Feng Bo, Tang Xin, Chen Hong, Sun Xia, Wang Ningli
Beijing MEM Eye Care System, No. 65-2, Fuxing Road, Beijing, 100036, China.
Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology and Visual Sciences Key Laboratory, No. 1 Dongjiaominxiang Street, Dongcheng District, Beijing, 100730, China.
Int Ophthalmol. 2018 Oct;38(5):2117-2126. doi: 10.1007/s10792-017-0712-2. Epub 2017 Oct 10.
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.
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.
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.
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.
虹膜角膜内皮（ICE）综合征是一种罕见疾病，中国患者的独特特征可能导致诊断困难。我们的目的是描述58例连续中国患者ICE综合征的临床特征和变异情况。
回顾性分析2008年至2011年在我院眼科青光眼门诊就诊的连续ICE综合征患者的临床资料。ICE综合征的诊断标准为角膜内皮呈现“锤击银”外观，以及镜面显微镜检查显示ICE细胞，其特征为缺乏六边形外观、细胞内有暗区以及明暗反转模式。汇总并检查角膜、虹膜、瞳孔和前房角的一般特征。
共确诊58例ICE综合征患者：26例为钱德勒综合征（CS），23例为科根-里斯综合征，9例为进行性虹膜萎缩（PIA）。青光眼的发生率为98%。23例（39.7%）患者患有非典型ICE综合征。13例虹膜轻度受损的患者眼压相对较高，而角膜内皮相对保持完整。裂隙灯检查显示10例患者（17.2%）虹膜完整，前房角有大量黑色色素沉着。在25例前房角仅部分关闭的患者中，80%的前房角色素沉着。
CS是中国患者ICE综合征最常见的变异类型，PIA最不常见。裂隙灯检查时虹膜轻微改变或完整是ICE综合征的一个特征。
