Cogans syndrome has been reported in patients from 2 to 60 years of age with a median age of onset of 25 years and an equal sex distribution. We report a case of cogans syndrome in 12 year old girl who intialy present with bilateral interstitial keratitis with audiovestibular and cardiac symptoms. Clinical hallmarks are interstitial keratitis ik and vestibuloauditory dysfunction 1. Video 32 oct anterior segment imaging cirrus hdoct image with a visible angle recess blue arrow.
Topical treatment was modified with the addition of. The purpose of this report is to describe a case of cogan s syndrome cs with atypical ophthalmic features, such as diffuse anterior scleritis and bilateral peripheral ulcerative keratitis. Cogan syndrome cardiomyopathy chromosome 4, monosomy 4q chromosome 10q26 deletion syndrome chromosome 18 trisomy cogan reese syndrome carpenter syndrome chromosome 4, monosomy distal 4q chromosome 11, partial monosomy 11q chromosome 18 ring cohen syndrome cat eye syndrome chromosome 4, partial trisomy distal 4q chromosome 11, partial trisomy. To report two cases of iridocorneal endothelial ice syndrome, coganreese syndrome crs or iris nevus and progressive iris atrophy pia. Study conducted to report a case of cogan reese syndrome. Dec 06, 2007 cogans syndrome is defined as a chronic inflammatory disease of unknown origin, an autoimmune disease, characterized by bilateral sensorineural hearing loss, vestibular symptoms, inflammatory ocular manifestations with variable risk of developing into a systemic disease. Some evidence suggests an autoimmune mechanism, but the true etiology unknown. Associations between cs and systemic vasculitis, as well as aortitis, also exist 24. Cogan reese syndrome nord national organization for rare. Chaglasian, od osd, glaucoma and quality of life am j ophthalmol 2012. Chromosomal aberrations in iris melanomas british journal. Medical treatment was effective despite a fail trabeculectomy. Jul 26, 2016 yet coganreese syndrome was named for him and algernon reese many years ago. Resultsfiftyeight patients with ice syndrome were identified.
At that visit, her iop was 57 mmhg, and there was evidence of anterior chamber inflammation. Cogans syndrome is a rare autoimmune disorder that primarily affects the vision, balance, and hearing of the individual diagnosed with it. Cogan s syndrome is a rare, idiopathic, chronic inflammatory disorder characterized vestibuloauditory dysfunction and interstitial keratitis. When a new designation, ice syndrome, was suggested as a unifying term to replace coganreese syndrome, chandler syndrome, and essential iris atrophy, cogan was quoted by william spencer in another editorial in archives. Iridocorneal endothelial syndrome chandlers syndrome progressive iris atrophy cogan. Medical treatment of cogan s syndrome depends on how extensive the disease is at the time of diagnosis. Coganreese syndrome genetic and rare diseases information.
Photographsfirsttakenin1992demonstrated some ectropian uvea and nodules covering the iris from the 7to 3. Iris nodules observed in cogan reese syndrome also require differential diagnosis with other conditions showing similar iris changes such as neurofibromatosis, iris. The first karyogram of an iris melanoma revealed extra copies of chromosomes 2, 7 and 18, and loss of chromosome 15. Cohen syndrome is an inherited disorder that affects many parts of the body and is characterized by developmental delay, intellectual disability, small head size microcephaly, and weak muscle tone hypotonia. Cogan reese syndrome nord national organization for. It was first described by an ophthalmologist, dr david g. Sudhir patil introduction secondary angle closure glaucomas are a separate entity from primary angle closure glaucoma. A unique case of keratoconus with coganreese syndrome and secondary glaucoma. Introduction cogan reese syndrome is a clinical variation of the iri. He was introduced to ophthalmology by his mother, herself a practitioner and a pioneering ophthalmologist.
Some of the trains that run on this route are amritsar katihar express, amritsar dibrugarh express and etc. Coganreese syndrome presents with the corneal findings and pigmented nodules on the iris. To date, only eight iris melanomas have been cytogenetically analysed and described worldwide. Coganreese syndrome is characterized by proliferation of the. The pathology, pathogenesis, clinical manifestations, and treatment of cs will. Coganreese syndrome cornea jama ophthalmology jama. Search results for cogan s syndrome about 1 items found relating to cogan s syndrome. Cogans syndrome is a rare, idiopathic, chronic inflammatory disorder characterized vestibuloauditory dysfunction and interstitial keratitis. Diagnosis and management of iridocorneal endothelial syndrome. You can visit yahoogroups site and search for amrapali zodiac ajrapali. The classic form of the disease was first described by d. Access to the latest issue is reserved only for the paid subscribers.
He believed the essence of investigation was originality, curiosity, and perseverance, and fostered that spirit in the talented clinicians and basic scientists he recruited to howe. Cogans syndrome causes doctor answers on healthcaremagic. Cogans syndrome becomes atypical when the eye andor ear involvement is of a different type or when. He attended local school until 1925, when he enrolled at dartmouth college, where he combined a collegiate schedule with the first two years of medical school. He had cystoid macular edema which failed to respond initially to steroid, methotrexate and azothiaprine however resolved after treatment with mycophenolate mofetil. Cogans syndrome is defined as nonsyphilitic interstitial keratitis an inflammation of the eye and bilateral audiovestibular deficits hearing problems and dizziness. Fourteen patients ten women, four men with iris nevus coganreese syndrome, all having unilateral glaucoma, were studied clinically. It is not clear that cogans syndrome is a primary vasculitis. A unique case of keratoconus with coganreese syndrome and. Cogan syndrome cs is a chronic inflammatory disorder characterized by recurrent interstitial keratitis ik and audiovestibular symptoms including hearing loss, tinnitus, and vertigo cogan 1945. Diagnosis and management of iridocorneal endothelial.
Chandler syndrome most common hammered silver endothelium and about 40% have some iris atrophy halos from corneal edema glaucoma less severe if present progressive essential iris atrophy severe iris changes and pas iris nevus coganreese syndrome diffuse iris nevus or nodules some iris atrophy treatment. Association between cogans syndrome and systemic vasculitis as well as aortitis also exists. Treatment of coganreese syndrome usually involves the use of drops in the eyes to control the glaucoma and swelling edema. The main subtypes of this syndrome are the progressive iris atrophy, the coganreese syndrome, and the chandler syndrome. Associations between cs and systemic vasculitis, as well as aortitis, also exist.
A patient was diagnosed with cogans syndrome nearly 2 years after first presentation. Photographs first taken in 1992 demonstrated some ectropian uvea and nodules covering the iris from the 7 to 3. Cogan syndrome is a rare vasculitis of young adults that is primarily characterized by 1,4,6. While there have been some cases of coganreese syndrome reported in children, the disease is typically observed in females in the midadult years. The longterm outcome of the surgical treatment in ice syndrome patients has been shown to be better in those with a gdi. Cogans syndrome is a rare disorder of unknown origin characterized by inflammatory eye disease and vestibuloauditory symptoms, which primarily affects young white adults, without a hereditary pattern 1. May 29, 2019 cogan s syndrome cs is a chronic inflammatory disorder that most commonly affects young adults. Secondary angle closure glaucoma is usually caused by multiple factors, so identi. Cogans syndrome cs is a chronic inflammatory disorder that most commonly affects young adults.
Recurrent corneal ulceration secondary to an iris cyst. Information and translations of cogan syndrome in the most comprehensive dictionary definitions resource on the web. Seizures often develop within the first ten days of life and usually before three months old. Major characteristics of coganreese syndrome include a matted or smudged appearance to the surface of the iris nevus, yellow or brown lumps or nodules on the iris nodular iris nevi, the attachment of portions of the iris to the cornea peripheral anterior synechiae, and increased pressure in the eye glaucoma.
Photograph taken in 1992 demonstrating nodules characteristic of the iridocorneal endothelial syndrome coganreese variant covering the iris from the 7 to. Cogan syndrome is a rare disorder characterized by recurrent inflammation of the front of the eye the cornea and often fever, fatigue, and weight loss, episodes of vertigo dizziness, tinnitus ringing in the ears and hearing loss. It is more common in caucasians than in other races. Iris nodules observed in coganreese syndrome also require differential diagnosis with other conditions show ing similar iris changes such as neurofibromatosis, iris. Seizures seizure types frequently include brief tonic seizures stiffening of the body, focal. Antibodies against a peptide antigen cogan peptide have been found in sera from patients with cogans syndrome. The chainlike structures possess a single crystalline structure and preferential growth direction along botanima 1 0 0 crystal orientation. While there have been some cases of cogan reese syndrome reported in children, the disease is typically observed in females in the midadult years. Cogan, in 1945, who reported on a syndrome of nonsyphilitic interstitial keratitis ik and vestibuloauditory. The treatment of iris melanoma depends on many factors including. Introduction coganreese syndrome is a clinical variation of. Syndrome of interstitial keratitis associated with vestibuloauditory symptoms, characterized by abrupt onset of vertigo, tinnitus typical of menieres disease, and usually rapid development of bilateral deafness, caused by affection of the 8th cranial nerve. Treatment of cogan s syndrome is difficult, and the only information we find in the literature is based upon clinical case reports, as no organised series of treatments have been published.
Iridocorneal endothelial syndrome masquerading as iris melanoma. The classic ophthalmic feature is interstitial keratitis causing patchy, granular corneal stromal infiltrates that may result. Introduction coganreese syndrome is a clinical variation of the iri. David glendenning cogan was born in fall river, massachusetts, where his father was an episcopal minister. Coganreese syndrome, iridocorneal endothelial syndrome. The occurrence of iris cyst in this case of coganreese syndrome is unique, and could be related to the disease pathogenesis, or a rare coincidental finding. Female patient, 55 years old with diagnosis of cogan reese syndrome. Cogan syndrome affects young adults, with 80% of patients between 14 yr and 47 yr. Ohtahara syndrome ohtahara syndrome is a very rare epilepsy syndrome. A collection of disease information resources and questions. Major characteristics of cogan reese syndrome include a matted or smudged appearance to the surface of the iris nevus, yellow or brown lumps or nodules on the iris nodular iris nevi, the attachment of portions of the iris to the cornea peripheral anterior synechiae, and increased pressure in the eye glaucoma. Antibodies directed against the cogan peptide showing similarity with autoantigens, including cd148, were identified. Onset of the disease is generally a brief episode of inflammatory eye disease, most commonly interstitial keratitis. Coganreese syndrome carpenter syndrome chromosome 4, monosomy distal 4q chromosome 11, partial monosomy 11q chromosome 18 ring cohen syndrome cat eye syndrome chromosome 4, partial trisomy distal 4q chromosome 11, partial trisomy 11p14.
Better a descriptive name, if that is possible, and an eponym if it is not possible. The ice syndromes predominantly affect caucasian, young to middleaged women, and involve one eye. Cystoid macular oedema in cogans syndromea case report. Corneal endotheliitis is characterized by corneal edema, kps, and mild anterior chamber reaction with no signs of infig. David cogan was an influential teacher, cherished by his countless student, of which several have become prominent. Cogan s syndrome is rare and presents a challenge in terms of diagnosis and treatment. To describe chandler syndrome cs, specify the clinical features, list the differential diagnoses in the presence of unilateral ocular hypertension, with inclusion of. Coganreese syndrome progressive growth of endothelium over iris a 47yearold woman wasfirstdiagnosedas having iridocorneal endothelial syndrome in 1988.
Cogans syndrome, typified by the combination of interstitial keratitis and immunemediated sensorineural hearing loss, is a rare condition, and commonly associated with a diagnostic delay. Some babies may have seizures in the womb during the last three months of pregnancy. Association between cogan s syndrome and systemic vasculitis as well as aortitis also exists. Photographs taken in 1997 figure 3 and figure 4 illustrate a clear progression of the nodules over. Reese ocular surface disease lissamine green stain. To describe chandler syndrome cs, specify the clinical features, list the differential diagnoses in the presence of unilateral ocular hypertension, with inclusion of a flow chart. Cogan syndrome cs is a chronic inflammatory disorder characterized by recurrent interstitial keratitis ik and audiovestibular symptoms including hearing loss.
Other features common in this condition include worsening nearsightedness myopia, breakdown degeneration of the lightsensitive tissue at the back of the eye retinal dystrophy, an. Mar 25, 20 cogans syndrome cs is the combination of hearing loss, vertigo, and inflammation in the eyes of uncertain cause, which can be associated with a large, more often than medium, size vessel vasculitis in 1015% of patients. This variant of ice syndrome is distinguised by its cogan reese subtype of ice syndrome. Sep 30, 2014 cogan reese syndrome is one type of iridocorneal endothelial ice syndrome.
Cogan syndrome is a rare autoimmune disease involving the eye and the inner ear. Coganreese syndrome is one type of iridocorneal endothelial ice syndrome. If you have problems viewing pdf files, download the latest version of adobe reader. A 47yearold woman was first diagnosed as having iridocorneal endothelial syndrome in 1988. This report documents, what we believe to be a new association of. Cogans syndrome cs is a rare autoimmune vasculitis. Now thought of as a syndrome with a common fundamental pathophysiological basis but a spectrum of clinical manifestations, it is historically subdivided into essential progressive iris atrophy, chandler syndrome, and cogan reese irisnevus syndrome.
The documents contained in this web site are presented for information. To report two cases of iridocorneal endothelial ice syndrome, cogan reese syndrome crs or iris nevus and progressive iris atrophy pia. Pdf diagnosis and management of iridocorneal endothelial. Major characteristics of coganreese syndrome include a matted or smudged appearance to the surface of the iris nevus, yellow or brown lumps or nodules on the iris nodular iris nevi, the attachment of portions of the iris to the cornea peripheral anterior synechiae. Cogans syndrome is rare and presents a challenge in terms of diagnosis and treatment. Cogans syndrome is an uncommon, rheumatic disease characterized by inflammation of the ears and eyes. Cogan reese syndrome is a clinical variant of iridocorneal endothelial ice syndrome see this term characterized by variable iris atrophy, pigmented and. Clinical hallmarks are interstitial keratitis ik and vestibuloauditory dysfunction. This peptide antigen shares sequence homology with cd148 and connexin 26, which are expressed on endothelial cells and in the inner ear. Cogans syndrome causes symptoms that are related to those of syphilic keratitis although there is no evidence of pox transmission in cogans syndrome. Cogan s syndrome cs is a rare autoimmune vasculitis.
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