Congen—Reese综合征一例

虹膜角膜内皮（iridocorneal endothelium,ICE）综合征是波及角膜内皮、前房角、虹膜等眼前节组织的一组疾病,包括进行性虹膜萎缩、Chandler综合征、Cogen—Reese综合征。ICE共同的临床特点有角膜水肿、进行性前房角粘连、虹膜组织的破坏和眼压升高等。Congen—Reese综合征是ICE的一个临床类型,特点是具有典型的虹膜色素痣样结节、角膜水肿、瞳孔异位和虹膜萎缩。现将我们诊断的Congen—Reese综合征1例报告如下。     

虹膜角膜内皮综合征研究进展

虹膜角膜内皮综合征(ICE综合征)是一组累及虹膜、角膜、前房角的致盲性疾病。该疾病多单眼发病,少数为双眼,表现为角膜内皮异常进行性虹膜基质萎缩、虹膜周边前粘连、房角关闭以及继发性青光眼的一组疾病。临床上分为原发性进行性虹膜萎缩、Chandler综合征、Cogan-Reese综合征。本文就其病因、病理、临床表现、治疗的研究进展进行综述。     

虹膜角膜内皮综合征误诊为原发性开角型青光眼一例

虹膜角膜内皮综合征（iridocorneal endothelial syndrome,ICE综合征）是角膜内皮异常、进行性虹膜基质萎缩、广泛周边虹膜前粘连、前房角关闭及继发性青光的一组疾病,其病因及发病机制目前尚不清。根据虹膜情况,     

虹膜角膜内皮综合征3例

0引言 虹膜角膜内皮综合征（ICE）是以角膜内皮细胞变性、虹膜基质异常及继发青光眼为主要改变的一组疾病，临床少见，预后欠佳，最终以眼压失控及角膜内皮失代偿而致失明。早期诊断，合理治疗可延缓病程的发展，提高患者生活质量。现将临床上遇到的3例报告如下。     

虹膜角膜内皮综合征研究进展及治疗现状

原发性进行性虹膜萎缩、Chandler综合征、虹膜色素痣综合征 (Cogen- Reese综合征 )以往分别作为独立临床现象来考虑其所发生的虹膜萎缩 ,1979年 Yanoff将此三种疾患统称为虹膜角膜内皮综合征 (iridocorneal endothelialsyndrom e,简称 ICE综合征 ) [1 ,2 ]。 ICE综合征临床上并不多见 ,但由于它们特征性的眼部表现并经常引起继发性青光眼而受到重视。现就 ICE综合征的临床及病理特点、发病机理、鉴别诊断、治疗综述如下。一、临床及病理特点ICE综合征在临床上比较少见 ,自 190 3年 Harm s[3]首次报告原发性虹膜萎缩后 ,全世界约有 2 …     

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     

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     

八例虹膜角膜内皮综合征的临床观察与分析

目的 :探讨虹膜角膜内皮 (ICE)综合征的发病机制。方法 :对 8例 ( 1 0只眼 )ICE综合征患者的临床表现、分型及治疗效果进行分析和数据统计。并介绍目前国内外一些新的研究进展。结果 :8例ICE综合征患者临床表现为三型 :原发性进行性虹膜萎缩 7只眼 ,Chandler氏综合征 1只眼 ,Cogan Reese综合征 2只眼。 1 0只眼均继发闭角型青光眼 ,2只眼并发大泡性角膜病变。 9只眼行一次或多次滤过手术 ,1只眼行角膜移植术。结论 :ICE综合征的基本病变是角膜内皮层存在的ICE细胞 ,它们的过度增生导致房角粘连、虹膜萎缩、继发性青光眼和大泡性角膜病变。目前滤过手术及角膜移植术只能在一定程度上缓解眼压及改善角膜质量 ,但不能根本有效地阻止ICE细胞的扩散。     

虹膜角膜内皮综合征继发青光眼1例

虹膜角膜内皮综合征(iridocorneal endothelial syndrome,ICE综合征)是一组临床上少见且愈后较差的眼病,以角膜内皮异常、虹膜基质萎缩、虹膜周边前粘连、房角关闭及继发青光眼为主要特征。因发病率较低,发病机制复杂,目前尚待进一步研究。现将我院收治的1例ICE综合征继发青光眼的病例分析如下。患者女性,46岁。入院前1个月患者无明显诱因出现     

虹膜角膜内皮综合征的研究进展

虹膜角膜内皮综合征(iridocorneal endothelial syndrome， ICES)是一种罕见的眼部疾病，是一组以角膜内皮、前房角和虹膜的结构及增生异常为特征的疾病。常见的临床特征包括角膜水肿、继发性青光眼、虹膜萎缩、瞳孔异常等。常发生于年轻女性，多单眼发病。其发病机制尚不明确，症状多样，且致盲率高。该疾病诊断困难，且尚无理想治疗方法。本文旨在回顾有关ICES的特点、诊断及治疗等方面的文献，以期对疾病的诊治提供帮助。     

不典型的虹膜角膜内皮综合征1例

虹膜角膜内皮综合征是一种少见的眼科疾病,多为单眼发病,女性多见,因临床表现多样误诊漏诊率较高。本例患者表现为难以控制的高眼压,角膜水肿导致角膜内皮形态不易观察,且伴有虹膜少量新生血管,青光眼手术前未能明确诊断,术后角膜清亮后进一步完善检查,最终确诊为虹膜角膜内皮综合征（Chandler综合征）。     

The iridocorneal endothelial syndrome

The iridocorneal endothelial syndrome represents a unique group of ocular pathologies (Chandler syndrome, progressive iris atrophy, and Cogan-Reese syndrome) characterized by the proliferation of corneal endothelial cells that migrate toward the iridocorneal angle and iris surface causing, to a degree varying according to the subtype, corneal edema and decompensation and secondary glaucoma, whether by obstructing the angle or producing peripheral anterior synechiae by contraction of the basement membrane of the migrating cells over the surface of the iris. A triggering factor, possibly viral, induces the corneal endothelial cells to proliferate and behave like epithelial cells. Diagnosis is made based on typical ocular findings on the cornea and iris. Iridocorneal endothelial syndrome is more frequent in young women, with unilateral involvement in most cases. In vivo confocal microscopy is an excellent diagnostic tool, especially in borderline presentations like early cases of Chandler syndrome, which affects the cornea predominantly. Typical clinical management consists of treating the corneal edema and decompensation, where endothelial keratoplasty techniques have replaced in many cases the need for a penetrating keratoplasty and treating the secondary glaucoma, which usually requires surgical intervention.     

虹膜角膜内皮综合征的治疗新进展

虹膜角膜内皮综合征（ iridocorneal endothelial syndrome, ICES）是临床上比较少见的眼部疾病之一,其发病机制尚不明确,症状复杂,有很高的致盲率。患者常因青光眼、视力减退、眼痛和虹膜异常来就诊。 ICES至今尚无理想的治疗方法,临床上只能针对其并发症采取相应的药物、手术治疗。本文将对近期有关ICES治疗进展的文献进行综述,同时对极易出现误诊的疾病进行鉴别,以期对ICES的治疗提供帮助。     

Iridocorneal endothelial syndrome and glaucoma

Iridocorneal endothelial (ICE) syndrome is a group of ocular conditions characterized by corneal proliferative endotheliopathy in which secondary corneal edema, peripheral anterior synechiae, and abnormalities of the iris stroma are the common features. The etiology remains unclear, but may be related to viral infection with Herpes simplex or Epstein-Barr virus. The pathogenesis of the ICE syndrome is believed to result from an abnormality of the corneal endothelial cells (causing corneal edema), with secondary spreading of the cells over the trabecular meshwork region (causing anterior synechiae and elevated intraocular pressure [IOP]) and across the surface of the iris (responsible for the formation of iris holes, pupillary distortion, and iris noduli). The disease complex, which includes essential iris atrophy, Chandler's syndrome, and iris nevus (Cogan-Reese) syndrome, is almost always unilateral, nonfamilial, and typically occurs in females during young adulthood. ICE syndrome is commonly progressive and frequently complicated by secondary glaucoma and corneal decompensation. In Chandler's syndrome, iris changes are less pronounced and corneal edema more frequent than in essential iris atrophy or Cogan-Reese syndrome. Glaucoma associated with ICE syndrome is often difficult to manage and is usually treated with medications and/or filtering surgery. Glaucoma filtering surgery is usually successful when done early, but may fail due to endothelialization of the fistula by the abnormal corneal endothelium.     

Cirugía combinada de catarata y queratoplastia lamelar endotelial en el síndrome iridocórneo endotelial

The case is reported of a 59-year-old woman, who reported decreased visual acuity (VA) in the left eye (LE). On examination, a corrected VA of finger count/ 30 cm was observed in LE. Corneal oedema and folds in the Descemet's membrane were observed using the slit lamp. Anterior synechiae, iris atrophy, and corectopia were also found. Iridocorneal endothelial syndrome (ICE) was diagnosed. The treatment option was a combination of cataract surgery and automated endothelial keratoplasty with Descemet's membrane dissection (DSAEK). There were no complications during the operation, with a successful anatomical and functional recovery, with a VA of 0,8 being observed after one year. This result supports the efficacy of DSAEK in ICE syndrome, encouraging more studies to be carried out that should also support its efficacy in this syndrome.     

Corneal endothelial cell changes after cataract surgery in patients on systemic sympathetic α‐1a antagonist medication (tamsulosin)

Purpose: The purpose of this study was to assess the incidence of intraoperative floppy iris syndrome (IFIS) and the morphology of the corneal endothelium after cataract extraction in Caucasian male patients exposed to the α‐1_a adrenergic receptor antagonist tamsulosin. Methods: In a clinical prospective study, 23 male patients (23 eyes) treated with tamsulosin due to benign prostatic hyperplasia and 25 male patients (25 eyes) with no tamsulosin treatment had cataract surgery. The divide‐and‐conquer technique was used with the Infinity OZil^® machine. A combination of Healon and Healon5 was used in all patients, but the use of additional Vision Blue, iris retractors or intracameral phenylephrine in the tamsulosin group was at the discretion of the surgeon. The endothelial cell density, variation in endothelial cell size (CV), percentage of hexagonal cells and central corneal thickness (CCT) were recorded at baseline and at 3 months postoperatively. Results: In the tamsulosin‐treated group, 19 of 23 eyes (83%) developed IFIS, compared with no IFIS in the control group. Compared with the control group, the tamsulosin group showed significantly less dilatation at the start of the operation, significant miosis during surgery and significantly greater corneal endothelial cell loss 3 months postoperatively (12% versus 3%; p < 0.001). Conclusion: Intraoperative floppy iris syndrome during cataract surgery is significantly associated with tamsulosin‐treated male patients. Patients on tamsulosin showed less preoperative dilatation, significant miosis during surgery, and had significantly greater postoperative endothelial cell loss compared with nontreated patients despite recommended precautions.     

不同类型白内障囊外摘除术（ECCE）对核硬度≥IV级伴低角膜内皮细胞密度白内障患者的疗效分析

目的　对比并分析单切口和双切口白内障囊外摘除术（extra capsular cataract extraction,ECCE）对核硬度≥IV级伴低角膜内皮细胞密度白内障患者的疗效。方法　回顾性分析我院2017年1月至2019年12月收治的晶状体核硬度≥IV级且角膜内皮细胞密度<1000 个·mm^-2的白内障患者共92例94眼的临床资料,其中46例46眼采用双切口ECCE治疗设为A组,46例48眼采用单切口ECCE治疗设为B组。术后随访6个月,比较两组手术时间及手术前后患者视力、散光度、角膜内皮细胞密度、角膜内皮细胞丢失率、六边形细胞比例及并发症发生情况。结果　两组患者术前基线资料、手术时间及术后视力、术后散光度、术源性散光度等相比,差异均无统计学意义（均为P>0.05）。术后6个月,A组角膜内皮细胞密度为（780.73±110.14）个·mm^-2,显著多于B组的（706.15±84.07）个·mm^-2,差异有统计学意义（P=0.00）;A组角膜内皮细胞丢失率和六边形细胞比例分别为（4.08±0.52）%和（10.14±6.60）%,均显著少于B组的（10.89±1.40）%和（27.86±9.53）%,差异均有统计学意义（均为P=0.00）。随访期内,A组患者术后角膜水肿发生率为8.70%,显著低于B组患者的37.50%（P<0.05）;A组未见大泡性角膜病变,角膜内皮细胞密度＜600个·mm^-2者4眼;B组6眼出现大泡性角膜病变,未见角膜内皮细胞密度＜600个·mm^-2者。结论　相较于单切口ECCE,双切口ECCE治疗核硬度≥IV级伴低角膜内皮细胞密度白内障患者可有效保护角膜内皮细胞,降低术后早期角膜水肿发生风险,且两种手术方式整体疗效接近。     

A review of the evidence for in vivo corneal endothelial regeneration

Human corneal endothelium has long been thought to be a nonmitotic cell layer with no endogenous reparative potential. Pathologies that damage endothelial function result in corneal decompensation and, if untreated, blindness. The mainstay of treatment involves partial or complete corneal replacement, amounting to 40% of all corneal transplants performed worldwide. We summarize the case reports describing complications postoperatively in the form of (sub)total graft detachment and those resulting in postoperative bare stroma. Complications during cataract and glaucoma surgeries leading to an uncovered posterior cornea are also included. We discuss the newer treatment strategies that are alternatives for current Descemet membrane endothelial keratoplasty and Descemet stripping automated endothelial keratoplasty, including partial grafts and stripping of the diseased cell layer. In more than half of the cases reviewed, corneal transparency returned despite incomplete or no corneal endothelial cell transplantation. We question the existing paradigm concerning corneal endothelial wound healing in vivo. The data support further clinical study to determine the safety of simple descemethorexis in central endothelial pathologies, such as Fuchs endothelial corneal dystrophy, where presence of healthy peripheral cells may allow successful corneal recompensation without the need for donor cells.     

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。