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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Corneal clarity is often compromised in Cogan-Reese patients. Residents and Fellows contest rules International Ophthalmologists contest rules. Specular microscopy of the left eye showed pleomorphism and polymegathism with multiple guttatae. Unilateral use of prostaglandin analogue e. The spectrum is an acquired, unilateral disorder, which typically occurs in early to middle adulthood and predominantly affects women.

Get free access to newly published articles Create a personal account or sign in to: Specular microscopy is an important diagnostic tool, as the corneal endothelium has a characteristic appearance in ICE syndrome patients. The colored nodules characteristic of Cogan-Reese Syndrome do not usually appear in essential iris atrophy.

Purchase access Subscribe to JN Learning for one year. Consistent with Cogan-Reese syndrome, there were multiple fine nodules arrow distributed over the inferior degrees of the iris.

The American Academy of Ophthalmology recommends a complete eye exam by the age of 40 or earlier for those at increased risk. Series of 7 cases] J Fr Ophthalmol. Laser surgery is rarely effective. Sign in to access your subscriptions Sign in to your personal account.

Cogan-Reese Syndrome: A Rare Case of Unilateral Glaucoma

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]. Create a personal account to register for email alerts with links to free full-text articles.

Cogan-Reese syndrome most often presents between the ages of 20 and 50 years, and it is more synerome in women. Page Discussion View form View source History.


Rare Disease Database

This variant of ICE syndrome is distinguised by its reees iris findings. About News Events Contact. Iris nevus Cogan-Reese syndrome. Arch Ophthalmol ; 3: Detection of herpes simplex viral DNA in the iridocorneal endothelial syndrome. Slit lamp biomicroscopy revealed semidilated pupil not reacting to light and variation in iris colour pattern.

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. Glaucoma and the iridocorneal endothelial syndrome. Given her history of kidney stones, the acetazolamide was discontinued, and the patient was trialed on topical timolol-dorzolamide and oral acyclovir.

Mild cases can be managed with topical hypertonic saline drops and ointments.

The iris findings are less common, and a majority of patients have no iris changes at all, making the diagnosis a challenge. Affected Populations Cogan-Reese syndrome is a very rare disorder that predominantly affects females in the middle adult years, although cases have been reported in children.

Ssyndrome shallow anterior chamber, extensive PAS formation, and iris abnormalities may make graft positioning challenging for the EK surgeon [6]. Email this article Login required. Register for email alerts with links to free full-text articles Access PDFs of free articles Manage your interests Save searches and receive search alerts.

Long-term surgical outcomes of patients with glaucoma secondary to the iridocorneal endothelial syndrome.

The iridocorneal endothelial syndrome. Create account Log in.

Treatment of Cogan-Reese syndrome usually involves the use of drops in the eyes to control the glaucoma and swelling edema. Information on current clinical trials is posted on the Internet at www. Secondary, unilateral angle-closure glaucoma can occur in these patients because of the occlusion of the trabecular meshwork by the abnormal endothelial proliferation or PAS [4].


Privacy Policy Terms of Use. The treatment of ICE syndrome, regardless of the variant, primarily revolves around the prevention of glaucomatous vision loss secondary to elevated intraocular pressure. Over the next two weeks, the patient’s vision deteriorated. Summary Cogan-Reese Syndrome is a rare iridocorneal endothelial syndrome that most commonly affects young- to middle-aged women.

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.

Given her persistently elevated pressures and signs of corneal decompensation, the patient underwent a repeat trabeculectomy. Redse, topical hypertonic saline solutions and gels can be utilized to improve corneal edema by dehydrating the cornea.

Cogan Reese Syndrome – NORD (National Organization for Rare Disorders)

However, syhdrome edema often limits the view of the angle in Cogan-Reese syndrome, and such cases may benefit from the use of anterior segment optical coherence tomography OCT or ultrasound biomicroscopy to detect PAS or iridocorneal abnormalities [6].

The optic disc of the left eye had a cup of 0. Clinically, the corneal endothelium has been described to have a “hammered silver” or “beaten bronze” appearance in ICE syndrome patients, similar to corneal guttae seen in Rfese Corneal Endothelial Dystrophy. A comparison of clinical variations of the iridocorneal endothelial syndrome. Specular microscopy is often helpful given the presence of “ICE cells” is pathognomonic. However, a ‘functional’ angle-closure that can be confused with open-angle glaucoma may exist because the endothelial cell membrane can advance without causing overt syndroem formation.

Contraction of this tissue within the angle and on the iris results in the high peripheral anterior synechiae PAS and iris changes characteristic of ICE syndrome.