By R. J. Balkan
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Additional info for At the Crossing: Pediatric Ophthalmology And Strabismus (Noao Meeting Proceedings) (Noao Meeting Proceedings)
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. 1-3 Despite these important studies, many clinicians remained pessimistic and unconvinced that animal studies translated to children with congenital cataracts. 5 published their now-classic article documenting that good visual results can be obtained if a clear image is established in early infancy. The cause of previous failures was now realized to be dense, irreversible amblyopia caused by the malapropos stimulus of a blurred image during the critical period of visual development; namely, during the first few months of life.
It is important to remember that galactosemia is a treatable systemic disease that, when untreated, can result in brain damage and death. Further, if treated early, galactosemia cataracts may be reversible. Screening tests would include urinalysis with non-glucose-specific tests for reducing substances and screening for albumin. A 24-hour collection of urine can be screened for detection of certain genetic diseases. More specific tests would include galactose-1-phosphate uridyl transferase and galactokinase assays if galactose-related cataracts are suspected.
Ellis Fig. 12. Nuclear cataract. Fig. 13. Gyrate atrophy (hyperornithinemia), cataract. pmd 40 12/5/2003, 12:43 PM Etiology of pediatric cataracts 41 Fig. 14. Gyrate atrophy (hyperornithinemia), peripheral retina. Fig. 15. Neurofibromatosis Type II, cataract. D. Ellis competence to perform the examination, the patient’s pediatrician is informed as to the diagnosis of cataract and is queried about his own physical examination. If these examinations are entirely normal and the patient appears to be healthy except for the cataracts, little or no further investigation is required in the majority of cases.