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Key Findings

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  • Causes

    • Hydrocephalus

    • Glioma of the optic nerve

    • Craniosynostosis

    • Certain neurologic diseases

    • Toxins, such as methyl alcohol

    • Certain inborn errors of metabolism

    • Long-standing papilledema or papillitis

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Clinical Findings

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  • Optic atrophy is found in children most frequently after neurologic compromise during the perinatal period; an example would be a premature infant who develops an intraventricular hemorrhage

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Diagnosis

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  • Direct examination of the optic nerve by ophthalmoscopy reveals an optic nerve head with a cream or white color and possibly cupping

  • Neuroimaging is necessary to delineate CNS abnormalities

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Treatment

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  • Treatment of the underlying condition is indicated

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