0:00

So this was a newborn infant who presented

0:03

with respiratory distress.

0:04

And so we have a radiograph of the chest, abdomen, pelvis.

0:08

Um, we have bilaterally, bilaterally, low lung VINs.

0:13

Um, the cardiac silhouette is at the upper limit of normal,

0:15

but the most important finding here is the funky course

0:18

of the CIC tube.

0:19

So a course is below the diaphragm as we expect,

0:22

but typically we would expect, uh, stomach

0:25

to be located in the left upper abdomen.

0:28

This enteric tube is pushed into the center of the abdomen

0:32

and we have increased soft tissue fullness in this, um,

0:35

left upper abdominal quadrant.

0:38

If you look super carefully, make your, uh, window

0:41

and level super contrasty,

0:42

you can see some super subtle calcifications also in this

0:45

left upper abdominal quadrant.

0:47

And not only that, not only our stomach,

0:50

but all of our loops of bowel are displaced into the pelvis

0:53

and a little bit towards the left.

0:54

So this is pretty concerning that there's some sort

0:56

of abnormal soft tissue mass in the left upper

0:59

abdominal quadrant.

1:00

So the next thing we're gonna recommend to look for,

1:03

number one, to confirm

1:05

that there is indeed a mass in the left

1:07

upper abdominal quadrant.

1:08

We wanna determine the organ of origin

1:10

to better plan other cross-sectional imaging to follow.

1:14

So we're gonna start with an ultrasound

1:16

of the left upper abdominal quadrant

1:18

and see where this mass is arising from.

1:22

So this patient, uh, later that same day,

1:24

underwent complete abdominal ultrasound

1:27

to better assess that mass.

1:28

And so here the tech is trying to look at the pancreas

1:31

and we see a little bit of pancreatic head.

1:33

We see right kidney, we see some liver here.

1:36

There is this abnormal something in this left upper

1:39

abdominal quadrant.

1:40

It's hard to tell exactly what's going on,

1:42

on just one single image.

1:43

So let's keep scrolling through our abdomen,

1:45

follow our follow our abdominal ultrasound protocol.

1:48

So here we're in the sagittal plane,

1:49

we're looking at the left hepatic lobe,

1:52

and there is a very heterogeneous mass in the left upper

1:56

abdominal quadrant on the transverse images you could

1:59

convince yourself or, or question is

2:01

that just the heterogeneous content in the stomach?

2:03

But this is a mass

2:05

and I will, I will convince you when I show you the, uh, uh,

2:08

color doppler images later.

2:11

We, uh, have an umbilical vein, uh, which normally starts

2:16

to close, uh, postnatally.

2:17

So we're okay that there's no flow in that umbilical vein.

2:20

We also like that there's flow in the left portal vein

2:23

that's normal on a day of life, zero or day of life one.

2:27

As we're keeping scrolling,

2:28

there is some sludge in the gallbladder.

2:30

I feel like that's a normal finding in all of our patients.

2:33

We have a normal right adrenal gland.

2:36

We have a normal looking right kidney.

2:38

A reminder that newborns normally have, uh,

2:41

hyper coic kidneys compared to the appearance later in life.

2:44

So it's allowed to be brighter than the

2:46

adjacent liver parenchyma.

2:47

We do want cortico medullary differentiation

2:49

to be preserved in these infants, however.

2:52

So here comes the money

2:54

and how we can figure out the organ of, uh,

2:57

origin of this mass.

2:58

So number one, we start to see this, um,

3:01

this heterogeneous mass in the left upper abdominal quadrant

3:05

explaining that, uh,

3:06

increased soft tissue density in the left upper

3:08

abdomen at x-ray.

3:10

But this is number one IVC number two left hepatic vein.

3:15

So this is a huge left hepatic vein

3:18

that looks like it is extending, um, into that mass.

3:22

I'll show you on the cinematic images to convince you

3:24

that it's that big mass is being drained

3:26

by the left hepatic vein.

3:28

We have more gallbladder sledge here

3:30

as we're going through our stills.

3:31

Um, we have no billary ductal dilatation.

3:33

Our main portal vein has normal antegrade flow.

3:37

We have doppler imaging showing, uh, uh,

3:41

normal antegrade portal venous flow,

3:43

normal hepatic arterial flow.

3:45

This patient did have respiratory distress

3:47

and so this was the, um,

3:49

that's why these hepatic arterial waveforms looked a little

3:52

bit unusual like that.

3:54

We have a patent left portal vein, um,

3:57

and here is this very large left hepatic vein.

4:01

Um, uh, we have patent IVC,

4:06

uh, everywhere that the sonographer sampled.

4:08

We have patent IVC, now we're getting dedicated images of

4:11

that right kidney and right adrenal

4:13

glands which look normal.

4:16

We'll move on to the left to make sure

4:18

that the left kidney is not the organ of origin

4:20

for this mass after we see a normal urinary

4:24

bladder in the pelvis.

4:26

So here we are gonna focus on that, uh,

4:29

large heterogeneous mass in the left

4:30

upper abdominal quadrant.

4:31

And we see these epigenic foci to suggest

4:34

that indeed those subtle calcifications we questioned on

4:37

the x-ray were real.

4:39

So, uh, very heterogeneous, partially calcified mass.

4:43

It is large.

4:44

So up to, uh, over eight centimeters in diameter in this,

4:47

uh, left upper abdominal quadrant of this infant.

4:51

It is very vascular.

4:53

So when she, the, when we interrogate with color doppler,

4:57

uh, we have large peripheral blood vessels, um,

5:00

with spectral analysis.

5:01

Some of these have low resistance arterial waveforms.

5:05

Some of them have sort of unusual venous waveforms,

5:07

little pulsatility in it.

5:10

We have a normal spleen, so the spleen is not the organ

5:13

of origin of this big mass.

5:15

And then hopefully we see left kidney soon. Here we go.

5:17

So here is left kidney

5:18

where we have normal reinform appearance of the left kidney

5:22

and we actually see some normal left adrenal gland.

5:25

A reminder that it's normal to see the adrenal gland up

5:27

until about six weeks of age of newborn infants.

5:30

So normal left kidney, the left kidney is not the organ

5:33

of origin in this infant.

5:36

So that leaves us with a left upper abdominal quadrant mass.

5:39

I love this image. We have normal adrenal,

5:41

normal left kidney, normal spleen,

5:43

and this, uh, separate mass.

5:46

So that leaves us with, um, the liver

5:50

as the organ of origin.

5:51

And my learning point from this case was when you have a

5:55

huge left hepatic

5:56

Vein draining your mass,

5:58

then the liver is the organ of origin.

6:00

So if it's, if it's being drained by left hepatic vein,

6:04

that means it is, uh,

6:06

coming from the, coming from the liver.

6:08

So this is a large mass.

6:10

Um, here's our large left, uh,

6:12

hepatic vein on this transverse cinematic gray scale men

6:15

and superior inferior extending into this

6:18

highly vascular mass.

6:20

So a very vascular mass, um,

6:23

in the abdomen arising from the liver.

6:26

Um, in a newborn, a zero day old infant

6:29

that is separate from the adrenal gland,

6:32

that is gonna be a congenital hepatic hemangioma.

6:35

Um, congenital hepatic hemangiomas are, uh,

6:39

they develop in utero

6:41

and they are largest in size at the time of birth.

6:45

Um, and so that is what, that's

6:47

what this is most likely gonna be.

6:49

Um, again, we know that this is liver and origin

6:51

because this left hepatic vein is draining this large,

6:55

very vascular, very exophytic mass

6:57

that's arising from the superior aspect

7:00

of the left hepatic lobe.

7:01

So the next study we're gonna do is some additional

7:04

cross-sectional imaging with post contrast imaging

7:07

to better characterize, um, characterize the mass

7:10

and delineate the vasculature as well as, um, uh,

7:14

plan preoperatively to resect this lesion.

7:18

So this patient, uh, next underwent abdominal

7:21

and pelvic MRI prior to

7:23

and following contrast administration

7:25

to better see the extent of this lesion.

7:27

So this, uh,

7:28

upper left hand image is a coronal T two fat saturated

7:32

or stir image,

7:34

and it nicely corresponds to the appearance,

7:36

uh, at ultrasound.

7:37

It's a very heterogeneous mass lesion in

7:40

the left upper abdomen.

7:41

Um, and you can see that large left hepatic vein extending,

7:45

um, into the area with that big huge flow void there.

7:49

The axial T two weighted, um, sequence shows again

7:53

that mass, um, with huge, uh, flow void,

7:58

so very vascular, um, mass lesion in the left upper abdomen.

8:02

And I think it's pretty easy, uh, to tell

8:05

at the MRI even more so than the ultrasound

8:08

that this lesion is separate from spleen.

8:11

It is separate from adrenal gland,

8:13

it is separate from, uh, left kidney.

8:15

So this is a liver primary

8:17

exophytic lesion arising from the left hepatic.

8:19

Well, one other thing I want to point out on this.

8:22

Um, MRI look at this cardiomegaly.

8:25

So this vascular mass is causing a large amount of extra,

8:30

extra cardiac, um, shunting.

8:32

And so this, this patient is at, at risk for, um,

8:36

overflow circulation

8:37

or heart failure, um, related

8:39

to this extra cardiac shunting.

8:41

So, um, that is the real reason why this patient would

8:44

eventually go on to uh, resection.

8:46

Since this is a resectable lesion

8:48

because it's exophytic, thank goodness, um,

8:51

because this is a congenital hepatic men post contrast, you

8:54

Can see these large enhancing

8:57

very dysmorphic looking vessels at the peripheral aspect

9:00

of this large congenital hemangioma.

9:02

If we got delayed imaging, we would see some fill in, um,

9:06

of this, of this central part of this mass.

9:09

Um, but mostly we just see internal non enhancement,

9:13

um, in this infant.

9:15

Um, a couple of points of congenital hemangioma, um, again,

9:19

they're largest in size at the time of birth.

9:22

Typically they are the, uh, rich subtypes.

9:25

So the rapidly involuting congenital hemangioma,

9:28

they will involute spontaneously.

9:30

The issue with infants who have large congenital such

9:34

as this is they can cause very bad problems related

9:38

to shunting, and these patients can have congestive heart

9:40

failure related to over circulation.

9:43

Um, and so this patient did go to resection

9:46

of this lesion eventually,

9:47

it was a very complicated procedure

9:49

or transplant surgeons actually were involved

9:51

with resecting this very large mass.

9:53

Um, but these typically congenital hemangiomas are

9:56

typically solitary.

9:58

Um, again, they're largest

9:59

and present at the time of birth,

10:02

unlike infantile hemangiomas, which are multiple

10:05

and are not present at the time of birth.

10:08

So that's one way you can tell congenital versus

10:10

infantile hemangiomas.

10:12

Um, and we'll talk a little bit about the other differences

10:15

that are important to note about infantile versus congenital

10:18

hemangiomas on a subsequent companion case.