AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

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 should not be used to describe optic disc swelling with underlying infectious, infiltrative, or inflammatory etiologies.

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, except during infancy before the fontanelles close.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

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.
• Visual symptoms often are absent, but the following symptoms can occur:
• • Some patients experience transient visual obscurations (graying-out of their vision, 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 is preserved except in very advanced disease.

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. An abduction deficit secondary to a sixth nerve palsy sometimes may be seen in association with increased intracranial pressure.
• 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 the individuals may be obliterated when the intracranial pressure rises above 200 mm water.
  • 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
• 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

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

Toxic optic neuropathy
Optic disc infiltrates
Other optic nerve tumors
Diabetic papillitis

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Lab Studies

• Blood tests usually do not contribute to the diagnosis of papilledema. If the diagnosis is in doubt, CBC, blood sugar, angiotensin-converting enzyme, erythrocyte sedimentation rate (ESR), and syphilis serology may be helpful to look for signs of infectious, metabolic, or inflammatory diseases.

Imaging Studies

• Urgent neuroimaging (eg, CT scan, MRI) of the brain with contrast should be performed in an attempt to identify a CNS mass lesion.
• B-scan ultrasonography may be useful to rule out buried disc drusen.
• Fluorescein angiography can be used to help establish the diagnosis. Acute papilledema exhibits increased dilation of the peripapillary capillaries with late leakage of the dye.

Other Tests

• Perimetry
• • Visual fields should be tested. They commonly show enlargement of the blind spot. With extreme disc edema, a pseudo–bitemporal hemianopsia may be seen.
  • With chronic papilledema, constriction of the visual field, especially inferiorly, gradually can occur, which eventually may progress to a loss of central acuity and total blindness.
• Stereo color photographs of the optic discs are useful to document changes.

Procedures

• A lumbar puncture should be performed following a normal MRI to assess the opening pressure of the CSF and to obtain CSF for analysis to rule out neoplastic and infectious etiologies. It may provide some therapeutic benefit as the CSF pressure is reduced temporarily.

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical Care

• Therapy, whether medical or surgical, is tailored to the underlying pathological process and the progression of the ocular findings.
• Specific therapy should be directed to the underlying mass lesion if present.
• Diuretics: The carbonic anhydrase inhibitor, acetazolamide (Diamox), may be useful in selected cases, especially cases of idiopathic intracranial hypertension. (In the presence of venous sinus thrombosis, diuretics are contraindicated. In this scenario, evaluation by a hematologist is recommended.)
• Weight reduction is recommended in cases of idiopathic intracranial hypertension.
• Corticosteroids may be effective in cases associated with inflammatory disorders (eg, sarcoidosis).

Surgical Care

• The underlying mass lesion, if present, should be removed.
• Lumboperitoneal shunt or ventriculoperitoneal shunt can be used to bypass CSF.
• Optic nerve sheath decompression can be used to relieve worsening ocular symptoms in cases of medically uncontrolled idiopathic intracranial hypertension. This procedure probably will not ameliorate persistent headaches if present.

Consultations

Besides an ophthalmologist, a neurologist should be involved in monitoring the patient, and a neurosurgeon may be needed to help evaluate any underlying mass or to perform a shunting procedure.

Diet

Dietary restrictions and consultation with a dietitian in case of idiopathic intracranial hypertension is recommended.

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Diuretics may be helpful in cases of elevated intracranial pressure. Diamox and other carbonic anhydrase inhibitors can decrease the production of CSF.

Drug Category: Carbonic anhydrase inhibitors

Can be used in selected cases because they decrease the production of CSF and, thus, lower intracranial pressure.

Drug Category: Corticosteroids

May be useful in cases where inflammatory lesions lead to a secondary elevation in CSF pressure. These drugs are effective in these cases because of their potent anti-inflammatory effects.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Further Outpatient Care

• The patient should be examined weekly until stabilization of the ocular findings occurs. Well-developed papilledema takes 6-10 weeks to regress, following lowering of intracranial pressure.

In/Out Patient Meds

• See Medication.

Complications

• Unrelenting papilledema eventually may lead to permanent blindness.

Prognosis

• The visual prognosis is generally good if the intracranial pressure is controlled.

MISCELLANEOUS

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• Nonarteritic ischemic optic neuropathy (Foster Kennedy syndrome) and Leber hereditary optic neuropathy are 2 conditions where the appearance of usually bilateral sequential disc edema without intracranial pathology occurs.

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

• Acheson JF, Sanders MD. Common Problems in Neuro-ophthalmology. 1997: 78-84.
• Cullom RD, Chang B. The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease. 2nd ed. Lippincott-Raven;1994: 270-272.
• Kline LB. Optic Nerve Disorders, Ophthalmology Monographs. American Academy of Ophthalmology;1996: 37-53.
• Mathews MK, Sergott RC, Savino PJ. Pseudotumor Cerebri. Current Opinion in Ophthalmology. 2003;14:364-370.
• Miller NR, Newman NJ. The Essentials: Walsh & Hoyt's Clinical Neuro-ophthalmology. 5th ed. Lippincott Williams & Wilkins;1998: 166-195.
• Vaphiades MS. The Disk Edema Dilemma. Survey of Ophthalmology. 2002;47:183-188. [Full Text].
• Yanoff M, Duker JS. Ophthalmology. 1999: 11.5.1-5.4.

Article Last Updated: Jun 22, 2005