0:00

So this is the background. Uh,

0:03

this is a patient who is very tall. That's why we have, uh, chest x-ray in two,

0:08

two, uh, different images,

0:11

optical region and the base.

0:16

So basically what we are seeing here is some postoperative change.

0:22

Uh, standard tummy, um, heart size, relatively okay.

0:26

But there is this density, additional density. This is the aota.

0:29

The descending AOTA is okay, but in addition to that,

0:32

there is this per spinal opacity. Uh, the patient,

0:36

patient also presented with dysphagia.

0:39

So we wanted to do a fluoro study and we did the upper,

0:44

uh, we tried to do upper gi. I've never, uh, succeeded.

0:48

So here is the contrast. We gave a contrast orally,

0:53

and this is the dilated esophagus.

0:56

This is the initial CNA loop

1:00

where you see these very dismal, uh,

1:05

esophagus. There is some intra esophagal reflux,

1:10

very abnormal dilated esophagus.

1:14

There is some normal peristalsis in the cervical portion, but the,

1:18

but distal to that, you see this dilated, uh,

1:22

very tortuous esophagus, which doesn't want to move much.

1:27

And then there is no contrast past, uh, gastroesophageal junction.

1:34

And, uh, again, I want to remember the patient has undergone,

1:39

uh, sternotomy for some reason. And then few years later,

1:44

this patient is with us, uh,

1:47

where we do not see contrast passing through the GE junction.

1:53

So let's show the questions.

1:57

So in a patient who has undergone sternotomy in the past, uh,

2:02

has isop figures looking like that.

2:05

So they definitely have some congenital problems or syndromes.

2:11

So we need to think about whether this can potentially be cancer.

2:15

Is there a tra eso fistula or web or alesia?

2:20

Cool eso vigil webs are extremely rare. Uh,

2:25

they are usually associated with congenital short, uh,

2:29

stenotic esophagus. Uh,

2:32

and the webs are usually located in the upper esophagus. Um,

2:35

the congenital stenosis is seen in lower esophagus,

2:39

so you will see very short esophagus involving the distal portion of the

2:44

esophagus. But the cleft is located in the cervical esophagus.

2:47

So a good thought that there could be a web which could potentially completely

2:51

obstruct the transit of the radi of the contrast into the stomach. However,

2:56

here we are dealing with Akia.

2:59

Um,

3:02

the correlation between the, uh,

3:05

sternotomy and Akia is because this patient had, has down syndrome,

3:11

uh, very unique, very unique association. If you know this, you know this.

3:14

If you don't, then you have to read. And now you know about this. So,

3:17

Alesia happens in patients who have down syndrome because there is some, uh,

3:22

genetic dran derangement in down syndrome with nervous,

3:26

nervous system abnormality,

3:28

which leads to functional problems in distal esophagus.

3:33

Um, as you recall, uh,

3:35

down syndrome can have esophagal Leia during Leia Hersh syndrome.

3:40

All these are obstructive in nature. However,

3:43

instead of having an organic cause of obstruction in patients who have down

3:48

syndrome, you can have functional obstruction in the form of Alesia. Uh,

3:53

the treatment is usually medical. However,

3:56

Botox injection options are op are also available nowadays,

4:00

and many patients get Botox injections.

4:02

They have to repeatedly undergo Botox injections for, um,

4:06

maintaining the patency, pneumatic dilatation and eso faco.

4:09

Myotomy is also an option if they have very thick muscular layer. Uh,

4:14

one of theologies thought to be, uh, common, uh,

4:18

in patients who have down syndrome is autoimmune disorder,

4:21

and there is a thought process that there could be some autoimmune process

4:25

involving the distal esophagus. But, um, they usually,

4:28

patients will present with, um, dysphagia for solids first followed by liquids.

4:34

So this particular patient had av canal defect and had undergone, uh,

4:38

surgery for the same, and five or six years later, he presented with, uh,

4:44

slow, uh, dysphagia, early dys, first to solids swallowed by liquids.

4:49

And uh, he was diagnosed with Akia. Okay. Okay. Thank you.