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Key Findings

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  • Papilledema (optic nerve edema) is associated with increased intracranial pressure due to any cause, such as tumor, craniosynostosis, or intracranial infection

  • Occurs almost equally in boys and girls and sometimes is associated with obesity or upper respiratory tract infection

  • Other associated causes are viral and other infections, corticosteroid use or withdrawal, sinus infection, trauma, tetracycline use, growth hormone supplementation, and venous sinus thrombosis

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Clinical Findings

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  • Hydrocephalus and intracranial tumors are common causes of papilledema

  • In idiopathic intracranial hypertension (pseudotumor cerebri), neuroimaging is normal but papilledema, headaches, and pulsating tinnitus may be present

  • Early in the illness the patient may not notice a change in vision, although the blind spot may be enlarged

  • Transient obscuration of vision (amaurosis fugax) may occur as the process becomes more long-standing

  • Further effects on vision occur as the papilledema becomes chronic and ultimately leads to optic atrophy

  • Diplopia (double vision) may occur if increased intracranial pressure results in cranial nerve VI palsy

  • Workup and treatment are directed toward finding the underlying systemic or central nervous system cause

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Diagnosis

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  • Direct visualization of the optic nerve by ophthalmoscopy reveals an elevated disc with indistinct margins, increased vessel diameter, vessel tortuosity, and hyperemia

  • Hemorrhages and exudates are present in more severe cases

  • Observed changes may be subtle to striking

  • Optic nerve head changes are usually bilateral and generally symmetric

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Treatment

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  • Idiopathic intracranial hypertension may be treated using acetazolamide or a corticosteroid

  • Discontinue medications that are suspected of causing papilledema

  • Administer anticoagulation if venous sinus thrombosis is present

  • Diagnostic lumbar puncture may also be curative

  • Optic nerve sheath fenestration and ventriculoperitoneal shunt are surgical interventions used when conservative measures fail

  • Strabismus surgery, Botox (botulinum toxin type A) injection of extraocular muscles, and amblyopia treatment may be necessary in cases of associated cranial neuropathies resulting in strabismus

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