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Optic Disc Swelling (including Papilloedema)

Background
Optic disc swelling can be caused by a number of conditions; papilloedema relates more specifically to optic disc
swelling secondary to raised intracranial pressure.
Disc swelling is distinct from disc atrophy which refers to a loss of nerve fibres at the optic nerve head and which
results in a pale disc. Atrophy may be primary (where it occurs without prior disc swelling) or secondary (where it
is preceded by disc swelling).
This article provides an account of how to assess a swollen disc and an overview of the most common (and
some less common) causes of optic disc swelling.
See separate articles Optic Atrophy and Macular Oedema to find out more about these conditions.

Finding optic disc swelling

If you suspect a swollen disc on fundoscopy, there are several points to note that will be helpful in guiding your
diagnosis and referral. The separate article Examination of the Eye provides more detail on the method of
fundoscopy and examination of optic nerve function.

History
Take a full systemic, neurological and ophthalmic history. Specifically ask about any headaches and their
characteristics (note the headache and nausea/vomiting, worse on waking, coughing and bending pulsatile
tinnitus of raised intracranial pressure).

Examination of the disc

(See the 'Internet and further reading' link below for good images of papilloedema.)
DO NOT DILATE the patient's pupils until you have ascertained the presence or absence of a relative
afferent pupillary defect (RAPD) - see 'Evaluation of optic nerve function', below. This is a very valuable
sign which must not be missed.
Look around the margin of the disc: are there any clear segments of the disc margin or is it swollen all
around? An isolated area of 'swelling' may actually be myelination of nerve fibres around the nerve
head.
What colour is the disc? Pallor suggests a whole additional range of conditions (compare to the other
side). If the swelling is severe, you may not be able to distinguish the disc from the background retina.
If you have a very good view, look for the presence of a spontaneous venous pulsation: this is a
pulsation seen in the veins just as they emerge from the optic nerve before radiating out. This is
absent in 20% of normal patients but not unilaterally.
Have a look at the rest of the fundus for other significant findings (eg pallor, haemorrhages, abnormallooking vasculature).
Don't forget to examine the fellow eye, even in the absence of symptoms.

Evaluation of optic nerve function

Check visual acuity (VA) with a Snellen chart.

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Check for a relative afferent pupillary defect (RAPD) using the swinging flashlight test:
Make sure that you are in a dark room, use a bright light source and ask the patient to gaze
into the distance (such as a far wall) to avoid physiological constriction of the pupil and to
maximise your chances of spotting an abnormal response.
Shine the light source from one eye to the other in rapid succession.
Normally, both pupils should constrict briskly when the light is shone in either eye.
In the presence of an RAPD, stimulation of the normal eye elicits a brisk constriction of both
pupils but when the light is shone on the diseased eye, both pupils dilate. What happens is
that the dilatation produced by withdrawing the light from the normal eye outweighs the
weak constriction produced by shining light on the diseased eye - this is why it is called a
relative afferent pupillary defect. (An afferent pupillary defect occurs where there is a critical
optic nerve lesion or optic nerve transection: the patient is then blind in that eye - "it's all
black when I cover my good eye" and neither pupil will constrict when the light is shone on
the affected side).
Check for colour impairment (dyschromatopsia). Ideally, this is done using Ishihara's colour test
booklet: cover the good eye first and flick through the book, allowing about five seconds per number;
then compare with the fellow eye. If the booklet is not available, ask the patient to compare the colour
of a bright red object (such as a child's toy). Descriptions of things looking "washed out" should ring
alarm bells; this is called red desaturation and is an early sign of optic nerve disease.
Assess brightness sensitivity: shine a light in each eye and ask the patients to compare the
brightness. A useful measure is to suggest: "If I were to give you a pound for this brightness" (shine
light in good eye), "how much would you give me for this ...?" (shine light in bad eye).
Carry out a confrontational visual field test, specifically looking for an enlarged blind spot.
Complement your examination with an assessment of the neurological system and the cranial nerves.

Staging of papilloedema [1]

If the disc swelling is confirmed to be papilloedema, there is a staging system used by some:

Early - in addition to blurred disc margins, there are distended retinal veins and dilated disc capillaries. Acute - as
above with associated haemorrhages and cotton wool spots. The retinal vessels may be obscured by retinal
oedema. Chronic - there is variable disc swelling and a reduction of the acute signs above. However, more longterm changes such as shunt vessels at the disc appear. Atrophic/late - the disc is pale, swelling is variable, the
vessels are attenuated.

Investigation of optic disc swelling [1]

Urgent neuro-imaging needs to be organised; MRI with gadolinium enhancement is ideal. Further investigations
may be be needed including magnetic resonance venography (MRV) to check the cerebral venous sinuses,
lumbar puncture (LP) to check the opening pressure as well as CSF biochemistry and microbiology, and possibly
fluorescein angiography of the fundus if there is diagnostic uncertainty.
Ultrasonography or spectral domain coherence retinal tomography (provides data to form a 3D image) may play
a role in diagnosing papilloedema, especially in differentiating from pseudopapilloedema (the former) and in mild
cases (the latter). [2] [3]

Causes of optic disc swelling [4]

Raised intracranial pressure: papilloedema [5] [6]
This may be due to obstruction of the ventricular system, space-occupying lesions, impairment of CSF
absorption, diffuse cerebral oedema or idiopathic (benign/essential) intracranial hypertension. Some medications
have been associated with this, eg tetracycline, minocycline, lithium, isotretinoin, nalidixic acid and
corticosteroids (both use and withdrawal).

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Presentation: headache (worse on waking and when coughing), may have nausea/vomiting and may
have other neurological symptoms. In contrast to other forms of disc swelling, visual acuity (VA) not
impaired initially but in later stages, may complain of fleeting visual loss lasting seconds, related to
posture (transient visual obscurations). There may be diplopia if there is a VI cranial nerve palsy. There
may be a history of head trauma. Take note of the overweight woman of child-bearing age on the oral
contraceptive pill - they account for 90% of cases of essential intracranial hypertension.
Ocular findings: disc swelling that is nearly always bilateral (it may be asymmetrical), normal (early)
or reduced (late) VA, enlarged blind spot, impaired colour vision, may have a relative afferent pupillary
defect (RAPD) or a VI cranial nerve palsy.
Systemic findings: neurological signs depending on the cause of the raised intracranial pressure.
Additional notes: patients with suspected papilloedema should be considered to have an intracranial
mass until proved otherwise. Imaging of the brain and orbits is mandatory and a lumbar puncture may
also be performed if imaging is normal. Not all patients with raised intracranial pressure develop
papilloedema - this depends on the site and size of the tumour and, in infants with open fontanelles, it
may fail to occur altogether. Patients who have previously had papilloedema may also fail to redevelop
it in the future.

Space-occupying lesions of the optic nerve head

Also see separate article on Eye and Optic Nerve Tumours.

Presentation: reduced vision; may complain of diplopia if globe movement is restricted. Large lesions
may also cause epiphora (tearing) and discomfort as a result of proptosis and the patient may
complain of a red eye due to congested blood vessels.
Ocular findings: may range from few or no findings to a proptosed eye which is congested
(conjunctiva is red and may be oedematous) and has a limited range of movement. VA reduced in later
stages and there may be an RAPD.
Systemic findings: depends on the nature of the lesion. Look for neurological signs, any pointers
towards possible malignant disease and evidence of hyperthyroidism.

Optic neuritis
This term refers to inflammation of the optic nerve, which can occur as a result of demyelinating or infective
disease processes. The optic nerve head is occasionally swollen but pallor of the disc is the norm. Also see
separate article Acute Optic Neuritis.

Demyelinating optic neuritis [7]

Presentation: subacute monocular visual impairment (bilateral symptoms can occur but are rare)
and there may also be some mild globe discomfort, particularly on moving the eye. Demyelinating
optic neuritis can occur in isolation but is more commonly part of a systemic problem which can
include multiple sclerosis (MS), Devic's disease (which is characterised by bilateral optic neuritis) and
Schilder's disease.
Ocular findings: reduced VA, RAPD, optic disc pallor, dyschromatopsia and visual field defects (most
frequently altitudinal or arcuate). Palsies involving the III, IV and VI cranial nerves may be present.
Systemic findings: this depends on where else the demyelination has occurred. For example, a
trigeminal or facial nerve palsy may be found in brain stem lesions.
Additional notes: there is a close association between optic neuritis and MS:
16% of patients who have optic neuritis but a normal MRI scan will go on to develop MS
within five years.
50% of patients presenting with optic neuritis will have demyelinating lesions on MRI scan.
In 70% of established MS cases, there will be evidence of a previous episode of optic
neuritis.
There are a number of factors that increase the risk of a patient who presents with optic neuritis
developing subsequent MS: winter onset, HLA-DR2 positivity and worsening of symptoms with
increase in body temperature (exercise, hot bath - Uhthoff's phenomenon). VA often returns over the
course of several weeks (in 85% of cases, to 6/12 or better) but other functions often remain abnormal
and a mild RAPD may persist.

Parainfectious optic neuritis

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Presentation: severe visual loss, usually bilateral, 1-3 weeks following a viral infection (eg measles,
mumps, chickenpox, whooping cough, glandular fever). Children are more frequently affected than
adults and this may occur after immunisations. There may have been other neurological phenomena:
headaches, ataxia or seizures.
Ocular findings: disc may be swollen or normal.
Systemic findings: look for features of meningo-encephalitis.
Additional notes: spontaneous complete visual recovery is the norm in the majority of patients - only
a small number will need systemic steroids if the visual loss is severe.

Infectious optic neuritis

Varicella-zoster virus: primary optic neuritis is uncommon unless immunocompromised. Secondary
optic neuritis arises from viral spread where acute retinal necrosis has occurred. These patients will
be treated with intravenous (IV) antivirals.
Sinus infections: occasionally, direct spread of infection (possibly due to sinus wall defects) or
occlusive vasculitis may occur following spheno-ethmoidal sinusitis. Patients will complain of severe
headaches and recurrent episodes of unilateral visual loss.Treatment is with systemic antibiotics but
surgical drainage of the sinus may also be needed.
Cat-scratch fever: this self-limiting infection has a good prognosis with visual recovery occurring
within 1-4 weeks of starting antimicrobial therapy.
Lyme disease: optic neuritis may occasionally occur. Peripheral neurological manifestations may
mimic MS. A course of IV ceftriaxone is the management of choice.
Syphilis: acute optic neuritis may occur both in the primary or secondary stages. Involvement may be
unilateral or bilateral.

Ischaemic anterior optic neuropathy [5]

Non-arteritic
Nature: this is a partial or total infarction of the optic nerve head due to occlusion of the posterior
ciliary arteries. Patients tend to be in the 45-65 year-old age group and predisposing factors include
hypertension, diabetes, hypercholesterolaemia, collagen vascular disease, antiphospholipid antibody
syndrome, sudden hypotensive episodes and cataract surgery.
Presentation: sudden painless monocular visual loss (often discovered on waking).
Findings: 70% of patients have moderate-to-severe reduction in VA, most will have a visual field
defect (typically altitudinal), dyschromatopsia proportional to the level of visual impairment and a pale
oedematous disc.
General management following referral: fasting lipids and glucose, exclusion of autoimmune
diseases. Carotid artery Doppler ultrasound scans will also be valuable to rule out a source of emboli.
Address other factors relating to cardiovascular diseases, including advice on smoking cessation.
Prognosis: most patients experience no further reduction in VA but 30-50% of them go on to
experience the same problem in the fellow eye within months or years. Non-arteritic ischaemic optic
neuropathy never occurs in the same eye twice.

Arteritic (giant cell arteritis)

See separate article Giant Cell (Cranial) Arteritis for more detail.

Nature: granulomatous inflammation of vessels involving the elastic tissues of the media and the
adventitia. There is a predilection for the temporal, ophthalmic, posterior ciliary and vertebral arteries.
Presentation: one or more of jaw claudication, scalp tenderness, neck pain, malaise, temporal artery
tenderness, visual reduction or loss in the patient over 55 years of age (some argue that this condition
does not occur before the age of 60 or 65 years). Episodes of amaurosis fugax may occur prior to
infarction of the optic nerve head. Patients may also complain of flashing lights and periocular pain.
Findings: VA may still be normal. Pale or swollen optic disc. Palpate the temporal artery: a tortuous,
tender artery with no palpable pulse are worrying findings.

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Initial management on suspicion of diagnosis: this is a medical emergency - failure to diagnose

and treat adequately can result in blindness of one or both eyes:
Once diagnosis is suspected, take a baseline set of bloods to be processed urgently,
including plasma viscosity or ESR (depending on local availability) and CRP - if the CRP is
normal, this is almost certainly not giant cell arteritis.
It is also helpful to do an FBC and a CXR, as these patients will be treated with IV
methylprednisolone initially and oral steroids subsequently.
Refer the same day: treatment will be initiated if the suspicion is strong, even before a
temporal artery biopsy is performed. This may confirm the diagnosis (although the disease
is patchy and may not occur in the segment biopsied) and will support the decision to start
the patient on high-dose oral steroids (in the region of 60-80 mg per day) after three pulses
of IV steroids (1 mg methylprednisolone on three consecutive days).
A temporal artery biopsy will remain positive up to 10 days after starting treatment but
should ideally be performed within three days (by the ophthalmologists or vascular
surgeons).
Starting patients on low doses of steroids and using a watch and wait approach in the
community is inappropriate and could reasonably result in medical negligence procedures
should vision be lost.

Prognosis: good if treatment is initiated promptly. If there is visual loss, nothing can be done for that
eye but the fellow eye's vision can be saved with treatment.

Accelerated (malignant) hypertension

Presentation: decreased VA and episodes of temporary visual loss. May be asymptomatic.
Ocular findings: attenuation of arterioles (copper wiring), arteriovenous nipping (narrowing of the
veins as the arteries pass over them) and signs of vascular leakage (haemorrhages and exudates).
Disc swelling occurs in the presence of very high blood pressure.
Systemic findings: very high blood pressure (usually greater than 200 systolic and 100 diastolic).
Additional notes: this medical emergency constitutes one of the most common causes of optic disc
swelling. [8] Although the mainstay of treatment is clearly to lower the blood pressure, this must be
done progressively, as a sudden drop can precipitate vascular occlusion.

Central retinal vein occlusion [9]

Presentation: this common condition may be ischaemic or non-ischaemic and involves occlusion of
the central retinal vein, resulting in a backlog and stagnation of blood, which leads to generalised
(including disc) oedema. Presentation is with a painless reduction in VA which ranges from very mild
to profound. This is monocular but the fellow eye can be affected in unfortunate patients. Rarely,
patients complain of discomfort and a red eye.
Ocular findings: mild-to-severe, sudden or progressive (over weeks) reduction in VA (if severe,
RAPD may also be present), dilated, tortuous veins, diffuse retinal haemorrhages and disc oedema.
Later on, there may be conjunctival congestion and disc oedema.
Systemic findings: evidence of cardiovascular disease. Other causes include clotting disorders,
blood dyscrasias, paraproteinaemias, vasculitides, systemic lupus erythematosis and the oral
contraceptive pill.
Additional notes: in the UK, these patients tend to be managed conservatively with regard to the eye
but there should be efforts made to address underlying predisposing factors. Some patients will
benefit from fluorescein angiography and laser treatment several months down the line - this decision
will be made by the ophthalmology team based on their clinical findings. However, the prognosis for
visual rehabilitation is generally quite poor, particularly for the ischaemic form.

Diabetic papillopathy
Presentation: progressive monocular visual loss.
Ocular findings: modest decrease in VA (6/12 or better), disc swelling may be unilateral or bilateral,
visual field defects (general constriction or scotoma), RAPD and dyschromatopsia.
Systemic findings: evidence of peripheral diabetic vasculopathy.
Additional notes: some controversy regarding treatment (many just observe) but spontaneous
resolution does frequently occur over several months. These patients will be closely monitored for
evidence of other diabetic retinopathy or maculopathy.

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Leber's optic neuropathy

Presentation: unilateral, acute, severe but painless visual loss with similar symptoms occurring in the
fellow eye within two months - typically in males in their twenties (rarely: females aged 10-60 years
old). Patients may have family history, as this is an inherited disorder.
Ocular findings: dilated capillaries on (swollen) disc surface and visual field defects (scotoma).
Systemic findings: none.
Additional notes: prognosis is generally poor, although a small number of patients will have some
degree of recovery in due course (this may be years).
Other causes of a swollen disc include: [10]
Pan or posterior uveitis.
Posterior scleritis.
Compressive optic neuropathy (eg thyroid eye disease).
Irvine-Gass syndrome (see separate Macular Oedema article).
Meningitis.
Infiltration or inflammation (eg sarcoid, leukaemia).
Carbon dioxide retention.
Parahypothyroidism.
Uraemia.
You may find these other separate articles useful:
Eye in Systemic Disease (more detail on some of the conditions outlined below, eg hypertensive
retinopathy).
Diabetic Retinopathy and Diabetic Eye Problems.
Non-diabetic Retinal Vascular Disease.
Retinal Artery Occlusions.
Retinal Vein Occlusions.
Idiopathic Intracranial Hypertension.
Rising Intracranial Pressure.

Pseudopapilloedema [11]
Nature: this is apparent disc swelling due to an underlying benign process. This could be due to
colloid bodies (drusen) buried within the optic nerve head, an unusual angulation of the disc or a small
disc from which the neurones emerge with a residual myelin sheath.
Presentation: these patients are usually asymptomatic but drusen can occasionally cause a
progressive loss of peripheral vision (and, rarely, central vision too).
Ocular findings: apparent disc swelling (often bilateral) in the absence of any other findings. Small
discs may look "crowded" with the vessels sprouting out like a bunch of flowers. Residual myelin
sheath is often limited to one part of the disc only.
Additional notes: treat as true optic disc swelling until proven otherwise. The investigations outlined
above should help guide the diagnosis. An ultrasound scan of the eye may identify the drusen; this can
be confirmed using a relatively new diagnostic imaging technique known as optical coherence
tomography (OCT). [12]

Management
If papilloedema is suspected (ie optic disc arising from raised intracranial pressure), there is an urgent need to
rule out an intracranial mass. Otherwise, optic disc swelling that is not thought to be papilloedema should be
referred according to the severity of the symptoms. All patients should have an ophthalmological assessment
and, unless there is good reason to think otherwise, it is prudent to assume that the swollen disc you are looking
at is papilloedema until proven otherwise. Ultimately, the underlying cause needs to be addressed.

Further reading & references

Diagnosing Idiopathic Intracranial Hypertension, Department of Ophthalmology and Visual Sciences, University of Iowa,
2001; (good images of the different stages of papilloedema)

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1. Denniston AKO, Murray PI; Oxford Handbook of Ophthalmology (OUP), 2009
2. Neudorfer M, Ben-Haim MS, Leibovitch I, et al; The efficacy of optic nerve ultrasonography for differentiating papilloedema
from Acta Ophthalmol. 2011 Sep 22. doi: 10.1111/j.1755-3768.2011.02253.x.
3. Vartin C V, Nguyen AM, Balmitgere T, et al; Detection of mild papilloedema using spectral domain optical coherence Br J
Ophthalmol. 2011 Jun 7.
4. Kanski J. Clinical Ophthalmology, ASystematic Approach, 5th Edition, 2003, Butterworth Heinemann.
5. Kunimoto DY, Kanitkar KD, Makar MS; The Wills Eye Manual, 4th Edition, 2004, Lippincott, Williams and Wilkins.
6. Gossman MV et al; Papilledema, Medscape, Dec 2009
7. Brass SD, Zivadinov R, Bakshi R; Acute demyelinating optic neuritis: a review. Front Biosci. 2008 Jan 1;13:2376-90.
8. Barnard S; Introduction to diseases of the optic nerve
9. Kooragayala LM, Central Retinal Vein Occlusion, Medscape, Jan 2011
10. Jackson TL; Moorfields Manual of Ophthalmology, Mosby (2008)
11. Mustonen E, Kallanranta T, Toivakka E; Neurological findings in patients with pseudopapilloedema with and without Acta
Neurol Scand. 1983 Oct;68(4):218-30.
12. Johnson LN, Diehl ML, Hamm CW, et al; Differentiating optic disc edema from optic nerve head drusen on optical
coherence tomography. Arch Ophthalmol. 2009 Jan;127(1):45-9.

Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical
conditions. EMIS has used all reasonable care in compiling the information but make no warranty as to its
accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions.
For details see our conditions.
Original Author:
Dr Gurvinder Rull

Current Version:
Dr Gurvinder Rull

Peer Reviewer:
Dr Helen Huins

Document ID:
577 (v24)

Last Checked:
17/11/2011

Next Review:
15/11/2016

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