0:00

This is a 4-year-old, Dr. Shupack,

0:03

that complaints of headaches, has speech delay,

0:06

and weirdly,

0:07

I don't think we are ever able to figure it out,

0:09

has a left facial palsy.

0:11

Which didn't really fit well with the imaging,

0:13

but we do have a very large mass.

0:15

And as discussed in other vignettes,

0:16

one of the first things you want to do is decide,

0:19

is it extra-axial

0:21

or is it intra-axial?

0:23

If it's intra-axial, it may be

0:24

come from the brainstem or from the hypothalamus.

0:26

Clearly, that's not the case.

0:28

There is a plain of separation between it and the brain stem.

0:32

But on the other hand, we also want to decide,

0:35

is it of pituitary origin?

0:37

Or is it a suprasellar mass coming down?

0:39

And when you look very carefully here

0:41

and we scroll around a little bit,

0:43

there is pituitary tissue that's enhancing.

0:45

A little bit U or J-shaped in the pituitary fossa,

0:49

separate from the lesion.

0:50

So, the lesion most likely came from

0:52

the top down and grew into the sella,

0:54

grew down behind the clivus,

0:57

marginated by the membrane of Liliequist,

0:59

and it is not equal in signal intensity

1:02

to cerebrospinal fluid.

1:04

Doesn't have calcium in it.

1:05

Looks like a big protonation cystic mass.

1:08

It's bright, but not like cerebrospinal fluid.

1:11

Look at the T1 inversion recovery axial image.

1:14

It's as bright as the white matter.

1:16

Although, clearly, it is not white matter.

1:18

So, it has a very proteinaceous or blood tinge

1:22

or machine oil type of appearance.

1:24

And this is typical in a 4-year-old of adamantinoma,

1:29

the craniopharyngioma, which this is.

1:31

These are currently the first two decades of life and they

1:33

often present as this patient does,

1:36

as we'll see later on when we talk about surgery

1:38

in a separate vignette,

1:39

signs of increased intracranial pressure.

1:41

They have visual disturbances.

1:43

I'm sure this child has a visual field cut.

1:46

It's highly likely these patients,

1:48

with this size craniopharyngioma,

1:50

will have some element of pituitary hypofunction.

1:53

They may get indirect stock effect with a

1:55

little bit of prolactin elevation.

1:57

The TSH could be low.

1:59

So this could be a catastrophic scenario.

2:01

And occasionally, these can even rupture,

2:03

like dermoids, and lead to chemical meningitis.

2:06

So, this is the adenomatous form of craniopharyngioma.

2:09

The other type is the papillary form of craniopharyngioma.

2:12

And in a separate vignette,

2:14

let's talk about how to tackle this huge lesion.