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Int Ophthalmol. 2009 Feb;29(1):11-8. doi: 10.1007/s10792-007-9187-x. Epub 2008 Feb 23.

In-vivo confocal microscopy of iridocorneal endothelial syndrome.

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Department of Ophthalmology, Eye and ENT Hospital of Fudan University, Shanghai, China.



We carried out a study by in-vivo confocal microscopy to investigate the appearance of iridocorneal endothelial (ICE) syndrome, and discuss its diagnostic potential.


Twelve patients, each with unilateral ICE syndrome, had both their eyes examined by in-vivo confocal microscopy. The images were recorded and analyzed by the use of proprietary software. Endothelium density, average endothelial area, coefficient of variation of cell size, percentage of hexagonal cells, and nerve fiber diameter were measured in both the anterior and posterior stroma. Corneal thickness was also measured for both eyes. A non-parametric test was used to compare differences between the affected eye and the contralateral healthy one.


In-vivo confocal microscopy highlighted two main patterns of abnormal "epithelioid-like" endothelium, both characterized by marked hyperreflective nuclei and loss of regularity in cellular size and shape. The first pattern was relatively regular cell size and shape, conserving a pattern similar to that of normal endothelial cells. However, the cells lost normal hexagonality and presented prominent uniform "cobblestone-like" nuclei occupying the central area of the cells. The second type was more irregular in cellular size and shape, with hyperreflective diversely shaped nuclei adjacent to the boundaries of the cells. Cells with two nuclei could be found in both types. Compared with the contralateral eye, the stromal nerve fibers in affected eyes were unusually thicker and distorted. Nerve diameters in the anterior stroma of affected eyes and contralateral eyes were 5.7 +/- 0.5 microm and 3.2 +/- 0.2 microm, respectively; those in the posterior stroma were 10.8 +/- 0.3 microm and 6.6 +/- 0.4 microm, respectively (both P < 0.001).


Application of confocal microscopy indicates that ICE syndrome is characterized by pleomorphic epithelioid-like endothelial cells with hyperreflective nuclei. The technique has great potential in diagnosing ICE syndrome, especially in cases with corneal edema.

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