There are several major etiologies of corneal endothelial dysfunction that eventually result in the development of bullous keratopathy (BK), such as pseudophakic BK, glaucoma surgery–related BK, pseudoexfoliation syndrome–related BK, laser-induced BK, cytomegalovirus corneal endotheliitis, and Fuchs endothelial corneal dystrophy (FECD). Fuchs endothelial corneal dystrophy is a major cause of corneal endothelial dysfunction, especially in white individuals, and corneal guttae with corneal epithelial edema develop even in the early stages of FECD. Cornea guttae can be observed both in FECD and in the late stage of corneal endothelial dysfunction induced by other etiologies. However, a distinctive characteristic of FECD is the massive accumulation of extracellular matrices in the Descemet membrane (DM) (ie, numerous cornea guttae), which are presumably produced by dysregulated corneal endothelial cells (CECs). Clinical findings from specular microscopy images and histological findings from transmission electron microscopy images obtained from the same guttae region indicate that in vivo guttae are covered by thin, elongated CECs that appear degenerated.1 However, there is an ongoing debate in regard to the biological meaning of the presence of cornea guttae, ie, whether they are the result or the cause of functionally dysregulated CECs. In fact, CECs in patients with FECD develop endoplasmic reticulum stress, thereby enhancing the formation of unfolded protein and triggering the intrinsic apoptotic pathway in the cells, and epithelial to mesenchymal transition (EMT) tends to occur.2,3 These events produce an accumulation of extracellular matrices on and in the DM, a basement membrane of CECs, that protrude into the anterior chamber.
Kinoshita S. The Ongoing Puzzle of the Biological Behavior of Cornea Guttae. JAMA Ophthalmol. 2018;136(8):893–894. doi:10.1001/jamaophthalmol.2018.1475
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