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General Information

Definition

Optic disc swelling produced by increased intracranial pressure.

Symptoms

Episodes of transient, usually bilateral visual loss (lasting seconds), often precipitated after rising from a lying or sitting position (altering intracranial pressure); headache; double vision; nausea; vomiting; and, rarely, a decrease in visual acuity (a mild decrease in visual acuity can occur in the acute setting if associated with a macular disturbance). Visual field defects and severe loss of central visual acuity occur more often with chronic papilledema.

Signs

(See Figure 10.15.1.)

Critical

Bilaterally swollen, hyperemic discs (in early papilledema, disc swelling may be asymmetric) with nerve fiber layer edema causing blurring of the disc margin, often obscuring the blood vessels.

Other

Papillary or peripapillary retinal hemorrhages (often flame shaped); loss of venous pulsations (20% of the normal population do not have venous pulsations); dilated, tortuous retinal veins; normal pupillary response and color vision; an enlarged physiologic blind spot or other visual field defects by formal visual field testing.

In chronic papilledema, the hemorrhages and cotton–wool spots resolve, disc hyperemia disappears, and the disc becomes gray in color. Peripapillary gliosis and narrowing of the peripapillary retinal vessels occur, and optociliary shunt vessels may develop on the disc. Loss of color vision, loss of central visual acuity, and visual field defects (especially inferonasally) may be observed.

NOTE:

Unilateral or bilateral sixth cranial nerve palsy may result from increased intracranial pressure.

10-15.1 Swollen optic disc with obscured blood vessels and blurring of the disc margin.

Gervasio-ch010-image018

Flame-shaped hemorrhages may occur.

Differential Diagnosis

Differential Diagnosis of Disc Edema or Elevation
NOTE:

Optic disc swelling in a patient with leukemia is often a sign of leukemic optic nerve infiltration. Urgent initiation of therapy including corticosteroids and chemotherapy/radiation is required to achieve best visual outcomes.

Etiology

  • Primary and metastatic intracranial tumors.
  • Hydrocephalus.
  • Idiopathic intracranial hypertension: Often occurs in young, overweight females. See 10.16, IDIOPATHIC INTRACRANIAL HYPERTENSION/PSEUDOTUMOR CEREBRI.
  • Subdural and epidural hematomas.
  • Subarachnoid hemorrhage: Severe headache, may have preretinal hemorrhages (Terson syndrome).
  • Arteriovenous malformation.
  • Brain abscess: Often produces high fever and mental status changes.
  • Meningitis: Fever, stiff neck, headache (e.g., syphilis, tuberculosis, Lyme disease, bacterial, inflammatory, neoplastic).
  • Encephalitis: Often produces mental status abnormalities.
  • Cerebral venous sinus thrombosis.

Work Up

Workup
  1. History and physical examination, including blood pressure measurement.
  2. Ocular examination, including a pupillary examination and assessment for dyschromatopsia, posterior vitreous evaluation for white blood cells, and a dilated fundus examination. The optic disc is best examined with a slit lamp and a 60-diopter (or equivalent), Hruby, or fundus contact lens.
  3. Emergency MRI with gadolinium and magnetic resonance venography (MRV) of the head are preferred. CT scan (axial, coronal, and parasagittal views) may be done if MRI not available emergently.
  4. If MRI/MRV or CT is unrevealing, perform LP with CSF analysis and opening pressure measurement if no contraindication.

Treatment

Treatment should be directed at the underlying cause of the increased intracranial pressure.