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Case: Infantile Hepatic Hemangioma

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0:01

So this is our companion case

0:02

to the congenital Heman Genoma case we saw earlier today.

0:05

So this was a four month old infant who had a urinary tract,

0:09

um, infection who came to imaging for renal ultrasound.

0:13

And so the tech starts off

0:14

with our typical retroperitoneal protocol

0:17

where we're looking at the kidneys.

0:19

But incidentally, when we're looking at the kidneys,

0:22

we happen to catch part of the liver parenchyma,

0:24

and we see these multiple hypo coic lesions scattered

0:28

diffusely throughout the liver parenchyma.

0:30

So everywhere we look, we start to see more

0:33

of these hypo coic liver lesions.

0:35

Um, we'll continue to follow our ultrasound protocol,

0:39

but basically normal right kidney, normal urinary bladder,

0:43

normal left kidney.

0:44

So the pertinent finding in this case is not

0:47

with the kidneys or urinary bladder at all,

0:49

but, um, incidental low density lesions scattered throughout

0:53

the liver and the text switch

0:55

to our linear high frequency transer to, to, um, better try

0:58

to see these hypo code lesions throughout the liver.

1:01

So the next thing we'll do is number one, we will, uh, look

1:05

for a primary tumor in the abdomen.

1:08

The most common, uh, uh, disease or, or,

1:12

or thing that we will see that will have multiple liver

1:14

lesions scattered throughout the liver is metastasis.

1:17

So we'll look for neuroblastoma

1:19

and wilms tumor as a primary, uh, site of cancer

1:23

that would present with liver metastases.

1:26

The good news is we don't see a primary

1:28

malignancy in this patient.

1:29

We have normal looking kidneys at ultrasound,

1:31

and we don't see any senal masses in this patient.

1:35

So the next thing we'll do is we'll ask our clinical

1:37

colleagues to do a good cutaneous examination looking

1:40

for any cutaneous infantile hemangiomas.

1:43

And then this patient, um, uh, was seen

1:45

by our vascular anomalies clinic

1:47

and went on to MRI as the next step for characterization

1:50

of these liver lesions.

1:53

So this patient went on to MRI to better characterize these,

1:56

uh, multiple liver lesions

1:57

and ensure there was no primary malignancy.

2:00

So we'll start with this, uh, axial T two fat saturated, um,

2:04

MRI sequence on the top left hand corner,

2:06

you can see normal thymus

2:08

surrounding this normal sized heart.

2:10

We do have bilateral atelectasis in this MRI

2:13

that was performed under sedation.

2:15

Um, and this is a nice correlate to

2:17

what we saw sono graphically.

2:19

So, um, at ultrasound there are a bunch

2:21

of hypo coic liver lesions scattered throughout the liver.

2:24

But on T two weighted imaging, we have T two, um,

2:27

hyper intense liver lesions in numerable of them, um,

2:32

scattered throughout all lobes of the liver.

2:35

This is helpful for us to tell.

2:36

There's no primary adrenal mass to suggest neuroblastoma,

2:41

and it's helpful for, um, confirming

2:43

that there's no primary, um, uh, liver lesion, I'm sorry,

2:47

splenic lesion to suggest this is metastatic wounds disease.

2:50

So this is the axial T two fat that, um, the coronal, uh,

2:55

the coronal T two weighted, uh,

2:57

series shows similar appearance of innumerable varying size,

3:01

but small, um,

3:03

T two hyperintense lesions scattered

3:05

diffusely throughout the liver.

3:07

On our post contrast, which is this, uh,

3:09

bottom right hand side, I'm gonna blow it up.

3:11

We did dynamic post contrast to be able

3:13

to look at the vascular enhancement of this.

3:15

So we'll start with your arterial phase

3:17

or aorta is enhancing first,

3:20

and we have just very subtle hyper enhancement of,

3:24

of these lesions scattered

3:25

throughout the, throughout the liver.

3:27

Um, it's, they're pretty hard to tell that,

3:29

that there indeed are lesions here,

3:32

but here, here for an example is one such lesion

3:35

that is hyper enhancing subtly compared

3:37

to the background liver parenchyma.

3:40

And as we continue through our dynamic post con, um,

3:43

post contrast series axial, um, the really,

3:47

now the liver just looks very heterogeneous,

3:49

looks homogeneously enhancing.

3:51

Um, I don't really see any lesions as we go on

3:54

through portal venous and late portal venous phase

3:57

of imaging, but we did this

3:59

with a hepatocyte specific contrast agent, um, to be able

4:03

to help characterize these lesions.

4:05

And so on our hepatobiliary phase MRI, we see

4:08

that there is low signal

4:10

or no contrast retention

4:12

of these innumerable lesions scattered throughout the liver.

4:16

So this is a classic appearance for infantile hemangiomas

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and, um, when you have such diffuse involvement,

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you could consider this to be hemangiomatosis,

4:26

so innumerable infantile hemangiomas throughout the liver.

4:30

So in contrast to congenital hemangiomas,

4:33

infantile hemangiomas are usually multiple, um,

4:37

when there is liver involvement

4:38

and they are associated

4:40

with the q the identical lesion on the surface of the skin.

4:43

So cutaneous, infantile hemangiomas also pathologically,

4:48

these are vascular tumors.

4:49

They stay glute one positive at pathology

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and it is important to make this diagnosis

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because infantile hemangiomas are sensitive

4:58

to beta blocker therapy.

5:00

So they treat these infants with propanolol if,

5:03

if there is such extensive involvement such as this case,

5:06

because that will cause the infantiles to involute.

5:11

Um, so they'll shrink

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and they all, they, they won't cause problems anymore.

5:15

Um, one other interesting thing about infantile hemangiomas

5:18

is that they are a vascular tumor that express type three,

5:22

um, dia oto sase.

5:24

So it's a, it's a enzyme

5:26

or a a, it's a, a substance that these tumors express

5:30

that inactivate thyroid home hormone.

5:33

So this is the other reason it's important to distinguish

5:36

between congenital hemangiomas and infantile heman is

5:39

because these patients might have problems related

5:41

to hypothyroidism.

5:43

So that's another thing you can tell your clinicians

5:45

to be on the lookout for when you make the diagnosis

5:48

of infantile hemangiomas.

5:50

So congenital, hes present at birth or large

5:54

and cause problems related to vascular shunting,

5:57

Infantile hemangiomas present within the few,

6:00

the first few weeks to months of life.

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So not present at birth, but rather they develop later on

6:05

and they cause issues not only related to shunting, but also

6:09

because of, um, hypothyroidism.

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They can also cause mass effect.

6:12

And if they're on the surface

6:13

of the skin in an important area, they can grow, um,

6:17

as the child grows

6:19

and they can cause issues related to ulceration

6:22

or, um, as they enlarge they might cause compression

6:25

of important vascular structures.

6:27

Um, so a case of companion case of infantile heman illness.

Report

Text

Faculty

Judy H. Squires, MD

Associate Professor of Radiology

UPMC Children's Hospital of Pittsburgh

Tags

Vascular

Ultrasound

Pediatrics

Neonatal

MRI

Liver

Congenital

Body