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Today, we are honored to welcome Dr. Alka Singhal

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17 00:00:41,760 --> 00:00:43,920 for a lecture on ultrasound evaluation

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of fetal non-cardiac thoracic anomalies.

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Dr. Singhal has over 25 years of global radiology

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experience and is presently the Associate Director

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Radiology at Medanta, Division of Radiology and Nuclear

0:56

Medicine at Medanta Medcity Hospital, Delhi, India.

1:00

Her areas of focus include the abdomen, OB, and fetal USG

1:04

with 3D and 4D imaging, Doppler, vascular studies, neck

1:08

parathyroid, thyroid, and musculoskeletal ultrasound.

1:12

Dr. Singhal is dedicated to quality, accuracy,

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and patient satisfaction, and we're glad

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she's here today to share her expertise.

1:19

At the end of the lecture, please join Dr. Singhal

1:22

in a Q&A session where she will address

1:24

questions you may have on today's topic.

1:26

Please remember to use the Q&A feature

1:28

to submit your questions so we can get to

1:29

as many as we can before our time is up.

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With that, we're ready to begin today's lecture.

1:34

Dr. Singhal, please take it from here.

1:37

Thank you.

1:38

Warm

1:38

welcome to everyone.

1:40

So today we are going to discuss entirely different

1:44

and topic, and that's a non-fetal cardiac.

1:48

It looks like a very simple topic, and it's one of the

1:52

rare abnormalities that we see in our everyday scanning.

1:56

However, there just between the various sheets there are.

2:00

Lethal diagnosis.

2:02

There are non-lethal diagnosis.

2:03

There are diagnosis that will regress over a period of time.

2:07

So it's very important for us as ultrasound, uh,

2:10

ultrasonologist, uh, or who's remote and reporting

2:14

to have an understanding of which pathologies

2:17

are usually likely to regress and which require

2:21

intervention and which require counseling.

2:24

So let's put it in the categories, and let's look at it now.

2:28

At the outset, I'd like to thank my colleagues

2:31

who really supported me with this presentation.

2:34

Thank you so much.

2:35

So, coming to the various lung lesions,

2:38

and, uh, so we have, you can have lesions.

2:42

We are looking at the thoracic rib cage.

2:44

Now we are, what do we have there?

2:45

We have the heart, we have the lungs.

2:48

So beyond the, beyond the heart and the lungs.

2:52

Rib cage and the heart and the lungs, primarily the three.

2:55

So, coming to the thoracic cavity lesions, or the

2:58

pulmonary lesions, that's one way to classify.

3:00

We're going to currently focus mainly

3:02

on the pulmonary lesions today.

3:05

So what are the main points to understand

3:07

fetal thoracic abnormalities are?

3:10

Relatively rare, and they're easy to identify.

3:13

That's the key.

3:14

They're really easy; a simple four-chamber view in

3:18

good zoom and magnification is enough to solve the case.

3:24

And most of these lesions regress spontaneously, and

3:27

lesions which do not regress, they can be treated prenatally

3:31

or postnatally, and they happen to have good outcomes.

3:34

So let's understand.

3:36

How do we go about imaging?

3:37

As we all know, ultrasound, ultrasound, ultrasound is

3:40

the first-line imaging, and, of course, as a problem-

3:43

solving tool, we do use MRI for the next level of imaging.

3:48

Now what's a major concern?

3:50

That's one diagnosis that we definitely want

3:53

to not, uh, you know, miss, is definitely want

3:56

to make that diagnosis, is pulmonary hypoplasia.

3:59

Yeah.

4:00

Which is definitely responsible for poor perinatal

4:03

outcome, increased morbidity, and mortality.

4:06

Now, let's understand, how do we go

4:09

step by step in doing ultrasound?

4:11

What roles do ultrasound have in terms of diagnosis, in

4:15

terms of seizure follow-up, in terms of interventions,

4:19

and in terms of course, post management and other others?

4:22

Right?

4:23

So let's look at it.

4:26

So ultrasound definitely helps us in understanding the

4:29

natural course of the disease, and because, and we have to

4:33

see the lesion and whether they are static, or whether

4:36

they are progressing, or whether they're regressing.

4:40

So accordingly, you would call the patient for

4:42

a serial follow-up and take it on from there.

4:46

It has in prognosticating the lesion, whether that lesion

4:50

is the sole abnormality in the thorax and entire fetal body,

4:56

and, of course, any associated anomalies and amniotic fluid

5:01

there, thereby you are in a position to counsel the family,

5:05

uh, the couple as to what, what is the

5:08

best way going forward and where, where

5:11

required, we can do a prenatal therapy.

5:15

We can do, uh, planning of the place of delivery

5:18

and preparing neonatologists and pediatric surgeons

5:21

for appropriate management can be planned all the time.

5:25

Right?

5:26

So what are the objectives?

5:28

Most important factor is to evaluate the amount of normal

5:33

fetal tissue that is available, so we'll learn different

5:37

circumstances and areas and ratios to evaluate that.

5:41

Once you find out the pathology.

5:43

Can we determine what kind of pathology is it, and then how

5:47

much is the normal lung parenchyma that is available?

5:52

So first of all, of course, thoracic cage normality.

5:55

Skeletal normalities, we'll exclude for any, any.

5:58

Is there any restriction because of which

6:00

the lungs are not able to expand and grow normally?

6:04

An assessment of the lung hypoplasia and the

6:07

amount of normal tissue available has to be done.

6:10

Nature and the appearance of the

6:11

lung or the mediastinal lesion.

6:13

So whether there is any increased echogenicity, there's

6:16

a hyperechoic lesion, or whether there are any cysts.

6:19

Of course, we will be, in each and every case,

6:23

mediastinal shift and the dome of the diaphragm,

6:26

whether it's normal or, uh, breached or eventrated.

6:31

That's very important so that mediastinum, uh,

6:34

could be pulled or could be pushed; that we have to.

6:45

Blood supply, whenever you find any lesion,

6:48

color Doppler rule is very, very important.

6:50

We have to see whether the abnormal lesion is taking

6:54

any vascularity, is it revealing good vascularity or

6:57

not, and where is the vascularity coming from?

7:01

Is it from the pulmonary tree or is it from the systemic tree?

7:05

Because often you can have abnormalities where you

7:07

can have bowel herniation into the thorax and you can

7:10

find the SMA that is pulled upwards, and it's just a.

7:14

That may be the only cue as to what is the

7:17

abnormality, and, of course, we look at the venous

7:19

drainage and presence of hydrops, polyhydramnios,

7:23

and other associated abnormalities as well.

7:26

Now, just to understand, of course,

7:29

embryology is where we begin with.

7:31

Then we come to anatomy, then we come to

7:33

pathology, and then we come to diagnosis.

7:35

So understanding what is responsible for

7:37

the expansion of the growth of the lungs.

7:40

So, of course, fetal, uh, adequate

7:42

thoracic space in the rib cage cavity.

7:44

Then the fetal movements, the exercise.

7:48

Which stimulates every cell growth,

7:50

even for US patterns everywhere.

7:52

As we understand, the fluid in the lung, it acts as

7:55

a fluid stent, distending the developing alveoli

7:59

and allowing the capillary oxygen permeation.

8:02

So that's another very important factor.

8:04

So it may be simply less

8:05

amniotic fluid or any other factor.

8:08

There could be less expansion of the chest, or they

8:11

could be, for some reason, the fetus is not very active,

8:14

or less breathing movements could be leading to the same.

8:17

So we check for the fluid volume, we check for

8:20

the diaphragm abnormalities, and we check generally.

8:28

So what section?

8:30

Of course, we start our, uh, we've

8:32

done the BPD measurements.

8:35

We've done the head, we've done the face, we've done the

8:37

neck, or whichever way the baby presents as the anatomy.

8:40

So once we come to the chest and the thorax, of course,

8:43

we take an actual sweep from top to bottom,

8:46

and we look at the cycles, and we assess those things.

8:48

So as we are at the four-

8:50

chamber actual view, that view is very, very diagnostic.

8:55

Very, very diagnostic.

8:57

The fetal lungs are assessed by obtaining

8:59

the actual thoracic section at the level

9:02

of the four-chamber view of the heart.

9:04

Now, fetal lungs, what is the normal

9:06

echogenicity of the fetal lung?

9:08

So they're uniformly and slightly more echogenic,

9:13

more white, more hyperechoic than the liver on

9:17

the right side or the spleen on the left side.

9:20

So accordingly, you can see if the

9:22

echogenicity is slightly more, then that's okay.

9:24

But if the echogenicity is way more,

9:27

then it is probably pathological.

9:30

Of course, the fetal lung echogenicity

9:33

increases with advancing gestational age.

9:36

That also we must remember, and

9:39

it's easy to observe and, uh, see.

9:44

Once we've seen the most important striking thing

9:48

when you put your probe on the four-chamber view, apart from this,

9:51

because it's comprised of the chest, the rib cage, the shape

9:55

you've assessed, the spine, the ribs, and everything, then

9:58

we've seen the heart and the lungs, the echogenicity.

10:01

As we've done the sweep, we've, we've compared

10:04

the echogenicity with the liver and the spleen.

10:06

Now, the most important is the relative

10:09

proportion of the lung and the heart and their axis.

10:12

The heart.

10:14

So the heart normally occupies 25 to 30% of the,

10:20

um,

10:24

No, the heart normally occupies 25 to 30% of the

10:28

thoracic volume, and it's positioned in the left

10:32

anterior quadrant, the mediastinal location, right?

10:35

So the axis of the heart is important

10:38

in assessing a mediastinal shift.

10:39

So normally, the only thing that is there on the

10:42

right-hand side is just mainly the right

10:45

atrium and slightly back the right ventricle.

10:48

So when you remember this right area, and when you draw a

10:52

line from the spine to the mid epigastrium, then you can assess

10:57

the chamber orientation; that will help you understand.

11:00

What is going on, right?

11:03

So axis can be assessed, and, of course, fetal lung

11:06

volume can be calculated by using 2D, 3D, or 4D

11:09

ultrasound, which can be used to calculate, uh,

11:13

in cases of abnormalities, to assess the available

11:16

normal residual lung volume, like I discussed.

11:20

So best, we use this cross-sectional imaging, and

11:23

you see the heart, you see the, uh, uh, the, uh,

11:29

hmm.

11:30

Draw the line, and then you can see that mainly the

11:33

right atrium is on the right-hand side, and so the remaining

11:39

two-thirds is on the left and one-third on the right.

11:43

So with this, we move on to, and as we are doing the

11:47

sweep, we are going to assess the echogenicity.

11:50

So the liver and the spleen, they are normally slightly

11:53

hypoechoic as compared to the lung, and the lung is slightly

11:59

hyperechoic as compared to the liver and the spleen.

12:01

And there is a hypoechoic, well-delineated

12:06

contrast of the diaphragm that we can

12:08

appreciate, which helps us identify, right?

12:14

So that's the dome of the diaphragm,

12:16

very nicely seen on both the sides.

12:18

We see the bladder, we see the stomach, we see the heart.

12:21

We see the ribs here.

12:23

Okay?

12:24

Right.

12:25

So what are the abnormalities that we can

12:28

diagnose on ultrasound, and what is it that we

12:30

should be aware of and be looking out for?

12:34

In other words, so we could have the commoner ones,

12:37

which could be bronchopulmonary malformations, bronchial,

12:41

congenital lobar hyperinflation, foregut duplication cysts,

12:45

congenital pulmonary airway malformation, bronchopulmonary

12:48

sequestration, hybrid lesions, and Scimitar syndrome.

12:53

Other masses could be pulmonary blastoma

12:57

pulmonary blastoma, teratoma, or C-H-A-O-S.

13:01

So these ones are the lesser known.

13:04

Now, however, we being radiologists, being ultrasound

13:08

professionals, the commonest way that we tend to

13:11

identify an abnormality is in terms of the ultrasound

13:15

appearances, and then we go and see where it fits in, and

13:20

then we give possible differential diagnosis, and that

13:24

leads to a diagnosis that hints towards, so based on.

13:30

Which is more important for us to

13:32

understand what are the echogenic lesions?

13:34

The echogenic lesions are the congenital pulmonary airway malformation.

13:38

CPAM are also called congenital lobar bronchial obstruction, bronchopulmonary

13:45

sequestration, because here you have hyperechogenic

13:48

lung, then you can have hybrid lesions, and CHAOS,

13:53

or C-H-A-O-S, congenital high airway obstruction.

13:58

Right.

13:59

The non-echogenic, basically not hyperechoic, just cysts, could

14:03

be just simple foregut duplication cysts or Scimitar syndrome.

14:08

And rarely you could have mediastinal

14:10

germ tumors, or variants of that type.

14:13

Coming to the first pathology

14:16

that we discussed is bronchial atresia.

14:18

So what is bronchial atresia?

14:20

It's a narrowed or a completely blocked airway,

14:23

can occur anywhere in the branching bronchi.

14:26

So depending on the level where the

14:28

blockage occurs, the fluid can accumulate in

14:31

the lung, and the lungs can get very large.

14:33

So obviously, the higher the level of

14:35

obstruction, you’ll have much larger

14:39

distention of the lung parenchyma.

14:42

And implications, so we can have extensive lobar

14:46

involvement and presence as an echogenic lung mass.

14:49

This is still because the bronchus got blocked.

14:54

And obviously because that

14:57

is not able to decompress.

14:58

So mediastinal shift is there

15:00

to the opposite side with eversion.

15:03

It distends with a lot of fluid.

15:05

It then causes mediastinal shift

15:09

to the opposite side with eversion of the diaphragm, right?

15:13

Common locations are the apicoposterior

15:16

segment of the left upper lobe,

15:18

or right upper lobe, or right middle lobe.

15:23

Usually seen between five to 15 weeks.

15:26

It would also have arterial blood supply and

15:28

may be associated with other abnormalities.

15:31

So what do we see on ultrasound?

15:33

Hyperechogenic lung, big dilated bronchi resulting

15:37

in mediastinal shift to the contralateral side.

15:41

Coming to the next condition.

15:46

So this is also known as congenital

15:51

CLE.

15:52

It's a rare abnormality.

15:54

It can be confused with CPAM, bronchopulmonary sequestration,

15:58

or a hybrid lesion, or a congenital diaphragmatic hernia.

16:02

There are two possible factors: intrinsic.

16:04

There is an abnormal airway or

16:06

abnormal bronchial cartilage, right?

16:09

Second is extrinsic, when there's a compression

16:12

from a nearby abnormality, such as a, um,

16:16

uh, bronchogenic or any other cyst, right?

16:20

What do we see on ultrasound?

16:22

We see a hyperechogenic area, usually in the upper lobes, and

16:25

may be associated with mediastinal shift, depending on the

16:30

severity of the lesion. So, of course, the more severe the lesion,

16:34

you could also have depression of the

16:35

dome of the diaphragm, and, of course, next,

16:38

where you can have pleural effusions, hydrops.

16:41

We can have polyhydramnios and commonly associated

16:44

with other congenital heart disease.

16:47

So you can have increased echogenicity and

16:50

compressed opposite lung, severe mediastinal

16:53

shift, and, of course, flow seen on Doppler.

16:58

Now coming to

17:04

coming to CPAM.

17:05

So

17:06

one of the most important, uh,

17:09

basically, uh, areas of study and diagnosis

17:12

and differential diagnosis with many other

17:14

modalities that we keep on discussing.

17:16

What is it?

17:18

So what is it?

17:18

Basically congenital cystic adenomatoid malformation.

17:22

So that means we have got some multiple cysts.

17:25

So the cystic change that has happened

17:28

at the pulmonary parenchyma, right?

17:30

So cystic mass of pulmonary tissue with bronchial

17:33

products such as air; it's typically unilateral,

17:37

and it's typically unilateral, and only really

17:40

2 to 35% you can have bilateral, right?

17:44

So, right.

17:48

So, and it may be associated with abnormal

17:50

hematoma or dysplastic lung tissue.

17:55

So anomaly of the lung.

17:58

You have a right upper lobe.

17:59

Middle lobe and right lower lobe, and left lobes.

18:01

Got two lobes there.

18:03

So what happens in congenital pulmonary malformation,

18:07

so you have a cluster of cysts.

18:09

Depending upon how large they are, they can

18:12

displace the mediastinum to the contralateral side.

18:17

So let's look at it.

18:18

So what will you see on ultrasound?

18:20

The findings will vary depending upon what.

18:23

Depending upon the size of the cyst, like

18:27

we see adult polycystic dysplastic kidney or

18:30

multicystic dysplastic kidney, or just tiny cysts.

18:32

So similar to that, we have here in the lungs, depending

18:37

upon the size of the cyst, easy to understand, right?

18:40

So you can have microcystic, where they’re so tiny cysts

18:44

that with the naked eye you don’t see cysts.

18:48

You just see a solid-looking echogenic lesion.

18:52

So then you can have macrocystic, where you have

18:54

larger cysts that you can actually appreciate,

18:58

and you can have intermediate sizes.

19:02

Okay.

19:03

Right.

19:03

So let's just see there.

19:05

Measurements and classification.

19:07

So we have a Stocker classification.

19:10

So type I has got larger cysts, type II, so smaller

19:15

cysts, so you still see some cysts and some echogenic parenchyma.

19:20

Type I, we see cysts, and type III, where you have

19:24

microcysts, which appears as a solid echogenic lung tissue

19:29

there.

19:29

Okay.

19:30

So now let's look at type I in detail and

19:34

understand what sizes and what classifies it.

19:38

So type I is the most common,

19:41

the one with the largest cysts, right?

19:43

So seen in 70% of the cases, largest cysts, and

19:47

the sizes are between two to 10 cm.

19:50

They're pretty large, right?

19:52

To be able, you could think initially, if you just see

19:56

a solitary cyst, you could think whether it's the stomach in the

19:58

diaphragm, in the chest, or any other differential diagnosis.

20:02

That also, if it's just one, there's a

20:04

whole challenge of differential diagnosis.

20:06

But if you see many, the diagnosis

20:09

kind of points towards CPAM, right?

20:14

So they may be single or surrounded by smaller cysts, and large

20:18

CPAM will produce a mediastinal shift depending

20:20

upon the size and the volume of the pathology, and they

20:23

could be, of course, really associated with hydrops

20:26

depending upon the severity of the disease process.

20:31

So, like, as you can see here, so when you

20:35

have a large macrocystic, uh, abnormality, it's

20:39

pushing the heart to the contralateral side,

20:45

compressing it.

20:46

Okay.

20:47

So there's a large macrocystic, uh, CPAM, right?

20:53

So, so you, what do you have?

20:55

You have hyper.

20:56

So we come into the, uh, uh, CCAM type I.

21:03

So the most important differential diagnosis,

21:05

of course, when you see the cysts, it's easy.

21:08

We are looking at a CCAM.

21:11

And when you have the intermediate sizes,

21:13

then you have differential diagnosis as BPS,

21:16

bronchopulmonary sequestration.

21:18

However, when you put your color Doppler on, so the

21:21

flow is from pulmonary circulation and not from

21:24

systemic circulation, it points towards CPAM.

21:28

Okay.

21:29

So that's what a normal heart is,

21:31

and that's an echogenic heart.

21:33

That's a normal parenchyma.

21:35

That's again, we see an echogenic, and then

21:37

we see, again, we see cysts here, right?

21:41

Again, you see a heart that is being shifted, and you

21:45

see an echogenic parenchyma, some cysts as well, right?

21:50

So coming again, echogenic parenchyma with cysts.

21:56

Microcystic again.

21:58

So you have heart here, and you have echogenic lung,

22:05

and you have cysts as well.

22:08

So, like we will discuss the measurement later,

22:11

but just to show, so we measure the lung

22:15

area, and we divide it by the head circumference.

22:19

Head circumference.

22:20

We all know, we are all doing biometry.

22:23

It's simple as that.

22:24

Whether the gestational age, you measure the

22:26

head circumference, you take a note of that.

22:28

And how do we, how do we calculate area?

22:32

Area is what, length into breadth.

22:38

Right.

22:39

And then the product will give you the area.

22:42

So we will see the amount of lung tissue that you have

22:45

compressed and available, and we'll measure that, right?

22:49

So this is

22:50

microcystic

22:54

CCAM, so this assists

23:04

cyst vascular clarity.

23:14

Or is it from the

23:18

Okay,

23:18

so that's going to answer the case for you.

23:23

Right?

23:23

So coming to type II, the only difference

23:26

is now the size of the cyst is getting smaller.

23:30

We are looking at cysts of the order

23:31

of the size 0.5 to 2 centimeters.

23:34

So basically not more than 2 centimeters.

23:37

If it's more than 2 centimeters cyst,

23:39

we would classify it as type I, right?

23:43

So this is often associated with other

23:45

congenital anomalies, especially renal.

23:48

And look out.

23:50

Look out.

23:50

Look out.

23:52

That will prognosticate, that will help

23:54

you in counseling and management of that.

23:57

Pathology primarily that you are looking at, type I

24:01

and type II are also known as macrocystic type,

24:04

because you can actually see the cysts in these.

24:07

The type III that we will discuss next,

24:09

of course, we cannot see. So, of course, when the cyst

24:12

is not more than, uh, 2 centimeters, that's when we

24:17

will label it as type II, and we will, uh, calculate

24:22

normal lung volume available and

24:26

divide it by the head circumference.

24:29

Okay, so we do not measure the abnormal tissue.

24:32

We measure the normal available compressed

24:35

pulmonary tissue. Coming to type III.

24:39

So it's also called the microcystic type.

24:42

Seen in less than 10% cases of CPAM, and actually

24:47

less than five millimeters, so, which is half

24:50

a centimeter, so they cannot be distinguished

24:52

individually, and you only see a solid echogenic mass.

24:57

Typically, it involves the entire lobe

25:00

and definitely has a poorer prognosis.

25:02

And that's just an illustration of the pathology.

25:06

So you can have a pathology like this where you

25:09

have an echogenic area in the lung and mediastinal

25:17

shift, right?

25:18

So here again, you have an echogenic lung

25:21

and shift to the mediastinum, and this is the

25:26

normal right lung parenchyma that you can see.

25:32

Is the role of serial ultrasound here?

25:34

So, as we know, 26 weeks.

25:36

By 26 weeks, or, uh, by third trimester, many of these resolve.

25:42

Serial antenatal ultrasound is important, as

25:45

most of these masses regress in size.

25:48

Regression may, however, be partial or be

25:52

complete, so at every ultrasound, uh, scan, we

25:55

are going to do the lung area and we are going

25:58

to do the head circumference ratio and prognosis.

26:01

It leads to the resolution of the mediastinal

26:04

shift also as the lung lesion results in hydrops.

26:09

Of course, if it develops early before 26 weeks

26:12

and it's a poor outcome, and it's an indicator,

26:15

you may need to do fetal, uh, in utero fetal

26:19

therapy, which may be, as we'll discuss later.

26:21

It may be simply just aspirating the larger

26:23

cyst or other shunts and other procedures.

26:27

Hydrops is, of course, more common in microcystic

26:30

type, which has a poor prognosis.

26:34

Okay?

26:36

Right.

26:36

So CPAM, however, is not associated with

26:39

syndromes, and the risk of anomalies is very low.

26:42

Two common complications is what you have to see.

26:45

How much is the hydrops, and how much is

26:46

the mediastinal shift. In the absence of any

26:49

hydrops, long-term outcome is usually good.

26:55

So this is just another piece of CPAM. We have

26:59

a good view of hydrops as well.

27:03

And that's the echogenic lung

27:08

and

27:09

a cyst along.

27:12

So.

27:13

Incidence of fetal hydrops is 10%

27:16

in case of a large cystic mass.

27:18

If fetal hydrops is present, chances of survival are low.

27:21

And mediastinal shift resolves with the regression

27:24

of the mass, like we discussed for the management.

27:27

So fetal macrocystic lesions may be drained

27:31

into the fetal chest, diverting

27:34

the fluid from the cyst into the amniotic sac.

27:39

So that is called a thoraco-

27:43

amniotic shunt that you create.

27:45

Or you could just do a one-time

27:47

aspiration of the cyst and let it be.

27:51

Coming to the next.

27:52

So the prognosticating factor, like we

27:55

discussed, we are going to do the volume ratio.

27:58

So the volume of the CPAM is this ratio

28:01

divided by the head circumference.

28:03

So if CVR is more than 1.6,

28:06

chances of developing hydrops are 75%.

28:09

So you have to understand which ratios are used.

28:12

LHR is also going to come, and there is a CVR.

28:16

So don't mix the two. In CVR,

28:19

what are we measuring?

28:20

We are measuring the abnormal lung, and

28:22

we are dividing by the head circumference.

28:25

So we'll discuss the other, so when we are, obviously,

28:27

when the abnormal over the cohort or the control,

28:32

we are taking so that if the abnormal is more so,

28:35

if CVR is more than 1.6, chances of high drops.

28:39

Is, uh, high and 75%, and the prognosis is poor.

28:44

So, um, and the ratio is more than 1.6, right?

28:51

Coming to the next pathology,

28:52

which is pulmonary sequestration.

28:55

Now, what is it?

28:56

It's a, it's a, basically a FOG abnormality.

28:58

So, and these basically, uh.

29:02

We'll have the most important key factors is it'll

29:05

have an independent systemic supply, which is not seen

29:09

in CPAM, and this could be intralobar or extralobar.

29:13

Intralobar sequestration is engaged with the ULA, and

29:16

extralobar sequestration is associated with the pleural

29:22

effusion and is prone to torsion and spontaneous regression.

29:26

So what, uh, what do we mean by this?

29:29

So we, what do you mean by here?

29:30

We have, normally bronchopulmonary sequestration is an

29:33

echogenic lesion, which has vascularity from the system.

29:36

However, we see a bunch of cysts and we see echogenicity.

29:39

So it's a hybrid lesion of CCAM

29:42

and bronchopulmonary sequestration.

29:45

So we have cysts, we have echogenic lung

29:49

parenchyma, and the vascularity from the systemic

29:52

circulation.

29:54

So, uh, it can be diagnosed intrauterine, is really

29:58

diagnosed in utero. Extra could be intrathoracic or intra-

30:03

So the defined feeding vessel is very, very important.

30:07

Appropriate color Doppler settings, appropriate zoom,

30:10

appropriate sensitivity of the color Doppler, all adjustments,

30:15

the plane, keep in the area close to the skin surface.

30:19

You might have to maneuver the baby.

30:21

Uh, you might have to get the mother to roll.

30:25

So that you have your area of interest as superficial

30:28

to the skin to be able to elucidate clearly the

30:33

vascularity, because if you don't demonstrate this

30:34

vascularity right now, and however there was a

30:38

vascularity in that, that doesn't work, right?

30:43

So here comes a lot of skill and demonstration,

30:48

demonstrating the vascularity in that echogenic paradigm.

30:52

This mass is usually triangular shaped and is located, uh,

30:57

basally at the base of the chest, more often on the left side.

31:00

Most of them are small or moderate, and color

31:02

Doppler is used to demonstrate a systemic vessel.

31:05

Very, very appropriate.

31:07

Very, very important and appropriate

31:10

machine settings technique.

31:12

All will go into your experience to demonstrate this

31:16

finding, which is the key in the differential diagnosis.

31:20

Right, so here, see we want to have the area

31:24

that we want to examine here, anteriorly close.

31:28

We are measuring this abnormality, and then here we

31:32

are trying to demonstrate the vascular clarity is coming

31:35

from the systemic circulation and supplying there.

31:39

This is the key to the diagnosis.

31:42

Very, very

31:43

important.

31:45

Here you have echogenic tissue.

31:51

Scan machine settings and the flow,

31:56

right?

31:57

So in a left-sided BPS, again, you have an echogenic area.

32:00

Lesion is posterior, close to the

32:02

normal appearance stomach bubble.

32:04

Okay?

32:05

Again, you see the vascularity is coming from the systemic.

32:11

Again, another triangular red chip area

32:14

close to the spine, and then the vascularity

32:17

is coming from the systemic circulation.

32:21

That is the key.

32:22

Again, you have an echogenic area, which is taking

32:25

the vascularity from the systemic circulation.

32:30

So these are all cases of bronchial sequestration.

32:35

Right now.

32:38

In these hybrid lesions, they can be.

32:40

How do you diagnose?

32:41

Whenever we see an echo lung, we check for any mediastinal

32:45

shift, check for the blood supply to it.

32:47

And if a segment of the lung is being

32:49

supplied by the systemic circulation, we know

32:52

that we are dealing with pulmonary sequestration, right?

32:56

So again, echogenic lung, and here we have a system.

33:02

Again, that's an echogenic lung there.

33:06

And that's the feeding vessel from the systemic circulation.

33:12

See how much the technique is important?

33:17

Thank you.

33:18

So again, you have

33:25

lung and the vascularity being demonstrated.

33:28

So the

33:30

differential diagnosis for CCAM.

33:34

Common bronchial cyst or a thoracic

33:36

lymphatic cyst or bronchial atresia.

33:39

The bronchial cysts are usually in the

33:41

posterior mediastinum, usually single.

33:43

So that's why I said, so if you have multiple,

33:45

your task is easy, but you may have single, so

33:47

you have a lot of differential to put through.

33:50

Lymphatic cysts usually do have

33:53

lymphatic cysts anywhere in the body.

33:54

That's streaky kind of an appearance that

33:57

tells you that you are looking at a lymphatic

34:00

collection or cyst, and of course bronchia.

34:04

So you can have cysts in thymic bronchial cyst.

34:09

And coming to the next bronchus,

34:11

these could be either intralaryngeal or inline.

34:14

The posterior mediastinum, rarely associated with

34:17

anomalies and usually not symptomatic in neonatal

34:22

maturity, are small and located in the posterior

34:25

mediastinum, the above-defined thin-walled unilocular cyst.

34:30

Really, they could be multilocular.

34:32

And this is just another case of bronchogenic cyst.

34:37

And however, prognosis is good, karyotyping is

34:40

not recommended, and really large bronchogenic

34:44

mediastinal cysts can compress the trachea or the bronchi.

34:48

Now.

34:50

Now the next most common differential that

34:53

you will come across and you have to

34:55

do is differentiate the CPAM and Schneider.

35:01

Because in the diaphragmatic hernia you can have the abdominal

35:05

organs herniating, you can have cystic organs or

35:09

solid organs herniating into the thorax, which could

35:12

be stomach, bowel, liver, gallbladder, and others.

35:16

And then again, they could also

35:18

cause mediastinal shift and others.

35:21

So it's a challenge to differentiate.

35:23

Let's separate the two, and let's get

35:26

this very much clarified and sorted.

35:30

Right.

35:31

So to summarize, CPAM, it's rare.

35:34

It's unilateral in 97% cases due to failure of the

35:39

bronchial alveolar development, and it's got a very

35:42

low association with any aneuploidies or syndromes.

35:45

And the CVR.

35:47

That is the abnormal lung, CPAM volume ratio.

35:50

It's not the normal lung here, lesser the CPAM

35:53

volume, obviously lesser the abnormal tissue

35:56

volume, better the prognosis, better the

35:58

prognosis and occurrence of hydrops less, right?

36:03

Spontaneously resolves in 80% cases.

36:07

Management, prenatal macrocystic variant.

36:10

If you have a dominant cyst, you can aspirate

36:12

it, or you could institute a shunt, as

36:15

we discussed, outcome is usually very good.

36:18

Spontaneous regression happens usually by the third

36:21

trimester and rarely requires a postnatal resection.

36:26

Clear till now.

36:27

Now let's move on to the next diagnosis.

36:31

CDH that we are going to come to more cases.

36:35

Congenital diaphragmatic hernia is relatively more common.

36:39

It can be left-sided or right-sided or bilateral or

36:43

central, and it is due to defective development of

36:46

the diaphragm. We would understand anterior, et cetera.

36:51

It could be associated with any, and it can be.

36:57

Left or right.

36:58

Evolving lesion. Depends on the,

37:01

and basically, what do we see?

37:04

You will see abdominal viscera herniating into the

37:09

thorax, which could be cystic, like stomach or colon.

37:13

Could be solid-looking like small

37:14

bowel, and then the small bowel.

37:17

You'll often see that SMA also turns upwards into the thorax.

37:20

You can see liver, you can see the.

37:23

Portal vein branches, gallbladder, and other

37:27

structures that will tell you what's going on.

37:29

Liver comes into thorax, and of course you can have a mix

37:34

of bowel and liver, so you could be very heterogeneous

37:36

appearing and nonhomogeneous contents seen in.

37:42

Right, of course.

37:43

Additional features due to this, what is happening, you

37:47

will see upturned SMA, tortuous IVC, hepatic vessels, and

37:51

the gallbladder in the thorax, and contralateral shift of the mediastinum.

37:57

So

38:01

Revision of the ultrasound features.

38:03

We're just going to discuss the

38:04

ultrasound features of CDH.

38:07

Now, what is in CPAM? We saw the cysts.

38:10

These largest cysts we saw as hypoechoic cysts, right?

38:14

And microcystic is appearing as a solid echogenic lesion.

38:18

And of course, we have contralateral mediastinal

38:20

shift and mass effect lesion, and heart

38:22

is, of course, against the chest wall.

38:26

Now, what is happening in CDH?

38:29

So basically, you will not see the stomach in the

38:34

abdomen. The stomach is not there where it's supposed to be.

38:40

It is located in the subthoracic region here.

38:43

The stomach has gone up on the

38:45

left-sided cases, small bowel.

38:47

If you see in the thorax, how do you identify it?

38:50

If you are patient, you may be able to observe peristalsis.

38:54

You have to just put your

38:55

probe and just wait a little bit.

38:58

And then, of course, you can see the fluid

39:00

contents, and you can have an upturned SMA.

39:03

You can see the hepatic vessels in the thorax.

39:06

You can see the gallbladder in the thorax and shift, but

39:10

with moderate cardiac axis deviation, right?

39:13

Not that gross, but CPAM,

39:15

you can have gross cardiac axis deviation,

39:18

gross contralateral mediastinal shift, and

39:21

heart is almost like squeezed to the chest wall.

39:24

Of course, color Doppler, all these

39:26

vascular structures are all normal, right?

39:28

We are not having any power or anything, the threads, right?

39:33

So, so whenever you have to use the color Doppler is

39:38

a very good problem-solving tool that will help you

39:41

identify the hepatic vasculature, or see stenosis in the vessels,

39:45

and your patience to observe for any peristalsis, uh, in the.

39:52

Presumed bowel loops into the correct

39:54

that will also help you solve the case.

39:57

Let's understand the diaphragmatic hernias in more detail.

40:02

So

40:04

moving on.

40:05

So, of course, diaphragmatic hernias are more common.

40:09

They result due to the failure of closure of

40:11

the pleuroperitoneal canal, which closes at

40:14

nine to ten weeks of gestation.

40:17

So the pleural space and the peritoneal

40:20

space, they have some potential openings which

40:23

normally close during embryonic development.

40:27

Right.

40:28

And, of course, it has a high mortality rate.

40:30

Nearly 35% of, uh, and, uh, 10 to 20% are

40:34

associated with other chromosomal abnormalities.

40:37

Right.

40:38

Let's see.

40:39

So what happens is there is a breach, there is

40:43

a shift of the abdominal contents up into the.

40:47

And, of course, contralateral shift and changes.

40:51

You can see the liver in the chest.

40:53

You can see all the other organs, and depending upon

40:57

which side, okay, it can happen through posterolateral

41:02

foramen, anterior midline pericardial, or esophageal hiatus.

41:08

So these are the three places where you can have the

41:10

defect. The left-sided hernias, these are the most common.

41:15

Stomach is not seen in the normal position.

41:17

That's the most important.

41:18

What alerts you is that I can't find the stomach bubble.

41:23

I can't find the stomach.

41:25

You just first.

41:26

You simply wait.

41:27

Okay, let me wait.

41:28

Maybe it's not distended.

41:29

So I waited, and I waited.

41:31

I still can't see.

41:32

But, of course, if you would have done a, uh,

41:36

nice four-chamber view, but again, it would

41:38

also depend upon the time of the scan.

41:40

It may actually not be distended

41:42

at that time when you did this scan.

41:44

So patience is required, follow through, review just

41:48

in the 10 minutes, 20 minutes, 30 minutes.

41:52

Loops of bowel may also be seen in the chest. Left-

41:55

liver hernia may also be seen. Abdominal circumstances

41:58

less because abdominal contents move into other

42:02

abnormalities in the head, spine, and other areas.

42:07

Post-associated findings, you will see IUGR, and bladder

42:10

may be displaced because they’re all pulled up.

42:13

Polyhydramnios in most cases, more often in the third trimester.

42:18

Cardiac size is smaller.

42:19

Left-sided lung is also smaller, and what

42:22

happens in right-sided diaphragmatic hernias?

42:25

These are often less common and difficult

42:28

to detect because the echogenicity of the

42:34

lung parenchyma and the liver parenchyma.

42:37

Just a very slight subtle difference.

42:41

Visualization of the vein and the ductus venosus,

42:44

of course, is very helpful in diagnosis.

42:46

Any time when we want to identify the liver, we

42:48

just look at the portal vein and the biliary

42:51

architecture and vascular signature. Gallbladder is often

42:55

seen herniated along with the liver, hydrops.

42:59

So here you have heart, you have the normal

43:03

lung, you have echogenic lung, uh, you have the

43:06

spaced-out wall contents, abdominal contents into

43:10

the thorax, and you have the liver and the stomach.

43:14

So this is bilateral hernia.

43:19

It has to be, of course, you just

43:21

see cystic contents into the thorax.

43:23

So again, it brings us to all the differential

43:25

diagnoses of CPAM, bronchogenic cyst, thymoid cyst.

43:30

And, of course, like we discussed bowel loops, we

43:33

do observe peristalsis. Color Doppler will show

43:37

the vascularity and support prognosis, of course,

43:41

detected 24 weeks of gestation or near birth.

43:46

Uh, or earlier is poor prognosis.

43:50

So in third trimester or beyond, we have still good chances.

43:54

Large size of bowel loops are present along with the stomach.

43:57

Then, of course, it's not good prognosis.

43:59

Stomach is dilated.

44:01

Presence of liver in the chest.

44:03

It's bilateral mediastinal shift to

44:05

significant presence of ascites now.

44:10

So just a summary in a few cases.

44:13

Thanks to my friends and Khalid.

44:14

So we have a, um, left-sided diaphragmatic hernia.

44:19

You can see that you have left-sided,

44:22

uh, eventration of the diaphragm.

44:24

You have left-sided CDH.

44:26

You can just see the bowel there.

44:28

And you have bilateral CDH, and then

44:35

Okay, so then again, left-sided,

44:37

CDH with the bowel is upturned.

44:39

Upturned SMA, that you can see.

44:42

And then again, you have a right-sided diaphragmatic hernia.

44:47

There you go.

44:55

So penetration of the

44:56

diaphragm.

44:58

We have to observe the contour of the diaphragm.

45:03

So the relatively increased echogenicity of the lungs

45:06

helps us to delineate the diaphragm and evaluate it.

45:10

So that's what we observed. Brian observed

45:16

that you can just observe and diagnose.

45:18

Now, coming to the next entities, CHAOS.

45:22

C-H-A-O-S.

45:24

So congenital high obstruction of the airway due to

45:26

laryngeal or tracheal atresia, or a membrane,

45:31

leads to accumulation of fluid into the tracheal

45:33

bronchial tree and enlargement of the lung.

45:36

Here is congenital airway obstruction

45:40

syndrome causing high obstruction.

45:43

Obviously, it leads to large, hyperechoic lungs.

45:52

Basically, you just see like a very narrow,

45:55

like a butterfly sign, or what do you say?

45:58

So small centrally placed heart and large

46:01

echogenic lungs on the side, dilated bronchi.

46:05

These are the two dilated bronchi and flattened

46:08

domes of the diaphragm because of large expansion

46:11

of the lungs and pushing of the heart as well.

46:14

So congenital high airway obstruction.

46:17

So again, like I said, the heart appears to be

46:19

small, elongated, without abnormal

46:22

cardiac axis, like almost zero axis.

46:25

The heart is squeezed in between the two echogenic lungs,

46:27

almost compressed, and other features of CHAOS are there.

46:32

Polyhydramnios can also be seen.

46:35

So this is just an illustration of a case.

46:39

So if you can just see, so, uh, the heart is almost

46:42

like a straight axis, and, uh, two echogenic lungs,

46:45

they're just squeezing the heart, and it's almost like an

46:50

upright.

46:52

Okay.

46:55

MRI,

46:55

of course, helps in locating the exact site of

47:00

obstruction and allows good evaluation of the larynx and the

47:03

trachea, and can be used in strategy for management as well.

47:09

Right?

47:10

So, like we said, now this is another, uh,

47:14

assessment, uh, measurement, which is called LHR,

47:17

which is the lung area to the head circumference ratio.

47:21

So what this is, this measures

47:24

to, to, uh, in CDH, what do we do?

47:29

We do the compressed normal lung tissue here.

47:32

So we are going to take two measurements to get the volume, to

47:34

get the area, and we are going to use the head parameter.

47:39

So it is lung area later to the CDH is originally obtained.

47:44

So we are trying to get the normal lung.

47:46

We're taking the product, the longest two

47:47

perpendicular linear measurements, and

47:50

it's divided by the head circumference.

47:52

So this is how we do it.

47:54

So this is the normal one, length into width, multiply

48:00

product of these two divided by the head circumference.

48:03

This is the normal one.

48:04

So this is CDH.

48:07

So lung area to head circumference ratio here.

48:13

Okay,

48:13

So again, this is how we are calculating

48:17

the lung area to head circumference ratio.

48:21

You can do it by trace method or normally with

48:25

standard two longest diameters multiplying to one another.

48:31

How do we assess it?

48:32

Assess.

48:33

So if LHR is one or less, prognosis is poor.

48:37

Poorer.

48:37

Stillbirths in the third trimester.

48:40

Such candidates, uh, require prenatal intervention.

48:45

LHR is between 1.0 and 1.4.

48:51

Oxygenation often needed.

48:52

LHR is greater than 1.4.

48:54

Prognosis is better.

48:56

I think we're getting close to 10:20.

48:58

Okay.

49:00

Right.

49:00

So like we discussed, so this is the

49:02

contralateral normal lung assumed.

49:04

So we are going to have two measurements of

49:06

this, and we have the head circumference.

49:09

And so the area of the contralateral lung is measured in the

49:12

four-chamber view with the lung close to the probe, right?

49:16

So like the universal rule of ultrasound.

49:20

So the area of interest is as close

49:22

to the skin surface as we can get it.

49:25

Standard method of measurement of the head circumference.

49:27

So this is the correct way to measure

49:29

it, not the, not when this is oblique.

49:33

Right.

49:34

Okay.

49:35

So this we already discussed, what is the summary

49:39

of the LHR ratio, and we can do fetal therapy,

49:44

which is the EXIT, to prevent hypoplasia.

49:47

So.

49:49

Fetal hydrothorax is basically, hydrops could be associated

49:54

due to various long list of differential diagnoses,

49:57

could be unilateral or bilateral and associated

50:00

with various other abnormalities, and you'll basically

50:03

see large effusions can cause a bat-wing appearance.

50:07

Unilateral will need to meet mediastinal

50:09

shift and displacement. Fetuses with hydrops

50:12

have smaller dimensions of right and left

50:14

ventricles, and this is how you would see them.

50:18

Prognosis is, uh, good if it's

50:20

not associated with other anomalies.

50:23

And last case is pulmonary hypoplasia.

50:29

Adequate lung development is essential for the postnatal

50:32

survival, so absolute decrease in lung volume, decreased

50:37

ratio of the total lung volume and the fetal body weight.

50:41

So it's rarely primary in origin,

50:44

and unilateral is more common.

50:46

Usually it's bilateral, secondary to prolonged

50:49

oligohydramnios, skeletal dysplasia, neuromuscular disorders.

50:54

And what do we do?

50:56

So what measurement do we take?

50:57

We did the thoracic parameter and

51:00

the thoracic-to-abdominal ratio less than 0.8.

51:05

Uh, is, uh, significant. Lung length and

51:08

fetal lung volume can also be measured, and

51:11

echogenicity does not correlate with lung maturity.

51:14

We have to remember that.

51:16

So that's how we just discussed.

51:19

So, of course, bilateral has got definitely no, it's fatal.

51:23

There is no prognosis.

51:26

Pulmonary hypoplasia.

51:27

Prognosis for unilateral.

51:29

Of course, you have chances of survival, though it

51:33

has a high mortality. And just some interesting cases.

51:38

So macrocystic.

51:40

This is a case of macrocystic

51:43

CPAM, where you can see cysts there.

51:46

I was just going to put them as a quiz, but they're

51:49

very easy, and the same patient, when followed up,

51:53

the cysts have regressed and was better.

51:57

So again, so do not just go on aggressive counseling.

52:01

We have to follow them through and always

52:05

draw your lines and see the answers.

52:08

It is easy.

52:10

Ask your application specialist how to do

52:12

that, and congenital diaphragmatic hernia, where

52:16

you've got all the contents in the chest.

52:18

You can see other abnormalities as well, cysts.

52:21

And then again, you go through a

52:23

long list of differential diagnoses.

52:24

The solitary cyst is the one that

52:26

gives you the longest challenge.

52:28

Multiple is always easy.

52:31

Again, so you can have a cyst to conclude

52:34

a proper diagnosis in an early stage.

52:37

Help in making good obstetrical decisions,

52:41

careful evaluation of the associated abnormalities and

52:46

chromosomal anomalies is important because it's required

52:49

for the complete prognostication, and ultrasound-guided

52:53

interventions are a possibility and more so in times to come.

52:57

Yes.

52:58

Thank you so much for your patient listening, and

53:01

I'm happy to take any questions you may have.

53:05

Thank you so much.

53:05

If anyone's got any questions, go ahead

53:07

and put those in the Q and A box right now,

53:09

and Dr. Singh will be happy to answer them.

53:12

We'll wait a minute to see if we got any.

53:17

Thank you very much.

53:19

Great.

53:20

Thank you so much for your lecture.

53:22

Um, we are, um, thrilled to have you

53:25

back on the new conference stage.

53:27

Love, love having you here.

53:28

Dr. Singh.

53:28

Oh, we have a question.

53:30

Here they come.

53:33

Okay.

53:37

Thank you so much.

53:37

The first one is a compliment.

53:39

Wonderful, ma'am.

53:40

Thank you so much.

53:41

That's wonderful.

53:43

And the second one's also.

53:44

Thank you.

53:44

And the third one.

53:48

Differentiation of pulmonary hypoplasia and eventration

53:52

of the right-sided pulmonary hypoplasia.

53:55

In eventration, you will have mediastinal shift, so, uh,

54:02

it's very, you will see the contents in the thorax.

54:05

You will see the right-sided,

54:07

you'll see the liver in the thorax.

54:09

You will, you can see the gallbladder.

54:11

You can see the portal vein radicals, and color Doppler.

54:15

Oh.

54:16

Using eventration.

54:18

Yeah.

54:18

So basically, and diaphragmatic hernia, so you can just

54:24

evaluate that.

54:26

Yeah.

54:27

Thank you.

54:28

Great.

54:29

Great.

54:30

Well, thank you so much, Dr. Singh.

54:31

Um, we've loved to have you back here.

54:34

You can access the recording of today's

54:36

conference and all our previous conferences

54:38

by creating a free MR online account.

54:41

Be sure to join us next week on Thursday, June 1st,

54:43

at 12:00 PM Eastern, featuring Dr. Jeffrey Scott

54:46

Pinnell for a lecture on cerebrospinal fluid

54:49

dynamics, leaks, and communicating hydrocephalus.

54:52

You can register for this free

54:53

lecture at mrionline.com and follow us on social

54:56

media for updates on future conferences.

54:59

Thanks again and have a great day.

55:01

Thank you.