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  • 1.  bilateral optic nerve pallor

    Posted 20 days ago
    Dear all,

    I am writing to seek your expertise and would appreciate any suggestions on further workup for a patient of mine.

    I am currently seeing a 63-year-old male who presented with floaters in his left eye. His pertinent history includes rheumatic fever as a child and a childhood injury to his right eye that required multiple surgeries (including cataract and glaucoma valves), which ultimately resulted in the right eye being NLP for years.

    On examination, his vision was 20/20 OS with normal color vision and normal formal visual fields; the foveal decibel was 36. However, the OCT nerve shows 62 microns for the RNFL with GCC thinning, and the nerve appeared slightly pale. IOP was normal.

    Regarding his systemic health, he underwent an aortic valve replacement and a CABG last week due to rheumatic fever damage; notably, my examination took place prior to this surgery. His CBC and lipid panel were normal. Other normal labs included ACE, RPR, FTA, Quantiferon Gold, TSH, B12, folate, MOG, and Aquaporin 4. Pertinent positive labs included an ANA with a titer of 1:40, as well as positive SSA and SSB.

    An MRI of the brain and orbits revealed mild atrophy of the optic chiasm, severe optic atrophy of the right eye due to the prior injury, and mild atrophy of the left optic nerve.

    I am unsure if there are any other necessary tests to perform at this stage or if I should simply continue to follow him for now. Your suggestions and insights would be very welcome.

    Thank you very much for your time and assistance.

    Best regards,

    Robert Bellinoff

    Robert Bellinoff, MD

    Division Head, Eye Department, Mercy Medical Group

    Site Medical Director of Midtown Medical and Surgical Specialties

     

    Mercy Medical Group

    3000 Q Street

    Sacramento, CA 95816

    (916) 733-3311 (O)

    (916) 733-3307 (F)

     

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  • 2.  RE: bilateral optic nerve pallor

    Posted 20 days ago
    Sir 
    I know what I will say sounds strange but after the era of COVID I noticed some reactivation of rheumatic fever cases with optic neuritis , in different ages including adults with known history and with cardiac affection with the rheumatologist we got them back on long acting penicillin , this case is different to have other markers positive but just before I came to Boston last week I had a case of optic neuritis with ASOT positive , RF factor positive and ACE positive , she is still under work up by immunologist but she has near history of COVID like illness , I recommended Echo heat with Doppler also 
    I think Rheumatic fever markers should be repeated with detailed history about exposure to COVID ,
    Does COVID trigger or uncover older and hidden immune conditions , I starting to believe so especially with Rheumatic fever , note that rheumatic fever still has a significant incidence in Egypt not like USA or Europe 





  • 3.  RE: bilateral optic nerve pallor

    Posted 20 days ago

    Hi Bob,

    What a tough case. It sounds like you've done an appropriate and thorough work-up.

    1) floaters: did exam or OCT sow a PVD?

    2) left optic nerve: given the normal visual function this sounds like a recovered optic neuropathy, my top differential for this is prior injury from trauma, optic neuritis, or nutritional deficiency. The next mechanism I think about I call "pre-clinical optic neuropathy"  and think about slowly progressive conditions that may cause OCT thinning before visual deficit such as glaucoma, MS, sarcoid, and perhaps certain nutritional deficiencies such as copper and folate. Lastly would be something genetic as we know that conditions like DOA can be highly variable and some genetic conditions have mild optic neuropathies.

    One thing to remember is a patient with only one eye and has optic neuropathy has an optic neuropathy on all their optic nerves and thus is equivalent to a bilateral optic neuropathy.

    I would guess that a patient like this with such an extensive history in the right eye has probably had VF and OCT in the left eye previously and reviewing them may offer some clues to progression vs stability. Is there any pattern to the GCC thinning?

    If the nerve has been stable for 5+ years on review of records I might not do any further testing, if unclear if it has been stable it might be worth doing a CT chest and lab for soluble IL-2 receptor which has better sensitivity for sarcoid. Copper deficiency is very rare as an isolated nutritional deficiency especially in the setting of normal diet and no risk factors for malabsorption.

    Best, 

    Drew



    ------------------------------
    Andrew Carey
    Associate Professor
    Wilmer Eye Institute, Johns Hopkins Medicine
    Baltimore MD
    ------------------------------



  • 4.  RE: bilateral optic nerve pallor

    Posted 20 days ago
    family history? LHON?





  • 5.  RE: bilateral optic nerve pallor

    Posted 20 days ago
    Wallerian degeneration?
    Irene Vanek
    Sent from my iPhone





  • 6.  RE: bilateral optic nerve pallor

    Posted 20 days ago
    Has the patient noted decreased vision in the seeing eye or is it just floaters? With normal acuity, color vision, and field, it's hard to get excited. I suppose you could perform a VEP to get another functional parameter to follow. Otherwise, I think you have to follow the patient. It's interesting that the color vision is normal (how tested? HRR? Ishihara? Others?)-you stated that his IOP is normal; does he have any cupping at all?

    N





  • 7.  RE: bilateral optic nerve pallor

    Posted 20 days ago
    Is he myopic? Tilted disc?  The RNFL doesn't fit with the rest of the exam. 
    Deb 
    Sent from my iPhone - sorry for any crazy autocorrect errors 





  • 8.  RE: bilateral optic nerve pallor

    Posted 20 days ago
    Dear all,

    Thank you for the quick responses and suggestions regarding this case. To provide more detail based on your questions:

    1. I have not yet asked about family history or ordered LHON genetics, but I plan to do so once the patient is discharged from the hospital.
    2. The patient is not myopic; his uncorrected visual acuity in the left eye was 20/20.
    3. The disc was not tilted; there was a shallow cup of approximately 0.5.
    4. The GCC thinning was more pronounced superiorly, with values in the low 60s superiorly and high 60s inferiorly.
    5. Color vision was tested using Ishihara plates.

    I appreciate your continued insights.

    Best regards,

    Bob

    Robert Bellinoff, MD

    Division Head, Eye Department, Mercy Medical Group

    Site Medical Director of Midtown Medical and Surgical Specialties

     

    Mercy Medical Group

    3000 Q Street

    Sacramento, CA 95816

    (916) 733-3311 (O)

    (916) 733-3307 (F)

     








  • 9.  RE: bilateral optic nerve pallor

    Posted 20 days ago
    Other than the slightly pale optic nerve. There doesn't seem to be any clinical evidence for underlying optic neuropathy other than the thinning of the nerve fiber layer. If there is cupping, this could represent pre-perimetric glaucoma. you should also look at the size of the optic nerve on the o. C t in terms of the disk area as this may be a congenitally hypoplastic nerve. I would likely just observe unless there was clearly evidence for progressive thinning of the nerve fiber layer, development of visual field loss, change in acuity, color vision, Et cetera.

    Matt 



    Sent from my Galaxy