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Mild Severity Dandy-walker spectrum

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This is a head ultrasound on a newborn that had

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an abnormal fetal ultrasound with concern for an

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abnormal development of the

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posterior cranial fossa.

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And similar to the fetal ultrasound,

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it can be difficult to fully characterize the

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cerebellar vermis.

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Here's the fourth ventricle,

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and this is the cerebellar vermis.

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And it's hard to know where the

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cerebellar vermis stops.

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So, this is definitely a time where

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MRI is more beneficial.

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In this case,

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we can see there is hypoplasia of the inferior

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aspect of the cerebellar vermis,

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but the vermis is present nearly in its entirety.

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We can see the superior medullary velum,

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right here, inferior medullary velum.

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A normal cerebellar vermis looks sort

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of like the character for Pac-Man.

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It's about trying to eat the brainstem.

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And in this case, most of Pac-Man is here,

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except Pac-Man has micrognathia.

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There is a hypoplastic inferior portion

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of the cerebellar vermis.

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So, there's just a very mild inferior vermian

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hypoplasia and mild enlargement of

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the posterior cranial fossa,

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where this CSF space is in free continuity

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with the fourth ventricle.

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So we can look at this on a T2-weighted image

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axial here, and you can see inferiorly,

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we're not seeing any cerebellar vermis

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between the cerebellar hemispheres.

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Superiorly,

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we see a normal vermis.

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So, this is a case where there's near complete

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development of the cerebellar vermis.

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But while it's nearly completely there,

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people sometimes referred to this

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as a Dandy-Walker variant.

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The problem is that a Dandy-Walker variant

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doesn't tell you if this is a mild hypoplasia of

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the vermis or more moderate or more severe

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hypoplasia of the cerebellar vermis.

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This case,

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I would consider it to be a very mild

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Dandy-Walker spectrum malformation,

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and I avoid the term Dandy-Walker variant

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because it is not a single entity.

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But if we qualify the description of this as

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being a mild Dandy-Walker spectrum malformation,

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I think it gives appropriate description to

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whoever's reading it that there are some

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features in the Dandy-Walker spectrum,

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but it is milder.

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I think that's important because there's a lot

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of confusion about terminology of the posterior

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cranial fossa malformations. In particular,

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the Dandy-Walker spectrum malformations.

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And not every person is going to

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say the exact same thing.

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And,

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you know,

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not every book that you read is

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going to say the same thing.

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So, if you're appropriately descriptive

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saying a mild severity Dandy-Walker spectrum

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malformation, I think that's helpful.

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I think that's appropriately descriptive.

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And by such as this,

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very often, can be clinically silent.

Report

Description

Faculty

Asim F Choudhri, MD

Chief, Pediatric Neuroradiology

Le Bonheur Children's Hospital

Tags

Ultrasound

Pediatrics

Neuroradiology

MRI

Idiopathic

Congenital

Brain

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