Chronic Papilledema from Cerebral Venous Thrombosis

Chronic papilledema from superior sagittal sinus thrombosis.

In the right eye, the optic nerve is diffusely elevated with nerve fiber layer edema. There is some mild tortuosity to the retinal vessels, and the smaller retinal vessels are obscured at the margins of the optic nerve. This is characteristic of Frisén grade 3 papilledema. Just temporal to the optic nerve are very faint dark lines concentric to the optic nerve. These are retinal folds (Paton lines), which can be seen in papilledema.

In the left eye, the optic nerve is diffusely elevated with nerve fiber layer edema. There is also some mild tortuosity, but the retinal vessels do not appear obscured by the nerve fiber layer edema. This is characteristic of Frisén grade 2 papilledema.

Because idiopathic intracranial hypertension is a diagnosis of exclusion, all patients with suspected papilledema need to be worked up for secondary causes of intracranial hypertension with an MRI ± MRV brain, lumbar puncture with opening pressure, and cerebrospinal fluid (CSF) studies.

Image credit: Schiefer, Wilhelm, Hart (Eds.). Clinical Neuro-Ophthalmology - A Practical Guide. Springer: Berlin/Heidelberg/New York, 2007. Used for educational purposes.

Very Severe Papilledema

Very severe papilledema.

In the right eye, there is diffuse elevation of the optic nerve with nerve fiber layer edema. The optic nerve is hyperemic with cotton-wool infarcts, most prominently at the superior and inferior poles of the optic nerve. There are multiple flame (splinter) hemorrhages throughout the posterior pole. There are telangiectatic vessels on the optic nerve. The retinal vessels are tortuous. The retinal vessels at the optic nerve are not clearly visible at all. This is characteristic of Frisén grade 5 papilledema.

The left eye has similar descriptive features and also has Frisén grade 5 papilledema.

This case of papilledema was secondary to tetracycline use. Treatment of pseudotumor cerebri secondary to drug use after confirmation of the diagnosis includes discontinuation of the offending drug and conventional treatment for intracranial hypertension, which may include observation, weight loss, medical therapy, and/or surgical therapy. Other medications that have association with pseudotumor cerebri include other tetracyclines (minocycline, doxycycline), vitamin A toxicity (>100,000 IU/day), all-trans retinoic acid (ATRA), Accutane, synthetic growth hormone, lithium, and steroid use/withdrawal.

Image credit: American Academy of Ophthalmology. Used with permission for educational purposes.

Severe Papilledema

Severe papilledema.

The optic nerve is diffusely elevated with nerve fiber layer edema present 360°. The optic nerve is diffusely hyperemic and there are telangiectatic vessels on the surface of the nerve. There is a flame (splinter) hemorrhage on the temporal aspect of the optic nerve. The retinal vessels are dilated and tortuous, suggestive of venous congestion. The smaller retinal vessels are partially obscured at the edge of the optic nerve but the larger central retinal vessels are still visible. This is characteristic of Frisén grade 3 papilledema.

On an unrelated note, if you look carefully at the pattern of white striae in the retina you can see the different bundles of nerve fibers (arcuate, papillomacular, and nasal) as they course from the retina to the optic nerve.

Image credit: FitzGibbon EJ. EyeWiki. American Academy of Ophthalmology. Accessed 04-11-2019. Available online. Used with permission for educational purposes.

Moderate Papilledema

Moderate papilledema.

The optic nerve is diffusely elevated with involvement of the entire optic nerve. The optic nerve is hyperemic. Some of the retinal vasculature is tortuous. There are no telangiectatic vessels, cotton-wool spots, or hemorrhages. There are no retinal or choroidal folds. The retinal vessels are still clearly visible. This appearance is characteristic of Frisén grade 2 papilledema.

Image credit: FitzGibbon EJ. EyeWiki. American Academy of Ophthalmology. Accessed 04-11-2019. Available online. Used with permission for educational purposes.

Mild Papilledema

Mild papilledema.

The margins of the optic nerve are still visible but there is nerve fiber layer edema (whitish feathery halo) more prominently seen superiorly, inferiorly, and nasally. There are no hemorrhages or cotton-wool spots. The retinal vasculature is intact and visible. There are no choroidal or retinal folds. Because the temporal margin of the optic nerve is still intact, this would fall in the category of Frisén grade 1 papilledema.

Image credit: FitzGibbon EJ. EyeWiki. American Academy of Ophthalmology. Accessed 04-11-2019. Available online. Used with permission for educational purposes.

Severe Papilledema

Papilledema in pseudotumor cerebri.

The right eye demonstrates diffuse nerve fiber layer edema (white feathery appearance) and there is diffuse elevation of the optic nerve. Telangiectatic vessels lie on the surface of the optic nerve (one more prominently seen temporally). The major retinal vessels are obscured by the thickened nerve fiber layer centrally but not completely obliterated by the edema. There are no obvious retinal or choroidal folds. This is characteristic of Frisén grade 4 papilledema.

The left eye demonstrates diffuse nerve fiber layer edema with diffuse elevation of the optic nerve. The disc is hyperemic and there are no obvious telangiectatic vessels. Nasally, there is a thin and faint whitish vertical/circumferential line that most likely indicates the extent of subretinal fluid emanating from the optic nerve. Some of the smaller retinal vessels are obscured by the thickened nerve fiber layer, but the largest retinal vessels remain completely visible. This is characteristic of Frisén grade 3 papilledema.

Image credit: Hoyt WF. Bilateral papilledema with pseudotumor cerebri. Neuro-Ophthalmology Virtual Education Library. University of Utah, 2012. Available online. Used for educational purposes.

B-Scan Ultrasound in Papilledema

B-scan ultrasound in papilledema.

A. Diameter of the optic nerve sheath in a normal person.

B. Diameter of the optic nerve sheath in a patient with IIH.

C. Diameter of the optic nerve sheath in the same patient as (B) following lumbar puncture with therapeutic removal of CSF.

Image credit: Bäuerle J, Nedelmann M. B-mode sonography of the optic nerve in neurological disorders with altered intracranial pressure. Perspectives in Medicine 2012;1:404-407. Available online.

30 Degree Test For Papilledema

30° test.

A-scan (figure) or B-scan (not shown) ultrasonography is used to measure the optic nerve sheath diameter in primary (left) and eccentric gaze (right). A reduction in the nerve sheath diameter >20% is considered a positive result, strongly suggesting increased fluid around the optic nerve.

Image credit: Wang R, Kini A, Al Othman B, Lee AG. Pseudotumor cerebri and papilledema: an overview of this perplexing syndrome and its hallmark presentation. Glaucoma Today. 2019 Jan/Feb:34-46. Available online.

MRI Findings in Papilledema

Empty Sella

T1 sagittal MRI showing empty sella.

Flattening of Posterior Globe

T2 axial MRI showing protrusion of the optic nerve head and flattening of posterior globe.

Increased CSF in Perioptic Space

T2 axial and coronal MRI showing dilation of optic nerve sheaths and increased CSF in perioptic space.

Narrowed Distal Transverse Venous Sinuses

MRV showing narrowing of right transverse venous sinus.

Image credit: Degnan AJ, Levy LM. Pseudotumor cerebri: brief review of clinical syndrome and imaging findings. AJNR Am J Neuroradiol 2011;32:1986-1993. Available online.

Visual Field in Papilledema

The prototype visual field defect in patients with IIH and mild visual field loss.

The inferior nasal arcuate defect is the prototypical visual field defect in IIH. The arcuate pattern is better seen in the Total Deviation plot.

Image Credit: Wall M. Update on Idiopathic Intracranial Hypertension. Neurologic Clinics 2017;35:45-57. Available online.

Fluorescein Angiography in Papilledema

Fluorescein angiography of the left eye with papilledema.

Note the progressive increasing hyperfluorescence of the optic nerve with enlarging and blurring of the hyperfluorescent area, consistent with leakage.

Image credit: Mollan SP, Markey KA, Benzimra JD, et al. A practical approach to diagnosis, assessment and management of idiopathic intracranial hypertension. Practical Neurology 2014;14:380-390. Available online.

Chronic Papilledema

Chronic Papilledema.

A, B: Optic discs in chronic papilledema, with the development of refractile bodies (arrows) representing lipid exudates from chronic microvascular leakage.

C, D: Visual field patterns confirm the presence of mild diffuse depression in sensitivity and superior and inferior arcuate defects.

Image credit: Parts A, B courtesy of Anthony C. Arnold, M.D.; parts C, D courtesy of Steven A. Newman, M.D. American Academy of Ophthalmology. Used with permission for education purposes.

Chronic Papilledema

Chronic papilledema.

The optic nerves remain elevated with blurred margins, with feathering of the retinal nerve fiber layer. The nerve can become more atrophic and pale. Retinochoroidal (optociliary) shunt vessels can form due to the chronic outflow obstruction (black arrow in left image). Refractile bodies representing lipid exudates can also be seen (black arrow in right image).

Image credit: Acheson JF. Idiopathic intracranial hypertension and visual function. Br Med Bull. 2006;79-80:233-244. Used for educational purposes.

Papilledema (video): Lecture from Dr. Andrew Lee, M.D.

Video credit: Lee AG. Papilledema. Video. YouTube. Available online. Accessed 04-10-2019.

Papilledema with Pseudopapilledema Features

Papilledema with pseudopapilledema features.

Papilledema is optic nerve edema secondary to increased intracranial pressure. The optic nerves are hyperemic, with whitish opacification of the peripapillary retinal nerve fiber layer. Telangiectatic vessels are seen on the temporal optic disc in the left eye. In severe cases of papilledema the retinal vasculature will become obscured by the nerve fiber layer edema.

In this case, the appearance of the optic nerve is confounded by yellowish refractile bodies on the nasal aspect of both optic nerves and the anomalous branching appearance of the retinal vessels in the left optic nerve. There is some nerve fiber layer whitening most pronounced in the superior aspect of the right optic nerve, and the retinal vessels are not obscured.

Differentiating papilledema from pseudopapilledema is extremely difficult based on clinical appearance alone. The presence of pseudopapilledema does not exclude papilledema. Ancillary testing that may help differentiate between papilledema and pseudopapilledema include OCT, autofluorescence, fluorescein angiography, and ultrasound. In cases where the diagnosis remains in doubt clinically, the patient should be counseled regarding whether to proceed with further workup for intracranial hypertension (MRI/MRV brain, LP with opening pressure, CSF studies) or to observe. This is often guided by the patient’s symptoms and the clinical suspicion for increased intracranial pressure.

Image credit: American Academy of Ophthalmology. Used with permission for educational purposes.