Papilledema

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Papilledema is swelling of the optic nerve(s) due to increased intracranial pressure.

Disease Entity

Disease

Papilledema refers to swelling of the optic disc from increased intracranial pressure (ICP)]. It must be distinguished from optic disc swelling from other causes which is simply termed "optic disc edema". Papilledema must also be distinguished from pseudo-papilledema such as optic disc drusen. Since the root cause of papilledema is increased intracranial pressure (ICP) this is an alarming sign which may presage such entities as brain tumor, CNS inflammation, or idiopathic intracranial hypertension (IIH).

Etiology

As noted above, papilledema is due to increased intracranial pressure from any cause. Normal ICP is typically less than 250 mm water when measured with a manometer in the recumbinat position. Papilledema results from orthograde axoplasmic flow stasis at the optic nerve head leading to edema of the nerve from the increased intracranial pressure pressing on the nerve behind the eye. Continued pressure can result in loss of axons and eventual optic atrophy. With optic atrophy there is little or no edema seen even with continuing increased intracranial pressure since there are no fibers to swell.  Increased intracranial pressure can be caused by (1) a space occupying lesion such as a tumor or hemorrhage, by (2) decreased CSF drainage due to blockage (obstructive hydrocephalus) or decreased absorption (infection, hemmorhage, venous sinus thrombosis or venous outflow obstruction), by (3) Idiopathic Intracranial Hypertenion (IIH, also called pseudtumor cerebri) , (4) rarely by increased CSF production such as from a choroid plexus tumor, and lastly by decreased skull volume (craniosynostosis).  The time course for development of papilledema may be weeks if there is only a slow and mild rise in intracranial pressure, but severe and rapid changes in pressure can cause papilledema to present within a day.

Risk Factors

Risk factors for papilledema are those that raise intracranial pressure. This includes space occupying lesions such as tumor or subarrachnoid hemoorhage, decrease of absorption of cerebrospinal fluid, change in the dynamics of cerebrospinal flow through the ventricles (for example by obstruction of the 4th ventricle), or rarely by increase of flow of cerebrospinal fluid.

General Pathology

Optic disc edema with neuronal swelling, venous dilation. Axoplasmic flow stasis and edema of the nerve head are noted.

Pathophysiology


Primary prevention

Diagnosis

Fundoscopy noting disc swelling (usually bilateral unless one disc is atrophic) in the presence of increased intracranial pressure is the key to diagnosis. Other diagnostic tools include ultrasonography, fluroescein angiography, CT or MR imaging, When presented with the possibility of papilledema the first approach is imaging to rule out space occupying lesion. This is best done by MRI and an accompanying MRV can help rule out a venous sinus thrombosis. Occasionally severe papilledema is noted on MRI as flattening of the sclera near the optic nerve and even a raised area at the disc head. A lumbar puncture with opening pressure and fluid sent for cytology and culture is done to confirm the diagnosis of increased intracranial pressure.


Disc Appearance

If spontaneous venous pulsations are present this suggests that ICP is probably not high at this time, however the ICP may have been high before simply due to diurnal variations in intracranial pressure.

Early papilledema - disc hyperemia possibly with dilated disc capillaries. Nerve fiber layer (NFL) opacification and swelling. Blurred disc margin(s). Venous pulsations are absent.  More severe developed papilledema will have elevation of the disc and opacified NFL obscuring disc vessels. There may be dilated veins, disc splinter hemorrhages, cotton wool spots, or hard exudates. With severe disc leakage a macular star may be seen, this is more prominent on the disc side of the macula. Later retinochroroidal folds called Paton's lines may be present. With chronicity of papilledema funduscopy reveals all of the above plus the start of disc pallor along with decreasing disc swelling with the loss of axons.  Sometimes optociliary shunts may be noted.  In the wake of papilledema there may be RPE pigmentary changes around the disc, disc gliosis and residual chroidal folds.


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Papilledema Grading System (Frisen Scale) Reference: Frisen L. Swelling of the optic nerve head: A staging scheme. J Neurol Neurosurg Psychiatry 1982; 45:13-18

Stage 0 - Normal Optic Disc   Blurring of nasal, superior and inferior poles in inverse proportion to disc diameter. Radial nerve fiber layer (NFL) without NFL tortuosity.

Rare obscuration of a major blood vessel, usually on the upper pole.

Stage 1 -Very Early Papilledema  Obscuration of the nasal border of the disc.  No elevation of the disc borders. Disruption of the normal radial NFL arrangement with

grayish opacity accentuating nerve fiber layer bundles. Normal temporal disc margin. Subtle grayish halo with temporal gap (best seen with indirect ophthalmoscopy).

Concentric or radial retrochoroidal folds.

Stage 2 - Early Papilledema Obscuration of all borders. Elevation of the nasal border. Complete peripapillary halo.

Stage 3 - Moderate Papilledema Obscurations of all borders. Increased diameter of optic nerve head. Obscuration of one or more segments of major blood vessels

leaving the disc. Peripapillary halo-irregular outer fringe with finger-like extensions.

Stage 4 - Marked Papilledema  Elevation of the entire nerve head. Obscuration of all borders. Peripapillary halo. Total obscuration on the disc of a segment of a major

blood vessel.

Stage 5 - Severe Papilledema  Dome-shaped protrusions representing anterior expansion of the optic nerve head. Peripapillary halo is narrow and smoothly demarcated.

Total obscuration of a segment of a major blood vessel may or may not be present. Obliteration of the optic cup.

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Examples:


Mild Papilledema

   Moderate Papilledema

  Severe Papilledema

Resolved Papilledema (post shunt)

History

Headache is typical and is often worse on wakening.

Ask about medicines that may cause increased intracranial pressure (such as tetracyclines, vitamin A and derivatives, steroids - especially changes in the amount of steroid taken)



Physical examination

In addition to the usual ophthalmic exam including visual fields one should check blood pressure to rule out malignant hypertension.  A neurological exam is appropriate.

Signs


Symptoms

Early papilledema has few symptoms. Headache is common with increased intracranial pressure and often worse in the morning. Nausea and vomiting are noted with increased intracranial pressure, especially with an acute rise in pressure. This is made worse by straining, coughing or bending. Visual fields often demonstrate increased size of the blind spot. When papilledema is severe, the patient may note short periods lasting seconds of losing vision which are called transient visual obscurations. These are observed when raising to stand or changing position. Farsightedness (hypermetropia) may increase because of pressure from the dilated optic nerve sheath flattening the back of the eye. Increased intracranial pressure can lead to hearing vascular noises, whooshing sounds, or pulsatile tinnitus.  Long standing papilledema may result in peripheral visual field loss as seen in IIH. Increased intracranial pressure can also lead to cranial nerve palsies, usually an abducens palsy. It is thought that the long course of the 6th cranial nerve is compressed by the increased CSF pressure.

Clinical diagnosis

Frisen scale

Diagnostic procedures

Fluorescein angiography can detect disc leakage. Autofluorescence can demonstrate drusen and B scan ultraound may also be helpful to find drusen.

Stereo disc images obtained to document and follow disc status.

Perimetry commonly shows enlarged blind spots. In severe chronic papilledema peripheral field loss may be seen.

CNS imaging study (CT or MRI with contrast) to identify a central nervous system mass lesion. 

MR venography should always be considered to look for venous sinus thrombosis.

Lumbar puncture (after imaging demonstrates no risk for herniation) to document increased ICP and to look for neoplastic, infectious or inflammatory causes. At a minimum, opening pressure along with glucose, protein, cell count and differential, culture are obtained.

Laboratory test


Differential diagnosis

Papilledema must be distinguished from optic disc edema and anonymously elevated discs such as buried drusen, tilted disc, hypoplastic disc.

These all can cause disc swelling:

   Intraocular inflammation causing optic disc edema (such as sarcoidosis, toxoplasmosis, VKH, other uveitis)

   Central Retinal Vein Occlusion

   Optic neuritis

   Diabetic papillopathy

   Hypertensive disc edema

   Non-arteritic ischemic optic neuropathy

   Infiltrative optic neuropathy and tumors (lymphoma, leukemia)

   Compressive optic neuropathy (optic nerve sheath meningioma)

   Scleritis

   Thyroid ophthalmopathy

   Hypertension

Management

The primary cause of the increased intracranial pressure must be addressed.  If a mass is present, primary therapy should be directed towards that. If  medications (tetracyclines, vitamin A analogues, etc.) are felt to be causative they should be discontinued.

Medications are used to decrease intracranial pressure by helping increase CSF absorption or decreasing CSF production.  Diuretics used include Acetalzolamide (diamox - a carbonic anhydrase inhibitor) and  Lasix  These are the mainstay of treatment in IIH along with surgical interventions.   Steroids can reduce inflammation if that is a component of the increased ICP and may help stabilize vision loss.  Steroids (especially with change in dosage) have also been causative of increased ICP.

Weight reduction in IIH  Weight loss (even 10-15% loss) in IIH can lead to normalization of intracranial pressure.


General treatment


Medical therapy

Carbonic anhydrase inhibitors (acetazolamide), lasix, See above

Medical follow up

Surgery

If a mass is causative, it should be addressed.

Ventriculoperitoneal (VP) and  lumboperitoneal (LP) shunts allow CSF fluid to drain and normalized ICP.

Optic nerve sheath fenestration- either unilateral or bilateral may be considered if vision is threatened. This will decompress the optic nerve but will usually not help with headache symptoms.

Surgical follow up

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Complications

Surgical complications include shunt failure and closure or scarring of optic nerve fenestrations.

Prognosis

Chronic papilledema leads to axon loss with constriction of the visual field, loss of disc substance and in the end, loss of central acuity.Patients (especially those with IIH) need to be followed to prevent vision loss.

Additional Resources

The Neuro-Ophthalmology Virtual Education Library:  library.med.utah.edu/NOVEL/

The North American Neuro-ophthalmolgy Society (NANOS):   www.nanosweb.org

References

1. Ehlers JP, Shah CP, eds. Papilledema. In: The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease. 5th ed. Baltimore, Md: Lippincott Williams & Wilkins; 2008:252-254.

2. Pane A, Burdon MA, Miller NR. The Neuro-ophthalmology Survival Guide. Mosby Elsevier 2006

3. Miller NR, et.al. eds. Walsh and Hoyt's Clinical Neuro-Ophthalmology, 6th Ed.   Lippincott Williams and Wilkins 2005. pp 237-291

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

5.Vaphiades MS. The disk edema dilemma. Surv Ophthalmol. Mar-Apr 4. 2002;47(2):183-8.

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

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

Original article contributed by: Edmond J. FitzGibbon, M.D.
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