0:01

So this patient is just under one year

0:03

of age, with a history of sepsis and had a CT

0:05

scan of the chest, abdomen, and pelvis to

0:07

evaluate for a source of the sepsis.

0:10

And so we'll scroll downwards and go right

0:12

to the abdomen and the liver in particular,

0:15

where there are a lot of abnormalities.

0:19

And on face value, we're really just seeing

0:22

numerous liver cysts, or cystic lesions within

0:26

the liver, multifocal involving the right

0:28

hepatic lobe, and involving the left hepatic lobe.

0:32

We'll look at it a little bit closer.

0:34

Let's follow the portal vein and

0:36

let's follow some of its branches.

0:38

And you can see, for example, the left portal

0:40

vein; some of its branches are going right

0:43

towards the center of these cystic spaces.

0:46

So this one over here, we can follow this

0:48

branch backwards, coming from the portal

0:49

vein, going right towards the center.

0:51

This one here is at the center.

0:53

This one here is at the center.

0:55

And so, what we're really seeing are

0:58

multiple cystic lesions that are enveloping

1:02

or surrounding these portal veins, these

1:05

portal vein branches in a very young child,

1:09

below one year of age.

1:12

And so this entity is almost

1:13

pathognomonic for Caroli's disease.

1:18

And this is a congenital abnormality

1:22

and it results in multifocal regions

1:26

of either saccular or cystic dilatation

1:30

of the intrahepatic bile ducts.

1:33

It can be diffuse; it can be involving just

1:35

one lobe, potentially involving a segment

1:37

of one lobe, and if it's associated with

1:40

liver fibrosis and portal hypertension,

1:43

it's known as Caroli's syndrome.

1:45

If it's not associated with that,

1:46

we call it Caroli's disease.

1:49

In imaging findings, we're seeing numerous

1:51

liver cysts; these will communicate with

1:53

the biliary tree; they're essentially

1:54

abnormalities of the biliary tree themselves.

1:57

And one of the findings that's been

1:58

described, I believe first on ultrasound,

2:01

but that I think sort of holds true for

2:03

any sort of cross-sectional imaging you

2:04

do thereafter, is the central dot sign

2:07

where you see the portal radical really

2:09

at the center of this saccular dilatation.

2:13

This is often associated with diseases

2:15

of the kidney as well, such as autosomal

2:17

recessive polycystic kidney disease, medullary

2:20

sponge kidney, and medullary cystic disease.

2:22

You can see this patient definitely

2:23

has some sort of cystic disease

2:24

involving the right kidney.

2:27

And the last thing I'll mention is that when

2:28

you look at some classification schemes,

2:31

sometimes they'll qualify this entity as a

2:34

type 5 choledochal cyst, but it's thought that

2:38

the pathophysiology of this is different enough

2:41

from the other choledochal

2:43

cysts that it's sort of classified as its own

2:46

entity and known as Caroli's disease.

2:48

So if you see that sort of put into that

2:51

category, don't be surprised

2:52

that it's sometimes put in that category,

2:54

but it's thought to be unique enough in

2:56

the way that it forms that it can be sort

2:59

of classified as its own separate entity.

3:02

And so again, this is a case of

3:04

Caroli's disease in a very young

3:06

child who has a history of sepsis.