0:01

This is a CT scan of the head

0:02

in a three and a half-month-old with seizures,

0:06

and they came into a community hospital,

0:10

and it was thought to have blood related to a potentially

0:16

hemorrhagic contusion or subarachnoid hemorrhage

0:19

overlying the right cerebral hemisphere.

0:21

So, they were sent to a tertiary care center.

0:26

When looking at this,

0:27

this doesn't really look like what I would

0:29

expect blood products to look like.

0:32

I'm not seeing edema.

0:34

I'm also seeing asymmetric prominence of

0:36

CSF overlying the right cerebral hemisphere.

0:39

So that doesn't, that could be related

0:42

to trauma, but it doesn't quite add up yet.

0:46

Looking at it in the MR,

0:49

here's the T2-weighted image,

0:50

we're seeing prominence of the CSF

0:53

space overlying the right cerebral hemisphere.

0:56

We're seeing a paucity of cortical

0:59

veins overlying the right cerebral

1:00

hemisphere, you can see on the left we

1:02

have a cortical vein here, here, here.

1:05

So, we're seeing a paucity of cortical veins

1:07

overlying the right cerebral hemisphere.

1:11

And we're seeing this leptomeningeal

1:16

enhancement overlying portions of the

1:17

right parietal lobe and occipital lobe.

1:20

And then in the right frontal lobe,

1:23

we're seeing these transmantle collaterals.

1:26

So, in the absence of significant cortical

1:30

veins, in this region, the venous blood

1:34

has to either go to the deep venous system

1:37

through these transmantle collaterals,

1:39

going eventually into the internal cerebral veins,

1:42

or through these leptomeningeal collaterals,

1:44

where they sort of transmit laterally or

1:47

perpendicular to the surface,

1:48

to find a place where there's some venous drainage.

1:53

All of this results in chronic venous ischemia

1:56

and congestion, resulting in volume loss

1:59

and dystrophic mineralization as seen here.

2:02

Well, we can notice

2:04

that because of those transmantle collaterals,

2:07

we're seeing some of the veins that

2:12

drain into the internal cerebral vein,

2:14

the right internal cerebral vein is bigger.

2:16

You can see the right thalamostriate

2:18

vein is much larger than the left.

2:21

Seven years later, we can see that those

2:24

veins have continued to hypertrophy.

2:26

The right internal cerebral vein remains

2:28

markedly enlarged, and this is a compensatory

2:34

mechanism, and there has not been

2:37

significant progression of the volume loss.

2:40

At this time, we still see the transmantle

2:45

collaterals in the frontal lobe.

2:46

And predominantly leptomeningeal collaterals

2:48

in the parietal and occipital lobe.

2:50

Here's where we're starting to see some transmantle

2:52

collaterals in the right temporal lobe.

2:56

So, this shows the evolution of Sturge-Weber

2:59

syndrome does not always result in severe

3:03

chronic stepwise progression of the volume loss.

3:07

Every single patient is different.

3:10

There is a different degree of involvement,

3:12

a different degree of collateral formation.

3:14

And it may be that these enlarged deep draining

3:19

veins end up decreasing the resistance and

3:23

decreasing the amount of venous congestion.

3:26

That's unclear, but what this shows is

3:29

that the evolution of Sturge-Weber syndrome

3:33

is different in every single patient.