1/5. gyrate atrophy of choroid and retina.gyrate atrophy of choroid and retina is a rare disorder of autosomal recessive nature. There occurs patchy and progressive atrophy of the choroid and retina at the equatorial region with central area being less affected. Here in this case report, one woman of about 47 years attended at the retina clinic, Tenennt Institute of ophthalmology, Glasgow University with the history of gradual loss of vision. On fundus examination, sharply defined bizarre shaped atrophic areas of fundus was seen in both the eyes. Velvet like fine granular pigments were present in the macula, the zone of healthy retina and the periphery. The colourless, elongated, glittering crystals were scattered over the dark brown pigments visible through 90 dioptre lens. Bone corpuscles pigments were not found. fluorescein angiography showed hyperfluorescence in the area of gyrate atrophy. Her plasma ornithine level and plasma tiramine level were 1 90 U mol/l and 357 U mol/l. respectively. A rigid schedule of low protein diet including near total elimination of arginine with supplementation of essential amino acids was advised since the diagnosis was established.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/5. Serpiginous choroidopathy: an unusual association with Crohn's disease.Serpiginous choroidopathy, an inflammatory chorioretinopathy characterized by areas of choroidal atrophy and scarring, has previously been described in association with various systemic granulomatous disorders, but has not been linked to Crohn's disease. There are reports of ocular posterior segment abnormalities in patients with this chronic, granulomatous, inflammatory bowel disease, but these have not included serpiginous choroidopathy. This is the first report suggesting a link between serpiginous choroidopathy and Crohn's disease. A 22-year-old woman presented with serpiginous choroidopathy in whom Crohn's disease was diagnosed soon after. After starting treatment with oral prednisolone, no further visual loss or progression of the chorioretinal lesions occurred. The T-cell mediated nature of both inflammatory disorders and the temporal association in their onset suggests a possible link between serpiginous choroidopathy and Crohn's disease.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/5. Calcification at the posterior pole in scleritis. A case report.We report a case with unilateral recurrent anterior scleritis which developed sclerochoroidal calcification at the posterior pole without any overt sign of posterior scleritis during the follow-up period of nine years. We believe that subtle posterior scleral inflammation might lead to sclerochoroidal calcification which might be dystrophic in nature.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/5. Ophthalmologic findings in long-chain 3-hydroxyacyl-coa dehydrogenase deficiency caused by the G1528C mutation: a new type of hereditary metabolic chorioretinopathy.OBJECTIVE: The purpose of the study was to determine the nature and course of ophthalmic abnormalities in long-chain 3-hydroxyacyl-coa dehydrogenase (LCHAD) deficiency, a recently discovered disorder of mitochondrial fatty acid beta-oxidation. STUDY DESIGN: The study design was a cohort (case series). PARTICIPANTS: A retrospective review of the records of 15 children who had died during their first 2 years was performed. Also performed were a longitudinal reanalysis and cross-sectional clinical examination of four long-term survivors aged 5 to 31 years. MAIN OUTCOME MEASURES: visual acuity, refraction, visual fields, ophthalmoscopy, fluorescein angiography, biometry, corneal topography, electroretinography (ERG), visual-evoked potentials (VEPs), color vision, and dark adaptation were measured. RESULTS: In seven children, ophthalmoscopic findings were within normal limits at 3 days to 13 months of age (median, 4.8 months). In 11 children, a granular retinal pigment epithelium (RPE), with or without pigment clumping in the macula, was seen at 4 months to 5 years of age (median, 9 months). Two long-term survivors, 16 and 31 years of age, eventually had circumscribed atrophy of the choroid, RPE, and retina, which coincided with a posterior staphyloma type 1. They had progressive axial myopia starting at 6 and 12 years of age and later paracentral scotomas leading to poor central vision. They suffered from early difficulty with mesopic vision, glare, and a severe generalized color vision deficiency that started as a tritanomaly. A third survivor was mildly myopic at 5 years of age. All four surviving patients had visually insignificant, flake-like supranuclear opacities in the lens. The ERG initially was normal but deteriorated during the first decade and later was unrecordable. The VEP responses remained fairly normal. Initially, angiography showed no blockade of the choroidal fluorescence because of the thin RPE. Filling of choroidal vessels was delayed, and the choriocapillaris and, later, larger choroidal vessels in the posterior pole became nonperfused. CONCLUSIONS: In LCHAD deficiency, the fundus is normal at birth (stage 1). Soon, however, pigment dispersion occurs in the RPE (stage 2), followed by circumscribed chorioretinal atrophy, occlusion of choroidal vessels, and deterioration of central vision, often with relative sparing of the peripheral fundus (stage 3). Finally, posterior staphylomas and central scotomas may develop (stage 4). Developmental cataract, progressive myopia, and deterioration of visual fields and color vision are new findings in LCHAD deficiency. The chorioretinopathy and abnormal ERG precede the development of myopia and posterior staphyloma, which, in turn, coincide with the loss of macular vision. The authors postulate that the RPE or choriocapillaris is primarily affected. awareness of the characteristic ocular features is important because of an opportunity for dietary treatment, genetic counseling, and prenatal diagnosis.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/5. Long-term follow-up of severe central serous chorioretinopathy using indocyanine green angiography.BACKGROUND: The severe types of central serous chorioretinopathy (CSC) have a chronic nature, suggesting that a pathological process persists subclinically. indocyanine green (ICG) angiography recently revealed intrachoroidal dye leakage and its static nature in CSC. As the intrachoroidal dye leakage was suspected to be relevant to the disease process, the long-term persistence of intrachoroidal ICG leakage was examined in four patients of the severe types of CSC. methods: ICG angiography was performed periodically over more than three years in three patients and two years in one patient. One patient had CSC with bullous retinal detachment, and the other three had chronic CSC or diffuse retinal pigment epitheliopathy. RESULTS: Intrachoroidal ICG leakage persisted in all the patients. However, a change in location of persistent intrachoroidal leakage or disappearance of intrachoroidal leakage regardless of no progression of retinal pigment epithelial alteration was noted in one eye of two patients. CONCLUSIONS: pathology causing intrachoroidal ICG leakage persisted subclinically for a long period. However, location and extent of the intrachoroidal leakage could change during a long-term follow-up period.- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |