The Histopathology of the Iridocorneal-Endothelial Syndrome

In vivo confocal microscopic characteristics of iridocorneal endothelial syndrome

“…Many clinicopathological studies have described the microstructural pathological changes of ICE syndrome, which can be summarized as: alterations in the endothelial cell size, shape and density, with prominent apical surface abnormalities such as microvilli, filipodia and ‘blebs’, and a tendency to divide and develop into multiple endothelial layers 13,14 . Unsurprisingly, the observations of the endothelium in the current study are generally consistent with previously published specular or confocal microscopical descriptions, as well as with ex vivo in situ characteristics 5,10,14−16 . However, in this study we suggested that in vivo confocal microscopy may not only be used to diagnose ICE syndrome, but may be superior to specular microscopy in defining this diagnosis, especially in cases of marked corneal oedema and particularly in regard to the facility to image all corneal layers in detail and accurately measure cellular and subcellular structures using proprietary software 2,5,12,17 .…”

“…Using the z‐scan of in vivo confocal microscopy we observed endothelial cell distribution over the z‐axis in up to five consecutive frames (subject 4), which is likely to correspond to multilayered endothelium. Previously such changes have been described ex vivo in advanced stages of disease requiring penetrating keratoplasty or trabeculectomy 2,14,15 . Interestingly, for subjects 1 and 2 no more than two layers of endothelial cells were observed.…”

On the pathology of the iridocorneal-endothelial syndrome: The ultrastructural appearances of ‘subtotal-ICE’

“…18 Briefly, a population of well-differentiated cells with epithelial features such as abundant tonofilaments and desmo somes was observed in many specimens. These cells' were larger and more pleomorphic than normal and were sometimes multilayered.…”

In-vivo confocal microscopy of iridocorneal endothelial syndrome

“…It includes Chandler's syndrome, progressive essential iris atrophy, and Cogan-Reese syndrome, a spectrum of diseases characterized by corneal endothelial abnormality, progressive iris atrophy, anterior synechia, and secondary glaucoma [2][3][4]. anomalies, and viruses such as Epstein-Barr and herpes simplex [5][6][7].…”

“…anomalies, and viruses such as Epstein-Barr and herpes simplex [5][6][7]. Much pathological research has shown that abnormal transformed ''epithelialized'' corneal endothelial cells, called ''ICE'' cells, are responsible for the onset of the disease; these cells are characterized by microvilli, filopodia, and ''blebs'' on the cellular surface, coexpression of cytokeratin and vimentin in the cytoplasm, and a tendency to divide and develop into multiple endothelial layers [3,4,[8][9][10]. Pathological examination cannot reveal in-vivo changes of the endothelium and other structures of the cornea in ICE syndrome, however.…”