Case #18 (08-06-2004)

[Andrew_Doan]

CC: 34 y.o. 5? 7?, 250 lbs. woman awoke with painless loss of vision OS.

HPI: She saw her local ophthalmologist who noted 1+ vitreous cells, a visual field defect OS, and a head MRI was normal. The diagnosis of retrobulbar optic neuritis was made.

Her vision OS continues to decline over 3 weeks, and she complains of photopsias x 1 week and 3 days of a frontal HA.

PMH/FH/POH: non-contributory.

EXAM
Best corrected visual acuities:20/15 OD and 20/60 OS.
Pupils: 1.5 LU RAPD OS.
VF: See Below.
EOM: Full OU. No Pain.
IOP: 18 mmHg OD, 17 mmHg OS
Anterior segment: Normal.
DFE: normal macula and periphery. 1+ Vitreous Cell OS. See photos of optic nerves below.

Automated Humphrey Visual Field OD

Automated Humphrey Visual Field OS

Goldmann Visual Field OD

Goldmann Visual Field OS

Optic Nerve Examination OD

Mild optic nerve head edema superiorly with obscuration of nerve fiber layer and vessels.


Optic Nerve Examination OS

Mild optic nerve head edema with obscuration of nerve fiber layer and vessels. There is an incidental finding of a melanocytoma and peripapillary nevus.


Feel free to discuss the following:

What tests should you order (I'll post labs when asked for them)?

What's the differential diagnosis?

What's the diagnosis?

What is the treatment of choice, surgically and/or medically?

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[optcom]

Lab tests,
IVFA
ESR
ACE
FTA-ABS, RPR
PPD

 

[Andrew_Doan]

Lab tests,
IVFA
ESR
ACE
FTA-ABS, RPR
PPD

What's your differential? And why are you ordering these tests?

 

[Andrew_Doan]

This is a difficult case. It's helpful to localize the lesion/defect before formulating a differential, i.e., is the field defect characteristic for a lesion in the retina, optic nerve, chiasm, optic tract, or brain?

Any thoughts?

 

[PBEA]

pre chiasm, nerve or retina. you said orbital CT was normal. I can't help but think the incidental finding of melanocytoma is involved causing incidental VF defect, not unlike ONH drusen. Distribution of defect doesn't make sense though. Speaking of which was Bscan done? Anyway I'd be leaning towards NAION, or for that matter what's wrong with original dx of post optic neuritis?

 

[Andrew_Doan]

pre chiasm, nerve or retina. you said orbital CT was normal. I can't help but think the incidental finding of melanocytoma is involved causing incidental VF defect, not unlike ONH drusen. Distribution of defect doesn't make sense though. Speaking of which was Bscan done? Anyway I'd be leaning towards NAION, or for that matter what's wrong with original dx of post optic neuritis?

Head and orbit MRI was done and was normal.

Retrobulbar optic neuritis in a young woman is common, but being painless is atypical.

NAION in a young woman is not common.

Optic nerve lesion could produce a VF defect like that, but it's really an enlarged blind spot with inferior and superior loss. It's difficult to have an optic nerve lesion produce that type of large, round VF defect.

With mild optic nerve edema OU, NAION would be unlikely there too b/c having both eyes affected in a young patiet is really rare.

Previous MRI did not note enhancement of optic nerves; thus, an inflammatory lesion is unlikely, which makes this unlikely to be retrobulbar optic neuritis.

With the mild optic nerve swelling OU, we thought it could be pseudotumor cerebri, which is common in young, over-weight woman. A spinal tap was done to check the opening pressure and it was normal.

We must focus now on the retina. We didn't see any retinal pathology on exam. Any thoughts what to do next to determine if the retina is not functioning? Any ideas what this is?

 

[PBEA]

ERG
IVFA

maybe BRAO

 

[Andrew_Doan]

ERG
IVFA

maybe BRAO

We did a multifocal ERG (MERG)

RIGHT EYE

LEFT EYE

 

[Andrew_Doan]

Any thoughts about the diagnosis?

 

[Eyesore]

AZOOR

 

[Andrew_Doan]

AZOOR

Yes, this is correct.

Does anyone know when you call it AZOOR vs Acute Idiopathic Blind Spot Enlargement?

I know Dr. Gass and Jampol have suggested that the white dot syndromes are a spectrum of diseases from MEWDS, AZOOR, AIBSE, MFC, etc...

It seems to me that AZOOR is the same as AIBSE.

 

[shredhog65]

did this patient eventually develop pigmentary changes?

difficult Dx!

 

[Andrew_Doan]

did this patient eventually develop pigmentary changes?

difficult Dx!

No pigmentary changes. We only saw the mild optic nerve swelling OU.

 

[Andrew_Doan]

Case posted: http://www.eyerounds.org/case18.htm