Cataracts and ketotic hypoglycemia.

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.