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Query: UMLS:C0086543 (cataract)
 29,165 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The treatment of the infant who is born with sensory deprivation amblyopia is one of the most difficult therapeutic problem sets facing the ophthalmologist today. This group of anomalies has many forms, the most common of which seems to be unilateral and bilateral cataracts. Substantial advances have been made, however, in many aspects of therapy for infants who have this set of problems. We have come to realize that it is essential to address these conditions at the first moment that is consistent with the safety of the child. New techniques in cataract extraction have reduced the time course of surgical treatment, reduced the frequency of secondary cataracts, and sustained the patency of the visual pathway. Unfortunately, practitioners who engage in the surgery may not have the back-up skills in advanced contact lens fitting, correction of aniseikonia, and, in some instances, the strabismological complications which are encountered. Further, improved means of assessment of visual acuity, binocular vision, and other visual characteristics of these infants are only in the developmental stages at this time. Thus, we propose to present the problem in an orderly manner. We shall first review the types of cases and problems. We will then discuss surgical techniques and the information which must be determined during examination under anesthesia (EUA) for the purposes of visual correction. We will then describe the designs of the spectacle lens and/or spectacle/contact lens combinations that are used to correct the resultant aniseikonia and anisometropia. We will address the approaches to patching and techniques on how to best achieve binocular vision in these patients. Separately, means and methods of assessment of vision by preferential looking, visual evoked response, optokinetic nystagmus, and tests of other visual functions will be explored. In summary, we will try to tie together the entire therapeutic regimen that can be used in the treatment of these patients.
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PMID:Helping the aphakic neonate to see. 408 71

In the evaluation of the genetic heterogeneity of congenital cataract and in establishing the nosologic diagnosis the cooperation of the ophthalmologist and geneticist is necessary. The importance of ophthalmologic syndromology for genetic counseling is shown in cases of Fraser's syndrome (McKusick No. 21,900), Usher's syndrome (N. 27,690), a syndrome involving acromegaly, cutis verticis gyrata and corneal leukoma (No. 10,210), a syndrome with congenital cataract, microphthalmia and nystagmus (No. 21,255), and a presumably new dominant hereditary cataract-vitiligo syndrome.
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PMID:Genetic counseling in congenital eye disorders. 641 63

In a study of congenital cataract in northern India, 76 patients (146 eyes) were evaluated for morphological patterns and etiology. Partial cataracts were found to be three times more common than total cataracts, the lamellar types being the most common among the partial cataracts. Hereditary cataracts had a 19.7% incidence. The majority (46.05%) of cases of congenital cataract were of idiopathic origin. A few cases were associated with metabolic disorders (Marfan's and Marchand's). Strabismus and nystagmus were the most common associated ocular abnormalities (28.94% and 15.79%), and mental retardation was the most commonly associated systemic abnormality. Rubella cataract was seen far less frequently than expected.
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PMID:Congenital cataract: etiology and morphology. 641 11

The records of 469 institutionalised mentally retarded subjects were examined. Keratoconus and blindness were present in 8 per cent and 4 per cent of the ninety-one subjects with Down syndrome. Only one case of keratoconus was found among the 378 subjects with mental deficiency of other aetiology. The incidence of strabismus, nystagmus and cataract was also increased in subjects with Down syndrome, especially in females. Cases of acute keratoconus associated with Down syndrome reported in the world literature are summarised. A total of about forty cases has ben reported between the ages of nine and fifty-three years. Sight has been preserved equally well after both conservative management and keratoplasty in eyes with and without cataracts.
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PMID:Keratoconus and blindness in 469 institutionalised subjects with Down syndrome and other causes of mental retardation. 646 Jan 9

We report about 8 patients all showing a nystagmus rotatorius or giratoire and differences of the horizontal component. In syndromes of the nervous system monocular nystagmus was combined with a scew-deviation or tremor of the lids. Similar to the syndromes of the fasciculus longitudinalis posterior defects in smooth or quick movements on both sides were different. After operation of a congenital cataract a vertical nystagmus was transformed in a nystagmus latens. In congenital squint syndromes monocular vertical nystagmus may be seen if one eye or both are closed. Separate pathways from the vestibular system only lead to the vertical-moving muscles of one eye.
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PMID:[Right-left differences in nystagmus direction. Observations in congenital and acquired ophthalmoneurologic syndromes]. 651 78

The vision of cats which were monocularly deprived during early infancy, of kittens, and of young human infants shares two limitations: detection in the nasal visual field is far poorer than detection in the temporal visual field, and optokinetic nystagmus (OKN) is difficult to elicit when a pattern moves nasally to temporally. Here we report similar limitations on the vision of children who had a dense central cataract in one eye during early infancy. Extensive static perimetry with one of these children whose visual acuity was good in both eyes revealed that her threshold for detection all along the horizontal meridian was higher in her aphakic than in her normal eye, with this difference much more pronounced in the nasal visual field than in the temporal visual field. Three children who developed cataracts after 6 months of age showed no such discrepancy between thresholds in the temporal and nasal fields. We tested the symmetry of OKN in 12 children treated for unilateral congenital cataract. In every test of an aphakic (n = 4) or normal eye (n = 12), OKN occurred significantly more often when stripes moved temporally to nasally than when they moved nasally to temporally. In contrast, no asymmetry was observed in any of 13 children treated for traumatic cataracts incurred after 3 years of age. We conclude that children treated for unilateral congenital cataract, like young human infants and monocularly deprived cats, show asymmetric OKN and relatively poor detection in the nasal visual field.
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PMID:Peripheral vision and optokinetic nystagmus in children with unilateral congenital cataract. 663 23

The visual outcome in cases of congenital cataract, managed both surgically and conservatively, is outlined. Final vision was better in cases with partial cataract, about 40% of whom attained 6/24 or better vision. With total cataracts, none could achieve this level of vision, with the majority (84%) attaining only 6/60 or less acuity. Visual results were also better in patients without nystagmus, 34% of whom attained 6/24 or better vision, while in cases with nystagmus, only 15% could come up to this level. Cataracts necessitating early surgery had a worse prognosis than cataracts necessitating late surgery. Visual results were best in cases with bilateral cataracts, partial cataracts, absence of associated ocular anomalies, the absence of nystagmus and in patients requiring later surgery. The important causes of nonimprovement of vision were amblyopia and after-cataract. Retinal detachment, retinopathy, and degenerative myopia were some of the less frequent causes of poor vision, while mental retardation was another important factor. Full cooperation from the parents is absolutely essential in the proper visual rehabilitation of the handicapped child.
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PMID:Congenital cataract: management and results. 664 86

An autosomal dominant syndrome of mild foveal hypoplasia (visual acuity, 20/50), congenital nystagmus, corneal pannus, and presenile cataracts was seen in four patients from a single family. There is variable expressivitly of this trait. The differential diagnosis includes aniridia, ocular albinism, and isolated foveal hypoplasia. Cataract surgery is indicated for affected patients with substantial visual impairment.
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PMID:Autosomal dominant foveal hypoplasia and presenile cataracts. A new syndrome. 706 45

Of 40 patients with ketotic hypoglycemia, 15 (nine boys and six girls) developed cataracts. The mean age at onset of the first hypoglycemic attack was 20 months, and the average age at the time the cataracts were discovered was 3 1/4 years. The average birth weight of 14 children was 2060 g. The cataracts were bilateral in all but one case. Seven patients (11 eyes, bilateral in four patients) developed complete cataracts. Despite aphakic correction and occlusion therapy, the major cause of visual loss after cataract surgery was stimulus deprivation amblyopia. Other ocular abnormalities included strabismus and jerky horizontal nystagmus. Neurologic impairment--epilepsy, psychomotor retardation, and/or electroencephalographic abnormalities--was present in over one half of the patients. All children with ketotic hypoglycemia should be referred promptly for an ophthalmic examination so that appropriate therapy can be implemented early.
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PMID:Cataracts and ketotic hypoglycemia. 717 78

The eye symptoms in dysostosis craniofacialis Crouzon can be divided into obligate and facultative signs; the latter are nystagmus, megalocornea, coloboma of the iris, corectopia, ectopia of the lens and cataract. There is no causal relationship between these symptoms and the dysostosis craniofacialis. The common eye findings in this disease use proptosis, divergent strabism, hypertelorism with dystopia canthi lateralis as well as in about 80% an atrophy of the optic nerve. It is considered until now that the premature synostosis of cranial sutures is the leading and only pathogenetical factor in this disease. This theory can no longer be sustained because of its inability to explain the above mentionned eye findings. We want to stress on a malformation of the prosencephalic head organisator leading to a dyschondroplasia that mainly affects the base of the skull. This frontal dysplasia causes a characteristic displacement of the building materials and thereby produces the common eye findings in this disease.
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PMID:[The development of eye symptoms in dysostosis craniofacialis Crouzon--a contribution to pathogenesis (author's transl)]. 719 65


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