eMedicine Specialties > Ophthalmology > Optic Nerve

Papilledema

Mitchell V Gossman, MD, Partner and Vice President, Eye Surgeons and Physicians, St Cloud
Joseph Giovannini, MD, Chief of Ophthalmology, Eye Surgery Center, David Grant Medical Center, Travis Air Force Base

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)

Differential Diagnoses

Other Problems to Be Considered

Optic disc infiltrates
Other optic nerve tumors
Diabetic papillitis

Workup

Laboratory Studies

• Blood tests usually do not contribute to the diagnosis of papilledema. If the diagnosis is in doubt, CBC count, blood sugar, angiotensin-converting enzyme, erythrocyte sedimentation rate, 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.
• Consider magnetic resonance (MR) venography to detect venous sinus thrombosis.
• 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. Autofluorescence may reveal disc drusen.

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

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 and can be curative. Bariatric surgery may be considered in cases refractory to conventional methods of weight loss.¹
• Corticosteroids may be effective in cases associated with inflammatory disorders (eg, sarcoidosis).
• Consider withdrawing causative medications, as weighed against other medical necessities and alternatives.

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

Carbonic anhydrase inhibitors

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

Acetazolamide (Diamox, Diamox Sequels)

The conversion of carbon dioxide to bicarbonate plays a key role in the production of both aqueous humor and CSF. Carbonic anhydrase inhibitors act by inhibiting the conversion of carbon dioxide to bicarbonate, thus inhibiting the production of both aqueous humor and CSF. Dosage should be individualized; most patients cannot tolerate more than 1 g/d because of the adverse effects (eg, dizziness, metallic taste, lethargy, paresthesias). Diamox sequels may be better tolerated than tablets.

Dosing

Adult

250 mg to 1 g/d PO in divided doses q6-12h; dosage >1 g/d does not usually increase the effect, but higher doses are occasionally needed

Pediatric

Infants and children: 5-10 mg/kg/dose PO q6h

Interactions

May have an additive effect with other diuretics; caution while administering with a high dose of aspirin due to acidosis

Contraindications

Sulfa allergy; kidney and liver failure; renal stones; depressed serum sodium or potassium

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

All sulfonamide derivatives may lead to a number of serious, although uncommon, adverse reactions (eg, Steven-Johnson syndrome)

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.

Prednisone (Deltasone)

Prednisone, like other corticosteroids, can cause profound and varied metabolic and immunologic effects. Its usefulness in these cases stems from its strong anti-inflammatory properties.

Dosing

Adult

Initial: 60-80 mg/d, may be given as single daily dose or in divided doses; dosage should be individualized

Pediatric

1 mg/kg/d PO

Interactions

Immunosuppressive effects of corticosteroids are additive to other immunosuppressive drugs; when on corticosteroid therapy, patients should avoid immunizations and should not be vaccinated against smallpox

Contraindications

Systemic fungal infections; relative contraindications include peptic ulcer disease, tuberculosis, active infections, psychosis, and pregnancy

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Long-term corticosteroid treatment should not be considered; a 2-6 week course may be effective, recurrence can occur as the medication is tapered; the lowest effective dose should be used because of the widespread metabolic and immunologic effects seen; adverse effects include hyperglycemia, hypokalemia, increased intraocular pressure, cataract formation, decreased wound healing, and growth suppression in children; larger doses may cause elevation of blood pressure, salt, and water retention; reduction in dose must be gradual if administered for more than a few days because of the potential for drug-induced adrenocortical deficiency; corticosteroids blunt the immune response, patients on these drugs may be more susceptible to a variety of infections; psychic derangements may be associated with the corticosteroids; an enhanced effect of these drugs exists in patients with cirrhosis and hypothyroidism; patients subject to unusual physiologic stress during the course of treatment should be treated with supplemental corticosteroids in most cases

Follow-up

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.

Inpatient & Outpatient Medications

• See Medication.

Complications

• Unrelenting papilledema may eventually lead to permanent blindness.

Prognosis

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

Miscellaneous

Medicolegal 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

1. Nadkarni T, Rekate HL, Wallace D. Resolution of pseudotumor cerebri after bariatric surgery for related obesity. Case report. J Neurosurg. Nov 2004;101(5):878-80. [Medline].

2. Acheson JF, Sanders MD. Common Problems in Neuro-ophthalmology. 1997:78-84.

3. Cullom RD, Chang B. The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease. 2^nd ed. Lippincott-Raven: 1994:270-272.

4. Kline LB. Optic Nerve Disorders, Ophthalmology Monographs. American Academy of Ophthalmology. 1996;37-53.

5. Mathews MK, Sergott RC, Savino PJ. Pseudotumor cerebri. Curr Opin Ophthalmol. Dec 2003;14(6):364-70. [Medline].

6. Miller NR, Newman NJ. The Essentials: Walsh & Hoyt's Clinical Neuro-ophthalmology. 5^th ed. Lippincott Williams & Wilkins; 1998:166-195.

7. Vaphiades MS. The disk edema dilemma. Surv Ophthalmol. Mar-Apr 2002;47(2):183-8. [Medline]. [Full Text].

8. Yanoff M, Duker JS. Ophthalmology. 1999:11.5.1-5.4.

Keywords

papilledema, optic nerve sheath, optic nerve head, optic disc swelling, elevated intracranial pressure, acute papilledema, papillitis, pseudotumor

Contributor Information and Disclosures

Author

Mitchell V Gossman, MD, Partner and Vice President, Eye Surgeons and Physicians, St Cloud
Mitchell V Gossman, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Medical Association, American Society of Cataract and Refractive Surgery, Minnesota Medical Association, North American Neuro-Ophthalmology Society, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Coauthor(s)

Joseph Giovannini, MD, Chief of Ophthalmology, Eye Surgery Center, David Grant Medical Center, Travis Air Force Base
Joseph Giovannini, MD is a member of the following medical societies: American Academy of Ophthalmology and American Society of Cataract and Refractive Surgery
Disclosure: Nothing to disclose.

Medical Editor

Edsel Ing, MD, FRCSC, Assistant Professor, Department of Ophthalmology & Vision Sciences, University of Toronto: Consulting Staff, Toronto East General Hospital
Edsel Ing, MD, FRCSC is a member of the following medical societies: American Academy of Ophthalmology, American College of Physician Executives, American Society of Contemporary Ophthalmology, Canadian Ophthalmological Society, Contact Lens Association of Ophthalmologists, North American Neuro-Ophthalmology Society, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Managing Editor

Brian R Younge, MD, Professor of Ophthalmology, Mayo Clinic School of Medicine
Brian R Younge, MD is a member of the following medical societies: American Medical Association, American Ophthalmological Society, and North American Neuro-Ophthalmology Society
Disclosure: Nothing to disclose.

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

The authors and editors of eMedicine gratefully acknowledge the contributions of previous coauthor, Georgia Chrousos, MD, to the development and writing of this article.

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