1/241. Extensive chorioretinal atrophy in Vogt-Koyanagi-Harada disease.PURPOSE: To report extensive chorioretinal atrophy during the long-term course of Vogt-Koyanagi-Harada (VKH) disease not treated properly in the initial phase. CASES: Four patients with VKH disease were examined more than 10 years after onset of the disease. OBSERVATIONS: They presented initially with classic features of VKH disease, except 1 patient who had developed bilateral, acute angle-closure glaucoma as the initial sign. Two patients received systemic corticosteroid therapy at the acute phase of the disease. During the follow-up of 13-34 years subsequent to onset, these patients had chronic recurrent anterior uveitis with apparently stable depigmented fundus. Eventually, they developed diffuse, extensive chorioretinal atrophy that resulted in severe visual loss. One patient had an unusual familial occurrence of the disease. CONCLUSIONS: Failure to prescribe proper corticosteroid therapy in the initial phase of VKH disease may lead to chronic recurrent uveitis. Long-standing uveitic reactions may eventually result in severe visual loss due to extensive chorioretinal degeneration.- - - - - - - - - - ranking = 1keywords = pigment (Clic here for more details about this article) |
2/241. Episcleritis associated with pigmentary retinal degeneration in an HTLV-I carrier.Human T-cell lymphotropic virus type I (HTLV-I) has been reported as the cause of a kind of endogenous uveitis (HTLV-I associated uveitis; HAU). We observed a case of episcleritis in a HTLV-I carrier with pigmentary retinal degeneration. HTLV-I infection might be associated with the development of episcleritis and pigmentary retinal degeneration. patients with episcleritis or pigmentary retinal degeneration should be examined for HTLV-I infection.- - - - - - - - - - ranking = 7keywords = pigment (Clic here for more details about this article) |
3/241. retinal degeneration associated with ectopia lentis.Two brothers had retinal degeneration, lens subluxation, and myopia since early life. There was no evidence of marfan syndrome, homocystinuria, or other systemic disease. They had nystagmus, myopia, inferior dislocation of the lens, and posterior subcapsular opacities in both eyes. Fundus examination showed attenuated retinal vessels, macular atrophy with occasional pigment accumulation as clumps, and perivascular sleeves. electroretinography revealed decreased photopic and scotopic responses. The visual fields were constricted. We believe this to be the first report of retinal degeneration with bilateral lens subluxation in a family. It appears to be inherited in an autosomal recessive fashion.- - - - - - - - - - ranking = 1keywords = pigment (Clic here for more details about this article) |
4/241. Focal retinal pigment epithelial dysplasia associated with fundus flavimaculatus.BACKGROUND: One or more focal dysplastic lesions of the retinal pigment epithelium (RPE) occurred in 15 eyes of 10 patients with fundus flavimaculatus. methods: review of patient records including an attempt to obtain follow-up information concerning a history of previous ocular trauma. RESULTS: Mild antecedent ocular trauma occurred to the eye with a dysplastic lesion in two patients. Dysplastic lesions were most frequently solitary and located temporal to the macula. Subretinal neovascularization accompanied two of the dysplastic lesions. The lesions were multifocal and present bilaterally in two patients. CONCLUSIONS: In fundus flavimaculatus, progressive lipofuscin storage is responsible for engorgement and hypertrophy of the RPE. Dysplastic lesions of the RPE probably result from reactive hyperplasia and fibrous metaplasia of RPE cells in response to acute disruption of fragile, hypertrophied RPE cells that may be enormously enlarged in the area of yellow flecks. This disruption may occur in response to trauma, focal inflammation, or other localized stimuli. patients with fundus flavimaculatus should be cautioned concerning the possible role of trauma in causing dysplastic changes in the RPE and visual loss.- - - - - - - - - - ranking = 70.774214340717keywords = retinal pigment, pigment (Clic here for more details about this article) |
5/241. Histopathological findings of X-linked retinoschisis with neovascular glaucoma.BACKGROUND: X-linked retinoschisis (XLRS) is rarely complicated by neovascular glaucoma. Only a few reports of XLRS histopathological findings with neovascular glaucoma have been published. methods: A 41-year-old man with XLRS complicated by neovascular glaucoma in his left eye was examined with electroretinography, B-scan, ultrasound biomicroscopy and computed tomography. He was examined by ophthalmoscopy and fluorescein angiography in the other eye. An enucleation was performed in his left eye due to uncontrollable high intraocular pressure and persistent ocular pain. We examined the enucleated eye histopathologically. RESULTS: Examination of the enucleated eye showed nuclear sclerosis of the lens, pigmented retrolental membrane and retinoschisis which separated the inner layer of the retina and made a large space in the vitreous cavity without any apparent detachment of the outer layers of the retina. Sclerotic vessels were present histopathologically in both the inner and outer layers of the retina. There was a peripheral anterior synechia, ectropion uveae and a fibrovascular membrane, which contained many lumina of neovascularization, indicating marked rubeosis iridis. Small cystic spaces were observed in both the schitic retina in the peripheral region and the foveal schisis at the outer layer of the retina. The photoreceptor cells had become markedly atrophied and multiple regions of calcification were observed. The optic nerve showed severe atrophy with gliosis, but the central retinal artery and vein were still open within the nerve. CONCLUSIONS: These histopathological findings suggest that rubeosis iridis may have developed secondarily to retinal ischemia due to occlusion of the retinal blood vessels.- - - - - - - - - - ranking = 2.905420795729keywords = detachment, pigment (Clic here for more details about this article) |
6/241. Progressive cone dystrophies.patients with progressive generalized cone dystrophy often present nystagmus (or strabism) and complain of photophobia, decrease in visual acuity or disturbances in colour perception. The most classic fundus abnormality is the bull's eye maculopathy or a pallor of the optic disc. Minimal macular changes are sometimes seen, which may progress to a bull's eye type of macular degeneration. The photopic ERG is always very affected, whereas at first the scotopic ERG seems normal. Progressive deterioration of the visual functions is accompanied by increasing fundus lesions and rod involvement, as suggested by the modifications of the dark adaptation curve and the scotopic ERG. However, the progression of typical generalized cone dysfunction is very slow. On the contrary, in some cases of so-called Stargardt's disease with peripheral participation, a very rapid progression has been observed. In such cases a normal ERG does not necessarily mean that the disease will remain localized to the macular area. No definite prognosis can be made on one single ERG. In 3 cases with sector pigmentary retinopathy the photopic ERG was more affected than the scotopic ERG. However, these cases are probably primary cone-rod dystrophies. Although there is no electrophysiological control, our clinical impression is that the evolution, if possible, is very slow.- - - - - - - - - - ranking = 1keywords = pigment (Clic here for more details about this article) |
7/241. bardet-biedl syndrome.This case report describes the presentation of a patient with bardet-biedl syndrome. bardet-biedl syndrome is an autosomal recessive condition that includes retinal dystrophy, dystrophic extremities (commonly polydactyly), obesity, hypogenitalism, and renal disease. Cognitive deficit has also been considered part of the syndrome. The historically associated laurence-moon syndrome includes spastic paraparesis but not the obesity and polydactyly. They are now considered separate conditions. The most common feature of bardet-biedl syndrome is retinal dystrophy. The appearance of the retina in the condition is quite variable with typical retinitis pigmentosa being present in only a minority of cases. The associated optic atrophy can be primary in nature and might play a role in the decreased central vision. diagnosis of the condition is important for visual prognosis and low vision management. The renal disease often goes undetected until specific radiological testing is done after diagnosis of bardet-biedl syndrome. This is significant in that early death often occurs in this condition because of the renal disease.- - - - - - - - - - ranking = 1keywords = pigment (Clic here for more details about this article) |
8/241. Unilateral macular retinoschisis with stellate foveal appearance in two females with myopia.Stellate foveal retinoschisis is considered the hallmark of X-linked retinoschisis. We describe the finding of unilateral retinoschisis with stellate foveal appearance in 2 females with myopia who had no evidence of familial disease. Optical coherence tomography (OCT) and fluorescein angiography were obtained in both cases. Neither patient had a family history of low vision, night blindness, or retinal detachment. visual acuity in the affected eyes was 20/50 and 20/25, respectively. Both fellow eyes had normal fundi, except for mild myopic changes. With OCT, widespread retinal splitting was detected at the posterior pole in the affected eyes, whereas the fellow eyes were normal. In one case, OCT showed that foveal retinoschisis represented the macular involvement of a flat inferior retinoschisis, although this was not clinically apparent. Macular retinoschisis with stellate foveal appearance may rarely be associated with pathologic myopia. OCT was useful to establish the true extension of these macular changes.- - - - - - - - - - ranking = 2.8935758436382keywords = detachment, retinal detachment (Clic here for more details about this article) |
9/241. Reattachment of retina and retinoschisis in pit-macular syndrome by surgically-induced vitreous detachment and gas tamponade.The origin of the subretinal fluid in pit-macular syndrome is unknown. Using optical coherence tomography, the authors observed that an optic disk pit is not a true pit but a cystic cavity covered with a superficial layer of the optic disk in a patient with retinal detachment and retinoschisis. The cyst was connected to the intraretinal space of the retina, which was split in several layers, or the retinoschisis in the papillomacular area. vitrectomy and gas tamponade were performed to treat the patient. During surgically-induced vitreous detachment, strong vitreoretinal attachment at the disk margin and the fovea was observed. After surgery, the cystic space at the optic disk disappeared and the retinal detachment and retinoschisis reattached. Vitreous traction may play a role to introduce the fluid from the optic cyst to the subretinal space through the superficially split retina.- - - - - - - - - - ranking = 15.314255665921keywords = detachment, retinal detachment (Clic here for more details about this article) |
10/241. retinal detachment and retinoschisis detected by optical coherence tomography in a myopic eye with a macular hole.The authors describe a myopic patient with a full-thickness macular hole that was accompanied by both retinal detachment and retinoschisis. A 51-year-old woman presented with a localized retinal elevation of three disc diameters around the macular hole in a myopic eye. Optical coherence tomography (OCT) showed features of both retinal detachment and retinoschisis. After vitreous surgery and postoperative intraocular gas tamponade, the macular elevation resolved, and separation of the neurosensory retina from the retinal pigment epithelium band disappeared on the OCT images. OCT is a useful tool for examining macular conditions associated with a macular hole in myopic patients.- - - - - - - - - - ranking = 27.563677738336keywords = retinal pigment, detachment, pigment, retinal detachment (Clic here for more details about this article) |
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