11/22. Pigment changes of the retina in chronic progressive external ophthalmoplegia (CPEO).A study was made of the retinal functions in 4 patients with chronic progressive external ophthalmoplegia, general myopathy, EEG anomalies and pigment changes of the fundus oculi (ophthalmoplegia-plus). Three of them exhibited typical, granular pigmentations in a linear or reticular arrangement at the periphery. All four showed slight to moderate pigment epithelial defects in the maculae, mostly only discernible with fluorescence angiography. In all 4 cases, a slight decrease of the visual acuity, a mildly abnormal ERG, mild concentric restriction of the field of vision and, in two cases, an abnormal dark-adaptation curve led to the conclusion of a mild diffuse, widely disseminated receptor affection of the retina (both rods and cones). The EOG appeared normal in 3, and at the lower limit of normal in 1 case. On the basis of a detailed study of the literature we can conclude that the retinal lesions in chronic progressive external ophthalmoplegia may vary from benign pigmentations without functional impairment to genuine 'retinitis pigmentosa' with all gradations of rod-cone or cone-rod dystrophy. Emphasis is laid on the possibility of a correlation between the mitochondrial abnormalities encountered in ocular myopathy and ophthalmoplegia-plus on the one hand, and the retinal abnormalities on the other, with special reference to a possible disorder of the utilization of pyruvate in the citric-acid cycle and a loose coupling of the oxidative phosphorylation.- - - - - - - - - - ranking = 1keywords = fluorescence (Clic here for more details about this article) |
12/22. Bilateral slowly progressive big blind spot syndrome.A 33-year-old woman was found with bilateral enlargement of the blind spot without optic disc edema. Her bilateral peripapillary retinas appeared slightly decolored and elevated with obscure margins and fluorescein angiography indicating only slight hyperfluorescence in the late venous phase. Four years after her initial visit, both of her blind spots became enlarged further and dark adaptation was remarkably difficult. Peripapillary retinal lesions appearing as retinal pigmentary degeneration were evident with very definitive margins. The lesions showed strong hyperfluorescence in the arteriovenous phase owing to window defects. From these findings, it appears that bilateral big blind spots without optic disc edema may possibly result from peripapillary retinal degeneration, which may become evident at a much later date.- - - - - - - - - - ranking = 2keywords = fluorescence (Clic here for more details about this article) |
13/22. Inflammatory pigmented paravenous retinochoroidal atrophy.A 47-year-old Japanese man had a progressive degeneration of the retina and choroid along the retinal veins associated with uveitis of two years' duration. The lesion was characteristic of paravenous retinochoroidal atrophy: a contiguous atrophy of the retinal pigment epithelium and choroid of one-half to one disc diameter in size was present along most of the veins from the posterior pole to the far periphery. fluorescein angiography showed a window defect in the retinal pigment epithelium, with hyperfluorescence representative of retinal pigment epithelium and choriocapillaris degeneration. Good visual acuity was attained after extracapsular cataract extraction for complicated cataract and vitrectomy for severe vitreous opacity had been performed in both eyes. The cause of this new inflammatory disease was unknown.- - - - - - - - - - ranking = 1keywords = fluorescence (Clic here for more details about this article) |
14/22. Degenerative changes in a retina affected with autosomal dominant retinitis pigmentosa.The eyes of a 17-year-old male donor who was affected with autosomal dominant retinitis pigmentosa with variable expressivity have recently become available for study. Initial macroscopic examination of the fundus revealed bone spicules located in 180 degrees of the postequatorial fundus centered on the inferonasal quadrant. light microscopic examination of the retina showed degeneration within each quadrant characterized by an absence of rods and cones in the equatorial areas, and the presence of photoreceptors in the more peripheral and central retina. Ultrastructural examination disclosed photoreceptors that were abnormal in all regions when compared to a control eye from a 26-year-old donor. Intact rods were restricted to the peripheral quadrants, and intact cones were identified in the fovea and far periphery. In areas of intermediate degeneration, many outer segments were either shortened and disorganized or absent. Regions of severe degeneration were characterized by the complete loss of the photoreceptors and apposition of the external limiting membrane to the retinal pigment epithelium. The density of rods and cones was found to be substantially lower than normal in all regions. In areas of relatively intact photoreceptor outer segments, we found ultrastructural evidence of recent phagocytic activity, and fluorescence microscopy revealed no unusual accumulation of lipofuscin within the pigment epithelium or subepithelial debris. The choroid and inner retina were normal throughout the eye. The normal condition of the choroid, retinal pigment epithelium, and inner retina implies that the primary disorder resides within the photoreceptor cell.- - - - - - - - - - ranking = 1keywords = fluorescence (Clic here for more details about this article) |
15/22. Retinal pigment epithelial window defect.The clinical and histopathologic features of a solitary retinal pigment epithelial window defect are presented. fluorescein angiography disclosed an area with early choroidal fluorescence but no late staining. Histopathologic studies showed an intact but attenuated retinal pigment epithelium with partial to complete loss of melanin granules, partial atrophy of the photoreceptor cells, and sclerosis of the choriocapillaris.- - - - - - - - - - ranking = 1keywords = fluorescence (Clic here for more details about this article) |
16/22. Central pigmentary retinal dystrophy and its angiographic classification.Twenty-one cases of central pigmentary retinal dystrophy were compiled and their clinical features were described. On the basis of fluorescein fundus angiography, these cases were classified into three types according to the changes in the choriocapillaris. In Type 1, the choriocapillaris showed annular or bow-shaped atrophy around the vascular arcade in the posterior pole of the fundus, but little change in the foveal region. Type 2 exhibited round or irregularly shaped atrophy of the choriocapillaris in the center of the posterior pole of the fundus. Type 3 had no atrophy of the choriocapillaris and showed only hyperfluorescence in the posterior pole of the fundus, due to damage of the retinal pigment epithelium. These types differed from each other not only from the morphological point of view, but also in other clinical findings that included age of onset, progression of visual impairment, pattern of color vision defects or recordability of the electroretinogram. It was discussed that central pigmentary retinal dystrophy consists of heterogenous conditions.- - - - - - - - - - ranking = 1keywords = fluorescence (Clic here for more details about this article) |
17/22. indocyanine green angiography in Stargardt's flavimaculatus.PURPOSE: We studied the indocyanine green videoangiographic characteristics of eyes in patients with Stargardt's flavimaculatus and fluorescein angiographic evidence of a dark choroid. methods: Affected individuals underwent ophthalmic examination and fluorescein angiographic examination. indocyanine green videoangiography was performed on eight patients with classic Stargardt's flavimaculatus. Two additional asymptomatic patients with mild manifestations of Stargardt's flavimaculatus, both of whom were related to one patient with the classic phenotype, were also examined with indocyanine green videoangiography. RESULTS: Choroidal detail was evident in all patients examined with indocyanine green videoangiography, and varying degrees of choroidal vascular closure were documented in the maculas of eight patients. Retinal pigment epithelial flecks were found to block indocyanine green videoangiographic fluoresence progressively. Late indocyanine green videoangiographic imaes frequently showed retinal pigment epithelial involvement in areas of retina thought to be uninvolved clinically and by fluorescein angiography. Peripapillary crescents of hypofluorescence, which in some patients were not noted clinically or by fluorescein angiography, were observed in all ten patients examined with indocyanine green videoangiography. In one asymptomatic patient, retinal pigment epithelial flecks could be identified only with indocyanine green videoangiography. CONCLUSIONS: indocyanine green videoangiography in conjunction with fluorescein angiography can be a valuable tool in the recognition and further understanding of Stargardt's flavimaculatus.- - - - - - - - - - ranking = 1keywords = fluorescence (Clic here for more details about this article) |
18/22. Multifocal Best's vitelliform dystrophy.Three members of a family had multifocal, macular and extramacular--Best's vitelliform dystrophy. The lesion in one patient was observed over a ten-year period. A striking symmetry of locale and evolution of these lesions is noted between the eyes of a patient as well as among the three members of the family. The pseudohypopyon of the vitelliform cyst and the vitelliform deposits showed fluorescence before fluorescein injection. A hypofluorescent halo surrounded most lesions.- - - - - - - - - - ranking = 1keywords = fluorescence (Clic here for more details about this article) |
19/22. Clinical course of newly developed or progressive patchy chorioretinal atrophy in pathological myopia.Regional chorioretinal atrophy in the posterior fundus (patchy chorioretinal atrophy) in pathological myopia impairs vision severely when it covers the macula. The aim of this study was to assess the course of development and progression of patchy chorioretinal atrophy in pathological myopia. The location and progression of patchy chorioretinal atrophy that was either newly developed or had progressed during the follow-up period (mean 5.25 years) were analyzed. A total of 41 lesions of patchy atrophy were newly developed in 30 eyes of 25 patients. These lesions were more likely to occur in marginal regions of a posterior staphyloma but frequency per unit area was highest in the macula. There were 138 lesions of patchy chorioretinal atrophy that progressed in 75 eyes of 53 patients. Sixty percent of the lesions of patchy chorioretinal atrophy in marginal regions of a posterior staphyloma spread toward the center. Seventy percent of the lesions of patchy chorioretinal atrophy in the macula spread in all directions. fluorescein angiography of newly developed patchy chorioretinal atrophy showed hyperfluorescence in 50% and hypofluorescence in 27%. Fluorescein angiography of progressive lesions of patchy chorioretinal atrophy showed hypofluorescence in 69%. fluorescein angiography of some progressive areas of patchy chorioretinal atrophy, which showed a change from hyperfluorescence to hypofluorescence within several years, suggested that damage to the retinal pigment epithelium preceded the progression of the patchy chorioretinal atrophy. In conclusion, the patchy chorioretinal atrophy is most likely to occur in the macula and to enlarge in all directions. And it is suggested that the patchy chorioretinal atrophy which shows hyperfluorescence by fluorescein angiography should be kept under observation because our data suggest that this finding indicates progression in the future.- - - - - - - - - - ranking = 6keywords = fluorescence (Clic here for more details about this article) |
20/22. Vitelliform dystrophy in a 64-year-old man.A 64-year-old white man had many large bilateral vitelliform-like lesions. A normal electroretinogram, abnormal elector-oculogram, and a pattern of blocked choroidal fluorescence were obtained. The yellowish appearance of the lesions progressed to absorption of yellow material and mottling with maintenance of relatively good visual acuity.- - - - - - - - - - ranking = 1keywords = fluorescence (Clic here for more details about this article) |
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