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elevated optic discs

  • 1.  elevated optic discs

    Posted 05-15-2025 15:25

    I've seen a thin 11 years old girl with elevated optic discs a few times during the past 3 months.  Her optic disc appearances are unchanged from their appearances in the attached photos from November.  Elevated optic disc appearances were first noticed during optometric examination 8 months ago while she was being evaluated for a very wide range of symptoms that began more than 1.5 years ago.  Symptoms seem overall mild as judged by the absence of behavioral changes and include aches and pains involving pretty much every part of her body, joint popping, migrator muscle pains, chills, a bad taste in her mouth (before treatment with Diamox was given), GERD, perioral blisters, fatigue, and more.  MRI/MRV c and s marred by motion artifact did not show a significant lesion or any classic signs of PTCS.  The only sign to consider is some enhancement of posterior sclera on each side is difficult to understand in the absence of signs or symptoms of scleritis/uveitis.  Buried drusen were found with sonography on each side.

    The unsettling caveat is from the LP that was done when she presented to an ER before we met.  An opening pressure was (attempted?) but not recorded.  CSF composition including titers for many infectious processes (treponemes, viruses) was normal aside from an increased number of RBCs.  In spite of my inquiries, an explanation for the absent OP was not offered.  While an opening pressure was not recorded, a closing pressure was recorded and here's the rub: 35 cm water.  What to make out of this isolated closing pressure?

    Would you just repeat the LP?  Or just follow her?  I saw her yesterday and observed a happy kid with otherwise normal findings.

    Thanks,

    Steven Kane



  • 2.  RE: elevated optic discs

    Posted 05-15-2025 15:32
    I had a patient like this last year. It was only after they developed a cataract that the subtle vitritis causing optic nerve swelling became manifest.

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  • 3.  RE: elevated optic discs

    Posted 05-15-2025 16:04
    It may be pseudopapilledema, scan for drusen





  • 4.  RE: elevated optic discs

    Posted 05-15-2025 18:21

    Was she sedated for the LP?

    I have seen pediatric patients who had markedly elevated opening pressure with normal optic discs and venous pulsations present when the LP was done with sedation.

    Deb

     






  • 5.  RE: elevated optic discs

    Posted 05-16-2025 10:08

    Hi Deb.  How do you fit elevated ICP and the presence of venous pulsations into your patient's story?  Symptoms and signs?  Some kind of artifact?  I usually am happy when a measure of ICP is reassuring and not if otherwise, like with this lone elevated closing pressure.

    Steven




  • 6.  RE: elevated optic discs

    Posted 05-16-2025 13:01

    The CSF pressure measurement was erroneously elevated due to sedation/anesthetic and possibly positioning (I don't recall whether the patients were prone or in the lateral decubitus position). All I can say if the diagnosis of PTCS/IIH is a package deal and one test alone does not confirm a definite diagnosis unless other signs are symptoms are present.

    Cheers,

    Deb






  • 7.  RE: elevated optic discs

    Posted 05-15-2025 16:11


    It would be helpful to know the patient's visual parameters --- best-corrected acuity OU, color vision OU, and the results of formal perimetry OU, including images of the fields.  It's not possible to evaluate the etiology of optic disc swelling (unilateral or bilateral) in the absence of this data.

    The fundus photographs show two additional findings:

    1)  Moderate diffuse pallor of the swollen right optic disc.  This may indicate some degree of progression into the atrophic stage of chronic disc swelling.

    2)  2-3+ attenuation of all retinal arterioles in the right eye as well as the inferotemporal retinal arterioles in the left eye.  This degree of diffuse arteriolar attenuation is particularly striking in view of the patient's age.

    Stephen C. Pollock, MD






  • 8.  RE: elevated optic discs

    Posted 05-15-2025 16:21

    You have presented a youth with buried disc drusen, no symptoms typically associated with elevated ICP, normal brain imaging, and a poorly performed or documented lp. My approach to this type of patient would include repeating the lop if it can be done accurately and without too much drama and following her with acuity, color, fields, Oct, and history. Fields and Oct may be abnormal in the presence of disc drusen, but shouldn't worsen dramatically.



    ------------------------------
    Russ Edwards
    ------------------------------



  • 9.  RE: elevated optic discs

    Posted 05-16-2025 09:07

    Hi Stephen,

    Fair enough.  20/25+ OU, AOHRR 5/6 OU, normal pupil reactivities, attached fields not striking to my eyes.  ARNFL 144 OU.  The difference in colors between discs is photographic, not physiologic, at least for my direct ophthalmoscope.  The question of arteriolar narrowing is not supported by direct visualization.  They really look similar.  The question of enhancement of posterior sclera OU is unanswered.  I believe the LP was done with sedation according to mom.  I wondered if sedation could have been waning as the closing pressure was measured, producing Valsava artifact.  Curious if the group feels that OCT is more reliable or sensitive than sonography for detecting buried drusen.  I've tended toward the latter for my patients who are nearly all children.

    Steven




  • 10.  RE: elevated optic discs

    Posted 05-16-2025 12:23
    Hi Steven

    I feel if the drusen in seen on the usg as a high reflective echo then it's definitely seen on the OCT as a hyperreflective / hyperreflective margin
    Sadly in children it's not seen easily on both these investigations.
    Therefore in children the term drusen can not be as freely used for pseudopapilloedema as in adults.
    In children the OCT mostly shows crowding, axonal flow stasis, PHOMS ( more so nasally)and a very normal asymptomatic child.If one is lucky a few horizontal hyperreflective lines may be seen , but a " true blue drusen" on OCT is a rarity in a child. But I have heard Steffen and group say that it happens up north in their population .

    Despite all this,an Edi OCT is a must.
    It tells a lot of things, some things  published known to the world and some known only to a busy clinician with no time to publish !

    Please share the images, we might get to hear from the experienced group members about the interpretations.

    Best
    Shikha


    Dr Shikha Bassi
    Sankara Nethralaya 
    Chennai
    India





  • 11.  RE: elevated optic discs

    Posted 05-16-2025 14:45

    Thanks for the additional clinical data, Steven.  Very helpful and much appreciated.

    Although bilateral drusen have been well documented by ultrasound, I'm still not entirely certain that the patient has isolated disc drusen and that she should simply be followed as such.  There remain several aspects of the clinical picture that leave me a bit uncomfortable (though I fully recognize that one or more of these could turn out to be artifacts of testing):

    1)  The posterior scleral enhancement

    2)  The mean deviations on HVF perimetry (-7.14 OD;  -8.59 OS)

    3)  The single LP measurement of 35 cm of water

    4)  The size of the right optic disc.  Although the left disc is at least consistent with what one would expect with buried drusen (small disc size, very small cup), the right disc is definitely enlarged.  To be more precise, the ratio of [horizontal disc diameter] to [distance from temporal disc margin to foveal center] is 0.93.  The size of the right disc is at least suggestive of acquired disc swelling.

    I'll be interested to hear what your follow-up evaluations disclose.

    Best regards,

                Steve

    Stephen C. Pollock, MD






  • 12.  RE: elevated optic discs

    Posted 05-21-2025 13:12

    Hi,

    Regarding the question of whether OCT is more reliable or sensitive than ultrasound for detecting buried drusen, a group of us recently studied these modalities (and others) and published our results in AJO (PMID: 36516916). 

    To save you a few clicks, our results were:  "The EDI-OCT had the highest sensitivity and accuracy (95%, 97%) to detect ODD, compared with FAF (84%, 92%), US (74%, 86%), and fundus photography (38%, 66%), respectively. All image modalities had high specificity (> 97%) and precision (> 93%). The EDI-OCT also had highest examiner confidence (96%) compared with all others (88%)."

    So EDI-OCT appears to be the best test to detect ODD. If I recall, there were no ODD that were detected by ultrasound that were missed by EDI-OCT, but lots the other way around.  You were asking about deep drusen, and we included both superficial and buried ODD in this study.  Photos and FAF would probably not be as good for detecting deep ODD specifically, I would think.

    One really nice advantage that EDI-OCT has over ultrasound is that it can not only DETECT drusen, but can also characterize them in really nice detail (shape, size, depth, number (single vs. conglomeration), relationship to adjacent tissues, presence of PHOMS or not, etc.) and allows you to follow the optic nerve head over time in a more standardized way than U/S. OCT can also allow you to measure interstitial edema in the RNFL (the "PHOMS-Up thickness", poster at the last NANOS; paper pending), which can probably help with distinguishing between papilledema (interstitial edema) and ODD pseudopapilledema (no interstitial edema).  

          Alex




  • 13.  RE: elevated optic discs

    Posted 05-15-2025 17:53
    I agree completely with Russ
    Peter





  • 14.  RE: elevated optic discs

    Posted 05-16-2025 07:46
    Looks like pseudo-papilloedema. EDI-OCT can be done to check for deep drusen. 
    Monitoring alone should suffice. Usually these discs don't change much in the short term, but can change over decades.

    Regards,
    Shiva
    -----
    Dr Shivanand J Sheth (MS, FICO, FAICO, FRANZCO)

    Paediatric Ophthalmology | Strabismus | Neuro-ophthalmology

    Head of Ocular Motility Unit

    Consultant in Strabismus and Neuro-Ophthalmology
    The Royal Victorian Eye and Ear Hospital 
    32 Gisborne Street, East Melbourne, VIC - 3002 

    Research Head, Department of Ophthalmology

    Consultant in Paediatric Ophthalmology
    The Royal Children's Hospital
    50 Flemington Road, Parkville, VIC - 3052 






  • 15.  RE: elevated optic discs

    Posted 05-16-2025 12:03

    If it was my child, I would opt for observation is there is no afferent dysfunction :) The discs don't look too concerning especially for a young child. 




  • 16.  RE: elevated optic discs

    Posted 05-16-2025 12:18
    There's something more going on here. This child has a litany of complaints. Maybe they are functional. Maybe she is under some kind of stress, or she is being abused. Lots of testing ratchets up the anxiety and stress. Maybe a psychiatry referral would be helpful. (Of course, in addition to seeing her in followup for her eye issues)
    Shelley Cross





  • 17.  RE: elevated optic discs

    Posted 05-18-2025 12:57

    There's nothing like having a referral with the MRI and LP already done, it simplifies the decision making of course (MRI or not, and especially LP or not). In this case, I would definitely get at least an MRI/MRV because sometimes it isn't so easy to differentiate pseudopapilledema from real papilledema.

    In this case, the absence of dilated vasculature on the nerve itself, the "lumpy" disk margins, the blurred disk margin, but no blurring of crossing retinal vessels leads me to conclude it's pseudo-papilledema. I refer these routinely to retina to do a B-scan looking for drusen, sometimes they're there, sometimes they're not. Once I've ruled out the tumor, in the asymptomatic patient with normal fields I will often do serial exams, including photos and fields every time, and almost always they show carbon copy replicas of the appearance, so much so that I've misled myself thinking I was flipping through the same photo. This avoids the trauma, morbidity of the LP, and the hang-wringing that goes with an LP with OP of 27.

    So is this hypocritical? You've opened the Pandora's box of possible true papilledema, you get the imaging, but then not proceed to the LP? Especially if the patient is heavy?

    I've seen two cases where I had referrals for papilledema in older patients, I confidently monitored them after the MRI and LP (normal), and then lo and behold, they came back with true papilledema on top of pseudopapilledema.

    Mitch