Papilledema

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Papilledema
Papilledema.jpg
Fundal photograph showing severe papilledema in the left eye
Specialty Ophthalmology, optometry, neuro-ophthalmology, neurology, neurosurgery

Papilledema or papilloedema is optic disc swelling that is caused by increased intracranial pressure due to any cause. [1] The swelling is usually bilateral and can occur over a period of hours to weeks. [2] Unilateral presentation is extremely rare.

Contents

In intracranial hypertension, the optic disc swelling most commonly occurs bilaterally. When papilledema is found on fundoscopy, further evaluation is warranted because vision loss can result if the underlying condition is not treated. [1] Further evaluation with a CT or MRI of the brain and/or spine is usually performed. Recent research has shown that point-of-care ultrasound can be used to measure optic nerve sheath diameter for detection of increased intracranial pressure and shows good diagnostic test accuracy compared to CT. [3] Thus, if there is a question of papilledema on fundoscopic examination or if the optic disc cannot be adequately visualized, ultrasound can be used to rapidly assess for increased intracranial pressure and help direct further evaluation and intervention. Unilateral papilledema can suggest a disease in the eye itself, such as an optic nerve glioma.

Signs and symptoms

Fundal photograph showing severe papilloedema in the right eye. Fundal photograph showing severe papilloedema in the right eye.jpg
Fundal photograph showing severe papilloedema in the right eye.

Despite being classically referred to as a key symptom of rising intracranial pressure, papilledema is often not present in patients seen in an acute setting such as an emergency room. Many urgent cases of increased ICP only have identifiable papilledema after a day or more. [1]

Early on, papilledema may be discovered on examination with an ophthalmoscope without any changes in patient vision. It can progress to enlargement of the blind spot, blurring of vision, a concentric blind spot pattern, or diplopia (double vision). Ultimately, total loss of vision can occur, as well as other patterns of permanent injury to the optic nerve. [1]

Papilledema (right) revealed by scanning laser ophthalmoscopy (top) and laser Doppler imaging (bottom). Healthy contralateral eye (left). Papilledema revealed by scanning laser ophthalmoscopy and laser Doppler holography.gif
Papilledema (right) revealed by scanning laser ophthalmoscopy (top) and laser Doppler imaging (bottom). Healthy contralateral eye (left).

The signs of papilledema that may be seen using an ophthalmoscope include:

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.

Causes

Raised intracranial pressure [1] as a result of one or more of the following:

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.

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 fundus photography, and possibly slit lamp examination.

It is important to determine whether the observed condition is due to optic nerve head drusen, which can cause an elevation of the optic nerve head that can be mistaken for papilledema. For this reason, optic nerve head drusen is also called pseudopapilledema.

Treatment

Historically, papilledema was a potential contraindication to lumbar puncture, as it indicates a risk for tentorial herniation and subsequent death via cerebral herniation, however newer imaging techniques have been more useful at determining when and when not to conduct a lumbar puncture. [10] Imaging by CT or MRI is usually performed to elicit whether there is a structural cause i.e., tumor. An MRA and MRV may also be ordered to rule out the possibility of stenosis or thrombosis of the arterial or venous systems.

The treatment depends largely on the underlying cause. However, the root cause of papilledema is the increased intracranial pressure (ICP). This is a dangerous sign, indicative of a brain tumor, CNS inflammation or idiopathic intracranial hypertension (IIH) that may become manifest in the near future.

Thus, a biopsy is routinely performed prior to the treatment in the initial stages of papilledema to detect whether a brain tumor is present. If detected, laser treatment, radiation and surgeries can be used to treat the tumor.

To decrease ICP, medications can be administered by increasing the absorption of cerebrospinal fluid (CSF), or decreasing its production. Such medicines include diuretics like acetazolamide and furosemide. These diuretics, along with surgical interventions, can also treat IIH. In IIH, weight loss (even a loss of 10-15%) can lead to normalization of ICP.

Meanwhile, steroids can reduce inflammation (if this is a contributing factor to increased ICP), and may help to prevent vision loss. However, steroids have also been known to cause increased ICP, especially with a change in dosage. However, if a severe inflammatory condition exists, such as multiple sclerosis, steroids with anti-inflammatory effects such as Methylprednisolone and prednisone can help.

Other treatments include repeated lumbar punctures to remove excess spinal fluid in the cranium. The removal of potentially causative medicines including tetracyclines and vitamin A analogues may help decrease ICP; however, this is only necessary if the medication is truly felt to contribute to the ICP increase.

Related Research Articles

<span class="mw-page-title-main">Cerebrospinal fluid</span> Clear, colorless bodily fluid found in the brain and spinal cord

Cerebrospinal fluid (CSF) is a clear, colorless body fluid found within the tissue that surrounds the brain and spinal cord of all vertebrates.

<span class="mw-page-title-main">Idiopathic intracranial hypertension</span> Medical condition

Idiopathic intracranial hypertension (IIH), previously known as pseudotumor cerebri and benign intracranial hypertension, is a condition characterized by increased intracranial pressure without a detectable cause. The main symptoms are headache, vision problems, ringing in the ears, and shoulder pain. Complications may include vision loss.

<span class="mw-page-title-main">Optic nerve</span> Second cranial nerve, which connects the eyes to the brain

In neuroanatomy, the optic nerve, also known as the second cranial nerve, cranial nerve II, or simply CN II, is a paired cranial nerve that transmits visual information from the retina to the brain. In humans, the optic nerve is derived from optic stalks during the seventh week of development and is composed of retinal ganglion cell axons and glial cells; it extends from the optic disc to the optic chiasma and continues as the optic tract to the lateral geniculate nucleus, pretectal nuclei, and superior colliculus.

<span class="mw-page-title-main">Lumbar puncture</span> Procedure to collect cerebrospinal fluid

Lumbar puncture (LP), also known as a spinal tap, is a medical procedure in which a needle is inserted into the spinal canal, most commonly to collect cerebrospinal fluid (CSF) for diagnostic testing. The main reason for a lumbar puncture is to help diagnose diseases of the central nervous system, including the brain and spine. Examples of these conditions include meningitis and subarachnoid hemorrhage. It may also be used therapeutically in some conditions. Increased intracranial pressure is a contraindication, due to risk of brain matter being compressed and pushed toward the spine. Sometimes, lumbar puncture cannot be performed safely. It is regarded as a safe procedure, but post-dural-puncture headache is a common side effect if a small atraumatic needle is not used.

<span class="mw-page-title-main">Cerebral edema</span> Excess accumulation of fluid (edema) in the intracellular or extracellular spaces of the brain

Cerebral edema is excess accumulation of fluid (edema) in the intracellular or extracellular spaces of the brain. This typically causes impaired nerve function, increased pressure within the skull, and can eventually lead to direct compression of brain tissue and blood vessels. Symptoms vary based on the location and extent of edema and generally include headaches, nausea, vomiting, seizures, drowsiness, visual disturbances, dizziness, and in severe cases, death.

<span class="mw-page-title-main">Intracranial pressure</span> Pressure exerted by fluids inside the skull and on the brain

Intracranial pressure (ICP) is the pressure exerted by fluids such as cerebrospinal fluid (CSF) inside the skull and on the brain tissue. ICP is measured in millimeters of mercury (mmHg) and at rest, is normally 7–15 mmHg for a supine adult. The body has various mechanisms by which it keeps the ICP stable, with CSF pressures varying by about 1 mmHg in normal adults through shifts in production and absorption of CSF.

<span class="mw-page-title-main">Amaurosis fugax</span> Medical condition

Amaurosis fugax is a painless temporary loss of vision in one or both eyes.

Cushing reflex is a physiological nervous system response to increased intracranial pressure (ICP) that results in Cushing's triad of increased blood pressure, irregular breathing, and bradycardia. It is usually seen in the terminal stages of acute head injury and may indicate imminent brain herniation. It can also be seen after the intravenous administration of epinephrine and similar drugs. It was first described in detail by American neurosurgeon Harvey Cushing in 1901.

<span class="mw-page-title-main">Intraparenchymal hemorrhage</span> Medical condition

Intraparenchymal hemorrhage (IPH) is one form of intracerebral bleeding in which there is bleeding within brain parenchyma. The other form is intraventricular hemorrhage (IVH).

Optic neuropathy is damage to the optic nerve from any cause. The optic nerve is a bundle of millions of fibers in the retina that sends visual signals to the brain. [1].

<span class="mw-page-title-main">Optic disc drusen</span> Medical condition

Optic disc drusen (ODD) are globules of mucoproteins and mucopolysaccharides that progressively calcify in the optic disc. They are thought to be the remnants of the axonal transport system of degenerated retinal ganglion cells. ODD have also been referred to as congenitally elevated or anomalous discs, pseudopapilledema, pseudoneuritis, buried disc drusen, and disc hyaline bodies.

<span class="mw-page-title-main">Central retinal vein</span> Vein in the retina of the eye

The central retinal vein is a vein that drains the retina of the eye. It travels backwards through the centre of the optic nerve accompanied by the central retinal artery before exiting the optic nerve together with the central retinal artery to drain into either the superior ophthalmic vein or the cavernous sinus.

<span class="mw-page-title-main">Cerebral venous sinus thrombosis</span> Presence of a blood clot in the dural venous sinuses or cerebral veins

Cerebral venous sinus thrombosis (CVST), cerebral venous and sinus thrombosis or cerebral venous thrombosis (CVT), is the presence of a blood clot in the dural venous sinuses, the cerebral veins, or both. Symptoms may include severe headache, visual symptoms, any of the symptoms of stroke such as weakness of the face and limbs on one side of the body, and seizures, which occur in around 40% of patients.

<span class="mw-page-title-main">Foster Kennedy syndrome</span> Medical condition

Foster Kennedy syndrome is a constellation of findings associated with tumors of the frontal lobe.

<span class="mw-page-title-main">Cerebrospinal fluid leak</span> Medical condition

A cerebrospinal fluid leak is a medical condition where the cerebrospinal fluid (CSF) surrounding the brain or spinal cord leaks out of one or more holes or tears in the dura mater. A cerebrospinal fluid leak can be either cranial or spinal, and these are two different disorders. A spinal CSF leak can be caused by one or more meningeal diverticula or CSF-venous fistulas not associated with an epidural leak.

Optic papillitis is a specific type of optic neuritis. Inflammation of the optic nerve head is called "papillitis" or "intraocular optic neuritis"; inflammation of the orbital portion of the nerve is called "retrobulbar optic neuritis" or "orbital optic neuritis". It is often associated with substantial losses in visual fields, pain on moving the globe, and sensitivity to light pressure on the globe. It is often an early sign of multiple sclerosis.

<span class="mw-page-title-main">Bonnet–Dechaume–Blanc syndrome</span> Medical condition

Bonnet–Dechaume–Blanc syndrome, also known as Wyburn-Mason syndrome, is a rare congenital disorder characterized by arteriovenous malformations of the brain, retina or facial nevi. The syndrome has a number of possible symptoms and can, more rarely, affect the skin, bones, kidneys, muscles, and gastrointestinal tract. When the syndrome affects the brain, people can experience severe headaches, seizures, acute stroke, meningism, and progressive neurological deficits due to acute or chronic ischaemia caused by arteriovenous shunting.

The monitoring of intracranial pressure (ICP) is used in the treatment of a number of neurological conditions ranging from severe traumatic brain injury to stroke and brain bleeds. This process is called intracranial pressure monitoring. Monitoring is important as persistent increases in ICP is associated with worse prognosis in brain injuries due to decreased oxygen delivery to the injured area and risk of brain herniation.

Increased intracranial pressure (ICP) is one of the major causes of secondary brain ischemia that accompanies a variety of pathological conditions, most notably traumatic brain injury (TBI), strokes, and intracranial hemorrhages. It can cause complications such as vision impairment due to intracranial pressure (VIIP), permanent neurological problems, reversible neurological problems, seizures, stroke, and death. However, aside from a few Level I trauma centers, ICP monitoring is rarely a part of the clinical management of patients with these conditions. The infrequency of ICP can be attributed to the invasive nature of the standard monitoring methods. Additional risks presented to patients can include high costs associated with an ICP sensor's implantation procedure, and the limited access to trained personnel, e.g. a neurosurgeon. Alternative, non-invasive measurement of intracranial pressure, non-invasive methods for estimating ICP have, as a result, been sought.

<span class="mw-page-title-main">Visual impairment due to intracranial pressure</span>

Spaceflight-associated neuro-ocular syndrome (SANS), previously called spaceflight-induced visual impairment, is hypothesized to be a result of increased intracranial pressure (ICP), although, experiments directly measuring ICP in parabolic flight have shown ICP to be in normal physiological ranges during acute weightless exposure. The study of visual changes and ICP in astronauts on long-duration flights is a relatively recent topic of interest to space medicine professionals. Although reported signs and symptoms have not appeared to be severe enough to cause blindness in the near term, long term consequences of chronically elevated intracranial pressure are unknown.

References

  1. 1 2 3 4 5 Blumenfeld, Hal (2022). Neuroanatomy through clinical cases (3rd ed.). Sunderland, MA, USA: Sinauer Associates. p. 143. ISBN   978-1-60535-962-5.
  2. ↑ K., Khurana, A. (2015). Comprehensive ophthalmology. Khurana, Aruj K., Khurana, Bhawna. (6th ed.). New Delhi: Jaypee, The Health Sciences Publisher. ISBN   9789351526575. OCLC   921241041.{{cite book}}: CS1 maint: multiple names: authors list (link)
  3. ↑ Ohle, Robert; McIsaac, Sarah M.; Woo, Michael Y.; Perry, Jeffrey J. (2015-07-01). "Sonography of the Optic Nerve Sheath Diameter for Detection of Raised Intracranial Pressure Compared to Computed Tomography A Systematic Review and Meta-analysis". Journal of Ultrasound in Medicine. 34 (7): 1285–1294. doi:10.7863/ultra.34.7.1285. ISSN   0278-4297. PMID   26112632. S2CID   25301791.
  4. ↑ Cameron AJ (1933). "Marked papilloedema in pulmonary emphysema". Br J Ophthalmol. 17 (3): 167–9. doi:10.1136/bjo.17.3.167. PMC   511527 . PMID   18169104.
  5. ↑ Garrity JA, Herman DC, Imes I, Fries P (1993). "Optic Nerve Sheath Decompression for Visual Loss in Patients With Acquired Immunodeficiency Syndrome and Cryptococcal Meningitis With Papilledema". American Journal of Opthamology. 116 (4). doi:10.1016/S0002-9394(14)71407-2.
  6. ↑ Fahmy JA (1972). "Papillodema associated with ruptured intracranial aneurysms". Acta Opthalmologica. 50 (6): 793-802. doi:10.1111/j.1755-3768.1972.tb06618.x.
  7. ↑ Krispel CM, Keltner JL, Smith W, Chu DG, Ali MR (2011). "Undiagnosed papilledema in a morbidly obese patient population: A prospective study". Journal of Neuro-Ophthalmology. 31 (4): 310–315. doi: 10.1097/WNO.0b013e3182269910 . PMID   21799447. S2CID   6759222.
  8. ↑ Rich Phillips (2012-02-10). "Astronaut feels space's toll on his body". CNN . Retrieved 2012-03-01.
  9. ↑ Stein JD, Kim DS, Mundy KM, Talwar N, Nan B, Chervin RD, Musch DC (2011). "The association between glaucomatous and other causes of optic neuropathy and sleep apnea". Am. J. Ophthalmol. 152 (6): 989–998.e3. doi:10.1016/j.ajo.2011.04.030. PMC   3223261 . PMID   21851924.
  10. ↑ Gower DJ, Baker AL, Bell WO, Ball MR (1987). "Papilledema and lumbar puncture risk". J. Neurol. Neurosurg. Psychiatry. 50 (8): 1071–4. doi:10.1136/jnnp.50.8.1071. PMC   1032242 . PMID   3655817.
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