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Papilledema

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Kalsang Dolma, M.B.B.S.[2]

Synonyms and Keywords: Papilloedema

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Kalsang Dolma, M.B.B.S.[2]

Overview

Papilledema is defined as swelling of the optic disc 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.

Pathophysiology

Papilledema is in general the result of transmission of increased intracranial pressure[1] to the anterior end of optic nerve through optic nerve sheath.

Causes

Papilledema can be caused by conditions which increase intracranial pressure. An increase in the volume of any of the cranium contents, the brain, cerebrospinal fluid and / or blood can cause increased intracranial pressure.

Epidemiology and Demographics

Papilledema occurs in approximately 50% of patients with a brain tumor.

Differentiating Papilledema from other Diseases

Papilledema should be distinguished from pseudopapilledema

Natural History, Complications and Prognosis

Papilledema regresses if the underlying cause is treated and if not, can lead to vision loss.

Diagnosis

History and Symptoms

Patients with papilledema usually present with signs and symptoms of increased intracranial pressure (headache, nausea, vomiting, diplopia, vision loss and altered consciousness) and can lead to vision loss if the underlying condition is not treated.

Physical Examination

The signs of papilledema include blurring of the margins of the optic disc, edema, and hemorrhages on fundoscopy.

Treatment

Medical Therapy

The mainstay of treatment of papilledema is to treat the underlying cause which is often increased intracranial pressure. Drugs are used to decrease intracranial pressure which either decrease cerebrospinal fluid (CSF) production or increase the outflow of CSF.

Surgery

Surgical treatment of papilledema depends on the underlying cause. Various surgeries including shunt surgeries, craniotomies and optic nerve sheath fenestration can be done depending on the cause.

References

  1. Tso MO, Hayreh SS (1977). “Optic disc edema in raised intracranial pressure. IV. Axoplasmic transport in experimental papilledema”. Arch. Ophthalmol. 95 (8): 1458–62. PMID 70201. Unknown parameter |month= ignored (help)


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Historical Perspective

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References

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Classification

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References


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Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Kalsang Dolma, M.B.B.S.[2]

Overview

Papilledema is in general the result of transmission of increased intracranial pressure[1] to the anterior end of optic nerve through optic nerve sheath.

Pathophysiology

  • 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.
  • Persistent and extensive optic nerve head swelling, or optic disc edema, can lead to loss of these fibers and permanent visual impairment.
  • Papilledema may be absent in cases of prior optic atrophy. In these cases, the absence of papilledema is most likely secondary to a decrease in the number of physiologically active nerve fibers.

References

  1. Tso MO, Hayreh SS (1977). “Optic disc edema in raised intracranial pressure. IV. Axoplasmic transport in experimental papilledema”. Arch. Ophthalmol. 95 (8): 1458–62. PMID 70201. Unknown parameter |month= ignored (help)

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Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]: Associate Editor(s)-in-Chief: Kalsang Dolma, M.B.B.S.[2]

Overview

Papilledema can be caused by conditions which increase intracranial pressure. An increase in the volume of any of the cranium contents, the brain, cerebrospinal fluid and / or blood can cause increased intracranial pressure.

Causes

Common Causes

Causes by Organ System

Cardiovascular Hypertension, Hypertensive encephalopathy, Hypertensive retinopathy
Chemical / poisoning Methanol, Ethylene glycol
Dermatologic No underlying causes
Drug Side Effect Ciclosporin, Cisplatin, Corticosteroid, Dexamethasone, Growth hormone treatment, Insulin-like growth factor 1, Interleukin 11, Isotretinoin, Lithium, Medroxyprogesterone, Minocycline, Oprelvekin, Prednisone, Tetracycline
Ear Nose Throat No underlying causes
Endocrine Cushing’s disease, Diabetic retinopathy, Thyroid opthalmopathy
Environmental No underlying causes
Gastroenterologic Acute liver failure
Genetic Camurati – Engelmann disease, Crouzon syndrome, Fabry’s Disease, Tuberous sclerosis, Vogt-Koyanagi-Harada syndrome
Hematologic Acute lymphocytic leukemia
Iatrogenic No underlying causes
Infectious Disease Brucellosis, Epidural abscess, Infective endocarditis, Meningitis, Meningoencephalitis, Paragonimiasis, Syphillis, Toxoplasmosis
Musculoskeletal / Ortho Paget’s disease
Neurologic Cavernous Sinus Thrombosis, Arachnoid cyst, Arnold chiari malformation, Brain Abscess, Brain cyst, Brown – symmers disease, Cerebellar abscess,

Cerebral abscess, Cerebral venous sinus thrombosis, Dandy-Walker syndrome, Epidural hematoma, Foster kennedy syndrome, Intracranial bleeding Medulloblastoma, Myoclonic epilepsy, Parinaud’s syndrome, Subarachnoid hemmorhage, Subdural empyema, Subdural hematoma, Hydrocephalus, Cerebral edema, Gullian-Barre syndrome

Nutritional / Metabolic Nutritional optic neuropathy
Obstetric/Gynecologic Eclampsia
Oncologic Adult low grade infiltrative suoratentorial astrocytoma, Brain Stem Gliomas, Brain tumor, Chondroma, Choroid plexus neoplasm, Malignant Astrocytomas, Neuroectodermal tumor primitive, Osteoma, Tumor lysis syndrome, Acute lymphocytic leukemia
Opthalmologic Amaurosis fugax, Chorioretinitis, Glaucoma, Neuropapillitis, Optic disc drusen, Optic disc vasculitis, Optic neuritis, Orbital lesion, Orbital optic nerve tumors, Papillitis, Scleritis, Age related macular degeneration, Anterior ischemic optic neuropathy, Central retinal vein occlusion, Optic neuropathy
Overdose / Toxicity Arsenicals, Carbon monoxide toxity, Ethylene glycol
Psychiatric No underlying causes
Pulmonary Hypercapnia, Obesity hypoventilation syndrome, Respiratory acidosis
Renal / Electrolyte Acid Base Imbalance, Hypocalcemic tetany
Rheum / Immune / Allergy Sarcoidosis, Sympathetic ophthalmia, Systemic Lupus Erythematosus
Sexual No underlying causes
Trauma Head trauma
Urologic No underlying causes
Dental No underlying causes
Miscellaneous Idiopathic intracranial hypertension, POEMS syndrome, Deafness- peripheral- neuropathy- arterial disease

Causes in Alphabetical Order

References

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Differentiating Papilledema from other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Kalsang Dolma, M.B.B.S.[2]

Overview

Papilledema should be distinguished from pseudopapilledema

Differentiating of Papilledema from other Diseases

Papilledema should be distinguished from pseudopapilledema which is caused by

  • Optic disc drusen:[1] These are globules of mucoproteins and mucopolysaccharides that progressively calcify. They are thought to be the remnants of the axonal transport system of degenerated retinal ganglion cells. Optic disc drusen have also been referred to as congenitally elevated or anomalous discs, pseudopapilledema, pseudoneuritis, buried disc drusen, optic nerve head drusen and disc hyaline bodies. Ophthalmoscopy and B-scan ocular ultrasonography allow appropriate diagnosis of optic disc drusen, which mimics papilledema.[2]This is important to avoid unnecessary interventions and anxiety

References

  1. Sahin A, Cingü AK, Ari S, Cinar Y, Caça I (2012). “Bilateral optic disc drusen mimicking papilledema”. J Clin Neurol. 8 (2): 151–4. doi:10.3988/jcn.2012.8.2.151. PMC 3391621. PMID 22787500. Unknown parameter |month= ignored (help)
  2. Khonsari RH, Wegener M, Leruez S, Cochereau I, Milea D (2010). “[Optic disc drusen or true papilledema?]”. Rev. Neurol. (Paris) (in French). 166 (1): 32–8. doi:10.1016/j.neurol.2009.05.003. PMID 19540541. Unknown parameter |month= ignored (help)

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Epidemiology and Demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Kalsang Dolma, M.B.B.S.[2]

Overview

Papilledema occurs in approximately 50% of patients with a brain tumor.

References


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Natural History, Complications and Prognosis

Please help WikiDoc by adding more content here. It’s easy! Click here to learn about editing.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Papilledema regresses if the underlying cause is treated and if not, can lead to vision loss.

References


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Diagnosis

Diagnosis

History and Symptoms | Physical Examination | CT | MRI | Other Imaging Findings | Other Diagnostic Studies

Treatment

Treatment

Medical Therapy | Surgery | Primary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case Studies

Case #1

Related Chapters

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