Papilledema
Encyclopedia
Papilledema is optic disc
swelling that is caused by increased intracranial pressure
. The swelling is usually bilateral and can occur over a period of hours to weeks. Unilateral presentation is extremely rare.
In intracranial hypertension, papilledema most commonly occurs bilaterally. When papilledema is found on fundoscopy, further evaluation is warranted as vision loss can result if the underlying condition is not treated. Further evaluation with a CT or MRI of the brain and/or spine is usually performed. Unilateral papilledema can suggest orbital pathology, such as an optic nerve glioma.
The signs of papilledema that are 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.
s for signs of papilledema should be carried out whenever there is a clinical suspicion of raised intracranial pressure
, and is recommended in newly onset headache
s. This may be done by ophthalmoscopy
or slit lamp
examination.
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 cell
s 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.
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 lasix. 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 exist, 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 ICP increase.
Optic disc
The optic disc or optic nerve head is the location where ganglion cell axons exit the eye to form the optic nerve. There are no light sensitive rods or cones to respond to a light stimulus at this point. This causes a break in the visual field called "the blind spot" or the "physiological blind spot"...
swelling that is caused by increased intracranial pressure
Intracranial pressure
Intracranial pressure is the pressure inside the skull and thus in the brain tissue and cerebrospinal fluid . 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...
. The swelling is usually bilateral and can occur over a period of hours to weeks. Unilateral presentation is extremely rare.
In intracranial hypertension, papilledema most commonly occurs bilaterally. When papilledema is found on fundoscopy, further evaluation is warranted as vision loss can result if the underlying condition is not treated. Further evaluation with a CT or MRI of the brain and/or spine is usually performed. Unilateral papilledema can suggest orbital pathology, such as an optic nerve glioma.
Signs and symptoms
Papilledema may be asymptomatic or present with headache 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 discOptic discThe optic disc or optic nerve head is the location where ganglion cell axons exit the eye to form the optic nerve. There are no light sensitive rods or cones to respond to a light stimulus at this point. This causes a break in the visual field called "the blind spot" or the "physiological blind spot"...
- blurring of optic margins
- elevation of optic disc
- Paton's lines = radial retinal lines cascading from the optic disc
On visual field
Visual field
The term visual field is sometimes used as a synonym to field of view, though they do not designate the same thing. The visual field is the "spatial array of visual sensations available to observation in introspectionist psychological experiments", while 'field of view' "refers to the physical...
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 eyeHuman eye
The human eye is an organ which reacts to light for several purposes. As a conscious sense organ, the eye allows vision. Rod and cone cells in the retina allow conscious light perception and vision including color differentiation and the perception of depth...
s for signs of papilledema should be carried out whenever there is a clinical suspicion of raised intracranial pressure
Intracranial pressure
Intracranial pressure is the pressure inside the skull and thus in the brain tissue and cerebrospinal fluid . 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...
, and is recommended in newly onset headache
Headache
A headache or cephalalgia is pain anywhere in the region of the head or neck. It can be a symptom of a number of different conditions of the head and neck. The brain tissue itself is not sensitive to pain because it lacks pain receptors. Rather, the pain is caused by disturbance of the...
s. This may be done by ophthalmoscopy
Ophthalmoscopy
Ophthalmoscopy is a test that allows a health professional to see inside the fundus of the eye and other structures using an ophthalmoscope . It is done as part of an eye examination and may be done as part of a routine physical examination...
or slit lamp
Slit lamp
The slit lamp is an instrument consisting of a high-intensity light source that can be focused to shine a thin sheet of light into the eye. It is used in conjunction with a biomicroscope...
examination.
Causes
- Raised intracranial pressureIntracranial pressureIntracranial pressure is the pressure inside the skull and thus in the brain tissue and cerebrospinal fluid . 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...
: brain tumorBrain tumorA brain tumor is an intracranial solid neoplasm, a tumor within the brain or the central spinal canal.Brain tumors include all tumors inside the cranium or in the central spinal canal...
, pseudotumor cerebri or cerebral venous sinus thrombosisCerebral venous sinus thrombosisCerebral venous sinus thrombosis is a rare form of stroke that results from thrombosis of the dural venous sinuses, which drain blood from the brain. Symptoms may include headache, abnormal vision, any of the symptoms of stroke such as weakness of the face and limbs on one side of the body, and...
, Intracerebral hemorrhage - Respiratory failureRespiratory failureThe term respiratory failure, in medicine, is used to describe inadequate gas exchange by the respiratory system, with the result that arterial oxygen and/or carbon dioxide levels cannot be maintained within their normal ranges. A drop in blood oxygenation is known as hypoxemia; a rise in arterial...
- HypotoniaHypotoniaHypotonia is a state of low muscle tone , often involving reduced muscle strength. Hypotonia is not a specific medical disorder, but a potential manifestation of many different diseases and disorders that affect motor nerve control by the brain or muscle strength...
- Accutane (Isotretinoin), which is a powerful derivative of Vitamin AVitamin AVitamin A is a vitamin that is needed by the retina of the eye in the form of a specific metabolite, the light-absorbing molecule retinal, that is necessary for both low-light and color vision...
, rarely causes Papilledema. - Hypervitaminosis A, in some people who take megadoses of nutritional supplements and vitamins.
- Hyperammonemia, elevated level of ammonia in blood (including cerebral edema/intracranial pressure)
- Guillain-Barré syndromeGuillain-Barré syndromeGuillain–Barré syndrome , sometimes called Landry's paralysis, is an acute inflammatory demyelinating polyneuropathy , a disorder affecting the peripheral nervous system. Ascending paralysis, weakness beginning in the feet and hands and migrating towards the trunk, is the most typical symptom...
due to elevated proteinProteinProteins are biochemical compounds consisting of one or more polypeptides typically folded into a globular or fibrous form, facilitating a biological function. A polypeptide is a single linear polymer chain of amino acids bonded together by peptide bonds between the carboxyl and amino groups of...
levels - Foster Kennedy syndromeFoster Kennedy syndromeFoster Kennedy syndrome refers to a constellation of findings associated with tumors of the frontal lobe....
(FKS) - Chiari Malformation
- Tumors of the frontal lobeFrontal lobeThe frontal lobe is an area in the brain of humans and other mammals, located at the front of each cerebral hemisphere and positioned anterior to the parietal lobe and superior and anterior to the temporal lobes...
- Acute Mountain Sickness and High Altitude Cerebral Oedema
- Lyme diseaseLyme diseaseLyme disease, or Lyme borreliosis, is an emerging infectious disease caused by at least three species of bacteria belonging to the genus Borrelia. Borrelia burgdorferi sensu stricto is the main cause of Lyme disease in the United States, whereas Borrelia afzelii and Borrelia garinii cause most...
(Lyme meningitisMeningitisMeningitis is inflammation of the protective membranes covering the brain and spinal cord, known collectively as the meninges. The inflammation may be caused by infection with viruses, bacteria, or other microorganisms, and less commonly by certain drugs...
specifically, when the bacterial infection is in the central nervous system, causing increased intracranial pressure). - Malignant HypertensionMalignant hypertensionMalignant hypertension or hypertensive emergency is severe hypertension with acute impairment of an organ system and the possibility of irreversible organ-damage...
- Medulloblastoma
- Orbital A.Ocular venous drainage block:central retinal venous block, cavernous sinus trombosis B.Local lesion:optic neuritisOptic neuritisOptic neuritis is the inflammation of the optic nerve that may cause a complete or partial loss of vision.-Causes:The optic nerve comprises axons that emerge from the retina of the eye and carry visual information to the primary visual nuclei, most of which is relayed to the occipital cortex of the...
, ischemia of the nerve head, toxin (methanol), infiltration of the disc by glioma,sarcoidosis and lymphoma
Pathophysiology
As the optic nerveOptic nerve
The optic nerve, also called cranial nerve 2, transmits visual information from the retina to the brain. Derived from the embryonic retinal ganglion cell, a diverticulum located in the diencephalon, the optic nerve doesn't regenerate after transection.-Anatomy:The optic nerve is the second of...
sheath is continuous with the subarachnoid space
Subarachnoid space
In the central nervous system, the subarachnoid cavity is the interval between the arachnoid membrane and pia mater....
of the brain
Brain
The brain is the center of the nervous system in all vertebrate and most invertebrate animals—only a few primitive invertebrates such as sponges, jellyfish, sea squirts and starfishes do not have one. It is located in the head, usually close to primary sensory apparatus such as vision, hearing,...
(and is regarded as an extension of the central nervous system
Central nervous system
The central nervous system is the part of the nervous system that integrates the information that it receives from, and coordinates the activity of, all parts of the bodies of bilaterian animals—that is, all multicellular animals except sponges and radially symmetric animals such as jellyfish...
), 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 cell
Ganglion cell
A retinal ganglion cell is a type of neuron located near the inner surface of the retina of the eye. It receives visual information from photoreceptors via two intermediate neuron types: bipolar cells and amacrine cells...
s 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. 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
Acetazolamide
Acetazolamide, sold under the trade name Diamox, is a carbonic anhydrase inhibitor that is used to treat glaucoma, epileptic seizures, Idiopathic intracranial hypertension , altitude sickness, cystinuria, and dural ectasia...
and lasix. 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 exist, such as multiple sclerosis
Multiple sclerosis
Multiple sclerosis is an inflammatory disease in which the fatty myelin sheaths around the axons of the brain and spinal cord are damaged, leading to demyelination and scarring as well as a broad spectrum of signs and symptoms...
, 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 ICP increase.