Use term 'papilledema' with caution to avoid unnecessary testing

August 1, 2004

Papilledema is a term that is best reserved for optic disc edema that is due to increased intracranial pressure. All other forms of optic disc swelling should probably be referred to as optic disc edema to avoid clinical confusion with the more specific term, papilledema. Ophthalmologists should therefore be cautious about using the term papilledema when they really mean ischemic optic neuropathy or optic neuritis. This is especially important when referring patients to other consultants (for example, neurology) because these patients may end up with inappropriate, expensive, or unnecessary testing.

Patients with papilledema often complain of headache that may be worsened by maneuvers that increase intracranial pressure, such as cough or Valsalva's maneuver. There may be associated nausea and vomiting. Pulse-synchronous tinnitus, presumably related to intermittent venous sinus compression, may also be present. Local ischemia due to the optic disc edema may produce transient visual obscurations lasting seconds at a time. Diplopia may occur due to a non-localizing sixth-nerve palsy associated with increased intracranial pressure.

Typical clinical featuresClinically, the typical features of papilledema usually allow differentiation from other causes of optic disc edema. The central visual acuity is usually spared in papilledema, as opposed to most other optic neuropathies in which the visual acuity is typically decreased (for example, optic neuritis, ischemic or inflammatory optic neuropathy). Papilledema may produce visual acuity loss if associated with macular hemorrhage or subretinal neovascular membrane formation, concomitant ischemic optic neuropathy, or choroidal folds.

The ophthalmologist's role is to recognize papilledema and refer appropriately. A semi-urgent neuroimaging study, usually magnetic resonance (MR) imaging, often with contrast-enhanced MR venography, is recommended. If the imaging is negative, then a lumbar puncture with opening pressure should be performed. Pseudotumor cerebri (idiopathic intracranial hypertension) is a diagnosis of exclusion (negative neuroimaging and spinal fluid analysis with elevated intracranial pressure).

Ophthalmologists should recognize that patients with papilledema who present with acute or progressive visual loss should be referred for consideration for surgical decompression (for example, optic nerve sheath fenestration or shunting procedure).OT