0:00

I'm going to move on to another collagen

0:02

vascular disease, Ehlers-Danlos type IV.

0:04

It is the second most common type of collagen

0:07

vascular disease that causes dissection.

0:09

It's much rarer than FMD.

0:12

The patients have a characteristic faces of

0:14

large eyes, small chin and sunken cheeks,

0:17

and lobeless ears.

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They sometimes present with uterine or

0:22

intestinal rupture because they have

0:25

fragility in those organs.

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As far as the cerebrovascular system,

0:30

it tends to involve the aorta and proximal

0:33

branches. And you'll see enlarged vessels,

0:37

dissections, AV fistulas, aneurysms.

0:39

It can be diagnosed by skin biopsy.

0:42

And there are other types of Ehlers-Danlos,

0:44

but we're going to focus on

0:45

Ehlers-Danlos type IV.

0:46

And these are classic appearance of the carotid arteries.

0:49

This dolichoectatic,

0:52

irregular, kind of sausage shape appearance,

0:56

as you can see in these slides.

0:59

So now, I'm going to show you a case

1:01

of a 64 year old male with known Ehlers-Danlos

1:05

who had known previous strokes and presented

1:08

to us with right-sided weakness.

1:10

And we'll look first at the non-contrast CT

1:13

and we can see some of the old strokes.

1:14

The right PCA infarction has a

1:17

left cerebellar infarction,

1:20

also had a small left PCA

1:22

territory infarction.

1:23

Hard to see on this non-contrast CT.

1:26

I'm not seeing any hemorrhage or mass effect

1:28

or any loss of gray-white differentiation elsewhere.

1:32

So, let's take a look at the vessels.

1:35

So these are the coronal reconstructions

1:38

of the CTA,

1:39

and we're going to take a look at the vessels.

1:42

And you can see the right carotid artery

1:45

is very tortuous and mildly

1:47

dilated and irregular.

1:49

And the left internal carotid artery is

1:52

dilated and irregular and tortuous.

1:55

And then, we'll take a look at the vertebral arteries,

1:57

and you can see the right vertebral artery

2:00

basically looks like a sausage.

2:02

It's markedly enlarged and irregular,

2:05

so is the left side.

2:06

Both look pretty similar.

2:08

And this is a classic appearance

2:11

of Ehlers-Danlos.

2:14

And then, we're going to look at

2:16

the intracranial arteries,

2:17

and

2:19

these are maximal intensity projection images.

2:23

And you can see that the intracranial arteries

2:25

are also kind of dolichoectatic

2:28

and enlarged and tortuous.

2:30

So, unlike FMD, Ehlers-Danlos tends to

2:35

involve the intracranial arteries as well.

2:37

You can see that again on the coronal images,

2:40

the posterior circulation,

2:42

you can see enlargement of the vertebral

2:44

arteries and tortuosity of the basilar artery.

2:47

And again, some mild enlargement of the

2:49

distal ICAs and the proximal MCAs

2:51

and the proximal ACAs.

2:54

All right,

2:55

so let's take a look at the raw data.

2:57

So, we'll take a look at all of the vessels,

3:01

but let's start with the right common

3:04

carotid artery, and you can see,

3:07

actually, it looks pretty normal.

3:09

And then we get up to the bifurcation,

3:11

and we're going to follow that internal up,

3:14

and the internal gets smaller and then gets

3:17

enlarged, and there's a big loop,

3:19

and it's very large at the skull base.

3:21

And then, we're going to follow the left

3:23

internal carotid artery up,

3:25

and we'll look at the left common,

3:27

which looks pretty normal.

3:29

And then the left internal carotid artery is

3:31

enlarged and tortuous and irregular and

3:36

curving around just like the right side.

3:39

And then, let's look at the vertebral arteries.

3:42

So on the right side,

3:44

which are even more markedly abnormal.

3:47

So, you can see the brachiocephalic artery and the

3:51

right subclavian artery, which are tortuous.

3:54

And then the right vertebral artery

3:57

comes off here, and it's huge.

3:59

And there's this huge thrombo pseudoaneurysm.

4:03

And if you look closely

4:06

right here,

4:08

there's this vessel that comes off the

4:10

vertebral artery and then drains into the veins.

4:12

So, there's a fistula there.

4:15

And then if you follow the right vertebral

4:16

artery up, it's just tortuous.

4:18

Remember, again, that I said that foramen transversarium

4:22

are enlarged, so it tells you it's probably congenital.

4:25

There's a little flap with a pseudoaneurysm.

4:29

Again, following this right vertebral artery up,

4:31

irregular all the way up,

4:32

multiple areas of, you know, possible flaps.

4:36

Again, same thing as we follow it up, a flap.

4:40

Tortuous, irregular,

4:43

even intracranially, it's enlarged.

4:46

And then the left side,

4:47

it's going to be kind of the same thing.

4:49

So, left subclavian.

4:57

Hard to see here.

5:01

Yeah, so here's the left subclavian.

5:04

And then, here's the left vert coming off.

5:07

And it's enlarged and tortuous and it has kind

5:12

of a corkscrew appearance in some places.

5:15

Again, the enlarged foramen transversarium,

5:18

just very similar to the left side.

5:22

And then, what you'll note are all

5:23

these prominent veins.

5:24

I think that's because of the fistula below.

5:26

So, you have enlarged tortuous vessels,

5:28

get dissections, we get AV fistulas.

5:32

and this is kind of a classic appearance

5:35

of Ehlers-Danlos.

5:36

And we'll just look intracranially quickly.

5:37

If we look at the posterior circulation,

5:39

we said the verts were enlarged.

5:41

Here's the tortuous basilar artery.

5:44

The proximal PCAs are a little bit enlarged.

5:48

The rest of the PCAs look okay.

5:49

And then the right ICA,

5:51

again,

5:53

a little bit dolichoectatic,

5:55

enlarged and tortuous

5:57

going into the proximal MCA,

5:59

and then it looks more normal.

6:01

And then the left ICA,

6:02

also

6:05

same thing, mildly enlarged up into the proximal.

6:09

MCAs and proximal ACA.

6:12

So, this is a classic case of Ehlers-Danlos.