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Optic Nerve Sheath Meningioma - Review

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Let's contrast the optic nerve glioma

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with the optic nerve meningioma.

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As you can see on this post-gad T1-weighted scan above,

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there is abnormal enhancement along the optic nerve sheath complex

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which seems to spare the optic nerve itself.

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This will be indicative of an optic nerve meningioma.

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The optic nerve sheath complex is shown to

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be expanded on the T2-weighted scan.

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And once again, we see the enhancement of the optic

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nerve sheath, but with sparing of the nerve.

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This is a T1-weighted scan showing that expansion

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of that optic nerve sheath complex.

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Again, the clinical distinction between optic nerve glioma

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and optic nerve meningioma is that optic nerve

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meningiomas have visual loss earlier in the course and

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are generally in middle-aged to adult patients,

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as opposed to optic nerve gliomas which are far and away

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more common in the pediatric population with

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neurofibromatosis type one. On this final case,

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we see optic nerve sheath enhancement.

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And in this situation, where it's not that enlarged,

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one has a differential diagnosis which includes

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lots of infectious inflammatory conditions,

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including sarcoidosis. We saw a case of leukemia.

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We have the idiopathic orbital

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inflammation of pseudotumor,

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which also can cause optic nerve sheath enhancement.

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There are collagen vascular diseases which also can

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lead to contrast enhancement of the optic

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nerve sheath. And in addition,

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one can see this with spread of ocular tumors along

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the subarachnoid space such as retinoblastoma

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or uveal melanomas.

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Here's another example of an optic nerve sheath meningioma.

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Note that on the T2-weighted fat-suppressed scan above,

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we have sparing of the optic nerve,

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but enlargement of the optic nerve sheath

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along the superioral portion.

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Here's the nerve which is similar in signal intensity

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and caliber to the contralateral side.

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But we do have this expansion of the optic nerve

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sheath itself with contrast enhancement.

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We also see sparing of the nerve,

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but the gross enlargement of the optic nerve sheath

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complex by this optic nerve sheath meningioma.

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The appearance of the optic nerve spared with

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enhancement on either side of it is called the tram track sign.

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Some people also call it the oreo cookie or hydrox cookie sign,

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and this is secondary to the meninges and the optic

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nerve sheath enhancing while the optic

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nerve is spared. Now, as I mentioned,

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there is a relatively broad differential diagnosis

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which is listed down below here,

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including all kinds of infiltrative granulomatous

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disorders as well as things that

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can spread via the subarachnoid space.

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Reckonid space as opposed to optic nerve gliomas which

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are associated with neurofibromatosis type one.

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Optic nerve meningiomas may be part of the misms

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syndrome of neurofibromatosis type two,

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which includes multiple inherited schwannomas and

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meningiomas and ependymomas. Calcification of optic

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nerve meningiomas occurs

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in about one fourth of cases.

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So this is a differential diagnosis which is quite

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broad and the vast majority of cases will

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simply be optic nerve meningiomas.

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But it sometimes behooves the clinician to perform a

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lumbar puncture and check the CSF to make sure that

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this is not a subarachnoid space lesion that

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is simulating an optic nerve meningioma.

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When one considers causes of the optic nerve sheath

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complex enlargement, it's good to separate them into

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neoplastic and non-neoplastic lesions of the tumors.

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The most common are going to be in children,

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the optic nerve glioma and in adults,

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the meningioma with leukemia, lymphoma, and metastases,

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much less common.

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When we look at non-neoplastic causes of optic nerve

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sheath complex enlargement, we're going to be seeing

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most commonly optic neuritis in association with

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multiple sclerosis or de novo or

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as part of the NMO syndrome.

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The other thing that can cause optic nerve

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sheath complex enlargement is idiopathic

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intracranial hypertension or so-called

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pseudotumor cerebri. In this case,

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the patient may have papilledema and present with severe headache.

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When we think about the coneal space,

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we're talking about the muscles within the orbit

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and they include the muscles described here.

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The medial rectus muscle, lateral rectus muscle, inferior rectus muscle,

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superior rectus muscle with the levator palpebrae

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muscle outside the muscle cone and above the superior rectus muscle.

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And we have the oblique muscle with its tendinous insertion at the trochlea.

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Here, the inferior oblique muscle is seen underneath the orbit.

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So the lesions of the muscle cone or conal lesions are relatively few.

Report

Description

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Tags

Orbit

Neuroradiology

Neuro

Neoplastic

MRI

Head and Neck

CT

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