Papilledema

Papilledema (or papilloedema) is optic disc swelling that is caused by increased intracranial pressure. The swelling is usually bilateral but can be unilateral which is extremely rare and can occur over a period of hours to weeks.

In intracranial hypertension papilledema can occur in only one eye or it can be more severe in one eye than the other or may not occur at all despite evidence of raised intracranial hypertension. When papilledema is present it is a pathological state and requires investigation to prevent loss of vision. Papilledema can often be a tell tale sign of intracranial hypertension. When one has papilledema in one eye one must also be sure that there is no local problem in the orbit (eye socket) that is causing unilateral papilledema. For example a tumor of the optic nerve behind the eyeball can also cause such swelling or blockage of circulation in the eye socket. It is important to have ultrasound and MRI studies to be sure.

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Signs and symptoms

Papilledema may be asymptomatic in the early stages. However it may progress to enlargement of the blind spot, blurring of vision, visual obscurations (inability to see in a particular part of the visual field for a period of time) and ultimately total loss of vision may occur

The signs of papilledema that are seen using an ophthalmoscope include venous engorgement (usually the first signs)
loss of venous pulsation
hemorrhages over and / or adjacent to the optic disc
blurring of optic margins
elevation of optic disc
Paton's lines = radial retinal lines cascading from the optic disc
Headaches

On visual field examination, the physician may elicit an enlarged blind spot; the visual acuity may remain relatively intact until papilledema is severe or prolonged.

Diagnosis

Checking the eyes for signs of papilledema should be carried out whenever there is a clinical suspicion of raised intracranial pressure, and is recommended in newly onset headaches. This may be done by ophthalmoscopy or slit lamp examination.

Causes

Raised intracranial pressure: brain tumor, pseudotumor cerebri or cerebral venous sinus thrombosis, Intracerebral hemorrhage
Respiratory failure
Hypotonia
Accutane (Isotretinoin), which is a powerful derivative of Vitamin A, rarely causes Papilledema.
Guillain-Barré syndrome due to elevated protein levels
Foster Kennedy syndrome (FKS)
Chiari Malformation
Tumors of the frontal lobe
Acute Mountain Sickness and High Altitude Cerebral Oedema
Lyme disease (Lyme meningitis specifically, when the bacterial infection is in the central nervous system, causing increased intracranial pressure).

Pathophysiology

As the optic nerve sheath is continuous with the subarachnoid space of the brain (and is regarded as an extension of the central nervous system), increased pressure is transmitted through to the optic nerve. The brain itself is relatively spared from pathological consequences of high pressure. However, the anterior end of the optic nerve stops abruptly at the eye. Hence the pressure is asymmetrical and this causes a pinching and protrusion of the optic nerve at its head. The fibers of the retinal ganglion cells of the optic disc become engorged and bulge anteriorly. Persistent and extensive optic nerve head swelling, or optic disc edema, can lead to loss of these fibers and permanent visual impairment.

Treatment

The treatment depends largely on the underlying cause. For instance, raised intracranial pressure may improve with glucocorticoids, acetazolamide or surgical shunting.

Translation

The word "Papilledema" occurs as such in the following languages: English, Italian.

Translation(s) in other languages: German: Stauungspapille, French: Papilloedema, Croatian: Edem optičkog diska, Portuguese: Papiledema, Swedish: Papillödem.