The iridocornealendothelial ice syndrome is characterized by glaucoma, corneal failure, and iris destruction. 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. Common clinical features include corneal edema, secondary glaucoma, iris atrophy, and pupillary anomalies, ranging from distortion to polycoria. Corneal specular and confocal microscopic examinations were performed in all three patients. The unilaterality of the presentation must include a differential diagnosis of unilateral. Median followup after glaucoma surgery was 24 1536 months. Cystoid macular edema associated with iridocorneal endothelial syndrome. Confocal microscopy in the iridocorneal endothelial syndrome. Ice is a group of conditions related to changes in corneal cells and the iris. Iridocorneal endothelial syndrome, or ice syndrome, is a primary abnormality of the corneal endothelium. The documents contained in this web site are presented for information purposes only. Glaucomaassociated corneal endothelial cell damage.
Iridocorneal endothelial syndrome is a rare ocular disorder characterized by abnormal endothelization of angle structure and iris producing characteristic manifestation including secondary angle. 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. Glaucoma associated with iridocorneal endothelial syndrome. Iridocorneal endothelial syndrome in thai patients. To describe chandler syndrome cs, specify the clinical features, list the differential diagnoses in the presence of unilateral ocular hypertension, with inclusion of. Ice syndrome is a group of disorders with three clinical variants. Purpose to report the demographic profile, clinical features, and prevalence of glaucoma and its management in patients with iridocorneal endothelial ice syndrome. Iridocorneal endothelial syndrome linkedin slideshare. Pk for iridocorneal endothelial syndrome yields visual.
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. The medical history, clinical pictures of the cornea, iris and anterior chamber angle, intraocular pressure, specular mi. Iridocorneal endothelial ice syndrome occurs mainly in young and middleaged women and. Iridocorneal endothelial ice syndrome describes a group of progressive corneal proliferative endotheliopathies comprised of chandlers syndrome, coganreese syndrome and essential. Ice syndrome is a spectrum of conditions of the eye, where the inner layer of the cornea appears abnormal. 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. Pseudocapsulorrhexis in a patient with iridocorneal endothelial. Diagnosis and management of iridocorneal endothelial.
Always seek professional medical advice about any treatment or change in treatment plans. Trabeculectomy failed, and the intraocular pressure was controlled with a glaucoma drainage. The main outcome measures were visual outcome, prosthesis. If you have problems viewing pdf files, download the latest version of adobe reader. In most cases the iridocorneal endothelial ice syndrome affects women unilaterally and shows endothelial degeneration, thickening of descemets membrane, iris atrophy, and glaucoma. It damages the cornea instead of being a single layer of cells covering the. Treatment list for iridocorneal endothelial syndrome. Aims to report the appearances of iridocorneal endothelial ice syndrome from real time, white light confocal microscopy. 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. 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. Results ice syndrome was present in 223 eyes of 203 subjects, 124 61% were female and.
The spectrum of the ice syndrome includes the chandlers syndrome, the essential iris atrophy, and the coganreese syndrome. The median presenting iop irrespective of antiglaucoma treatment. Iridocorneal definition of iridocorneal by medical. Loss of cells from the cornea can cause corneal swelling, and the iris and pupil can. 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. The iridocorneal endothelial ice syndrome is characterized by extension or proliferation of corneal endothelium and descemet membrane over the. Ice the iridocorneal endothelial syndromes youtube. 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. 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.
Because iridocorneal endothelial syndrome causes pressure to build up inside the eye, it can lead to glaucoma. The list of treatments mentioned in various sources for iridocorneal endothelial syndrome includes the following list. She had corneal edema in both eyes and grouped posterior corneal vesicles in the left eye. A 40yearold woman had a 3year history of cloudy vision and pain in both eyes. Iridocorneal endothelial ice syndrome is a rare condition, and unique characteristics in chinese patients can make diagnosis difficult. 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. 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. Iridocorneal endothelial ice syndrome linkedin slideshare. To report two cases of iridocorneal endothelial ice syndrome, coganreese syndrome crs or iris nevus and progressive iris atrophy pia. A this patient presented with elevated pressure, a round central pupil, and a thickened, hammered silver appearance to the corneal endothelium.
In ice syndrome, abnormal corneal endothelial cells proliferate, forming a membrane that migrates out over the anterior chamber angle and the iris. Methods three consecutive patients, each with ice syndrome, were examined prospectively. The syndrome almost always involves cells moving from the cornea to the iris. We present a unique case of an ice syndrome, which we would subclassify. Pdf diagnosis and management of iridocorneal endothelial.
Unique variations and characteristics of iridocorneal. A 31yearold white man had transient blurry vision oculus sinister os upon awakening in the morning for the past 2 months. Iridocorneal endothelial syndrome presenting with large. Glaucoma and the iridocorneal endothelial syndrome. What is iridocorneal endothelial syndrome, or ice syndrome. Visible changes in the iris swelling of the cornea, and the development. Iridocorneal endothelial ice syndrome is a disorder with a broad spectrum of clinical manifestations. Investigators evaluated graft survival, visual acuity and iop. The retrospective study included patients with iridocorneal endothelial ice syndrome who underwent pk between 2000 and 20. Diagnosis and management of iridocorneal endothelial syndrome. In the first patient, a penetrating keratoplasty was performed and the cornea was. Ice the iridocorneal endothelial syndromes vision tube.
No therapy is available which targets the pathogenesis of ice syndrome, meaning medical treatment is aimed at controlling iop and corneal. 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. The iridocorneal endothelial ice syndrome is a disease spectrum which. Iridocorneal endothelial syndrome and glaucoma j fr ophtalmol. Surgical results in iridocorneal endothelial syndrome. Iridocorneal endothelial syndrome genetic and rare diseases nih. More common in women and usually diagnosed in people between ages 30 and 50, iridocorneal endothelial syndrome, or ice syndrome, has three main features. Introduction coganreese syndrome is a clinical variation of the iri. Bilaminar interepithelial bodies within fingerprint. We initiated topic treatment with dorzolamide, timolol and dexamethasone, with good control of intraocular pressure. In iridocorneal endothelial ice syndrome, there is a dysfunction with this inner layer of cells. Definition of iridocorneal endothelial syndrome in the dictionary.
A group of eye diseases characterized by three main features. The purpose of this study is to report the intermediateterm surgical outcomes of patients with iridocorneal endothelial syndromerelated glaucoma. 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. A collection of disease information resources and questions answered by our. Diagnosis and management of iridocorneal endothelial syndrome martasacchetti,1 flaviomantelli,2 marcomarenco,3 ilariamacchi,4 oriellaambrosio,1 andpaolorama1. Iridocorneal endothelial ice syndrome includes 3 closely related entities iris. Department of ophthalmology, division of glaucoma, doheny eye institute, keck school of medicine, university of southern california summary. The most common feature of ice syndrome is the movement of endothelial cells off the cornea onto the iris. Pdf iridocorneal endothelial syndrome researchgate. 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.
Glaucoma associated with iridocorneal endothelial syndrome in 203. 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. Cystoid macular edema associated with iridocorneal. It includes coganreese syndrome, chandlers syndrome, and progressive iris atrophy. Information and translations of iridocorneal endothelial syndrome in the most comprehensive dictionary definitions. Iridocorneal endothelial syndrome genetic and rare. Longterm surgical outcomes of patients with glaucoma.
Pdf the iridocorneal endothelial ice syndrome is a rare ocular disorder that includes a group of conditions characterized by structural and. The clinical variants are distinguished primarily on the basis of the changes in the iris. Specular photomicroscopy of the corneal endothelium in this disease shows a. Iridocorneal endothelial syndromecoganreese syndrome. The clinical data of consecutive patients with ice syndrome who were seen between 2008 and 2011 at the glaucoma clinic of our. The iridocorneal endothelial syndrome is a disease spectrum which includes chandler syndrome, progressive iris atrophy and coganreese syndrome.
Instead of being a single layer of nonregenerating cells, it starts to replicate and spread. Our purpose was to describe the clinical characteristics and variations of ice syndrome in 58 consecutive chinese patients. 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. Iridocorneal endothelial syndrome ice is a rare eye condition. Iridocorneal endothelial syndrome and secondary glaucoma. 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. Iridocorneal endothelial syndrome ice university of.