[Significant ptosis in the young child: timing of surgery].
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Biomedical subjects
Publications and source records attributed to D Goddé-Jolly.
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The authors emphasize the importance of the relations between strabismus and amblyopia. Strabismus is often the symptom which leads to the discovery of amblyopia. Amblyopia may cause strabismus, as in organic amblyopias induced by congenital lesions of the retina or visual pathways, and visual deprivation amblyopias due to corneal opacities and/or congenital cataracts. Unilateral clouding of normally transparent media (cornea, lens, vitreum) prevents normal stimulation of the retina which is necessary to the development of binocular vision. Anisometropic amblyopias also belong to this category. More often, amblyopia is the consequence of strabismus: it is then termed "functional strabismic amblyopia". Loss of the parallelism of the two visual axes disrupts the functional balance between the images received from the two eyes. The two images are different; one is suppressed. This suppression may eventually lead to amblyopia. Amblyopia was discovered in 593 of 1 757 studied cases of strabismus. Amblyopia was the cause of strabismus in 87 patients and was functional in the remaining 506. Among these latter, visual acuity was inferior or equal to 1/10 in 241 cases and between 2/10 and 7/10 in 265. 147 children in the first group and 136 in the second could be treated. Among these patients, who were treated before the age of seven, complete recovery was obtained in approximately 1/2 of cases and improvement in more than 1/4 of first group patients and 1/3 of second group patients. Results were all the better that treatment was initiated earlier and that patients were younger.(ABSTRACT TRUNCATED AT 250 WORDS)
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A case of Richner-Hanhart syndrom with tyrosinemia is being reported. The diagnosis was suggested from clinical manifestations of this syndrom : superficial opacities of the cornea in an infant preceeding hyperkeratosis of the palms and soles and mild mental retardation. It has been confirmed by the high levels of blood tyrosine. The introduction of a diet low in tyrosine and alanine has lead to a rapid improvement and finally a complete cure of the ophthalmological and dermatological symptoms. The normal metabolism of tyrosine is recalled as well as the specific metabolic aberration responsable for this syndrom (deficiency of cytosol tyrosine amino-transferase). This case is being compared with those which have been previously reported.
The different kinds of penalizations which are used in strabismus therapy--occlusion, atropine penalization, optic penalization, filters, partial occlusion of the glasses (sectors)--are being discussed with their advantages and disadvantages. Their indications and their results are reported according to the age of the child and the degree of the amblyopia.
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