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This is a CT scan of the head

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in a three-month-old child with seizures,

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with a right-sided facial birthmark and clinical findings

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strongly suggestive of Sturge-Weber syndrome.

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Looking at the CAT scan, we can see volume

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loss in the right cerebral hemisphere

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with areas of increased density.

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This increased density is likely

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related to dystrophic mineralization

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associated with the volume loss that

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would be seen in Sturge-Weber syndrome.

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Now, while there's volume loss in the right

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cerebral hemisphere, we're seeing prominence

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of CSF space overlying both cerebral

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hemispheres, and MRI was performed, and we

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can actually, if we look closely, we can

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see the dura here and we see the cortical

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veins and we see a separation between the

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cortical veins and the inner table cortex.

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and we're seeing near complete

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flare suppression of signal here.

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So this is either a subdural hygroma

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or a proteinaceous effusion with

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very low proteinaceous content.

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Let's see what else is going on.

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So we're not definitely seeing

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a subdural collection overlying

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the right cerebral hemisphere.

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But we're also not seeing as many

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of these cortical veins as we did.

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We're seeing more little serpentine

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vessels that are potentially collateral

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vessels, but we're not seeing this

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normal constitution of cortical veins.

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overlying the right cerebral hemisphere.

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That is a characteristic feature

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of Sturge-Weber syndrome.

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If we look at the post-contrast imaging, we're

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seeing extensive leptomeningeal enhancement

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overlying the right cerebral hemisphere,

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related to leptomeningeal collateral vessels.

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Now, we're also seeing hypertrophy

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of the right choroid plexus.

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It's unclear whether that's related to the

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collateral venous flow and venous congestion,

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or whether that is a primary process.

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What else are we seeing?

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In addition to this, we look at the globes.

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This is the left eye, the normal eye, the

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axial length from the cornea to the back of

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the retina is approximately 20 millimeters.

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19 to 20 millimeters is

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approximately normal for a newborn.

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In the right eye, it's 22 millimeters.

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That's larger than we would expect.

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Separate from that, the anterior

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chamber between the cornea and the lens

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is approximately three millimeters.

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On the right, the interchamber is approximately

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four millimeters, so it's asymmetric.

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The corneal width is 13 millimeters on

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the right, is 12 millimeters on the left,

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but I think it looks larger on the right.

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And again, while it may sound like it's

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only a millimeter difference, those are very

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profound differences in a child this age.

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This here with the increased size of the

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eye and increased depth of the interior

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chamber is suggestive of glaucoma.

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Now, the diagnosis of glaucoma is made by

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measuring the intraocular pressure, but This

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here is an imaging appearance that is strongly

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suggested that there is right-sided glaucoma.

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If I zoom in on the orbits on the T1 post

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contrast imaging, I see this thin rim of

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enhancement along the posterior aspect

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of the right globe, along the retina.

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This is likely related to retinal angiomatosis.

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So retinal angiomatosis is a feature associated

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with Sturge-Weber syndrome, ipsilateral to the

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port-wine stain, ipsilateral to the cerebral

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abnormalities, and is associated with glaucoma.

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So between this enhancement, the enlarged

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axial length, and the increased depth of the

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anterior chamber, and potentially increased

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width of the cornea, this is strongly suggestive

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that this patient has right-sided glaucoma.

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This is important to know because this patient

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needs to see an ophthalmologist, potentially

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a glaucoma specialist, as soon as possible.

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Sturge-Weber patients normally get

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eye exams, but this patient needs

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an examination as soon as possible.

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Glaucoma and Sturge-Weber syndrome are

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notoriously very challenging for the

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ophthalmologist to manage, but it also

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can be potentially vision-threatening.

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So this is the type of finding where if

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you see this, even though it's just a

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millimeter or so difference, it's something

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that can really help guide management

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and help get them to an ophthalmologist

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faster than they normally would have.

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So this patient has Sturge-Weber syndrome with

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significant involvement in the right cerebral

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hemisphere with dystrophic mineralization,

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leptomeningeal enhancement, ipsilateral

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choroid plexus hypertrophy, ipsilateral retinal

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angiomatosis, ipsilateral enlarged right eye,

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and signs suggestive of right-sided glaucoma.