86例虹膜角膜内皮综合征临床特征及误诊分析

Analysis of the Clinical Features and Misdiagnosis of 86 Cases of Iridocorneal Endothelial Syndrome

目的:探讨虹膜角膜内皮综合征患者的临床特征及常见误诊原因。方法:回顾性系列病例分析。统计1993—2015年于青岛眼科医院就诊并确诊为虹膜角膜内皮综合征的患者86例(86眼),记录患者性别、年龄、主诉、病程、并发症、误诊情况等,按照虹膜角膜内皮综合征的诊断及分型标准,对患者就诊时的临床特征、误诊情况等进行分析。结果:86例均单眼患病,男女比1:1.2,年龄20~73(50.1±11.8)岁。91%患者就诊原因以视力渐进性下降伴眼红为主诉,病程0.17~10(2.4±2.3)年;1%患者以瞳孔变形为主诉,病程1年余;6%患者主诉双眼黑眼珠颜色不一就诊,病程0.5~2(1.4±0.6)年;2%患者体检发现眼压高并及时就医。初诊正确率为93%,其中95%表现明显虹膜萎缩,体征被归类为原发性进行性虹膜萎缩,Chandler综合征、Cogan-Reese综合征分别占4%、1%。角膜内皮细胞形态典型表现为不规则、空泡状、中央区黑而边缘较亮。不典型表现是无明显虹膜萎缩。超声生物显微镜及房角镜检查周边虹膜广泛前粘连及部分局部丝状前粘连,小梁网棕色色素沉着1~3级。68%患者已出现青光眼及角膜内皮功能失代偿。27%患者首次就诊出现误诊,其中20%为外院误诊。8%误诊为慢性闭角型与原发性开角型青光眼,12%误诊为青睫综合征、Fuchs综合征、葡萄膜炎。角膜内皮细胞检查缺失及虹膜不典型临床表现是误诊的主要原因。结论:视力渐进性下降是虹膜角膜内皮综合征患者就诊的主要原因,就诊时间较晚、病程长,超过50%患者就诊时已出现并发症;约95%患者属于原发性进行性虹膜萎缩,最易误诊为原发性青光眼及葡萄膜炎,角膜内皮细胞检查缺失及虹膜不典型表现为主要误诊原因。

Objective: To observe and analyze the clinical features and misdiagnosis of iridocorneal endothelial (ICE) syndrome. Methods: In this retrospective case analysis, 86 eyes of 86 patients who were diagnosed with ICE syndrome between 1993 and 2015 in Qingdao Eye Hospital. Sex, age, maincomplaints, course of disease, types, complication and misdiagnosis were recorded and analyzed. Results: Eighty-six cases were unilateral. The ratio of male to female was 1:1.2, and the age ranged from 20 to 73 years, average 50.1±11.8 years. 91% of patients with progressive visual loss and red eye were the main complaints. The course of disease ranged from 0.17 to 10 years, average 2.4±2.3 years. 1% of patients complained about pupil deformation, the course of disease was 1 year. 6% of patients complained black eye foggy, the course of disease ranged from 0.5 to 2 years, average 1.4±0.6 years. 2% of patients with high intraocular pressure founding by physical examination were treated in time. 95% of patients with obvious iris atrophy were classified as primary progressive iris atrophy, Chandler and Cogan-Reese syndrome were 4% and 1%. The typical morphology of corneal endothelial cell showed irregular, cavitation, central black area and brighter edge. The atypical sign was no significant iris atrophy. 68% of patients had developed glaucoma and corneal endothelial dysfunction. Ultrasawnd biomicroscepy and angle mirror examination showed peripheral iris extensive anterior and local filamentous adhesion, angle pigment classification level was 1 to 3. 27% of patients were misdiagnosed for the first visit and 20% of these patients were misdiagnosed in other hospitals. 8% of patients were misdiagnosed as primary glaucoma, 12% of patients were misdiagnosed as Posner-Schlossman syndrome, Fuchs syndrome and uveitis. Absence of corneal endothelial cell morphology examination and atypical clinical manifestation were main causes of misdiagnosis. Conclusions: Progressive visual loss is the main reason for patients with ICE syndrome, long course of disease and delay treatment. More than 50% of patients have complications at visiting time. 95% of patients are primary progressive iris atrophy. Most easily misdiagnosed as primary glaucoma and uveitis. Absence of corneal endothelial cell morphology examination and atypical clinical manifestation are main causes of misdiagnosis.