Cases reported "Retinal Diseases"

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1/51. A case report of frosted branch angiitis and its visual electrophysiology.

    We reported a 5-years old boy with frosted branch angiitis in both eyes. The visual acuities of the two eyes were suddenly lost to 5/50. The fluorescein angiography, electroretinograms (ERGs) and pattern evoked potentials (PVEPs) had been tested at the acute and the recovery stage of the disease. At the onset of the disease, fluorescein angiography showed obvious dye leakage from dilated retinal veins. The PVEPs and whole field ERGs including rod, maximum, OPs and cone responses were seriously decreased and prolonged, indicating that the visual pathway's and retinal functions were greatly damaged. The patient had an excellent response to systemic prednisone. One month after the treatment, the visual acuities recovered to 60/50 in both eyes. The fluorescein angiography showed that leakage from blood vessels was diminished, but the lesions at macula and optic disc were persisted a longer time even to the end of treatment. Through 6 months medication, nearly all of the electrophysiological abnormalities had been resolved.
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2/51. diagnosis of vitreoretinal adhesions in macular disease with optical coherence tomography.

    PURPOSE: To compare the relative incidence of vitreoretinal adhesions associated with partial vitreous separation within the macula diagnosed with optical coherence tomography (OCT) with that of those diagnosed with biomicroscopy. methods: The authors obtained linear cross-sectional retinal images using OCT in patients with selected macular diseases. Additional studies included biomicroscopy, fundus photography, fluorescein angiography, and B-scan ultrasonography. RESULTS: Optical coherence tomography was performed on 132 eyes of 119 patients. Vitreoretinal adhesions within the macula were identified using OCT in 39 eyes (30%) with the following diagnoses: idiopathic epiretinal membrane (n = 13), diabetic retinopathy (n = 7), idiopathic macular hole (n = 7), cystoid macular edema (n = 7), and vitreomacular traction syndrome (n = 5). Biomicroscopy identified vitreoretinal adhesions in only 11 eyes (8%). Two distinct vitreoretinal adhesion patterns were identified with OCT, each associated with partial separation of the posterior hyaloid face: focal (n = 25) and multifocal (n = 14). CONCLUSIONS: Optical coherence tomography is more sensitive than biomicroscopy in identifying vitreoretinal adhesions associated with macular disease.
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3/51. indocyanine green angiographic findings of chorioretinal folds.

    PURPOSE: To analyze indocyanine green (ICG) angiographic findings of chorioretinal folds. methods: Eight patients (9 eyes) in whom chorioretinal folds had been diagnosed were enrolled in this study. color photography, fluorescein angiography (FA) and ICG angiography (IA) were performed. RESULTS: indocyanine green angiography demonstrated choroidal venous congestion and a filling delay of the choroidal vessels in one case with an orbital tumor. In one posterior scleritis case, IA showed a filling delay of choroidal vessels in the early phase and multiple patchy hypofluorescent lesions scattered in the posterior pole during the late phase. Idiopathic cases showed choroidal venous dilatation. No abnormalities of the choroidal vasculature in the form of radial folds, were revealed in two cases of AMD. Linear hyperfluorescent areas suggestive of chorioretinal folds seen on IA were less numerous and wider than those observed on FA in some eyes. On the other hand, they were equally numerous and wider on IA than those on FA in other eyes. CONCLUSION: indocyanine green angiography is useful for evaluating both pathological conditions of the choroidal vasculature and the width of chorioretinal folds at the level of the choroidal vasculature.
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4/51. Clinical and electrophysiological findings in autosomal dominant vitreoretinochoroidopathy: report of a new pedigree.

    PURPOSE: To report the clinical and electrophysiological findings in a three-generation pedigree with autosomal dominant vitreoretinochoroidopathy. methods: Sixteen members of a three-generation pedigree with autosomal dominant vitreoretinochoroidopathy were examined clinically, including measurement of the corneal diameter. In 14 persons, Goldmann perimetry, axial length determination and electro-oculography were carried out. electroretinography, according to ISCEV standards, was performed in 11 of 12 affected persons. RESULTS: Characteristic annular peripheral pigmentary changes were present in all affected members, as well as chorioretinal atrophy varying from a tigroid aspect to marked atrophy. Four patients presented a microcornea and shallow anterior chamber without microphthalmia. The visual fields appeared to narrow with ageing. The electro-oculography was pathological in the affected patients and normal in the unaffected. The electroretinographic amplitude responses tended to worsen with age, with maintenance of near normal latencies. CONCLUSION: The clinical presentation of autosomal dominant vitreoretinopathy is variable. electrooculography seems to be a discriminative test. The condition may be associated with anterior segment abnormalities other than presenile cataract, such as microcornea, shallow anterior chamber and angle closure glaucoma.
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5/51. Ischemic maculopathy in patients with acquired immunodeficiency syndrome.

    PURPOSE: To describe the characteristics of ischemic maculopathy in patients with human immunodeficiency virus (hiv) infection, as a means of understanding this uncommon disorder more fully. methods: This is a multicenter, retrospective review of clinical data available for five hiv-infected patients who were given the diagnosis of ischemic maculopathy. RESULTS: All cases had been diagnosed on the basis of fluorescein angiograms obtained after patients complained of vision loss. Four of the five patients had bilateral macular disease. visual acuity at presentation in the nine affected eyes ranged from 20/20 to count fingers. Vision loss was gradual in both eyes of one patient and was abrupt in onset in seven eyes. Each of the seven eyes with abrupt vision loss had opacification of the superficial retina and/or intraretinal hemorrhages near the fovea. fluorescein angiography revealed enlargement of the foveal avascular zone and mild staining of the juxtafoveal vessels in affected eyes. Six eyes had active or clinically inactive cytomegalovirus retinitis at presentation, and a seventh eye developed cytomegalovirus retinitis 2 weeks later. All patients were receiving anticytomegalovirus drugs when they developed visual symptoms. visual acuity remained stable in five eyes, became worse in two eyes, and improved in two eyes; final visual acuity ranged from 20/25 to count fingers. CONCLUSIONS: Ischemic maculopathy may cause profound and permanent vision loss in hiv-infected individuals. fluorescein angiography should be considered in all hiv-infected patients with unexplained loss of vision. The pathogenesis of ischemic maculopathy remains unknown.
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6/51. Multifocal electroretinography in multifocal choroiditis and the multiple evanescent white dot syndrome.

    PURPOSE: To study and compare the findings on multifocal electroretinography (MERG) between multifocal choroiditis (MFC) and the multiple evanescent white dot syndrome (MEWDS). SUBJECT AND methods: patients were recruited prospectively from the Department of ophthalmology & Visual Sciences at the University of iowa hospitals & Clinics. They were evaluated using Goldmann visual fields (GVF) and MERG. patients were diagnosed as having either MFC or MEWDS based on their clinical findings before MERG testing. RESULTS: Nineteen patients (23 eyes) were included in the study. Eleven patients were diagnosed with MFC and eight patients with MEWDS. Fourteen eyes with MFC and seven eyes with MEWDS were tested with MERG during the acute phase of their respective conditions. Fourteen patients (8 MFC and 6 MEWDS) were followed serially with MERG. patients with MEWDS demonstrated focal depression corresponding to GVF defects with subsequent near total recovery of the MERG to baseline. patients with MFC typically demonstrated diffuse loss of function over the entire test field. Focal scotomata, in addition to the diffuse depression, could be identified in 7 of 14 patients. patients with MFC demonstrated only partial or no recovery of MERG following acute episodes, which was significantly different from the course followed by patients with MEWDS (P < 0.001, Fisher's exact test). CONCLUSION: Multifocal electroretinography differentiates MFC from MEWDS. patients with MFC have permanent damage to the retina with diffuse depression of MERG. patients with MEWDS, however, typically demonstrate greater focal loss initially on MERG followed by nearly full recovery of first order retinal function.
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7/51. Retinal visualization in an eye with corneal crystals using indocyanine green videoangiography.

    PURPOSE: To report a patient in whom clear imaging of the retina, impossible with conventional methods, was obtained using indocyanine green (ICG) videoangiography. DESIGN: Interventional case report. methods: A 31-year-old patient with nephropathic cystinosis and complaints of decreased vision in the left eye underwent a complete ophthalmologic evaluation, including conventional photography, fluorescein angiography, and ICG videoangiography. RESULTS: With conventional methods (direct and indirect ophthalmoscopy, photography, and fluorescein angiography), the view of the left retina was obscured by densely packed corneal cystine crystals. During ICG angiography, clear imaging of the retina was obtained with near-infrared illumination. CONCLUSION: indocyanine green videoangiography can be a useful tool in cases with cystinosis and other corneal opacities, where visualization and imaging of the retina are important but impossible with conventional methods.
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8/51. Eales' disease with neurological manifestations.

    Eales' disease is a primary retinal perivasculitis of an undetermined etiology seen predominantly in the Indian subcontinent. However, neurological involvement is rare. We report here a patient of retinal perivasculitis with neurological dysfunction. Our patient is a 39 years male who developed acute diminished vision right eye in March 99, which progressed for four days and remained static. In April 99 he developed acute diminished vision left eye, which progressed to near total blindness in 48 hours. He was undergoing ophthalmic evaluation. Fourty five days later he developed incoordination and weakness left half of body. The examination revealed bilateral retinal perivasculitis with pyramidal signs and left sided cerebellar signs. Investigations revealed an ESR of 40 mm at the end of first hour. His CT head revealed bilateral basal ganglionic infarcts. MRI head revealed enhancing lesions both basal ganglia and right parietal region. cerebrospinal fluid examination showed xanthochromic fluid with markedly elevated protein and lymphocytic pleocytosis. His workup for connective tissue disorders was negative. He was put on ATT with steroids. Eales' disease is presumed allergy to tuberculoprotein. A trial of ATT with steroids has been tried with varying results. Our patient had bilateral retinal perivasculitis and neurological dysfunction. He had lymphocytic pleocytosis with markedly elevated protein in the CSF.
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9/51. 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.
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10/51. Macular hole formation following ruptured retinal arterial macroaneurysm.

    PURPOSE: To report four cases of macular hole formation following ruptured retinal arterial macroaneurysm. DESIGN: Case-control study. methods: review of clinical records of 75 consecutive eyes (73 patients) with macroaneurysm. Macroaneurysms were confirmed using either fluorescein angiography or indocyanine green angiography or both that indicated a telangiectatic retinal vascular change. Clinical data of patients with macroaneurysm with and without macular hole (MH) formation were compared retrospectively. RESULTS: Of 75 eyes, four (5.3%; four patients) with macroaneurysm had a macular hole. In one of the four eyes, a macular hole was detected 2 days after the rupture. The distance from the macroaneurysm to the center of the fovea was significantly closer in the four eyes with a macular hole (MH group, 1.1 /- 0.4 (mean /- standard deviation [SD]) disk diameter) than in the 71 eyes without a macular hole (non-MH group, 1.9 /- 0.9 disk diameter) (P =.0196). The incidence of subretinal hemorrhage in the macula was significantly greater in the MH group (100%) than in the non-MH group (35.2%) (P =.0195). CONCLUSIONS: Macular hole formation associated with ruptured macroaneurysm is not as uncommon as previously thought. We suggest that there might be early onset of macular hole formation following ruptured macroaneurysm. Macroaneurysms with early-onset macular hole are present near the center of the macula and are accompanied by subretinal hemorrhage in the macula.
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