Histopathology of Primary Endothelial-Epithelial Dystrophy of the Cornea*

Retrocorneal membranes. I. Their origin and structure.

“…However, as stated by Chi, Teng, and Katzin (1960), endothelial mitoses are numerous during this time, particularly during the 2nd post-operative day, but unless the injury, or the gap in Descemet's membrane, is extremely small the daughter cells fail to cover it before the 5th or 6th days when the juvenile membrane appears. The latter grows, first by spreading as a sheet of two to four cells in thickness over the injured area of Descemet's membrane, but later by expanding both laterally and in thickness to form the mature stage.…”

“…As there has been no correlation of the isolated reports, the structures here called retrocorneal membranes have previously been known by a variety of names, e.g. stromal overgrowths (Duke-Elder, 1954); fusiform scars (Morton, Ormsby, and Basu, 1958); laminated membranes (Chi, Teng, and Katzin, 1958); fibrous tissue plugs (Stocker, 1953); post-graft membranes (Werb, 1962). The last was suggested by Werb because these membranes are most commonly noticed clinically after penetrating keratoplasty when they are seen to lie behind the graft.…”

Comprehensive Assessment of Genetic Variants WithinTCF4in Fuchs' Endothelial Corneal Dystrophy

“…1 Guttae, which are microscopic collagenous excrescences of the endothelial basement membrane, are the clinical hallmark of the disease and allow easy phenotyping of affected individuals. 2 In severe cases, extensive guttae and endothelial cell loss lead to failure of the fluid pumping function of the endothelium, resulting in corneal edema and vision loss, and allogeneic transplantation is the only available therapeutic alternative. In the United States 3 and other predominantly Caucasian countries, 4,5 FECD is the most common indication for corneal transplantation.…”

Involvement of ZEB1 and Snail1 in excessive production of extracellular matrix in Fuchs endothelial corneal dystrophy

“…The phenotypic clinical features of FECD are characterized by the presence/formation of corneal guttae, which are collagenous excrescences of the corneal endothelial basement membrane (Descemet's membrane). 1 As the disease progresses, an increasing density of guttae and an aberrant thickening of Descemet's membrane becomes evident; this is caused by the abnormal/excessive accumulation of extracellular matrix (ECM). This accumulation of ECM is accompanied by a gradual loss of corneal endothelial cells (CECs), thereby leading to a functional impairment of the endothelium to pump excess fluid out of the corneal stroma.…”