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Wk 1, Case 5 - Review

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So this was probably the hardest one. Okay. And so I couldn't make 'em,

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couldn't make it easy for everyone. He had to throw a couple of hard cases in.

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But I think that what I, in the discussion that I gave, I, I,

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I remember looking at this case and I,

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and I think the patient came in with vision loss or something,

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or headaches or something like that.

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And I think what it does help me do is really refine my search pattern.

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So when I'm looking at re a regular brain, Mr. Um, you know, like most men,

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I think I have attention deficit disorder. My,

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my brain kind of goes from one thing to another thing.

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So what I do is when I look at something, I,

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I have to be very structured in my reports.

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And so in the sense that I try to keep my reports as consistent as possible.

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And anytime I look at any study,

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whether it's a brain M r I or even a skull-based m R I or a neck m r i and they

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actually have a sagal images,

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the first thing where I go to is I look at the sagal images and I go through the

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midline structures.

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So the first structure I look at is I look at the corpus Callum,

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then I look at the optic chiasm, then I look at the, the, uh, pituitary gland,

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then I look at the pineal region.

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Then I look at the tip of the cerebellum to make sure that there's no, uh,

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chiari malformation. So in this particular case, if you do your search pattern,

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then the abnormality becomes obvious.

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And that is the diffusely thickened optic chiasm.

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So this optic chiasm is way too thick. Um,

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and when you look at your next brain MRIs,

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you'll be able to really appreciate how thick that chiasm is.

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And then when we looked at the other sequences here,

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and when you look at the flare sequence, and I'll narrow it down a little bit,

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then what you start to notice is that if you look at the post, the, uh,

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post chias asthmatic segment of the optic chiasm, you can see it's bright.

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And now you start seeing these areas of increased signal involved in the medial

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portion of the right cerebellar p dunkle.

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And then when you look a bit a little bit lower,

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you can see this abnormal signal here involving the right lateral aspect of the

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pons.

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So when you put this diffuse enlargement of the optic

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nerve and you sort of combine it with all of these lesions that you end up

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seeing these areas of high T two signal, here's one involving the right half,

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the splenium of the corpus callosum,

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then you can come up with a diagnosis of of neurofibromatosis type one.

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So this turned out to be a very, uh, unusual rare case of NF type one.

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But you can make the diagnosis based on the enlarge of the optic chiasm and then

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also on the, uh,

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high areas of flare and T two signal,

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which have pathologically been able,

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have been pathologically shown to represent hematomas.

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So this is the case I showed earlier about the optic neuritis,

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and this is more of the classic case that you would expect to see in optic nerve

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glioma. So this is somewhat similar to what we saw before.

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See that diffusely thickened optic chiasm here, see how thick that is.

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And on the coronal T two weighted images, here's diffuse

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Enlargement of the optic nerve and look what it's doing to the optic sheath.

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It's, uh, diffusely enlarged.

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This is a coronal T two weighted images demonstrating increased enlargement of

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the pre-K asthmatic segment of the optic nerve.

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Here's a T one weighted image with contrast demonstrating diffuse enhancement.

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This is that enlargement on T two and then on the SAG T one weighted images,

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excuse me. And sometimes this is defined.

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This is referred to as a sausage SA shaped appearance of the optic nerve.

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That's indicative of an optic nerve glioma.

Report

Patient History
Vision loss left eye.

Findings
Pre- and postcontrast MR was performed of the brain and orbits.

Brain: Sagittal T1-weighted images demonstrate corpus callosum to be intact. No evidence of Chiari malformation. No abnormal pineal region masses. Pituitary gland is not enlarged. Diffusion imaging demonstrates no evidence of recent infarct. Gradient-echo imaging shows no evidence of hemosiderin staining. Axial FLAIR and T2-weighted images demonstrate ill-defined areas of increased FLAIR and T2 signal involving the left basal ganglia, right side of the splenium of the corpus callosum, medial right cerebral peduncle, right anterolateral pons, and anterior 4th ventricle. Contrast-enhanced T1-weighted images show no abnormal enhancing intraaxial lesions. These findings are unchanged when compared to the prior study.

Orbits: Thin-section imaging through the optic nerves demonstrates diffuse enlargement of the optic chiasm extending posteriorly to involve the post-chiasmatic optic nerves and optic tracts with asymmetrical increased signal along the proximal postchiasmatic optic nerve tracts. No abnormal enhancement is identified. The retrobulbar segments of the optic nerves appear to be within normal limits. The postcontrast-enhanced T1-weighted images demonstrate no definite evidence of abnormal enhancement involving the right anteromedial pons and medial right cerebral peduncle. The region of Meckel's cave and cavernous sinuses appear to be within normal limits and symmetric.

Conclusions
1. Findings are consistent with neurofibromatosis type 1 associated with optic nerve glioma involving the optic chiasm extending posteriorly to involve the post-chiasmatic optic nerve radiations.
2. Multiple intraparenchymal focal areas of increased FLAIR and T2 signal which are most likely due to hamartomatous lesions typically seen in neurofibromatosis.
3. No change in the ventricular size, shape, and configuration

Case Discussion

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Suresh K Mukherji, MD, FACR, MBA

Clinical Professor, University of Illinois & Rutgers University. Faculty, Michigan State University. Director Head & Neck Radiology, ProScan Imaging

Tags

Neuroradiology

MRI

Head and Neck