Papilledema Clinical Presentation

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]}