Cases reported "Retinal Degeneration"

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1/18. 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.
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ranking = 1
keywords = neovascularization
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2/18. 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.
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ranking = 1
keywords = neovascularization
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3/18. indocyanine green angiography in Sorsby's fundus dystrophy.

    PURPOSE: To report the indocyanine green angiographic findings in patients with a fundus dystrophy characterized by subretinal deposits, macular atrophic or neovascular degeneration and peripheral chorioretinal atrophy which is most likely Sorsby's fundus dystrophy. methods: A series of 11 clinically affected patients and 4 asymptomatic carriers, belonging to one autosomal dominant pedigree were examined with stereoscopic funduscopy, fluorescein and indocyanine green angiography. RESULTS: Subretinal deposits were found in 20 eyes of 10 patients. These deposits stained slightly on indocyanine green angiography, causing a reticular pattern. Two eyes had a disciform lesion and 3 geographic atrophy in the macula. A peripheral disciform lesion was found in 1 eye. indocyanine green angiography identified peripapillary choroidal neovascularization in 2 eyes. Peripheral chorioretinal atrophy was found in 8 eyes of 4 patients, associated with peripheral plaques that could only be identified by indocyanine green angiography in 6 eyes of 3 patients. CONCLUSION: indocyanine green angiography in Sorsby's fundus dystrophy may indicate the presence of homogeneously staining, well-demarcated peripheral areas of hyperfluorescence associated with chorioretinal atrophy. These plaques correspond in our opinion to choroidal neovascularization which is otherwise unsuspected.
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ranking = 2
keywords = neovascularization
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4/18. Hereditary retinal dystrophies and choroidal neovascularization.

    BACKGROUND: choroidal neovascularization infrequently occurs in patients affected by hereditary retinal dystrophies. methods: We studied eight patients suffering from different hereditary retinal dystrophies (Best's disease, reticular dystrophy, butterfly-shaped dystrophy, gyrate atrophy, and retinitis pigmentosa) who developed choroidal neovascularization. All patients underwent complete ophthalmic evaluation, electrophysiology, colour vision testing, and fluorescein angiography. In some patients, ICG video-angiography was also performed. Laser treatment was carried out in only one patient. RESULTS: The mean duration of follow-up was 41.7 months (range 6-148 months). At CNV diagnosis, the mean VA was 0.23 (range 0.02-0.6). At the last follow-up, mean VA was 0.34 (range HM to 0.9). At the last follow-up, fluorescein angiography showed a focal, atrophic scar in seven eyes, a fibrotic membrane in two eyes and a still active membrane in two cases. CONCLUSION: We emphasize the relatively favourable visual prognosis in patients suffering from inherited retinal dystrophies complicated with choroidal neovascularization. Therapeutic approaches other than laser treatment could be attempted in these patients.
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ranking = 7
keywords = neovascularization
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5/18. Best's vitelliform macular dystrophy.

    A patient with Best's vitelliform macular dystrophy in his right eye had an intact vitelliform disk and drusen of the optic papilla. Although the foveola elevated by a few tenths of a millimeter, resolution was only slightly affected and the retinal receptors remained remarkably well-aligned, suggesting that mechanisms subserving receptor alignment still functioned. Hue discrimination and visual acuity were only slightly affected and the electroretinogram and visually evoked response showed essentially normal values. The vitelliform disk in the left eye ruptured by direct, blunt trauma. This same eye later developed subretinal macular neovascularization, as its visual acuity reduced, retinal sensitivity decreased, receptor orientation showed abnormalities, and hue discrimination became less efficient; the electroretinogram and visually evoked response remained normal.
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ranking = 1
keywords = neovascularization
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6/18. indocyanine green angiographic interpretation of reticular dystrophy of the retinal pigment epithelium complicated by choroidal neovascularization.

    A 37-year-old woman presented with flashes in her left eye and bilateral visual distortion. Fundal examination revealed a reticular 'fishnet' pattern of retinal pigmentation in both eyes consistent with reticular dystrophy of the retinal pigment epithelium. In the left eye there was a small haemorrhage and a shallow serous macular detachment. fluorescein angiography demonstrated subfoveal choroidal neovascularization. indocyanine green angiography (ICG) revealed more extensive involvement than fluorescein angiography, with small areas of intense hyperfluorescence amongst reticular areas of hypofluorescence. These changes, as interpreted in light of the known histopathological localization of ICG, are consistent with varying stages of dysfunction of the retinal pigment epithelium in this disease.
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ranking = 5
keywords = neovascularization
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7/18. Senile mecular degeneration. A clinicopathologic correlation of two cases with neovascularization beneath the retinal pigment epithelium.

    The histopathologic findings in two cases of senile macular degeneration were correlated with the premortem clinical studies, from the onset in both cases and through the disciform stage in one case. In case 2, the postmortem eyes were obtained only one month after ophthalmoscopic, fluorescein angiographic, and fundus photographic studies were performed, all on the same day. Our report not only adds to the few clinicopathologic correlations of the lesions in senile macular degeneration reported in the literature, but also presents the first reconstruction to scale of subretinal pigment epithelial (sub-RPE) neovascularization. Our results confirm the diagnosis of sub-RPE neovascularization and serous detachment of the retinal pigment epithelium. Drusen were correlated as to number and general location but not individually. Serous detachment of the neurosensory retina was found histopathologically but not clinically.
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ranking = 6
keywords = neovascularization
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8/18. Vitreoretinal disorders in anterior megalophthalmos.

    PURPOSE: To describe vitreoretinal abnormalities in patients with anterior megalophthalmos (AM) and to report the results of vitreoretinal surgery in these cases. methods: In a retrospective, noncomparative case series, a diagnosis of AM was made according to the following criteria: megalocornea, iris hypoplasia, lens subluxation, cataract formation at an early age, and absence of congenital glaucoma. Vitreous biomicroscopy and funduscopy were performed in all patients. Patient records were also reviewed. RESULTS: Twenty-four patients with AM (12 male, 12 female), aged 4 months to 64 years, were included in this study. Funduscopy was possible in 46 eyes. Vitreous degeneration was the most common abnormal posterior segment finding, present in 34 eyes (73.9%). Nine eyes (19.5%) had optically empty vitreous with strands. Lattice degeneration was seen in five eyes (10.8%). Spontaneous vitreous hemorrhage occurred in three eyes, and peripheral retinal neovascularization was detected in two. retinal detachment occurred in 18 eyes (37.5%); retinal breaks were detected in eight of these before surgery. Thirteen eyes underwent a total of 18 procedures. Retinal reattachment was finally achieved in 12 of the 13 eyes. CONCLUSIONS: Anterior megalophthalmic eyes seem to be affected by a type of vitreoretinopathy predisposing to retinal detachment. Current vitreoretinal surgical techniques usually achieve good anatomic results in these cases.
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ranking = 1
keywords = neovascularization
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9/18. Sequential observation of fundus changes in patients with long standing membranoproliferative glomerulonephritis type II (MPGN type II).

    Specific fundus changes have been reported in patients with membranoproliferative glomerulonephritis type II (MPGN type II). We studied the clinical course of this retinopathy in four patients who all had a long follow-up with several fundus examinations. Sequential observation was indicative of a slow progression of the retinopathy. Most eyes maintained in the chronic stages a nearly normal visual acuity, and a full visual field despite the existence of marked drusen and atrophic changes. The prognosis however must be somewhat guarded, since choroidal neovascularization developed in three eyes and caused bilateral severe visual loss in one patient.
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ranking = 1
keywords = neovascularization
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10/18. Hemorrhagic peripheral pigment epithelial disease.

    Hemorrhage under the retinal pigment epithelium and neurosensory retina occurred in the temporal periphery of ten patients with aging macular degeneration and one patient with histoplasmosis syndrome. The macula was not the source of bleeding. Peripheral subretinal neovascularization, commonly seen histopathologically, may be the cause. The visual recovery was poor, and most patients failed to regain lost vision once peripheral hemorrhage occurred. The differential diagnosis includes retinal detachment, uveal effusion, hemorrhagic retinoschisis, choroidal melanoma, and metastatic tumor.
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ranking = 1
keywords = neovascularization
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