Fuchs综合征的临床特征及漏诊和误诊分析

Clinical features and missed and inaccurate diagnosis of Fuchs syndrome

目的探讨Fuchs综合征的临床特征和漏诊及误诊的可能原因。方法回顾分析1999年6月至2003年12月诊治的Fuchs综合征患者85例的临床资料。对所有患者详细询问病史,进行双眼裂隙灯显微镜检查,详细记录角膜后沉着物(KP)的性质及分布、虹膜颜色及脱色素、并发症等改变。查阅以往的病史记录,统计分析诊断的一致性及漏诊、误诊的原因。结果患者年龄13～72岁,双眼受累者76例,均无睫状充血、虹膜后粘连和虹膜异色;KP呈中等大小或星形,呈三角形分布者13例,角膜后弥漫分布或瞳孔区分布者72例;均有前房闪辉,42例有前房细胞;虹膜均有不同程度的脱色素改变;44例出现晶状体后囊下混浊,19例有眼压升高。既往诊断疾病有9种,主要为葡萄膜炎、前葡萄膜炎、并发性白内障等。结论Fuchs综合征是一种以虹膜脱色素、典型KP形状和分布为特征的葡萄膜炎;缺乏对临床特征的认识是漏诊、误诊的重要原因。

Objective To investigate the clinical manifestations and possible reasons of the missed and inaccurate diagnosis of Fuchs syndrome. Methods The clinical data of 85 patients with Fuchs syndrome who were diagnosed and treated from June 1999 to December 2003 were retrospectively analyzed. The disease history of each patient was carefully recorded. Slit lamp microscopy was performed on the patients. The character and distribution of keratic precipitates （KP）, color of the iris, depigmentation, and complications were noted in detail. The data recorded in other hospitals were analyzed and the reasons of missed and inaccurate diagnosis were statistically analyzed. Results The age of the patients ranged from 13 to 72 years, and binocular involvement was noted in 76 patients. None of the patients showed ciliary congestion and iris synechiae. Middle-sized or stellated KP was found. Triangle-distributed KP was seen in 13 patients, and diffuse distribution behind the corneal or in the pupil area was in 72. Anterior-chamber flare was observed in all of the patients, while anterior-chamber cells were only noted in 42 patients. The iris with different degrees of depigmentation was found in all the patients. Complicated cataract and increased intraocular pressure oecured in 44 and 19 patients, respectively. The diagnosis in other hospitals mainly included uveitis, anterior uveitis and complicated cataract. Conclusions Fuchs syndrome is characterized by depigmented iris and typical KP. Missed and inaccurate diagnosis is mainly due to the unawareness of its clinical features.