0:00

We're going to only go over a few

0:02

of the congenital lung lesions.

0:04

Um, many of these are diagnosed on fetal MRI

0:07

or fetal ultrasound,

0:08

and so we know to be looking for them postnatally.

0:12

Occasionally we will see an unexpected, um,

0:15

congenital lung lesion in an infant who presents

0:17

for radiographs for respiratory distress.

0:20

We're not gonna go over all of these.

0:23

The first one I wanna, uh, talk about is bronchi atresia.

0:26

So this is actually a spectrum, um,

0:28

of a completely atretic bronchus, um,

0:32

two congenital lobar over inflation.

0:34

On the, on the less severe, um, end of the spectrum,

0:37

patients with bronchi atresia have a

0:39

completely plugged airway.

0:40

If you image them prenatally, that lung is gonna be full

0:43

of amniotic fluid, so it's gonna be a fluid filled portion

0:47

of, um, eretic lung beyond the eretic bronchus.

0:51

Um, if you image them later,

0:53

you're gonna have an overinflated appearance of that lung

0:57

as the, um, amniotic fluid has drained from that, uh,

1:01

obstructed part of the lung

1:02

and it's gonna look hyperinflated.

1:04

So it's gonna look like an a congenital lobe bar over

1:06

inflation appearance.

1:08

The more common location is the left upper lobe,

1:11

and I have to say the most common presentation is not

1:14

actually in utero or in a fetus or a newborn,

1:17

but rather in an older child.

1:19

So this was a, uh, an older, uh, teenager who had, uh,

1:23

incidental finding of bronchi atresia.

1:26

You can see this mucus filled, um, eretic bronchus

1:28

and beyond that, over inflation of the lung beyond.

1:31

So this is the much more common presentation rather than in

1:34

the fetal or newborn period

1:36

where you have overinflation at the lung beyond the eretic,

1:40

um, mucus plugged, uh, bronchus.

1:42

And this is that CT correlate showing you a plugged

1:44

fluid-filled, um, bronchus

1:46

with air trapping and over inflation.

1:48

Beyond in newborns, uh, we see congenital over overinflation

1:54

as a less severe spectrum of bronchi atresia.

1:57

Um, again, because it's a similar process,

1:59

the left upper lobe is the most common location.

2:02

Um, the thought is that there's some sort of like abnormal,

2:06

um, cartilage in that airway causing air to be able

2:10

to get into that overinflated, uh, segment of lung,

2:14

but knock it out like a ball valve mechanism.

2:16

Typically these patients

2:18

with congenital low bar overinflation will present

2:20

within six months of age.

2:21

So we will see this in the newborn period.

2:25

Um, this is what it looks like on ct.

2:26

Again, it's just, uh, normal structural lung beyond

2:30

that narrowed airway, but it is overinflated in that lobe

2:35

and it, this is the most common location

2:37

of left upper lobe congenital lobe bar overinflation.

2:41

Um, just like bronchi atresia,

2:43

if you image these patients immediately after birth

2:46

or in utero, that uh, overinflated lung will be full

2:50

of amniotic fluid actually.

2:51

So it can look like an opacity when you image these patients

2:54

immediately following birth.

2:56

Um, typically these patients do well without

2:58

Having to have any sort of inter intervention.

3:01

If that lung becomes so overinflated,

3:03

it becomes symptomatic, then the patient might

3:05

have to go to lobectomy.

3:07

Uh, we're getting towards the end of our lung lesions

3:09

that we're going to review.

3:10

The next thing to talk about is A-C-P-A-M

3:13

or a congenital pulmonary airway malformation.

3:16

These used to be called congenital cystic adenoid

3:20

malformations, but pathologically,

3:21

that's not what these look like.

3:23

So they have changed the name to CPA.

3:25

Um, there are different types

3:27

of CPAs depending on the appearance of it.

3:30

Um, if it has more, uh,

3:32

like solid components versus smaller cysts

3:34

versus larger cysts.

3:35

And it's important if you have a large cyst type CPAM, um,

3:40

in a patient with a known history of the patient

3:42

or the family has a history of ER one

3:45

to make sure you put pleural preliminary blastoma in your

3:48

differential diagnosis of a large cyst type CPAM

3:51

'cause they can look identical at imaging.

3:55

Um, these are the subtypes.

3:56

I don't go crazy saying this is a type two versus a type

3:59

one I just described.

4:01

This is a large cyst type CPAM

4:03

and give a differential of pleural pulmonary blastoma

4:06

or if it's solid or a smaller cyst subtype.

4:11

Again, these can present, um, on fetal ultrasound.

4:14

So this is an example of, uh,

4:16

this is just heart here on the left side.

4:18

This is a amniotic fluid-filled congenital pulmonary airway

4:22

malformation on this fetal, um, ultrasound

4:25

and postnatally, uh, this was a large cyst subtype, uh,

4:28

congenital pulmonary airway malformation.

4:31

So these patients, we can sometimes see the abnormality on

4:35

x-ray when they are born,

4:36

but oftentimes they don't actually go to CT

4:39

for pre-surgical planning until at least six months of age.

4:42

They, they let the patient grow a little bit

4:44

to make their surgery easier

4:46

and then they will also wait

4:47

until it's not viral respiratory season.

4:50

So they won't get the CT in the winter

4:52

and then they will wait until the patient is at least six

4:54

months of age before they do their definitive preoperative

4:57

imaging unless there's something else going on clinically.

5:01

This patient, for example, had a very large cyst subtype,

5:04

um, congenital pulmonary airway malformation,

5:06

and so this patient went on to CT sooner

5:08

because of the mass effect associated with this.

5:11

Um, again, notice how yes, there are some air-filled parts

5:15

of this lung, but a lot of this is full of fluid

5:18

because this is a brand new baby.

5:19

And so the, the baby was breathing in amniotic fluid.

5:22

So these will initially be full of fluid, not air.

5:27

Contrast that with, uh, sequestration.

5:28

So bronchopulmonary sequestration don't communicate

5:31

with a tracheal bronchial tree.

5:33

Usually these are solid in appearance as opposed to, uh,

5:37

CPAs that are, have some sort of cystic component.

5:40

The most important thing to diagnose a sequestration is

5:43

to find the systemic, um, arterial blood supply.

5:47

These are much more common in the lower lobes

5:50

and it's important to distinguish between intra lobar

5:52

and extra lobar sequestration.

5:54

The extra lobar sequestration frequently will be located in

5:58

the upper abdomen and then you have to distinguish

6:00

between is this a neuroblastoma or some other sort of mass

6:03

or is this a sequestration

6:04

where the surgeons can wait a little

6:06

bit before they take it out.

6:08

Um, this is an example where you can see this solid, uh,

6:11

lesion in the lung

6:13

and you see this beautiful feeding vessel coming off

6:15

of the like junction of the thoracic

6:17

and abdominal aorta to feed this, uh, right lower lob.

6:20

So Sion, you can also have hybrid lesions,

6:24

a congenital lung lesion that has components of both.

6:27

So it will have a systemic feeding artery

6:29

and then we'll have various cystic components.

6:31

But the good news is we just have

6:32

to be descriptive with what we see.

6:35

We tell our surgeon the arterial vascular supply,

6:37

make sure there's nothing coming off from the abdominal

6:40

aorta for inferiorly

6:41

that would change their surgical approach.

6:43

And then we'll describe what the lesion itself looks like,

6:46

whether it has like large cysts, small cysts, and

6:48

or solid components.

6:50

The pathologist can tell exactly what type of lesion it is.

6:55

Last but not least, anytime you are looking at a chest x-ray

6:58

in a patient in the NICU

7:00

or elsewhere, um, you, I want you to still pay attention

7:03

to the upper abdomen.

7:04

So the last thing I do

7:05

before I close any chest x-ray is I take my mouse

7:08

and I make, make the windowing and leveling super contrasty

7:12

and I look at the upper abdomen

7:13

because you can find unexpected

7:16

pneumoperitoneum such as this case.

7:18

Don't forget that these infants are typically supine when

7:20

they're imaged portably.

7:21

This is a continuous diaphragm sign of pneumoperitoneum,

7:25

totally unsuspected in this infant who got a chest x-ray.

7:28

So we'll talk about necrotizing enteritis later,

7:31

but this is pneumatosis intestinalis On this supine view we

7:34

have a continuous diaphragm sign of pneumoperitoneum.

7:38

So don't forget to look below the diaphragm.

7:40

If you're concerned about pneumoperitoneum,

7:42

you can always request a left lateral decubitus,

7:44

or I actually prefer cross table lateral, um,

7:47

'cause these patients are supine

7:49

and then they can not move the patient to confirm

7:52

that pneumoperitoneum, um,

7:54

that we incidentally see on our chest radiograph.