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

Abstract

SUMMARYThe iridocomeal-endothelial syndrome (ICE syn The iridocorneal-endothelial syndrome (ICE syn drome) is characterised by a 'hammered-silver' appearance of the corneal endothelium, corneal

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 .…”

“…Iridocorneal endothelial syndrome is an uncommon and, unfortunately, often under‐diagnosed condition, due to the variability of severity of presentation. 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 .…”

“…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.…”

“…Their study confirmed the value of the specular microscope for qualitative endothelial observations. This seminal study was followed by a number of reports correlating specular microscopic observations with ex vivo microscopy, including electron microscopy, and the definition ‘characteristic ICE cells’ was quickly accepted into ophthalmic practice 5,9,14 . Subsequently, no other corneal diseases appear to have been associated with such changes, such that this appearance has been accepted as a sine qua non clinical marker of ICE syndrome.…”

Ring melanoma---a rare cause of refractory glaucoma

“…These diagnoses were supported by endothelial specular microscopy (ESP). This method had previously been advocated as providing a definitive diagnosis of ICE syndrome,13 but at present ICE has been shown to have pleomorphic manifestations on ESP 14. It may be that ring melanoma deposits on the corneal endothelium mimic the in vivo ESP appearance of ICE cells during one of its transitional stages.…”