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1/6. choroidal neovascularization in long-standing case of Vogt-Koyanagi-Harada disease.

    The eyes in a case of Vogt-Koyanagi-Harada disease (VKH) with long-standing uveitis for 26 years after the onset were studied histopathologically. It was found that typical granulomatous inflammation was persistent in the uveal tract and the choroidal neovascularization occurred in the peripheral fundus accompanied by proliferation of the retinal pigment epithelial cells (RPE). Some of the new vessels under the pigment epithelium extended into the vitreous. It was concluded that the ocular inflammation of VKH was essentially granulomatous even in this long-standing case. Disappearance of choroidal melanocytes, existence of epithelioid cells containing pigment granules, and accumulation of lymphocytes and plasma cells in the lesion indicated that the inflammation was an autoimmune reaction against uveal melanocytes, although the trigger initiating the disease remains unknown. It was further concluded that the peripheral fundus as well as the peripapillary and macular areas was a predilected site for choroidal neovascularization in chronic uveitis. The choroidal neovascularization may develop in such a way that the uveal inflammation damages the Bruch's membrane and choriocapillaris and consequently causes retinal ischemia, thus stimulating the endothelium of the choriocapillaris and the overlying RPE to proliferate. There is a close relation between choroidal neovascularization and proliferation of RPE. choroidal neovascularization may cause reactive proliferation of the RPE and vice versa.
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2/6. Subretinal neovascularization in the Vogt-Koyanagi-Harada syndrome.

    Two Hispanic patients with Vogt-Koyanagi-Harada (VKH) syndrome each developed a disciform lesion involving the macula of one eye several months after the onset of symptoms. Each had extraocular manifestations which included pleocytosis of the cerebrospinal fluid. The disciform lesions were associated with retinochoroidal anastomoses, a finding not previously reported in VKH syndrome. Each patient had a separate extramacular disciform lesion in the same eye. Two other Hispanic patients with diffuse bilateral intraocular inflammation had ocular findings consistent with VKH syndrome. One of these patients developed bilateral peripapillary disciform lesions and the other developed a disciform macular scar in one eye. fluorescein angiography in each patient showed early irregular hyperfluorescence with late intense staining. The disciform detachments occurred in areas of reactive proliferation of the retinal pigment epithelium, and we postulate that growth of subretinal new vessels occurred through areas of Bruch's membrane that were damaged by the inflammation.
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3/6. Retinal arteriovenous anastomoses in the Vogt-Koyanagi-Harada syndrome.

    A 15-year-old black girl had acute bilateral uveitis and other features of the Vogt-Koyanagi-Harada syndrome. Several months after the onset of her illness, she developed acquired retinal arteriovenous anastomoses in both eyes. By using fluorescein angiography, we documented that the anastomoses were collateral vessels developing in areas of damaged retinal pigment epithelium and retinal capillaries.
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4/6. indocyanine green video-angiographic findings in Harada's disease.

    indocyanine green (ICG) video-angiography was performed in 8 cases of Harada's disease and the findings were compared with those of fluorescein angiography. ICG video-angiography was confirmed to be far superior to fluorescein angiography in evaluating the choroidal vasculature seen in Harada's disease. Before retinal detachment appeared, the early phase of ICG video-angiography revealed decreased numbers of large fluorescent choroidal vessels as well as patchy areas of hypofluorescence. When retinal detachment became prominent, the large fluorescent choroidal vessels seen on ICG video-angiography were significantly fewer in number and patchy areas of hypofluorescence could be seen in the early phase. In the late phase, pooling of dye leakage became prominent. When retinal detachment subsided, the number of fluorescent choroidal vessels had increased. These results suggest that the decreased number of large fluorescent choroidal vessels seen in the early phase of ICG video-angiography indicate circulatory disturbance of the choroidal vessels, the degree of which may correspond to the exudative retinal detachment characteristic of Harada's disease. Patchy areas of hypofluorescence indicated blockage due to multiple choroidal granulomas and Dalen-Fuchs nodules.
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5/6. indocyanine green angiographic findings in Vogt-Koyanagi-Harada disease.

    PURPOSE: To report the indocyanine green angiographic findings associated with Vogt-Koyanagi-Harada disease and compare them with fluorescein angiographic findings and monochromatic scanning laser images. methods: In a prospective study, indocyanine green angiography, by scanning laser ophthalmoscopy or infrared fundus photography, was performed in ten consecutive patients (20 eyes) with Vogt-Koyanagi-Harada disease during the acute stage before and recovery stage after corticosteroid treatment. Findings were compared with fluorescein angiographic features and monochromatic scanning laser imaging. RESULTS: During the acute stage of the disease, indocyanine green angiography disclosed a dark background in the early phase and multiple, non-uniform hypofluorescent lesions in the midphases. Lesions were more numerous and extensive than areas either of serous retinal detachment on monochromatic scanning laser imaging or of punctate hyperfluorescence on fluorescein angiography. During the recovery stage, the abnormal dark background on indocyanine green angiography at initial examination resolved, with choroidal vessels visible in all cases, but nonuniform hypofluorescent lesions persisted in most eyes. fluorescein angiography disclosed hypofluorescent patchy areas, and confocal infrared laser imaging showed some bright reflective lesions in three patients with especially severe clinical symptoms. On final examination after an average of 17.7 months, both angiographies still disclosed abnormal findings in these three patients. CONCLUSIONS: indocyanine green angiographic findings suggest that choroidal inflammation may cause a transient choroidal circulatory disturbance during the acute stage of Vogt-Koyanagi-Harada disease. In more severe cases, this dysfunction may secondarily damage the retinal pigment epithelium.
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6/6. Concurrent bilateral posterior scleritis and Vogt-Koyanagi-Harada disease in a patient with positive rheumatoid factor.

    We examined a 67-year-old man who complained of headache and pain and decreased visual acuity in both eyes. The patient had dilated conjunctival and episcleral vessels, intracameral cells, shallow anterior chambers, hyperemic swollen optic disks, exudative retinal detachment and choroidal detachment. fluorescein angiography showed leakage of dye from the optic disks and choroid into the subretinal space. Scleral thickening was found by computed tomography. These abnormal findings disappeared within 1 week after treatment with a systemic corticosteroid. Laboratory test results showed positive rheumatoid factor. Sunset glow fundi and vitiligo developed a few months after the treatment. We believe that concurrent bilateral posterior scleritis, Vogt-Koyanagi-Harada disease and positive rheumatoid factor, as demonstrated in our patient, may be rare.
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