What Is Papilledema

Understanding papilledema: causes, symptoms, diagnosis, and treatment

Learn about the causes, symptoms, diagnosis methods, and treatment options for papilledema, a condition characterised by optic nerve swelling due to increased pressure inside the skull.

Overview

Papilledema is a condition where there is swelling of the optic nerve head, also known as the optic disc.1 It is typically caused by increased pressure inside the skull, which can occur due to various reasons such as brain tumours, infections, high blood pressure, or a condition called idiopathic intracranial hypertension (pseudotumor cerebri).2 The increased pressure puts strain on the optic nerve, leading to symptoms like blurry vision, headaches, nausea, and changes in vision. Treatment involves addressing the underlying cause, such as medication to reduce the pressure inside the skull or surgical interventions like optic nerve sheath fenestration. Prompt medical attention is crucial to identify and manage papilledema effectively.1

Causes papilledema can happen for different reasons. here are some common causes of papilledema:3

Brain Tumours: When there's a tumour in the brain, it can take up space and increase the pressure inside the skull. This pressure can affect the optic nerve and lead to papilledema.

Infections: Certain infections, like meningitis and  encephalitis, can cause inflammation in the brain. This inflammation can, in turn, increase the pressure inside the skull and result in papilledema.

High Blood Pressure: When your blood pressure is consistently high, it can strain the blood vessels in the brain. This strain can contribute to increased pressure inside the skull and lead to papilledema.

Idiopathic Intracranial Hypertension (Pseudotumor Cerebri): This is a condition of increased pressure with an unknown cause. The pressure builds up in the skull, causing papilledema.

Signs and symptoms4, 7 

Papilledema can cause certain signs and symptoms that indicate something is not right with your eyes and brain. Here are some common signs and symptoms of papilledema:

Blurry Vision: Your vision may become blurry or hazy, making it difficult to see clearly. This blurriness can involve  both close and far-away objects.

Headaches: You may experience frequent headaches, which can sometimes be more intense in the morning, but can also be  persistent and may not go away easily with over-the-counter painkillers.

Nausea and Vomiting: You may feel nauseous or experience episodes of vomiting. These symptoms can be persistent and unrelated to eating.

Changes in Vision: Your vision may go through some changes, such as seeing flickering lights or experiencing double vision. You may also find it harder to focus or have trouble with peripheral vision (side vision).

It's important to note that papilledema itself doesn't cause pain or vision loss, but it is a sign that something is affecting the optic nerve due to increased pressure in the skull. If you notice any of these symptoms, it's crucial to seek medical attention from an eye specialist or healthcare professional to determine the cause and receive appropriate treatment.

Management5, 8 

The management  of papilledema involves addressing the underlying cause and relieving the pressure on the optic nerve. Here's what you can expect: 

Identifying and Treating the Underlying Cause: The first step is to identify and treat the condition or factor that is causing the increased pressure in your skull. For example, if a brain tumour is the cause, you may need surgery to remove it. If it's due to high blood pressure, lifestyle changes and medications may be prescribed to manage your blood pressure.

Medications: In some cases, your doctor may prescribe medications to reduce the pressure inside your skull. Diuretics, also known as water pills, may be given to help your body eliminate excess fluid and reduce pressure. 

Monitoring and Follow-up: Regular check-ups with your doctor are important to monitor the progress of your condition. They may conduct repeat eye examinations and imaging scans, such as optical coherence tomography, to ensure the papilledema is improving and the underlying cause is being effectively managed. Surgical Interventions: In some cases, surgery may be needed to address specific problems. For example, if there is a blockage or too much fluid in the brain, a tube, called a shunt, can be placed to drain the extra fluid and alleviate  the pressure. Surgery may also be necessary to treat specific causes, such as removing a brain tumour or performing a procedure called optic nerve sheath fenestration. Optic nerve sheath fenestration involves creating small openings in the protective covering of the optic nerve, which is an extension of that covering the brain, which helps to relieve pressure on the nerve. This pressure can occur in conditions such as papilledema or optic nerve swelling. By making these small openings, the excess fluid around the brain and spinal cord can be drained, reducing the pressure on the optic nerve and potentially improving vision. 

It is crucial to follow your doctor's recommendations. Remember, each case is unique, and the specific treatment plan will depend on the underlying cause and individual circumstances. 

Diagnosis 4, 6 When a doctor suspects papilledema, they will order or perform certain tests to confirm the diagnosis. Here's what you can expect during the diagnosis of papilledema: 

Eye Examination: The doctor will carefully examine your eyes using a special instrument called an ophthalmoscope. This allows them to look at the back of your eyes and specifically at the optic nerve head (optic nerve disc, which is the exit point of nerve fibres on leaving the eye). They will check for signs of swelling of the optic nerve head (disc edema) or any other changes in the appearance of the optic nerve head.

Visual Field Test: This test measures your peripheral vision, or the ability to see objects at the sides while looking straight ahead. You may be asked to focus on a central point and respond to visual cues presented in different parts of your field of vision. This helps the doctor assess if papilledema is affecting your vision or if there are any visual field defects. 

Imaging Scans: To determine the underlying cause of papilledema, the doctor may order imaging scans such as an MRI or CT scan. These scans provide detailed images of your brain, allowing the doctor to look for any abnormalities, such as cerebral venous thrombosis, venous sinus thrombosis, or pseudotumor cerebri, that may be causing increased pressure in the skull.

Optical Coherence Tomography (OCT): OCT is a special non-invasive type of scan that helps doctors look inside your eye. It uses light waves instead of sound waves to create detailed pictures of  different parts of your eye, including  the retina and optic nerve. It's like taking a picture of the inside of your eye, but much more detailed. With OCT, doctors can check the health of your optic nerve and see if there are any calcium or protein deposits in the optic nerve head, which can make the nerve swell. They can also find out if there is any damage or problem with the optic nerve itself. This damage can be caused by different things like inflammation, injuries, certain medicines, or diseases like glaucoma or multiple sclerosis. When the optic nerve is damaged, it can affect your vision and lead to the problems of  blurriness  or impaired  colourful vision, or trouble seeing things on the sides. 

Lumbar Puncture: In some cases, a lumbar puncture (also spinal tap), may be performed. During this procedure, a small needle is inserted into your lower back to collect a sample of cerebrospinal fluid (the fluid bathing/surrounding the brain and spinal cord). The fluid pressure is then measured and signs of infection or other abnormalities are checked for, which can help differentiate true papilledema from pseudo papilledema (which is defined as the abnormal rise of the optic disc resulting in its apparent swelling but with no true oedema of the retinal nerve fibre layer which can be caused by such factors as optic nerve drusen (a round cluster of extracellular deposits of calcium, hyaline, and other proteins), optic neuropathy, or other anatomical variations. Typically, pseudo papilledema does not cause visual symptoms or headaches. It can be differentiated from true papilledema through careful examination by an eye specialist and additional tests like optical coherence tomography.

These diagnostic tests help the doctor confirm the presence of papilledema and determine the underlying cause.

FAQs

How can I prevent papilledema?

To prevent papilledema, focus on maintaining overall health and addressing underlying conditions that can increase skull pressure. Manage blood pressure through a balanced diet, exercise, and medications if necessary. Adopt a healthy lifestyle with a nutritious diet, regular exercise, sufficient sleep, and stress management. Seek prompt medical attention for head injuries or infections to prevent complications. Attend regular check-ups, including eye examinations and CSF pressure monitoring, to detect early signs of papilledema and address underlying causes.

How common is papilledema?

Papilledema can happen to anyone, regardless of age, gender, or background, depending on the underlying cause. One common cause of papilledema is a condition called idiopathic intracranial hypertension. This condition is more common in women who are overweight and of childbearing age. In the UK, according to a 2015 study published in “Eye and Brain" the number of cases per year is estimated to be around 1.56 for every 100,000 people in the general population. Among women aged 15-44, the number increases to about 2.86 per 100,000; if those women are obese, the number goes up to 11.9 per 100,000. The occurrence of this condition can vary in different countries. While it  is the usual cause, papilledema can also affect individuals who are not overweight and elderly. In these cases, it is important to undergo a thorough evaluation to find out the specific cause of papilledema.

Who is at risk of papilledema?

While papilledema can occur in anyone, certain groups of people have a higher risk. These include individuals with brain tumours, infections like meningitis or encephalitis, consistently high blood pressure and being overweight or obese. Certain medical conditions like idiopathic intracranial hypertension also make individuals more susceptible.

When should I see a doctor?

Seek medical attention as soon as possible if you experience any of the following:

  • Blurry vision or changes in vision.
  • Frequent and persistent headaches, especially if they are more intense in the morning.
  • Nausea and vomiting that occur without any apparent reason.
  • Difficulty focusing or seeing flickering lights.
  • Pulsatile tinnitus - whooshing or thumping noise that you hear in synchrony   with your pulse.

Even if you are unsure whether your symptoms are related to papilledema, it's better to consult a doctor who can assess your condition and provide the necessary guidance. Remember, early detection and intervention are key to managing papilledema effectively.

Summary

Papilledema describes a condition with an optic disc swelling, typically caused by increased pressure inside the skull, which can occur secondary to various causes that include brain tumours, brain or meningeal infections, high blood pressure, or idiopathic intracranial hypertension (pseudotumor cerebri). The increased pressure inside the skull puts strain on the optic nerve, leading to symptoms of blurry and other vision changes, headaches, and nausea/vomiting. Management involves addressing the underlying cause, medications to reduce the pressure inside the skull or surgical interventions as optic nerve sheath fenestrations. Prevention measures, and prompt medical attention are critical to effectively identify and manage papilledema.

References

  1. Rigi M, Almarzouqi SJ, Morgan ML, Lee AG. Papilledema: epidemiology, etiology, and clinical management. Eye Brain. 2015:  17(7): 47-57. Available from:  EB-69174-papilledema--epidemiology--etiology-and-clinical-management (nih.gov)
  2. Chen JJ, Bhatti M, and  Tariq. Papilledema. International Ophthalmology Clinics 2019; 59(3): 3-22. Available at: Papilledema : International Ophthalmology Clinics (lww.com)
  3. Franjić S. Eye Pain is Very Common in Ophthalmic Patients. MAR Ophthalmology 2021; 2.5. [Internet]. Available at: https://www.medicalandresearch.com/assets/articles/documents/DOCUMENT_20210528161547.pdf
  4. Friedman DI, Troung MN. ‌In Ravishankar K, Evans RW, Wang SJ (eds).  Modern Day Management of Headache: Questions and Answers. New Delhi: Jaypee Brothers Medical (P) Ltd; 2017. 403-19  Available at: https://www.google.co.uk/books/edition/Modern_Day_Management_of_Headache/PrlEDwAAQBAJ?hl=en&gbpv=1&dq=The+increased+pressure+puts+strain+on+the+optic+nerve
  5. ‌Sharma M, Chhabra A, Raina N. Optic nerve sheath fenestration: A second lease at sight. Indian Journal of Ophthalmology 2023; 71(7): 2845-49. Available at: Optic nerve sheath fenestration: A second lease at sight : Indian Journal of Ophthalmology (lww.com) 
  6. Grant TL. Papilledema from Idiopathic Intracranial Hypertension: A Girl with Headaches and Diplopia. In Heidary G, Phillips, PH (eds). Fundamentals of Pediatric Neuro-Ophthalmology. Papilledema from Idiopathic Intracranial Hypertension: A Girl with Headaches and Diplopia. New York: Springer, Cham; 2023. 35-38 Available at: DOCUMENT_20210528161547.pdf (medicalandresearch.com)
  7. Markey KA, Mollan SP, Jensen RJ, Sinclair AJ. Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions. The Lancet 2016; 15(1): 78-91. Available from: Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions - The Lancet Neurology
This content is purely informational and isn’t medical guidance. It shouldn’t replace professional medical counsel. Always consult your physician regarding treatment risks and benefits. See our editorial standards for more details.

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