Papilloedema (Raised Intracranial Pressure)

Overview

Papilloedema presents as bilateral swelling of the optic nerve head (ONH) secondary to increased intracranial pressure (ICP). The swelling of the ONH may be asymmetric and can develop over a few hours to weeks. Papilloedema requires an accurate diagnosis and urgent management as it is potentially sight and life threatening.

Raised ICP causes a reduction in brain perfusion and results in ischaemic damage. The increased pressure is transferred down the optic nerve sheath from the intracranial cavity, causing axoplasmic flow stasis and ischaemia. This leads to axonal oedema and interstitial fluid accumulation within the ONH tissue, resulting in swelling and elevation.

ICP can be caused by several mechanisms including an increase in brain volume (e.g. due to trauma or ischaemia), an increase in mass (e.g. intracranial tumours), an increase in cerebrospinal fluid (e.g. due to increased production or decreased absorption), an increase in blood volume (e.g. cardiovascular complications), the use of some medications and skull deformities. It may also have no known cause, termed idiopathic intracranial hypertension (IIH). IIH predominately affects women of childbearing age who are overweight however it may affect others outside of this group including children, the elderly and those without obesity. It may also be associated with recent weight change.

Symptoms of papilloedema can vary from person to person, but may include the following: a headache worse in morning upon waking or being recumbent, transient visual loss, diplopia, pulsatile tinnitus, nausea or vomiting. Central vision acuity is relatively well preserved in most cases.

Malignant hypertension is another cause of papilloedema and that is covered elsewhere in this resource. Please use the link at the bottom of the page to navigate to this page.

Acute Papilloedema

Acute papilloedema is characterised by blurry disc margins due to a thickened peripapillary RNFL and elevation of the optic disc on clinical examination. Additional clinical features include increased vessel tortuosity, dilated disc capillaries, vessel obscuration, peripapillary haemorrhages, loss of spontaneous venous pulsation, adjacent retinal and choroidal folds (Paton's folds) and disc hyperaemia.

Fundus autofluorescence imaging shows dense hypo-autofluorescence extending beyond the peripapillary area.

OCT imaging shows marked elevation of the ONH and thickening of the RNFL with "rainbow fringes" on the RNFL analysis. In some cases, a triangular shaped hypo-reflective “wedge” may be seen in the peripapillary region (termed a “V contour), however this is not always present. There may also be cystic spaces in the peripapillary retina and an anterior bowing of the RPE/ Bruch's membrane layer at the optic canal opening.

Visual field testing in the acute stages shows enlargement of the blind spot.

Chronic Papilloedema

In chronic cases, visual acuity can be affected and visual fields can constrict. Optociliary shunts may also be present.

Continued pressure on the ONH results in the death of axons and subsequently secondary optic atrophy. Disc pallor with RNFL thinning will occur and can be associated with visual field defects. The field defects can be unpredictable however the papillomacular bundle is typically spared until the later stages of disease, so visual acuity usually remains relatively good until this time.

A link to more information on secondary optic atrophy is included at the bottom of this page.

Case Examples

  • Case 1: Obvious papilloedema and IIH

    A 23 year old overweight Caucasian female with best corrected visual acuity of 6/7.5 (20/25) in both eyes. She has symptoms of headaches (worse in the morning), nausea and blurred vision.

    Fundus photos (right and left eye)

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    Fundus photos (right and left eye)

    Retinal photos show indistinct disc margins, vessel obscuration, disc hyperaemia and barely visible cups. There are also flame (splinter) haemorrhages radiating out from the disc in both eyes, more apparent in the right eye.

    Red free images (right and left eye)

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    Red free images (right and left eye)

    A red free filter helps to highlight the retinal haemorrhages.

    Stereoscopic image (right optic disc)

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    Stereoscopic image (right optic disc)

    Please use a stereoviewer or 15 prism dioptres base out to view this image.

    The stereoview shows the elevation of the optic nerve head. On this magnified image, the peripapillary haemorrhages are also easier to discern.

    Stereoscopic image (left optic disc)

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    Stereoscopic image (left optic disc)

    Please use a stereoviewer or 15 prism dioptres base out to view this image.

    The stereoview shows the elevation of the optic nerve head. On this magnified image, the peripapillary haemorrhages are also easier to discern.

    Cirrus RNFL analysis

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    Cirrus RNFL analysis

    The "rainbow fringes" seen on the RNFL thickness map are characteristic of papilloedema. The TSNIT curve shows the RNFL thickness in both eyes to be significantly outside the normative database in all clockhours. The tomograms show elevation of the optic nerves and a thickening of the RNFL.

    30-2 SITA-Standard visual field

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    30-2 SITA-Standard visual field

    The 30-2 test shows an enlarged blindspot in both eyes, with superior and nasal defects in the right eye as well.

  • Case 2: Subtle papilloedema and IIH

    A 38 year old, obese female with worsening "sensations" in her peripheral vision, worse on waking in the morning. The patient has a complex medical history including type 2 diabetes, sleep apnoea and systemic hypertension. Her best corrected visual acuity was 6/6+ (20/20+) in both eyes. This patient was referred urgently to hospital and the diagnosis of IIH was confirmed.

    Fundus photos (right and left eye)

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    Fundus photos (right and left eye)

    Funduscopy showed elevated discs with blurred nasal margins (right>left). A flame shaped haemorrhage is seen superior to the right disc and there are mild Patons lines superotemporal to the left disc.

    Red free images (right and left eye)

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    Red free images (right and left eye)

    Funduscopy showed elevated discs with blurred nasal margins (right>left). Disc haemorrhages are seen superior and nasal to right disc and there are mild Paton’s lines superotemporal to the left disc.

    Stereoscopic image (right optic disc)

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    Stereoscopic image (right optic disc)

    Please use a steroviewer or 15 prism dioptres base out to view the image.

    Stereoscopic viewing demonstrates the elevation of the disc.

    Stereoscopic image (left optic disc)

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    Stereoscopic image (left optic disc)

    Please use a steroviewer or 15 prism dioptres base out to view the image.

    Stereoscopic viewing demonstrates the elevation of the disc.

    3-D representation of the optic nerves

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    3-D representation of the optic nerves

    A 3-D representation of the elevated optic nerves does not necessarily provide diagnostic assistance, however it may be useful in patient explanations.

    Spectralis OCT volume and line scans (right eye)

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    Spectralis OCT volume and line scans (right eye)

    OCT line scans show elevation of the superior, nasal and inferior neuroretinal rims in both eyes. The opening of Bruch's membrane is also anteriorly displaced

    Spectralis OCT volume and line scans (left eye)

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    Spectralis OCT volume and line scans (left eye)

    OCT line scans shows elevation of the superior, nasal and inferior neuroretinal rims in both eyes. The opening of Bruch's membrane is also anteriorly displaced

    Cirrus RNFL analysis

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    Cirrus RNFL analysis

    OCT RNFL analysis shows ‘rainbow fringes’ around the optic nerve with marked RNFL thickening on the TSNIT curve and quadrant thickness measurements .

    Cirrus Ganglion Cell Analysis

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    Cirrus Ganglion Cell Analysis

    The GCA thickness is relatively unaffected.

  • Case 3: Subtle papilloedema

    A 24 year old Middle Eastern male with symptoms of headaches on wakening that last 1-2 hours and intermittent visual disturbances in his temporal vision. He reports no nausea or vomiting and his best corrected visual acuity is 6/6 (20/20) in each eye. This patient was referred urgently to hospital and a diagnosis of papilloedema was confirmed.

    Fundus photos (right and left eye)

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    Fundus photos (right and left eye)

    Retinal photos show average sized discs (left eye smaller than the right) with a tilted insertion (left more than right). The discs have small cups and margins are indistinct in the right eye and nasally in the left. The vessels are slightly obscured in the left.

    Red free images (right and left eye)

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    Red free images (right and left eye)

    The red free image show no retinal haemorrhages.

    Stereoscopic image (right optic disc )

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    Stereoscopic image (right optic disc )

    Please use a stereoviewer or 15 prism dioptres base out to view this image.

    A stereoscopic image of the right disc shows a slightly indistinct inferior disc margin.

    Stereoscopic image (left optic disc)

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    Stereoscopic image (left optic disc)

    Please use a stereoviewer or 15 prism dioptres base out to view this image.

    A stereoscopic image of the left disc shows an indistinct nasal disc margin.

    Spectralis OCT line scans (right optic disc)

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    Spectralis OCT line scans (right optic disc)

    OCT imaging shows a peripapillary intraretinal cystic space temporally in the right eye.

    Spectralis OCT line scans (left optic disc)

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    Spectralis OCT line scans (left optic disc)

    OCT imaging shows peripapillary intraretinal cystic spaces nasal to the disc in the left eye. The disc appears more heaped than the right eye with minimal retinal nerve fibre layer protrusion. This appearance is highly suggestive of papilloedema.

    Cirrus RNFL analysis

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    Cirrus RNFL analysis

    RNFL analysis shows the RNFL thickness is thicker than the normative database in most quadrants.

  • Case 4: Resolving papilloedema

    A 46 year old Middle Eastern male with best corrected visual acuity of 6/6+ (20/20+) in each eye. He reports mild symptoms of nausea in recent days. The clinical findings were suspicious for papilloedema at his initial visit and he was promptly referred for further investigations. Subsequent testing revealed raised intracranial pressure and a lumbar puncture was undertaken. The following images capture the resolution of the clinical signs of papilloedema over a period of 7 years.

    Fundus photos and fundus autofluorescence images (initial presentation)

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    Fundus photos and fundus autofluorescence images (initial presentation)

    Optic nerve photos show indistinct disc margins at the nasal part of the left disc and central glial tissue. The right disc appears relatively normal. Fundus autofluorescence did not show any evidence of disc drusen.

    Cirrus RNFL analysis

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    Cirrus RNFL analysis

    RNFL analysis of the left eye show "rainbow fringes". The TSNIT curve confirms increased RNFL thickness in the left eye, particularly inferiorly and superonasally (compared both with the right eye and the normative database).

    Flicker presentation - retinal photos pre- and post-lumbar puncture

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    Flicker presentation - retinal photos pre- and post-lumbar puncture

    Flicker comparison shows a reduction in the blur of the left disc margins following treatment.

    Flicker presentation - resolution of papilloedema over time

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    Flicker presentation - resolution of papilloedema over time

    Funduscopic flicker comparison over 7 years shows a reduction in the disc elevation and blurring of the margin after treatment was conducted with the disc appearance remaining stable since.

    Spectralis OCT Volume and line scans at follow up (left optic disc)

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    Spectralis OCT Volume and line scans at follow up (left optic disc)

    Comparative OCT line scans through the optic nerve showed marked ONH elevation prior to treatment that was reduced following treatment.

Differential Diagnosis

Optic nerve head drusen

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Small crowded discs

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Tilted disc syndrome

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Non-arteritic anterior ischaemic optic neuropathy (NAAION)

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Artertic anterior ischaemic optic neuropathy (AAION)

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Optic neuritis (papillitis)

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Diabetic papillopathy

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References

Arturo Carta, Stefania Favilla, Marco Prato, Stefania Bianchi-Marzoli, Alfredo A. Sadun, Paolo Mora; (2012) Accuracy of Funduscopy to Identify True Edema versus Pseudoedema of the Optic Disc. Invest. Ophthalmol. Vis. Sci. 2012;53(1):1-6.

Chiang, J., Wong, E., Whatham, A., Hennessy, M., Kalloniatis, M. and Zangerl, B. (2015), Differentiating optic nerve head swelling. Clin Exp Optom, 98: 12-24.

Mollan SP, Markey KA, Benzimra JD, et al (2014) A practical approach to, diagnosis, assessment and management of idiopathic intracranial hypertensionPractical Neurology14:380-390.

Rigi, M., Almarzouqi, S. J., Morgan, M. L., & Lee, A. G. (2015). Papilledema: epidemiology, etiology, and clinical management. Eye and brain, 7, 47–57.