Median followup after glaucoma surgery was 24 1536 months. Iridocorneal endothelial ice syndrome includes 3 closely related entities iris. The iridocorneal endothelial syndrome is a disease spectrum which includes chandler syndrome, progressive iris atrophy and coganreese syndrome. A 31yearold white man had transient blurry vision oculus sinister os upon awakening in the morning for the past 2 months. We present a unique case of an ice syndrome, which we would subclassify. More common in women and usually diagnosed in people between ages 30 and 50, iridocorneal endothelial syndrome, or ice syndrome, has three main features. Treatment list for iridocorneal endothelial syndrome. The shiley eye institute is the only academic institution in the san diego area with comprehensive programs for the clinical care of patients with eye disorders, cutting edge research on surgical techniques and treatments of eye diseases, education in the field of ophthalmology and innovative outreach to. Specular photomicroscopy of the corneal endothelium in this disease shows a. Iridocorneal endothelial syndrome genetic and rare diseases nih. If you have problems viewing pdf files, download the latest version of adobe reader. Iridocorneal definition of iridocorneal by medical. Introduction coganreese syndrome is a clinical variation of the iri.
Glaucomaassociated corneal endothelial cell damage. Loss of cells from the cornea can cause corneal swelling, and the iris and pupil can. A collection of disease information resources and questions answered by our. To describe chandler syndrome cs, specify the clinical features, list the differential diagnoses in the presence of unilateral ocular hypertension, with inclusion of a flow chart. Iridocorneal endothelial syndrome and secondary glaucoma. Department of ophthalmology, division of glaucoma, doheny eye institute, keck school of medicine, university of southern california summary. Pseudocapsulorrhexis in a patient with iridocorneal endothelial. The iridocorneal endothelial ice syndrome is typically a unilateral condition characterized by a corneal endothelial abnormality that is variably associated with corneal edema, anteriorchamber angle changes, alterations in the iris, and secondary glaucoma. Longterm surgical outcomes of patients with glaucoma. Results ice syndrome was present in 223 eyes of 203 subjects, 124 61% were female and. Iridocorneal endothelial syndrome ice essential iris atrophy. She had corneal edema in both eyes and grouped posterior corneal vesicles in the left eye.
A 30yearold female with iridocorneal endothelial syndrome ice who had been followed for several years with elevated intraocular pressure iop recently presented to my glaucoma clinic. These are characterized by an abnormal, beaten metal appearance of the corneal endothelium, with or without corneal edema, and iris abnormalities of varying degrees of severity ranging from mild stromal atrophy to severe distortion, stretching, and polycoria. Iridocorneal endothelial syndrome and glaucoma j fr ophtalmol. The iridocornealendothelial ice syndrome is characterized by glaucoma, corneal failure, and iris destruction. A 40yearold woman had a 3year history of cloudy vision and pain in both eyes. Longterm surgical outcomes of patients with glaucoma secondary to iridocorneal endothelial. Investigators evaluated graft survival, visual acuity and iop. The iridocorneal endothelial ice syndrome is a disease spectrum which. Iridocorneal endothelial ice syndrome describes a group of progressive corneal proliferative endotheliopathies comprised of chandler. Iridocorneal endothelial syndrome is a rare ocular disorder characterized by abnormal endothelization of angle structure and iris producing characteristic manifestation including secondary angle. It includes coganreese syndrome, chandlers syndrome, and progressive iris atrophy. Iridocorneal endothelial syndrome presenting with large. Iridocorneal endothelial syndrome abnormal corneal endothelium that is responsible for variable degrees of iris atrophy, secondary angleclosure glaucoma in association with characteristic peripheral anterior synechiae pas, and corneal edema.
The clinical data of consecutive patients with ice syndrome who were seen between 2008 and 2011 at the glaucoma clinic of our. Ice is a group of conditions related to changes in corneal cells and the iris. Common clinical features include corneal edema, secondary glaucoma, iris atrophy, and pupillary anomalies, ranging from distortion to polycoria. Methods three consecutive patients, each with ice syndrome, were examined prospectively. The purpose of this study is to report the intermediateterm surgical outcomes of patients with iridocorneal endothelial syndromerelated glaucoma. Iridocorneal endothelial ice syndrome is a group of disorders characterized by abnormal corneal endothelium that causes variable degrees of iris atrophy, secondary angleclosure glaucoma, and corneal edema. It damages the cornea instead of being a single layer of cells covering the. Ice the iridocorneal endothelial syndromes youtube. This patient presented with unilateral corectopia, pas, hammered silver appearance of the corneal endothelium os, areas of iris atrophy os, and history of intraocular pressure spikes os. Visible changes in the iris swelling of the cornea, and the development. Iridocorneal endothelial syndromecoganreese syndrome.
Iridocorneal endothelial syndrome is a rare eye diseases that causes swelling inside the cornea and changes in the iris the colored part of your eye. The most common feature of ice syndrome is the movement of endothelial cells off the cornea onto the iris. The iridocorneal endothelial ice syndrome is a rare ocular disorder that includes a group of conditions characterized by structural and proliferative abnormalities of the corneal endothelium, the anterior chamber angle, and the iris. The unilaterality of the presentation must include a differential diagnosis of unilateral. Introduction iridocorneal endothelial ice syndrome is a rare disorder characterized by proliferative and structural abnormalities of the corneal endothelium, progressive obstruction of the iridocorneal angle, and iris anomalies such as atrophy and hole formation the consequences of these changes are corneal decompensation and glaucoma. Glaucoma today iridocorneal endothelial syndrome may. Chandlers syndrome is a rare eye disorder in which the single layer of cells lining the interior of the cornea proliferates. Confocal microscopy in the iridocorneal endothelial syndrome. The purpose of the study was to report a case of iridocorneal endothelial syndrome with an initial presentation of a large diurnal fluctuation of intraocular pressure iop which peaked early in the morning. Ice syndrome is a group of disorders with three clinical variants. Our purpose was to describe the clinical characteristics and variations of ice syndrome in 58 consecutive chinese patients. While the ice syndrome is known to be associated with glaucoma and bullous keratopathy, to our knowledge, only two cases of ice syndrome complicated with cystoid macular edema cme. Cystoid macular edema associated with iridocorneal endothelial syndrome.
We initiated topic treatment with dorzolamide, timolol and dexamethasone, with good control of intraocular pressure. Iridocorneal endothelial syndrome, or ice syndrome, is a primary abnormality of the corneal endothelium. The clinical variants are distinguished primarily on the basis of the changes in the iris. The iridocorneal endothelial ice syndrome is characterized by extension or proliferation of corneal endothelium and descemet membrane over the. Always seek professional medical advice about any treatment or change in treatment plans. Pdf iridocorneal endothelial syndrome researchgate. The main outcome measures were visual outcome, prosthesis. She initially sought treatment 5 years ago with uncontrolled iop in the high 30s and a humphrey visual field with almost complete loss of the superior field. Trabeculectomy failed, and the intraocular pressure was controlled with a glaucoma drainage.
Diagnosis and management of iridocorneal endothelial. The list of treatments mentioned in various sources for iridocorneal endothelial syndrome includes the following list. Iridocorneal endothelial ice syndrome describes a group of progressive corneal proliferative endotheliopathies comprised of chandlers syndrome, coganreese syndrome and essential. Glaucoma associated with iridocorneal endothelial syndrome. Purpose to report the demographic profile, clinical features, and prevalence of glaucoma and its management in patients with iridocorneal endothelial ice syndrome. The medical history, clinical pictures of the cornea, iris and anterior chamber angle, intraocular pressure, specular mi. A this patient presented with elevated pressure, a round central pupil, and a thickened, hammered silver appearance to the corneal endothelium. To describe chandler syndrome cs, specify the clinical features, list the differential diagnoses in the presence of unilateral ocular hypertension, with inclusion of. Iridocorneal endothelial syndrome ice university of.
Iridocorneal endothelial ice syndrome is a rare condition, and unique characteristics in chinese patients can make diagnosis difficult. Iridocorneal endothelial ice syndrome linkedin slideshare. Iridocorneal endothelial syndrome in thai patients. Diagnosis and management of iridocorneal endothelial syndrome. Iridocorneal endothelial syndrome genetic and rare. Iridocorneal endothelial ice syndrome is a disorder with a broad spectrum of clinical manifestations. Iridocorneal endothelial syndrome ice syndrome is a unique ophthalmic disorder that involves an irregular corneal endothelium that can lead to varying degrees of corneal edema, iris atrophy, and secondary angleclosure glaucoma.
Surgical results in iridocorneal endothelial syndrome. The median presenting iop irrespective of antiglaucoma treatment. Iridocorneal endothelial syndrome ice is a rare eye condition. What is iridocorneal endothelial syndrome, or ice syndrome.
Diagnosis and management of iridocorneal endothelial syndrome martasacchetti,1 flaviomantelli,2 marcomarenco,3 ilariamacchi,4 oriellaambrosio,1 andpaolorama1. Glaucoma and the iridocorneal endothelial syndrome. Pk for iridocorneal endothelial syndrome yields visual. Aims to report the appearances of iridocorneal endothelial ice syndrome from real time, white light confocal microscopy.
Information and translations of iridocorneal endothelial syndrome in the most comprehensive dictionary definitions. The syndrome almost always involves cells moving from the cornea to the iris. Ice syndrome is a spectrum of conditions of the eye, where the inner layer of the cornea appears abnormal. Iridocorneal endothelial syndrome linkedin slideshare. Corneal specular and confocal microscopic examinations were performed in all three patients. If you have problems viewing pdf files, download the latest version of adobe. The spectrum of the ice syndrome includes the chandlers syndrome, the essential iris atrophy, and the coganreese syndrome.
Unique variations and characteristics of iridocorneal. Iridocorneal endothelial ice syndrome occurs mainly in young and middleaged women and. To report two cases of iridocorneal endothelial ice syndrome, coganreese syndrome crs or iris nevus and progressive iris atrophy pia. Iridocorneal endothelial ice syndrome occurs mainly in young and middleaged women and typically presents as a unilateral disease characterized by abnormalities of the iris and corneal endothelium. In most cases the iridocorneal endothelial ice syndrome affects women unilaterally and shows endothelial degeneration, thickening of descemets membrane, iris atrophy, and glaucoma. In iridocorneal endothelial ice syndrome, there is a dysfunction with this inner layer of cells. In ice syndrome, abnormal corneal endothelial cells proliferate, forming a membrane that migrates out over the anterior chamber angle and the iris. This loss of cells from the cornea often leads to swelling of the cornea, distortion of the iris, and variable degrees of distortion of the pupil the adjustable opening at the center of the iris that allows varying amounts of light to enter the eye. Definition of iridocorneal endothelial syndrome in the dictionary. Because iridocorneal endothelial syndrome causes pressure to build up inside the eye, it can lead to glaucoma. Cystoid macular edema associated with iridocorneal. Other clinical cases on show were fuchs endothelial dystrophy, iridocorneal endothelial syndrome and a child suffering from icthyosis who requires intense hourly, ocular lubrication day and night and who also has had a corneal graft in one eye. A group of eye diseases characterized by three main features.
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