An Overview of Papilledema

Papilledema is the term used for swelling of one or both optic nerve discs. The optic nerve disc, also called optic nerve head, is a small oval-shaped area on the back of the eye, marking the site of entrance of the optic nerve into the eyeball. Papilledema is a sign of some diseases that affect the brain. The conditions that cause papilledema can cause serious damage to the optic nerve or to the brain, resulting in vision loss or brain damage if not treated.

Often, papilledema is detected as part of the routine eye examination that you would have if you see an optometrist, an ophthalmologist, or a neurologist. It is a diagnostic finding that your healthcare provider can see with a quick non-invasive inspection of your eyes using a specialized eye exam device. Your practitioner would also check you to see if you have papilledema and complaints that could be related to one or more of the medical problems that can cause swelling of the optic discs.

Symptoms

Papilledema itself is not a disease, but it is a sign of disease. The conditions that cause papilledema may cause some symptoms, particularly if there is a lot of swelling in your optic discs.

Symptoms associated with papilledema can include:

• Blurred vision or loss of vision: This occurs due to physical pressure on the optic nerve, which controls your vision. Some people with optic nerve compression experience blurred vision or loss of vision from one or both eyes. Unlike many other neurological conditions, which cause symmetrical loss of vision from both eyes, papilledema is associated with loss of vision in only one eye or asymmetric loss of vision in both eyes.
• Headaches: Often, papilledema is associated with headaches and head pressure, as the conditions that produce swelling of the optic nerves may also cause pressure within the skull, causing pain. Your pain may worsen and improve as you change your head position, although each person with papilledema may have a different head position associated with comfort or with pain.
• Pain behind one or both eyes: The increased pressure that produces papilledema may be more concentrated near one or both eyes, causing pain that is most severe behind the eyes rather than all over the head.
• Fatigue: Papilledema is often associated with extreme fatigue and sleepiness.

Causes

There are a number of conditions that cause papilledema. The swelling itself is caused by a build-up of cerebrospinal fluid (CSF) or by physical pressure coming from the brain or from inside the eye. CSF is a fluid that surrounds the brain and spinal cord and does not communicate with blood or fluid elsewhere in the body. When cerebrospinal fluid builds up, it can cause pressure within the skull or on the optic nerves because it cannot "escape" from within the skull or the spine, thereby leading to papilledema.

Conditions that can cause papilledema include:

Brain Tumor

Brain tumors can develop in different areas of the brain, causing physical compression or obstruction of CSF flow within the skull. Physical pressure or CSF pressure can cause the optic disc to swell or to appear swollen.

Meningitis or Encephalitis

An infection of the brain (encephalitis) or the meninges that protect the brain (meningitis) can cause inflammation, resulting in swelling or fluid pressure that produces papilledema. 

Head Trauma

If you have a head injury, you may experience bleeding, bruising, or swelling of the injured areas of your brain. Generally, head trauma does not cause papilledema, but it can if the injury is near the eyes or if the swelling throughout your brain is severe.

Bleeding in the Brain

A brain hemorrhage is a bleed in the brain, and it can occur if a blood vessel bursts or due to trauma or a stroke. Often, brain hemorrhages result in swelling or excessive blood within the skull that can manifest with papilledema.

Idiopathic Intracranial Hypertension/Pseudotumor Cerebri

A condition that is caused by too much fluid production or by obstruction of fluid in the brain, this can occur as a result of antibiotics, the use of high doses of vitamin A, or without a known cause. Pseudotumor cerebri is given that name because the papilledema may be similar to symptoms when someone has a brain tumor, but it occurs without a tumor or a mass in the brain. This condition can cause permanent vision loss due to persistent pressure on the optic nerves. The fluid needs to be removed, usually with a lumbar puncture, which is also known as a spinal tap.

Cerebral Edema

Swelling and inflammation in the brain can occur due to a variety of causes, such as a large ischemic stroke, lack of oxygen to the brain, multi-organ failure, and fluid imbalance. Edema can also occur as the body’s response to excessive brain damage after a large stroke or when the fluid and electrolytes in the body are not at the proper concentration. Papilledema may worsen or improve depending on the degree of the brain swelling.

Diagnosis

Papilledema is a clinical diagnosis that your healthcare provider makes by looking at your optic nerve with an ophthalmoscope or with other equipment that is specialized for looking directly behind the eyes. This is a non-invasive diagnostic procedure during which your practitioner will look at your eyes, often by placing a magnifying device close to—but not actually touching—your eye.

These diagnostic devices can visualize the inside of your eye behind your pupil. This is not an imaging test; it is a "live" test, meaning that your healthcare provider looks at your eye to evaluate it in person. However, some types of specialized equipment can also take a photo of your eye so that your practitioner can share the results to consult with your other healthcare providers or compare changes in your eye over time.

The appearance of the swelling makes your optic nerve disc look as if it is a squished or enlarged, and sometimes there may be distended (abnormally widened), irregularly shaped blood vessels, or cloudy fluid as well.

Once papilledema is diagnosed, an imaging study of the brain, such as an MRI or CT scan, is usually performed to diagnose its underlying cause. In cases in which the brain imaging study is normal, a lumbar puncture should follow, with measurement of the opening pressure and analysis of the spinal fluid.

Treatment

Treatment of papilledema is specific to the underlying cause and involves procedures or medications that will eventually reduce the pressure in the optic discs.

Treatment options include:

Medication

If you have an infection or organ damage, medications to control those systemic health problems can reduce papilledema and prevent irreversible damage to the brain and eyes. There are also medications that can be used to help decrease the pressure inside the brain and consequently in the optic disc helping with the symptoms.

Lumbar Puncture

A lumbar puncture is normally considered a diagnostic procedure used to evaluate cerebrospinal fluid. With pseudotumor cerebri or increased intracranial pressure due to excessive fluid, a lumbar puncture can be used as a therapeutic procedure to remove the fluid, with the purpose of relieving the pressure. A lumbar puncture, also known as a spinal tap, involves placement of a needle in your lower back to collect fluid from around your spine. It is a mildly uncomfortable procedure, but it is safe, and recovery generally takes a few hours.

Some people develop a post-lumbar puncture headache as the body adjusts to the lowered CSF pressure after fluid removal. But if you have papilledema, you are likely to experience relief of your headaches after the procedure rather than a new headache. However, your healthcare providers might not do a lumbar puncture if you have a mass in your brain, as the changes in CSF flow and pressure can cause minor changes in the position of the mass and possibly dangerous displacement of the brain itself.

Stop Taking Medications That Cause Pressure

If your papilledema is caused by increased intracranial pressure due to medication, you need to stop taking that medication and include it in your medical record as a mediation that you cannot tolerate. Generally, stopping the medication prevents the worsening of the problem, but it is not enough to completely solve the problem after it has already developed, and you may also need to have a lumbar puncture to decrease the CSF build-up.

Shunting

If you have recurring papilledema due to chronically increased intracranial pressure, you may need to have a drain known as a ventriculoperitoneal (VP) shunt or lumboperitoneal shunt surgically placed to maintain a normal fluid volume and pressure. The VP shunt is usually needed for people who have had a brain tumor that caused blockage of the fluid flow in the brain while the lumboperitoneal shunt is more commonly used for patients with idiopathic intracranial hypertension/pseudotumor cerebri who failed clinical treatment.

Surgery

If you have a tumor or another anatomic cause of your papilledema, you may need to have it surgically removed to relieve the pressure and to prevent permanent vision loss and brain damage.

A Word From Verywell

If you or your child has papilledema, there are many possible causes. In general, your healthcare providers will perform a series of testing to help identify what is causing the papilledema. If symptoms such as blurred vision or vision loss have occurred, you need to have urgent treatment. 

Most of the time, papilledema can be treated effectively, but repeat treatment may be necessary. Be sure to maintain regular follow-up visits with a practitioner who examines your eyes, even after you have been effectively treated for papilledema.