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  • 1.  13-yo with atypical presentation of presumed OPG

    Posted 5 days ago

    Dear colleagues,

     

    I would appreciate your input on the following challenging case of presumed OPG.  The following link provides imaging and photos: https://uwmadison.box.com/s/nle1tiaej3ta8gik7euwhn67uf9eunnp

     

    13-yo CM with 2-wk h/o progressive visual loss OD ~ 2 wks. PMH is significant for congenital pulmonary valve stenosis and undescended testes, otherwise unremarkable. 

     

    3/3/2026: sudden onset HA with nausea and worsening blurriness, right frontal HA. Ophth exam 20/50 and 20/20, Ishihara 8/14 and 17/17, severely constricted VF on confrontation (able to count finger only in the center VF on CVF). (+) APD OD. Profound disc edema 360, otherwise unremarkable DFE. The orbital MRI showed an expansile lesion along the right optic nerve towards optic chiasm and tract with mild enhancement, consistent with OPG. Pt was discharged with IV port for chemotherapy (LGG-14C03: carboplatin and vincristine, dexamethasone).

     

    3/8/2026: acute visual decline to NLP OD on cycle 1, day 3 chemo, and new pain with eye movements. Now with new findings of -3 supraduction and -2.5 adduction OD, and more profound ODE with new extensive disc heme. A repeat orbital MRI showed a similar expansile lesion with minimal enhancement. The lesion appears to have pinged on the posterior globe more prominently than before. 

     

    Workup: (-) CBCD, CMP, ANA, ANCA, TP-PA, HIV, HSV/VZV, quantiferon-gold, CXR. 

     

    Question: 

    The radiographical findings are consistent with OPG. However, the rapid change to NLP, newly developed massive disc heme, and worsening ODE, and newly developed painful ophthalmoplegia are very unusual. The chemo is held. The pain with eye movement has not responded to IVMP (250 mg 2/day, ~ 40 kg); so low suspicion for inflammatory process. Also found a couple of case reports of vincristine causing unilateral or bilateral optic neuropathy in a setting of leukemia treatment. However, vincristine toxicity can't explain painful ophthalmoplegia. 

     

    We are thinking about orbital biopsy. Any suggestions on investigation and/or intervention will be greatly appreciated.

     

    Thanks,

     

    Judy

     

    Yanjun (Judy) Chen, M.D., Ph.D.

    Associate Professor of Ophthalmology, Neurology, and Neurosurgery

    University of Wisconsin School of Medicine and Public Health

    2870 University Ave, Suite 108B

    Madison, WI 53705

    Ph: 608-263-4823

    Fax: 608-263-7694

    Email: ychen344@wisc.edu

     



  • 2.  RE: 13-yo with atypical presentation of presumed OPG

    Posted 5 days ago
    Hi Judy,

    The images on Box don't seem to work, I logged in and it says the link was removed or not available.

    It sounds like this is likely OPG apoplexy which has been described as a rare complication.

    I had a similar case in an adult with sporadic orbitla segment OPG  last year, she had poor vision prior to presenting to our hospital and opted for observation, then after being stable for 6 eyars developed pain and ophthalmoplegia, MRI showed new enhancement of the prior non-enhancing lesion and diffusion restriction. Work-up was negative for secondary causes of orbital inflammatory syndrome. We treated her with oral steroids tapered over 6 weeks, she had rapid resolution of pain and motility returned to normal and MRI showed resolution of enhancement. I was initially worried about transformation into a more aggressive tumor, but she has remained stable and deferred biopsy.

    You can ask your radiologist to look at the diffusion weighted images to see if there are signs of infarct. Susceptbility weighted imaging to look for hemorrhage is usually limited in the orbit due tissue interfaces.

    Here is the largest case series I could find: Optic pathway-hypothalamic glioma hemorrhage: a series of 9 patients and review of the literature. J Neurosurg . 2018 Dec 1;129(6):1407-1415. doi: 10.3171/2017.8.JNS163085.

    Abstract
     OBJECTIVE
    Hemorrhage (also known as apoplexy) in optic pathway gliomas (OPGs) is rare. Because of the variable presentations and low incidence of OPG hemorrhages, little is known about their clinical course and the best treatment options. The aim of this work was to review risk factors, clinical course, and treatment strategies of optic glioma hemorrhages in the largest possible number of cases.
    METHODS
    A total of 34 patients were analyzed. Nine new cases were collected, and 25 were identified in the literature. Data regarding demographics, radiological and histological features, treatment, and outcome were retrospectively reviewed.
    RESULTS
    The majority of patients were younger than 20 years. Only 3 patients were known to have neurofibromatosis. The histopathological diagnosis was pilocytic astrocytoma in the majority of cases. Five patients had intraorbital hemorrhages, whereas 29 patients had intracranial hemorrhage; the majority of intracranial bleeds were treated surgically. Six patients, all with intracranial hemorrhage, died due to recurrent bleeding, hydrocephalus, or surgical complications. No clear risk factors could be identified.
    CONCLUSIONS
    Intracerebral OPG hemorrhages have a fatal outcome in 20% of cases. Age, hormonal status, neurofibromatosis involvement, and histopathological diagnosis have been suggested as risk factors for hemorrhage, but this cannot be reliably established from the present series. The goals of surgery should be patient survival and prevention of further neurological and ophthalmological deterioration.

    Best,

    Drew

    PS: If you want to collaborate on a case series, let me know.



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  • 3.  RE: 13-yo with atypical presentation of presumed OPG

    Posted 5 days ago
    This could also be an apoplectic event from rupture of an intrinsic  cavernoma, As I have encountered these several times over the years, affecting either the optic nerve or chiasm And I had one referred to me that was read as a glioma elsewhere and was recommended to undergo unnecessary chemotherapy and radiation therapy prior to seeing me.

    Matt



    Sent from my Galaxy




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  • 4.  RE: 13-yo with atypical presentation of presumed OPG

    Posted 5 days ago

    Hi Drew and Matt,

     

    Thank you for your input. I will load the image again tomorrow. In our patient, the initial MRI showed an unusual-looking "ring enhancement" within the tumor, which was no longer present on the repeat MRI 5 days later (after the NLP and painful ophthalmoplegia).

     

    Apoplexy makes sense – I was wondering about sudden hemorrhage inside the tumor causing intense pressure to posterior orbit, perhaps with resulting pressure/ischemia to the EOMs in the area. I will check with neuroradiology again to see if an orbital CT is indicated to look for hemorrhage if MRI is unrevealing.

     

    So no concern for vincristine optic neuropathy?

     

    Thanks,

    Judy

     

    Yanjun (Judy) Chen, M.D., Ph.D.

    Associate Professor of Ophthalmology, Neurology, and Neurosurgery

    University of Wisconsin School of Medicine and Public Health

    2870 University Ave, Suite 108B

    Madison, WI 53705

    Ph: 608-263-4823

    Fax: 608-263-7694

    Email: ychen344@wisc.edu

     

     




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  • 5.  RE: 13-yo with atypical presentation of presumed OPG

    Posted 5 days ago
    I believe vincristine optic neuropathy is usually a bilateral toxic optic neuropathy presenting with atrophy, not hemorrhagic disc edema 



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  • 6.  RE: 13-yo with atypical presentation of presumed OPG

    Posted 4 days ago

    Pt completed a CT orbit this morning with no radiographic evidence of intralesional hemorrhage. 

    Case reviewed with peds neuroradiology with the following impression:
     
    1. The lesion appears to be optic nerve origin, most consistent with OPG, though behaving more aggressively than usual
    2. There is diffusion restriction within the lesion suggesting hypercellularity or ischemia
    3. No radiographic findings of cavernous sinus/ophthalmic vein thrombosis
    4. The lesion does not appear to be orbital cavernous hemangioma on the MRI
    5. There is no clear radiographic (MRI/CT) sign of hemorrhage

    We are talking to oculoplastics to get a biopsy. 

    Best,

    Judy


    Yanjun (Judy) Chen, M.D., Ph.D.

    Associate Professor of Ophthalmology, Neurology, and Neurosurgery

    University of Wisconsin School of Medicine and Public Health

    2870 University Ave, Suite 108B

    Madison, WI 53705

    Ph: 608-263-4823

    Fax: 608-263-7694

    Email: ychen344@wisc.edu

     




    Original Message


  • 7.  RE: 13-yo with atypical presentation of presumed OPG

    Posted 4 days ago
    I wonder if it outgrew its vascular supply and became necrotic causing acute inflammatory process...



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  • 8.  RE: 13-yo with atypical presentation of presumed OPG

    Posted 4 days ago

    Hi Drew,

     

    It is quite possible. I wonder if the visual decline has been longer than the reported 2 wks given pt's young age. The tumor size could have slowly built up, and the optic nerve function finally tipped off at the first presentation on 3/4/2026, when he preserved only tunnel vision despite VA of 20/40. Therefore, the change from 20/40 to NLP could be only a small step within the same process.

     

    Just an update, the painful ophthalmoplegia finally improved after 2 days of IVMP, with improvement in both pain level and ocular motility.

     

    Also found the following case report of 34 hemorrhaged OPG. 17/34 were pilocystic astrocytoma (WHO I) and 4/34 were pilomyxoid astrocytoma (WHO II), 5/34 were orbital. Vision improved 9/15 pts with VF deficits. The authors cautioned about the surgical intervention/biopsy. One pt with orbital OPG improved without surgical intervention.

     

    "Optic pathway-hypothalmaic glioma hemorrhage: a series of 9 patients and review of the literature. J Neurosurg 2018;129:1407-1415"

     

    What intervention did your patients receive? Given the interval improvement and the information from the above article, I am now a bit hesitant about the biopsy.

     

    (BTW – also found this article: "Unilateral optic neuropathy following vincristine chemotherapy. Journal of Pediatric Ophthalmology and Strabismus. Norton and Stockman 1979;16(3):190-3")

     

    Thank you,

     

    Judy

     

    Yanjun (Judy) Chen, M.D., Ph.D.

    Associate Professor of Ophthalmology, Neurology, and Neurosurgery

    University of Wisconsin School of Medicine and Public Health

    2870 University Ave, Suite 108B

    Madison, WI 53705

    Ph: 608-263-4823

    Fax: 608-263-7694

    Email: ychen344@wisc.edu

     




    Original Message


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