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Incidentally Discovered NF2

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This is an MR of the brain

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in a 10-year-old child

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who was in a motor vehicle accident,

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had trauma, and afterwards, had headaches.

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Sos an MRI was performed.

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The MRI shows signs of a Chiari type 1 malformation.

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I can see there's crowding at the level of

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the foramen magnum with cerebellar tonsil ectopia.

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Now, also noted on this study,

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if we look on this high-resolution

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postcontrast image,

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we see this area of enhancement

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in the right internal auditory canal.

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And then, we also see an area of enhancement

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at the funds of the left internal auditory canal.

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This bilateral internal auditory canal

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enhancement is diagnostic of

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neurofibromatosis type 2.

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This represents bilateral vestibular schwannomas.

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Now, there's always a differential

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for enhancement.

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This is a location where patients

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with CNS tumors that are prone to leptomeningeal spread,

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you can get metastatic deposits here.

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I've seen that with ependymomas and medulloblastomas.

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However, there's no signs of that.

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So, this patient has bilateral enhancement

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in the internal auditory canals.

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But if we look closer,

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we're actually seeing, additionally,

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an enhancing lesion on the left ocular motor nerve.

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This is the Basilar artery.

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Here's the posterior cerebral artery on the left

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and we have an approximately 4 mm schwannoma

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of the left ocular motor nerve.

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So, this is a reminder that

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neurofibromatosis type 2,

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while is most characteristically described by

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bilateral vestibular schwannomas,

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actually can result in schwannomas of a variety of cranial nerves.

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In this case,

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the left oculomotor nerve.

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There are other entities seen with

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neurofibromatosis type 2,

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including meningiomas and spinal ependymomas.

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And we looked through the images to,

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for that,

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but we're not seeing anything at present.

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Again, this is just a 10-year-old child and this

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was actually an incidental finding

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in a trauma follow-up of bilateral vestibular schwannomas.

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So, this patient warrants a genetic evaluation,

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imaging surveillance,

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bilateral hearing surveillance with

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baseline audio grams and routine surveillance.

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So, and this may result in a need to screen

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siblings and other first

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degree family members.

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So, this is an incidentally noted case

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of neurofibromatosis type 2.

Report

Description

Faculty

Asim F Choudhri, MD

Chief, Pediatric Neuroradiology

Le Bonheur Children's Hospital

Tags

Syndromes

Pediatrics

Neuroradiology

Neuro

Neoplastic

MRI

Congenital

Brain

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