization of experimentally transplanted corneas with corneal equivalents generated by tissue engineering and the recent addition of clinical data on the replacement of a diseased cornea with a synthetic corneal equivalent (keratoprosthesis) also resulted in several publications. More recently, the database has allowed to develop innovative algorithms to determine the optimal shape of an implant according to the clinical parameters of the recipient. On the other hand, we also demonstrated that the 3D shape of the cornea can be used as a biometric characteristic (such as fingerprints) for identification of individuals for various applications ranging from forensics to secure border crossings. Consequently, a new multimodal database (cornea + iris + eventually retina) was created for the purpose of biometric identifications. This database provides a unique set of anatomical and functional tools for the analysis of the cornea. It is characterized by the scientific quality and large quantity of accumulated information on the cornea and the high-level tools to exploit its content.
Conclusions: The common infrastructure is easily accessible to all VHRN members on request. The database will also be accessible online in 2018 (see http://cvl.concordia.ca for more information).
Background: Congenital hereditary endothelial dystrophy (CHED) is characterized by blindness at birth or in early infancy resulting from bilateral corneal opacification, and is linked to mutation in the Slc4a11 gene. A Slc4a11 knockout (KO) mouse, generated by gene deletion (Vithana et al. Nat Genet 2006), was acquired in order to study this disease. To confirm the phenotype of this Slc4a11 KO mouse model as a function of age, using the wild type (WT) mouse as a control.
Methods: Genotyping was performed by PCR (REDExtract-N-AmpTM Tissue PCR Kit, Sigma-Aldrich, Oakville, ON). Slc4a11 WT and KO mice populations aged from 5 to 50 weeks were studied (n=5 animals per age group; 5-year age intervals). Slit lamp examination, anterior segment-ocular coherence tomography (OCT930SR; Thorlabs, Inc., Newton, NJ), corneal endothelial cell staining, and scanning (SEM) and transmission (TEM) electron microscopy were used to assess the morphological and cellular differences between the two groups. The expression of basolateral membrane transporter NaBC1 within the corneal endothelium was also assessed using immunohistochemistry.
Results: Diffuse and progressive corneal opacification was observed at the slit lamp in the Slc4a11 KO mice, starting at 10 weeks. The central corneal thickness (CCT) also increased progressively as a function of time. In comparison, Slc4a11 WT corneas remained clear over the entire study period. Early TEM results showed vacuole degeneration of the corneal endothelium in the 15-week KO mouse, which was not seen in the same age WT mouse.
Conclusions: The corneal phenotype of this Slc4a11 KO mouse is representative of the clinical manifestations of CHED in human subjects, confirming the usefulness of this model for studying pathophysiology and therapeutic alternatives for Slc4a11-associated corneal dystrophies.