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Papilledema is swelling of the optic disk due to increased intracranial pressure. All other causes of optic disk swelling, such as that caused by malignant hypertension or thrombosis of the central retinal vein, do not involve increased intracranial pressure and therefore are not causes of papilledema. Papilledema requires an immediate search for the cause. There are no early symptoms, although vision may be disturbed for a few seconds. Diagnosis is by ophthalmoscopy with further tests, usually brain imaging, to determine cause. Treatment is directed at the underlying condition.

Papilledema is a sign of elevated intracranial pressure and is almost always bilateral. Causes include the following:

• Brain tumor or abscess
• Cerebral trauma or hemorrhage
• Meningitis
• Arachnoidal adhesions
• Cavernous or dural sinus thrombosis
• Encephalitis
• Idiopathic intracranial hypertension (pseudotumor cerebri), a condition with elevated CSF pressure and no mass lesion

Symptoms and Signs

Vision is usually not affected initially, but seconds-long graying out of vision, flickering, or blurred or double vision may occur. Patients may have symptoms of increased intracranial pressure, such as headache or nausea and vomiting.

Ophthalmoscopic examination reveals engorged and tortuous retinal veins, a hyperemic and swollen optic disk (optic nerve head), and retinal hemorrhages around the disk but not into the retinal periphery. Isolated disk edema (eg, caused by optic neuritis, ischemic optic neuropathy) without elevated CSF pressure is not considered papilledema.

Papilledema

In the early stages, visual acuity and pupillary response to light are usually normal and become abnormal only after the condition is well advanced. An enlarged blind spot may be detected on visual field testing. Later, nerve fiber bundle defects may be apparent.

Diagnosis

• Clinical evaluation
• Immediate neuroimaging

The degree of disk swelling can be quantified by comparing the plus lens numbers needed to focus an ophthalmoscope on the most elevated portion of the disk and on the unaffected portion of the retina.

Differentiating papilledema from other causes of a swollen optic disk, such as optic neuritis, ischemic optic neuropathy, hypotony, central retinal vein occlusion, uveitis, or pseudo swollen disks (eg, optic nerve drusen), requires a thorough ophthalmologic evaluation. If papilledema is suspected clinically, MRI with gadolinium contrast or, alternatively, CTwith contrast is done immediately to exclude causes such as an intracranial mass. Lumbar puncture and measurement of CSF pressure should be done if a mass lesion has been ruled out. Lumbar puncture in the setting of intracranial mass lesions can result in brain stem herniation. B-scan ultrasonography is the best diagnostic tool for pseudo disk edema of optic nerve drusen.

Treatment

• Treatment of underlying disorder

Urgent treatment of the underlying disorder is indicated to decrease intracranial pressure. If intracranial pressure is not reduced, secondary optic nerve atrophy and vision loss eventually occur, along with other serious neurologic sequelae.

Last full review/revision July 2008 by James Garrity, MD

Content last modified July 2008