Papilledema

Papilledema, also known as papilloedema, is optic disc swelling that is secondary to elevated intracranial pressure.[1, 2, 3] In contrast to other causes of optic disc swelling, vision usually is well preserved with acute papilledema.[1] Papilledema almost always presents as a bilateral phenomenon and may develop over hours to weeks.[4]

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.[1, 4]

The disc swelling in papilledema is the result of axoplasmic flow stasis with intra-axonal edema in the area of the optic disc.[1, 4, 5]  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.

Frequency

United States

Rare

International

Rare

Mortality/Morbidity

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.

The visual prognosis is generally good if the intracranial pressure is controlled.

Most symptoms in a patient with papilledema are secondary to the underlying elevation in intracranial pressure, as follows[1, 5, 6] :

• 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.

• Inquire about potential causative medications.

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.[4]

Frisén scale

Papilledema can be graded using the Frisén scale[7] but remains subjective, as follows[4] :

• 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[4] :

• 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[4] :

• 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[4] :

• 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.

Potential causes include the following[4] :

• Any tumors or space-occupying lesions of the CNS

• Idiopathic intracranial hypertension (also known as pseudotumor cerebri)[8]

• Decreased CSF resorption (eg, venous sinus thrombosis, inflammatory processes, meningitis, subarachnoid hemorrhage)

• Increased CSF production (tumors)

• Obstruction of the ventricular system

• Cerebral edema/encephalitis

• Craniosynostosis

• Medications, for example, tetracycline, minocycline, lithium, Accutane, nalidixic acid, and corticosteroids (both use and withdrawal)

Unrelenting papilledema may eventually lead to permanent blindness.[1, 4]

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.[1] Patients with cerebral venous sinus thrombosis can be tested for hypercoagulation.[4]

Urgent neuroimaging (eg, CT scan, MRI) of the brain with contrast should be performed in an attempt to identify a CNS mass lesion.[1, 4]

Consider magnetic resonance (MR) venography to detect venous sinus thrombosis.[4]

Optical coherence tomography can be used to document the elevation of the nerve fiber layer and can be used in a serial fashion.[9]

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.[4]

Perimetry

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.[4]

Stereo color photography

Stereo color photographs of the optic discs are useful to document changes.[4]

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.[4]

Therapy, whether medical or surgical, is tailored to the underlying pathological process and the progression of the ocular findings.[1, 4]

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.[4, 10]   Bariatric surgery may be considered in cases refractory to conventional methods of weight loss.[11]

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.

Dietary restrictions and consultation with a dietitian in case of idiopathic intracranial hypertension is recommended.

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.

Class Summary

Can be used in selected cases because they decrease the production of CSF and, thus, lower intracranial pressure.

Acetazolamide (Diamox, Diamox Sequels)

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.

Class Summary

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.

Prednisone (Deltasone)

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.