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This is an MRI of the brain in an 11-year-old

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with Sturge-Weber syndrome, and we can see

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bilateral occipital volume loss with cortical thinning,

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subcortical white matter volume loss,

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ex vacuo prominence of the occipital horns

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of both lateral ventricles, susceptibility

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weighted imaging shows dystrophic mineralization

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involving these regions bilaterally in both

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occipital lobes, and then post-contrast imaging

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shows that there is leptomeningeal enhancement

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in both occipital lobes in these regions.

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Now, this is of interest because

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classically, Sturge-Weber syndrome

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is felt to be an ipsilateral or

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unilateral finding, a unilateral finding

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ipsilateral to the facial port-wine stain.

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And that is true, except for when it's not true.

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So, this patient has bilateral

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occipital Sturge-Weber syndrome.

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And we can see there's also some other,

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you know, venous drainage variants.

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This would normally be considered

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a developmental venous anomaly.

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Given the setting of Sturge-Weber syndrome,

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it's unclear whether some of

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those are collateral vessels or not.

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This patient had difficult-to-control seizures.

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And if we look at this sagittal image,

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we can see the parietal-occipital sulcus here,

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calcarine fissure, and we can see the cuneus

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of the occipital lobe, just the volume loss.

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We're seeing cortical thinning.

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We're not seeing significant white

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matter as we do see elsewhere.

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So, this is predominantly an occipital,

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maybe posterior parietal finding,

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but the patient had seizures.

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So, one of the techniques that they used in this

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patient was to perform a corpus callosotomy.

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They did a midline transection of the

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corpus callosum, which we can see on

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this diffusion tensor fiber tracking.

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We see no midline fibers traversing the corpus

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callosum in a transversely oriented direction.

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Normally, transversely oriented fibers would

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be red, and we're not seeing any transversely

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oriented fibers traversing the midline.

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Also of note on the post-contrast T1-weighted

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imaging, we're seeing extensive venous channels

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within the diploic space, within the bones.

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This is a finding that has been

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associated with Sturge-Weber syndrome.

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It may be related to collateral formation.

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It may be related to the underlying venous

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development abnormality, but that is definitely

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a finding that is a known association with

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Sturge-Weber syndrome and is important to

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notice and potentially has surgical implications

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if they're going to do a craniotomy because

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that may result in greater than expected

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bleeding for craniotomy through this region.

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Diffusion tensor fiber tracking,

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also known as tractography, ends up showing that

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there are no fibers crossing the midline

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on the forceps minor or forceps major.

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So, this is a way of using the diffusion

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tensor imaging with these directional

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encoded fractional anisotropy map, and the

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diffusion tensor fiber tracking to confirm

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the midline transaction of the corpus

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callosum, which was performed to treat the

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seizures related to bilateral occipital

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involvement in Sturge-Weber syndrome.