0:00

This was a one day old infant who presented

0:03

with abdominal distension.

0:05

Um, and uh, we have this abdomen radiograph,

0:10

and you can tell there is this repo type enteric tube

0:13

with the tips projecting over the gastric body.

0:15

But we have diffused abnormal dilation

0:18

of all bowel loops throughout the abdomen.

0:20

We have a lack of bowel gass in our rectum.

0:23

Um, but if we draw our, um, our, uh, equator along

0:27

where we think the cus will be, we can see that there are

0:31

as many dilated bowel loops above the equator.

0:34

So in the northern hemisphere

0:35

as we have in the southern hemisphere.

0:36

So this is concerning for a distal BGAs obstruction.

0:40

And that's the appearance that we're gonna have, um, um,

0:43

when we have such diffuse st dilation, gaseous dilation

0:46

of bowel throughout the abdomen.

0:47

This is a distal BGAs obstruction pattern.

0:50

So this ne the next step in the workup

0:51

of this patient will be contrast enema.

0:54

So this patient came to our fluoroscopy suite

0:57

for contrast enema later that same day,

1:00

or maybe it was the next day.

1:01

We always start with a scout radiograph just to get a lay

1:05

of the land, make sure the interior tube is in the

1:07

appropriate position, and we have a similar abnormal.

1:10

So after our scout radiograph,

1:12

a tube is placed in the rectum of the infant

1:14

and water soluble contrast is slowly administered.

1:17

This is the, uh, uh,

1:19

left lateral decubitus lateral view of the rectum.

1:22

And in the setting

1:24

of suspected distal BGAs obstruction pattern, this is one

1:27

of the more important images, um, to get.

1:29

So we inject contrast

1:31

and we slowly watch the, uh, the contrast column, um, as it,

1:36

uh, moves in a retrograde manner.

1:39

Um, and this, this area right here in the rectum is

1:42

something that we're gonna pay close attention to

1:45

as we continue to fill this colon in a retrograde fashion

1:48

to see if this goes away or not.

1:50

Um, so, uh, one of the differential diagnoses

1:52

of a distal bowel gas obstructive pattern

1:55

is hirschprung disease.

1:56

And that is where you have an a ganglionic, uh,

1:59

section of rectum.

2:01

Um, so it is unable to relax

2:03

and so you'll have focal narrowing of bowel at that level.

2:07

And so as we continue to inject

2:09

or instill contrast in a retrograde fashion, we are sort

2:14

of an o bleak angle here.

2:15

We have this clear transition point.

2:17

So we have, uh, a narrowing, focal narrowing

2:20

of the rectum at this level with upstream dilation of colon.

2:24

So this is that, um, sort of funneled appearance

2:26

of colon at, uh, contrast enema in a case of from disease.

2:31

And so we'll just make sure that this never does, um,

2:34

open up very well.

2:36

It's continuing to be narrowed vocally right here

2:38

with upstream dilation.

2:41

Um, a lateral rectum filling shot.

2:43

So this is where you look at your rectal sigmoid ratio.

2:46

So normally the rectum is where

2:49

is the stool repository prior to defecation.

2:52

And so the rectum will be larger than the sigmoid colon.

2:55

In this case, the rectal sigmoid ratio is reversed,

2:58

And so the rectum is smaller than the sigmoid colon.

3:01

So this is the classic appearance with hirschprung disease.

3:05

Of course, we continue filling

3:06

to see if there are any additional areas of,

3:09

um, of narrowing.

3:11

There's a positive arrow sign showing you

3:13

that focal narrowing of the rectum at that transition

3:16

of a ganglionic to ganglionic.

3:19

Um, um, uh, rectum

3:22

and sigmoid colon will continue to fill, um, uh,

3:26

with water soluble contrast.

3:28

Um, as much as we can approximately, again,

3:31

we are making sure that this never opens up.

3:34

Um, and it's continuing to be abnormally narrowed here.

3:38

Um, patients with hirschprung disease can be cha it can be

3:41

challenging to get that contrast to reflux all the way

3:43

to the proximal ileum.

3:45

Um, but, uh, as this case is, um, so this is the case

3:49

of hirschprung disease with abnormal rectal sigmoid ratio

3:53

and dilation of, uh, sigmoid colon upstream Because of

3:57

that achy gli, uh, section of of colon.