0:00

We're picking up on this 29-year-old with nausea,

0:03

vomiting, and headaches.

0:05

T1 non-contrast,

0:06

T2 and T1 with contrast,

0:08

or T1-appearing image.

0:10

And we've got a cystic mass in the posterior fossa.

0:13

It's not producing obstruction to the fourth ventricle,

0:16

important pertinent negative.

0:17

And it has a nodule.

0:19

The nodule has a little cyst in it on the T2,

0:22

and the nodule enhances on the T1, FLAIR contrast.

0:27

We also established in a prior vignette,

0:29

there is a solid subpial nodule in

0:33

the left cerebellar hemisphere.

0:34

This is the second most common appearance

0:36

of Von Hippel-Lindau,

0:38

about 33% of the time.

0:40

And the solid lesions are usually smaller.

0:41

We've got another solid lesion down low right there.

0:44

So we've got a total of three lesions.

0:47

And we established in a prior vignette that this most

0:50

likely is going to be Von Hippel-Lindau

0:52

or cerebello-retinal hemangioblastomatosis.

0:56

So tell me the criteria.

0:58

Feed me the criteria for Von Hippel-Lindau.

1:00

So the criteria for Von Hippel-Lindau include CNS

1:02

and retinal hemangioblastomas, as we discussed,

1:05

hemangioblastoma and one of the following: either renal

1:08

cysts, pancreatic cysts, hepatic cysts, epididymal cysts,

1:13

and even possibly lung cysts.

1:15

Additionally,

1:16

pheochromocytoma and renal cancer are additional criteria.

1:21

Now, there's another set of criteria related to family history.

1:23

What would be that criteria?

1:24

So, the family history in one of the following.

1:26

So, hemangioblastoma, visceral lesions,

1:30

pheochromocytoma and renal cancer.

1:33

All right.

1:33

So in this patient, we're going to focus on three areas,

1:36

and that would be

1:38

the globe,

1:40

the ear, and the cerebellum.

1:43

We've already started with the cerebellum.

1:44

So what are we looking for in the ear?

1:46

So, in the ear,

1:46

you're looking for endolymphatic sac tumor.

1:49

And what are we looking for in the globe?

1:50

Either a retinal...

1:52

mass within the globe,

1:54

possible retinal detachment,

1:56

any enhancing...

1:57

avidly enhancing lesion.

1:59

Yeah.

1:59

And so, if somebody shows up with a retinal detachment,

2:02

or say phthisis bulbi, a shrunken globe,

2:05

and they've got a cerebellar mass,

2:06

like a cystic mass with a nodule,

2:08

first thing you ought to think of is cerebello

2:11

retinal hemangioblastomatosis,

2:13

otherwise known as Von Hippel-Lindau syndrome.

2:16

Let's move on, shall we?

2:17

Let's RNP out.