1/11. Cogan's syndrome: organ-specific autoimmune disease or systemic vasculitis? A report of two cases and review of the literature.Cogan's syndrome is a rare disorder of unknown origin characterized by inflammatory eye disease and vestibuloauditory symptoms. Typically, young adults suffer from interstitial keratitis and sudden onset of tinnitus and hearing loss. Few cases (around 150) have been published and thus it is difficult to determine the percentage of patients with underlying systemic disorders such as systemic vasculitis. The variety of systemic manifestations is large and includes fever, splenomegaly, lymphadenopath, and musculoskeletal complaints. systemic vasculitis can be seen in around 10% of cases and may involve the large vessels, appearing as Takayasu-like vasculitis with affection of the aortic valve but also the coronary arteries and the small kidney vasculature. Evaluating the exact extension of the systemic features determines the choice of treatment. While corticosteroids have proved to be of short-term benefit, long-term treatment with immunosuppressive drugs is controversial. Auditory function in deaf patients has often been restored successfully with cochlear implants. To illustrate the nature of the syndrome, we present two patients with a wide clinical spectrum of symptoms from local disease restricted to the eyes and ears to a widespread vasculitis affecting arteries of the brain, kidney and the upper and lower extremities. We then review the typical aspects as well as the etiology of the disease.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/11. Structural alteration and destructive corneal disease.There is good evidence that maintenance of the structural integrity of the human cornea carries with it significant advantages over and above the preservation of normal function. The breakdown in the protective layers of the cornea and invasion by blood vessels and lymphatics would seem to add greatly to the risk of recurrent inflammatory disease. There is a solid rationale, therefore, for the development of therapeutic principles aimed at preventing these structural changes from occurring.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/11. Chronic and delayed-onset mustard gas keratitis: report of 48 patients and review of literature.PURPOSE: To report the clinical features of 93 eyes of 48 patients with chronic and delayed-onset mustard gas keratitis. Clinicopathologic correlation in 5 eyes and a review of related literature are presented. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Forty-eight Iranian survivors of Iraqi chemical warfare with chronic or delayed-onset mustard gas keratitis. methods: We reviewed the symptoms, clinical findings, course, and treatment of our patients and reviewed the literature. In 5 patients, histopathologic features of corneal and conjunctival specimens were evaluated. MAIN OUTCOME MEASURES: Ocular findings, clinical course, treatment measures, and histopathologic studies. RESULTS: Of 48 patients, 31 (64.6%) had chronic symptomatology, whereas 17 (35.4%) experienced delayed-onset lesions. visual acuity at referral ranged from hand motions to 20/20. Ocular surface changes included chronic blepharitis and decreased tear meniscus in all patients, limbal ischemia (81.3%), and conjunctival vascular abnormalities (50%). Corneal signs in order of frequency were: scar or opacity (87.5%), neovascularization (70.8%), thinning (58.3%), lipoid deposits (52.1%), amyloid deposits (43.8%), and epithelial defects and irregularity (31.3%). Many patients received conservative treatment; others underwent allograft stem cell transplantation (20 eyes of 17 patients), penetrating keratoplasty (12 eyes of 12 patients), and lamellar keratoplasty (4 eyes of 3 patients). Conjunctival specimens were evaluated by light microscopy. Decreased goblet cell density, attenuated or thickened epithelium, scarring in the substantia propria associated with plasmacytic and lymphocytic infiltration, and dilated lymphatic vessels were noted. Excised corneal buttons disclosed absence of epithelium and Bowman's layer, fibrovascular pannus, stromal scarring, and vascularization. CONCLUSIONS: mustard gas causes chronic and delayed destructive lesions in the ocular surface and cornea, leading to progressive visual deterioration and ocular irritation. The pathophysiologic features of these changes are not clearly identified. Excised conjunctival and corneal specimens revealed a mixed inflammatory response without any specific features. Based on the clinical appearance of the lesions and the histopathologic findings, an immune-mediated component seems possible. This article contains additional online-only material available at.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/11. Hemicrania with massive autonomic manifestations and circumscribed eyelid erythema.OBJECTIVES: To describe a unilateral headache that in addition to the typical shortlasting unilateral neuralgiform headache attacks with conjunctival injection, tearing, sweating and rhinorrhea (SUNCT) syndrome - traits with excessive and ipsilateral autonomic phenomena - had circumscribed eyelid erythema and adjacent ocular redness. OBSERVATIONS: A 60-year-old female had excessive, right-sided lacrimation and local pain at 15 years of age. Due to steadily increasing discomfort, with lacrimation and swelling over the outer part of the upper eyelid, the right lacrimal gland was removed at 20 years of age, with a suspicion of lacrimal gland adenoma. Preoperatively, symptomatic side mild-degree eyelid erythema/rhinorrhea were integral parts of the attack. After years with minor complaints, she, in the mid-twenties, experienced more long-lasting pain attacks, and pain soon became the main problem. A marked, distinct erythema on the lateral part of the right-sided eyelids and marked, localized 'eye redness' in the adjacent area were main ingredients of the attacks together with eyelid edema and viscous rhinorrhea. There were visible vessels below the eye, and telangiectasia of the upper eyelid. CONCLUSIONS: This headache has many similarities with SUNCT - but has several, grossly deviating traits: the temporal aspects, excruciatingly intense pain attacks, and above all marked, lateral eye-lid erythema, and adjacent, massive ocular reddening. This constellation probably alienates it from SUNCT.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/11. Cogan's syndrome with aortitis, aortic regurgitation, and aortic arch vessel stenoses.Cogan's syndrome of interstitial keratitis and vestibuloauditory dysfunction is rare. Systemic vasculitic manifestations occur, and 10% of patients with this syndrome have aortic valvular disease. A patient with Cogan's syndrome is presented who had aortitis of the ascending thoracic aorta, severe aortic valve regurgitation, orificial stenosis of the right coronary artery, and orificial stenoses of all three aortic arch vessels. Histopathology confirmed aortitis. aortic valve replacement combined with coronary and aortic arch vessel reconstruction was required for correction.- - - - - - - - - - ranking = 6keywords = vessel (Clic here for more details about this article) |
6/11. Late corneal opacities in the syndrome of interstitial keratitis and vestibulo-auditory symptoms.Progressive clouding of the cornea may be a delayed complication of the interstitial keratitis-vestibuloauditory syndrome. Two illustrative cases are presented. The first case illustrates progressive opacification about aberrant vessels in the deep stroma presenting a characteristic dendritiform pattern. The second case shows that the opacification is due, in this case at least, to formation of connective tissue with inclusion of lipid crystals and fat along with the blood vessels. Descemet's membrane is also thickened several fold.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
7/11. Spontaneous hyphaema and corneal haemorrhage as complications of microbial keratitis.Hyphaema developed spontaneously in 16 of 458 patients with microbial keratitis treated at two centres on the East and West Coasts of the united states. Chronic corneal conditions were often present, and three cases had rubeosis iridis. Inflamed iris vessels were assumed to be the source of the haemorrhage. The hyphaemas tended to persist longer than is usual, particularly when coincident with a hypopyon. Recurrent hyphaemas are reported in two patients from outside this series. Spontaneous corneal haemorrhage was seen in three cases. Subepithelial bleeding settled rapidly, but a combined midstromal and pre-Descemet's haematoma cleared more slowly. Anterior segment bleeding was significantly associated with advanced age, female sex, infection with Gram-positive organisms, and hypopyon.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/11. Anterior segment fluorescein angiography in inflammatory diseases of the cornea.To study the vascular changes in inflammatory diseases of the cornea 22 patients with various corneal inflammations were examined by means of anterior segment fluorescein angiography. Simple avascular central and marginal corneal ulcers stained with fluorescein in the late phase of angiography. An inflamed limbus and an early microscopic pannus adjacent to the ulcer were seeen in simple corneal ulcers. Progressive pannus with pronounced fluorescein leakage was observed in chronic corneal ulcer, disciform keratitis, Mooren's ulcer, and complicated acute keratoconus. In sclerokeratouveitis and in gutter associated with rheumatoid arthritis the corneal vessels showed less leakage. The iris vessels showed fluorescein leakage as a sign of irritative iritis during the active stage of simple and chronic corneal ulcers, in disciform keratitis, Mooren's ulcer, and in graft rejection. It is concluded that anterior segment fluorescein angiography gives valuable information of the vascular architecture, flow and leakage in inflammatory diseases of the cornea.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
9/11. temporal bone findings in Cogan's syndrome.We report on the pathological findings in temporal bones taken from a 92-year-old man who had been diagnosed as suffering from Cogan's syndrome before death. Extensive endolymphatic hydrops was observed in the cochlea of the right ear. The saccule showed a collapse following intense dilation. Outpouching of the utricle and crus commune was found and the perilymphatic space of the posterior semicircular canal was occupied by the fibrotic tissue. The left ear showed neither hydrops nor fibrosis. There was absorption of the enchondral bone in both ears, but in the blood vessels there were no pathological changes.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
10/11. A case of coronary stenosis developing after successful aortic valve repair in Cogan's syndrome.Cogan's syndrome has been described as entity of progressive deafness and interstitial keratitis with variable cardiovascular involvement leading to aortic insufficiency or orificial stenosis of coronary or aortic arch vessels. So far, aortic valve replacement either with mechanical or biological prosthesis was favored for correction of the valvular lesion. A patient with primary successful aortic valve repair followed by coronary revascularization for left coronary ostial stenosis occurring after the first operation is presented.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
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