Unique variations and characteristics of iridocorneal endothelial syndrome in China: a case series of 58 patients.

PURPOSE: Iridocorneal endothelial (ICE) syndrome is a rare condition, and unique characteristics in Chinese patients can make diagnosis difficult. Our purpose was to describe the clinical characteristics and variations of ICE syndrome in 58 consecutive Chinese patients.

METHODS

The clinical data of consecutive patients with ICE syndrome who were seen between 2008 and 2011 at the glaucoma clinic of our ophthalmology department were retrospectively reviewed. The diagnostic criteria for ICE syndrome were a "hammered-silver" appearance of the corneal endothelium and specular microscopy showing ICE cells characterized by the absence of a hexagonal appearance, dark areas within the cells, and a light-dark reversal pattern. The general characteristics of the cornea, iris, pupil, and anterior chamber angles were compiled and examined.

RESULTS

Fifty-eight patients with ICE syndrome were identified: 26 had Chandler's syndrome (CS), 23 Cogan-Reese syndrome, and nine progressive iris atrophy (PIA). The incidence of glaucoma was 98%. Twenty-three (39.7%) patients had atypical ICE syndrome. Intraocular pressure was relatively high in 13 patients with slightly damaged irises, while the corneal endothelium remained relatively intact. Slit lamp examination showed an intact iris in ten patients (17.2%), with massive deposition of black pigment in the anterior chamber angle. In the 25 patients in whom the anterior chamber angle was only partially closed, 80% of the anterior chamber angles were hyperpigmented.

CONCLUSIONS

CS is the most common variation of ICE syndrome in Chinese patients, and PIA is the least common. A slight change or an intact iris under slit lamp examination is a characteristic of ICE syndrome.