0:01

This is an adolescent with a history

0:03

of Von Hippel-Lindau syndrome,

0:04

who's had several prior resections of

0:06

cerebellar hemangioblastomas.

0:08

We can see the encephalomalacia from the

0:12

prior resection, this hypointense appearance

0:14

on T2-weighted imaging, likely related to

0:16

hemosiderin from the prior resection, and

0:20

some degree of cerebellar volume loss.

0:22

At the present time, we can see several

0:27

enhancing lesions, which, given the

0:29

history of Von Hippel-Lindau syndrome,

0:31

likely represent small hemangioblastomas.

0:36

Here's another lesion.

0:38

Here are two additional lesions and

0:41

another one in the inferior aspect

0:43

of the left cerebellar hemisphere.

0:44

Note that there is a lesion in the

0:46

region of the obex, which is for

0:49

some reason a common place for these.

0:52

Hemangioblastomas do occur

0:53

in Von Hippel-Lindau syndrome.

0:56

There are other little enhancing areas.

0:58

So some of which we have to

0:59

look to see, are they a vessel?

1:01

This looks like a vessel, but this is not.

1:05

Given the setting of Von Hippel-Lindau

1:06

syndrome, where we know there's a genetic

1:08

predisposition for developing hemangioblastomas,

1:11

every single one of these

1:14

little enhancing areas is suspicious

1:16

for an additional hemangioblastoma.

1:19

These are multicentric lesions that can have

1:21

multiple of them throughout the neural axis,

1:24

in particular the cerebellum and in the spine.

1:27

The prototypical hemangioblastoma that

1:29

is described is a cystic lesion with an

1:33

enhancing neural nodule that has some imaging

1:36

similarities to a pilocytic astrocytoma.

1:39

They very often can be a solid enhancing

1:42

lesion, especially in the spine.

1:45

Especially in the spine.

1:46

They're very often just solid enhancing lesions.

1:50

It's important to note that

1:52

these lesions enhance because of

1:54

the vascularity of the lesion.

1:57

So they have a propensity to bleed.

2:00

It is not just a regular solid tumor.

2:03

These are highly vascular lesions.

2:06

So it's important to be aware of that.

2:10

Now, if we look

2:15

the right globe, We've seen abnormality.

2:20

Well, how do we make sense of it?

2:22

Well, one of the things that can often help is

2:25

an understanding is look at what you do know.

2:27

So the left globe, we have the vitreous

2:30

here, which is hyperintense in T2-weighted

2:32

imaging, and it suppresses on FLAIR imaging.

2:37

We have a normal morphology.

2:40

In contrast, the right globe, we have several

2:45

different components, including this area

2:47

here, where it is intermediate hypointense

2:52

signal on T2-weighted imaging without any

2:54

appreciable suppression on FLAIR imaging.

2:58

We have a crescentic area of T2 hyperintense

3:00

signal that does appear to

3:02

suppress on FLAIR imaging, and we have

3:04

this hypointense interface here, and

3:09

this likely is a susceptibility artifact.

3:12

And so this is an appearance that

3:14

is seen with intravitreous silicone

3:17

injection seen after vitrectomy.

3:20

So this patient with Von Hippel-Lindau

3:22

syndrome had a right orbital lesion.

3:25

These orbital lesions typically are

3:28

evaluated with things such as fluorescein

3:30

angiography and optical coherence

3:31

tomography, which are performed by

3:35

ophthalmologists, often retina specialists.

3:37

And so the primary evaluation is typically

3:41

not from a radiologic perspective.

3:46

However, this is the post-treatment appearance

3:51

after a vitrectomy of the right globe.

3:54

So it's important to be aware of that, and

3:56

it's important to look at the globes in

3:59

patients with suspected Von Hippel-Lindau

4:01

syndrome to see if we can see some potential

4:04

enhancing component, which may result in

4:07

accelerated visits to the ophthalmologist.

4:10

But ophthalmology surveillance is, with

4:14

dilated fundoscopic exams, is one of

4:17

the key follow-up recommendations for

4:19

patients with Von Hippel-Lindau syndrome.

4:22

So this patient has multifocal multicentric

4:26

cerebellar hemangioblastomas in the setting

4:29

of several previously resected cerebellar

4:31

hemangioblastomas, as well as prior right-sided

4:35

detrectomy for a right-sided ocular lesion.