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 10 yo with acute vision loss OS

Anne Kao's profile image
Anne Kao posted 04-19-2026 19:14

10 yo healthy boy with vision loss OS. 

Beginning 5 days prior to onset he noticed intermittent bright stars in vision.  On day of presentation he developed blurry (not dark) vision while playing soccer on a hot day, stopped playing, got a little better, resumed playing and at end of game vision was dark OS.  Maybe a little sore when he moved his left eye while playing soccer, but not otherwise.  No diplopia, no headache.

No recent illnesses, except for 2-3 episodes of severe abdominal pain in the 2.5 months preceding presentation that would awaken him from sleep, but better by morning. Otherwise ROS negative.  Does not eat a lot but eats a relatively healthy diet.  No recent travel, no family history of vision problems.  He has an outdoor cat and is active outdoors, was playing outside after storms about a month ago, but did not ingest any dirty water.

Vision 20/20 OD, HM OS.  3-4+ RAPD OS.  Fundus examination OD normal; OS showed very mild optic nerve edema inferiorly and superiorly, macula notably pale – looked like a CRAO – but vessels unremarkable (no tortuosity, no unusual caliber, no signs of embolus nor vasculitis). 

MRI showed mild edema of and around optic nerve where it enters globe, mild enhancement of tissue posterior to sclera and superior to optic nerve, no enhancement of nerve itself. 

IVMP 30mg/kg/day was started about 24 hours after initial presentation with no improvement in vision.  IVIg was then started with no improvement in vision. 

All tests of serum and CSF to date have been negative: ESR, CRP, CBC (normal diff), ANA, ACE, ANCA, MOG, NMO, syphilis, quant gold, B12/folate, COVID PCR, Bartonella IgG and IgM, leptospira, CSF protein/glucose/cx/meningitis panel/NMO/MOG/toxo

Hypercoag panel pending

Only abnormal tests are slightly low serum and CSF IGG 

Repeat MRI showed stable edema of nerve, interval decrease in enhancement adjacent to nerve

Any thoughts regarding etiology or further testing would be greatly appreciated!

Anne Kao's profile image
Anne Kao

Correction: initial CBC showed mild increase in neutrophils, no eosinophilia

Pamela Chavis's profile image
Pamela Chavis

Was a toxoplasmosis screen done? 

Andrew Carey's profile image
Andrew Carey
Do you have an OCT of the macula to confirm retinal ischemia? Viral retinitis can also cause retinal whitening and have scleritis / optic nerve involvement. Is there any AC cell? Did you do viral PCR on CSF?
Posterior scleritis can have secondary involvement of the optic nerve, the MRI description could be consistent with scleritis which has a different differential than optic neuritis , more in the realm of vasculitis.
Are the sinuses okay? Is there chest imaging? How about urine protein: cr ratio? RF, IgG4? If there is tissue in the orbit enhancing you could consider biopsy.
Has he had a colonoscopy? Inflammatory bowel disease and bechets can look like this.
Best, 
Drew
Ahmad Najafi's profile image
Ahmad Najafi

En Face OCTA slabs of the macula have the highest information yield if macular perfusion is concerned.

John Chen's profile image
John Chen

I agree with the recommendations posted so far.  If the workup for infectious, inflammatory, and vasculitis etiologies is all negative and the OCT confirms there was retinal ischemia (inner retinal whitening/thickening), I would consider evaluating for DADA2, one of the monogenic autoinflammatory syndromes, by testing plasma ADA2 enzyme activity.

Best,
John

Sherif Ahmed Kamel Abdelbar's profile image
Sherif Ahmed Kamel Abdelbar

I would investigate POHS , with Crohn’s and. Celiac disease 

I would also make a cardiological consultation with ECHO and carotid Doppler 

sherif 

Anne Kao's profile image
Anne Kao

Thank you so much everyone for your thoughts and insights. 

He hasn't had any ancillary testing (such as OCT) done, since he has been admitted.  He will likely be discharged soon and we'll bring him to clinic.

DADA2 was certainly fresh in my mind, given the recent Walsh, and we placed a genetics consult on Saturday.

I have not yet examined him at a slit lamp, but with regard to AC cell -- clear view to fundus and eye was white and quiet.  no photophobia and the soreness he endorsed sounded pretty minimal.

The MRI findings were very subtle, so there isn't anything to biopsy at this point.

Toxo PCR in CSF negative.  Viral PCR in CSF negative.  

The peds hospitalist initiated a workup for IBD, but it was after his course of steroids so she thought it would likely be low yield.  She noted that he did not have anemia.  

I will follow up on some of the other suggestions, very grateful for your input.  I am still struck by the fact that his inflammatory markers all have been normal.

Shivanand Sheth's profile image
Shivanand Sheth

We had a similar presentation in a 13 year old - Turned out to have cilioretinal artery occlusion with a pale disc and macula. Cardiac Echo revealed a Patent foramen ovale which was the source of the tiny emboli - So agree with cardiac workup/ECHO