0:02

Hello and welcome to Case Crunch Rapid case

0:04

review for the core exam hosted by Medality.

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In this rapid fire format, faculty will show

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key images along with a multiple-choice

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question, and you'll respond with your

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best answer via the live polling feature.

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After a quick answer explanation,

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it's onto the next case.

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You'll be able to access the recording of today's case

0:23

review and previous case reviews by creating a free

0:25

account using the link provided in the chat today.

0:29

We are honored to welcome Dr. Judy Squires

0:31

for a pediatric board prep case review.

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Dr. Squires completed radiology residency at

0:37

University of Cincinnati College of Medicine,

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followed by a pediatric radiology fellowship at

0:42

Cincinnati Children's Hospital Medical Center.

0:45

She is currently associate professor of radiology

0:47

at the UPMC Children's Hospital Pittsburgh, where

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she serves as chief of pediatric ultrasound and

0:53

director of the UPMC radiology residency program.

0:57

Questions will be covered at the end of time

0:58

allows, so please remember to use that

1:00

Q and A feature to submit your questions.

1:03

With that, we are ready to begin today's board review.

1:06

Dr. Squires, please take it from here.

1:09

Welcome, everybody.

1:10

I'm Judy Squires.

1:11

I'm a pediatric radiologist in Pittsburgh.

1:13

And, um, we're gonna, I have no relevant disclosures.

1:17

We're starting with a, let's make sure your stuff works.

1:20

Question.

1:20

So for our first question, are you, and then select

1:26

the answer that is the most relevant for you.

1:37

Lots of people studying for core.

1:39

Our residents are also in the home stretch

1:42

of studying, so hopefully these are high

1:44

yield and, um, you learn something and also.

1:47

Be engaged the whole entire time.

1:49

Um, this is gonna be a high yield image,

1:51

rich, case-based pediatric thoracoabdominal,

1:54

case-based with questions, um, session.

1:58

I'm gonna have a lot of companion cases, um,

2:00

to simulate, uh, what you're gonna encounter

2:02

when you're taking the, um, core examination.

2:05

Without further ado, let's start with our first case.

2:07

So this is a 2-year-old who

2:09

presented with abdominal pain.

2:12

We have two ultrasound images.

2:14

I'll give you just a couple of

2:15

seconds to familiarize yourself.

2:20

And then let's move on to the question.

2:22

So if there are no other complications,

2:25

what is the first-line treatment?

2:37

So I was hoping for an easy first, uh,

2:40

question to, uh, start this off and

2:43

most of you guys got the correct answer.

2:45

So the, um, first-line

2:48

treatment is a contrast or an air enema.

2:52

Um, so this is a classic donut sign or,

2:55

uh, in this, in the transverse plane of the

2:57

intussusception or in the sagittal plane, they

3:00

call it a pseudo kidney sign or a hotdog sign.

3:02

So this is an ileocolic intussusception.

3:04

We know that because we see both trapped mesenteric

3:07

fat and lymph nodes within the intussusception.

3:10

Um, the other thing to note, you can kind of see the

3:13

scale on the side of, um, both of the ultrasound images.

3:15

The diameter of this intussusception is.

3:18

Pretty large.

3:19

So an ileocolic intussusception will measure larger

3:21

than a small bowel, small bowel intussusception.

3:24

Um, so this is like three or four centimeters as opposed

3:26

to a small bowel, small bowel intussusception, which

3:28

can also give you a "target" appearance, but the diameter is

3:31

smaller and you won't see that trapped fat in lymph nodes.

3:34

So, um, the next step for this patient, as long as

3:36

there's no evidence of perforation or hemodynamic

3:38

instability, is to move on with an enema reduction.

3:42

If there's any evidence of, um, perforation,

3:44

we would skip the, uh, fluoroscopic reduction

3:47

and we would recommend surgery next.

3:50

So a follow-up question.

3:53

So this patient underwent the procedure.

3:56

The question is, what is the maximum pressure that

3:59

should be used during the air enema reduction?

4:11

120 is the magic number.

4:13

Yep.

4:13

So the correct answer is B and, um,

4:16

we want to balance ensuring reduction.

4:19

So enough pressure to reduce the intussusception,

4:22

but not increase the risk of perforation.

4:24

Great job. Next case.

4:28

We have a newborn infant who presents with emesis,

4:38

so the image stays the same.

4:40

You get to see the question.

4:41

Now, what is the next step in evaluation of this infant?

4:54

So we have a split response here.

4:56

So the correct answer is not that I actually clicked

4:59

on my slide, so it advances upper GI and the key.

5:03

Piece of information in this patient's history

5:05

is that this patient presented with emesis.

5:09

So once you have an infant with emesis, you can

5:11

basically ignore everything else that the image shows

5:14

or that the question says, because that patient.

5:18

Um, is that the primary concern is mal-

5:21

rotation with midgut volvulus, which, um, is,

5:24

uh, an important, uh, piece of information

5:26

to know that volvulus or green vomiting history.

5:30

Um, remember that in patients with, uh, mal-rotation

5:33

and midgut volvulus, oftentimes the radiographs are

5:35

completely normal, so they're not particularly

5:37

helpful in the assessment of these infants.

5:40

Um, so the next step, um, will always be

5:42

either an upper GI emergently or these

5:46

days a lot of institutions are doing an.

5:48

Upper abdominal ultrasound, so an abdomen-limited.

5:51

Um, an epigastric ultrasound, right lower

5:54

abdominal quadrant ultrasound would be, um,

5:57

looking for the appendix, not necessarily the

5:59

midgut anatomy that we need to see on ultrasound.

6:02

So continued, so the following images are obtained

6:07

during the fluoroscopic upper GI examination.

6:15

Just a couple more seconds to

6:17

check out what's happening,

6:22

and then we'll move to the question.

6:23

So what is the next step in management of this patient?

6:32

A patient with emesis who went to

6:34

upper GI, and these are the images I.

6:47

Emergency surgery is exactly right.

6:49

So of course, in real life you're gonna do,

6:52

um, multiple things all at the same time.

6:53

You're gonna try to suck out that contrast.

6:55

You're gonna fluid resuscitate the infant, but the

6:58

first thing you need to line up is call the surgeon

7:01

because this patient needs to undergo emergency surgery.

7:04

To be able to, um, reduce that, um,

7:07

twisting of the bowel and, um, hopefully

7:10

return perfusion and save bowel.

7:12

So, um, the images are, uh, showing you on the left side

7:15

of the screen a lateral view, showing you a corkscrew

7:18

of the, um, proximal small bowel, the frontal view.

7:22

The image on the right is showing you an abnormal

7:24

duodenal jejunal junction, which is too low.

7:27

And then it's also showing you that, um, that kind

7:29

of corkscrew appearance on the fluoroscopic study.

7:32

Um.

7:33

Uh, holdup of contrast at the proximal.

7:36

The second and third portion of the duodenum is

7:39

enough to question malrotation with midgut volvulus.

7:41

So that alone is enough to take a patient to the OR.

7:45

You don't necessarily have to see that corkscrew sign.

7:48

So, um, uh, malrotation with midgut volvulus

7:51

is a surgical emergency.

7:54

Great job.

7:56

But wait, there's more.

7:58

So our next question in this series,

8:00

what is the radiologic sign demonstrated

8:03

on this CT in a 7-year-old boy?

8:15

Oh my gosh, you guys are making me so proud.

8:18

So now you know, we're talking about

8:19

malrotation in midgut volvulus.

8:20

So this is the Whirlpool sign.

8:22

Some people like to call it a hurricane sign,

8:24

or I think of it as a hurricane of doom.

8:27

Um, so this is the Whirlpool, the, um, CT

8:30

equivalent of the corkscrew sign on the

8:32

upper GI that we saw.

8:34

So on CT, it looks, uh, looks like

8:36

a swirling of mesenteric vessels of

8:39

the duodenum, um, in the upper abdomen.

8:42

And you can see there's a little bit of

8:43

contrast that's, that is sneaking through there.

8:45

Um, just a reminder of what those other signs are.

8:48

The pseudo kidney is with an intussusception.

8:50

Um, that's the ultrasound appearance in the

8:52

sagittal plane of an ileocolic intussusception.

8:54

And then the drooping lily sign is, uh,

8:57

the term used to describe the lower pole

8:59

of a duplex collecting system in a kidney.

9:03

Last but not least, I think this is the

9:05

last of the, um, uh, subportions, uh, in

9:09

this theme of this, um, portion of the talk.

9:12

So again, we have that red flag history.

9:15

So we have a newborn infant

9:16

with emesis, green emesis.

9:23

I'm gonna let that play one more time

9:25

and then we'll go to the question.

9:35

So what is that arrow pointing at?

9:49

Let's see how we did.

9:50

So most people said the correct answer,

9:52

which is the superior mesenteric artery.

9:55

So ways to remember that the um.

9:59

SMA is the structure that everything is twisting around.

10:02

If they show you an image, the SMA (superior

10:05

mesenteric artery) has that echogenic halo around it.

10:08

So it's like an angel.

10:09

So SMA and an angel.

10:12

Um, so all of the midgut structures in the duodenum,

10:15

uh, mesentery, bowel, they're all twisting

10:17

around that superior mesenteric artery.

10:19

Um, oftentimes the duodenum, um.

10:23

In the setting of malrotation, make-up,

10:24

ulous will be dilated with fluid, and

10:27

we can see that easily at ultrasound.

10:29

The superior mesenteric vein almost always in the

10:32

setting of malrotation, is collapsed because of that twisting.

10:35

So you get venous obstruction

10:37

prior to arterial obstruction.

10:39

So just a reminder of the other structures

10:41

that, um, are important to know on an

10:43

ultrasound looking at midgut anatomy.

10:45

Um, so again, oftentimes both the stomach

10:48

and duodenum are fluid-filled, so they're,

10:50

uh, mostly anechoic on these images.

10:52

Uh, the superior mesenteric artery, again, is

10:54

pointed out by that white arrow, and then

10:56

the aorta is that posterior structure.

10:59

So with normal rotation on ultrasound, we see

11:01

the duodenum, the third portion of the duodenum.

11:04

In a retroperitoneal location, so it courses normally

11:07

between the aorta and the superior mesenteric artery.

11:11

In patients with malrotation, it courses anterior, or it

11:14

doesn't cross completely between the SMA and the aorta.

11:18

With malrotation and midgut volvulus, you will see

11:20

the duodenum being pulled anterior to the superior

11:23

mesenteric artery and twisting in a clockwise direction

11:26

as you're scanning from superior to inferior, as

11:29

we're doing on this cinematic image on the left.

11:33

All right.

11:33

Great news.

11:34

Our malrotation and midgut volvulus

11:36

topic is over—case number three.

11:39

We have a newborn term infant.

11:46

I'm gonna keep that image but cropped a

11:47

little bit for you guys to see the question.

11:50

What disorder is most commonly

11:52

associated with this abnormality?

12:04

Y'all are awesome.

12:05

This is a classic duodenal atresia patient.

12:10

So on this supine abdominal radiograph,

12:11

we have a double bubble sign.

12:14

So that is gaseous distension of

12:15

both the stomach and duodenum.

12:18

In real life, we place, uh, the neonatologist places an

12:20

NNG tube, and so oftentimes in real life we don't see.

12:24

Um, patients present with this dilation of the

12:27

stomach and duodenum because it's been decompressed,

12:29

but this was a nice example we had not too long ago.

12:32

Um, so the radiograph, uh, double bubble sign

12:35

raises concern for duodenal atresia, which in turn

12:38

raises concern for Trisomy 21 or Down syndrome.

12:41

Um.

12:42

Tr, uh, double bubble.

12:44

I'm sorry.

12:45

A, uh, duodenal atresia is not the only

12:47

cause of a double bubble on a radiograph.

12:49

You could also have an annular pancreas causing

12:51

obstruction at the second portion of the duodenum

12:53

level, or a preduodenal portal vein.

12:58

Um, just another, uh, some other associations with

13:01

Trisomy 21 congenital heart disease, uh, is, uh, is, uh.

13:07

Higher-end prevalence in this patient population,

13:09

and most commonly, it's an atrial septal defect.

13:11

Hirschsprung disease is also, uh, common.

13:14

There are increased risks of, uh, ALL acute, uh,

13:18

lymphocytic leukemia, hypothyroidism, hypotonia

13:21

on exam, and infants, and then subpleural cysts.

13:25

That's a hint for later.

13:27

Up next, we have another abdominal radiograph.

13:30

It's another newborn with vomiting.

13:38

Just a couple more seconds.

13:43

So now this question, what is the next

13:46

step in the evaluation of this infant?

13:59

Alright, y'all are on it tonight so that this,

14:02

uh, supine frontal view of the abdomen is

14:05

showing you a distal bowel gas obstruction so

14:08

there aren't ginormous, dilated loops of bowel,

14:11

mostly in the upper abdomen as you would.

14:13

With proximal bowel obstruction, there are kind of

14:15

diffusely dilated loops of bowel throughout the abdomen.

14:18

So in the upper abdomen and uh, pelvis, there are

14:22

dilated loops of bowel, kind of similarly distributed.

14:24

So this is a distal bowel gas obstruction.

14:27

In the absence of a history of bilious emesis, we go to

14:30

contrast Enema X. We don't do, um, barium enemas.

14:35

We do water-soluble enemas because we want to, uh,

14:39

number one, not cause more constipation with um.

14:42

By using barium for our diagnostic study,

14:45

but also with water-soluble contrast.

14:47

Most of us use a hyper, a slightly

14:49

hyperosmolar agent, which can help with the

14:51

constipation or distal obstruction in infants.

14:56

So, uh, keeping on with that patient, a

14:59

water-soluble contrast enema is performed.

15:03

The image is on the right-hand of the screen.

15:06

What is the diagnosis?

15:17

Great job everybody.

15:18

So, um, in a patient with a diffusely small

15:22

colon, we also have some meconium filling defects.

15:25

In this diffuse micro meconium

15:28

I is the primary consideration.

15:30

We did not have any reflux

15:32

contrast, um, into the distal ileum.

15:35

Um, but uh, this is enough to make that diagnosis

15:38

because we did not have huge dilated proximal small

15:41

bowel loops to make us think that this was, um,

15:44

ileal atresia; also good news that was not an option.

15:48

Um, a couple of other things

15:49

to point out in these answers.

15:51

Small left colon is the same thing as

15:54

functional immaturity of the colon; is

15:55

the same thing as meconium plug syndrome.

15:58

So.

15:59

Three different names for the same entity.

16:01

Um, I hate the term meconium plug syndrome because,

16:04

uh, there's absolutely no relation to cystic fibrosis.

16:08

Unlike meconium ileus, if you have a patient who

16:11

presents, um, with meconium ileus, cystic fibrosis,

16:14

um, is the underlying diagnosis until proven otherwise.

16:18

So all patients with meconium

16:19

ileus will get worked up for CF.

16:22

This enema appearance.

16:24

Uh, you can see in the sighting of total colonic Hirschsprung,

16:27

but that is an incredibly rare diagnosis.

16:29

Um, meconium ileus is much more common.

16:33

Just a comparison example.

16:35

So we have an infant, a supine

16:36

frontal view of the abdomen.

16:38

You can see there's relatively

16:40

diffuse dilation of loops of bowel.

16:43

Um, this patient presented with vomiting,

16:45

not green vomiting, or bilious vomiting.

16:48

And also the history is important in this one.

16:50

This patient was an infant of a diabetic mother.

16:53

So we went to water-soluble

16:54

contrast enema in this infant.

16:56

And you can see there is a transition

16:58

point at the level of the splenic flexure.

17:01

So this is the example that, um, you'll see

17:03

in the setting of functional immaturity of the

17:05

colon, which is also called small left colon.

17:08

Um, it is common in patients of, uh, infants of diabetic

17:11

mothers and in patients who were treated, uh, who were.

17:15

Born to a mother who was treated

17:16

prior to birth with magnesium.

17:18

Um, the classic fluoroscopy finding is

17:20

that transition from small to bigger colon

17:23

at the level of the splenic flexure.

17:26

All right, now this one is for you guys.

17:30

So we have another abdomen radiograph, and

17:34

this one is a little bit tricky, so we have a

17:36

little bit of a blown-up view of the same x-ray.

17:43

And let's go to the question, which has a nice

17:46

arrow sign for you, what is marked by the arrow?

17:59

So this one is a little bit tricky to

18:01

see, but you guys are mostly right.

18:03

So there is this super subtle little rim of

18:06

calcification that that arrow is pointing to, and it's

18:09

right at that, um, area in the abdomen where you don't

18:13

see a whole bunch of bowel gas and there's a dilated.

18:15

Single dilated loop of bowel.

18:17

So this is the clot meconium pseudocyst that is,

18:20

um, it, they describe it as an eggshell calcification

18:23

and it's related to in utero bowel perforation.

18:27

So it's a, a contained kind of

18:29

collection of meconium, if you will.

18:31

And this is most commonly seen just like

18:33

meconium ileus in the setting of cystic fibrosis.

18:37

Great job. Next case.

18:41

So this is a two-week-old, former 26-week

18:45

premature, 26-week gestational age premature infant.

18:52

What is the next step in management of this patient?

19:07

Maybe I made it too easy with that history,

19:09

but yes, you guys are totally right.

19:12

So this radiograph is showing you

19:14

classic pneumatosis intestinalis.

19:16

So it's not just modeled lucency,

19:17

it's like you would expect with stool.

19:19

There are linear, um, lucencies at the

19:23

outside of all of these loops of bowel.

19:25

So, um, in the setting of pneumatosis

19:28

intestinalis in a newborn, uh, I'm sorry, a

19:31

former premature infant in the NICU, um.

19:34

First, you categorize it as surgical versus non-surgical.

19:38

So with surgical neck, there will

19:40

be evidence of, um, perforation.

19:43

So pneumoperitoneum seen on what other,

19:45

whatever kind of imaging, um, or on exam,

19:48

there will be evidence of peritonitis.

19:50

If it's just medical necrotizing enteritis, so

19:53

no bowel perforation, you, um, stop feeding the

19:56

infant temporarily and you feed them parenterally.

19:59

You place the, place an NG tube to suction,

20:02

resuscitate with IV fluids, and then

20:04

you administer empiric antibiotics.

20:06

So great job, companion case of sorts.

20:20

What sign is present?

20:32

We have some expert, um, pediatric abdomen

20:36

radiographers in this, in this, uh, in this session.

20:39

So all of the above.

20:40

So this is a case of.

20:42

Intestinal perforation in a newborn, it can be

20:45

kind of hard to see the football sign, which is

20:47

the, the line of the falciform ligament, um, that

20:50

we see in the setting of pneumoperitoneum because

20:52

we have air on both sides of that ligament.

20:55

Whereas typically we don't see it because

20:57

there's soft tissue on both sides because

20:58

the liver is up abutting the abdominal wall.

21:01

Um, but you can kind of see it just to the right of.

21:04

The, um, umbilical venous catheter.

21:07

So we also have a continuous diaphragm sign.

21:09

The regular sign is where you see

21:10

air on both sides of the bowel.

21:12

And then there's even gas extending between

21:14

the layers of the tunica vaginalis, which is

21:17

often still patent in premature infants and, um,

21:19

infants up until the age of one year of life.

21:22

Um, so there's, uh, scrotal gas

21:24

tracking from the abdominal gas.

21:27

Great job.

21:29

Our next case is another newborn infant.

21:36

I'm gonna leave this for just a couple seconds

21:38

so you can actually see this nice and big,

21:42

and then we'll move on to the question.

21:45

What is the most likely diagnosis?

21:57

So most of you guys got this correct.

21:59

This is surfactant deficiency.

22:01

So there are a few, um, important things to look

22:03

for to be able to help you make this diagnosis.

22:06

Um, so surfactant deficiency, they will also

22:09

call respiratory distress syndrome or RDS

22:12

when they, um, shorten it to the acronym.

22:14

Um, you can tell this patient is premature.

22:16

Um.

22:17

A hint that this patient is premature,

22:20

not term, is that we don't see ossification

22:22

centers at the humeral heads on either side.

22:25

So if we don't see it, it could be

22:27

a term infant or preterm infant.

22:29

If we do see humeral head ossification

22:31

centers, we know the patient is term.

22:34

Um, other things that are helpful are, are

22:36

lung volumes, and this patient's are low.

22:38

Um, my favorite description of surfactant

22:41

deficiency is somebody threw sand on the cassette

22:44

set and then took a, took a picture of the x-ray.

22:46

So you have all these granular opacities

22:48

diffusely throughout the lungs.

22:50

Um, the other description I love with

22:52

surfactant deficiency is it's the best

22:55

air bronchograms you're ever gonna see.

22:56

So you have these beautiful central

22:58

lucencies of air bronchograms with

23:00

little tiny collapsed alveoli everywhere.

23:03

Um, next case, a one-day-old infant.

23:10

So this is the same image, but magnified

23:13

on the bottom left of your screen.

23:18

What is this most likely diagnosis?

23:31

So you guys are awesome.

23:33

This is pulmonary interstitial emphysema.

23:35

Yes.

23:36

This patient also had a pneumothorax, but

23:38

great news, that was not an answer choice.

23:40

So pulmonary interstitial emphysema can

23:42

be tricky to diagnose because it can be

23:44

hard to tell, well, is the lucency the

23:47

abnormality or is the opacity the abnormality?

23:49

But once you see, um, uh, it's almost like air

23:52

bronchograms because there's air traveling along

23:54

the interstitial and along the lymphatics.

23:57

Um, the etiology is thought to be related to stiff

23:59

lungs with the positive pressure ventilation.

24:01

So alveolar ruptures, and then it tracks along

24:03

the interstitial, same infant, different question.

24:09

Where is the tip of the catheter marked by the arrow?

24:23

So the answer here is the aorta.

24:26

So number one, this catheter is on the

24:28

left side of the patient for the most part.

24:31

Um, you can also see, I'll just go back a

24:33

slide that it courses inferiorly before it,

24:36

then courses superiorly up into the, um.

24:40

Uh, central and leftward aspect of the patient.

24:43

So this is an umbilical arterial catheter.

24:46

So remember you have two umbilical arteries,

24:48

and so if you have an umbilical arterial

24:50

catheter in the frontal projection, it's gonna

24:52

take that inferior swoop before it goes up.

24:54

I love this lateral view from the literature

24:57

because it shows you the course of the

24:59

umbilical artery, um, on the lateral view, so

25:01

it goes posterior and then up along the aorta.

25:05

I find there's a lot of confusion with umbilical

25:08

venous anatomy because, um, oftentimes on the frontal

25:12

projection, the UVC will be projecting over the liver.

25:15

So I'll hear people say the tip is in the IVC, and

25:18

that's not true unless it's very far superiorly.

25:20

So don't forget your umbilical vein anatomy.

25:23

First of all, you only have one umbilical vein.

25:25

It courses from the umbilical vein.

25:28

Towards the junction of the portal, um, uh, the

25:30

main portal vein, left portal vein, and then it

25:32

goes posteriorly through the ductus venosus until it

25:35

meets the, um, confluence of the hepatic veins.

25:39

And then it, it will go into the um, uh,

25:42

like IVC at the caval-atrial junction,

25:44

the lower caval-atrial junction level.

25:46

So just a reminder of important anatomy.

25:50

So this companion case, where

25:51

would we put this catheter?

25:53

So this is an example of an umbilical venous

25:56

catheter, and this is abnormally low lying.

25:59

So this is probably near the confluence of the portal

26:01

veins, possibly in the ductus venosus pretty superficially.

26:04

We care about this because depending on what they are

26:07

administering into the UVC, it can cause problems with

26:09

the liver because, um, you know, uh, some of the, uh.

26:14

Medications they give, uh, little newborns are either

26:17

super acidic, very alkaline, or very hyperosmolar.

26:21

So it can cause issues with the liver

26:22

parenchyma, and you can get collections

26:24

related to, um, these VCs within the liver.

26:29

All right.

26:29

Switching things a little bit.

26:30

So now we have a three-month-old infant

26:33

who had a prenatally diagnosed abnormality,

26:41

and let's go to the question.

26:45

What is the most likely diagnosis?

26:58

So super great.

26:59

Most of you guys got this correct diagnosis.

27:01

This is congenital lobar overinflation.

27:04

It used to be called congenital lobar emphysema, but

27:07

pathologically, there was no alveolar distraction.

27:10

So now everybody.

27:11

Uh, the correct terminology is overinflation.

27:14

Um, this looks like air trapping not true cysts,

27:18

which can help you distinguish between A-C-P-A-M-A

27:20

congenital pulmonary airway malformation, um, or

27:24

some, uh, like a sequestration type of a thing.

27:27

Um.

27:28

The left upper lobe is the most common location,

27:31

and you can see there's preserved architecture.

27:33

Each secondary pulmonary lobe is

27:35

just hyperlucent and too expanded.

27:38

We don't see the plugged airway.

27:40

That would make us say a diagnosis

27:42

of bronchial atresia and good news.

27:43

That was not an answer option.

27:45

Um, the left upper lobe again is the most

27:47

common location, followed by middle lobe

27:49

and right middle lobe and right upper lobe.

27:52

Great job.

27:54

We have another prenatal abnormality.

27:57

At this time in a seven-week-old.

28:08

So let's go to this question and

28:11

good news, the image is still there.

28:14

What is the next step in the evaluation of this infant?

28:29

So we have a little bit of a split here.

28:31

So, um, when we are working up, uh, congenital

28:35

lung abnormalities, um, the next step, um.

28:39

Postnatally, obviously, is a CT angiography, and

28:44

the reason why is we are trying to, number one,

28:47

diagnose the image, um, the abnormality, and

28:50

then also for presurgical planning, if relevant.

28:53

So, um, this, uh, chest radiograph is showing

28:56

you this, uh, lesion in the left lower lobe.

29:00

There are multiple LOEs of abnormal cystic lucency.

29:03

So, um, we are either dealing with a

29:06

congenital pulmonary airway malformation.

29:09

A sequestration or slash hybrid lesion because we know

29:13

that there's some sort of communication with the airway

29:15

since the lesion contains air, it's cystic, right?

29:19

Um, or alternatively, and much less

29:21

likely is pleural pulmonary neuroblastoma.

29:24

So, um, to distinguish CPAM from a

29:27

hybrid lesion or sequestration, uh.

29:30

We need to see that feeding

29:32

artery arising from the aorta.

29:34

And so, to do that, we do a CT angiography

29:37

closer to the time that the surgeon

29:39

potentially is gonna take the lesion out.

29:41

So right around the age of six months or so, um.

29:45

With the, uh, if the answer were pleural, pleural,

29:47

pulmonary neuroblastoma, number one, we would not

29:49

see that systemic artery that we see in that,

29:51

uh, scrolling CT image on the bottom left.

29:54

They also might give you a history that the patient

29:56

or a family member has a DICER1 mutation.

29:59

Uh, PPS are very strongly associated with DICER

30:02

1 mutation, and they have a classic evolution.

30:05

Over time, they start out cystic, and then as

30:07

the disease progresses, it becomes more solid.

30:12

Moving on to our next patient.

30:15

This is a seven-month-old who had a history

30:18

of multiple recurrent viral illnesses.

30:27

So let's go to the question.

30:30

What is the most likely diagnosis?

30:43

So we have a pretty even split

30:45

between RSV and the correct answer.

30:48

The correct answer is.

30:50

Knee-high or neuroendocrine cell hyperplasia of infancy.

30:54

And this has a pretty characteristic CT appearance where

30:57

you get ground-glass opacities in the lingula and right

31:00

middle lobe as well as centrally in both lower lobes.

31:03

Um, oftentimes if you get inhalation and

31:06

exhalation imaging with a high-resolution

31:08

chest CT, um, you can get air trapping in the,

31:11

um, non-ground-glass opacities of the lungs.

31:14

So this is one of those CT patterns that we

31:16

can actually help, um, with, um, diagnosing

31:20

interstitial lung disease in children.

31:22

And, um, it was formally known as

31:25

persistent tachypnea of the newborn.

31:27

Now it's called knee-high because there

31:30

are an increased number of neuroendocrine

31:31

cells at pathology, and the architecture is.

31:35

Uh, otherwise preserved.

31:36

Um, in the lung parenchyma, this typically presents by

31:39

the age of two, and the classic history is recurrent

31:42

respiratory illnesses or difficulty breathing.

31:45

Um, a longstanding history of respiratory problems.

31:48

Um, one thing that's interesting about this disease is

31:50

that it's typically not responsive to steroids, unlike

31:53

some of the other airway, um, like RSV and, uh, asthma.

31:58

Um, and the treatment is more oxygen and supportive.

32:00

And typically the children grow

32:02

out of this just on their own.

32:05

This is a companion case.

32:07

Remember we talked about there are,

32:09

um, subpleural cysts that we can see in

32:11

association with patients with Down syndrome.

32:14

Um, so this is the comparison example of that.

32:17

It has this classic, um.

32:19

A small cyst, it almost looks like

32:21

honeycombing, except the abnormality is

32:23

the cyst rather than the fibrotic change.

32:26

Um, the other thing that's a little bit

32:28

different about the, uh, lung growth disorder

32:30

of Trisomy 21 is that it typically involves

32:33

the anterior and medial aspects of the lung.

32:36

Um, so it's, um, involving the subpleural

32:38

regions and along the fissures and the cysts

32:40

are always small, so one to two millimeters.

32:43

So classic, um, subpleural cysts

32:45

associated with Trisomy 21.

32:50

Moving on down into the abdomen.

32:53

This is an 11-year-old girl who

32:55

presented with abdominal pain.

33:01

I'm gonna let the cinematic image play a few more times

33:14

and then we will go to the question.

33:17

What is the measured structure?

33:30

I feel like you can't have a pediatric talk

33:32

that includes anything in the abdomen without.

33:35

An appendix somewhere in there.

33:36

Um, so this is showing you an

33:38

abnormally enlarged appendix.

33:40

Um, it does not compress if you, um, use

33:43

all your resources and look at the way

33:45

the technologist has labeled the images.

33:48

The, um, split screen, right-hand side of

33:50

the split screen is labeled with compression.

33:52

So it's a non-compressible appendix.

33:54

The other thing to point out is that the mesenteric.

33:57

Fat around this appendix is edemic.

33:59

Um, which also helps us to know

34:01

this is an abnormal appendix.

34:03

And then finally on the cinematic view,

34:04

you can see that that's not an anechoic, just

34:06

complete black fluid near the appendix.

34:09

That's a little bit edemic.

34:10

So this may be a case of complicated appendicitis.

34:14

Again, it's also blind-ending.

34:17

Um, it can be confusing, um, a small bowel,

34:20

small bowel intussusception versus an appendix.

34:22

But again, you'll have more of a donut look and

34:24

you'll be able to see, um, uh, two different,

34:27

uh, Oreo cookies within itself if it's a

34:30

small bowel, small bowel intussusception.

34:32

The other thing that's helpful to distinguish

34:34

a small bowel, small bowel intussusception

34:35

from an appendix is that, um, with a

34:38

small bowel, small bowel intussusception.

34:40

Um, number one, typically that is,

34:43

um, intermittent abdominal pain.

34:46

Um, number two, you won't see that academic,

34:48

um, peri-per appendiceal or, or peri-bowel.

34:52

Um, the mesenteric fat around the abnormal

34:54

structure will not be so inflamed with the

34:55

small bowel, small bowel intussusception.

34:57

Um, the.

34:59

If you think it's a small bowel, small bowel

35:00

intussusception rather than an appendix or an ileocolic

35:03

intussusception, typically we will ultrasound, um,

35:06

to ensure that that has resolved because the vast

35:09

majority of cases, um, spontaneously resolve all by

35:12

themselves as long as there's no pathologic lead point

35:15

and the length is smaller than three centimeters.

35:19

Case number 12.

35:21

More radiographs.

35:23

So we have a brand new infant who was delivered at 32

35:28

weeks gestational age due to non-immune hydrops fetalis.

35:35

So we have a supine abdomen, radiograph of the chest

35:38

and AB radiograph of the chest and abdomen with a

35:41

zoomed in view of the left upper abdominal quadrant.

35:46

Wait, there's more.

35:49

So you have Mr. Images and I'm gonna let

35:52

that cinematic series play several times.

36:05

Last, last play through,

36:11

and we'll go to our question.

36:14

What is the most likely diagnosis?

36:29

All right, so this one I will admit is super sneaky.

36:33

So there are several things that we need to point out.

36:36

So the correct answer is congenital hemangioma.

36:40

So let's walk through these images together.

36:42

So the first thing I want you to see, let's

36:44

actually go back to the still images, if you will.

36:46

Humor me.

36:47

Um, look at this cardiac, uh, uh, heart

36:51

on the very first slide of that sinning.

36:54

Let's also go back to the chest radiograph.

36:56

There is cardiomegaly on this frontal

36:59

view that includes the chest, so.

37:01

So, um, the other things to point out, um, especially

37:05

on the, uh, coronal, which I'm gonna go back to,

37:07

apologize if I'm giving you a headache, is you can see

37:10

this little claw of liver tissue wrapping around this

37:13

very heterogeneous mass and we see this ginormous.

37:18

Um, left hepatic vein draining the structure.

37:21

So the venous drainage of a mass can be

37:24

super helpful for determining the organ

37:26

of origin in addition to that claw sign.

37:29

So let's put it all together.

37:31

We have a brand new baby.

37:32

We, um, know this infant presented with, um, hydrops.

37:37

Fetalis, so presumably.

37:38

In utero, high output heart failure.

37:41

Um, and you can see all that crazy

37:43

anasarca, uh, along the abdominal wall too.

37:45

So this patient, um, is basically in CHF.

37:48

So this is a congenital hemangioma.

37:50

So these are present at birth.

37:52

We can see them on fetal ultrasounds, fetal

37:55

MRIs, and they are largest at the time of birth.

37:59

The vast majority of congenital hemangiomas,

38:00

involute all by themselves spontaneously.

38:03

Um.

38:05

They can also cause obviously, uh,

38:07

high output heart failure related to

38:10

massive shunting within this, um, mass.

38:13

Unfortunately, there's really not any great

38:14

medical therapy for these lesions at this time.

38:17

The treatment really is only resection.

38:20

This patient was, um, fortunate because

38:22

it was a relatively pedunculated mass, and

38:24

so the surgeons resected it, which, uh,

38:26

obviously resolved all of the shunting issues.

38:29

Or if it's not amenable to resection,

38:31

um, coiling can be considered.

38:34

So I want to spend just a little bit of

38:36

time talking about the vascular anomalies.

38:38

Um, I love this graphic.

38:40

Um, based on the current is a

38:42

classification, which is back in 2016.

38:44

So this is now standard terminology.

38:47

So when we're talking about newborns with, uh,

38:50

uh, liver mass, since this is our thoracoabdominal

38:53

case-based, um, session, um, those are always gonna be

38:57

vascular neoplasms, not just vascular malformations.

39:01

Um, and the two, um.

39:03

Uh, most common by far and away

39:05

subtypes are the congenital hemangioma.

39:08

Um, so the rapidly involuting congenital

39:09

hemangioma, those are typically solitary.

39:12

Again, they're present and largest at birth.

39:14

And then the other subtype is infantile hemangioma.

39:18

More on that in a second.

39:19

One quick word about hemangioendotheliomas.

39:22

If you see that in a textbook, I kind of want you to

39:24

maybe find a more UpToDate version, something that,

39:27

one of 'em that uses the UpToDate classification.

39:30

The International Society for the Study of

39:32

Vascular Anomalies because hemangioendotheliomas are not

39:35

really a disease of newborns if they're in the liver.

39:39

So a osa, FORM, hemangioendothelioma is more

39:41

an extremity, a musculoskeletal mass that can,

39:45

if it's a visceral location, grow into

39:47

the liver, but it's not a liver primary mass.

39:51

Epitheloid hemangioendotheliomas

39:53

don't happen in babies.

39:55

So that's, that's not an option.

39:56

You'll see that in a teenager

39:57

or a younger adult.

40:00

Okay.

40:02

Companion sort of case.

40:04

A five-month-old boy, a history of prematurity and

40:08

multiple skin lesions, and on a renal ultrasound,

40:13

multiple hypoechoic liver lesions were discovered.

40:17

I'm not showing you a renal ultrasound.

40:19

I'm showing you MRI images.

40:24

So let's go to the question.

40:28

In this infant, what is the most likely diagnosis?

40:41

So most people said congenital hemangiomas.

40:44

And if you remember, usually those are solitary lesions.

40:47

Um, these are infantile hemangiomas.

40:50

If you remember in our history, let's just go back

40:53

and show you this patient had multiple skin lesions.

40:56

Um, and so we can assume I. Probably that this patient

41:01

also had multiple cutaneous infantile hemangiomas.

41:04

So infantile hemangiomas are, um, those are the

41:07

ones that are innumerable and patients will,

41:09

um, have, uh, cutaneous infantile hemangiomas.

41:12

Also, in fact, when patients have five or more

41:15

cutaneous hemangiomas, infantile hemangiomas,

41:18

it is recommended that they go, they undergo

41:20

screening liver and, uh, abdomen ultrasound,

41:23

looking for visceral involvement as well.

41:26

Um, we did not see a primary tumor on any of these

41:30

images, although I completely understand that.

41:33

Um, I didn't give you any cinematic images.

41:35

I didn't show you normal adrenal glands, um, to

41:38

show you that this was definitely, um, not adrenal.

41:41

Uh, neuroblastoma metastases.

41:44

Um, they like to, uh, uh, ask about the pathology

41:49

findings of infantile hemangiomas because they're

41:51

identical, whether it's cutaneous or a liver hemangioma.

41:55

So they stay in GLUT1 positive.

41:57

They are identical.

41:58

Um, a funny thing about infantile hemangiomas is that

42:02

patients can present with hypothyroidism because

42:04

these tumors present type III deiodinase,

42:09

which, um, uh, like basically nullifies the thyroid

42:12

hormone, so patients can present with hypothyroidism.

42:15

It's important to make the correct diagnosis

42:18

because, um, unlike congenital hemangiomas, uh,

42:22

there is a medical therapy for these lesions.

42:24

So, beta blockers, um, and specifically propranolol

42:28

is the treatment of choice for these infants.

42:30

So remember, um, in infants.

42:33

The two most common vascular abnormalities are

42:36

involving the liver are vascular neoplasms, and that

42:39

is congenital hemangioma if it's one lesion, and then

42:43

infantile hemangiomas if there's multiple lesions or the

42:46

patient and/or the patient has multiple skin lesions.

42:52

Home stretch y'all a little bit longer.

42:55

Hang in there.

42:56

We now have a three-year-old former

42:58

30-week gestationally age infant.

43:00

And the history that's key here is this

43:02

patient has elevated alpha fetoprotein.

43:08

Give you just a little bit to look at these images.

43:19

So our question, what is the first

43:23

step in the treatment of this mass?

43:36

So we have a tie between, well, most people picked

43:39

the correct answer, which is chemotherapy.

43:41

So this is a big, ugly heterogeneous mass.

43:45

Um, so this is a classic hepatoblastoma.

43:49

The history that's also helpful is, um, the age.

43:52

This is a three-year-old, um, and the

43:55

alpha-fetoprotein level was elevated.

43:57

So by three years of age, alpha

43:59

fetoprotein level should be normal.

44:00

They should not be high.

44:02

So, um, in the setting of a large

44:04

liver mass, it's hepatoblastoma.

44:06

So, um.

44:08

When we're working up and treating

44:10

a patient with suspected hepatoblastoma, the

44:13

first thing we would do is ask ourselves,

44:15

can we just straight up resect this?

44:16

Is this a resectable mass?

44:18

And this is way too big.

44:19

It's pushing on, um, the IVC.

44:22

It's abutting the portal bifurcation.

44:24

Um, so not, not resectable upfront.

44:27

So they would do a, uh, biopsy first.

44:30

See what subtype it is and then start chemotherapy.

44:33

The great news is hepatoblastomas are very

44:35

chemo-sensitive, so they usually shrink.

44:38

Um, and, uh, then, uh, resection would occur.

44:42

A reminder that, uh, we stage hepatoblastomas

44:46

using the PRETEXT staging system or

44:48

the pre-treatment extent of tumor.

44:51

Um, and that is because, uh, the surgeon

44:54

needs to know if it is resectable versus not.

44:57

Um, and I think of PRETEXT as the

44:59

amount of liver that will be left.

45:01

If a surgeon goes in and resects all the tumor,

45:04

so in this case, only the left side of the,

45:07

the liver would be remaining after the surgeon

45:10

goes in and takes all of this huge mass out.

45:12

Um, so this would be a PRETEXT at, uh, at least two.

45:16

Um, I have not given you enough, um,

45:18

images to be able to give it a good stage,

45:20

but all right, this is our last case.

45:24

We are almost there.

45:26

This is a 3-year-old boy who presented

45:29

with abdominal pain and a limp.

45:40

Maybe just a little bit more

45:41

time to look at this radiograph

45:48

and our question, what is represented by the arrow?

46:04

Y'all are awesome.

46:05

So the correct answer is C, abnormal

46:08

uptake related to adrenergic tissue.

46:11

So the big finding here, especially on this

46:13

radiograph, was there are abnormal calcifications

46:17

in the right upper abdominal quadrant.

46:18

So could be a liver mass, it could be a

46:22

kidney mass, it could be an adrenal mass.

46:25

On our CT, however, we see there is, um,

46:28

no claw sign of the, uh, the coronal CT

46:32

shows us no claw sign with the right kidney.

46:34

So this is a SRE primary, um, mass.

46:38

And then on our bone windows, we can see there

46:40

is a very heterogeneous appearance of bone.

46:44

So that arrow is not looking at esophageal uptake.

46:47

So it's not free tecate or something.

46:49

This is osseous metastasis.

46:51

And the other thing to recognize is.

46:53

This is an MIBG.

46:55

Um, so it's, it's not a technetium-based study.

46:59

It is, um, an I-131, uh, imaging study.

47:03

And just a reminder, in the setting of

47:05

neuroblastoma, especially bone marrow

47:07

uptake is never normal on an MIBG.

47:10

So if you can see bone, it is metastasis

47:13

in the setting of neuroblastoma.

47:15

Um, importantly in the setting of neuroblastoma, we

47:18

use a Curie score to stage these patients because

47:21

there's some evidence that, um, Curie score at

47:24

presentation and then the change with therapy

47:26

has implications for, um, long-term survival.

47:30

Um, so with Curie scoring, we break up the skeleton

47:33

into different body parts, add up all the areas that

47:37

have, um, abnormal uptake in a sign, a Curie score.

47:41

So that was my last case.

47:43

Great job, everybody.

47:44

I'm gonna look at our questions now.

47:46

Let's see.

47:48

Why not Car disease?

47:50

Okay, we're gonna go back to the,

47:51

um, case of infantile hemangiomas.

47:54

So if we thought that these multiple T2

47:57

hyperintense, uh, things throughout the

48:00

liver were, um, uh, Cohl's syndrome, we would

48:04

expect that signet ring appearance.

48:06

So there would be little, uh, low signal foci

48:10

within the, each of the little cystic spaces,

48:13

and they would be, um, coming off the bile ducts.

48:16

Um.

48:17

The other thing is these are scattered.

48:19

These are scattered all diffusely throughout the liver.

48:22

Um, this is our hepatobiliary phase, so

48:25

we have T two fat sat on the left, T one

48:29

pre, um, in the middle, and then this image

48:31

on the right is our hepatobiliary phase.

48:34

So, um, hepatocytes.

48:36

Uh, should retain the hepatobiliary contrast agent.

48:40

Um, if it was cirrhosis disease and these are like

48:43

little myelomas or something, we would expect those

48:45

to kind of have little puddles of hepatobiliary

48:48

agent, so not cirrhosis for a couple of reasons.

48:54

Let's see.

48:55

I'm gonna stop sharing my

48:56

screen so I can see the Q and A.

48:58

Oh, there we go.

48:59

Yeah, correct.

49:01

Knee-high is similar to Dip Neck.

49:03

I'm not, I'm not familiar with that acronym.

49:05

I don't see small nodules.

49:08

So knee-high neuroendocrine cell hyperplasia

49:10

of infancy is characteristic ground glass

49:13

opacities in that characteristic location.

49:15

So lingula, right?

49:17

And um, right middle lobe and then centrally

49:20

in the lower lobes with air trapping

49:22

in the dependent aspects of the lungs.

49:25

Um, so you don't typically see nodules with knee-high.

49:30

Um, what are our main differential

49:32

diagnoses with, right.

49:35

Call it gutter fluid with a normal appendix.

49:38

Uh, so you can have a small amount of

49:42

anti-coic, physiologic free fluid in

49:44

the right lower abdominal quadrant.

49:45

The case we looked at, it was too much in quantity,

49:49

so too much fluid, and it was academic, so we

49:51

knew it wasn't just normal physiologic fluid.

49:54

Um, it depends on the rest of the history.

49:56

So if you have complex fluid.

49:59

You can have, um, like a mass with, uh, could

50:02

potentially be in the right lower abdominal

50:03

quadrant, some sort of inflammatory bowel

50:06

disease impacting cecum or terminal ileum.

50:09

Um, if it's a patient with a history of cancer

50:11

and neutropenia, colitis could potentially give you

50:14

a little bit of free fluid you would expect on

50:16

ultrasound to see other associated abnormalities.

50:19

So with the mass, you would see what

50:20

looks kind of like an appendix, but a

50:22

little bit too fat and located medially.

50:24

Um, not in the right lower abdominal quadrant, titis,

50:28

you'd see a big fat cecum with Crohn's presentation.

50:32

You'd have a big fat terminal,

50:33

um, ileum, terminal ileitis.

50:37

Um, next question regarding the Devil Bubble.

50:40

Any way to tell the difference between an annular

50:42

pancreas and duodenal atresia on radiograph?

50:45

Typically with an annular pancreas, some error gets

50:49

past the second/third portion of the duodenum.

50:52

I. Um, because it's not a complete obstruction, unlike

50:54

duodenal atresia, where it's a complete obstruction.

50:57

Um, so if you can see, if you have a hugely dilated

51:01

stomach and proximal duodenum, but you see gas

51:03

distally, you could think of an incomplete obstruction.

51:06

Uh, a web could give you that appearance also.

51:08

But again, you'll see gas distally.

51:12

Um, okay.

51:15

Is biliary hematoma on the differential

51:17

diagnosis for infantile hemangioma, um, at imaging?

51:21

Yes, but the history there is key.

51:23

So if you see multiple liver lesions in the absence

51:26

of a primary tumor, so neuroblastoma worms, for

51:28

example, which are the most common, um, primary

51:31

tumors in an infant to present with mets to the liver.

51:34

Um.

51:35

Then it would be metastatic biliary

51:38

hematomas are not as common and also they're

51:40

not associated with the skin lesions.

51:43

So infantile hemangiomas on the skin really points

51:46

you towards infantile hemangiomas, um, of the liver.

51:50

In fact, some places if you have cutaneous,

51:52

infantile hemangiomas and liver lesions without a

51:55

primary tumor, they will assume that they are

51:59

infantile hemangiomas and not do further workup, um,

52:02

depending what else is going on with the patient.

52:04

So.

52:08

All right.

52:11

I think you got all the questions.

52:12

Dr. Squires, thank you so much for this case review.

52:16

That was excellent.

52:17

Awesome.

52:19

Thank you guys.

52:20

And thank you for everyone else for

52:22

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52:24

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52:30

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52:35

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