虹膜角膜内皮综合征12例的临床观察和分析

目的：分析虹膜角膜内皮综合征( iridocorneal endothelial syndrome,ICE综合征)的临床特点及发病机制,探讨其治疗及预后。
　　方法：选择2007-06/2015-02在第三军医大学附属西南医院住院治疗的12例12眼ICE综合征患者,其中原发性进行性虹膜萎缩7眼,Chandler综合征3眼,Congan-Reese综合征2眼。对所有患者的临床资料进行回顾性分析及随访。
　　结果：所有患者中,8眼行一次或多次滤过手术,4眼仅行青光眼阀门植入术。术后随访时间为15mo ~5a,平均30mo。12眼患者中,4眼抗青光眼术后眼压升高的时间为3~16(平均10)mo,远期观察眼压控制不佳。
　　结论：ICE综合征是一组临床上比较少见的严重眼病,其基本病变为角膜内皮层存在的ICE细胞过度增生导致房角粘连、虹膜萎缩及继发性青光眼等,目前青光眼滤过手术及青光眼阀门植入术只能在术后早期控制眼压,但远期效果不佳。

Clinical observation and analysis of 12 cases with iridocorneal endothelial syndrome

AIM: To summarize clinical characteristics and pathogenesis of iridocorneal endothelial syndrome(ICE)and investigate the treatment and prognosis.

METHODS: The clinical data of 12 cases(12 eyes)who received treatment in southwest hospital during Jun. 2007 to Feb. 2015 were analyzed retrospectively. The essential progressive atrophy of iris included 7 eyes, Chandler syndrome included 3 eyes, Congan-Reese syndrome included 2 eyes.

RESULTS: A total of 8 eyes were carried out once or multiple filtration surgery; 4 eyes were treated with glaucoma valve implantation. Postoperative follow-up time ranged from 15mo to 5y with the average of 30mo. Three months to 16mo after the surgery, the intraocular pressure of 4 patients were elevated again. Postoperative intraocular pressure was poorly controlled.

CONCLUSION:ICE syndrome is a group of clinically rare and serious eye disease. The excessive proliferation of ICE cells causes the existence of the corneal endothelial cells adhesion to the chamber angle and iris surface, which cause iris atrophy, secondary glaucoma, corneal endothelial decompensation. Currently, glaucoma filtration surgery and glaucoma valve implantation can only control intraocular pressure for several months, but the long-term prognosis is poor.