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.

Author: Tojalrajas Mikar
Country: Nicaragua
Language: English (Spanish)
Genre: Love
Published (Last): 2 August 2006
Pages: 254
PDF File Size: 11.39 Mb
ePub File Size: 18.23 Mb
ISBN: 724-3-49714-752-8
Downloads: 80904
Price: Free* [*Free Regsitration Required]
Uploader: Tojind

User Username Password Remember me. Laser surgery is rarely effective.

Rare Disease Database

Causes The cause of Cogan-Reese syndrome is not known. Purchase access Subscribe to the journal.

Iris nevus Cogan-Reese syndrome: The purpose is to present a case of iridocorneal endothelium Syndrome with glaucoma and discuss clinical presentation and management strategies.

Topical medication is the first line therapy for patients with elevated intraocular pressure from secondary angle-closure glaucoma in the setting of ICE syndrome. Photographs first taken in demonstrated some ectropian uvea and nodules covering the iris from the 7- to 3: Together we are strong. Unilateral use of prostaglandin analogue e. However, prompt diagnosis is important given the progressive nature and aggressive course of ICE.

ICE syndrome can result in advanced glaucomatous optic neuropathy with extensive vision loss if left untreated. The congenital cataracts, optic nerve and foveal hypoplasia, bilateral presentation, lack of corneal endothelial changes, and congenital nature differentiate aniridia from ICE syndrome. Many patients resse subtle disease, and do quite well with intraocular pressure control both topical and surgical.

Comparisons may be useful for a differential diagnosis:. Slit lamp photography, OU. Sign in to customize your interests Sign in coogan your personal account.

Superior and inferior arcuate visual field defects Mean deviation: Unfortunately, this often fails to adequately syyndrome intraocular pressure due to progressive obstruction of the trabecular meshwork. The patient may present with unilateral reduced vision, noticeable color changes to the iris, or unilateral secondary glaucoma due to corneal endothelium obstruction of the trabecular meshwork.


Cogan-Reese Syndrome: A Rare Case of Unilateral Glaucoma

Sign cgan to access your subscriptions Sign in to your personal account. For information about clinical trials conducted in Europe, contact: Treatment Management of Cogan-Reese, similar to that of the other ICE syndromes, is aimed at slowing or preventing vision loss from secondary angle-closure glaucoma by lowering the patient’s intraocular pressure.

Specular microscopy of the left eye showed pleomorphism and polymegathism with multiple guttatae. This resulted in well-controlled IOPs off drops.

Cogan-Reese Syndrome syyndrome a rare iridocorneal endothelial syndrome that most commonly affects young- to middle-aged women. 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.

The Academy uses cookies to analyze performance and provide relevant personalized content to users of our website. Humphrey visual fields showed substantial worsening from prior fields Figure 2.

The treatment of ICE syndrome, regardless of the variant, primarily revolves around the prevention of glaucomatous vision loss secondary to elevated intraocular pressure.

The colored nodules characteristic of Cogan-Reese Syndrome do not usually appear in essential iris atrophy. Aniridia is a bilateral congenital disease that presents with absence of the iris commonly a rudimentary iris stump existscataracts, glaucoma, and corneal pannus. Glaucoma and iridocorneal endothelial syndrome. A trabeculectomy with antifibrotic agents mitomycin-C or 5-fluorouracil or a glaucoma drainage device aqueous shunt have been found to be effective in controling IOP in ICE syndrome patients.

The decision was made to proceed with surgery given the persistently elevated IOP. Whenever a patient with suspected iris nevus syndrome or essential iris atrophy is subjected to glaucoma surgery, a biopsy specimen of the iris should be obtained for histological study. Progressive growth of endothelium over iris. Three years after her initial trabeculectomy, the patient underwent phacoemulsification of a cataract in her left eye with intraocular lens placement at an outside institution.


Three weeks prior to her presentation at UIHC, she presented to her local optometrist for evaluation of blurring of her vision OS. Brisk, reactive to light OS: Cogan-Reese syndrome is unique among the ICE syndromes due to the presence of pedunculated, pigmented iris nodules, commonly referred to as iris nevi [1].

Axenfeld-Rieger syndrome is a bilateral, heterogeneous congenital condition that is is autosomal dominant in most cases, but can also occur sporadically, and may include developmental abnormalities in the anterior chamber angle, iris, and trabecular meshwork. Multiple fine nodules distributed over the inferior degrees; see Figure 4 for higher magnification of these nodules. Iris nevus Cogan-Reese syndrome.

Testing and Imaging Making the diagnosis of an ICE syndrome is often challenging, as it can appear similar to other disease states, including posterior polymorphous corneal dystrophy. Purchase access Subscribe to the journal.

Orphanet: Cogan Reese syndrome

Purchase access Subscribe now. Coagn failures have been reported with trabeculectomies secondary to advancing endothelialization of the fistula. Cogan-Reese syndrome, also known as iris nevus syndrome, is a very rare disease that is considered one of the three variations of ICE syndrome [1]; the other two ICE variants are Chandler’s syndrome and Essential Iris Atrophy.