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SMA Syndrome

SMA Syndrome

Released Monday, 27th May 2024
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SMA Syndrome

SMA Syndrome

SMA Syndrome

SMA Syndrome

Monday, 27th May 2024
Good episode? Give it some love!
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Episode Transcript

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0:00

One of the scariest things that patients can

0:02

come in with is a clot to the

0:04

superior mesenteric artery. In other

0:06

words, acute mesenteric ischemia. And

0:08

if you've spent any time in the hospital, you know

0:11

these patients are in a great deal

0:13

of pain, they are extremely sick, their

0:15

guts are actually dying, and they can

0:17

become so acidotic they could code. And

0:20

so it is a true emergency. And so when

0:23

you learned about acute mesenteric ischemia, it became a

0:25

condition that stayed with you. There

0:27

is a lesser known condition known

0:29

as superior mesenteric artery syndrome, a

0:31

condition that made self-known to myself

0:34

just out of the textbooks and

0:36

in the wards just the last

0:38

few weeks. And so I

0:40

wanted to share with you this case that's

0:42

going to be a teaching point in the

0:44

topic of this week's podcast. I'm Dr. Niket

0:47

Slonpaul, your friendly-neighbor internist and gastroenterologist, and we're

0:49

not going to be talking about acute mesenteric

0:51

ischemia, which is basically just an myocardial

0:53

infarction but of the gut. We're going

0:56

to be talking about SMA syndrome and

0:58

how it presents and how to recognize

1:00

it. So cue that music. So

1:15

what is SMA syndrome or superior

1:17

mesenteric artery syndrome? Well, it's basically

1:19

a rare cause of duodenal obstruction.

1:22

And basically what's happening is the

1:24

duodenum is being extrinsically compressed between

1:26

the SMA and the aorta. And

1:29

we know that it's not that common. It

1:31

was first described back in a textbook

1:33

in 1842 and then there was a

1:35

case series in 1927 and

1:38

so it was called Wilke's syndrome, but

1:40

the term SMA syndrome is a little bit

1:42

more appropriate to help understand what's going on.

1:45

Now how common is this condition? Well,

1:47

I had a 20-something year old female who came

1:49

in a few weeks ago with symptoms of

1:51

this in the outpatient clinic. And after

1:53

a lot of workup, we were able to narrow

1:55

down that it was SMA syndrome. And

1:57

It turns out that the median age is... The

2:00

about twenty three but it ranges sixteen

2:02

to thirty nine and it is more

2:04

common and see males. But. If you

2:06

what to look at some estimation of how

2:08

almond it is and what's the epidemiology of

2:10

it. It's. Under one percent.

2:13

Do not see it that often. But. When

2:15

you hear this podcast and you fantasy it, walk

2:17

through the door they're gonna know exactly what's going

2:19

on. So. Let's begin with a little

2:21

anatomy lesson that's right for don't think about

2:24

the basic science. So. You can understand why

2:26

the suckers that is the all based on

2:28

the anatomy. Path. Of physiologies

2:30

Interesting, but when it's a pure

2:32

mechanical obstruction, it's even more fascinating

2:34

because really, all it is. Is.

2:36

A problem with internal plumbing. A

2:39

third portion of the duodenal runs between

2:41

be a order and the superior mesenteric

2:43

artery. The Do a demon

2:45

typically crosses and tear to the

2:47

aorta and right around the third

2:49

lumbar vertebrae. Now, the superior mesenteric

2:51

artery arises from the frontal aspect

2:53

of the aorta and around the

2:55

level of l one in the

2:57

vertebral body. Now. We know that

2:59

it's in taste, in sandy, in lymphatic

3:02

tissues, and here's what's important. To.

3:04

Normal angle between the Smh and

3:06

the aorta is anywhere between forty

3:08

and sixty five degrees. Simply.

3:10

Because of a mesenteric sad pad the

3:13

keep the mice and separated. They.

3:15

Might be wondering, why am I talking

3:17

about a fat pat? Well it turns

3:20

out this sad part is crucial to

3:22

the path of physiology and development of

3:24

Estimates Syndrome. You see, what happens is

3:26

the single becomes narrowed to a very

3:29

significant acute angle, and when that happens,

3:31

The. Do would deem them becomes compressed

3:33

between the superior mesenteric artery and the

3:36

aorta. With that compression you end up

3:38

developing a lot of symptoms. Sports Talk

3:40

about those. So. How are your patience

3:42

with Estimates Sims I'm going to present. Will.

3:45

Primarily for gonna look like they have

3:47

a small bowel obstruction. They may

3:49

have some of the gastric discomfort

3:51

after eating be me feel full

3:53

very easily and early society they

3:55

may have nausea, bilious emphasis. and

3:57

most significantly the number one spot

4:00

in their history that you have

4:02

to confirm is significant weight loss.

4:04

Now the weight loss actually precedes

4:06

the onset of symptoms. It's actually

4:08

the most common risk factor. You

4:10

see, significant weight loss from any

4:12

number of causes like medical conditions,

4:14

psychological conditions, or even bariatric surgery

4:17

can then affect that fat pad

4:19

and change the angle between the

4:21

aorta and the SMA, allowing for

4:23

the duodenum to become more compressed.

4:26

Now our patient who had come in

4:28

several weeks ago with the same condition

4:30

also had a dramatic amount of weight

4:32

loss from some psychological factors, including grief,

4:34

as well as using osemic off-label for

4:36

weight loss. And so she presented

4:38

with a host of symptoms, nausea,

4:40

vomiting, severe discomfort after eating, and so

4:42

she stopped eating. She even developed some

4:44

cyto-phobia, which was a fear of eating,

4:47

due to the pain and nausea vomiting.

4:49

What was interesting is that the physical

4:51

exam was pretty classical. She would

4:54

actually say that when she's having symptoms, she would

4:56

lay down in the prone position or

4:58

in the left lateral position and bring her knees

5:00

up to her chest, which, if

5:02

you look in the actual literature, takes

5:05

the angle and the tension off the

5:07

mesentery and the superior mesentery artery, opening

5:09

up to space and decompressing the duodenum.

5:12

Now on physical exam, she had some

5:14

distension, she had high pitched bowel sounds,

5:16

and a percussion splash. So of course

5:19

we became concerned for an obstruction, but

5:21

she had been sent from the emergency

5:23

room multiple times after having had

5:26

endoscopies, colonoscopies, and of course, having

5:28

CT scans, which basically ruled those

5:30

things out. In addition

5:32

to that, you also want to confirm

5:34

the patient doesn't have electrolyte abnormalities simply

5:37

from all the vomiting. If

5:39

you remember from our basic science

5:41

training, a great deal of vomiting

5:43

can lead to low volumes, high

5:46

aldosterone. So they get a hypochlorimic,

5:48

hypokalemic metabolic alkalosis. Now

5:50

our patient had gone to freestanding ERs,

5:52

which primarily are like urgent care. So

5:55

she was sent to us for tertiary

5:57

referral and consultation In which we reviewed the results.

6:00

The all the imaging. And so we were able

6:02

to then get a sense of what's going on. She's.

6:04

Seen outpatient gastroenterologist to done the

6:06

procedures and found that there was

6:08

nothing that was common. And remember

6:10

that Sesame Syndrome is uncommon. And.

6:12

Is usually lower on the differential, but when

6:14

it starts to become a high index of

6:17

suspicion, you want to obviously begin with making

6:19

sure other things have been ruled out. That's.

6:21

Why the station head and does cubbies and cat

6:24

scans? Or. Was unique about her case was

6:26

that the cats can didn't pick up the finding

6:28

simply because of the nature of the angle of

6:30

her a semi and aorta. But. Typically

6:32

for the diagnostic testing for this patient, you

6:35

start with an x ray to make sure

6:37

that it's not truly an obstruction. And.

6:39

They'll also have other image and has like

6:41

ultrasound and cats kittens. But. When you're

6:44

actually driving to make the diagnosis of a

6:46

semi syndrome, the first test you can do

6:48

is an upper Gi I series which was

6:50

so a delay of passage of contrast from

6:53

the do a denim into the small bowel.

6:55

Know if you wanna go ahead and confirm

6:58

that the estimate is actually having a problem

7:00

Said cutting off the do a denim. The.

7:02

Best test that you can do is

7:04

actually see team the in Geography or

7:06

Mrm geography and in this the criteria

7:08

is going to be the following: One.

7:12

Dude in obstruction with an abrupt

7:14

cut off in the third portion,

7:16

but they're still active Paracelsus. And

7:18

a widow mesenteric angle of less than

7:20

twenty five degrees. And the

7:22

highest extinction of the do with the number ligament

7:24

of traits. All three of these are

7:26

finding that radiologist will pick up and help

7:29

you confirm the diagnosis of Estimates Syndrome now

7:31

in our patient. It was sent in but

7:33

the angle was a little bit hard to

7:36

read because of her previous surgeries and

7:38

abdominal conditions as well. But again as the

7:40

Me syndrome was diagnosis. So what are going

7:42

to do about. Will. It depends on

7:44

how they present is the patients in

7:46

the emergency room. Like this one was

7:49

the rule that obstructions. Placed nz tubes

7:51

and corrected the electrolyte abnormalities. That's the

7:53

most important component of this because you

7:55

want to stabilize the patient from a

7:57

potassium chloride standpoint. In addition,

8:00

The be fluid hydration. It's critical because

8:02

many stations mail from an acute kidney

8:04

injury from violent vomiting, the wants to

8:06

stabilize unable to go home and their

8:08

fluid an electrolyte status as corrected. The

8:11

next step in management is to use

8:13

anti metics and slow wave came less

8:15

actually the initial therapy. You can try

8:17

that through entrail nutrition support but there

8:19

are some patients who might need parental

8:22

nutrition like Tpm. Once.

8:24

Those things have been tried and if

8:26

they don't improve and there were surgical

8:28

options like mobilizing the ligaments rights or

8:31

even more significant surgeries that will wreak

8:33

aunt who are the bowels configurations and

8:35

allow for the patients have less obstructive

8:37

symptoms and gain some weight back and

8:39

with that ladies and gentlemen brings us

8:42

to end of as the May Syndrome

8:44

syndrome that sounds like an obstruction but

8:46

it's completely to the weight loss which

8:48

in most cases is a good thing

8:50

for dramatic very quick weight loss is

8:52

the most common with. Factor: I'm doctrine

8:55

kept on com your friendly neighborhood interests

8:57

and gastroenterologist. Thanks for joining the the

8:59

sleek of.

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