Papilledema, also known as papilloedema, is optic disc swelling that is secondary to elevated intracranial pressure.[1, 2] In contrast to other causes of optic disc swelling, vision usually is well preserved with acute papilledema. Papilledema almost always presents as a bilateral phenomenon and may develop over hours to weeks.
An intracranial mass lesion and malignant hypertension should be excluded in all patients with papilledema. It is incorrect to use the term "papilledema" to describe optic disc swelling due to primary infection, infiltration, or inflammation of the optic nerve that does not cause increased intracranial pressure.
The disc swelling in papilledema is the result of axoplasmic flow stasis with intra-axonal edema in the area of the optic disc.[3] The subarachnoid space of the brain is continuous with the optic nerve sheath. Hence, as the cerebrospinal fluid (CSF) pressure increases, the pressure is transmitted to the optic nerve, and the optic nerve sheath acts as a tourniquet to impede axoplasmic transport. This leads to a buildup of material at the level of the lamina cribrosa, resulting in the characteristic swelling of the nerve head. 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.
Early detection and identification of cause may be life saving.
Race
No racial predilection exists.
Sex
Papilledema affects both sexes equally.
Age
Papilledema can present at any age, though, during infancy, before the fontanelles close, the finding of papilledema may fail to occur despite elevated intracranial pressure.
Most symptoms in a patient with papilledema are secondary to the underlying elevation in intracranial pressure, as follows:[3, 4]
Headache: Increased intracranial pressure headaches are characteristically worse on awakening, and they are exacerbated by coughing or other type of Valsalva maneuver.
Nausea and vomiting: If the rise in intracranial pressure is severe, nausea and vomiting may occur. This eventually may be followed by a loss of consciousness, pupillary dilation, and death.
Pulsatile tinnitus
Visual symptoms often are absent, but the following symptoms can occur:
Some patients experience transient visual obscurations (graying-out of their vision, usually both eyes, especially when rising from a lying or sitting position, or transient flickering as if rapidly toggling a light switch).
Blurring of vision, constriction of the visual field, and decreased color perception may occur.
Diplopia may be seen occasionally if a sixth nerve palsy is associated.
Visual acuity may be well-preserved, except in very advanced disease.
Papilledema is sometimes found at routine examination in an asymptomatic individual.
The history should be taken, and a physical examination, including vital signs, should be performed. In particular, check the blood pressure to exclude malignant hypertension.
The patient should be evaluated for neurologic problems and febrile illness. The patient’s height and weight should be recorded when elevated body mass index with idiopathic intracranial hypertension is suspected.
Visual acuity, color vision, and pupillary examination findings should be normal. A relative afferent pupillary defect is usually absent. Since an abduction deficit secondary to a false-localizing sixth nerve palsy sometimes may be seen in association with increased intracranial pressure, check cover test in cardinal fields of gaze and check for full motility.
Frisén scale
Papilledema can be graded using the Frisén scale[5] but remains subjective, as follows:
Stage 0 is a normal optic disc.
Stage 1 papilledema is a C-shaped halo of disc edema with preservation of the temporal disc.
Stage 2 papilledema is a circumferential halo of edema on the optic disc. (The optic cup is not obscured in stage 1 or 2 papilledema but may be in higher grades of papilledema.)
Stage 3 papilledema is elevation of the optic disc with partial obscuration of one of more segments of the blood vessels at the disc margin.
Stage 4 papilledema is characterized by almost complete obscuration of major blood vessels on the optic disc.
Stage 5 papilledema is partial or total obscuration of all blood vessels on the surface of the optic disc.
Fundus examination
Fundus examination may reveal the signs below.
Early manifestations of papilledema include the following:
Disc hyperemia
Subtle edema of the nerve fiber layer can be identified with careful slit lamp biomicroscopy and direct ophthalmoscopy. This most often begins in the area of the nasal disc. A key finding occurs as the nerve fiber layer edema begins to obscure the fine peripapillary vessels.
Small hemorrhages of the nerve fiber layer are detected most easily with the red-free (green) light.
Spontaneous venous pulsations that are normally present in 80% of individuals may be obliterated when the intracranial pressure rises above 200 mm water. Therefore, though the presence of spontaneous venous pulsations is very useful to exclude papilledema (except in cases of highly variable intracranial pressure), its absence is not very helpful.
Late manifestations of papilledema include the following:
As the papilledema continues to worsen, the nerve fiber layer swelling eventually obscures the normal disc margins and the disc becomes grossly elevated.
Venous congestion develops, and peripapillary hemorrhages become more obvious, along with exudates and cotton-wool spots.
The peripapillary sensory retina may develop concentric or, occasionally, radial folds known as Paton lines. Choroidal folds also may be seen.
Chronic manifestations of papilledema include the following:
If the papilledema persists for months, the disc hyperemia slowly subsides, giving way to a gray or pale disc that loses its central cup.
With time, the disc may develop small glistening crystalline deposits (disc pseudodrusen).
Asymmetric disc edema is occasionally be seen in conditions such as idiopathic intracranial hypertension, perhaps owing to asymmetry in the size of the bony optic canals.
Blood tests usually do not contribute to the diagnosis of papilledema. If the diagnosis is in doubt, CBC count, blood sugar, angiotensin-converting enzyme, erythrocyte sedimentation rate, and syphilis serology may be helpful to look for signs of infectious, metabolic, or inflammatory diseases. Patients with cerebral venous sinus thrombosis can be tested for hypercoagulation.
Urgent neuroimaging (eg, CT scan, MRI) of the brain with contrast should be performed in an attempt to identify a CNS mass lesion.
Consider magnetic resonance (MR) venography to detect venous sinus thrombosis.
Optical coherence tomography can be used to document the elevation of the nerve fiber layer and can be used in a serial fashion.[7]
B-scan ultrasonography may be useful to rule out buried disc drusen.
Fluorescein angiography is sometimes helpful in establishing the diagnosis. Acute papilledema exhibits increased dilation of the peripapillary capillaries with late leakage of the dye. Autofluorescence may reveal disc drusen.
Visual fields should be tested. They commonly show enlargement of the blind spot. With extreme disc edema, a pseudo–bitemporal hemianopsia may be seen.
With chronic papilledema, constriction of the visual field, especially inferiorly, gradually can occur, which eventually may progress to a loss of central acuity and total blindness.
Stereo color photography
Stereo color photographs of the optic discs are useful to document changes.
If no mass lesion is detected on MRI, a lumbar puncture can be performed to assess the opening pressure of the CSF and to obtain CSF for analysis to rule out neoplastic and infectious etiologies. The lumbar puncture may provide transient headache relief, as the CSF pressure is reduced temporarily.
Therapy, whether medical or surgical, is tailored to the underlying pathological process and the progression of the ocular findings.
Specific therapy should be directed to the underlying mass lesion if present.
Patients should sleep with the head of the bed elevated. Sleep apnea, if present, should be treated.
Diuretics: The carbonic anhydrase inhibitor, acetazolamide (Diamox), may be useful in selected cases, especially cases of idiopathic intracranial hypertension. (In the presence of venous sinus thrombosis, diuretics are a relative contraindication. In this scenario, evaluation by a hematologist is recommended.)
Weight reduction is recommended in cases of idiopathic intracranial hypertension and can be curative.[8] Bariatric surgery may be considered in cases refractory to conventional methods of weight loss.[9]
Corticosteroids may be effective in cases associated with inflammatory disorders (eg, sarcoidosis).
Consider withdrawing causative medications, as weighed against other medical necessities and alternatives.
The underlying mass lesion, if present, should be removed.
Lumboperitoneal shunt or ventriculoperitoneal shunt can be used to bypass CSF.
Optic nerve sheath decompression can be used to relieve worsening ocular symptoms in cases of medically uncontrolled idiopathic intracranial hypertension. This procedure probably will not ameliorate persistent headaches if present.
Besides an ophthalmologist, a neurologist should be involved in monitoring the patient, and a neurosurgeon may be needed to help evaluate any underlying mass or to perform a shunting procedure.
The patient should be examined weekly until stabilization of the ocular findings occurs. Well-developed papilledema takes 6-10 weeks to regress, following lowering of intracranial pressure.
Diuretics may be helpful in cases of elevated intracranial pressure. Diamox and other carbonic anhydrase inhibitors can decrease the production of CSF.
Clinical Context:
The conversion of carbon dioxide to bicarbonate plays a key role in the production of both aqueous humor and CSF. Carbonic anhydrase inhibitors act by inhibiting the conversion of carbon dioxide to bicarbonate, thus inhibiting the production of both aqueous humor and CSF. Dosage should be individualized and up to 2 g per day may be required in patients with idiopathic intracranial hypertension. However, many patients cannot tolerate more than 1 g/d because of the adverse effects (eg, dizziness, metallic taste, lethargy, paresthesias). Diamox sequels may be better tolerated than tablets.
Clinical Context:
Prednisone, like other corticosteroids, can cause profound and varied metabolic and immunologic effects. Its usefulness in these cases stems from its strong anti-inflammatory properties.
May be useful in cases where inflammatory lesions lead to a secondary elevation in CSF pressure. These drugs are effective in these cases because of their potent anti-inflammatory effects.
What is papilledema?What is the pathophysiology of papilledema?What factor reduces the mortality of papilledema?Which patient groups are at highest risk for papilledema?What is the prognosis of papilledema?What are the signs and symptoms of papilledema?What should be included in the physical exam for suspected papilledema?How is papilledema staged?Which findings on fundus exam are characteristic of early papilledema?Which findings on fundus exam are characteristic of late papilledema?Which findings on fundus exam are characteristic of chronic papilledema?What causes papilledema?What are potential complications of papilledema?What are the differential diagnoses for Papilledema?What is the role of lab studies in the workup of papilledema?What is the role of imaging studies in the workup of papilledema?What is the role of perimetry in the workup of papilledema?What is the role of stereo color fundus photography in the workup of papilledema?What is the role of lumbar puncture in the workup of papilledema?What is the focus of treatment for papilledema?What is the role of surgery in the treatment of papilledema?Which specialist consultations are needed for the treatment of papilledema?When are dietary restrictions indicated in the treatment of papilledema?What is included in the long-term monitoring of patients with papilledema?Which medications are used in the treatment of papilledema?Which medications in the drug class Corticosteroids are used in the treatment of Papilledema?Which medications in the drug class Carbonic anhydrase inhibitors are used in the treatment of Papilledema?
Mitchell V Gossman, MD, Partner and Vice President, Eye Surgeons and Physicians, PA; Medical Director, Central Minnesota Surgical Center; Clinical Associate Professor, University of Minnesota Medical School
Disclosure: Nothing to disclose.
Coauthor(s)
Joseph Giovannini, MD, Chief of Ophthalmology, Eye Surgery Center, David Grant Medical Center, Travis Air Force Base
Disclosure: Nothing to disclose.
Specialty Editors
Simon K Law, MD, PharmD, Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine
Disclosure: Nothing to disclose.
Chief Editor
Edsel Ing, MD, MPH, FRCSC, Associate Professor, Department of Ophthalmology and Vision Sciences, University of Toronto Faculty of Medicine; Active Staff, Michael Garron Hospital (Toronto East Health Network); Consulting Staff, Hospital for Sick Children and Sunnybrook Hospital, Canada
Disclosure: Nothing to disclose.
Acknowledgements
Georgia Chrousos, MD Clinical Professor, Department of Ophthalmology, Division of Neuro-Ophthalmology and Pediatric Ophthalmology Services, Georgetown University Medical Center
Disclosure: Nothing to disclose.
Brian R Younge, MD Professor of Ophthalmology, Mayo Clinic School of Medicine
Brian R Younge, MD is a member of the following medical societies: American Medical Association, American Ophthalmological Society, and North American Neuro-Ophthalmology Society
Disclosure: Nothing to disclose.
References
Ehlers JP, Shah CP, eds. Papilledema. The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease. 5th ed. Baltimore, Md: Lippincott Williams & Wilkins; 2008. 252-254.
Miller NR, Newman NJ, et al, eds. Walsh & Hoyt's Clinical Neuro-ophthalmology: The Essentials. 2nd ed. Lippincott Williams & Wilkins; 2008. 122-145.
