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This is an MRI of the brain

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in a two year old child

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with neurofibromatosis type 1.

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This was performed for routine surveillance.

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You can see an area of myelin vacuolization

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in the right globus pallidus.

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You can see myelin vacuolization in the deep

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cerebellar gray matter bilaterally,

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right greater than left.

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If we go superiorly,

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we see that this juxtacortical

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area of signal abnormality,

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this T2 hyperintense signal abnormality.

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On this T1 post contrast image,

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we see, within the area of signal abnormality,

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we see a small enhancing focus.

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So, this is concerning for a low grade glioma in

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the setting of neurofibromatosis type 1.

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But there's not a lot of mass effect,

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and there's a little bit of signal abnormality,

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which could be edema,

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but could just be myelin vacuolization.

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So in the setting neurofibromyatosis type 1,

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this enhancing area actually

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will just be followed.

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They will not typically go directly to resection

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for this, because we know some of these lesions,

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even if they're enhancing,

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may not get worse over the course of years.

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The enhancement and even the signal

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abnormality may go away.

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That's a part of the waxing and

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waning of these lesions.

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So, just immediately jumping to

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concerning findings and immediately jumping to

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surgery isn't always in the

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patient's best interest,

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especially in neurofibromatosis type 1.

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Now, in this patient with neurofibromatosis type 1,

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we also see extreme tortuosity of the

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optic nerves bilaterally.

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This is a two-year-old.

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This is very tortuous.

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That's not normal.

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And on this post contrast imaging of the orbits,

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we can actually see enhancement

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of the optic nerves.

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This subtle enhancement on the left,

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and there's enhancement on the right.

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The problem is,

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how do you know what's normal

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and what's not normal

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when you have a bilateral abnormality?

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Well,

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we can see here the optic chiasm in the optic

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tracts aren't enhancing as much as this

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segment of the right optic nerve.

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So this patient,

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while the optic nerves are not significantly enlarged,

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and on T2, we're not seeing significant

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signal abnormality.

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Given the tortuosity and the enhancement,

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this patient likely has bilateral

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optic pathway gliomas.

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So, these are the types of findings to be able to

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try and scrutinize for on every single patient

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with neurofibrimatosis type 1.