Papilledema

Papilledema
Papilledema. Image courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology
ICD-10	H47.1
ICD-9	377.0
DiseasesDB	9580
MeSH	D010211

Papilledema
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Overview
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Classification
Pathophysiology
Causes
Differentiating Papilledema from other Diseases
Epidemiology and Demographics
Natural History, Complications and Prognosis
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Template:Search infobox Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Kalsang Dolma, MBBS

Synonyms and Keywords: papilloedema

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. Papilledema is mostly seen as a symptom resulting from another pathophysiological process.

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.

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.

Common Causes

Hydrocephalus

Cerebral edema

Intracranial bleeding

Brain tumor

Cerebral venous sinus thrombosis

Idiopathic intracranial hypertension

Meningitis

Choroid plexus neoplasm

Hypertension

Causes by Organ System

Cardiovascular	Hypertension, Hypertensive encephalopathy, Hypertensive retinopathy
Chemical / poisoning	Methanol, Ethylene glycol
Dermatologic	No underlying causes
Drug Side Effect	Cisplatin, Corticosteroid, Growth hormone treatment, Insulin-like growth factor 1, Interleukin 11, Isotretinoin, Lithium, Minocycline, 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

Acid Base Imbalance Acute liver failure Acute lymphocytic leukemia Adult low grade infiltrative suoratentorial astrocytoma Age related macular degeneration Amaurosis fugax Anterior ischemic optic neuropathy Arachnoid cyst Arnold chiari malformation Arsenicals Brain Abscess Brain cyst Brain Stem Gliomas Brain tumor Brown - symmers disease Brucellosis Camurati-Engelmann disease Carbon monoxide toxity Cavernous Sinus Thrombosis Central retinal vein occlusion Cerebellar abscess Cerebral abscess Cerebral edema Cerebral venous sinus thrombosis Chondroma Chorioretinitis Choroid plexus neoplasm Cisplatin Corticosteroid Crouzon syndrome Cushing's disease Dandy-Walker syndrome Deafness- peripheral- neuropathy- arterial disease Diabetic retinopathy Eclampsia Epidural abscess Epidural hematoma Ethylene glycol Fabry's Disease Foster Kennedy syndrome Glaucoma Growth hormone treatment Gullian-Barre syndrome Head trauma Hydrocephalus Hypercapnia Hypertension

Hypertensive encephalopathy Hypertensive retinopathy Hypocalcemic tetany Idiopathic intracranial hypertension Infective endocarditis Insulin-like growth factor 1 Interleukin 11 Intracranial bleeding Isotretinoin Lithium Malignant Astrocytomas Medulloblastoma Meningitis Meningoencephalitis Methanol Minocycline Myoclonic epilepsy Neuroectodermal tumor primitive Neuropapillitis Nutritional optic neuropathy Obesity hypoventilation syndrome Optic disc drusen Optic disc vasculitis Optic neuritis Optic neuropathy Orbital lesion Orbital optic nerve tumors Osteoma Paget's disease Papillitis Paragonimiasis Parinaud's syndrome POEMS syndrome Respiratory acidosis Sarcoidosis Scleritis Subarachnoid hemmorhage Subdural empyema Subdural hematoma Sympathetic ophthalmia Syphillis Systemic Lupus Erythematosus Tetracycline Thyroid opthalmopathy Toxic optic neuropathy Toxoplasmosis Tuberous sclerosis Tumor lysis syndrome Vogt-Koyanagi-Harada syndrome

Differentiation of Papilledema from other Disorders

Papilledema should be distinguished from pseudopapilledema which is caused by

• Optic disc drusen
• Tilted optic disc

Epidemiology and Demographics

Papilledema occurs in approximately 50% of those with a brain tumour.

Diagnosis

Checking the eyes for signs of papilledema should be carried out whenever there is a clinical suspicion of raised intracranial pressure. Because of the (rare) possibility of a brain tumor, pseudotumor cerebri or cerebral venous sinus thrombosis, all of which can increase intracranial pressure, this examination has become common for patients suffering from headaches.

Physical Examination

Eyes

Images shown below are courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

• 

  Papilledema.

• 

  Pseudopapilledema.

There are 10 hallmarks of papilledema:

• Blurring of the disc margins
• Filling in of the optic disc cup
• Anterior bulging of the nerve head
• Edema of the nerve fiber layer
• Retinal or choroidal folds
• Congestion of retinal veins
• Peripapillary hemorrhages
• Hyperemia of the optic nerve head
• Nerve fiber layer infarcts
• Hard exudates of the optic disc

References

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