Dec 05, 2017 cogan syndrome is a rare autoimmune disease that affects the eyes and inner ears. Create a free personal account to download free article pdfs, sign up for alerts, customize your interests, and more. In a group of disorders collectively known as ice syndrome, corneal endothelial degeneration leads to corneal edema, alterations to the anterior chamber angle, and structural changes of the iris. Oct 25, 2020 cogan reese syndrome is a clinical variant of iridocorneal endothelial ice syndrome see this term characterized by variable iris atrophy, pigmented and. Purposeto present a case with coexistence of cogan reese syndrome and exfoliation syndrome. Central to the pseudoangle, endotheliumandnew abm were present on the inner surface of the trabecular meshwork.
Coganreese syndrome cornea jama ophthalmology jama network. Chandlers syndrome nord national organization for rare. Cogan reese syndrome is one type of iridocorneal endothelial ice syndrome. Backed by our structural warranty, every cogan product is a reflection of the steadfast reliability we value and you deserve. Endothelium also had extendedonto theanterioriridic surface, whereit had. Lisch nodules in neurofibromatosis, tapioca melanoma of the iris, inflammatory iris granulomata and cogan reese syndrome should be. Iris mammillations in two female siblings with congenital. This variant of ice syndrome is distinguised by its cogan reese subtype of ice syndrome. Iridocorneal endothelial syndrome and secondary glaucoma. Glaucoma associated with disorders of the corneal endothelium. This disorder differs from cogan corneal dystrophy, which is inherited as an autosomal dominant disorder. Cogan syndrome also cogan s 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.
Study conducted to report a case of cogan reese syndrome. This variant is most commonly unilateral and seen in middleaged females 2. Cogan reese syndrome iris nevus syndrome see the ice slide set, k26, for more details. The condition is actually a grouping of three closely linked conditions. Atypical cogan s syndrome audiovestibular dysfunction with other types of inflammatory eye disease is associated.
Cogans syndrome definition of cogans syndrome by medical. A rare variant of iridocorneal endothelial syndrome, the iris nevus syndrome cogan reese syndrome, is the result of corneal endothelial overgrowth across the angle and over the iris surface, which gives rise to multiple small iris nodules and secondary glaucoma. Cogan reese syndrome, ice syndrome, specular microscopy, ultrasound biomicro. Apr 26, 2016 ice syndrome is a group of disorders with three clinical variants. Get a printable copy pdf file of the complete article 3. A 32yearold woman presented with elevated intraocular pressure and diminution of vision in her right eye for 3 months.
Typical cogan s syndrome is a rare disease of young adults consisting of flares of interstitial keratitis and sudden onset of menierelike attacks nausea, vomiting, tinnitus, vertigo and hearing loss. Cogan reese syndrome is characterized by distortion of iris tissue and the development of small wartlike growths on the iris. May 31, 2019 resultsfiftyeight patients with ice syndrome were identified. Semantic scholar extracted view of the iris nevus and cogan reese syndromes. Pdf iridocorneal endothelial syndrome in a 16yearold. Cogans syndrome genetic and rare diseases information. The ice syndrome encompasses 3 clinical variationschandler syndrome cs, 1 progressive iris atrophy pia, 2 and cogan reese syndrome crs 3 that are believed to form a spectrum of disease manifestation. Descemets stripping automated endothelial keratoplasty in patient. Increased pressure within the eye and corneal swelling are also evident. Symptoms of the syndrome include irritation and pain in the eyes, decreased vision, hearing loss, and vertigo. Create a free personal account to make a comment, download free article pdfs, sign up for alerts and more. Cogan reese syndrome figure 2 is characterized by the presence of.
Summary cogan reese syndrome is a rare iridocorneal endothelial syndrome that most commonly affects young to middleaged women. Pdf endothelial keratoplasty in the management of irido. Cogan reese syndrome merupakan variasi klinis dari iridocorneal endothelial ice syndrome. Iridocorneal endothelial ice syndromes are a spectrum of disea. The ice syndromes predominantly affect caucasian, young to middleaged women, and involve one eye. Photographs first taken in 1992 demonstrated some ectropian uvea and nodules covering the iris from the 7 to 3. Full text is available as a scanned copy of the original print version.
Treatment of cogan reese syndrome usually involves the use of drops in the eyes to control the glaucoma and swelling edema. By biomicroscopic examination there was no abnormalities in the right eye. 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 reese syndrome nord national organization for. Decreased endothelial permeability in the iridocorneal endothelial syndrome. Association of cogan reese syndrome, exfoliation syndrome, and secondary openangle glaucoma. Consistent with cogan reese syndrome, there were multiple fine nodules arrow distributed over the inferior degrees of the iris. Specific findings on anterior segment oct that aid in detecting cogan reese syndrome include increased iris thickness 10.
Cogan reese syndrome is the most common form and is strongly associated with glaucoma. Sep 30, 2014 cogan reese syndrome is one type of iridocorneal endothelial ice syndrome. 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. Lifethreatening aortic insufficiency develops in 10% of reported cases. Cogan reese syndrome nord national organization for rare. This complex unilateral secondary angle closure glaucoma represents a variant of iridocorneal endothelial syndrome with unknown etiology though viral. Oct 15, 2019 register for email alerts with links to free fulltext articles access pdfs of free articles manage your interests save searches and receive search alerts. Cogan reese syndrome is an extremely rare eye disorder characterized by a matted or smudged appearance to the surface of the iris.
Cogan syndrome is a rare, rheumatic disease characterized by inflammation of the ears and eyes. Coganreese syndrome cornea jama ophthalmology jama. A yearold woman was first diagnosed as having iridocorneal endothelial syndrome in she underwent a trabeculectomy in photographs first. Glaucoma may occur as a secondary disorder to cogan reese syndrome. Pathogenesis of chandlers syndrome, essential iris atrophy and the. The three variations of ice syndrome chandler syndrome, essential iris atrophy, and cogan reese iris nevus syndrome. So when you choose a cogan product, you always get a superior solution made with the best quality materials, the latest technology, and the finest workmanship. Cogan reese syndrome is a clinical variant of iridocorneal endothelial ice syndrome see this term characterized by variable iris atrophy, pigmented and. Cogan reese syndrome memiliki gambaran nodul berpigmen pada iris dan tidak tampak struktur. Iridocorneal endothelial syndrome in thai patients. A syndrome of iris nodules, ectopic descemets membrane, and unilateral glaucoma. Cogan reese syndrome definition of cogan reese syndrome. Iris cyst in a case of cogan reese syndrome is being reported for the. Iridocorneal endothelial ice syndrome is a disorder with a broad spectrum.
Resultsfiftyeight patients with ice syndrome were identified. Cogan reese variant is characterized by multiple pigmented iris nodules 2,6. Coganreese syndrome genetic and rare diseases information. Association of coganreese syndrome, exfoliation syndrome. Essential progressive iris atrophy among the three clinical variants of ice syndrome, chandler syndrome appears to be the most common 5. Ice syndrome can be subdivided into progressive iris atrophy pia, chandlers syndrome cs, and cogan reese syndrome crs. Diagnosis and management of iridocorneal endothelial syndrome. Cogan reese syndrome has a clinical features of pigmented nodules on the iris and does not appear to have normal iris structure. Treatment management of cogan reese, similar to that of the other ice syndromes, is aimed at slowing or preventing vision loss from secondary angleclosure glaucoma by lowering the patients. Iris mammillations are dark brown, smooth, mound or domeshaped protuberances that are typically found on the anterior iris surface and are presumed to be congenital in origin.
Sign cgan to access your subscriptions sign in to your personal account. Express minishunt implanted in a pregnant patient with. This congenital anomaly is usually unilateral and can be hereditary or sporadic. Female patient, 55 years old with diagnosis of cogan reese syndrome.
Literature search the authors searched pubmed on january 8, 2018, for englishlanguage results using the following terms sin. Medical therapy and trabeculectomy have a low success rate. 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. Cogan reese syndrome is a clinical variation of the iridocorneal endothelial ice syndrome characterized by multiple small pedunculated round nodules on the anterior iris surface, iris atrophy, heterochromia, ectropion uveae, and ectopic pupil, as well as corneal edema and iridocorneal angle abnormalities. Surgical treatment of glaucoma in essential progressive atrophy of the iris. Hollo g, kothy p, konstas ag eur j ophthalmol 2012 janfeb. Cogan s syndrome cs is a rare chronic inflammatory disease, characterized by nonsyphilitic ocular keratitis and menierelike cochleovestibular dysfunction, with relapsing attacks of vertigo, sudden onset of tinnitus, vomiting, and progressive, mostly bilateral sensorineural hearing loss 65. The condition may also be associated with bloodvessel inflammation called vasculitis in other areas of the body that can cause major organ damage in 15% of those afflicted. Ultrasound biomicroscopy of chinese eyes with iridocorneal. A 47yearold woman was first diagnosed as having iridocorneal endothelial syndrome in 1988.
Sep 30, 2019 coganreese syndrome is a clinical variant of iridocorneal endothelial ice syndrome see this term characterized by variable iris atrophy, pigmented and. Cogan reese has been called iris nevis syndrome since these outcroppings resemble small nevi on the iris surface. Slitlamp examination revealed minimally raised darkly pigmented iris nodules fig a with shallow anterior chamber. Skip to search form skip to main content semantic scholars logo. Pathogenesis of chandlers syndrome, essential iris atrophy and the coganreese syndrome. A 65 yearold woman medically free presented with one eye with ice syndrome figure 1. Cogan syndrome definition of cogan syndrome by medical.
Bordering the island of endothelial free stroma, the advancing edge of. In cogan reese syndrome, contracture of the proliferating endothelial membrane on the iris face forms multiple pedunculated outcroppings of normal iris tissue. Pdf study conducted to report a case of cogan reese syndrome. Iridocorneal endothelial ice syndrome is a disorder with a broad spectrum of clinical manifestations. Chandler syndrome essential progressive iris atrophy the status of the other two are subject to debate. Aug 01, 2019 cogan reese syndrome predominantly affects the iris and may present with myriad manifestations such as iris nevus, peripheral anterior synechiae, matted appearance of iris, loss of iris crypts, fine iris nodules, ectropion uveae, heterochromia and secondary glaucoma 1,2,4. What do ice and ppmd ppmd stand for, and which fuchs are we. What do ice and ppmd ppmd stand for, and which fuchs. Higher magnification of the inferior iris revealed heterogeneously pigmented iris nodules distributed over the iris surface associated with pupillary ruff. Cogan reese syndrome, a unilateral puzzling cause of secondary. Surgery was uneventful and the morning iop after 2 days was 12 mmhg without treatment. The exact cause of cogan syndrome is not wellunderstood. Other symptoms may include joint or muscle pain or inflammation of the blood vessels.
Specular microscopy is an important diagnostic tool, as the corneal endothelium has a characteristic appearance in ice syndrome patients. Management of cogan reese, similar to that of the other ice syndromes, is aimed at slowing or preventing vision loss from secondary angleclosure glaucoma by lowering the patients intraocular pressure. Cogan reese syndrome definition of cogan reese syndrome by. Conclusion the nsaids was the only treatment received by the patient and throughout the years, her cme. Case reporta 72yearold caucasian woman presented for consultation due to uncontrolled intraocular press. Cogan syndrome can lead to vision difficulty, hearing loss and dizziness. For information about clinical trials conducted in europe, contact. Cogan reese syndrome, a unilateral puzzling cause of. The classic form of the disease was first described by d. These are characterized by an abnormal, beaten metal appearance of the corneal endothelium, with or without corneal edema, and iris abnormalities of varying degrees of severity ranging from mild stromal atrophy to severe distortion, stretching, and polycoria. Progressive essential iris atrophy, chandlers syndrome, and the iris.
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