Papilledema

Papilledema: Excerpt from The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter

Richard C. Mauer

Papilledema is optic disc swelling produced by increased intracranial pressure. The diagnostic assessment of papilledema is critical in that the underlying cause can range from a subarachnoid hemorrhage to a totally benign optic disc head drusen, giving a pseudopapilledema-type picture (1).

Approach

In evaluating papilledema, take care first to determine primarily if there is true disc edema or only pseudopapilledema (2).

 A. True papilledema must have increased intracranial pressure. A list of the most common underlying causes can be extensive. The most often cited causes include metastatic intracranial tumor, aquaductal stenosis, pseudotumor cerebri (often in young, overweight females), subdural hematoma, subarachnoid hemorrhage, arteriovenous malformations, brain abscess (often with high fevers), meningitis (with fever, stiff neck, headache), encephalitis (often with mental status changes), and sagittal sinus thrombosis.

 B. Pseudopapilledema is optic nerve head elevation caused by hyaline deposition within the optic nerve head itself. An elevated nerve exists, but not true disc edema. The vessels will have an anomalous branching pattern and tiny hyaline deposits can be seen in the optic nerve head ophthalmoscopically.

 C. Disc swelling without increased intracranial pressure (3,4)

1. Optic neuritis. An afferent pupillary defect exists along with decreased vision and pain on extraocular movement. Color vision will be decreased in this normally unilateral condition.

 2. Malignant hypertension. Blood pressure is markedly elevated here. The eye findings are characteristic: bilateral prominent disc edema, flame hemorrhages that extend peripherally, and cotton wool spots (Chapter 7.8).

 3. Central retinal vein occlusion is a unilateral disc swelling with very prominent flame and blot hemorrhages, without elevated increased blood pressure.

 4. Anterior ischemic optic neuropathy. Arteritic versus nonarteritic type: usually, in the arteritic type is found headache, stiff neck, temporal tenderness, jaw claudication, elevated sedimentation rate, and severe visual loss in one eye followed by visual loss of the other eye in 60% of cases. In nonarteritic, typically no symptoms are present except decreased vision. Associated systemic findings include systemic hypertension, diabetes mellitus, or collagen vascular disorders.

5. Infiltration of the optic nerve. Tuberculosis granuloma, leukemic infiltrate, sarcoidosis, and metastatic disease are the more common examples of infiltrative processes. The infiltration can be unilateral or bilateral and can lead to rapid loss of vision. Radiation therapy can be helpful to preserve vision.

 6. Leber’s optic neuropathy usually affects men in the second or third decade. This is unilateral progressive loss of vision with disc swelling.

 7. Diabetic papillitis is an ischemic infarction to the nerve in advanced diabetics. Often this is bilateral and causes mild disc elevation (Chapter 14.1).

History

 Headache, nausea, vomiting, diplopia, and transient loss of vision lasting seconds, especially with the head in dependent positions, raise the index of suspicion for increased cranial pressure. Mood swings can be present in cases with prolonged increased intracranial pressure. Rarely is a decrease in visual acuity seen in increased intracranial pressure; if truly present, then it would suggest other causes (e.g., vein occlusion, anterior ischemic optic neuropathy, and optic neuritis). The red flags in the history include true binocular diplopia with increasing headache, disorientation, and nausea and vomiting.

Physical examination

A focused physical examination should include vital signs, such as blood pressure. Examine the head: check for neck stiffness, temporal artery tenderness, pain in and around the eyes, and pain on ocular rotations, such as occurs in optic neuritis. Afferent pupillary defect is another red flag that almost always signifies an ocular cause of disc edema, retinal vein occlusion, anterior ischemic optic neuropathy, or optic neuritis. Always examine both eyes. Normally papilledema is bilateral, but can be present asymmetrically. In true disc edema, nerve fiber layer swelling is seen, which obscures the margins of the blood vessels. Tiny splinter hemorrhages will be seen in and around the optic nerve. If the other eye has no disc swelling, look for spontaneous venous pulsations (SVP). If these SVPs are present, there is normal intracranial pressure, therefore, no true papilledema. Very prominent retinal hemorrhages suggest malignant hypertension or central retinal vein occlusion, rather than papilledema. Disc elevation can be measured using the diopteric overcorrection in the direct ophthalmoscope. Basically, focus on the retina and add in plus (red) power until the optic nerve blurs. Three diopters equals 1 mm of elevation. Ocular rotations are limited in both third and sixth nerve palsy. Sixth nerve palsies show limited lateral gaze and third nerve palsies have limitation in medial gaze, elevation, and depression. When ptosis and a dilated pupil are seen, suspect an aneurysm at the posterior communicating artery in the circle of Willis as the underlying cause. Decreased visual acuity is another red flag and normally is only mildly depressed in true papilledema. If the vision is decreased severely, look for other causes that are not related to increased intracranial pressure.

Testing

A. Laboratory studies. If disc edema is found, suggested laboratory tests include sedimentation rate and C-reactive protein for temporal arteritis; white blood count to rule out underlying infection and leukemic infiltration of the optic nerve; a computed tomography (CT) or magnetic resonance imaging (MRI) scan to rule out a compressive lesion; cerebrospinal fluid (CSF) examination for signs of meningitis, tumor, or hemorrhage only after ruling out a compressive lesion with a scan.

B. Diagnostic imaging. If true papilledema is suspected, diagnostic imagining is mandatory. A CT scan with and without contrast should be ordered, possibly followed by MRI and MRI angiography, if the CT scan is inconclusive. MRI will be particularly helpful in imaging brainstem and cerebellar lesions, which can obstruct CSF flow. Despite the greater cost of the MRI and the greater specificity of intracranial pathology, the CT scan still is the preferred technique to image acute bleeding intracranially.

Diagnostic assessment

The critical workup in papilledema includes an accurate history and assessment of the visual system. Vision is often not significantly impaired in true papilledema; if present, it would suggest seeking other causes. An afferent pupillary defect indicates a localized optic nerve or retinal condition as the cause. A detailed examination of the optic nerve to look for optic nerve drusen or pseudopapilledema is also important. The finding of spontaneous venous pulsation indicates normal intracranial pressure and no imaging is mandatory. However, if absent, this does not rule out normal intracranial pressure. No imaging is mandatory; however, if needed, a lumbar puncture should be performed following imaging. Treatment should be directed toward the underlying cause of the elevated intracranial pressure.

References

1. Gordon RN, Burde RM, Slamovits T. Asymptomatic optic disc edema. J Neuroophthalmol 1997;17(1):29–32.

2. Hedges TR. Bilateral visual loss in a child with disc swelling. Surv Ophthalmol 1992;
36(6):424–428.

3. Moster ML. Unilateral disk edema in a young woman. Surv Ophthalmol 1995;39(5):
409–416.

4. Wall M. Optic disc edema with cotton-wool spots. Surv Ophthalmol 1995;39(6):
502–508.

Book Source Details

• Book Title: The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter
• Author(s): Robert B. Taylor (editor)
• Year of Publication: 2000
• Copyright Details: The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter, Copyright © 2000 Lippincott Williams & Wilkins.

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More About This Book: Title: The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter Authors: Robert B. Taylor (editor) Publisher: Lippincott Williams & Wilkins Copyright: 2000 ISBN: 0-78172-094-X

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