In cases of severe deformity, surgical treatment may be necessary to reconstruct the damaged. Iridocorneal endothelial syndrome in thai patients. A 45 year old female presented with redness, pain and diminision of vision in left eye. Coganreese syndrome is a subset of the larger disease category iridocorneal endothelial syndrome, which is characterized by abnormal proliferation of the corneal endothelium over the cornea, iridocorneal angle, and onto the surface of the iris. Diagnostic tests for coganreese syndrome including blood tests, urine tests, swabs, diagnostic tests, lab tests, and pathology testing. Orphanet is a european reference portal for information on rare diseases and orphan drugs. Medical treatment was effective despite a fail trabeculectomy. Atypical cogans syndrome audiovestibular dysfunction with other types of inflammatory eye disease is associated. Coganreese syndrome is an extremely rare eye disorder characterized by a.
While there have been some cases of coganreese syndrome reported in children, the disease is typically observed in females in the midadult years. It is natural to associate the site of pain as the source of pain. More detailed information about the symptoms, causes, and treatments of cogan reese syndrome is available below symptoms of cogan reese syndrome. The iris naevus coganreese syndrome british journal of. This variant is most commonly unilateral and seen in middleaged females 2. A very rare eye disorder where various eye anomalies may impair vision. Coganreese syndrome is one type of iridocorneal endothelial ice syndrome. The cornea is the clear layer in front of the iris and pupil. Vasculitis is a family of rare disorders characterized by narrowing of the vessels, which restricts blood flow and damages vital organs and tissues. Cogans syndrome is a rare, rheumatic disease characterized by inflammation of the ears and eyes. Cogans syndrome is a rare disorder characterized by inflammatory eye and inner earvestibular disease that can also involve inflammatory vasculitis. Coganreese syndrome is characterized by proliferation of the corneal. Results during 5 years of followup only slight progression, manifested in episodes of blurred vision, occurred because of conleal changes. Over time, it can lead to permanent vision and hearing loss.
A cogan mezzanine is the fastest and most economical way to increase manufacturing and storage capacity in your facility. Others suggest that the primary disorder involves the cells that line the cornea corneal endothelium, with the impact on the iris as a secondary or associated disorder. This should not be considered a variant of ice syndrome, but can be confused with the coganreese syndrome. Most cases are relatively mild, have an excellent prognosis, and do not require treatment. Cogan syndrome is a rare autoimmune disease involving the eye and the inner ear. The clinical manifestations of this disease, namely, iridic stromal matting, iridic nodule formation, and unilateral. Antibodies against a peptide antigen cogan peptide have been found in sera from patients with cogans syndrome. Treatment of coganreese syndrome usually involves the use of drops in the. Cogan syndrome definition of cogan syndrome by medical. Coganreese variant is characterized by multiple pigmented iris nodules 2,6.
Objective to evaluate the spectrum of iridocorneal endothelial syndrome, to our knowledge, never studied previously in orientals. Antibodies directed against the cogan peptide showing similarity with autoantigens, including cd148, were identified. The rapid evolution courses with persistent glaucoma1,2. Resultsfiftyeight patients with ice syndrome were identified. Cogan syndrome eye disorders msd manual consumer version.
Learn more about coganreese syndrome from related diseases, pathways, genes and ptms with the novus bioinformatics tool. The nevi in irisnevus syndrome represent true nevi histologically. Methods the inner ears of six patients with cogan syndrome were studied. The disease appears to result from an autoimmune reaction directed against an unknown common autoantigen in the cornea and inner ear. Coganreese syndrome is one of the iridocorneal endothelial ice syndromes, all of which usually affect one eye of young to middleaged women. Diagnosis and management of iridocorneal endothelial syndrome. Association of coganreese syndrome, exfoliation syndrome. Cogan syndrome is a rare condition that affects your eyes and ears. The cornea looks fragile but is almost as stiff as a fingernail. Cogan reese syndrome nord national organization for rare. Coganreese syndrome symptoms, causes, diagnosis, and treatment information for coganreese syndrome coganreese syndrome with alternative diagnoses, fulltext book chapters, misdiagnosis, research treatments, prevention, and prognosis. Pdf the iridocorneal endothelial ice syndrome is a rare ocular disorder that includes a group of.
Click on the link to view a sample search on this topic. Cogan reese syndrome 59 introduction t his study was conducted to describe a case of cogan reese syndrome, which is a rare disease classified as one of the variants of the iridocorneal endothelial syndrome, which affects young, healthy individuals and is not correlated to family history. The ice syndromes essential iris atrophy, chandlers syndrome, and coganreese syndrome are distinct from one another. Cogan reese syndrome nord national organization for. Associations between cs and systemic vasculitis, as well as aortitis, also exist 24. Study conducted to report a case of cogan reese syndrome. Finally, in coganreese syndrome different degrees of iris atrophy can. Clinical examination revealed the presence of coganreese syndrome and exfoliation syndrome od. To present a case with coexistence of coganreese syndrome and exfoliation syndrome. Coganreese syndrome is a clinical variant of iridocorneal endothelial ice syndrome see this term characterized by variable iris atrophy, pigmented and. Cogan syndrome features not only problems of the hearing and balance portions of the ear, but also inflammation of the front of the eye the cornea and often fever, fatigue, and weight loss. This peptide antigen shares sequence homology with cd148 and connexin 26, which are expressed on endothelial cells and in the inner ear.
This variant of ice syndrome is distinguised by its coganreese subtype of ice syndrome. Lifethreatening aortic insufficiency develops in 10% of reported cases. Europe pmc is an archive of life sciences journal literature. Cogan reese syndrome symptoms, diagnosis, treatments and. Bilateral iridocorneal endothelial syndrome presented as. Cogan syndrome affects young adults, with 80% of patients between 14 yr and 47 yr. Some researchers suspect that inflammation or chronic infection may be the cause of the disease. Pdf diagnosis and management of iridocorneal endothelial. The term iridocorneal endothelial syndrome ice was suggested by eagle and yanoff which denotes a spectrum of disease characterized by primary corneal endothelial abnormality.
Slit lamp biomicroscopy revealed semidilated pupil not reacting to light and variation in iris colour. Chandler syndrome, essential progressive iris atrophy, coganreese syndrome iris nevus syndrome represent a spectrum of ice syndrome. The condition may also be associated with bloodvessel inflammation called. It protects the iris and lens and helps focus light on the retina. The purpose is to present a case of iridocorneal endothelium syndrome with glaucoma and discuss clinical presentation and management strategies. Conclusion ice syndrome is considered as unilateral, although cases of bilateral involvement of the same variant have been described. These abnormalities include iris atrophy, decreased corneal endothelium, iris nodules, adhesion of the iris to the lens and glaucoma. Studies have shown that the ice syndromes represent a spectrum of disease that are linked by a common corneal endothelial abnormality. A 72yearold caucasian woman presented for consultation due to uncontrolled intraocular pressure iop of the right eye.
Coganreese syndrome is an extremely rare eye disorder characterized by a matted or smudged appearance to the surface of the iris. Bordering the island of endothelialfree stroma, the advancing edge of. The clinical manifestations of this disease, namely, iridic stromal matting, iridic nodule formation, and unilateral angleclosure glaucoma, appear to be secondary to an endothelial membrane on the anterior surface of the iris. Clinical hallmarks are interstitial keratitis ik and vestibuloauditory dysfunction 1. Gutierrezortiz c, pareja j, bolivar g, cedazo mt, busteros ji, teus ma eur j ophthalmol 2006 novdec.
Methods from 1986 to 1998, we examined 60 consecutive patients 20 men, 40 women with characteristic signs of. This variant of ice syndrome is distinguised by its cogan reese subtype of ice syndrome. The patient was completely evaluated for the complaints. Mr of the inner ear in patients with cogan syndrome. Treatment of icerelated glaucoma often requires glaucoma filtering. Female patient, 55 years old with diagnosis of cogan reese syndrome.
Cogans syndrome can lead to vision difficulty, hearing loss and dizziness. Photograph taken in 1992 demonstrating nodules characteristic of the iridocorneal endothelial syndrome coganreese variant covering the iris from the 7 to. Cogans syndrome is a rare autoimmune disease that primarily affects the eyes and inner ears, but can also cause inflammation of the blood vesselsa condition known as vasculitis. The light and electron micrscopic findings in an eye enucleated from a 38yearold woman with iris naevus coganreese syndrome are presented. Designed for bulk, oversized, or awkwardlyshaped items, our industrialgrade cantilever racking stands up to any storage situation.
Chandler syndrome, essential progressive iris atrophy, cogan reese syndrome iris nevus syndrome represent a spectrum of ice syndrome. Coganreese syndrome definition of coganreese syndrome. The ice syndromes predominantly affect caucasian, young to middleaged women, and involve one eye. A very rare syndrome characterized by eye abnormalities that lead to vision impairment. The presentation of coganreese syndrome with iris cyst in this case is novel, which could be related to the disease pathogenesis, or a rare coincidental finding. Cogan reese syndrome is a clinical variant of iridocorneal endothelial ice syndrome see this term characterized by variable iris atrophy, pigmented and. Pubmed is a searchable database of medical literature and lists journal articles that discuss coganreese syndrome.
No therapy is available which targets the pathogenesis of ice syndrome, meaning medical treatment is aimed at. Coganreese syndrome cornea jama ophthalmology jama. The light and electron micrscopic findings in an eye enucleated from a 38yearold woman with. Iris melanocytoma mimicking the coganreese syndrome with monocular pigment dissemination.
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