My long journey to diagnoses of Jugular Vein Compression by Styloid & C1

I am glad I have found this site. I am from Toronto, Canada and I have been suffering from this terrible Jugular Eagle variant since 2016. I just did not know it at the time. It has completely put my life to stop as I have a multitude of symptoms. I was finally diagnosed after several doctors dismissed me. As result, I had all the imaging you can think of; MRIs, MRVs, CT scans, Ultrasound, and finally Catheter Angiogram/Venogram.

I had to request an ultrasound for my neck after a second opinion on one of my MRI showed that I had signs of intercranial pressure but they did not know what was causing it. The ultrasound showed high velocity for both Jugular Veins ( 130 cm/s for the left one and 200 cm/s for the right) but it was missed by the radiologist and I had to point them out after learning how to read doppler ultrasound for vascular issues (narrowing = high velocity, reminds me of the fluid dynamics I took in physics classes). After confronting them with the high velocity, only then they agreed to take second look and agreed with me. Then a CT Scan of the neck, head and chest was ordered to investigate extrinsic compression of IJVs. The CT Scan did show that my right IJV was hypoplastic (naturally narrowed) and showed my left Dominant IJV was compressed by both Styloid and C1 transverse process but again the radiologist missed it so it was reported as unremarkable. I had to point it to them after learning how to read CT Scans for jugulars from YouTube videos. I also learned how to render 3Ds in 3D slicer same as many of you here (necessity is the mother of learning). So finally, I contacted a vascular surgeon who agreed to do dynamic Catheter Angiogram/Venogram with manometry and lo and behold he could see that 90% of my brain was drained by my left Jugular Vein which was severely compressed by primarily the styloid process and then C1 arch. I have attached the images below. So now, they do not know what to do. Vascular surgeon thought he could stent it and I told him that will cause complication since there is osseous compression and that I need removal of the left styloid and shaving of the C1 but here in Canada, no doctor seems to know or do that so I might as well pay out of the pocket in the states to get it done.

Also my Styloids are not long (the left one is about ~ 1.8 cm but thick) and I have one dominant left jugular vein which is compressed by the left Styloid process and to some degree the C1. My right jugular vein is small and hardly draining my brain so nothing can be done on it.

I have severe bilateral hearing loss, Vision problems (halos around lights, blurry, distortion of surfaces), Memory & Cognitive issues, Insomnia…etc. I can’t concentrate at all. I will give the full details of my symptoms shortly but I have not yet seen anyone here complaining about hearing loss and memory impairment. Does anyone suffer from SNHL as result of this Jugular compression. I have seen the some academic papers on IJVS showing that almost 50% of IJVs patients suffer from hearing loss.

Axial 2021 CT Scan of the neck showing styloid & C1 compression in the left dominant IJV



Sagittal 2021 CT Scan of the neck showing styloid & C1 compression in the left dominant IJV


2020 MR Angiogram Images with segmented Venous phase shows the imprint of the styloid compression on the dominant left Internal Jugular Vein.



3D Bone remodelling of the 2021 CT scan of the Neck and Head showing left dominant left jugular vain compression by Styloid process and C1 transverse process





2021 Catheter Angiogram Showing narrowed dominant left Jugular vein with blood flow issues







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WOW, KoolDude!

That’s a lot of imaging & I applaud you :clap: :clap: :clap: for your resourcefulness in learning how to read them. You’ve inspired me. I’m pretty good w/ 3D images but am a bit lost on all the other scan images (thanks for the arrows & subtitles on some images, BTW). You are in good company here as we have several current members from Ontario & other provinces who are in the same situation as you are regarding finding a doctor to do surgery. You can use the magnifying glass icon to search for posts by @Tomas10 & @bmcdiddie in particular.

Your journey has been long & I’m very sorry for the level of debilitation you’ve suffered. Again, being proactive has brought you this far. Your symptoms i.e. visual & hearing changes plus significant brain fog are a common theme among our IJV compressed ES members here. Many don’t have the advanced symptoms you do but still have similar complaints. As I type this, one of our executive directors who is Canadian is going to bat w/ the medical community on behalf of our Canadian members who have ES. Since her efforts are just beginning, it will likely take some time to see the results. In the meantime, if you were to come to the US for surgery, Dr. Hepworth in Denver, CO, is our most knowledgeable vascular ES surgeon. Sadly, he isn’t taking new patients until next year so a call to him now would be fruitless. Dr. Cognetti and Dr. Newman, both in Philadelphia, PA, & Dr. Hackman in Charlotte, NC, are all very experienced as well so you could try contacting any of them to find out if they feel they can help you.

Shaving C-1 is another story, however. There are very few surgeons in the US who do that as it can create some cranio-cervical instability. Often just getting the styloid removed & taking that pressure off the IJV allows the IJV to open enough to allow increased blood drainage from the brain. That in turn allows for healing of symptoms over time. It is not a quick fix & can take months & up to a year or more to realize the full results of a styloidectomy.

I do agree, in your case, that it’s not the length of your styloids but how thick they are that is causing your troubles. Unfortunately, even some of the best surgeons on our list base their decision about doing surgery on styloid length alone w/o considering the other features of a problematic styloid. This is not to discourage you but to give you a heads up if you do contact doctors here & are turned away. The answer is NEVER SURRENDER & NEVER GIVE UP!

https://forum.livingwitheagle.org/t/doctor-lists-no-discussion/10245/5

:hugs:

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KoolDude

I’m glad that you found this site as well. LivingWithEagle.org is part of a larger network of rare disease communities, BensFriends.org. Please have a look at that, and know that you are by no means alone in your struggle.

Because each rare disease/condition is unique, each of our 40+ sites is unique as well. What binds us together is the need for others who understand what we are going through, and the heartfelt desire to give support as well as get support.

Welcome to this community, and to the Ben’s Friends network for people whose lives have been touched by a rare disease.

Thank you for introducing yourself and sharing your story. That alone has made some other people feel less alone.

All the best to you

Seenie from Admin and Moderator Support

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Thanks Isaiah for the warm welcome and the useful guidance on how to find doctors. I won’t give up or give in to this disease but mine is unique in that I have alot of symptoms that I can count. For example, my whole right side is weak and my muscle get tight. Not to mention the dementia like symptoms I get often.

For the folks wondering if IJVS can cause a whole lot of weird symptoms, you need to watch this youtube video (Venous Compression Syndromes: From Diagnosis to Treatment - YouTube). it is 16 min long and talks about Venous Compression Syndrome which IJVS is part of. At 13:30 minute mark, there is 17 year old lady who has suffered from quadriparesis (weakness of all four limbs) and could not walk as result of severe right jugular vein stenosis and when it was stented, she was walking within 24 hours and running on a treadmill within 48 hours. So this syndrome can cause all kinds of weird symptoms and I have tons of it. Probably because my right jugular vein is naturally diminutive and the left one is extremely compressed by the Styloid process and C1. Impaired or blocked venous outlet is linked to venous congestion and brain hypo-perfusion potentially contributing to cognitive issues such as memory lapses, brain fog, insomnia, vision and hearing impairment, muscle issues. etc.

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Thanks @Mod_support for the kind welcome message. I hope I helped someone. I am equally glad that I found this site. I learned alot by just reading it.

@Isaiah_40_31

Regarding the shaving of C1, I found this study that details 10 patients who had both Styloid removal and C1 shaving/tubercula drilling. 9 patients felt better after the operation or experienced resolution of their symptoms with one patient experiencing transient swallowing difficulty with facial drooping. I am not saying C1 shaving is free of complications but this study appears to be promising in terms of the safety of it. I know many doctors do not do for the fear of complications but I have seen many folks in here not improve after styloid removal only.

It is also important to note that one of the writers is part of the doctors list here and his name is Dr. Peter Nakaji. The average hospital stay was 2.1 days (range 1 - 6 days) and the average follow up was 11.8 months ( range 1 - 42 months) according to the study. See the study link below.

Styloidogenic Jugular Venous Compression Syndrome: Clinical Features and Case Series

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I’m really impressed with your perseverance, advocating & technical abilities, you’ve done so well to get this far! So frustrating after all that hard work to find Canadian doctors are reluctant to do the surgery…
I would say that we do have people complaining of brain fog/ memory issues, usually those with vascular ES. And either hearing loss or hyperacusis (acute sensitivity to sounds) have been mentioned quite a bit too. I had brain fog & some hearing loss after bilateral jugular compression.
I couldn’t open the link to the research paper, so will have to have a look & see if I can find it elsewhere…we have got a section for posting research papers if you find any which might be of interest! I’m in the process of compiling a list of doctors who’ve authored research papers as this might be an avenue for members struggling to find doctors.
I’ll watch the video- thanks for that- really interesting the case you mentioned! We’ve had members who’ve had full body weakness & have asked if this could be related, but we weren’t sure if it was, now we know it could be!

Hi Jules,

Thanks to you and Isaiah for the useful links and guidance on this terrible condition. Many feel at home here where the world seems lonely and cruel simply because not many people understand this. Even family members think we exaggerating it. The domino effect to the body and the brain of this vascular version of eagles is huge and not many research exist as it is rare.

I have uploaded the PDF form of the research paper by Dr. Peter Nakaji et all below.

Styloidogenic Jugular Venous Compression Syndrome Clinical Features and Case.pdf (674.1 KB)

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@KoolDude, that’s quite a story. As one who lives in Canada, I can imagine what kind of hell you went through to get it all done…

Out of curiosity, I can’t but ask - do you think you might have loss of cervical lordosis, aka military neck, aka straight neck, aka cervical kyphosis too? (https://www.healthline.com/health/military-neck)

A while ago I noticed correlation between enlarged styloid processes and military neck among symptomatic cases, and started thinking if the extra calcification in some cases could be caused by the body trying to stabilize the neck (or vice versa-trying to avoid impingement and straightening the neck).

hi KD thank you for this information and the overview with imaging - very impressive!
I am also fairly certain that atlas / styloid is part of my clinical picture and have done similar imaging evaluation using radiant, slicer etc but not to the same degree you have

to date I have had only cta to evaluate and have included some pics where it is likely compression is happening (left side styloid / c1). it appears as if the internal jugular is being squished by the atlas on left side. my left side measures at 4.2 and right is 3.6 but thicker. I was told by a neurosurgeon thatt they could tie the IJV in a bow and my body would still function normally

just curious - where did you get the venography and ultrasound studies done? what type of doctor sent the referral to get them done , and what notes did they put on the req? also living in the Toronto area. thank you



Hi VDM,

Always glad to see a fellow Canadian in here. I am pretty sure I have that military neck but I never sought help or therapy for it. One of my many MRIs was for Cervical spine and it did show mild multi-disk degeneration on the cervical area which I think contributes to the development of this military neck. I also realized that my neck is not only super straight, it is slightly tilted to left. I am computer scientist by profession so I spend a lot of time sitting in front of computers with poor posture so this could have also contributed.

BTW, I wear soft neck color which seems to help alleviate some of the neck pain and facial numbness from the styloid compression.

Hi bmcdiddie,

Glad to see another Torontonian here. I am not a radiologist (just aspiring google doc) and can’t of course give medical opinion but given the images you provided, I do think your C1 arch/transverse process is doing the majority of the left jugular compression with some involvement from you left Styloid near the base of the skull where it originates.

As far as how I got the referral for the Venogram and the ultrasound, it is really a long story and included many doctors. First, I had MR Angiogram which showed a tale tale sign of intercranial hypertension mainly partial empty sella and CFS fluid in the optic nerve sheath but was reported as unremarkable. I then had to send that MRI to another radiologist in the states for a second read and did confirm that it showed high pressure in my brain. So I started googling what causes Intercranial pressure and I came across articles detailing how venous stenosis (narrowing) can cause it so that is when I started learning 3D slicer to segment the venous system from the MR Angiogram and it was clear as shown below that my dominant jugular vein was compressed and that the right transverse, sigmoid and jugular vein were congenitally stenosed. Since the MRA only covered the head, I needed something that can show at neck level. So since my personal doctor and I have bit of rocky relationship over the years since she could not find anything wrong from previous tests and thought that I was anxious and was fine otherwise, I had to fight her to send me CT Scan of the Neck but she refused and settled on ultrasound on the neck. She told me that they only one they use was Carotid Duplex Ultrasound so I requested her to give me that. Once, I was with the technologist who was doing the ultrasound, I requested her to do the Jugular Veins as well which she agreed. During the ultrasound, she was surprised to see the high speeds but since she was not allowed to give her opinion, she told me to turn my head left and right and I can see that she was seeing something. Anyways, long story short, the ultrasound was read as unremarkable and my doctor received yet another unremarkable report. that is when I had to learn how to read vascular ultrasound, and only then I was able to see that my jugulars were not evaluated at all. only Carotid arteries were evaluated. So I contacted the radiologist who did it and asked him to evaluate my ultrasound and confronted him with high speeds. Only then, an addendum to report was produced and CT Scan requested to further evaluate the causes of the high speed. Again they reported the CT Scan unremarkable…Anyways, I contacted a doctor in St Michael’s hospital who is a vascular surgeon and gave him the revised ultrasound report and the CT scan images I did and he agreed to do the Catheter Angiogram/Venogram to settle the story . His name is Dr. Julian Spears and he is head of the neurovascular clinic along with DR. Vitor Pereira who is vein specialist. They are very busy doctors so you will need to get them referred to you by any physician.

Regarding your doctor saying that you can tie it to a bow and you will be fine. I am missing the context here. total occlusion of the jugular vein can indeed kill you so I do not understand his point. May be he meant that you have 2 of them so you can live with one functioning and that is true but I do not know tying to the bow thing,

Sorry for the long story but I just want to show the battle I had to go through to get to a diagnoses in public healthcare system. Hope this long boring story helps you but I wanted to prepare you for battle in order to advocate for yourself.

2020 MRA Angio with segmentation of the venous system which gave me the clue that something was compressing my jugular vein ( at the time I did not know Styloid - I had to learn anatomy again).

image

Ultrasound on my left and right Jugular Vein with respective velocities (137 cm/s and 200 cm/s). Blue means the blood leaving from the brain to the heart and red is blood going to the probe i.e regurgitating to the back to the brain due to stenosis. Jugular Veins should all be blue as they take the blood back to the heart

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@KoolDude, likewise. Not many Canadians are here, but I guess we are all in the same territory both figuratively and literally…

I also have a soft collar (nothing fancy, a 10 cad wrap with a velcro). And also noticed that it sometimes helps, just eventually developed hypersensitivity to anything wrapped around my neck, sometimes even the scarf is simply annoying to wear…

Talking about the military neck… I don’t have any definitive conclusion yet, but have seen a number of CTs begging the question: what if the causation of the compressed jugulars is coming from the atlanto-occipital joint changes, in particular the skull having shifted (glided) too far backwards over the time?..

My one of the speculations is that if the military neck extends “the boom” too far forward while sitting or walking, “the mike” becomes too heavy to be supported by postural muscles, specifically designed for this job. That is where various extensor muscles kick in, but the extensors are made of different muscle fibre, and over the time they become shorter, unable to fully relax. That might prevent the skull from returning back to its designated position on the C1 vertebrae.

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Fellow Ontarian here but I am in Ottawa. I will however soon be seeing a surgeon in Toronto. I was lucky to have testing (catheter angiogram and venogram in different positions)here that showed I have severe bilateral compression of both my internal jugular veins. I’ll post more after my appointment if it is positive. I would really like to know who the Canadian in this group, that is advocating for adequate care. I could possibly help out.

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Hi AnnieK, our wonderful Seenie, Admin & an Executive Director of Ben’s Friends is going to advocate for better care…I’ve sent you an invite for the discussion.

Thank you!

Kooldude,

Your advocacy and perseverence, as well as abilities to read these scans are incredible!!! YOU have a lot to teach us all so stick around for awhile!!! :wink:

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@vdm, It is definitely a plausible hypothesis since you found a correlation between osseous jugular compression and military neck. I wonder if they both originated from the same underlying pathology or as you put if the military neck combines with genetic susceptibility contribute to the developing it. Since the styloid process itself serves as an anchoring site for muscles and ligaments in normal scenario, it is conceivable that a continuous pull or prolonged extensions on some of the muscles it anchors can make it deviate its original path and induce vascular compression. Genetics could also play a role in developing elongated styloids. I remember reading it somewhere.

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Hi Aniek,

Glad to see an Ontarian from the tulip city. I used to live in Ottawa in the early nineties, beautiful city.
Also happy to see that you are lending a helping hand to the Canadian group. We really need an advocacy group here in Canada.

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Hi Snapple2020,

We all learn from each other. I have learned so much from here and happy to help in anyway I can. It is too bad that we really have to act as pseudo-radiologists to guide the doctors and eventually get the care we deserve. I have noticed most of the sufferers of Eagle Syndrome be it vascular or the classic one, have to learn to interpret their imaging since medical establishment have no knowledge or are unwilling to help. We are lucky we live in information technology age where a few googling or youtubing can return lectures on any imaginable topic you might be interested in.

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