Papilledema is used to denote a swelling of the optic nerve head (disc) resulting from raised intracranial pressure. There is a lack of inflammation, infiltration or infection of the optic nerve itself. Any edema of optic nerve head arising from causes other than increased intracranial pressure is termed optic disc edema, and not papilledema.
Presentation
A patient with papilledema presents with symptoms of increased intracranial pressure like headache and brief transient obscuration of vision. The headache is characteristic of increased intracranial pressure, worse on awakening and exacerbated by coughing. There can also be nausea and vomiting if the intracranial pressure is very high. A pulsatile tinnitus is sometimes present [6].
Visual acuity is usually well preserved. Some patients may have monocular or binocular visual blackouts that last for a few seconds, especially when rising from a recumbent position to a sitting or standing position. There may be transient flickering of lights, dyschromatopsia and constriction of the visual field. Relative afferent pupillary defect is usually absent. Fundus examination shows bilateral signs of optic disc edema like blurring of the optic disc margins, filling in of the optic disc cup, edema of the nerve fiber layer, retinal or choroidal folds, venous congestion, peripapillary hemorrhages, cotton-wool spots, hyperemia of the optic nerve head and hard exudates of the optic disc [7].
Entire Body System
- Pseudotumor
To report a case of a 25-year-old girl with pseudotumor cerebri who presented with unilateral swollen optic disk. [ncbi.nlm.nih.gov]
What is pseudotumor cerebri? Pseudotumor cerebri occurs when the pressure inside your skull becomes increased for no discernible cause. [neuroeyeorbit.com]
What are papilledema and pseudotumor cerebri? Papilledema and pseudotumor cerebri are conditions related to pressure inside your skull which can cause swelling in your optic nerve. [retinaspecialists.com]
Symptoms - Papilledema * Headache * Nausea * Vomiting * Vision symptoms * Transient loss of vision * Reduced field of vision Causes - Papilledema * Pseudotumor cerebri (idiopathic intracranial hypertension) –Most common cause of papilledema –Young, obese [checkorphan.org]
- Inflammation
Often this condition is a warning sign of a more serious medical condition such as CNS inflammation, brain tumours, or idiopathic intracranial hypertension (pressure around the brain with no detectable cause). [fightingblindness.ie]
Optic disc edema or swelling is concerning because an elevation of intracranial pressure can indicate a brain tumor, hemorrhage or inflammation. However, it can also be due to idiopathic or unknown causes. [eu.floridatoday.com]
[…] like sarcoidosis, but there is usually evidence of cancer or inflammation elsewhere Leber hereditary optic neuropathy, but usually unilateral and optic disc is hyperemic Central retinal vein occlusion, but that has more hemorrhage and less optic disc [kellogg.umich.edu]
It can be a red flag suggesting disease entities that cause elevated ICP such as brain tumors, cerebrospinal inflammation (infectious and non-infectious), and idiopathic intracranial hypertension (IIH). [ncbi.nlm.nih.gov]
Diagnosis is... read more, uveitis Overview of Uveitis Uveitis is defined as inflammation of the uveal tract—the iris, ciliary body, and choroid. [msdmanuals.com]
- Fever
We describe a case of dengue fever having mild encephalopathy and papilledema at presentation. CASE REPORT: Twenty-year-old female presented with fever, headache and vomiting. [ncbi.nlm.nih.gov]
She denies fevers, neck stiffness, facial weakness, dysarthria, and personal history of cancer or venous thromboembolic events. Vital signs (VS) include BP 188/110, HR 112, T 98.8 Oral, RR 14, SpO2 99% on room air. [emdocs.net]
rash, bowel problems), meningitis ( fever, neck stiffness), cerebral venous thrombosis ( personal or family history of hypercoaguable states, smoking, or oral contraceptive pills), and medications that might precipitate high ICP such as tetracyclines [eyewiki.aao.org]
Gastrointestinal
- Nausea
Nausea and vomiting: If the rise in intracranial pressure is severe, nausea and vomiting may occur. [hydroassoc.org]
Symptoms include headache, nausea and vomiting, ringing or other noises in the ears, and vision problems including temporary vision blackouts or “gray-outs” that last up to 30 seconds and affect one or both eyes or flashing lights. [neuroeyeorbit.com]
After renal transplantation, the patient had complete resolution of headaches, nausea and the papilledema. [ncbi.nlm.nih.gov]
Symptoms of increased intracranial pressure include headache or nausea and vomiting. Vision problems are not common initially, but may include short flickers of... [openmd.com]
- Projectile Vomiting
Projectile vomiting. Loss of consciousness/ generalized motor rigidity. 9. [slideshare.net]
But you can watch for signs of raised intracranial pressure: Nausea Vomiting Headache Projectile vomiting is a sign of elevated ICP. It causes you to forcefully expel stomach contents up to several feet away. [visioncenter.org]
The patient can have nausea and projectile vomiting along with it. 2. Visual symptoms- transient vision loss lasting seconds may occur due to the edema causing transient optic nerve ischemia, thereby obscuring the vision. [eophtha.com]
Cardiovascular
- Thrombosis
[…] optic disk secondary to increased intracranial pressure → classically bilateral but (as in the video example) may be unilateral Optic disk swelling resulting from causes that do not involve increased intracranial pressure (eg, malignant hypertension, thrombosis [smartypance.com]
Management of Venous Sinus Thrombosis. Neurosurg Clin N Am. 2018 Oct;29(4):585-594. [PubMed: 30223971] 8. [ncbi.nlm.nih.gov]
Her magnetic resonance imaging showed features of raised intracranial pressure with thrombosis of the superior sagittal and bilateral lateral sinus thrombosis. [ruralneuropractice.com]
Skin
- Macula
it gives rise to severe If front of the macula, it gives rise to severe If front of the macula, it gives rise to severe located in syndrome), in infants of subdural haemorrhage Medullated or myelinated nerve fibers White -yellow spots seen superiorly [thamburaj.com]
Optic disc showed features of advanced papilledema with normal macula and retinal periphery in both eyes. Visual acuity was 20/200 in the right eye and counting fingers close range in the left eye. [ncbi.nlm.nih.gov]
This exam protocol encompasses the posterior pole, including the macula, optic disc, and peripheral retina. Read more [d-eyecare.com]
maculae luteae senilis sicca (16) degeneratio maculae senilis (15) degeneratio maculae senilis exsudativa (13) degeneratio maculae senilis haemorrhagica (13) degeneratio maculae senilis neovascularis (13) degeneratio maculae senilis sicca (16) [zeitzfrankozeitz.de]
Ears
- Tinnitus
A 25-year-old obese patient admitted to our ophthalmic department complaining of headaches, tinnitus, and transient visual obscurations for the last three months. [ncbi.nlm.nih.gov]
Two months later, CT venogram showed no thrombus, but he developed visual obscurations, tinnitus, and new onset papilledema, peripheral field defects with normal visual acuity. [n.neurology.org]
He denied headache or eye pain, tinnitus, TOVs, and diplopia. Findings from his medical history included obesity, lumbar disc disease, and depression. [jamanetwork.com]
Eyes
- Enlarged Blind Spot
Goldmann visual field revealed an enlarged blind spot in the right eye only ( Figure 2 ). [omicsonline.org]
Visual field testing may detect an enlarged blind spot. Later, visual field testing may show defects typical of nerve fiber bundle defects and loss of peripheral vision. [merckmanuals.com]
The visual hallmarks are relatively spared visual acuity in the setting of bilateral optic disc edema with enlarged blind spots, nasal visual field loss, or constriction of the visual fields. [medlink.com]
Visual field testing may detect an enlarged blind spot. Later, visual field testing may show peripheral vision loss with arcuate defects that follow the nerve fiber bundle defects. [msdmanuals.com]
- Blurred Vision
Presentation Pearls Diagnosis Treatment Media Patient will present as → a 57-year-old male with a history of hypertension who complains of an acute onset of intermittent headaches and blurred vision of the right eye. [smartypance.com]
An example of papilledema is blurred vision and headache because of a brain tumor. YourDictionary definition and usage example. Copyright © 2018 by LoveToKnow Corp Link/Cite Link to this page Cite this page MLA Style "Papilledema." YourDictionary. [yourdictionary.com]
Briefly, the syndrome is manifested by a patient who feels generally well, may have an occasional headache, may complain of blurred vision, and may have tinnitus, vertigo, or dizziness. [jamanetwork.com]
They can present with headaches, ringing in ears, flickering in the vision, double vision, vision loss (sometimes unilateral and sometimes bilateral), nausea, vomiting, visual field defects and sometimes a decrease in the vision or blurred vision. [metrolinaeye.com]
- Visual Impairment
She presented at 19 months of age with bicoronal and sagittal synostosis and advanced visual impairment and papilledema. Surgical intervention included cranial expansion and reconstruction with a multidisciplinary team. [ncbi.nlm.nih.gov]
Persistent and extensive optic nerve head swelling, or optic disc edema, can lead to loss of these fibers and permanent visual impairment. [en.wikipedia.org]
- Visual Impairment
She presented at 19 months of age with bicoronal and sagittal synostosis and advanced visual impairment and papilledema. Surgical intervention included cranial expansion and reconstruction with a multidisciplinary team. [ncbi.nlm.nih.gov]
Persistent and extensive optic nerve head swelling, or optic disc edema, can lead to loss of these fibers and permanent visual impairment. [en.wikipedia.org]
- Central Scotoma
scotoma (loss of vision in the middle of the visual fields) in the ipsilateral eye anosmia (loss of smell) ipsilaterally This syndrome is due to optic nerve compression, olfactory nerve compression, and increased intracranial pressure (ICP) secondary [en.wikipedia.org]
One week after admission, his visual acuity remained 20/40 bilaterally with resolution of his partial central scotoma while disc edema improved 1.5-2 + OD, 1.5 + OS. [surgicalneurologyint.com]
In the left eye, there was a dense central scotoma and severe generalized depression. Findings from fundus examination showed bilateral disc edema, mild on the right and marked on the left, with peripapillary hemorrhages in both eyes. [jamanetwork.com]
scotoma should raise a red flag that this is not papilledema. [e-tjo.org]
Neurologic
- Papilledema
Features of ONHD vs True Papilledema. [reviewofoptometry.com]
Papilledema is usually seen bilaterally but can be seen asymmetrically and rarely unilaterally. [ncbi.nlm.nih.gov]
Disease or Syndrome papilledema [ pa-pil-eh-dee-muh ] Subclass of: Disorder of the optic nerve Definitions related to papilledema: Papilledema refers to edema (swelling) of the optic disc secondary to any factor which increases cerebral spinal fluid pressure [openmd.com]
Papilledema, also known as papilloedema, is optic disc swelling that is secondary to elevated intracranial pressure. In contrast to other causes of optic disc swelling, vision usually is well preserved with acute papilledema. [optos.com]
- Headache
Abstract Headaches associated with papilledema may be both life-threatening as well as vision-threatening. [ncbi.nlm.nih.gov]
A 49-year-old man presented with a three-week history of progressive visual loss and headaches. Funduscopic examination revealed bilateral papilledema. [nejm.org]
headache in an obese woman of childbearing age who is found to have papilledema on examination with an otherwise normal neurologic exam. [emdocs.net]
However, her headaches did not improve. She was subsequently referred to a neurosurgical team for an intracranial shunting procedure to redirect CSF. [racgp.org.au]
Do you experience headaches or loss of vision? Give us a call at Metrolina Eye Associates, to schedule an appointment. [metrolinaeye.com]
- Stroke
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. [verywellhealth.com]
Stroke 2004;35:664-70. 6. Narayan D, Kaul S, Ravishankar K, Suryaprabha T, Bandaru VC, Mridula KR, et al. [ruralneuropractice.com]
Stroke 1985;16:199-213. [PUBMED] 2. Coutinho JM, Ferro JM, Canhão P, Barinagarrementeria F, Cantú C, Bousser MG, et al. Cerebral venous and sinus thrombosis in women. Stroke 2009;40:2356-61. 3. Bousser MG, Ferro JM. [advbiores.net]
Arnold-Chiari malformation, aqueductal stenosis, postinfectious) * Intracranial hemorrhage (papilledema may not be seen acutely because it takes about 24 hours to develop after the ICP increases) –Subdural hematoma –Subarachnoid hemorrhage –Hemorrhagic stroke [checkorphan.org]
For example, increased intracranial pressure caused by a brain hemorrhage leads to an aneurysm or a stroke. But if a brain abscess causes it, the possible complications are meningitis, thrombosis, and septicemia. [visioncenter.org]
- Foster-Kennedy Syndrome
However, it can be unilateral in Foster Kennedy syndrome and its pseudovariants. Note however that with the advent of motion correction (e.g. [radiopaedia.org]
Foster Kennedy syndrome is thought to be present in 1% to 2.5% of intracranial masses.2 Tumours of the frontal lobe can become quite large before they are discovered, and focal symptoms (e.g., memory loss or emotional lability) may be sparse. [cmaj.ca]
Foster Kennedy syndrome Other names Gowers–Paton–Kennedy syndrome, Kennedy's phenomenon, Kennedy's syndrome Frontal lobe (on the right) Specialty Neurology Foster Kennedy syndrome is a constellation of findings associated with tumors of the frontal lobe [en.wikipedia.org]
Special forms: ❖ Foster Kennedy syndrome: This refers to isolated atrophy of the optic nervedue to direct tumor pressure on one side and papilledema due to increased intracranial pressure on the other side. [brainkart.com]
- Paresthesia
Adverse effects of acetazolamide include distal paresthesias, fatigue, and metallic taste associated with carbonated beverages, increased urination, stomach upset, and rarely kidney stones. The general management of IIH is similar to glaucoma. [e-tjo.org]
At that level, perioral and extremity paresthesias were a frequent side effect. If prescribed at a dose of greater than 2 g per day, the potassium level should be checked regularly. [medlink.com]
Workup
Apart from a detailed fundus examination, fundus fluorescein angiography may help in the diagnosis of papilledema. B-scan ultrasonography of the eye may be helpful in ruling out buried disc drusen. Visual fields should be tested, especially in chronic cases.
As soon as papilledema is diagnosed, urgent neuroimaging is required. Computed tomography (CT) scan and magnetic resonance imaging (MRI) of the brain with contrast are required to detect an intracranial mass lesion. At times, magnetic resonance venography may be required to detect venous sinus thrombosis. If MRI fails to show a lesion, a lumbar puncture should be performed to assess the opening pressure of the cerebrospinal fluid and to obtain fluid sample for histopathological and microbiological analysis [8].
Blood tests have limited value except when required to rule out infectious, inflammatory or metabolic causes. In such cases, complete blood count, blood sugar, erythrocyte sedimentation rate, and syphilis serology may be done.
Visual Field Test
- Central Scotoma
scotoma (loss of vision in the middle of the visual fields) in the ipsilateral eye anosmia (loss of smell) ipsilaterally This syndrome is due to optic nerve compression, olfactory nerve compression, and increased intracranial pressure (ICP) secondary [en.wikipedia.org]
One week after admission, his visual acuity remained 20/40 bilaterally with resolution of his partial central scotoma while disc edema improved 1.5-2 + OD, 1.5 + OS. [surgicalneurologyint.com]
In the left eye, there was a dense central scotoma and severe generalized depression. Findings from fundus examination showed bilateral disc edema, mild on the right and marked on the left, with peripapillary hemorrhages in both eyes. [jamanetwork.com]
scotoma should raise a red flag that this is not papilledema. [e-tjo.org]
Treatment
The treatment of papilledema associated with visual loss depends largely on the cause, symptoms, signs, and progression of the underlying condition. Medical treatment usually consists of diuretics, especially carbonic anhydrase inhibitors. Weight reduction in obese patients with idiopathic intracranial hypertension is recommended. Serial lumbar punctures may also be effective in lowering the intracranial pressure. Corticosteroids may be useful in sarcoidosis. If medical treatment is not sufficient, optic nerve sheath decompression or a ventriculo- or lumboperitoneal shunt may need to be carried out [9] [10].
Prognosis
The prognosis for papilledema depends on the cause. Patients with metastatic brain tumors have the worst prognosis. Individuals suffering from ventricular obstructive disease may have a better prognosis with successful shunting. Patients with idiopathic intracranial hypertension usually have the best outcomes. The diagnosis of papilledema requires a prompt workup with neurological, neurosurgical, or neuroradiologic consultations. Long-standing papilledema leads to irreversible visual loss in the form of secondary optic atrophy, constriction of visual fields and poor color vision [5].
Etiology
Common causes of increased intracranial pressure leading to papilledema are [2]:
- Intracranial tumors or space-occupying lesions
- Idiopathic intracranial hypertension (Pseudotumor cerebri)
- Decreased cerebrospinal fluid drainage (eg. venous sinus thrombosis, inflammatory processes, meningitis, subarachnoid hemorrhage)
- Increased production of cerebrospinal fluid due to tumors
- Cerebral edema/encephalitis
- Sarcoidosis
- Obstruction of the ventricular system may lead to a very rapid rise in intracranial pressure and fulminant papilledema
- Craniosynostosis
- Drugs like tetracycline, minocycline, lithium, nalidixic acid, and corticosteroids (more commonly from withdrawal than from use)
Epidemiology
Papilledema affects both sexes equally and can present at any age. During infancy elevated intracranial pressure may not lead to papilledema because the fontanels are not closed. Among young adults, papilledema is more likely to be caused by idiopathic intracranial hypertension than by a tumor. Idiopathic intracranial hypertension is often seen in obese, young women [3]. Intracranial masses most commonly cause papilledema in adults.
Pathophysiology
The subarachnoid space of the brain is continuous with the optic nerve sheath. Therefor conditions increasing the intracranial pressure can lead to compression of optic nerve leading to papilledema. Usually a combination of mechanical and vascular factors results in edema, ischemia, and eventual visual impairment or loss [4].
Prevention
Sometimes a patient may be asymptomatic and papilledema may be detected incidentally. However, on detection of papilledema, urgent neurological workup is important to save vision and/or life. Patients with known etiological factors should have regular fundus examination or relevant neurological follow up. For chronic conditions, follow up can prevent or detect in time any future occurrences of papilledema.
Summary
Papilledema is the swelling of the optic nerve head in the absence of any other optic nerve pathology, due to elevated intracranial pressure usually from causes like intracranial tumors and idiopathic intracranial hypertension.
The vision in papilledema is usually well preserved till late in the course of the disease, unlike in the other causes of optic nerve head swelling. The signs and symptoms are typical for increased intracranial tension such as nausea, vomiting, headache, transient obscuration of vision and diplopia. The treatment is directed at management of the underlying cause of elevated intracranial pressure. Diuretics may be given for decreasing the intracranial pressure. Early treatment and resolution of papilledema leads to complete visual recovery. Long-standing or severe papilledema may result in bilateral optic nerve dysfunction and secondary optic atrophy [1].
Patient Information
- Definition: Papilledema is a swelling of the optic nerve, at the point where it enters the retina in the back of the eye. The optic nerve carries visual signals from the eye to the brain.
- Cause: It is caused by an increase in the pressure of the fluid within the skull, with no infection or inflammation of the optic nerve itself. The common causes of rise in the intracranial pressure are tumors of the brain, spine or skull or any structure inside the skull, infection of the brain or meninges, hemorrhage, trauma, an abnormal closure of the bones of the skull, or an accumulation of cerebrospinal fluid within the skull, known as hydrocephalus.
- Symptoms: Headache, that is worse on waking up and is exacerbated by coughing. Nausea and vomiting may be there. There may be temporary and transient blurring of vision, poor color perception, flickering sensation, or double vision.
- Diagnosis: The eye doctor will examine your retina and optic nerve head with an ophthalmoscope. Angiography of the eye with a dye and ultrasound of the eye may be required at times. Computerized tomography and magnetic resonance imaging of brain is commonly used to detect the underlying disease. A testing of the spinal fluid may be required.
- Treatment and follow up: Treatment is directed at treating the cause, eg. a brain tumor. Diuretic drugs are given to decrease the intracranial pressure. Corticosteroids may be given if there is an inflammation in the brain like with sarcoidosis. With complete treatment and resolution of papilledema, no permanent visual loss occurs. Even after the underlying disease is managed, it is important to continue consultations with the eye doctor until the papilledema goes away. Chronic papilledema may lead to blindness.
References
- Ehlers JP, Shah CP, eds. Papilledema. In: The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease. 5th ed. Baltimore, Md: Lippincott Williams & Wilkins; 2008:252-254.
- Rosenberg MA, Savino PJ, Glaser JS. A clinical analysis of pseudopapilledema. I. Population, laterality, acuity, refractive error, ophthalmoscopic characteristics, and coincident disease. Arch Ophthalmol 1979; 97:65.
- Friedman DI. Papilledema and pseudotumor cerebri. Ophthalmol Clin North Am 2001; 14:129.
- Sinclair AJ, Burdon MA, Nightingale PG, Matthews TD, Jacks A, Lawden M, et al. Rating papilloedema: an evaluation of the Frisén classification in idiopathic intracranial hypertension. J Neurol. Jan 12 2012.
- Corbett J.J., Thompson H.S. The rational management of idiopathic intracranial hypertension. Arch Neurol 1989; 46:1049-1051.
- Scott CJ, Kardon RH, Lee AG, Frisén L, Wall M. Diagnosis and grading of papilledema in patients with raised intracranial pressure using optical coherence tomography vs clinical expert assessment using a clinical staging scale. Arch Ophthalmol. Jun 2010; 128(6):705-11.
- Yanoff M, Duker JS. Ophthalmology. 1999:11.5.1-5.4.
- Hayreh SS, Hayreh MS. Optic disc edema in raised intracranial pressure. II. Early detection with fluorescein fundus angiography and stereoscopic color photography. Arch Ophthalmol 1977; 95(7):1245–54.
- Friedman DI, Jacobson DM. Idiopathic intracranial hypertension. J Neuro-Ophthalmol 2004; 24(2):138–45.
- Rubin RC, Henderson ES, Ommaya AK, Walker MD, Rall DP. The production of cerebrospinal fluid in man and its modification by acetazolamide. J Neurosurg 1966; 25(4):430–6.