At the Crossing: Pediatric Ophthalmology And Strabismus by R. J. Balkan

By R. J. Balkan

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Pmd 44 12/5/2003, 12:43 PM Monocular congenital cataracts 45 Monocular congenital cataracts Is binocular vision too much to ask? Kenneth W. Wright and Mehmet Cem Mocan Pediatric Ophthalmology and Strabismus, Los Angeles, CA, USA Introduction Historically, unilateral congenital cataracts have had a poor visual prognosis. As an ophthalmology resident 20 years ago, I remember many experts stating that visual rehabilitation in these children was impossible, some even saying that treatment was a waste of time.

Daily atropine should be avoided in these patients, as prolonged cycloplegia can induce amblyopia. Pupillary dilatation is usually reserved for infants with partial cataracts, waiting until they are one or two years of age before IOL can be used. These patients should be monitored carefully and, if significant amblyopia persists, cataract extraction should be performed. Surgical treatment of a visually significant congenital cataract Once a visually significant cataract is diagnosed in a neonate, time is of the essence and immediate action is necessary.

After a lens has become completely opacified, the original morphology cannot be determined unless prior photographs or written descriptions of the original appearance exist. D. Ellis Table 2. Examples of classic cataract syndromes and inheritance patterns Syndrome Inheritance pattern Comments Conradi Marfan I (AR) & II (AD) (AD) Mandibulofacial Hallerman-Streiff Pierre Robin Treacher Collins Trisomy syndromes Patau (trisomy 13) Edwards (trisomy 18) Down (trisomy 21) Nance-Horan (AD) stiff joints, stippled epiphyses subluxated lenses, hyperextensibility of joints, dilated aortic root typical facies (S) typical facies (XLR) Aniridia (AD or S) dental anomalies, microcornea, short metacarpals, developmental delay, maternal posterior Y-suture opacities iris, foveal, optic nerve hypoplasia Metabolic syndromes: Galactokinase deficiency (AR) Galactosemia (AR or XLR) Oculo-cerebro-renal (Lowe) (XLR) Hyperferritinemia (AD) G-6-PD deficiency (XLR) uniform lens opacification oil droplet cataract reversible early glaucoma, aminoaciduria, hyphema associated with cataract removal7 hereditary hyperferritinemia cataract syndrome (HHCS), crystals in lens cortex, elevated ferritine with normal serum iron levels8 glucose-6-phosphate dehydrogenase deficiency.

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