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.     

Iridocorneal endothelial syndrome (ICE-S): classification, clinical picture, diagnosis

The iridocorneal endothelial (ICE) syndrome includes progressive essential iris atrophy, the Cogan-Reese syndrome, Chandler's syndrome and mixed forms. The term ICE-S was proposed by Scheie and Yanoff in 1975 and by Yanoff in 1979. The capacity of migration of the abnormal corneal endothelial cell layer across the anterior chamber angle, and on to the anterior surface of the iris, possible on to the back surface of the iris and across the zonula fibers is responsible for corneal edema, secondary glaucoma, nevi, noduli and atrophy of the iris, and pupillary distortion. The contraction of the migrated membrane-like ICE tissue produces holes in the iris. The diseases are usually unilateral in young patients. The etiology is still not clear. Theories include membrane formation, low grade of inflammation and viral infection with Herpes simplex or Epstein-Barr virus. Glaucoma and edema of the cornea are the main therapeutic problems.     

Clinical and specular microscopic manifestations of iridocorneal endothelial syndrome

PURPOSE: To investigate the correlation between the clinical pictures and the specular microscopic findings in patients with iridocorneal endothelial (ICE) syndrome. METHODS: The records of 15 patients with ICE syndrome who presented at the National Taiwan University Hospital between 1993 and 1996 were examined. The medical history, clinical pictures of the cornea, iris and anterior chamber angle, intraocular pressure, specular microscopic findings, and the correlation between clinical and specular microscopic findings were assessed. RESULTS: Endothelial changes in specular micrographs were found in all the patients, even in those patients with minimal angle involvement by peripheral anterior synechiae. Corneal decompensation resulting in corneal edema and bullae formation was the main cause of visual impairment. Neither ICE grading nor endothelial cell density correlated with corneal edema or intraocular pressure, but they correlated with the angle involvement in ICE syndrome. The intraocular pressure was difficult to control in 8 of these patients, even after treatment with anti-glaucoma agents and trabeculectomy, especially in the patients with Cogan-Reese syndrome. CONCLUSION: Although specular microscopy provides an invaluable method for the diagnosis of ICE syndrome, it is not a reliable tool for predicting prognosis. Close follow-up of intraocular pressure and early detection of glaucoma are important steps to preserve visual functions in patients with ICE syndrome.     

Clinicopathologic features of Chandler's syndrome

The clinicopathologic features of Chandler's syndrome are elucidated based on a study of nine patients. Keratoplasty, trabeculectomy and/or iris specimens were studied by light, electron and, in six cases, specular microscopy. The prominent pathologic features were abnormalities of the endothelially-derived cells lining the posterior corneal surface, the inner surface of the trabecular meshwork, and the anterior iris surface. In all four corneal buttons, the endothelium was diffusely attenuated and focally absent, and posterior collagen layers were present. Extension of the endothelial cell layer and Descemet's membrane deposition over the trabecular meshwork were observed in two trabeculectomy specimens, and similar proliferation onto the anterior surface of the iris was evident in four iris specimens. Other abnormalities of the endothelial cells included increased intracytoplasmic filaments. These pathologic alterations are consistent with the concept of Chandler's syndrome as representing one variant of the iridocorneal endothelial (ICE) syndrome. Interestingly, although both endothelial proliferative and degenerative responses can be observed in Chandler's syndrome, their occurrence within the same eye is apparently not simultaneous.     

Chandler syndrome--a case report

Chandler's syndrome is a particularly rare form of unilateral secondary glaucoma, in which--besides other symptoms--corneal epithelial edema occurs even when intraocular pressure rises slightly to above 20 mmHg. For 3 years now the authors have had a patient under observation whose condition corresponds to Chandler's syndrome. A remarkable feature of this case is that corneal edema occurred at physiological pressure levels. The symptoms and course are described; a brief differential diagnostic review is given and therapeutic possibilities are pointed out.     

Ultrasound biomicroscopy of Chinese eyes with iridocorneal endothelial syndrome

AIM: To document the ultrasound biomicroscopic (UBM) findings in Chinese patients with iridocorneal endothelial (ICE) syndrome. METHODS: 21 patients with ICE syndrome and 15 normal subjects underwent UBM. UBM findings of anterior segment were compared between normal subjects and three clinical types of ICE syndrome: progressive iris atrophy (PIA), Chandler's syndrome (CS), and Cogan-Reese syndrome (CRS). RESULTS: Central anterior chamber depth was significantly less in patients with ICE syndrome (2.25 (SD 0.32) mm) than in normal subjects (2.76 (0.32) mm). Peripheral anterior synechiae were observed in all the ICE patients by UBM. Three out of four CRS subjects showed an "arborised" shape of iridocorneal angle. Two eyes out of 10 with CS presented bridge-shaped synechiae. A membrane-like mound was observed in iridocorneal angle in two patients: one with CRS and one with CS. UBM was found to be more effective in detecting peripheral anterior synechiae (PAS) and iris atrophy than slit lamp microscopy and gonioscopy, mainly because of corneal oedema in patients with CS. Four out of 11 patients with unilateral ICE syndrome had shallow or closed anterior chamber angles in their fellow eyes. Two of them successfully responded to laser peripheral iridotomy. CONCLUSIONS: UBM is an effective method to reveal the anterior segment features and provides a useful tool in the diagnosis of ICE syndrome. Different subtypes of ICE syndrome may have different UBM manifestations. UBM can help to identify angle closure in the fellow eye of unilateral ICE syndromes.     

Decreased Endothelial Permeability in the Iridocorneal Endothelial Syndrome

Five patients with the iridocorneal endothelial (ICE) syndrome were examined by fluorophotometry. All patients had typical abnormal corneal endothelium, peripheral anterior synechiae, and distortion of the iris (pupillary irregularity or anterior stromal traction tears) in one eye only. Fluorescein was deposited in the superior cornea of both eyes by iontophoresis, and the cornea and anterior chamber concentrations and total mass of fluorescein were measured hourly over the ensuing eight hours. In all five patients, the endothelial permeability to fluorescein was within normal limits in the normal eye. In four of the five abnormal eyes, endothelial permeability was markedly decreased. In these four patients, the permeability to fluorescein in the normal eye was approximately six times that in the abnormal eye. In the fifth patient, the endothelial permeability was normal in both eyes. The central corneal thicknesses were normal in both eyes of all five patients. These results indicate that in many eyes with the ICE syndrome, corneal endothelial permeability to solutes is markedly decreased. Decreased endothelial permeability to solutes has not been documented previously in any clinical corneal disorder and may be of importance in the pathophysiologic changes that accompany endothelial disease.     

Descemet stripping with endothelial keratoplasty for treatment of iridocorneal endothelial syndrome

PURPOSE: To describe use of Descemet stripping with endothelial keratoplasty (DSEK) to treat corneal edema associated with iridocorneal endothelial (ICE) syndrome. METHODS: In this retrospective, consecutive, interventional case series, the corneal endothelium was selectively replaced by the DSEK technique in eyes with ICE syndrome. Three eyes were treated at 1 center between June 2005 and July 2006. Descemet membrane and endothelium were stripped from the recipient and an 8- or 8.5-mm-diameter donor button consisting of posterior stroma and healthy endothelium was folded and implanted through a 5-mm incision. An air bubble was used to press the donor tissue against the recipient cornea, allowing it to attach without sutures. In 1 case with extensive peripheral anterior synechiae (PAS), after the PAS were broken, the anterior chamber was shallow, so a temporary anchor suture was placed in the peripheral edge of the donor tissue to help ensure that it would unfold in the correct orientation. RESULTS: DSEK successfully resolved corneal edema in 3 male patients with unilateral ICE syndrome who were 47 to 67 years of age. Follow-up ranged from 1 to 14 months. Best spectacle-corrected visual acuity at the most recent visit was 20/20 to 20/30, with a mean refractive cylinder of 1.2 D. CONCLUSIONS: Selective replacement of dysfunctional endothelium with DSEK can successfully treat corneal edema and associated visual loss and pain caused by ICE syndrome. Visual recovery is rapid and refractive changes are minimal compared with replacement of the full corneal thickness with a traditional penetrating keratoplasty.     

Confocal microscopy used as the definitive, early diagnostic method in Chandler syndrome

PURPOSE: To report the early, rapid diagnosis of the Chandler variant of the iridocorneal endothelial (ICE) syndrome using confocal light microscopy. METHODS: A 62-year-old man with a long history of unilateral glaucoma reported progressively blurred vision in his right eye. Examination of both eyes included visual acuity, slit-lamp examination, pneumotonometry, visual field, gonioscopy, and confocal microscopy. RESULTS: On examination, visual acuity was 20/80 and 20/20 and the IOPs were 26 and 12. The anterior segment OD revealed 1+ inferior and axial corneal edema, while the OS was normal to biomicroscopy and posterior segment. Chandler syndrome or Fuchs endothelial dystrophy was suspected. In the affected eye, confocal light microscopy clearly showed an "epithelium-like" transformation of the corneal endothelium with irregularly shaped cells and hyperreflective nuclei, establishing the diagnosis of Chandler syndrome. CONCLUSIONS: In the presence of corneal edema or haze, corneal endothelium can be clearly visualized by confocal microscopy. "Epithelium-like" endothelial cells with highly reflective nuclei characteristic of Chandler syndrome were easily identified by confocal light microscopy to establish the diagnosis, despite the presence of corneal edema. Thus, confocal microscopy is a sensitive tool for the rapid, early diagnosis of ICE syndrome and may help distinguish among its variants.     

Histopathological Features of the Trabecular Meshwork in a Case of Presumed Bilateral Chandler Syndrome

Background: We describe a patient who had no-table decrease in the number of corneal endothelial cells in both eyes and developed open angle glaucoma without evident iris atrophy and peripheral anterior synechia.Case: A 74-year-old woman. The trabeculectomized angle tissue and iris tissue of her left eye were observed under light and transmission electron microscopy. From Schwalbe's line to the anterior chamber angle, degenerated endothelium-like cells were observed and overgrowth of layers of collagen fiber with varied cycles and basement membrane-like material were noticed. In addition, the morphology of intra-trabecular space was atrophic and occlusive with marked degeneration and exfoliation of endothelial cells in the trabecular space. On the side of the anterior chamber, degenerated endothelium-like cells were observed, the morphology of which was considered to result from abnormal metabolic function in corneal endothelium. Overgrowth of basement membrane-like material onto iridic tissue was not observed.Conclusion: Although slit lamp examination revealed no obvious abnormality, the result of histological examination suggested that this was a case of Chandler's syndrome in a broad sense or was in the early stage of this disease. We also discussed differential diagnosis from other diseases.     

Histopathological features of the trabecular meshwork in a case of presumed bilateral Chandler syndrome

BACKGROUND: We describe a patient who had notable decrease in the number of corneal endothelial cells in both eyes and developed open angle glaucoma without evident iris atrophy and peripheral anterior synechia. CASE: A 74-year-old woman. The trabeculectomized angle tissue and iris tissue of her left eye were observed under light and transmission electron microscopy. From Schwalbe's line to the anterior chamber angle, degenerated endothelium-like cells were observed and overgrowth of layers of collagen fiber with varied cycles and basement membrane-like material were noticed. In addition, the morphology of intra-trabecular space was atrophic and occlusive with marked degeneration and exfoliation of endothelial cells in the trabecular space. On the side of the anterior chamber, degenerated endothelium-like cells were observed, the morphology of which was considered to result from abnormal metabolic function in corneal endothelium. Overgrowth of basement membrane-like material onto iridic tissue was not observed. CONCLUSION: Although slit lamp examination revealed no obvious abnormality, the result of histological examination suggested that this was a case of Chandler's syndrome in a broad sense or was in the early stage of this disease. We also discussed differential diagnosis from other diseases.     

A case of Chandler's syndrome revealed by ultrastructural studies of the trabecular meshwork

PURPOSE: To investigate ultrastructural changes in the aqueous outflow route and discuss the mechanisms associated with intraocular pressure (IOP) elevation in a patient with presumably early stage Chandler's syndrome. METHODS: A 47-year-old man underwent trabeculectomy because of elevated IOP. A specimen obtained during surgery was studied by transmission electron microscopy. RESULTS: Electron microscopy showed the presence of a monolayer composed of corneal endothelium-like cells and thick basement membrane-like material. Neovascularization was also observed in the corneoscleral trabeculum. CONCLUSIONS: Our results indicate that several mechanisms, including the formation of basement membrane-like tissue, infiltration of inflammatory cells and neovascularization, might contribute to the elevation of IOP in Chandler's syndrome. These may occur even when there is no history of conspicuous inflammatory reaction in the anterior ocular segments.     

IRIS NEVUS SYNDROME (REPORT OF 9 CASES)

This paper reports on 9 rases of iris nevus syndrome diagnosed clinically, eight of which were confirmed by microscopy. The clinicopathologic features, etiology and pathogenesis of iris nevus syndrome are discussed. It was verified that iris nevus syndrome and other clinical variations of iridocorneal endothelial(ICE) syndrome had the same characteristic corneal endothelial defect. Also this syndrome was considered to he related to Chandler‘s syndrome Clinically.     

Keratopathy in pseudoexfoliation syndrome as a cause of corneal endothelial decompensation: a clinicopathologic study

PURPOSE: To provide clinical and histopathologic evidence of a distinct keratopathy as a potential cause of corneal edema in patients with pseudoexfoliation syndrome. DESIGN: Retrospective observational case series. PARTICIPANTS: Twenty-two patients with clinically diagnosed pseudoexfoliation syndrome undergoing penetrating keratoplasty for irreversible corneal endothelial decompensation. METHODS: The clinical and histopathologic findings of the corneal buttons are described compared with classic Fuchs' endothelial dystrophy. RESULTS: Clinically, the patients showed diffuse corneal edema, a pleomorphic and numerically reduced corneal endothelium, and retrocorneal flakes of pseudoexfoliation material in three cases. Histopathologically, all corneal buttons showed an abnormal diffuse, irregular thickening of Descemet's membrane and focal accumulations of locally produced pseudoexfoliation material onto or within Descemet's membrane in seven cases. The absence of typical guttata, a higher degree of fibroblastic transformation and melanin phagocytosis of endothelial cells, and a more pronounced endothelial cell loss distinguished the pseudoexfoliation specimens from specimens with classical Fuchs' dystrophy even in the absence of the pathognomonic pseudoexfoliation material. CONCLUSIONS: In patients with pseudoexfoliation syndrome, a distinct type of corneal endotheliopathy may occur, which can lead to an early corneal endothelial decompensation and which might have been previously misdiagnosed as an "atypical nonguttata Fuchs' endothelial dystrophy." This pseudoexfoliation keratopathy may potentiate the known complications in pseudoexfoliation eyes.     

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.     

Pseudocapsulorrhexis in a patient with iridocorneal endothelial syndrome

We describe a patient with Chandler's syndrome variant of the iridocorneal endothelial syndrome in whom ectopic Descemet's membrane was found intraoperatively on the anterior surface of the lens. Initially, the membrane was confused with the anterior lens capsule during extracapsular cataract extraction, leading to the performance of a pseudocapsulorrhexis. Electron microscopy disclosed that the epilenticular membrane was composed of multiple layers of abnormal basement membrane consistent with the iridocorneal endothelial syndrome.     

虹膜角膜内皮综合征的临床研究现状

虹膜角膜内皮综合征(ICE综合征 )是关于角膜内皮异常改变和虹膜萎缩等病变的一组疾病。常见于中青年女性,多单眼发病,一般不涉及遗传倾向,早期无症状时无需治疗,当并发角膜水肿、青光眼时,主要采用对症治疗。在这组疾病中,其病因、临床表现、治疗等皆有许多相同点,也有一些相异之处,国际上尚未形成统一的标准,因此本文就ICE综合征的临床研究现状进行综述。     

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.     

Essential progressive iris atrophy. Report of two cases

Chandler's syndrome and essential progressive iris atrophy, we believe, are two entities of the same syndrome. We have presented a case which could be considered a perfect intermediate. The mechanical 'stretch' theory of Campbell et al., supported to an extent by the light and electron microscopic findings of Rodrigues et al., is untenable in view of the observation in our patient that the first, and broad, peripheral anterior synechia was opposite the position of the displacement of the pupil. Every case in which corneal abnormality, glaucoma and iris changes are found, should be checked explicitly, with specular microscopy and gonioscopy, for corneal endothelial changes and in order to locate the area of predilection of peripheral anterior synechiae.     

Distinguishing features of the iridocorneal endothelial syndrome and posterior polymorphous dystrophy: value of endothelial specular microscopy.

The literature suggests that posterior polymorphous dystrophy (PPD) may show features such as iridocorneal adhesions, glassy membranes, and pupillary ectropion which are typically ascribed to the iridocorneal endothelial (ICE) syndrome. This complicates diagnosis. PPD, unlike ICE, is familial, and ICE, unlike PPD, is usually progressive and frequently complicated by glaucoma: thus it is important to distinguish between them. To determine whether this could be achieved by specular microscopy, since the posterior corneal surface is abnormal in both conditions, 57 cases of ICE and 44 of PPD were repeatedly examined and photographed with the specular microscope. Progressive and/or static morphological features of the corneal endothelium and Descemet's membrane were found that were specific for each condition. Specular microscopy can thus provide a definitive diagnosis of ICE or PPD even in uncertain cases.