0:00

All right, next two cases are three A and three B. Cases are,

0:05

again, two, um, similar types of cases.

0:10

And uh,

0:11

case three A is zero day old female with antenatal diagnosis of renal disease

0:16

and oligo had ream and case B is zero day old male with

0:21

antenatal diagnosis of renal disease.

0:24

So let's look at the images now.

0:28

Alright, um, this is a,

0:33

this is a boy. This is a, uh, second case, actually three B.

0:38

This is a boy whose right kidney demonstrates multiple small cysts.

0:44

These look simple and there is some hy nephrotic component. As you can see,

0:48

there is dilation of the pelvis, dilated pelvis here.

0:53

That's the bladder. The bladder is super thick. This is poly catheter,

0:57

which has decompress the bladder. And now we are looking at the left kidney.

1:03

The left kidney also shows severe dilatation of the left renal pelvis.

1:09

Now the difference between the right and the left kidneys, um,

1:13

are increased ity of the renal chy as well as severe

1:18

hydronephrosis as is seen here. This is Kelly seal dilatation,

1:23

which you can see very well. However,

1:25

there are tiny cysts which are not so apparent as we are seen in the right

1:29

kidney.

1:31

So here we are dealing with both the kidneys are abnormal.

1:36

The right kidney shows multiple cysts, which are simple in nature.

1:40

The left kidney shows increased ity of the renal parenchyma with some tiny cysts

1:46

and severe HydroOne nephrosis. That's part of the ureter,

1:51

part of the left ureter, which you are saying there.

1:56

Then we will look at the girl's ultrasound,

2:02

who is also brand new baby. And this is the right kidney.

2:05

This right kidney looks exactly like the other one. Uh,

2:10

this whole right kidney is occupied by multiple cysts of varying sizes.

2:14

They all look simple. There are no, uh,

2:16

features of complications like septations, debes, et cetera.

2:20

This is the urinary bladder, which has a Foley, also decompressed.

2:26

And now we are looking at the left kidney,

2:27

which looks exactly like the right kidney and is replaced by multiple cysts of

2:31

varying sizes. Okay?

2:36

There is no in this baby.

2:40

So we are dealing with, uh,

2:42

two patients who have cysts in the kidneys or

2:46

damaged kidneys with increased immunogenicity.

2:50

Now I'm going to show you the x-ray

2:54

On the first baby you can see the lungs are hypoplastic.

2:59

There is pneumothoraxes,

3:02

and the shape of the chest is bell-shaped.

3:07

Remember, this baby was born to the mother who had oligo hyd,

3:14

and this is the second baby. Similar situation.

3:16

You have the pneumothoraxes bilaterally and the lungs look very

3:21

small in size. The size of the chest wall is also small.

3:26

So here we are dealing with two babies who have bilateral renal disease,

3:31

as well as bilateral hypoplastic lungs, bilateral pneumothoraxes.

3:36

So their lungs are not well developed because the kidneys are not functioning

3:40

well. So now let's, uh, bring the poll question.

3:47

So we have the option of pot sequence, autosomal, resistive,

3:52

polycystic kidney disease, bilateral multicystic disease,

3:55

or bilateral cystic neoplasms.

4:01

Pot sequence is the correct answer. So the babies,

4:07

um, were born to the moms who have oligo amnio.

4:12

They, they have pulmonary hypoplasia, hypoplastic lungs, bell-shaped wall,

4:16

and they present with pneumothoraxes.

4:19

As soon as the baby takes the first breath, there is not enough lung parenchyma,

4:24

and the lung expansion is short of reaching the chest wall.

4:29

So there are the most common complication is pneumothorax.

4:32

Powder sequence is consistent.

4:35

Withher amias is one of the important components.

4:38

So these babies can have club feet because there is no enough volume for them to

4:42

move in neutral. They can have contractors,

4:45

low setters and hypertherm.

4:48

Most of these babies have I U G R and the causes of, uh,

4:53

potter sequence is usually, um, the,

4:57

the renal disease, which affects both the kidneys.

5:00

So autism and ro resistive polycystic kidney disease,

5:03

posterioral valves leading to, um,

5:07

b common tract obstruction, um,

5:10

bilateral hydrosis, bilateral multicystic, dysplastic kidney,

5:15

bilateral renal agensis, and rarely twin, twin transfusion syndrome,

5:19

where one of the babies is a donor who gets the, uh,

5:23

hypoplastic lungs as well as, uh, uh, oligo hyperemia.

5:28

So the first baby, the boy had posterior valve,

5:32

and the girl has multicystic dysplastic kidneys.

5:36

Multicystic dysplastic kidneys is non heritable.

5:40

So they are unfortunate kids who get this condition. Unfortunately,

5:45

the multicystic dysplastic kidneys is not that rare and is seeing commonly

5:50

in the female, uh, female newborn babies.

5:53

Multicystic kidneys can be diagnosed in tro by ultrasound

5:58

when they're born. We usually try to do the, um,

6:02

regram to confirm there is some functioning tissue.

6:07

If there is only one unilateral multicystic dysplastic kidney that has a better

6:11

prognosis, that function, that nonfunctioning kidney usually invol.

6:16

The other kidney compensates and the patient can have normal renal function.

6:23

Now, multicystic dysplastic kidneys rarely can get infected.

6:26

They can be persistent enlargement and they can present with renal vascular

6:30

hypertension. In that case, they need to be surgically removed.

6:34

Bilateral multicystic dysplastic kidneys can be seen in patients who have

6:38

different types of syndromes like mekel, Hubert Jaws, vegar syndrome, et cetera.

6:43

When you have only one multicystic dysplastic kidney,

6:46

you have to closely look at the other kidney because the contra renal anomaly is

6:50

very common entities, especially patic junction obstruction, uh,

6:56

ent insertion of the ureter at the, uh, junction.

7:00

The junction and reflux, those are the common things.

7:05

Posterioral valves, they are seen only in boys. Again,

7:10

this is a sporadic condition. However, some in some families like uh,

7:14

h p s is seen in families. Same thing with posterioral valves. You can see,

7:20

uh, common, um,

7:21

entity in some of the families is posterioral valve presentation.

7:26

Posterioral valves can be seen in downs, babies,

7:29

and because of the other associations,

7:32

whatever the other associations are with down syndrome like breia,

7:36

cranial spinal defects, et cetera,

7:38

can be seen in patients who have posterioral valves.

7:41

And innately you can diagnose bilateral hydronephrosis and a keyhole sign

7:46

because of dilated posterior electron, because the there,

7:50

this entities happens in the second trimester of pregnancy.

7:55

The renal damage is extensive because of the constant VAs

8:00

reflux.

8:00

There is renal dysplasia increased in the cortical immunogenicity and cyst

8:04

formation. The the pathophysiology is very separate,

8:08

very different than what we see in multicystic dysplastic kidney.

8:14

Now on V C U G you can see thick,

8:16

thick trad bladder diverticular formation, dilated posterior,

8:21

which is the key feature on V C U G.

8:24

You can see reflux unilaterally or bilaterally.

8:28

Whenever you see unilateral reflux. The reason of the other urate are not being,

8:33

uh, not showing reflux, is it? It is already atretic.

8:38

You can also see peral diabetic lung,

8:41

the complications from the posterioral valves. If there is severe,

8:44

very severe obstruction that leads to ure or renal failure in a

8:49

newborn period. And because the system is obstructed,

8:52

they can have multiple infections.

8:56

This is another case. You can see there is dilated posterior ithra.

9:00

This is posterioral valve causing dilation of the posterior urethra.

9:04

There is severe reflux bilaterally.

9:07

And then you can also see a per urethra diverticula. This is in the or.

9:12

They tried to stent it and it was not possible.

9:14

This patient had to get peritoneal dialysis catheter on day

9:19

three of his life. So severe obstruction can lead to renal failure,

9:23

which needs peritoneal dialysis catheter. We cannot miss these critical cases.