In iridocorneal endothelial ice syndrome, there is a dysfunction with this inner layer of cells. Slit lamp biomicroscopy revealed semidilated pupil not reacting to light and variation in iris colour. The purpose is to present a case of iridocorneal endothelium syndrome with glaucoma and discuss clinical presentation and management strategies. At the last followup, best spectaclecorrected visual acuity ranged from 2067 to 2030. Deep lamellar endothelial keratoplasty for iridocorneal endothelial syndrome in phakic eyes.
Review article diagnosis and management of iridocorneal. It damages the cornea instead of being a single layer of cells covering the. Trabeculectomy failed, and the intraocular pressure was controlled with a glaucoma drainage. Essential iris atrophy is a rare clinical variant of ice syndrome. Iridocorneal endothelial syndrome essential iris atrophy. Atypical presentation of iridocorneal endothelial syndrome. Ice syndrome is characterized by proliferative and structural abnormalities of the corneal endothelium, progressive obstruction of the iridocorneal. In the first patient, a penetrating keratoplasty was performed and the cornea was. Descemets stripping with endothelial keratoplasty in. 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 corneal endothelial changes of fuchs endothelial dystrophy more closely resemble those of ice syndrome, but patients with fuchs do not have the anterior chamber angle or iris features seen in ice syndrome.
The cornea is the clear window in front of the coloured part of the eye the iris. Iridocorneal endothelial ice syndrome is a disorder with a broad spectrum of clinical manifestations. To report an unusual presentation of iridocorneal endothelial ice syndrome associated with band keratopathy and its management with ethylenediaminetetraacetic acid edta chelation and descemet membrane endothelial keratoplasty dmek. Glaucoma and the iridocorneal endothelial syndrome jama. Clinical and specular microscopic manifestations of. Iridocorneal endothelial syndrome ice is a group of ophthalmic disorders, characterized by structural and proliferative abnormalities of the corneal endothelium, affecting men and women with onset at young to middle age. It is a little frequent entity among the ophthalmologic diseases and among the glaucoma group it is of very low visual prognosis. The iridocorneal endothelial ice syndrome is characterized by extension or proliferation of corneal endothelium and descemet membrane over the. Iridocorneal endothelial syndrome and glaucoma american. Loss of the normal iris crypts leads to a smoothappearing iris. A group of eye diseases characterized by three main features. Coganreese syndrome is a clinical variant of iridocorneal endothelial ice syndrome see this term characterized by variable iris atrophy, pigmented and.
Iridocorneal endothelial syndromecoganreese syndrome. Cs is one of three syndromes affecting the eyes progressive iris atrophy and coganreese syndrome are the other two that make up the iridocorneal endothelial syndrome ice syndrome. 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. Glaucoma occurred in 33 50% of the patients with ice syndrome and was most common in the variants in which abnormal cells involve the entire posterior corneal surface disseminated ice and total ice. On the pathology of the iridocornealendothelial syndrome. It is necessary to carry out more studies permitting to obtain greater evidences about this. Yag membranectomy bonnie nga kwan choy1, carol pui yang chien2, jimmy shiu ming lai1, jonathan cheuk hung chan3 1department of ophthalmology, lks faculty of medicine, the university of hong kong, hong kong sar, peoples republic of china. While the ice syndrome is known to be associated with glaucomaand bullous keratopathy, to our knowledge, only two. We present the case of a 47yearold woman with bilateral iridocorneal endothelial syndrome. This page contains presentations for ophthalmology residents to use in studying for the okap and board exams. Early detection of iridocorneal endothelial syndrome ice. A 57yearold female patient presented with unilateral progressive painless visual impairment, corneal band keratopathy, and morphological.
Please explain your rationale for choosing the codes. A this patient presented with elevated pressure, a round central pupil, and a thickened, hammered silver appearance to the corneal endothelium. Iridocorneal endothelial syndrome genetic and rare. This variant of ice syndrome is distinguised by its coganreese subtype of ice syndrome. Confocal microscopy in the iridocorneal endothelial syndrome. 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.
Specular photomicroscopical and histological studies of the corneal. A 45 year old female presented with redness, pain and diminision of vision in left eye. The patient was completely evaluated for the complaints. 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. Pdf diagnosis and management of iridocorneal endothelial.
An interventional case is described to report on the clinical outcome of descemets stripping automated endothelial keratoplasty. Please read this short introduction with details regarding how to use the presentations author. 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. Glaucoma drainage device tube retraction and blockage in a patient with iridocorneal endothelial syndrome treated with nd. Iridocorneal endothelial ice syndrome is a rare and fascinating condition that can be challenging for ophthalmic surgeons to manage. Instead of being a single layer of nonregenerating cells, it starts to replicate and spread. 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. Because iridocorneal endothelial syndrome causes pressure to build up inside the eye, it can lead to glaucoma. The iridocorneal endothelial ice syndrome is characterized by glaucoma, corneal failure, and iris destruction. Iridocorneal endothelial syndrome and secondary glaucoma.
Review article diagnosis and management of iridocorneal endothelial syndrome martasacchetti, 1 flaviomantelli, 2 marcomarenco, 3 ilariamacchi, 4 oriellaambrosio, 1 andpaolorama 1 corneaandocularsurfaceunit,ospedalesanraa eleirccs,viaolgettina, m ilan,italy. Chandlers syndrome is a rare condition characterized by abnormalities in the corneal endothelium, iridocorneal angle obstruction, and iris abnormalities, such as. The records of 15 patients with ice syndrome who presented at the national taiwan university hospital between 1993 and 1996 were examined. Iridocorneal endothelial syndrome ice essential iris atrophy. Objective to evaluate the spectrum of iridocorneal endothelial syndrome, to our knowledge, never studied previously in orientals. Cystoid macular edema associated with iridocorneal. Methods from 1986 to 1998, we examined 60 consecutive patients 20 men, 40 women with characteristic signs of. The iridocorneal endothelial ice syndrome is a group of disorders characterized by abnormal physiology of the corneal endothelium, leading to varying degrees of progressive iris atrophy, corneal edema, andor synechial angle closure. Ppd, unlike ice, is familial, and ice, unlike ppd, is usually progressive and frequently complicated by glaucoma. Surgery by glaucoma drainage device implantation was performed. Corneal specular and confocal microscopic examinations were performed in all three patients. Keys to diagnosis and management a common pathological mechanism explains the corneal, iris, and angle abnormalities that result in a rare but important type of secondary angleclosure glaucoma. Iris disorders that could be confused with ice syndrome include axenfeldrieger syndrome, aniridia and iridoschisis.
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. Iridocorneal endothelial syndrome in a patient with keratoconus a. Pdf iridocorneal endothelial syndrome in a 16yearold. Aims to report the appearances of iridocorneal endothelial ice syndrome from real time, white light confocal microscopy. Iridocorneal endothelial syndrome ice essential iris.
Diagnosis and management of iridocorneal endothelial syndrome. It is one of the variants of iridocorneal endothelial syndrome essential iris atrophy, chandlers syndrome and. Objective to describe the clinical features of iridocorneal endothelial ice syndrome in a group of patients referred because of suspected iris melanoma. Chandler syndrome, essential progressive iris atrophy, coganreese syndrome iris nevus syndrome represent a spectrum of ice syndrome. Iridocorneal endothelial syndrome ice university of. The term iridocorneal endothelial syndrome ice was suggested by eagle and yanoff which denotes a spectrum of disease characterized by primary corneal endothelial abnormality. Visible changes in the iris swelling of the cornea, and the development. Iridocorneal endothelial ice syndrome describes a group of eye diseases that are characterized by three main features. Visible changes in the iris the colored part of the eye that regulates the amount of light entering the eye swelling of the cornea, and. Iridocorneal endothelial syndrome ice sd is a rare condition characterized by abnormalities in the corneal endothelium, iridocorneal angle obstruction, and iris abnormalities, such as iris atrophy. Iridocorneal endothelial syndrome ice syndrome is a unique ophthalmic disorder that involves an abnormal corneal endothelium that leads to varying. Ice syndrome is a spectrum of conditions of the eye, where the inner layer of the cornea appears abnormal. The iridocornealendothelial syndrome ice syn drome is characterised by a hammeredsilver appearance of the corneal endothelium.
What is the codes for iridocorneal endothelial syndrome. It comprises a spectrum of three clinical entities. Iridocorneal endothelial syndrome in a patient with. The inferior iris has become stretched and thin, resulting in fullthickness atrophic holes polycoria. Iridocorneal endothelial syndrome masquerading as iris. Iridocorneal endothelial syndrome in thai patients. Glaucoma occurred in 33 50% of the patients with ice syndrome and was most common in. Common clinical features include corneal edema, secondary glaucoma, iris atrophy, and pupillary anomalies, ranging from distortion to polycoria. 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. Distinguishing features of the iridocorneal endothelial. It includes coganreese syndrome, chandlers syndrome, and progressive iris atrophy. Specular photomicroscopy of the corneal endothelium in this disease shows a. The iridocornealendothelial syndrome ice syndrome is characterised by corneal failure, glaucoma and iris destruction.
Pdf the iridocorneal endothelial ice syndrome is a rare ocular disorder that includes a group of conditions characterized by structural and. The etiology of ice syndrome is not clear, and there is still no effective treatment for halting this disease. 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. The iridocorneal endothelial syndrome has heretofore been considered a unilateral disorder. The iridocorneal endothelial ice syndrome is a rare ocular disorder that includes a group of conditions characterized by structural and proliferative. Pdf iridocorneal endothelial syndrome researchgate. Corectopia, which is often present at this stage, is absent in this eye. Iridocorneal endothelial ice syndrome is a progressive clinical spectrum of corneal endothelial abnormalities affecting the cornea, iris, and iridocorneal angle. This condition was initially described by harms, in 1903 2, as essential progressive iris atrophy. The medical history, clinical pictures of the cornea, iris and anterior chamber angle, intraocular pressure, specular mi.