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Primary Sclerosing Cholangitis (BD)

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Here we have a patient who is a 50-year-old

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gentleman with worsening liver function tests,

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and an MRI was obtained to evaluate for this.

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I'm going to start off using

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the axial T2-weighted sequence.

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As we scroll downwards from the

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sequence, I'm going to focus on the bile

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ducts in the intrahepatic biliary tree.

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It's tough to really see if something

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objectively is going wrong here.

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If you were to look at it very critically

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on this slice, oh, maybe there's some bile

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ducts that are a little bit dilated, but

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I'm not going to draw any conclusions yet.

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Right on this slice, you start to see

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something here that looks a little bit dilated.

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So that could be a dilated bile duct.

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Maybe even this one; it's hard to know.

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Maybe even this one over here.

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So there's something, there are some clues

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that maybe something is going wrong here.

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On this slice, you can see a little bit

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of the bile ducts that are dilated here,

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but they're not completely dilated.

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It's just one small segment

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of it that's dilated.

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Similarly, posteriorly over here, and on this

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slice, you can definitely see the right hepatic

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ducts that are quite, quite dilated, right?

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So something's happening here where some

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of the intrahepatic biliary tree is affecting

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certain segments that look a little bit more

1:08

dilated than they should be, right?

1:11

If we sort of follow this all the way down

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the extrahepatic biliary tree over here.

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looks fairly okay, and we can follow

1:17

it to the ampulla fairly nicely.

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This whole area here was just a little bit

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of an artifact, probably a focus of gas.

1:24

Notice how it's non-dependent as opposed

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to a stone, which would be dependent.

1:29

Let's evaluate the same findings but

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on the coronal plane, and then we'll

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start looking at some more sequences.

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As we scroll through the coronal plane, I'm

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going to magnify for a second; you can see

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that there are some bile ducts here that look

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a little bit dilated, some bile ducts here

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that look a little bit dilated. You know,

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some bile ducts here that might be dilated,

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some bile ducts here, one over

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here that's quite dilated, and even out in

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the periphery, some ducts that are dilated.

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So again, I think the conclusion is probably

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very similar, but we're just viewing

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everything in a very different plane.

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We also did an MRCP sequence over here.

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So let's just have a look at that.

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And this is sort of a 3D MIP of the sequence.

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And I think this in itself is very instructive.

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And what this shows us is that the extra

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hepatic biliary tree looks fairly okay.

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The right hepatic duct here is a little

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bit dilated, but then look what happens.

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You can't follow it all the way through.

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In fact, it's sort of almost missing, but then

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you see it again over here, and it's dilated.

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And then maybe it's missing again.

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And then you see it again,

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more distally, and it's dilated.

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Similar pattern involving other

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portions of the bile ducts.

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Over here, it's relatively small in

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caliber, but then it's dilated again.

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Similarly over here, similarly in the

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left hepatic lobe, where, you know,

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you have certain segments

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of ducts that are dilated and other

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segments that you don't see well at all.

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Another sequence that I like to use in order to

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evaluate, particularly the intrahepatic bile

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ducts, is actually T1 post-contrast images.

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And so here we have our T1 axial T1

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post-contrast images, and they're sort of stacked up.

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You have the arterial phase first, in which

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you can certainly see some abnormalities.

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Look at the bile ducts here

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that are a little bit dilated.

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Look at this bile duct here that's dilated.

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There's some heterogeneous enhancement

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globally of the liver parenchyma.

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You can see another bile duct here that's

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dilated, one over here that's dilated.

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Let's look at it now in the oral venous phase.

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And what you're starting to see is

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that there are multifocal regions of

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intrahepatic biliary ductal dilatation

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over here, over here, over here.

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And what's interesting, as opposed

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to some of the other cases that we've

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seen, for example, choledocholithiasis,

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is it's not uniformly dilated.

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So we have a duct that's dilated

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here, and then it narrows over here.

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You hardly see it, maybe only

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a sliver of it over here.

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And then again, you see

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it dilated over here.

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And you see that pattern almost

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everywhere in this liver.

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Again, dilated ducts over here, but then they

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get narrowed again; you can hardly see them.

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And the extrahepatic biliary tree, if

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you follow it, looks fairly okay.

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And so this pattern

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of multifocal regions of intrahepatic

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biliary ductal dilatation and

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narrowing is quite characteristic

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of primary sclerosing cholangitis.

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Right.

4:18

This is an idiopathic inflammatory condition.

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It's thought to be potentially immune-mediated.

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And it affects the biliary tree.

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It can affect the intrahepatic ducts,

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it can affect the extrahepatic ducts,

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it can affect both at the same time.

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There's a strong association

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with this in ulcerative colitis.

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If you're looking at patients with ulcerative

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colitis, always remember to look at the

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intrahepatic tree to look for this entity.

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There is an increased risk of developing

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cholangiocarcinoma, and I'll talk a

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little bit about what that looks like

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in another case in this master course.

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And it's often seen in males about

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30 to 40 years of age, so that's sort

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of the patient demographic, so it can

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be seen in any number of patients.

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And over time, because you have these areas

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of biliary stricturing, you have impaired

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biliary excretion, and that can

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result in atrophy in portions of the liver.

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The caudate lobe, oftentimes as a result,

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can actually hypertrophy, so it's a very

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characteristic rounded appearance of the liver;

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it can almost look quite cirrhotic, but it has

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itself a very specific appearance where you

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have enlargement of the caudate lobe and atrophy

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of some of the other more hepatic segments.

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And so once again, this is a case of primary

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sclerosing cholangitis, multifocal intrahepatic

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biliary ductal strictures with resulting

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regions of focal intrahepatic ductal dilatation.

Report

Faculty

Mahan Mathur, MD

Associate Professor, Division of Body Imaging; Vice Chair of Education, Dept of Radiology and Biomedical Imaging

Yale School of Medicine

Tags

Syndromes

Other Biliary

Non-infectious Inflammatory

MRI

Liver

Iatrogenic

Gastrointestinal (GI)

Gallbladder

Congenital

Body

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