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1/119. The ocular manifestations of congenital infection: a study of the early effect and long-term outcome of maternally transmitted rubella and toxoplasmosis.

    PURPOSE: To study the spectrum of adverse ocular effects which result from maternally transmitted rubella and toxoplasma infection; further, to record the long-term visual and neurodevelopmental outcomes of these 2 major causes of fetal infection. STUDY DESIGN AND patients: A series of 55 patients with congenital infection have been studied prospectively on a long-term basis. The study group included a cohort of 34 cases with congenital rubella syndrome demonstrated by virus isolation, and 21 cases with a clinical diagnosis of congenital toxoplasmosis and serologic confirmation. All patients had specific disease-related ocular defects. Rubella patients were first identified during or following the last major rubella epidemic in 1963-1964, and some have been followed serially since that time. A separate study group of representative toxoplasmosis patients presented for examination and diagnosis at varying time periods between 1967 and 1991. OBSERVATIONS AND RESULTS: This study confirms that a broad spectrum of fetal injury may result from intrauterine infection and that both persistent and delayed-onset effects may continue or occur as late as 30 years after original infection. Many factors contribute to the varied outcome of prenatal infection, the 2 most important being the presence of maternal immunity during early gestation and the stage of gestation during which fetal exposure occurs in a nonimmune mother. RUBELLA: As a criteria of inclusion, all 34 rubella patients in this study exhibited one or more ocular defects at the time of birth or in the immediate neonatal period. Cataracts were present in 29 (85%) of the 34, of which 21 (63%) were bilateral. Microphthalmia, the next most frequent defect, was present in 28 (82%) of the 34 infants and was bilateral in 22 (65%). glaucoma was recorded in 11 cases (29%) and presented either as a transient occurrence with early cloudy cornea in microphthalmic eyes (4 patients), as the infantile type with progressive buphthalmos (1 patient), or as a later-onset, aphakic glaucoma many months or years following cataract aspiration in 11 eyes of 6 patients. Rubella retinopathy was present in the majority of patients, although an accurate estimate of its incidence or laterality was not possible because of the frequency of cataracts and nystagmus and the difficulty in obtaining adequate fundus examination. toxoplasmosis: Twenty-one patients with congenital toxoplasmosis have been examined and followed for varying time periods, 7 for 20 years or more. The major reason for initial examination was parental awareness of an ocular deviation. Twelve children (57%) presented between the ages of 3 months and 4 years with an initial diagnosis of strabismus, 9 of whom had minor complaints or were diagnosed as part of routine examinations. All cases in this study have had evidence of retinochoroiditis, the primary ocular pathology of congenital toxoplasmosis. Two patients had chronic and recurrent inflammation with progressive vitreal traction bands, retinal detachments, and bilateral blindness. Macular lesions were always associated with central vision loss; however, over a period of years visual acuity gradually improved in several patients. Individuals with more severe ocular involvement were also afflicted with the most extensive central nervous system deficits, which occurred following exposure during the earliest weeks of gestation. CONCLUSIONS: Although congenital infection due to rubella virus has been almost completely eradicated in the united states, the long-term survivors from the prevaccination period continue to experience major complications from their early ocular and cerebral defects. They may be afflicted by the persistence of virus in their affected organs and the development of late manifestations of their congenital infection. Congenital toxoplasmosis continues to be the source of major defects for 3,000 to 4,100 infants in the united states each year; the spectrum of defects is wide and may vary from blindness and severe mental retardation to minor retinochoroidal lesions of little consequence. Effective solutions for either the prevention or treatment of congenital toxoplasmosis have not been developed in this country but are under intensive and continuing investigation.
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ranking = 1
keywords = choroid
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2/119. Fibroglial proliferation in pars planitis.

    In a 51-year-old patient with long-standing pars planitis, light and electron microscopical examination of the intravitreal 'snowbank' revealed a fibrovascular layer adjacent to the hyperplastic nonpigmented epithelium of the pars plana and an extensive fibroglial proliferation within the vitreous base. The fibrovascular layer consisted of well-differentiated capillaries, probably emanating from the peripheral retina, interspersed with aggregated vitreous fibrils. The fibroglial portion of the 'snowbank' was composed of fibrous astrocyte-like cells which had secreted basement membranes and larger diameter collagen fibrils. This fibroglial tissue was in direct continuity with an ultrastructurally similar preretinal membrane. No significant choroiditis or cyclitis could be demonstrated. It is hypothesized that, in pars planitis, a primary inflammatory process of the peripheral retina and vitreous base may stimulate the observed preretinal and intravitreal fibroglial proliferation.
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ranking = 0.5
keywords = choroid
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3/119. Relapsing polychondritis.

    BACKGROUND: Relapsing polychondritis (RPC) is a rare, chronic, and potentially fatal multisystemic inflammatory disorder targeting cartilaginous structures. This disorder is frequently associated with rheumatoid arthritis, systemic vasculitis, connective tissue diseases, and/or hematologic disorders. RPC afflicts patients with recurrent and often progressive episodes of inflammation with the potential for destruction of the affected structures. tissues involved include the ears, joints, nose, larynx, trachea, eyes, heart valves, kidneys,and skin. Ocular manifestations commonly include episcleritis, scleritis, conjunctivitis, iridocyclitis, chorioretinitis, and proptosis. Lid edema, orbital inflammation, muscle palsies, and corneal melting may also occur. CASE REPORT: An 83-year-old man previously diagnosed with RPC presented to our clinic with acute unilateral chemosis, conjunctivitis, lid edema, proptosis, and extraocular muscle restriction. After orbital cellulitis was ruled out, further evaluation revealed posterior scleritis with choroidal detachment OS. A course of oral indomethacin and topical antibiotic-steroid combination drops was implemented in the treatment of the ocular manifestations. The quick positive response to the anti-inflammatory agents confirmed the diagnosis of ocular complications secondary to RPC. DISCUSSION: The presenting ocular signs and symptoms of RPC often resemble other commonly encountered ocular conditions. It is important for the eye care practitioner to be familiar with the ocular manifestations of RPC because the eyes are sometimes the initial site of involvement and may be a marker of severity. early diagnosis and intervention may significantly improve the patient's outcome. This case report with literature review will hopefully bring to light features of this disease which will help the eye care practitioner in the diagnosis and management of this condition.
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ranking = 0.5
keywords = choroid
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4/119. Massive suprachoroidal hemorrhage with retinal and vitreous incarceration; a vitreoretinal surgical approach.

    Suprachoroidal hemorrhage(SH) may cause the expulsion of the intraocular contents. Vitreous incarceration in the wound and retinal detachment with SH are extremely poor prognostic signs. Treatment modalities depend on the severity of eye damage. This particular patient had "kissing" hemorrhagic choroidal detachment which completely filled the vitreous cavity after cataract surgery. It seemed to be inoperable. Secondary surgery was delayed 3 days to lower IOP to normal levels. The eye underwent anterior drainage sclerotomy under constantly-maintained limbal or pars plana infusion fluid line pressure. The authors performed a pars plana vitrectomy, followed by perfluorocarbon liquid injection and a silicone oil tamponade. After this surgical approach, the patient attained an attached retina and a visual acuity of 5/200 at the 3 month follow-up.
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ranking = 3
keywords = choroid
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5/119. Visual outcome after surgical removal of choroidal neovascularization in pediatric patients.

    OBJECTIVE: To assess the visual outcome after surgical removal of choroidal neovascularization (CNV) in pediatric patients. methods: A retrospective, noncomparative, consecutive case series of 17 eyes undergoing surgical removal of CNV of various causes in patients aged 18 years and younger. RESULTS: The cause of the CNV was presumed ocular histoplasmosis syndrome in 11 eyes, idiopathic in 3, and optic nerve coloboma, ocular toxoplasmosis, and trauma in 1 eye each. Two eyes had CNV within 100 microm of the center of the foveal avascular zone (juxtafoveal CNV) and 1 eye had peripapillary CNV, while 14 eyes had CNV beneath the geometric center of the foveal avascular zone (subfoveal CNV). In eyes with subfoveal CNV, median preoperative Snellen visual acuity was 20/200 (range, 20/80-3/200). With a median follow-up of 27 months (range, 6-45 months), median final visual acuity was 20/50 (range, 20/20-2/200); 10 (72%) had improvement of 2 or more Snellen lines after surgery, and 6 eyes (43%) had final visual acuity of 20/40 or better. In 3 eyes with juxtafoveal CNV or peripapillary CNV, all eyes had improvement of 4 or more Snellen lines. Postoperative recurrent CNV developed in 6 (35%) of 17 patients; 2 of the eyes underwent a second vitrectomy and 4 received laser treatment for the recurrences. CONCLUSIONS: Pediatric patients may have good recovery of vision after surgical removal of CNV, and the removal of these membranes may be a viable alternative to laser photocoagulation in pediatric patients.
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ranking = 2.5
keywords = choroid
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6/119. Retinal pigment epithelial tear with vitreomacular attachment: a novel pathogenic feature.

    BACKGROUND: The development of tears of the retinal pigment epithelium (RPE) has classically been described with or without choroidal neovascularization (CNV) or after laser treatment. Tangential shear forces within the RPE or CNV are usually considered to cause the dehiscence. methods: Three patients with CNV and spontaneous RPE tear and additional vitreomacular traction were examined by fluorescein angiography (FA), optical coherence tomography (OCT) and kinetic ultrasound. RESULTS: From the pre-tear to the tear stage a sudden decrease in vision was observed. Fluorescein angiographic images demonstrated RPE-tear formation with blocked filling in the area of the contracted RPE and a well-demarcated hyperfluorescence in the bed of the torn RPE. OCT-scans demonstrated vitreomacular traction at the foveal area in all three cases. Kinetic ultrasound revealed vitreous attachments at the optic disc and fovea. CONCLUSION: Magnitude, variation of mechanical forces, and the continuous shear stress of the aged vitreous gel transmitted across vitreoretinal attachments may cause a chronic stimulus to retina and RPE. Vitreomacular traction may contribute to the subsequent formation of RPE tears via mechanical or cell mediator pathways.
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ranking = 0.5
keywords = choroid
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7/119. 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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ranking = 3
keywords = choroid
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8/119. Ocular melanocytosis and melanoma.

    The hypothesis that ocular melanocytosis is a precancerous condition that may lead to a choroidal melanoma should be seriously questioned for the following reasons: The incidence of malignant degeneration in a hyperpigmented eye is unknown and overreported. If the hypothesis were correct a bilateral melanoma would occasionally occur in patients with bilateral melanosis. No such case has been reported. In patients with unilateral melanosis the blue, unaffected eye may also develop a melanoma. A 67-year-old white woman with one dark and one blue eye provided the first such instance, although previous cases may not have been reported if their histologic picture was not unusual. The incidence of melanoma in our patients with unilateral melanosis was rare compared with the many melanomas developing in normal pigmented eyes (4/418). I found no statistically significant difference in the incidence of choroidal melanomas originating in the hyperpigmented or in the blue eye in patients with unilateral ocular melanocytosis.
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ranking = 1
keywords = choroid
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9/119. Vascular occlusions in the eye from cardiac myxomas.

    Vascular occlusion in the eyes from cardiac myxomas was diagnosed in two cases, belatedly in one and not until after death in the other. Suspicion of a myxomatous origin should be aroused by the combination of unexplained retinal (or choroidal) vascular disease occurring with multifocal neurological symptoms and with systemic symptoms suggesting atypical subacute bacterial endocarditis. The first patient had unilateral retinal artery occlusion by embolic material believed to have been myxomatous. Removal of the cardiac myxoma resulted in disappearance of this material (although the eye remained blind). The second patient who had had evidence of retinal artery occlusion in the clinical course of her multisystemic disease was found at autopsy to have extensive myxomatous involvement of the posterior ciliary arteries and of the choroidal arteries of both eyes and of the retinal artery in one eye.
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ranking = 1
keywords = choroid
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10/119. Hemosideric heterochromia iridum in malignant melanoma of the choroid.

    A case is reported in which hyperchromic heterochromia iridum developed due to blood staining of an eye with malignant melanoma of the choroid in which massive hemorrhage developed. It is suggested that a possibility of the malignant melanoma of the choroid be kept in mind where hemosiderin deposits are suspected to be the cause of heterochromia but no intraocular iron foreign body is present.
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ranking = 3
keywords = choroid
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