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1/214. vitrectomy in sickling retinopathy: report of five cases.

    The unique problems encountered in sickle patients include the need to remove peripheral vitreous if perfusing sea fans-which can bleed after vitrectomy-are present at the time of surgery. However, peripheral vitrectomy is riskier than central vitrectomy alone. If visualization of the sea fans is sufficient, it is safer to close the sea fans prior to vitrectomy in order to obviate the need for peripheral vitrectomy; then, only central vitrectomy is performed. When sea fans cannot be closed prior to vitrectomy, peripheral vitreous is removed to allow early photocoagulation of the sea fans before they bleed again. Four case presentations illustrate these principles. vitrectomy relieved severe vitreous traction that complicated retinal detachment in the fifth patient, thereby eliminating the need for a tight encircling structure, which can be poorly tolerated in patients with SC hemoglobin.
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ranking = 1
keywords = retinopathy
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2/214. Primary ocular Epstein-Barr virus-associated non-Hodgkin's lymphoma in a patient with AIDS: a clinicopathologic report.

    OBJECTIVE: To report an unusual case of chronic multifocal chorioretinitis with vitritis in a patient with acquired immunodeficiency syndrome (AIDS) that was resistant to antiviral and antitoxoplasmic medication and required a retinal biopsy for definitive diagnosis. methods: Vitreous biopsy, pars plana vitrectomy, and retinal biopsy were performed. The vitreous biopsy material was sent for bacterial, fungal, and viral culture, and the vitreous cassette was sent for cytology. The retinal biopsy material was divided and sent for polymerase chain reaction testing for toxoplasmosis and virology and pathologic tissue analysis. RESULTS: Vitreous cytology showed a mixed population of lymphocytes and histiocytes, but all other microbiologic and virologic studies were negative. Tissue analysis revealed an infiltrate of atypical mononuclear cells extending from the inner limiting membrane through the outer plexiform layer characteristic of a B cell, non-Hodgkin's lymphoma of the central nervous system (NHL-CNS). in situ hybridization for the Epstein-Barr virus (EBV) was positive. An extensive systemic evaluation did not show evidence of extraocular tumor. CONCLUSION: Although rare, primary ocular NHL-CNS can be seen in patients with AIDS, and its clinical presentation often closely resembles other disorders. To our knowledge, this case represents the first ocular NHL in which EBV is shown to be associated.
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ranking = 0.17128053476917
keywords = retinitis
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3/214. 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 = 0.25
keywords = retinopathy
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4/214. A Colombian family with X-linked juvenile retinoschisis with three affected females finding of a frameshift mutation.

    X-linked retinoschisis (XLRS) is a vitreoretinal disease responsible for most cases of juvenile macular degeneration in males. retinoschisis carrier females generally manifest no pathological symptoms. However, a large affected family from colombia presented three affected females with typical RS phenotype similar to their 27 affected male relatives. Fundus examination as well as electroretinograms (ERG) indicate that the disease in these three affected females is as severe as in their affected male counterparts. dna sequence analysis of the XLRS1 gene in the affected members of this family indicates a single base (G) deletion at the 639 base position (639delG). This deletion causes a frameshift during translation and results in a larger (235 amino acids) than normal peptide (224 amino acids) with grossly altered discoidin domain, which is considered critical for the cellular function of the protein. The co-segregation of this gene mutation with the RS phenotype and the RS carrier status as well as its complete absence in normal controls indicates that this genetic change is responsible for the RS pathology in this family. This (639delG) is a novel RS mutation and reported here for the first time. Furthermore, the analysis of the three affected females indicates that the RS pathology in affected females (a very rare occurrence) is due to XLRS1 mutations carried on both of their X chromosomes.
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ranking = 0.036204369628389
keywords = degeneration
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5/214. Three children with congenital toxoplasmosis: early report from a Swedish prospective screening study.

    The aim of this prospective study was to define the incidence of congenital toxoplasmosis in sweden. blood eluates collected on filter papers, Guthrie cards, from 40978 newborn babies were analysed for specific immunoglobulin m (IgM) and IgG antitoxoplasma antibodies. This is a preliminary report of three children with congenital toxoplasmosis, defined by the occurrence of antitoxoplasma-specific IgM antibodies. Two children were asymptomatic at birth. They were both normally developed at the age of 12 and 15 months, respectively. The third child had unidentified but uncomplicated symptoms of infection in the neonatal period. As a result of the screening congenital toxoplasmosis was confirmed and treatment instituted. Microphthalmus and peripheral chorioretinitis were detected in one eye. In spite of the chemotherapeutic treatment he developed hydrocephalus needing neurosurgical intervention at the age of 3 months. His development at 14 months was normal. The incidence in sweden of congenital toxoplasmosis detected by specific IgM antitoxoplasma antibodies in blood from filter papers is less than 1:10000.
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ranking = 0.17128053476917
keywords = retinitis
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6/214. Clinical features of Goldmann-Favre syndrome.

    A 21-year-old woman complained of progressive loss of visual acuity. She had also had night blindness since she was ten years old. At the eye examination, the vitreous was found to be degenerated in both eyes. The fundus findings were a large retinoschisis in the right macula, edema resembling retinoschisis in the left macula and annular degenerative changes in the midperiphery. ERG and dark adaption were abnormal. This vitreoretinal degeneration was diagnosed as Goldmann-Favre syndrome.
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ranking = 0.036204369628389
keywords = degeneration
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7/214. A case in the spectrum of the oculo-encephalo-hepato-renal syndrome.

    An 18-year-old male is presented with unprecedented central nervous system findings (cerebral dysplasia and sacral meningocele) possibly in the spectrum of the oculo-encephalo-hepato-renal syndrome. He had severe mental retardation, triplegia, epilepsy, retinitis pigmentosa, and chronic renal failure. magnetic resonance imaging demonstrated cerebral dysplasia (left dominant abnormal gyri, hypoplastic white matter, basal ganglia, and thalamus, and absence of the septum pellucidum) and the hypoplastic cerebellum and brainstem. A sacral meningocele was observed first at 16 years of age. His renal function gradually worsened after 11 years of age. His liver function was normal. The previously reported 72 cases with the oculo-encephalo-hepato-renal syndrome are reviewed.
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ranking = 0.62974824338993
keywords = retinitis pigmentosa, retinitis, pigmentosa
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8/214. Ocular toxicity of systemic medications: a case series.

    BACKGROUND: There are many visually threatening conditions that may result from long-term use of systemic medications. Many of these adverse side effects can be greatly reduced or prevented with close monitoring of patients. In view of current knowledge, updated clinical guidelines for appropriate monitoring of ocular toxicity from systemic medications need to be developed for the eye care practitioner. CASE review: There have been many reports of ocular toxicity from isoniazid, thioridazine, steroids, and amiodarone therapy. Clinical cases illustrating possible adverse ocular side effects are presented, which include INH-induced optic neuropathy, phenothiazine-induced retinopathy, steroid-induced glaucoma, and vortex epitheliopathy secondary to amiodarone. CONCLUSION: Optometrists should be aware of the potential for ocular side effects from systemic medications. eye care guidelines for monitoring ocular side effects from thioridazine, INH, steroids, and amiodarone use are suggested.
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ranking = 0.25
keywords = retinopathy
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9/214. 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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ranking = 0.25
keywords = retinopathy
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10/214. 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 = 0.17128053476917
keywords = retinitis
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