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Deep orbital haemangioma

  • 1.  Deep orbital haemangioma

    Posted 27 days ago
    This is a 43-year-old female known to have a left orbital haemangioma since 2013, but it was left untreated.  She now came to our hospital for second opinion.
    The haemangioma is too deep for the orbital surgeon and in an uncommon place for the neurosurgeon.
    The right eye has a Snellen visual acuity of 0.9 (18/20). The left eye sees 0.7 (14/20).
    There is a left optic atrophy (GCL more affected than RNFL) and associated visual field loss. However, there is still vision to be saved.
    What would you recommend?
    Thank you,
    Michel
    image
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  • 2.  RE: Deep orbital haemangioma

    Posted 27 days ago
    Coronal views?

    Russ Edwards





  • 3.  RE: Deep orbital haemangioma

    Posted 27 days ago
    Also, do you have older fields? is the vision loss progressing, or has it been stable for the past 13+ years?

    --
    Russ Edwards





  • 4.  RE: Deep orbital haemangioma

    Posted 27 days ago
    Steretotactic radiosurgery (Cyberknife or Gammaknife) can be an alternative to surgery in these cases. If there is severe neuro-axonal loss already then the risk of more post-op visual loss risk is higher.


    RB




  • 5.  RE: Deep orbital haemangioma

    Posted 27 days ago
    These are always tough calls. Depending on the exact location, endoscopic surgery can be done to decompress or take the lateral wall off all the way back to get at it from superior or inferior laterally. Another option would be to inject an anti-VEGF agent into the lesion. Finally, radiosurgery may be appropriate but will take a long time to reduce the lesion size.

    N



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  • 6.  RE: Deep orbital haemangioma

    Posted 27 days ago
    It doesn't have to be radiosurgery, they can do fractionated radiotherapy (~50 gy in  in 26 fractions) similar to what is used for meningiomas which will likely be lower risk optic nerve toxicity as the dose per fraction is lower and better tolerated by the optic nerve. 

    I think orbital decompression without resection is likely just kicking the can down the road.

    In the orbital apex, there is high risk of vision loss (60-80%) with surgery from a variety of mechanisms including traction on the nerve of the tumor is adherent, retrobulbar inflammation / hemorrhage.

    Best, 

    Drew




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  • 7.  RE: Deep orbital haemangioma

    Posted 27 days ago

    Michel,

     

    I operated on a similar case many years ago.  I used a transantral approach to the medial wall at the apex.  Once the bone was removed and the periorbital opened, the mass presented itself into the posterior ethmoidal space an was easily dissected free from the orbital tissues.

     

    Steve

     






  • 8.  RE: Deep orbital haemangioma

    Posted 27 days ago

    Many years ago when I was JSKs fellow and we were operating on optic nerve sheath meningiomas. our exposure with neurosurgical help was to remove the lateral wall back to the temporal dura.  

    that is not too difficult and then open the periorbita and hope that you decomopress the optic nerve

    but my question is (and remember I was a very aggressive optic nerve and orbital surgeon) what is the patient to Gain

    this goes back to my residency when I wanted to do a glaucoma procedure on an only eye (glaucoma surgery was frowned upon in NYC in those days) and my attending in a fatherly manner said "let 

    God blind him Tom not us" best to al and stay well

    tcs



    ------------------------------
    tsapoor md
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  • 9.  RE: Deep orbital haemangioma

    Posted 27 days ago
    The tumor appears to lie lateral to the optic nerve. A trans ethmoidal approach might take some pressure off the nerve but would not provide safe access to it. Lateral orbitotomy combined with a frontal craniotomy would give access. 

    But - this is based on the few cuts provided; any surgeon would want to review the whole series of scans before making any recommendations. 

    If this is in fact a cavernous hemangioma, it and the option neuropathy may be stable. I had a patient with mild optic neuropathy from a much larger cavernous hemangioma who declined surgery. I followed her for 20 years without change. I still recommend chasing down old records.

    Russ Edwards




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  • 10.  RE: Deep orbital haemangioma

    Posted 27 days ago
    If the tumor is lateral to the optic nerve, then a far posterior superolateral approach would be best, hopefully between the lateral rectus and the superior rectus/levator complex. The farther posterior the lesion, the more it tends to be stuck to surrounding tissue so a piecemeal approach with focal bipolar cautery to shrink might be ideal.



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  • 11.  RE: Deep orbital haemangioma

    Posted 27 days ago

    At the orbital apex, there is enough pressure build-up to affect optic nerve function.  Also, lmost all of the tissue is fixed in position except for that slippery hemangioma that will follow path of least resistance.  Just an N of 1, but a very satisfying result.

    Steve

     




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  • 12.  RE: Deep orbital haemangioma

    Posted 27 days ago
    I agree with all the previous comments. Neil your comments about "being stuck" to orbital apex tissues are best described by Jerry Harris article. My vote would be to consider radiation therapy given the location.

    Cavernous Hemangioma of the Orbital Apex: Pathogenetic Considerations in Surgical Management
    American Journal of Ophthalmology
    Volume 150, Issue 6, December 2010, Pages 764-773
    Gerald J. Harris

    Jim Garrity
    Mayo


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  • 13.  RE: Deep orbital haemangioma

    Posted 27 days ago
    A lateral orbital wall decompression has been helpful in several of these cases I have had over the years as it affords some breathing room.

    Matt

    Sent from my Galaxy






  • 14.  RE: Deep orbital haemangioma

    Posted 27 days ago
    Stereotactic radiosurgery (i.e. gamma knife, cyberknife)
    A 2024 systematic review and meta-analysis of SRS for orbital cavernous hemangiomas (6 studies, 100 patients) reported visual acuity improvement in 80%, visual field improvement in 71%, proptosis improvement in 94%, and tumor reduction in 77%. The complication rate was 13%, consisting only of minor events (orbital pain, periorbital chemosis).

    1. Punukollu et al (    J Neurooncol. 2024). Gamma knife radiosurgery for orbital cavernous hemangioma: a systematic review and single-arm meta-analysis. 

    Fractionated Stereotactic Radiotherapy (FSRT) (i.e. intensity modulated radiation therapy = IMRT)
    Two small series evaluated conventional fractionation. Ratnayake et al. treated 6 patients with 45–50.4 Gy in 1.8–2 Gy fractions, achieving 63% average tumor volume reduction at 12 months, with improvement in proptosis and visual field defects in all symptomatic patients and no complications. [6] Rootman et al. treated 5 patients with 40–50 Gy in conventional fractions, achieving approximately 60% tumor shrinkage with rapid visual field improvement by 3 months and no complications.

    2. Rootman et al (Ophthalmic Plast Reconstr Surg. 2012). Stereotactic fractionated radiotherapy for cavernous venous malformations (hemangioma) of the orbit. 
    3. Ratnayake et al (Ophthalmic Plast Reconstr Surg. 2019) Stereotactic Radiotherapy for Cavernous Venous Malformations of the Orbital Apex. 

    I would caution against proton therapy, it is more inflammatory than photons and in the tight orbital apex may cause vision loss due to compartment syndrome.

    Best,

    Drew



    Original Message


  • 15.  RE: Deep orbital haemangioma

    Posted 26 days ago
    I have been seeing a patient with deep orbital haemangioma since 2009
    Size was 11x12x7 mm then and in 2024 had increased to 18x1.6x11 mm and 21X18 mm in Feb 2025  underwent EBRT in Feb 2025
    Has a subjective improvement in vision last mri in June 26 the size is 13x8x5.3mm. She has a bilateral added vision loss due to stargadts disease. A challenging case to follow up and monitor.But she has done fairly well in life despite the odds , as it was mentioned that we can observe for years before considering the challenging treatments.


    Dr Shikha Bassi
    Sankara Nethralaya 
    Chennai
    India



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  • 16.  RE: Deep orbital haemangioma

    Posted 26 days ago

    We have successfully done many similar cases by an extended lateral orbitotomy to the superior orbital fissure and middle cranial fossa.

    An optional, but less desirable approach is an endoscopic transnasal orbital apex decompression to decompress the optic nerve, but with no tumor excision.

    Howard R Krauss MD




  • 17.  RE: Deep orbital haemangioma

    Posted 25 days ago
    Dear All

    I would appreciate some help with a young man with recurrent 6th nerve palsies that don't quite fit the paradigm reported for benign recurrent 6th nerve palsies:

    15 yo male without prior history

    2022 - presented with right abducens paresis without any cause apparent on extensive investigation. Resolved over 2 months
    2025 - when he developed episodic right abduction palsy, preceded by rhinitis, occurring daily for several hours without provocation. No persistence of rhinos after resolution of rhinitis. 
    2026 - significant reduction in frequency, now monthly and only lasts about 1 hour, otherwise no change in each event symptomatically. 

    Repeated investigation, including fine cuts through orbits in axial, coronal and oblique planes, does show any pathology. Full autoimmune and metabolic screens are normal. 

    Any suggestions or comments are welcome. 

    Kind regards


    Prof Owen B White MD PhD FNANOS FRACP
    Dept of Neurosciences
    Central Clinical School
    Monash University
    Melbourne, VIC, 3004
    Tel: _61 3 95760022
    Fax: +61 3 95760022
    Mob: +61 418822996
    Orcid ID: 00000-0002-2836-7344




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  • 18.  RE: Deep orbital haemangioma

    Posted 24 days ago
    could he have CSF rhinorrhea and CSF leak during which time he gets the sixth nerve paresis and then it self resolves for a bit.

    does he get headache with standing during these times or other cerebral hypotension symptoms

    has he had imaging during the sixth nerve palsies to show meningeal enhancement and low lying tonsils?

    There are types of ball valve mechanisms that could explain this, in areas of base of skull, like from a small meningocele herniating into these defects.

    However, only 1 hour of VIth nerve paresis, would be unusual for a VI th paresis related to stretching, but perhaps it could happen?

    Could he have IIH with a secondary leak?  Any reason for IIH?

    MG, Neuro-myotonia don't go with the history


    Jade




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  • 19.  RE: Deep orbital haemangioma

    Posted 24 days ago
    Sir , Does he play violent sport like kick boxing things like that I had a cases like that one of them the boxing caused traumatic PTC with rhinorrhea  and sixth palsy that need repeated shunt , guess what that boy went play boxing with the shunt without telling his parents till they discovered that , the second scenario which I many cases is just simple traumatic sixth with violent spirts and exercise with some time lapse between the trauma and the complaint , with misleading nasal sinus fluid discharge resembling CSF , false rhinorrea, so I got deceived many times by those kids playing violent sport without even telling their families or denying trauma that are sometimes repeated like falling of a bike without helmet , so better dig into the history Sir 
    Sherif 




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  • 20.  RE: Deep orbital haemangioma

    Posted 24 days ago
    What is his refractive eror?I have had youngish patients with"sixth nerve palsies" who actually had decompensated ET/accomodative spasm associated with high hyperopia and/or prolonged near work.
    Marilyn Kay



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  • 21.  RE: Deep orbital haemangioma

    Posted 24 days ago
    There is no history of any trauma.  The original episode was isolated and spontaneous lasting some weeks. Recurrent episodes come on spontaneously being preceded by approximately 20 mins of primarily "a stuffy nose" and rhinorrhea for no more than 20 minutes. 

    His abduction paresis then lasts 1-3 hours and resolves over minutes. There are no usual symptoms of CSF leak and I honestly have seen many dural leaks. I have not seen abduction paresis apart from prolonged spinal leaks with demonstrably low pressure and abnormal MRI. I find it difficult to believe this is the mechanism. 

    I wonder about migraine but this is not like any of the ophthalmoplegic migraine I have seen in the past

    I agree this does not sound like MG but I did wonder about neuromyotonia.  Seeing him during an attack might help but he lives 2 hours from my office (still suburban in Australia)

    There are no symptoms or signs of IIH and now events are diminishing in frequency. 

    Owen

    Prof Owen B White MD PhD FNANOS FRACP
    Dept of Neurosciences
    Central Clinical School
    Monash University
    Melbourne, VIC, 3004
    Tel: _61 3 95760022
    Fax: +61 3 95760022
    Mob: +61 418822996
    Orcid ID: 00000-0002-2836-7344



    Original Message


  • 22.  RE: Deep orbital haemangioma

    Posted 24 days ago
    The combination of CN6 palsy and autonomic features would suggest a pathology in the cavernous sinus where the sympathetic fibers and CN6 are in close proximity, perhaps a small aneurysm or schwannoma.

    Do you have high resolution cranial nerve protocol MRI with T2 steady state sequences (CISS or FIESTA)?

    You can have the patient video VOR and lateral gaze saccades using his phone during an episode and self test trigeminal sensation.

    If imaging is negative might want to try a course of indomethacin to break an episodic trigeminal autonomic cephalgia.

    Best,

    Drew





    Original Message


  • 23.  RE: Deep orbital haemangioma

    Posted 24 days ago
    Thanks Andrew

    All good thoughts

    Cavernous sinus fully investigated on three occasions without pathology showing. 

    Examined several times during attacks by an excellent strabismus with nothing else coming to mind. 

    What is curious is that here have gone from lasting weeks to days and now hours while frequency has come down from daily to now once a month or so over a couple of years

    Owen
    Sent from my iPad

    Prof Owen B White
    Director Ocular Motor Laboratory
    Central Clinical School
    Monash University
    Commercial Rd
    Prahran, VIC 3004
    Australia 
    Tel: +61 3 9576 0022
    Fax: +61 3 9576 0019
    Mob: +61 418 822 996



    Original Message


  • 24.  RE: Deep orbital haemangioma

    Posted 24 days ago
    There's a condition called benign recurrent sixth nerve palsy of childhood  brought on by flu ( ?nasal congestion etc) It has an excellent prognosis .
    But if it's  autonomic symptoms and not a viral prodrome ,it point towards a migraine variant which the child is outgrowing .
    BTW was any treatment in the form of steroids/ migraine prophylaxis /NSAIDs ever given over the years .

    Dr Shikha Bassi
    Sankara Nethralaya 
    Chennai
    India



    Original Message


  • 25.  RE: Deep orbital haemangioma

    Posted 23 days ago
    Many thanks to everyone for your replies and sharing your experience. In addition to surgery and radiotherapy, propranolol was also suggested.
    The neurosurgeon talked  to the patient last Friday. Although I have not heard back, yet, we will try the propranolol and then most likely stereotactic radiotherapy. I will update the thread later to report on the response to treatment.
    Kind regards,
    Michel



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