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Granulomatous Sinusitis with IgG4-related Ophthalmic Disease

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This patient presented with left-sided proptosis

0:03

with orbital inflammation.

0:05

On the T2-weighted scan,

0:08

we see that the left globe is anteriorly placed and there is

0:14

soft tissue swelling along the lateral

0:17

and medial aspect of the globe.

0:21

What concerns us, on this case, is the opacification

0:25

of the ethmoid sinuses.

0:27

When one sees lower signal intensity tissue

0:31

within the paranasal sinuses,

0:34

one has to worry about two things:

0:37

fungal infection and neoplasm.

0:40

In this case,

0:42

we have the bright signal intensity of what we would

0:44

normally expect of secretions and inflammation

0:47

in the left ethmoid sinus.

0:49

However, there is this component,

0:51

which is of lower signal intensity

0:54

which is extending medially into the extraconal space.

1:00

This is very worrisome for aggressive inflammatory

1:05

fungal invasive sinusitis affecting the orbit.

1:11

Looking at the post-gadolinium enhanced scans,

1:17

one sees portions of the ethmoid sinus

1:20

which are non-enhancing.

1:23

Normally, we expect to see some element of mucosal

1:26

enhancement with inflammation.

1:27

But within this portion of the ethmoid sinus,

1:31

the septations between the areas of the ethmoid

1:35

air cells are not showing enhancement.

1:38

We also see that the patient has a component, which is

1:42

outside the ethmoid air cell and extending into

1:45

the medial orbit. Scrolling through this case,

1:49

we worry about necrotic ethmoid sinus bone,

1:53

which is also of concern for aggressive

1:58

invasive fungal sinusitis.

2:02

You note also that the medial rectus muscle appears

2:07

to be showing some contrast enhancement.

2:09

And when we compare the retrobulbar fat

2:12

on the left side to the right side,

2:14

we see that there is enhancement there as well.

2:17

So this patient has orbital cellulitis, as well as myositis,

2:23

as well as a periosteal abscess,

2:27

as well as findings in the ethmoid sinus

2:31

that suggest fungal infection.

2:33

This patient was biopsied on multiple times, and also

2:38

had sinus surgery. Throughout the entire time,

2:44

no fungus was ever grown.

2:46

However, there were granulomas that were identified.

2:51

Normally,

2:52

you don't see invasive fungal sinusitis in a patient who

2:56

is immunocompetent. As it happened, this patient

3:00

had Crohn's disease and was on steroids and therefore

3:03

at high risk for fungal infection.

3:07

So, the patient was still treated with amphotericin and

3:10

intravenous antibiotics for antifungal medication.

3:14

Ultimately,

3:15

the pathologists and the consensus, among the infectious

3:19

disease community, was that this represented a case of IgG4

3:24

related ophthalmic disease, associated with Crohn's

3:29

disease, as opposed to fungal infection.

3:34

Nonetheless,

3:34

I think that anytime you see low signal intensity sinus

3:39

inflammation with absence of mucosal enhancement

3:43

within the ethmoid sinus,

3:45

you should at least raise the possibility

3:47

of fungal sinusitis. In this case,

3:50

it was signed out as granulomatous sinusitis associated

3:55

with IgG4 related ophthalmic disease.

Report

Description

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Tags

Paranasal sinuses

Orbit

Non-infectious Inflammatory

Neuroradiology

Neuro

MRI

Head and Neck

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