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Autosomal Dominant Polycystic Liver Disease

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This case is a 44-year-old gentleman,

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history is follow-up of liver masses.

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So here we have, uh, the liver MRI for this patient.

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We'll start off looking at our T2-weighted sequences.

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As we scroll through the T2-weighted sequences,

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this is a single-shot, uh, spin echo, uh, sequence.

0:18

We can see that there are numerous liver lesions

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scattered throughout the liver, variable size, some

0:24

larger than others, some have lobulated borders.

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We can see that they're fairly

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hyperintense on this sequence.

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And I'm just going to sort of look at one representative

0:36

lesion in order to describe it a little bit more.

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So if we see this large lesion over here, we can see

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that the internal content is very T2 hyperintense.

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But again, we're not going to use this sequence

0:49

to really judge the T2 signal of any lesion.

0:52

We're going to use the Turbospin echo sequence over here.

0:55

And on this one, indeed, look how bright

0:57

this content is of this, of this mass.

1:00

If we see this content this bright, it

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looks so similar to CSF, we're going to

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be, um, not too worried about this lesion.

1:08

It's probably going to be a cyst, maybe a

1:10

hemangioma, but we're going to look at some of

1:11

the other imaging sequences to sort that out.

1:14

Next, we're going to look at the

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T1 in and out of phase sequence.

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We're looking for areas of signal loss.

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On the out-of-phase sequence

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over here, areas of signal loss.

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On the in-phase sequence over here, and if we sort of take

1:25

a global survey of all these liver lesions, we can see

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that they look pretty similar in all the imaging sequences.

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If we look at that representative lesion in the right

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hepatic lobe, we can see that it has T1 hypointense

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signal, and that doesn't change between the out-of-phase

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sequence over here and the in-phase sequence over here.

1:48

Next up, we're going to look at the

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pre- and post-contrast sequences.

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And again, these lesions all on the pre-contrast

1:56

sequence look like they're hypointense, T1 hypointense.

2:00

We're going to look at that large lesion again.

2:02

This is the arterial phase image over here.

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We see that there's no contrast enhancement.

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Next up, we're going to look at

2:07

the T1 pre-contrast sequence.

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We can see all these lesions again have a T1 hypointense

2:14

signal with respect to the liver parenchyma.

2:17

This large representative lesion, again, very

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homogeneous in its signal and T1 hypointense with

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respect to the remaining liver parenchyma, as are the

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other liver lesions that are present for this patient.

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And finally, we're going to look at the dynamic

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post-contrast imaging sequences to assess if there's

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any internal enhancement within these lesions.

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And again, we'll look at the representative

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lesion in the right hepatic lobe.

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You can see the lesion here on

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the, uh, arterial phase images.

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You can see the lesion here on

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the portal venous phase images.

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And finally here on the equilibrium phase images.

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And it looks jet black on all these sequences.

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Um, no enhancement seen.

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And we can also see that around the rim of it, maybe

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there's a minimal, minimal imperceptible rim enhancement.

3:02

And that's okay.

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But internally, inside of it, there's

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absolutely no enhancement identified.

3:09

These findings are compatible with multiple cysts, and

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given the multiplicity of the lesions, you gotta think

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about autosomal dominant polycystic liver disease.

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Now, this is a, uh, finding that can be seen.

3:25

It's not that common.

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It can be seen with other cysts and organs, such

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as the kidneys, or it can be isolated as well.

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And often patients, uh, start off being

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asymptomatic, so no real symptoms, but as the

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cysts increase in size, you can start to get pain.

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Um, like any other cyst, they can rupture and cause

3:43

hemorrhage, uh, potentially get infected as well.

3:46

If symptomatic, patients will need to

3:48

undergo resection or again, marsupialization.

3:51

So, this is basically, uh, multiple liver cysts

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seen in patients, and typically we're talking

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about more than 10 or 20 liver cysts, um, and you

4:00

gotta start thinking about this, uh, this entity.

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

MRI

Liver

Idiopathic

Gastrointestinal (GI)

Body

Acquired/Developmental

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