eMedicine Specialties > Ophthalmology > Optic Nerve

Papilledema

Author: Mitchell V Gossman, MD, Partner and Vice President, Eye Surgeons and Physicians, St Cloud
Coauthor(s): Joseph Giovannini, MD, Chief of Ophthalmology, Eye Surgery Center, David Grant Medical Center, Travis Air Force Base
Contributor Information and Disclosures

Updated: Dec 17, 2008

Introduction

Background

Papilledema is an optic disc swelling that is secondary to elevated intracranial pressure. In contrast to other causes of optic disc swelling, vision usually is well preserved with acute papilledema. Papilledema almost always presents as a bilateral phenomenon and may develop over hours to weeks.

The term, as a matter of definition, is incorrect to be used to describe optic disc swelling with underlying optic nerve infectious, infiltrative, or inflammatory etiologies; but, it is correctly used if the underlying cause of elevated intracranial pressure is infectious, infiltrative, or inflammatory.

Pathophysiology

The disc swelling in papilledema is the result of axoplasmic flow stasis with intra-axonal edema in the area of the optic disc. The subarachnoid space of the brain is continuous with the optic nerve sheath. Hence, as the cerebrospinal fluid (CSF) pressure increases, the pressure is transmitted to the optic nerve, and the optic nerve sheath acts as a tourniquet to impede axoplasmic transport. This leads to a buildup of material at the level of the lamina cribrosa, resulting in the characteristic swelling of the nerve head. Papilledema may be absent in cases of prior optic atrophy. In these cases, the absence of papilledema is most likely secondary to a decrease in the number of physiologically active nerve fibers.

Frequency

United States

Rare

International

Rare

Mortality/Morbidity

Early detection and identification of cause may be life saving.

Race

No racial predilection exists.

Sex

Papilledema affects both sexes equally.

Age

Papilledema can present at any age, though, during infancy, before the fontanelles close, the finding of papilledema may fail to occur despite elevated intracranial pressure.

Clinical

History

Most symptoms in a patient with papilledema are secondary to the underlying elevation in intracranial pressure.

• Headache: Increased intracranial pressure headaches are characteristically worse on awakening, and they are exacerbated by coughing or other type of Valsalva maneuver.
• Nausea and vomiting: If the rise in intracranial pressure is severe, nausea and vomiting may occur. This eventually may be followed by a loss of consciousness, pupillary dilation, and death.
• Pulsatile tinnitus
• Visual symptoms often are absent, but the following symptoms can occur:
  • Some patients experience transient visual obscurations (graying-out of their vision, usually both eyes, especially when rising from a lying or sitting position, or transient flickering as if rapidly toggling a light switch).
  • Blurring of vision, constriction of the visual field, and decreased color perception may occur.
  • Diplopia may be seen occasionally if a sixth nerve palsy is associated.
  • Visual acuity may be well-preserved, except in very advanced disease.
• Papilledema is sometimes found at routine examination in an asymptomatic individual.
• Inquire about potential causative medications.

Physical

• The history should be taken, and a physical examination, including vital signs, should be performed. In particular, check the blood pressure to exclude malignant hypertension.
• The patient should be evaluated for neurologic problems and febrile illness.
• Visual acuity, color vision, and pupillary examination findings should be normal. A relative afferent pupillary defect is usually absent. Since an abduction deficit secondary to a false-localizing sixth nerve palsy sometimes may be seen in association with increased intracranial pressure, check cover test in cardinal fields of gaze and check for full motility.
• Careful dilated fundus examination should be performed to look for the following signs:
  • Early manifestations
    • Disc hyperemia
    • Subtle edema of the nerve fiber layer can be identified with careful slit lamp biomicroscopy and direct ophthalmoscopy. This most often begins in the area of the nasal disc. A key finding occurs as the nerve fiber layer edema begins to obscure the fine peripapillary vessels.
    • Small hemorrhages of the nerve fiber layer are detected most easily with the red-free (green) light.
    • Spontaneous venous pulsations that are normally present in 80% of individuals may be obliterated when the intracranial pressure rises above 200 mm water. Therefore, though the presence of spontaneous venous pulsations is very useful to exclude papilledema (except in cases of highly variable intracranial pressure), its absence is not very helpful.
  • Late manifestations
    • As the papilledema continues to worsen, the nerve fiber layer swelling eventually obscures the normal disc margins and the disc becomes grossly elevated.
    • Venous congestion develops, and peripapillary hemorrhages become more obvious, along with exudates and cotton-wool spots.
    • The peripapillary sensory retina may develop concentric or, occasionally, radial folds known as Paton lines. Choroidal folds also may be seen.
  • Chronic manifestations
    • If the papilledema persists for months, the disc hyperemia slowly subsides, giving way to a gray or pale disc that loses its central cup.
    • With time, the disc may develop small glistening crystalline deposits (disc pseudodrusen).

Causes

• Any tumors or space-occupying lesions of the CNS
• Idiopathic intracranial hypertension (also known as pseudotumor cerebri)
• Decreased CSF resorption (eg, venous sinus thrombosis, inflammatory processes, meningitis, subarachnoid hemorrhage)
• Increased CSF production (tumors)
• Obstruction of the ventricular system
• Cerebral edema/encephalitis
• Craniosynostosis
• Medications, for example, tetracycline, minocycline, lithium, Accutane, nalidixic acid, and corticosteroids (both use and withdrawal)

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