Cases reported "Cataract"

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11/515. Wilson's disease (hepatolenticular degeneration).

    Wilson's disease, or hepatolenticular degeneration, is a rare inherited disorder of copper metabolism which usually affects young people. Excess copper accumulates in the tissues, primarily in the liver, brain, and cornea. This copper deposition results in a wide range of hepatic and neurological symptoms, and may produce psychiatric illness. Hepatic involvement often occurs in childhood, while neurological deficits generally are detected at a later age. The disease is inherited in an autosomal recessive fashion. Ocular findings are of particular importance because the corneal copper deposition, forming the Kayser-Fleischer ring,is the only pathognomonic sign of the disease. The structure of the ring and the presence of copper have been well established. An anterior capsular deposition of copper in the lens results in a characteristic sunflower cataract in some of these patients. Other ocular abnormalities have been described but are much less common. The pathogenesis of the disease and the basic genetic defect remain obscure. It is clear that there is excess copper in the tissues, but the mechanism of its deposition is unknown. It is in some way associated with a failure to synthesize the serum copper protein ceruloplasmin normally. Another theory suggests that an abnormal protein with a high affinity for copper may bind the metal in the tissues. The diagnosis may be suggested by the clinical manifestations and confirmed by the presence of a Kayser-Fleischer ring. In the absence of these findings biochemical determinations are necessary. The most important of these are the serum ceruloplasmin, the urinary copper, and the hepatic copper concentration on biopsy. Treatment consists in the administration of the copper chelating agent, penicillamine, and the avoidance of a high copper intake. This usually results in marked clinical improvement if irreversible tissue damage has not occurred. maintenance therapy for life is necessary in order to continue the negative copper balance. The detection and prophylactic treatment of asymptomatic individuals with the disease is especially important. Seven cases of Wilson's disease have been presented in order to illustrate many of the features which have been discussed, with emphasis on the ocular findings.
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ranking = 1
keywords = anterior capsular, capsular, ocular
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12/515. Brachycephalosyndactyly with ptosis, cataract, colobomas, and linear areas of skin depigmentation.

    A male patient with brachycephalosyndactyly syndrome associated with ocular and skin anomalies is reported and it is suggested that this patient has a previously undescribed disorder.
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ranking = 0.0035365625304014
keywords = ocular
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13/515. cataract extraction and intraocular lens implantation in anterior megalophthalmos.

    This report describes the implantation of a standard posterior chamber intraocular lens (IOL) in a patient with bilateral cataract and anterior megalophthalmos. After extracapsular cataract extraction, the IOL was sutured to the posterior surface of the iris and anterior capsule. Different types of IOLs were used in each eye, and the surgical technique was adapted to the characteristics of the IOL. No complications were noted. Visual rehabilitation was successful. Extracapsular cataract extraction with a posterior chamber IOL sutured to the posterior surface of the iris and anterior capsule is a useful option in patients with anterior megalophthalmos and cataract.
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ranking = 0.10991414310597
keywords = capsular, ocular
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14/515. Fetal cataract in congenital toxoplasmosis.

    We report a case of the prenatal diagnosis of fetal cataract due to congenital toxoplasmosis. To the best of our knowledge, this is the first report of such a case. We discuss the long-term ocular sequelae of the condition and how they should affect prenatal counselling.
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ranking = 0.0035365625304014
keywords = ocular
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15/515. Contact zone of piggyback acrylic intraocular lenses.

    In a hyperopic cataract patient, surgery was performed with implantation of 2 foldable, acrylic, posterior chamber intraocular lenses (IOLs) in the bag. The IOLs showed a central contact zone during surgery. This contact zone remained after surgery and was documented 2 months postoperatively. The contact zone may induce multifocality similar to that seen with multifocal IOLs.
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ranking = 0.017682812652007
keywords = ocular
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16/515. Complete capsulorhexis opening occlusion despite capsular tension ring implantation.

    An 89-year-old woman and an 86-year-old woman had continuous curvilinear capsulorhexis, phacoemulsification, and implantation of a silicone plate-haptic intraocular lens. Because of presumed weak zonules (high age, pseudoexfoliation), a poly(methyl methacrylate) capsular tension ring was also implanted. Despite this, both patients reported deterioration in visual acuity that was the result of complete occlusion of the anterior capsule opening by fibrotic tissue 4 and 3 months postoperatively, respectively.
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ranking = 0.2341148886653
keywords = capsular, ocular
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17/515. Ocular injury caused by an air bag for a driver wearing eyeglasses.

    BACKGROUND: Although air bags have been shown to reduce the number of fatalities and serious injuries caused by motor vehicle accidents, there have been many reports of air bag-related ocular injuries. We recently treated air bag-related corneal laceration in a patient wearing eyeglasses at the time of a motor accident. CASE: A 38-year-old Japanese man was driving a car at approximately 40 km per hour when he struck a stopped 2-ton truck. He was wearing a three-point lap-shoulder seat belt. At impact, the driver's-side air bag deployed and struck the man on the left side of his face. He was wearing eyeglasses with glass lenses, and the air bag broke the left lens of his eyeglasses, and glass fragments lacerated his cornea. OBSERVATIONS: External examination showed multiple superficial abrasions of the skin and ecchymosis of the left side of his face. Slit-lamp examination of his left eye showed corneal laceration and hyphema. The lens had opacities and was covered with fibrin membrane. Repair of the corneal laceration and phacoemulsification of the lens were performed. Six months later, his best corrected visual acuity was 20/20 in the left eye. CONCLUSIONS: As cars are increasingly equipped with air bags, reports of air bag-related eye injuries have increased. To our knowledge, this is the first reported case of corneal laceration caused by a shattered lens in an air bag-related injury. Ophthalmologists should caution patients about the danger of eye injuries in air bag-equipped cars, and thought should be given to improving the materials for eyeglasses.
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ranking = 0.0035365625304014
keywords = ocular
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18/515. Ophthalmic manifestation of congenital protein c deficiency.

    Under normal conditions activated protein C is a natural anticoagulant that cleaves 2 activated coagulation factors, factor va and factor viiia, thereby inhibiting the conversion of factor X to factor xa and of prothrombin to thrombin. Additionally, activated protein C enhances tissue-plasminogen activator-mediated fibrinolysis by inhibition of plasminogen activator inhibitor-1. This results in an increase in circulatory plasminogen activator levels. protein c deficiency, a genetic or acquired thrombophilic abnormality, has been demonstrated to predispose to episodes of potentially blinding and lethal thromboembolic events. Heterozygous-deficient subjects usually remain asymptomatic until adolescence or adulthood. In homozygous-deficient patients, protein C activity is usually less than 1% (reference range, 70%-140%), resulting in thromboembolism as early as in the neonatal period. The major clinical symptoms in affected newborn infants have been purpura fulminans, vitreous hemorrhage, and central nervous system thrombosis. The age of onset of the first symptoms has ranged from a few hours to 2 weeks after birth, usually after an uncomplicated full-term pregnancy and delivery. In contrast to the genetic form, acquired neonatal protein c deficiency occurs particularly in ill preterm babies. Typical complications of prematurity such as respiratory distress syndrome, necrotizing enterocolitis, and neonatal sepsis may also be present. In the medical literature, there are only a few reports of homozygous protein c deficiency in neonates. We present 2 cases of homozygous protein c deficiency with ocular and extraocular manifestation.
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ranking = 0.0070731250608027
keywords = ocular
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19/515. Electric cataract: a case report and review of the literature.

    A case of electrically induced cataract in both eyes in a 12-year-old boy, after a high-voltage electric shock, is reported. He sustained skin burns on the neck, chest, abdomen, and inner left arm. The cataract developed first in the left eye and later on in the right eye. The child regained normal vision in both eyes after cataract extraction and aphakic correction with spectacles. The need for awareness of the possibility of this complication and screening of all cases of electrical injuries is stressed. The majority of cases respond well to surgery, but final visual acuity will depend on the other ocular damage due to electrical current. The clinical features and pathogenesis of this condition are briefly reviewed.
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ranking = 0.0035365625304014
keywords = ocular
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20/515. Severe, non X-linked congenital microcephaly with absence of the pyramidal tracts in two siblings.

    In two siblings (a female and a male neonate), severe microcephaly, bilateral absence of the pyramids, severe hypoplasia of the cerebral peduncles, and dysplasia of the inferior olives was found together with microphthalmia, facial malformations and multiple contractures of the extremities. In both cases, the cerebral hemispheres otherwise showed a more or less normal gyral pattern with the insula incompletely covered by the opercula, and a tom but otherwise intact corpus callosum. In case 2, congenital cataract was also observed. The present cases can be characterized as a rapidly fatal, familial syndrome, probably transmitted as an autosomal recessive trait, and have several features in common with the Neu-Laxova syndrome. They differ in having a less severe form of microcephaly, a rather normal cytoarchitecture of the cerebral cortex, an apparently normal corpus callosum, no gross cerebellar abnormalities, and no other organ malformations. The present cases belong to a group of heterogeneous syndromes which have microcephaly, ocular and facial malformations, multiple contractures, and ichthyosis-like skin in common.
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ranking = 0.0035365625304014
keywords = ocular
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