Cogan-Reese syndrome is a clinical variant of iridocorneal endothelial (ICE) syndrome (see this term) characterized by variable iris atrophy, pigmented and. This variant of ICE syndrome is distinguised by its Cogan-Reese sub-type of ICE syndrome. A YEAR-OLD woman was first diagnosed as having iridocorneal endothelial syndrome in She underwent a trabeculectomy in Photographs first.
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Asymmetric endothelial cell loss and atypical endothelial cell morphology is typically evident, which appears on a specular photomicrograph as dark, larger than normal endothelial cells, with a bright central spot.
Specular microscopy of the left eye showed pleomorphism and polymegathism with multiple guttatae. A comparison of clinical variations of the iridocorneal endothelial syndrome. In general, glaucoma is one of the leading causes of blindness in the world.
Iridocorneal Endothelial Syndrome and Secondary Glaucoma
There is a hypothesis that ICE syndromes stem from an in-vitro herpes infection localized sundrome the endothelial layer. Similar to our patient, many affected individuals require multiple glaucoma procedures to normalize IOP, in addition to corneal endothelial keratoplasty EK to address the significant corneal decompensation. Greater diagnostic evaluation for glaucoma can be accomplished with common testing devices utilized for any glaucoma patient.
The mechanism of glaucoma in ICE syndrome all three variants is believed to be related to a cellular membrane secreted by the abnormal endothelial cells.
Sign in to save your search Sign in to your personal account. Arch Ophthalmol ;93 A Rare Case of Unilateral Glaucoma. Unfortunately, this often fails to adequately control synddrome pressure due to progressive obstruction of the trabecular meshwork.
Rare Disease Database
The abnormal endothelial cells in ICE syndrome are thought to have a toxic effect on neighboring healthy cells, thus explaining the progressive nature of the disease . Eur J Ophthalmol ;24 5: However, prompt diagnosis is important given the progressive nature and aggressive course of ICE. An overgrowth of endothelium and Descemet membrane extending onto the iris surface may be a characteristic part of the process. Get free access to newly published articles Create a personal account or sign in to: Trace nuclear sclerosis OS: Purchase access Subscribe to the journal.
Material for histological study was available from ten patients and showed a nonmalignant rsese nevus of the anterior surface of the iris.
She underwent a trabeculectomy in This page has been accessedtimes. The corneal endothelium in ICE syndrome is typically described as “hammered silver” or “beaten bronze” in appearance when viewed through the slit lamp using specular reflection Figure 4A sjndrome.
All three disease entities are difficult to treat given their progressive nature and the obliteration of the iridocorneal angle that leads to refractory elevation in IOP. Some researchers suspect that inflammation or chronic infection may rese the cause of the disease.
Orphanet: Cogan Reese syndrome
Create a personal account to register for email alerts with links to free full-text articles. Making the diagnosis of an ICE syndrome is often challenging, as it can appear similar to other sydrome states, including posterior polymorphous corneal dystrophy. Transmission and Scanning Electron Microscopic examination of these cells has demonstrated a population of well-differentiated cells with epithelial features such as desmosomes, tonofilaments, and microvilli.
A Cause of Unilateral Glaucoma. The glaucoma tends to be more severe reesse progressive iris atrophy and Cogan-Reese syndrome. Normal OU Cup-to-disc ratio: Create account Log in.
Cogan-Reese Syndrome: A Rare Case of Unilateral Glaucoma
The American Academy of Ophthalmology recommends a complete eye exam by the age of 40 or earlier for those at increased risk. Stereo disc photographs and visual field analysis Humphrey or Goldmannalong with optic nerve and nerve fiber layer assessment heidelberg retinal tomogram HRT or optical coherence tomography OCTcan all be implemented in the initial work-up and ongoing evaluation for glaucoma progression in these patients. For information about clinical trials sponsored by private sources, contact: Specifically, if medical management fails, a trabeculectomy or implanting a glaucoma drainage implant GDI should be pursued.