虹膜角膜内皮综合征1例

患者 ,女 ,5 8岁。因左眼视物模糊 3个月 ,加重 10天而入院。入院时眼部检查 :视力右 1.0 ,左 0 .1。左眼角膜透明 ,周边前房深度 1/ 2 CT,左眼虹膜纹理疏松 ,显著变薄 ,3点钟方位可见一 2 mm× 3mm的大洞 ,中间有几束虹膜残留 ,瞳孔3mm× 3mm,晶状体轻度混浊 ,眼底视乳头色苍白 ,边界清楚 ,C/ D=0 .9,血管向鼻侧偏移。右眼无异常。前房角镜检查 :右眼为宽角 ,左眼房角广泛粘连、变窄。非接触式眼压计测眼压 :右 13mm Hg,左 32 mm Hg,角膜内皮照相及计数示 :右眼角膜内皮数 2 85 8.8个 / mm2 ;左眼 112 4个 / mm2 ,左眼内皮细胞边界模糊…     

虹膜角膜内皮综合征误诊1例

ICE综合征是以角膜内皮异常、进行性虹膜基质萎缩、广泛的周边虹膜前粘连、房角关闭及继发性青光眼为特征的一组疾病 ,无遗传倾向 ,罕有家族史 ,多见于白人 ,在我国比较少见。患者 ,女 ,5 5岁。主诉右眼视物模糊 2个月 ,曾先后在 2家县级及市级医院就诊 ,诊为虹膜睫状体炎。 2     

虹膜角膜内皮综合征1例

患者男　 30岁　左眼视物模糊 8个月 ,于 1998年 4月 15日就诊。于 8年前因患“虹膜炎”、“瞳孔变形”在地方医院治疗后视力恢复。无外伤及手术史。右眼视力 5 .2 ,左眼 4.9。眼睑及结膜无充血 ,左角膜混浊 ,表面不平 ,后弹力层皱褶明显。前房浅 ,kp (- ) ,房水闪光 (- )。虹膜纹理不清 ,部分萎缩 ,下方虹膜前粘连。瞳孔欠圆、上移 ,晶体透明。眼底正常。眼压正常。右眼未见异常。诊断 :左眼虹膜炎 ,全身和局部应用皮质激素治疗。于 1999年 6月再次来诊。右眼视力 5 .2 ,左眼光感 ,左眼球结膜充血 ,刺激症状不明显 ,角膜弥漫性混浊 ,水肿 ,…     

虹膜角膜内皮综合征

本文报道了１３例１８眼虹膜角膜内皮综合征。男性５例,女性８例,单眼发病８例,双眼发病５例,１０例继发青光眼。原发性进行性虹膜萎缩７例,Ｃｈａｎｄｌｅｒ综合征４例,虹膜痣２例。本文结合文献讨论了该病的临床特点,发病机理及治疗。     

虹膜角膜内皮综合征

虹膜角膜内皮综合征徐珊琪肖斯贤郑可薇汕头大学医学院附属第一医院眼科（５１５０４１）虹膜角膜内皮综合征是进行性原发性虹膜萎缩，Ｃｈａｎｄｌｅｎ氏综合征和Ｃｏｇｅｎ－Ｒｅｓｅ综合征（虹膜痣综合征）的总称，较少见，现将遇到３例报告如下。例１陈××男６１岁住...     

双眼虹膜角膜内皮综合征1例

1临床资料 患者，女，38岁，约3a前出现双眼视力下降，胀痛发红，以后逐渐反复加重，于2007年2月28日到我院就诊。测眼压：右眼43．3mmHg（1kPa=7．5mmHg），左眼36．3mmHg，拟诊“双眼青光眼”入院。既往无眼疾史，家族中无同类疾病患者。视力：右眼0．2，左眼0．4；双眼睑无红肿，睑球结膜无充血，双眼角膜轻度水肿，双眼整个角膜缘的角膜内皮混浊，前房闪辉（-），周边虹膜前粘连，大部分虹膜基质萎缩，可见虹膜血管及色素痣，双眼瞳孔不圆、变形移位，瞳孔缘外翻，晶状体轻度混浊，玻璃体无明显混浊。     

虹膜角膜内皮综合征六例

虹膜角膜内皮综合征六例徐珊琪虹膜角膜内皮综合征是指进行性原发性虹膜萎缩、Ｃｈａｎｄｌｅｎ综合征、ＣｏｇｅｎＲｅｓｅ综合征（虹膜痣综合征）等的总称。临床较少见。我们共遇到６例，男１例，女５例；年龄３０～６１岁。其中进行性原发性虹膜萎缩２例，Ｃｈａｎｄ...     

虹膜角膜内皮综合征临床研究进展

虹膜角膜内皮综合征临床研究进展山东枣庄市立医院眼科姚晓岚北京同仁医院眼科邓慧娟指导虹膜角膜内皮综合征是一种罕见病，简称ＩＣＥ综合征．它包括进行性虹膜萎缩Ｃｂａｎｄｌｅｒ综合征及Ｃｏｇｕ－Ｒ。ｅ综合征三种主要临床类型．一、历史及命名１８８６年Ｊｏｌｌｌ...     

虹膜角膜内皮综合征1例

1病例报告患者,男,31岁,右眼视力逐渐下降10mo,伴胀痛5mo,来我院就诊,以“右眼虹膜角膜内皮综合征”收入院治疗。眼部检查：PE：Vod0．1,     

共焦显微镜在不典型虹膜角膜内皮综合征诊断中应用

目的 探讨共焦显微镜检查在虹膜角膜内皮综合征临床诊断中的应用价值.方法 对8例(其中6例单眼患病,2例双眼患病)常规检查无法确诊的疑似虹膜角膜内皮综合征病例进行共焦显微镜检查.观察角膜内皮层病变结构.结果 活体共焦显微镜检查发现患眼角膜内皮细胞均偏大而且形状不规则,细胞核反光明显增高,可见散在双核、多核、偏位核及分叶核等改变,7例细胞边界模糊,部分病例有局灶内皮细胞缺失、胞内环形暗区、分裂状细胞等改变.此外,3例单眼患病病例的对侧眼在共焦显微镜下发现内皮细胞密度下降、大小不均等改变.最后确诊为原发性进行性虹膜萎缩型2例、Chandler综合征4例、Cogan-Reese综合征2例.结论 共焦显微镜可以在细胞水平上无创、高分辨率地观察到虹膜角膜内皮综合征患眼角膜内皮层特征性的显微结构改变,且不受角膜水肿的影响,大大提高了虹膜角膜内皮综合征诊断的准确性,并有助于早期诊断,具有很高的应用价值.     

In-vivo confocal microscopy of iridocorneal endothelial syndrome

Background: We carried out a study by in-vivo confocal microscopy to investigate the appearance of iridocorneal endothelial (ICE) syndrome, and discuss its diagnostic potential. Methods: Twelve patients, each with unilateral ICE syndrome, had both their eyes examined by in-vivo confocal microscopy. The images were recorded and analyzed by the use of proprietary software. Endothelium density, average endothelial area, coefficient of variation of cell size, percentage of hexagonal cells, and nerve fiber diameter were measured in both the anterior and posterior stroma. Corneal thickness was also measured for both eyes. A non-parametric test was used to compare differences between the affected eye and the contralateral healthy one. Results: In-vivo confocal microscopy highlighted two main patterns of abnormal “epithelioid-like” endothelium, both characterized by marked hyperreflective nuclei and loss of regularity in cellular size and shape. The first pattern was relatively regular cell size and shape, conserving a pattern similar to that of normal endothelial cells. However, the cells lost normal hexagonality and presented prominent uniform “cobblestone-like” nuclei occupying the central area of the cells. The second type was more irregular in cellular size and shape, with hyperreflective diversely shaped nuclei adjacent to the boundaries of the cells. Cells with two nuclei could be found in both types. Compared with the contralateral eye, the stromal nerve fibers in affected eyes were unusually thicker and distorted. Nerve diameters in the anterior stroma of affected eyes and contralateral eyes were 5.7 ± 0.5 μm and 3.2 ± 0.2 μm, respectively; those in the posterior stroma were 10.8 ± 0.3 μm and 6.6 ± 0.4 μm, respectively (both P < 0.001). Conclusions: Application of confocal microscopy indicates that ICE syndrome is characterized by pleomorphic epithelioid-like endothelial cells with hyperreflective nuclei. The technique has great potential in diagnosing ICE syndrome, especially in cases with corneal edema.     

虹膜角膜内皮综合征114例临床特征分析

目的分析虹膜角膜内皮(ICE)综合征患者的临床特征。方法回顾性病例系列研究。收集2014年1月至2020年5月在温州医科大学附属眼视光医院诊断为ICE综合征患者的临床资料。记录患者的临床类型、生活视力、临床特征及继发性青光眼情况。采用广义估计方程中的Wald χ2检验等方法对3种临床类型的临床特征进行比较。结果共纳入114例(127只眼)ICE综合征患者, 其中女性64例, 男性50例;就诊年龄为(49±13)岁。Chandler综合征(CS)53例(46.5%), 进行性虹膜萎缩(PIA)36例(31.6%), Cogan-Reese综合征(CRS)24例(21.0%), 无法明确临床类型1例(0.9%)。101例(88.6%)患者为单眼发病。在CS、PIA及CRS中分别有81.7%(49/60)、56.1%(23/41)及41.7%(10/24)患眼的生活视力低于0.3;角膜水肿在CS中最多见(52.5%, 32/61), 其次是PIA(29.3%, 12/41)以及CRS(20.8%, 5/24);瞳孔异位在CS、PIA、CRS中分别占78.7%(48/61)、95.1%(39/...     

单眼虹膜角膜内皮综合征患者对侧眼的临床表现

目的　了解单眼虹膜角膜内皮 (iridocornealendothelial,ICE)综合征患者对侧眼的临床表现。方法　观察有病历记录的 42例临床确诊为单眼ICE综合征患者对侧眼的临床表现。对其中 1 9例ICE综合征患者对侧眼的屈光状态、眼压、裂隙灯检查、房角镜检查、超声生物显微镜检查结果进行分析。并将 42例中的 35例ICE综合征患者对侧眼的角膜内皮细胞计数结果与 35例年龄匹配的正常人角膜内皮计数进行比较 ,t检验分析两组间角膜内皮细胞密度、六角形细胞比例及细胞大小变异度的差异。结果　在 1 9例ICE综合征患者的对侧眼中 ,88 9%为正视眼或远视眼 ,1例高眼压 ,50 0 %(6/ 1 2 )前房深度 <2 2mm ,3例虹膜萎缩或发育欠佳 ,3例前房角窄Ⅱ～窄Ⅲ和 (或 )前房角粘连关闭 ,2例前房角锥状粘连 ,1例视野检查发现相对旁中心暗点 ,31 6 %病例合并全身性疾病。 35例ICE综合征患者对侧眼的平均角膜内皮细胞密度为 (2 593 4± 432 7)个 /mm2 ,低于正常对照组 (2 784 9± 2 80 6)个 /mm2 ,两者比较差异有显著意义 (t=- 2 43 ,P =0 0 1 9) ;六角形细胞的百分率为 (56 5± 6 4) % ,低于正常对照组的 (58 8± 5 7) % ,两组比较差异无显著意义 (t=- 1 0 0 5 ,P =0 332 ) ;细胞大小变异度为 0 332± 0 0 3 ,低于正常对照组     

A case of atypical Cogan's syndrome with uncommon corneal findings.

PURPOSE: We report a case of atypical bilateral interstitial keratitis associated with Cogan's syndrome. METHODS: A 28-year-old man presented with a 2-year history of recurrent bilateral keratitis. Bilateral hearing loss preceded the ocular symptoms by 2 years. The patient also complained of skin nodules, headache, back pain, and arthritis. Corneal finding were consistent with superior stromal keratitis with stromal neovascularization and lipid deposition in the stroma. The patient's audiogram revealed cochlear pathology compatible with Cogan's syndrome (sensorineural deafness). RESULTS: The patient was treated with topical steroids but eventually required corneal transplantation in the right eye as a consequence of progressive loss of vision secondary to progressive lipid keratopathy. Visual acuity at the patient's most recent follow-up evaluation was 20/40. CONCLUSION: This case represents an unusual type of interstitial keratitis associated with Cogan's disease. The absence of ocular symptoms at the time of initial ear involvement and the atypical presentation of the keratitis were responsible for the delay in diagnosis in this patient, resulting in hearing impairment.     

Vogt-小柳-原田综合征漏误诊分析

目的：分析我国Vogt-小柳原田（VKH）综合征临床诊断中存在的问题。方法：对1996年1月－2000年12月于中山眼科中心葡萄膜炎专科就诊的,以往资料完整的123例VKH综合征患者的临床资料。特别是漏诊,误诊,发病至确诊时间和以往诊断等进行分析。结果：123例VKH综合征患者的盲目发生率为28．9％,在后葡萄膜炎期,前葡萄膜受累期和以前葡萄膜炎反复发作为特征的全葡萄膜炎期的漏诊,误诊率分别为96．7％,86．2％和74．0％,眼部发病至确诊时间平均为24．5个月。结论：我国VKH综合征患者的漏诊和误诊十分常见,诊断水平亟待提高。     

Familial ophthalmoplegia-plus syndrome with corneal endothelial disorder

The authors report two brothers with an ophthalmoplegia-plus syndrome characterized by chronic progressive external ophthalmoplegia associated with hypogonadism, primary testicular failure, neural deafness, cone-rod degeneration and mild optic atrophy. One brother, the proband, was a daily soft contact lens wearer who developed persistent epithelial and stromal edema of the right cornea and had bilaterally thickened corneas by ultrasonic pachymetry. Endothelial studies of both brothers revealed marked polymegathism, and pleomorphism. Stressing the inherently abnormal endothelium with contact lens wear may have resulted in further endothelial compromise and corneal edema in the proband.

Endothelial abnormalities may be another clinical finding within the spectrum of ophthalmoplegia-plus syndrome and should be searched for before fitting these individuals with contact lenses.     

急性视网膜坏死综合征的误诊分析

目的 分析急性视网膜坏死综合征(Acute retinal necrosis syndrome,ARN)临床诊断中存在的问题.方法 收集1998年1月至2006年10月中山眼科中心确诊住院治疗、以往资料完整的114例ARN病人的临床资料,重点分析初诊时误诊、漏诊原因,发病至确诊时间、确诊前诊断、治疗等情况.结果 114例(141眼)ARN病人的盲目率(42/141)为29.79%;初诊时,Ⅰ、Ⅱ、Ⅲ-Ⅳ期误诊率分别为61/70(87.14%)、23/25(93.0%)、13/19(73.68%),发病至确诊时间最短为3 d,最长为420 d,平均为46.16 d.结论 ARN综合征漏诊、误诊率较高,急需提高ARN的早期确诊率使病人保存部分有用视力.