Cases reported "Eye Diseases"

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1/53. Severe ocular and orbital toxicity after intracarotid etoposide phosphate and carboplatin therapy.

    PURPOSE: To report severe ocular and orbital toxicity after administration of intracarotid etoposide phosphate and carboplatin. METHOD: Case report. RESULTS: A 52-year-old man with glioblastoma multiforme underwent left intracarotid administration of eto poside phosphate and carboplatin inferior to the ophthalmic artery. Within 7 hours, a nonpupillary block angle-closure glaucoma developed secondary to uveal effusion in the ipsilateral eye, which was relieved by cycloplegia. Four days later, severe orbital inflammation resulted in a visual acuity of counting fingers, proptosis, optic neuropathy, and total external ophthalmoplegia in the eye. The patient's condition improved after a lateral cantholysis and administration of high-dose intravenous corticosteroids. Two weeks later, an anterior uveitis occurred in the left eye, which responded to topical corticosteroids. During a 2-month period, the patient recovered to a visual acuity of 20/70, near normal motility, and normal intraocular pressure, and the ocular and orbital inflammation resolved. Preexisting ipsilateral chemotherapy-induced maculopathy became more pronounced. CONCLUSION: Ocular and orbital toxicity after intracarotid etoposide phosphate and carboplatin therapy is infrequently reported.
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2/53. pupil abnormality in amyloidosis with autonomic neuropathy.

    darkness pupil diameters, light reflexes, and redilatation times have been recorded with infrared TV pupillometry in 12 consecutive patients with systemic amyloidosis associated with sensory motor and autonomic neuropathy. Nine of the patients had AL amyloidosis, two had familial amyloidosis associated with a transthyretin abnormality, and one was untyped. The pupils were abnormal in all 12 patients. On the basis of redilatation lag without pupillotonia, six patients had bilateral Horner's syndrome and in one of them amyloid deposits were found in a sympathetic ganglion and in the attached sympathetic chain obtained at necropsy. Four patients had bilateral tonic pupils with light-near dissociation and two had abnormally small pupils with reduced light reactions which could not be characterised. It seems that in patients with systemic amyloidosis generalised autonomic neuropathy is strongly associated with pupil abnormality as shown by tonic reactions with light-near dissociation, by redilatation lag, or by reduced size in darkness.
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3/53. 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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4/53. Two remarkable events in the field of intraocular foreign body: (1) The reversal of siderosis bulbi. (2) The spontaneous extrusion of an intraocular copper foreign body.

    Two unusual events concerning intraocular foreign bodies are presented. The first patient had an occult or unsuspected intraocular foreign body. He showed iridoplegia with mydriasis, siderosis iridis, and an intraocular piece of iron lying posteriorly near the retina. The foreign body was removed and the patient regained normal iris color and pupillary activity. His vision remains 20/15 six years postoperatively dispite ensuing retinal detachment one year after removal of the foreign body. The second patient was a young boy injured by a blasting cap explosion. He lost one eye from the injury and had a piece of intraocular brass in his left eye. In spite of the development of chalcosis and a mature cataract the lens gradually shrank in the pupillary space permitting a clear aphakic area and 20/25 vision. The brass fragment migrated forward and inferiorly and was finally extruded under the conjunctiva five years later, where it was removed and chemically analyzed by x-ray diffraction.
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5/53. Perioperative management of infants with the linear naevus sebaceous syndrome of Jadassohn: a report of two cases.

    Neurofibromatosis and tuberous sclerosis are the most well-recognized of the congential phakomatoses, a group of six hereditary neuro-oculo-cutaneous disorders. Although easily diagnosed at birth by a parasagittal line of facial sebaceous naevi, the linear naevus sebaceous syndrome (LNSS) of Jadassohn is the rarest phakomatosis, one often characterized by airway and anaesthetic considerations that do not apply to the other phakomatoses. In addition to its obvious cutaneous manifestations, LNSS is characterized by hemifacial asymmetry, an anatomic predictor of difficult trachael intubation, and intractable seizure activity, a condition that limits selection of anaesthetics. The perioperative management challenges of LNSS are depicted in the presentation of two cases of LNSS with different outcomes and contrasted with the major anaesthetic considerations in the perioperative management of other, more common phakomatoses.
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6/53. Pars plana vitrectomy for persistent, visually significant vitreous opacities.

    PURPOSE: To evaluate the role of vitrectomy in patients with persistent, visually disabling vitreous opacities. methods: Six consecutive eyes of five men (age 58-66 years) with pseudophakia or aphakia and vitreous opacities resulting in visual symptoms for more than 1 year that underwent vitrectomy were retrospectively reviewed. Postoperative questionnaires regarding functional performance and quality-of-life issues were completed by the participants to assess subjective patient satisfaction. RESULTS: Postoperative Snellen visual acuity was improved or equal to preoperative acuity in all cases (8-44 month follow-up) and there were no surgical complications. All patients expressed high satisfaction with overall visual function. Analysis of the National eye Institute Visual Function Questionnaire-39 indicated that general vision, near activities, distance activities, mental health, role difficulties, and peripheral vision were significantly improved (P < 0.05) following surgical intervention. CONCLUSIONS: vitrectomy may be indicated in a select group of patients with visually disabling vitreous floaters, although objective assessment of visual dysfunction from vitreous floaters requires further evaluation.
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7/53. 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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8/53. Laminated posterior vitreous cortex associated with idiopathic macular hole.

    PURPOSE: To report an unusual preretinal opacity associated with an idiopathic macular hole (MH) that was identified by optical coherence tomography. DESIGN: Observational case report. methods: A 59-year-old woman with a preretinal linear opacity running across an MH was examined by optical coherence tomography. RESULTS: Optical coherence tomography showed a full-thickness MH with a superiorly hinged flap. The preretinal opacity was identified as a part of a laminated vitreous cortex that was perpendicularly attached to the apex of the flap. A horizontal scan showed a partly double-layered posterior vitreous cortex. Two months later, the linear opacity disappeared coincident with a complete posterior vitreous detachment. The MH was closed by vitrectomy with peeling of the internal limiting membrane. CONCLUSIONS: Optical coherence tomography identified an unusual ophthalmoscopic presentation of a preretinal opacity as a part of laminated vitreous cortex associated with a "can-opener" MH.
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9/53. T13M mutation of lecithin-cholesterol acyltransferase gene causes fish-eye disease.

    BACKGROUND: Lecithin-cholesterol acyltransferase (LCAT) esterifies free cholesterol (FC) in plasma and plays a crucial role in the maturation of prebeta1-HDL (lipid-poor HDL) into alpha-migrating HDL (spherical HDL). Natural mutations of LCAT gene cause familial LCAT deficiency (FLD) or fish-eye disease (FED). The relationship between mutations and their phenotypes gives important clues to the functions of specific regions of LCAT. We investigated the first homozygous case with a substitution of threonine to methionine at codon 13 (T13M) of LCAT gene. methods: We evaluated LCAT activity, LCAT distribution among HDL subfractions and conversion of prebeta1-HDL to alpha-migrating HDL by native two-dimensional gel electrophoresis (N-2DGE). RESULTS: The proband had corneal opacity, severe hypo-alpha-lipoproteinemia, half-normal LCAT activity and near normal cholesteryl ester/total cholesterol (TC) ratio in plasma. These features were characteristic of FED. plasma prebeta1-HDL concentration was near normal, but not converted to alpha-migrating HDL during 37 degrees C incubation. As expected, alpha-migrating HDL (especially large particles) was markedly reduced. In the immunoblot against LCAT, the small alpha-migrating HDL from the proband had much less LCAT in this patient than in controls. CONCLUSION: T13M mutation of LCAT gene causes FED.
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10/53. Occupational exposures to pesticides containing organoarsenicals in california.

    The only organic arsenicals used in agriculture are methanearsonic acid (MSMA) and its sodium and ammonium salts and dimethylarsinic acid (cacodylic acid) and its sodium salt. They have an oral LD50 in the rat of 700-1,000 mg/kg and are classified as toxicity category 3 pesticides. During the three-year period 1975, 1976 and 1977 in california there were 34 reports by physicians of injury due to exposure to pesticides containing organic arsenicals of which nine resulted in systemic symptoms and the remainder being eye and skin irritations. There appeared to be prompt recovery from these exposures. They were caused primarily by use of faulty equipment, not using due care in its operation, poor work practices and improper use of protective equipment. There is no evidence that this group of chemicals is carcinogenic in animals or man.
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