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Birt‑Hogg‑Dubé Syndrome

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0:00

Then we have birth hug debate syndrome.

0:03

The problem with birth hug debate syndrome is

0:04

that it looks very similar

0:06

to LIP lymphocytic interstitial pneumonitis.

0:09

So we're gonna see these subpleural

0:12

or para mediastinal cys actually quite characteristic.

0:15

But very commonly you also see these para

0:17

bronchovascular thin cyst as well.

0:19

It looks very similar

0:20

to lymphocytic interstitial pneumonitis.

0:24

We do see a little bit more of a tendency

0:27

for bird hook base syndrome

0:28

to have these multi septated cysts like we see here.

0:31

Or we see the air cuff sign where the center

0:33

of a vessel is being surrounded by a cyst.

0:37

But I see this in LIP as well,

0:39

so you can't hang your hat on these findings.

0:42

People have also talked about the cyst having in Bur bay

0:45

having more of a lenticular shape.

0:46

But again, I've seen that in LIP as well.

0:50

The most helpful thing with Bert Ho Bay is that Bert Ho Bay,

0:54

for some reason likes to manifest

0:56

with these cyst along the para mediastinal structures.

0:59

So here's an XI image on the left.

1:01

Here's the mid on the right.

1:02

Look how these cysts are clustering along the para

1:04

mediastinal structures here.

1:06

And so if you see diffuse cystic lung disease

1:08

where the cyst like these areas along

1:11

where the mediastinum is,

1:12

you should be putting vertically big,

1:14

very high on your differential diagnosis.

1:17

For example here, vert hook, toay syndrome, again,

1:20

we see a little bit of air cuff here,

1:22

some lenticular cyst para bronchovascular.

1:25

It could be LIP over here except more anteriorly.

1:28

We see these para mediastinal cyst.

1:30

And so we the concentration of para mediastinal cyst make

1:32

of, think of vert hook debate,

1:34

which indeed this patient had another example

1:37

here of bur hug bay.

1:38

These cyst look non-specific.

1:40

These were bas or predominant a little bit

1:42

lenticular we see there.

1:44

But non-specific, and this is a truly

1:47

non-specific pattern here.

1:48

So mild cystic lung disease at the lung bases,

1:50

I would've favored LIP.

1:52

So for me, LIP is more common in bur ho base syndrome in my

1:55

clinic, no para media sten cyst, but,

1:58

and here's the min as well.

2:00

Mins are very helpful.

2:01

So mins minimum potentially projection images, these are

2:05

as helpful for cystic lung disease as MIPS are

2:09

for diffuse nodule lung disease.

2:10

I really like them 'cause it increases the conspicuity

2:14

of all the cysts within the lung parenchyma.

2:16

But these are basal predominant but otherwise nonspecific.

2:19

So we always look in the upper abdomen.

2:21

So this patient had bilateral renal tumors.

2:24

These are gonna be likely renal cell carcinomas.

2:28

And so even if I didn't know what histology was

2:30

with these renal tumors

2:31

and with the diffuse cystic lung disease at the lung basis,

2:34

this is bird hug base syndrome until proven otherwise.

Report

Faculty

Jonathan H. Chung, MD

Professor of Radiology and Division Chief of Cardiothoracic Imaging

UCSD - University of California San Diego

Tags

Syndromes

Lungs

Chest CT

Chest

CT