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Sturge Weber Syndrome: Ribbon-like Cortical Calcification

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This is a CT scan of the head

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in a six-year-old with known Sturge-Weber syndrome,

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with a right-sided facial port-wine stain.

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There is volume loss in the right occipital lobe,

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and we see this coarse calcification.

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That calcification actually follows the cortex.

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You can see this here on the coronal image.

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We see coarse calcification following the cortex

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in the right occipital lobe, and we see more

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finer calcification, but also volume loss in

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the right temporal lobe, and there's actually

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volume loss in the right parietal lobe as well.

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So, there's multiple areas of volume loss.

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The bone algorithm images can actually

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help us see that this calcium follows

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boundaries of where the cortex once normally was.

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This is the result of chronic venous

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ischemia that results in dystrophic

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mineralization in the areas of volume loss,

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which in this case is along the cortex.

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There's not as profound of involvement of

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the white matter because even though the

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adjacent white matter has similar chronic

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venous ischemia, the metabolic demands of

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the white matter are less than the gray matter,

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so they are less susceptible to the

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effects of this chronic venous ischemia.

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We can see that with that right-sided

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cerebral hemispheric volume loss,

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we can see asymmetric prominence of the

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diploic space overlying this volume loss.

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We see the outer table cortex,

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inner table cortex, and in between is the diploic space.

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The diploic space is asymmetrically

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prominent overlying this area of volume loss.

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Notice here, this is a more normal appearing

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diploic space for a child of this age.

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Using all the information we have, we can take

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the thin section images from the CT scan,

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make a three-dimensional rendering of the skull,

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take away half, and we can see

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the gyroid form appearance of this

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calcification in three dimensions.

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So, while we see a two-dimensional rendering

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here on the bone algorithm images, this three

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dimensional representation shows us that this

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is the result of dystrophic mineralization

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in the cortex of the right occipital lobe

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with significant volume loss and almost

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a geographic involvement of this area.

Report

Description

Faculty

Asim F Choudhri, MD

Chief, Pediatric Neuroradiology

Le Bonheur Children's Hospital

Tags

Syndromes

Pediatrics

Neuroradiology

Neuro

Congenital

CT

Brain

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