0:00

As you've probably gathered by now,

0:03

Dr. Yousem likes twofers. He likes a bargain.

0:06

So here we have a great bargain case.

0:09

As we scroll through the sagittal T1-weighted scan,

0:12

we see multiple abnormalities on the scan,

0:15

which are best demonstrated actually on the

0:17

post-gadolinium enhanced scans.

0:19

So first off, we see that the cerebellar tonsils extend below

0:23

the level of the frame and mag

0:25

them by greater than 5.

0:27

There is crowding at the foramen magnum.

0:29

That's indicative of Chiari 1 malformation.

0:33

Now, at first blush, when we're looking at

0:35

the post-contrast...

0:36

on the pre-contrast T1-weighted scan,

0:39

we might think that this represents a cord syrinx,

0:42

associated with Chiari 1 malformation,

0:45

and that might be your initial impression.

0:47

However, we also note that there is expansion at the

0:50

cervicomedullary junction on the

0:52

pre-contrast T1-weighted scan.

0:55

All becomes more clear on the post-contrast scan.

0:58

Now we see that the patient has a cystic and

1:01

solid mass at the foramen magnum.

1:05

But there is also an additional mass seen

1:08

projecting into the fourth ventricle.

1:10

This syrinx that we see on the

1:15

sagittal post-contrast scan,

1:18

actually, as we scroll,

1:21

we note that there is a nodule of contrast

1:25

enhancement on the edge of the spinal cord,

1:29

as well as an additional area of contrast

1:32

enhancement that is external to the spinal cord.

1:38

Let's look at the axial scans through the brain.

1:42

As we look at the axial scans through the brain

1:44

and we come to the posterior fossa,

1:46

we identify that mass that was associated

1:49

with the fourth ventricle.

1:50

However, it appears to be that there are multiple masses.

1:53

We see a mass in the lateral aspect of the left

1:56

side of the spinal cord...

1:57

of the cerebellum,

1:59

which has cystic as well as solid components.

2:02

We see an additional solid mass in the left side

2:06

of the cerebellar hemisphere and

2:09

additional masses, as well.

2:11

And we also see what looks like a potential

2:13

mass affecting the temporal bone.

2:16

So, multiple masses in the cerebellum.

2:19

Our differential diagnosis includes metastatic

2:22

disease versus hemangioblastomas.

2:26

With the finding of the cyst and mural nodule appearance,

2:32

we are more likely to go with hemangioblastoma

2:35

and Von Hippel Lindau disease.

2:37

Here is the mass at the C1-C2 level

2:41

corresponding with the mass seen on the sagittal scan.

2:44

So this patient indeed has multiple hemangioblastomas,

2:49

both in the posterior fossa, as well as in the

2:52

spinal cord with cyst and solid components,

2:58

and has an incidental cerebellar tonsillar descent.

3:02

Now, that cerebellar tonsillar descent is more likely

3:05

secondary to the effect of the masses

3:08

rather than on a congenital basis,

3:10

but we will return to this issue later on.

3:13

So, Von Hippel-Lindau disease, as I stated,

3:17

one third of patients with Von Hippel-Lindau disease

3:20

will have a spinal hemangioblastoma.

3:23

One third of patients who present with spinal

3:25

hemangioblastomas end up carrying the diagnosis

3:28

of Von Hippel-Lindau disease.