CASE REPORT
A 40-year-old female presented with complaints of sudden, painless visual disturbances in the right eye. A mastectomy was performed six years ago due to a breast carcinoma, which required radiation therapy.
Visual acuity was tested on the right eye by counting fingers from 1 meter away and on the left eye 0,9-1,0 by Snellen. Intraocular pressure was 18 mmHg in the right eye and 16 mm Hg in the left eye. A slit lamp biomicroscopy showed a normal appearance.
A posterior segment examination showed a swollen, enlarged, and blurred optic disc, with a dilated capillary net. The whole posterior segment was covered with a golden-yellow lesion on the right eye.
The optic disc in the left eye had a normal appearance but there were yellow, oval-shaped lesions (similar to that in the right eye) present in the posterior pole temporally area. FA showed dilated capillaries of the optic disc with leaking in the early phase and diffuse hyper fluorescence that continued during the late phase.
Large caliber intralesional vessels, relatively hyperfluorescent in the early phase of angiography, and diffuse hyperfluorescent in the late phase of angiography were presented together with the leaking from the whole posterior pole.
The ultrasonography B-scan (USG) showed a prominent optic nerve with a widened optic nerve sheath. The OCT demonstrated marked optic disc swelling.
Papilledema disease entity
Papilledema is a term that is exclusively used when disc swelling is secondary to increased intracranial pressure (ICP). It must be distinguished from optic disc swelling from other causes which are simply termed “optic disc edema”.
Papilledema must also be distinguished from pseudo-papilledema such as optic disc drusen. Since the root cause of papilledema is increased intracranial pressure (ICP) this is an alarming sign which may presage such entities as brain tumors, CNS inflammation, cerebral venous thrombosis, or idiopathic intracranial hypertension (IIH).
As noted above, papilledema is due to increased intracranial pressure from any cause. Normal ICP is typically less than 250 mm of water in adults when measured with a manometer and is less than 280 mm of water in children.
Papilledema results from orthograde axoplasmic flow stasis at the optic nerve head leading to edema of the nerve from the increased intracranial pressure pressing on the nerve behind the eye. Continued pressure can result in loss of axons and eventual optic atrophy secondary to intraneuronal ischemia ultimately causing vision loss.
With optic atrophy, there is little or no edema seen even with continuing increased intracranial pressure since dead fibers don’t swell.
Increased intracranial pressure can be caused by five patho-mechanisms:
- When the skull is too small for the brain (e.g. craniosynostosis)
- When the brain volume becomes too large for the skull such as a space-occupying lesion (e.g. tumor, hemorrhage), or brain edema ( e.g. trauma)
- When there is obstruction of the cerebrospinal fluid (CSF) flow (e.g. colloid cyst obstructing the foramen of Monroe)
- Increased production of CSF (e.g. choroid plexus papilloma)
- Reduced absorption of CSF (e.g. meningitis, cerebral venous thrombosis)
The time course for the development of papilledema may be weeks if there is only a slow and mild rise in intracranial pressure, but severe and rapid changes in pressure can cause papilledema to present within a few hours to a day.
The mechanism of Idiopathic intracranial hypertension is not clearly understood however proposed mechanisms include increased venous sinus pressure from venous sinus stenosis, and reduced absorption to name a few.
Fundus Explorer Pro
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From Choroida — the team behind this sitePapilledema MANAGEMENT
Management goals are:
(1) Treating the underlying cause, (2) saving vision, and (3) symptomatic relief (e.g. headache):
- treating the underlying cause
- If malignant hypertension was found, then the patient must be sent to the emergency department for further management. Even with malignant hypertension cases, the patient with papilledema should still proceed with neuro-imaging
- If a mass is present, primary therapy should be directed toward that lesion (e.g., surgery)
- If acute cerebral venous thrombosis was seen, the patient must be sent to the thrombosis team immediately. It is best to avoid acetazolamide in cerebral venous thrombosis as it can precipitate the condition, however, it can later be added on when the patient has started on anti-coagulation
- Medications (tetracyclines, vitamin A analogs, etc.) are felt to be causative so they should be discontinued.
- saving vision & 3. symptomatic relief (e.g. headache)
- treat the underlying cause
- Medications to lower ICP
Document what you see
Two smartphone imaging tools built for everyday clinic use — one for the slit lamp, one for the fundus.
From Choroida — the team behind this siteREFERENCES
- Lee A, Rigi M, Al marzouqi S, Morgan M. Papilledema: epidemiology, etiology, and clinical management 2015;7-47.
- Keira A Markey, Susan P Mollan, Rigmor H Jensen, Alexandra J Sinclair. Understanding idiopathic intracranial hypertension: mechanisms, management, and future directions.Lancet Neurol. 2016 Jan;15(1):78-91. doi: 10.1016/S1474-4422(15)00298-7. Epub 2015 Dec 8. PMID: 26700907DOI: 10.1016/S1474-4422(15)00298-7.
- Friedman DI, Liu GT, Digre KB. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 2013 Sep 24;81(13):1159-65. doi: 10.1212/WNL.0b013e3182a55f17. Epub 2013 Aug 21. PMID: 23966248.
- Jacks A, Miller N. Spontaneous retinal venous pulsation: etiology and significance. J Neurol Neurosurg Psychiatry. 2003;74:7–9.
- Reference: Frisen L. Swelling of the optic nerve head: A staging scheme. J Neurol Neurosurg Psychiatry 1982; 45:13-18.
Test yourself
A few questions straight from this article.
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According to the article, when is the term papilledema reserved?
Papilledema is used exclusively for disc swelling secondary to increased intracranial pressure; disc swelling from other causes is simply termed optic disc edema. -
Which pseudopapilledema does the article say must be distinguished from papilledema?
Papilledema must be distinguished from pseudopapilledema such as optic disc drusen, as well as from optic disc edema due to other causes. -
What normal intracranial pressure limits does the papilledema article give for adults and children?
Measured with a manometer, normal ICP is typically below 250 mm of water in adults and below 280 mm of water in children. -
What mechanism produces optic nerve head swelling in papilledema?
Raised intracranial pressure pressing on the nerve behind the eye causes orthograde axoplasmic flow stasis at the optic nerve head, which leads to edema of the nerve. -
Why may disc edema be minimal in long-standing papilledema despite persistently raised intracranial pressure?
Continued pressure causes axon loss and optic atrophy from intraneuronal ischemia; once atrophy sets in there is little or no edema because dead fibres don't swell. -
Among the causes of papilledema, by which mechanism does a choroid plexus papilloma raise intracranial pressure?
Of the five mechanisms of raised ICP listed, choroid plexus papilloma is the example of increased CSF production, whereas meningitis and cerebral venous thrombosis reduce absorption. -
How quickly can papilledema appear after a severe, rapid rise in intracranial pressure?
A slow, mild rise in pressure may take weeks to produce papilledema, but severe and rapid changes can cause it to present within a few hours to a day. -
A patient with papilledema is found to have malignant hypertension. What does the article advise about neuro-imaging?
Malignant hypertension warrants referral to the emergency department, but a patient with papilledema should still proceed with neuro-imaging. -
In papilledema from acute cerebral venous thrombosis, when may acetazolamide be added?
Acetazolamide is best avoided in acute cerebral venous thrombosis because it can precipitate the condition, but it can be added once the patient has started anticoagulation. -
Which causative medications does the papilledema article advise discontinuing?
Medications such as tetracyclines and vitamin A analogues are felt to be causative and should be discontinued as part of treating the underlying cause.
