Coganreese syndrome genetic and rare diseases information. Typical cogans syndrome is defined by nonsyphilitic interstitial keratitis associated with audiovestibular involvement similar to that of menieres disease with progressive hearing loss to complete deafness within 2 years. Cogan reese syndrome is characterized by proliferation of the corneal endothelium involving most of the iris and anterior chamber angle. The unifying abnormality in all three entities is the abnormal, unilateral, hammered silver appearance of the corneal endothelium. Medical treatment was effective despite a fail trabeculectomy. Ubm findings of anterior segment were compared between normal subjects and three clinical types of ice syndrome. Descemets stripping automated endothelial keratoplasty in. Cogan s syndrome cs is a chronic inflammatory disorder that most commonly affects young adults. Researchers arent sure about its exact origin, but many consider. Secondary glaucoma group of disorders in which the raised iop is associated with a primary ocular or systemic disease.
Cystoid macular edema associated with iridocorneal. Pathogenesis of chandlers syndrome, essential iris atrophy and the cogan reese syndrome. The patient was completely evaluated for the complaints. May 29, 2019 cogan s syndrome cs is a chronic inflammatory disorder that most commonly affects young adults. We report a case of an atypical cogans syndrome, the workup of the diagnosis, and treatment results. Direct slitlamp visualization of the cornea and anterior segment in cases of ice syndrome is inadequate for precise and objective assessment of the affected structures. Chandlers syndrome, and cogan reese iris nevus syndrome. Iridocorneal endothelial syndrome ice essential iris atrophy.
The common features of coganreese syndrome include fine iris nodules, peripheral anterior synechiae, loss of crypts, ectropion uveae and secondary glaucoma. Literature search the authors searched pubmed on january 8, 2018, for englishlanguage results using the following terms sin. The presentation of cogan reese syndrome with iris cyst in this case is novel, which could be related to the disease pathogenesis, or a rare coincidental finding. The light and electron micrscopic findings in an eye enucleated from a 38yearold woman with iris naevus coganreese syndrome are presented. Objective to evaluate the spectrum of iridocorneal endothelial syndrome, to our knowledge, never studied previously in orientals. J charamissurgical treatment of glaucoma in essential progressive atrophy of the iris. Oct 30, 2019 cogan s syndrome is a chronic longlasting disease. Biology of the corneal endothelium in health and disease eye. Glaucoma group of disorders characterized by a progressive optic neuropathy resulting in characteristic appearance of optic disc and a specific pattern of irreversible visual field defects associated with raised intraocular pressure. Cogan syndrome is a rare autoimmune disease involving the eye and the inner ear. Diagnosis and management of iridocorneal endothelial syndrome.
Iridocorneal endothelial syndromecoganreese syndrome. The subtype was classified as coganreese syndrome owing to the absence of corneal edema and iris hole formation as well as ectropion uveae and corectopia. Chandler syndrome, essential progressive iris atrophy, cogan reese syndrome iris nevus syndrome represent a spectrum of ice syndrome. Sep 07, 2018 this condition is called cogan syndrome after the american ophthalmologist david glendenning cogan 190893 who first described it.
Cogan reese syndrome, ice syndrome, specular microscopy, ultrasound biomicro. Cogan syndrome causes problems of hearing and balance and also inflammation of the cornea and often fever, fatigue, and weight loss. Cogan syndrome eye disorders merck manuals professional. While there have been some cases of cogan reese syndrome reported in children, the disease is typically observed in females in the midadult years.
Clinical hallmarks are interstitial keratitis ik and vestibuloauditory dysfunction 1. Distinguishing features of the iridocorneal endothelial. Optical coherence tomography characteristics of the iris. Get a printable copy pdf file of the complete article 2. Feb 01, 2015 cogan syndrome is a rare disease characterized by coexisting audiovestibular and ocular symptoms. Tan, pedunculated nodules may appear on the anterior iris surface, the nodules seen are composed of underlying iris stroma pinched off by abnormal cellular membrane 18. Associations between cs and systemic vasculitis, as well as aortitis, also exist 24. Iridocorneal endothelial syndrome in thai patients. Histopathology in the irisnevus coganreese syndrome. The purpose is to present a case of iridocorneal endothelium syndrome with glaucoma and discuss clinical presentation and management strategies. Cogan lived during one of the most exciting and creative eras in ophthalmology, and he was interested in every aspect of the field, exemplified by the eponyms cogan s plaque, cogan reese syndrome, cogan s syndrome of interstitial keratitis with vestibuloauditory symptoms, and cogan s lidtwitch sign. Coganreese syndrome is characterized by proliferation of the.
Treatment of coganreese syndrome usually involves the use of drops in the eyes to control the glaucoma and swelling edema. Cogan syndrome is a rare vasculitis of young adults that is primarily characterized by 1,4,6. The term iridocorneal endothelial syndrome ice was suggested by eagle and yanoff which denotes a spectrum of disease characterized by primary corneal endothelial abnormality. Cogan s syndrome becomes atypical when the eye andor ear involvement is of a. Cogan reese syndrome nord national organization for rare. Fiftyeight patients with ice syndrome were identified. Iris mammillations in two female siblings with congenital. Cogan reese syndrome is a clinical variant of iridocorneal endothelial ice syndrome see this term characterized by variable iris atrophy, pigmented and. Many had corneal edema, and all had changes in the iris consisting of one or more of the following. Symptoms come and go, or respond well to treatment, but usually the disease is prolonged or even lifelong. Coganreese syndrome cornea jama ophthalmology jama. The subtype was classified as cogan reese syndrome owing to the absence of corneal edema and iris hole formation as well as ectropion uveae and corectopia. Cogan reese syndrome presents with the corneal findings and pigmented nodules on the iris.
Cogan reese syndrome is one of the three clinical variants of iridocorneal endothelial syndrome ice characterized by nodular, pigmented lesions of the iris which are hallmark, and may be seen with the entire spectrum of corneal and other iris defects. Cogan reese syndrome nord national organization for. Chandler syndrome is the most common variant, and its hallmark is corneal edema. The management ice syndrome has been debatable for a long time. 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. Jun 16, 2010 rituximab is a monoclonal antibody inducing depletion of b lymphocytes and presently approved for the treatment of nonhodgkins lymphoma and rheumatoid arthritis. Study conducted to report a case of cogan reese syndrome.
Cogan syndrome is a rare condition that causes inflammation in your eyes and ears. Pdf diagnosis and management of iridocorneal endothelial. Photograph taken in 1992 demonstrating nodules characteristic of the iridocorneal endothelial syndrome coganreese variant covering the iris from the 7 to. Get a printable copy pdf file of the complete article 44m. A 45 year old female presented with redness, pain and diminision of vision in left eye. Photographs taken in 1997 figure 3 and figure 4 illustrate a clear progression of the nodules over. The development of coganreese syndrome is gradual, and may be preceded by symptoms of essential iris atrophy andor chandlers syndrome. Essential iris atrophys hallmark is large stretch holes and irregularities of the iris. 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.
Cogan reese syndrome, progressive essential iris atrophy, and chandler syndrome. Irisnevus syndrome cogan reese syndrome the extent of iris atrophy tends to be variable and less severe. Chandlers syndrome, cogan reese syndrome, and essential iris atrophy. Mr of the inner ear in patients with cogan syndrome. Pdf the iridocorneal endothelial ice syndrome is a rare ocular disorder that includes a. Coganreese syndrome is characterized by multiple small pedunculated round nodules on the anterior iris surface. Fourteen patients ten women, four men with iris nevus coganreese syndrome, all having unilateral glaucoma, were studied clinically. 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. Sep 12, 2018 ice syndrome includes many different conditions. Cogan reese syndrome pdf cogan reese syndrome is a clinical variant of iridocorneal endothelial ice syndrome see this term characterized by variable iris atrophy, pigmented. Mild eye disease may be treated with antiinflammatory medications, including steroids and nsaids that are applied to. 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. Cogan reese syndrome s hallmark is pigmented, pedunculated iris masses. Ultrasound biomicroscopy of chinese eyes with iridocorneal.
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. Researchers arent sure about its exact origin, but many consider it an autoimmune disease. To document the ultrasound biomicroscopic ubm findings in chinese patients with iridocorneal endothelial ice syndrome. Express minishunt can be considered a surgical option for iridocorneal endothelial syndrome. The iris naevus coganreese syndrome british journal of. This variant of ice syndrome is distinguised by its cogan reese subtype of ice syndrome. Slit lamp biomicroscopy revealed semidilated pupil not reacting to light and variation in iris colour. We report the case of a 38yearold woman who presented with severe medium and large vessel vasculitis.
Iridocorneal endothelial syndrome an overview sciencedirect. Nov 10, 2017 cogan syndrome is a rare condition that causes inflammation in your eyes and ears. Here is the first report of the use of this drug in a case of cogan s syndrome cs. Patients with coganreese syndrome showed more extensive, often arborized pas. Cogans syndrome becomes atypical when the eye andor ear involvement is of a different type or when. Treatment of icerelated glaucoma often requires glaucoma filtering surgery with. The common features of cogan reese syndrome include fine iris nodules, peripheral anterior synechiae, loss of crypts, ectropion uveae and secondary glaucoma. In our particular case, taking into account that the subject was a young, phakic, pregnant woman, whose intraocular pressure had to be closely controlled and we had to ensure that. The management ice syndrome has been debatable for a. Cogan reese syndrome definition of cogan reese syndrome. Iridocorneal endothelial syndrome ice essential iris. Female patient, 55 years old with diagnosis of cogan reese syndrome. Cogan reese variant is characterized by multiple pigmented iris nodules 2,6. The clinicalmanifestations of this disease, namely.
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. Detection of the folded iris surface, increase of iris thickness, and increase of the distance between the anterior and posterior iris layers with rtvueoct anterior segment examination may promote the correct diagnosis and may help the clinician to discriminate cogan reese syndrome from. Thepathological findings in patients with cogan s syndrome are however extremely diverse and suggest a mixed type of inflammatory. Its composition allows the ostium to remain open and the device triggers a milder postoperative inflammatory response. At that visit, her iop was 57 mmhg, and there was evidence of anterior chamber inflammation. While there have been some cases of coganreese syndrome reported in children, the disease is typically observed in females in the midadult years. The strange case of a triangular pupil american academy. The classic form of the disease was first described by d.
Coganreese syndrome is one type of iridocorneal endothelial ice syndrome. When cogan and reese described a similar condition associated with iris. With their finding that the isolated 12residue cogans peptide is homologous to four autoantigenslaminin, connexin 26, the collagen diseaseassociated ssaro, and the receptorlike phosphatase dep1cd148and one viral protein, claudio lunardi and colleagues sept 21, p 9151 have advanced greatly identification of the eye, inner ear, nervous system and endothelium autoantigens of cogan. Lisch nodules in neurofibromatosis, tapioca melanoma of the iris, inflammatory iris granulomata and cogan reese syndrome should be considered in the differential diagnosis. A rare form of artery inflammation arteritis of unknown cause that affects the ear. A 47yearold woman was first diagnosed as having iridocorneal endothelial syndrome in 1988. Almost half of patients develop systemic manifestations. Rituximab ameliorated severe hearing loss in cogans syndrome. Photographs first taken in 1992 demonstrated some ectropian uvea and nodules covering the iris from the 7 to 3. In this case report, the authors present a case of a bilateral iris mammillations in two siblings with congenital adrenal hyperplasia cah. The literature suggests that posterior polymorphous dystrophy ppd may show features such as iridocorneal adhesions, glassy membranes, and pupillary ectropion which are typically ascribed to the iridocorneal endothelial ice syndrome. Dec 02, 2019 cogan reese syndrome is a clinical variant of iridocorneal endothelial ice syndrome see this term characterized by variable iris atrophy, pigmented and.
This subset involves the presence of multiple iris nodules, surrounded by iris stromal loss of crypts and a. Coganreese syndrome cornea jama ophthalmology jama network. Cogans syndrome as an autoimmune disease the lancet. Articles from the british journal of ophthalmology are provided here. Express minishunt implanted in a pregnant patient with. It is not clear that cogans syndrome is a primary vasculitis. The more severe extent and height of pas observed by ubm in progressive iris atrophy and coganreese syndrome support the evidence of a more severe glaucoma observed in these ice subtypes than in chandlers syndrome 1, 42. Pdf the iridocorneal endothelial ice syndrome is a progressive clinical spectrum of corneal. The ice syndromes predominantly affect caucasian, young to middleaged women, and involve one eye. This subset involves the presence of multiple iris nodules, surrounded by iris stromal loss of crypts and a matted appearance. Oct 10, 2017 the general characteristics of the cornea, iris, pupil, and anterior chamber angles were compiled and examined. Methods from 1986 to 1998, we examined 60 consecutive patients 20 men, 40 women with characteristic signs of iridocorneal endothelial syndrome. A 23yearold male was referred to the emergency room with a 3month history of peripheral facial palsy, hearing loss, bilateral optic disc edema, and headaches refractory to painkillers in the last few weeks. Coganreese syndrome djo digital journal of ophthalmology.
The matted appearance of the iris and development of nodules on the iris distinguish coganreese syndrome from the other iridocorneal endothelial syndromes. Iridocorneal endothelial ice syndromes are a spectrum of diseases characterized by slowly progressive abnormalities of the corneal endothelium and features including corneal edema, iris distortion, and secondary angleclosure glaucoma. Cogan reese syndrome definition of cogan reese syndrome by. The patient was diagnosed with ice syndrome based on the observation of marked ectropion uveae, peripheral anterior synechia, and abnormalities of the corneal endothelium. Looking for online definition of cogan reese syndrome in the medical dictionary. The ice syndrome is usually unilateral although occasionally bilateral and. Sep 30, 2014 cogan reese syndrome is one type of iridocorneal endothelial ice syndrome. Cogan syndrome affects young adults, with 80% of patients between 14 yr and 47 yr. Patients with cogan reese syndrome showed more extensive, often arborized pas. The more severe extent and height of pas observed by ubm in progressive iris atrophy and cogan reese syndrome support the evidence of a more severe glaucoma observed in these ice subtypes than in chandlers syndrome 1, 42. 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. Methods the inner ears of six patients with cogan syndrome were studied. This variant is most commonly unilateral and seen in middleaged females 2. This does not usually cause marked pupillary displacement.
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