Cytokeratin expression in corneal endothelium in the iridocorneal endothelial syndrome.

The immunocytologic characteristics of two formalin-fixed, paraffin-embedded corneas from patients with the iridocorneal endothelial (ICE) syndrome and unaffected control corneas were studied. Binding of polyclonal antisera to Factor VIII, S-100 protein, involucrin, neuron specific enolase (NSE), and the lectins peanut agglutinin and Ulex europaeus agglutinin-1 was performed using the standard peroxidase-anti-peroxidase method. We detected reactive patterns of monoclonal antibodies to cytokeratins (34BE12 is a 56-58 kD mouse IgG reactive to stratified epithelia; Pkk1 is a 44-54 kD mouse IgG reactive to simple epithelia; and KL1 is a 55-57 kD mouse IgG reactive to epidermis and simple epithelia) using the standard avidin-biotin complex method. Staining properties were similar for the polyclonal antisera, lectins, NSE, and chromogranin in corneas with ICE syndrome and in the controls. However, the cytokeratins 34BE12, Pkk1, and KL1 were detected in the endothelium of the corneas with the ICE syndrome but not in the controls. These findings suggest that various cytokeratins are expressed in the corneal endothelium in the ICE syndrome that are not expressed in unaffected corneal endothelium.     

Progression and regression of partial corneal involvement in the iridocorneal endothelial syndrome.

We observed three women with partial corneal involvement in the iridocorneal endothelial syndrome for more than ten years. During this time, the peripheral anterior synechiae progressed in all three, with one patient developing glaucoma. In two patients, the abnormal endothelial cells spread to cover the entire posterior corneal surface; in the third, they disappeared almost entirely. The endothelial permeability to fluorescein remained abnormally low only in the two eyes with diffusely abnormal endothelium, and increased to normal in the third eye as the abnormal endothelium disappeared. The permeability in the third eye had been abnormally low only in the superior half of the cornea, where the abnormal endothelium was located. As the abnormal endothelium regressed, it was replaced by normal endothelium with a normal cell density similar to that of the unaffected fellow eye. Thus, for more than ten years, the partial endothelial involvement associated with the iridocorneal endothelial syndrome progressed substantially in two patients and regressed dramatically in a third.     

Descemet membrane endothelial keratoplasty in iridocorneal endothelial syndrome and posterior polymorphous corneal dystrophy

Objective

To report the clinical outcome of Descemet membrane endothelial keratoplasty (DMEK) in cases of corneal decompensation secondary to iridocorneal endothelial syndrome (ICE) or posterior polymorphous corneal dystrophy (PPCD).

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。     

Descemet's stripping with endothelial keratoplasty in iridocorneal endothelial syndrome.

An interventional case is described to report on the clinical outcome of Descemet's stripping automated endothelial keratoplasty performed for iridocorneal endothelial syndrome. A Descemet's stripping automated endothelial keratoplasty was performed in the eye of a 53-year-old woman with decompensated cornea secondary to iridocorneal endothelial syndrome. The transplant and the angle were evaluated with optical coherence tomography. Within 10 months postoperatively, the graft was clear and best-corrected visual acuity improved from 20/400 to 20/30. Descemet's stripping automated endothelial keratoplasty appears to be an effective measure to treat corneal decompensation, improve vision, and facilitate the examination of the optic disc and retina in patients with iridocorneal endothelial syndrome.     

共焦显微镜观察虹膜角膜内皮综合征及其继发性青光眼的手术治疗

目的应用共焦显微镜检查探讨虹膜角膜内皮综合征的临床特征及其继发性青光眼的手术治疗。方法 8例（8眼）虹膜角膜内皮综合征行共焦显微镜下双眼检查;描述角膜内皮的病变结构特点,分析双眼角膜内皮细胞密度、内皮细胞平均面积、六角形细胞比例及中央角膜厚度,并对6例继发性青光眼中的5例施行手术治疗。结果患眼角膜内皮细胞呈风筝样或上皮细胞样改变;细胞排列紊乱,大小不均;细胞内可见高反光的细胞核,部分可见双核。患眼和对侧眼的角膜内皮细胞密度、内皮细胞平均面积、六角形细胞比例分别为：789.7±75.8个/mm^2、2223.7±80.6个/mm^2;1106.9±89.4μm^2、379.8±20.6μm^2;17.2±1.2%、56.3±1.7%,下降明显。二者的中央角膜厚度相当。4例继发性青光眼手术后眼压控制正常,另1例手术无效。结论共焦显微镜能够发现ICE患者的角膜内皮细胞的特征性改变;对该病的诊断具有很高的临床意义。并发继发性青光眼要及时手术治疗控制眼压。     

Confocal microscopy in the iridocorneal endothelial syndrome

AIMS: To report the appearances of iridocorneal endothelial (ICE) syndrome from real time, white light confocal microscopy. METHODS: Three consecutive patients, each with ICE syndrome, were examined prospectively. Corneal specular and confocal microscopic examinations were performed in all three patients. In the first patient, a penetrating keratoplasty was performed and the cornea was examined by light and scanning electron microscopy. No surgery was performed in the remaining two patients. RESULTS: In the first patient corneal oedema prevented endothelial specular microscopy. Confocal microscopy performed before penetrating keratoplasty successfully revealed abnormal epithelial-like endothelial cells. Histological examinations of the cornea following penetrating keratoplasty revealed the presence of multilayered endothelial cells with epithelial features (microvilli). In the remaining two patients, specular microscopy showed the presence of ICE cells with typical dark/light reversal. Confocal microscopy demonstrated groups of endothelial cells with epitheloid appearances. In all three patients, the contralateral endothelial appearance was normal by specular and confocal microscopy, except for moderate endothelial polymegathism in one patient. Epithelial-like endothelial cells were characterised by prominent nuclei on confocal microscopy. CONCLUSIONS: The application of confocal microscopy indicates that the ICE syndrome is characterised by epitheloid changes in the endothelium. Confocal microscopy may be used to diagnose the ICE syndrome by demonstrating epithelial-like endothelial cells with hyperreflective nuclei. This technique is especially of value in cases of corneal oedema, since specular microscopy may fail to image the endothelium in such cases.     

Soft and RGP lens corneal swelling and deswelling with overnight wear

An overnight study was performed to compare the corneal swelling and deswelling for two very different modes of extended wear. A soft disposable lens, Acuvue (Johnson & Johnson), and a high Dk RGP lens, Super EX (Menicon), were investigated. A contact lens-inexperienced group participated in this study. Each subject wore the soft lens on one eye and the RGP on the other. The control overnight swelling was 3.7%. Overnight swelling for the RGP lens was 6.5%, and for the soft lens, 10.0%. Corneal deswelling time to the level of a 10 a.m. baseline with no lens wear occurred within 2 hours for the control. Corneal deswelling time for the RGP lens occurred in 3 hours, and for the soft lens, between 7 and 9 hours. Corneal deswelling time to the level of the control condition showed the RGP lens condition to deswell within 4 hours. By this same analysis, the soft lens condition did not return to control levels even by the end of the study.     

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。     

A new iridocorneal endothelial syndrome

Three iridocorneal endothelial syndromes are distinguished: essential progressive iris atrophy, iris nevus (Cogan-Reese) syndrome, and Chandler's syndrome. Obviously, there are patients in whom findings do not correspond fully to any one of the three syndromes; signs and symptoms which justify being considered as a new iridocorneal endothelial syndrome are described.     

Penetrating keratoplasty in iridocorneal endothelial syndrome

PURPOSE: To evaluate the clinical outcome of penetrating keratoplasty (PK) in iridocorneal endothelial (ICE) syndrome. METHODS: Clinical charts of patients who underwent penetrating keratoplasty for ICE syndrome between 1985 and 1999 were reviewed retrospectively. Glaucoma control, best corrected visual acuity pre- and post-PK, graft clarity, graft rejection episodes, improvement in pain, and additional procedures were analyzed. RESULTS: Fourteen cases were reviewed with an average follow-up of 58 months after PK. Initial grafts failed in seven patients (50%), in six cases because of rejection, and one owing to endothelial failure without signs of rejection. Repeat PKs were performed in six patients. At final follow-up, 12 grafts were clear. Glaucoma was controlled pre- and post-PK (average intraocular pressure, 16 mmHg for both eyes). Pre-PK, eight patients were using glaucoma medicines and nine had had glaucoma surgery. At the end of the follow-up, seven patients were using glaucoma medicines; six patients required glaucoma surgery after their initial PK. At the final follow-up visit, visual acuity in three patients (21%) was 20/40 or better, it ranged from 20/50 to 20/100 in four patients (29%) and 20/200 to 20/400 in five patients (36%), and in two patients with failed grafts (14%) it was counting fingers or worse. CONCLUSION: Clear grafts were achieved in 12 cases, although six patients (43%) underwent repeat PKs. All patients had glaucoma, which was controlled before and after PK by medical treatment and surgical procedures. Favorable outcomes can be achieved in patients with ICE syndrome but may require multiple corneal and glaucoma procedures.     

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。     

Results of penetrating keratoplasty in the iridocorneal endothelial syndrome

Five patients (four men and one woman ranging in age from 30 to 41 years) with the iridocorneal endothelial syndrome (four with Chandler's syndrome and one with essential iris atrophy) underwent penetrating keratoplasty for complaints relating to diminished visual acuity or pain. After follow-up periods ranging from one year to four years and seven months (average, 2.7 years), postoperative visual acuities ranged from 20/15 to 20/30. No evidence of recurrence of the corneal abnormalities observed before grafting was apparent in the donor corneas at the last examinations. Two patients who required medical treatment for intraocular pressure control preoperatively also required adjuvant therapy postoperatively. These results suggested that penetrating keratoplasty is a relatively safe and effective procedure for patients with diminished vision or other complaints related to corneal abnormalities in the iridocorneal endothelial syndrome. It does not, however, restore to normal the iris and angle structures affected adversely by the progression of the corneal endotheliopathy and therefore other measures may be required to control intraocular pressure.     

Corneal endothelial cell abnormalities in an early stage of the iridocorneal endothelial syndrome.

A corneal disc, obtained from a 52-year-old woman suffering from an early stage of the iridocorneal endothelial syndrome (ICE), was investigated by various morphological techniques to analyse the structural variations in the endothelial cells and to identify the collagen types within the abnormal layer of Descemet's membrane. Scanning electron microscopy of the posterior corneal surface revealed a mosaic of (a) flat hexagonal cells resembling irregular but normal endothelial cells, and (b) rounded hexagonal (ICE) cells with numerous surface microvilli. Degenerative changes were present in each cell type, but were more common in the flat hexagonal cells which contained intracytoplasmic spaces. By transmission electron microscopy the flat hexagonal cells exhibited many of the features of normal endothelial cells in terms of organelles and intercellular attachments, but lateral invaginations were absent. The ICE cells differed in that the apical surface was covered by microvilli and the cytoplasm contained tonofilaments, which were also observed by light microscopic immunocytochemical staining. Most commonly, intercellular attachments were rudimentary in both types of cell and intercellular spaces were dilated, but desmosomes were sometimes prominent in the ICE cells where interdigitations were pronounced. In some sectors, the basal surface of the ICE cells was indented by deposition of clumps of fibrillar collagenous material. An immunocytochemical study of the abnormal posterior deposits localised type IV collagen to the amorphous matrix and collagen types III and V, but not type I, to the collagen fibril bundles. Mononuclear inflammatory cells were identified between the ICE cells in the monolayer. The evidence suggests that some of the flat hexagonal cells were undergoing a degenerative change while others were transforming into ICE cells.     

5-Fluorouracil after trabeculectomy and the iridocorneal endothelial syndrome

Nine patients (nine eyes) with iridocorneal endothelial (ICE) syndrome underwent trabeculectomy and received postoperative subconjunctival injections of 5-fluorouracil (5-FU) to enhance bleb formation (total 5-FU dose, 30-105 mg; mean, 53.8 mg). Eight eyes had undergone prior unsuccessful trabeculectomy. Four eyes had intraocular pressure (IOP) less than or equal to 21 mmHg on zero to two glaucoma medications after 6 to 54 months of follow-up (mean, 25.3 months). Five eyes required repeat surgery within 2 to 13 months and were considered failures. All five eyes that failed received a Molteno drainage implant. Progressive endothelial proliferation may explain late onset bleb failure and the relative ineffectiveness of 5-FU in this condition.     

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.