Introduction
Papilledema is the only term that can be used when disk swelling is the direct result of increased intracranial pressure (ICP). It is very important to differentiate this condition from optic disk edema, which refers to optic disk swelling brought on by other conditions. Pseudo-papilledema, such as optic disk drusen, must be differentiated from true papilledema to diagnose papilledema properly. Since elevated intracranial pressure (ICP) is the underlying cause of papilledema, this is a concerning symptom that may be a precursor to other conditions, such as a brain tumor, inflammation of the central nervous system (CNS), cerebral venous thrombosis, or idiopathic intracranial hypertension.
What Are the Causes of Papilledema?
Papilledema is caused by increased intracranial pressure. When measured using a manometer, standard intracranial pressure is usually less than 250 mm of water in adults and less than 280 mm of water in children. Papilledema is caused by orthograde axoplasmic flow stagnation (axoplasmic flow is a biological mechanism that moves organelles to and from a neuron's cell body through its axon's cytoplasm.) at the optic nerve head, leading to nerve edema. Persistent pressure can lead to the loss of axons and eventual optic atrophy due to intraneuronal ischemia, resulting in vision loss. However, since dead fibers do not swell, there is minimal or no edema with optic atrophy, even with persistently elevated intracranial pressure. If intracranial pressure rises slowly and mildly, papilledema may develop over a period of weeks. However, papilledema may occur within a few hours to a day if intracranial pressure changes rapidly and dramatically. Five mechanisms that can lead to increased intracranial pressure causing papilledema are:
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When the brain is too large for the skull (for example, craniosynostosis).
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When the brain becomes too large for the skull as a result of a space-occupying lesion (such as a tumor or a hemorrhage) or brain edema (for example, trauma).
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When the flow of cerebrospinal fluid is obstructed, this condition is known as a cerebrospinal fluid blockage (for example, a colloid cyst obstructing the foramen of Monroe).
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Increased CSF production (for example, choroid plexus papilloma).
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Reduced CSF absorption (for example, meningitis, cerebral venous thrombosis).
What Are the Symptoms of Papilledema?
The symptoms of papilledema include:
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Headaches.
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Vomiting.
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Nausea.
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Visual problems, such as double vision.
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Ringing in the ears, which often sounds like a pulse.
What Are the Risk Factors for Papilledema?
Things that raise the pressure inside the head can cause papilledema. This includes lesions that take up space, like a tumor or subarachnoid hemorrhage, a decrease in the brain's ability to absorb cerebrospinal fluid, a change in the way cerebrospinal fluid flows through the ventricles (for example, if the 4th ventricle is blocked), or, very rarely, an increase in the brain's ability to make cerebrospinal fluid are the risk factors for papilledema.
How Is Papilledema Diagnosed?
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Physical Examination: First, the doctor will do a complete physical exam to check the health as a whole and look for any other signs. Next, the doctor will probably move their hands back and forth across the field of vision to find the blind spots. The doctor uses an ophthalmoscope to look at the optic nerve in each of the patient’s eyes through the opening in the front of the eye called the pupil. The doctor may say that the patient has papilledema. The optic disk, which is at the end of the optic nerve, looks unusually blurry or high up. If the patient has this condition, the doctor might also see blood spots in the eye.
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Diagnostic Tests: If a doctor thinks a problem with the patient’s brain causes papilledema, they will do more tests. First, the doctor may give an MRI (magnetic resonance imaging) or CT (computerized tomography) scan of the patient’s head to look for tumors or other problems in the brain or skull. Then, the doctor may take a tissue sample (biopsy) from the tumor to look for cancerous cells or drain some of the CSF to look for any problems.
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Ophthalmic Investigations: These include:
- Spectral-domain optical coherence tomography of the retinal nerve fiber layer (SD-OCT RNFL) may be an objective way to find minor disk swelling and see if the condition improves.
- Stereo disk images help to track the disk's status over time.
- Formal (and usually automated) perimetry often shows enlarged blind spots and visual field defects similar to glaucoma.
- Fluorescein angiography can find disk leakage in true optic disk edema, which is different from pseudo papilledema, in which the disk is stained, but there is no leakage. Also, pseudo papilledema can be seen on autofluorescence, B scan ultrasound, and enhanced depth imaging optical coherence tomography of the optic nerve head.
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Neuroimaging: The immediate goal of an urgent CT scan would be to rule out a space-occupying lesion. Then, combined with a CT venogram, the goal would be to rule out cerebral venous thrombosis in the right situation. Also, if meningitis is suspected, an MRI and MRV (magnetic resonance venography) with contrast to look for signs of high intracranial pressure.
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Lumbar Puncture: Usually done after neuroimaging shows there is no risk of herniation to record the opening pressure and CSF composition (ruling out neoplastic, infectious, or inflammatory causes). The patient will get the opening pressure, glucose, protein, cell count, and differential culture at the very least.
How Is Papilledema Treated in Children?
The treatment of papilledema depends on the underlying cause. The management strategies include:
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If malignant hypertension is discovered, the child is taken to an emergency and treated immediately. Even with malignant hypertension, papilledema patients need neuroimaging.
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If there's a mass, treatment is focused on the removal of the tumor (for example, surgery).
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If acute cerebral venous thrombosis is discovered, the patient must be sent immediately to the thrombosis team. Acetazolamide should be avoided in cases of cerebral venous thrombosis since it can potentially worsen the situation. However, once the patient has begun anticoagulation, it may be added.
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Tetracyclines, vitamin A analogs, and other medications are thought to be the causative agents and should be stopped.
What Should Be Done for Managing Idiopathic Intracranial Hypertension (IIH)?
Treatment for IIH depends on its severity. This condition ranges from asymptomatic to fulminant with serious eyesight loss. If there is no immediate visual threat, it is treated with conservative management, and drugs can be used. If the patient's vision is threatened, hospitalization and surgery are necessary.
Conservative Management: It includes the following measures:
Weight Management:
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It was determined that a weight loss recommendation of between five and ten percent was necessary to improve symptoms and indicators of high intracranial pressure.
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Avoiding precipitating medications.
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Take action to address the underlying risk factors like thyroid disease or obstructive sleep apnea.
Medical Management:
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Acetazolamide is the first therapy (a carbonic anhydrase inhibitor). The Idiopathic Intracranial Hypertension Trial (IIHT) showed that Acetazolamide paired with weight loss efficiently treated IIH and improved papilledema in patients with modest visual loss.
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Topiramate and Lasix are second-line treatments if Acetazolamide does not work.
Surgery:
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If there is merely vision loss, optic nerve sheath surgery is done.
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If headaches are only accompanied by visual loss, the child will be operated on for cerebrospinal fluid diversion.
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Dural venous sinus stenting may be explored for patients with high intracranial pressure and severe venous sinus stenosis.
Conclusion
In most cases, papilledema does not present a problem on its own. In most cases, the condition can be addressed by draining excess CSF fluid, which reduces swelling. After a few weeks, the symptoms resolve by themselves. Brain swelling or damage can be a significant medical emergency and even put one’s life in danger. Get treated as soon as possible if there is an underlying ailment causing the papilledema to avoid any long-term problems.
