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Septo-optic Dysplasia, Scizencephalic Cleft – Mild

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This is an MRI of the brain

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in an 11-month-old child

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with small optic nerves that were

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identified on ophthalmologic examination.

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On this coronal image,

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we see absence of the septum pellucidum and

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otherwise, relatively normal appearing lateral ventricles.

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If we go forward,

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we see a smaller than expected optic chiasm

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and smaller than expected optic nerves.

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So, those are the features that we've

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described with septo-optic dysplasia,

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absence of septum pellucidum and hypoplasia

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of the optic pathway.

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Now, there are additional features that you

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can sometimes see.

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In this patient,

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we see a focal contour irregularity along the

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lateral margin of the body of

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the right lateral ventricle,

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and that there's this gray matter-lined

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cleft that goes out to the periphery.

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And this is a schizencephalic cleft.

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This is a very subtle schizencephalic cleft.

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It's nearly what's called a closed

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lip schizencephaly.

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Now,

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a closed lip schizencephaly is where the gray

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matter margins are up upon one another,

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and an open lip is where they're widely apart.

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Now, from my perspective,

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it's parsing terms to debate whether this is

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open-lipped or closed-lip schizencephaly,

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because either way,

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the parenchymal defect is relatively small

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And so, we're missing a small amount of brain.

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So whether you call this a closed-lip schizencephaly

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or a very narrow open lip schizencephaly,

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likely has no clinical

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differentiating features.

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The issue is that there's not a large gap

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where there's brain parenchyma missing.

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So this is a patient with septo-optic dysplasia

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and a closed lip versus a narrow

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open lip schizencephalic cleft.

Report

Description

Faculty

Asim F Choudhri, MD

Chief, Pediatric Neuroradiology

Le Bonheur Children's Hospital

Tags

Pediatrics

Neuroradiology

MRI

Congenital

Brain

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