Quantitative Regional Differences in Corneal Endothelial Abnormalities in the Central and Peripheral Zones in Fuchs' Endothelial Corneal Dystrophy

Abstract

In Fuchs' endothelial corneal dystrophy, the corneal endothelium is damaged more severely in the center and paracentral zones than in the peripheral zone, and peripheral measurement can objectively grade the disease. In the peripheral zones, the inferotemporal endothelium is damaged more severely. These findings might provide a new understanding of the disease mechanisms.

Fuchs Corneal Dystrophy

“…However, a propensity for the development of guttae in the inferotemporal quadrant has been documented in multiple families using retroillumination photography 11,12 and specular microscopy. 13 Although the causes for this are unclear, the inferotemporal cornea is associated with a thinner cornea and potentially increased light exposure (Fig. 2).…”

Predicative Factors for Corneal Endothelial Cell Migration

“…21 These guttae are aberrant collections of banded DM that occur in variable sizes and distribution densities, 9 most commonly found within the interpalpebral fissure zone. 22 Disease progression is characterized by an increase in the size and density of guttae 23 and concomitant reductions in the population of healthy CECs. 24 In contrast, pseudophakic bullous keratopathy (PBK) is an acquired corneal endothelial disorder that may occur following intraocular cataract extraction surgery.…”

“…28 Although PBK is characterized by diffuse damage to the corneal endothelial layer, 29 a peripheral rim of healthy CECs and DM is usually retained in corneas affected by FED. 22 As such, it has been postulated that this reservoir of healthy CECs in FED corneas can potentially be exploited to repopulate the centrally diseased corneal endothelium either by migration or proliferation, on exposure to externally applied stimulants. 19 Topical Rho-associated protein kinase inhibitors (ROCK inhibitors) such as AR-12286, 30 AMA0076, 31 and Y-27632 32 have been studied extensively, with proven efficacy in the treatment of primary open angle glaucoma 33 and stimulation of CEC proliferation and migration under the appropriate culture conditions in vitro.…”

A Simple and Reliable Technique to Orient Donor Corneal Tissue Using the Radial Width of the Surgical Limbus

“…This additional level of spatial detail has the potential to enhance significantly our current understanding of endothelial variability. [4][5][6][7] Previous studies from our group have shown that the intracorneal coefficient of variation among different 3-mm diameter regions of the endothelium for basal mitochondrial respiration and glycolysis activity was 19.9% and 17.2% of the mean, respectively. 15 However, lack of unambiguous anatomical identifiers prevented us from orienting excised corneas with absolute certainty and exploring this variability by using a more refined sampling strategy.…”

“…It is already well-documented that the endothelium shows regional differences in cell density, 4 regenerating pluripotent stem cell populations, 5,6 and endothelial damage in eyes with Fuchs dystrophy. 7 However, the majority of ex vivo studies examine the regional physiological variability of the endothelium with radial (ie, central vs. peripheral) rather than axial (ie, temporal, superior, nasal, and inferior) orientation schemes. Thus, it is possible that additional and significant unrecognized sources of endothelial variability exist that may be identified with a more refined axial approach to regional variability.…”