30 Degree Test For Papilledema

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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

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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

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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

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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

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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

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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 with Pseudopapilledema Features

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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.

The Oculosympathetic Pathway

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The oculosympathetic pathway.

The oculosympathetic pathway is important for localizing lesions that may occur along its path. The common pathway consists of the first-order (central) neuron originating from the hypothalamus, descending down the brainstem and cervical spinal cord to the ciliospinal center of Budge-Waller at the level of C8-T2. The second-order (preganglionic) neuron then exits the spinal cord, travels around the apex of the lung, and travels up to the superior cervical ganglion, which is at the level of the jaw/carotid bifurcation. The third-order (postganglionic) neuron then branches to innervate the facial sweat glands, iris dilator muscle, and Müller and inferior tarsal muscles.

Image credit: Illustration by Christine Gralapp. American Academy of Ophthalmology. Used with permission for educational purposes.

Pharmacologic Testing for Horner Syndrome (video): Lecture from Dr. Andrew Lee, M.D.

Video credit: Lee AG. Pharmacologic Testing for Horner Syndrome. Video. YouTube. Available online. Accessed 02-27-2019.

Dilation Lag (video)

Two examples of dilation lag (Horner's syndrome). In the first example, the right pupil dilates much faster than the left pupil when the light is turned out. In the second example, the left pupil dilates much faster than the right pupil when the light is turned out.

Video credit: Digre KB, Jacobson D, Balhorn R. Dilation Lag. Video. [Neuro-Ophthalmology Virtual Education Library: NOVEL Web Site]. 2005. Available online. Accessed 02-27-2019.

Horner Syndrome

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Right Horner syndrome.

Note the anisocoria (right pupil smaller than left pupil) and right upper lid ptosis. There is not a prominent right lower lid ptosis present.

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

Horner Syndrome

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Right Horner syndrome.

Note the anisocoria (right pupil smaller than left pupil), and right upper eyelid ptosis. Right lower eyelid ptosis is not as pronounced in this case.

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

Horner Syndrome

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Right Horner syndrome.

Note the anisocoria (right pupil smaller than left pupil) and right upper and lower ptosis. In a child, the most common cause of congenital Horner syndrome is birth trauma to the brachial plexus, with acquired childhood Horner syndrome concerning for neuroblastoma. Apraclonidine should be avoided in small children due to the risk of CNS and respiratory depression.

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

Horner Syndrome

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Left Horner syndrome.

Note the anisocoria (right pupil larger than left pupil) suggesting miosis of the left pupil. There is left upper lid ptosis and a very slight left lower lid ptosis, consistent with paralysis of the Müller and inferior tarsal muscles, respectively. There is also some mild increased conjunctival injection in the left eye, which can occur due to the disruption of sympathetic innervation to the periocular blood vessels.

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

Hydroxyamphetamine Test

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Hydroxyamphetamine test for Horner syndrome.

A. Before drops administered (suspected right Horner syndrome).

B. After drops administered. Note the dilation of both pupils. This indicates an intact 3rd-order, postganglionic neuron and localizes the lesion to the 1st-order (central) or 2nd-order (preganglionic) neuron.

Image credit: Modified from clinical images courtesy of Lanning B. Kline, M.D. American Academy of Ophthalmology. Used with permission for educational purposes.

Apraclonidine Test

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Apraclonidine test for Horner syndrome.

A. Before drops administered (suspected left Horner syndrome).

B. After drops administered. Note the slight “reversal of anisocoria” in the left eye and the resolution of ptosis.

Image credit: Kanagalingam S, Miller NR. Eye Brain 2015;7:35-46. Available online. Used under the Creative Commons Attribution - Non Commercial (unported, v3.0) License.

Cocaine Test

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Cocaine test for Horner syndrome.

A. Before drops administered (suspected right Horner syndrome).

B. After drops administered. Note that there is some pupil dilation in the right eye, but the amount of anisocoria is ≥1 mm.

Image credit: Kanagalingam S, Miller NR. Eye Brain 2015;7:35-46. Available online. Used under the Creative Commons Attribution - Non Commercial (unported, v3.0) License.