Dilated Collaterals Cause Progressive Weakness on All Limbs as result of Bilateral Jugular Vein Occlusion - Interesting IJV Bypass Treatment

[DogLover]

@Dontgiveup I have ALL the symptoms you described in your original post, including severe leg, hip, back pain and stiffness which comes and goes and severe breathlessness and tachycardia and temperature changes. This is a picture of my collaterals.

Also at the front which I’ve tried to look into and it doesn’t seem normal to me. Does anyone else have this?

There also seems to be quite a lot of gubbins around the vertebral artery on the left hand side which I have wondered about as so many symptoms resemble vertebral artery insufficiency.

[KoolDude]

@DogLover My God. I think you have pretty bad dilated collaterals. One side is bit worse than the other, probably due to severe compression on that side although I can’t see it. BTW, I suffer from many symptoms as you such as memory impairment, brain fog, arrhythmia (Heart Block, Tachycardia when asleep), weakness/tightness/numbness on all the extremity, cognitive issues such as writing and reading issues (you will see a lot of spelling/omitted words in my posts if you carefully examine them), Insomnia and sleep jerks, bladder issues, I can go on and on…do not want to bore people with my endless list of symptoms. Although, we do not know the exact mechanism of how some of these symptoms are caused, I assume they primarily stem from blood circulation impairment related to IJVS & Nerve compression based on many studies, hence the cascading effect to multiple systems of the body, Central Nervous System, Peripheral Nervous System, Autonomic Nervous System (arrhythmias, temperature dysregulation, postural issues), Gastrointestinal System (Vagus Nerve involvement - Gastroparesis, intestine mobility issues), Endocrine System (Through raised Intercranial Pressure affecting pituitary gland) and lastly Glymphatic System (Vessels that clear the waste from the brain during sleep - impairment is linked to all kinds of neurodegenerative diseases and brain atrophy since CSF can’t be cleared due to impairment of venous outflow - The glymphatic pathway in neurological disorders. )

[KoolDude]

@DogLover I am wondering if you have calcification on your Carotid artery (shown below) or is it just an artifact on the 3D rendering of the CTV? I am wondering if you have axial images close to the Carotid Bifurcation so we can can confirm it, I know CTV is meant to visualize venous system as opposed to arterial but should show calcification regardless.

[DogLover]

Thanks I will read that.

Interestingly my compression of my right jugular isn’t that bad. But you can see the collaterals quite clearly here too, on the left and right.

Vertebral veins below seem to be doing quite a lot of work here too??

To my untrained eye my upright MRI also looks pretty congested above the jugular vein. Is this what reflux looks like on MRI I wonder.

[DogLover]

Well spotted! Yes I think so too but no one has mentioned this as an issue and when I googled it it says it’s fairly common!

[DogLover]

Your symptoms sound very similar to mine. My whole system seems shot! I had a lumbar puncture last week and since then then brain fog (fog - sounds so innocuous - more like brain concrete) seems a little better but other stuff is worse. Extreme pain in head on right after I cough and as soon as my leaking from eyes and nose starts. My heart rate is all over the place. Randomly peaked at 149 while sitting last night and stayed at about 100 for a few hours. It makes me think the cognitive and brain issues may be due to intracranial hypertension even though there was no indications on my MRIs. Has your hypertension resolved at all and did it make any difference?

[KoolDude]

You are right in that intercranial hypertension could partly explain the cognitive and brain issues. I never had lumber puncture so my hypertension has never been lowered/changed to see symptoms either improve or get worse. I never took Diamox (diuretics) either. I suspect I do intermittent leak CSF from the nose and the ears at times. I stayed away from things that increase CSF such as vitamin A intake and salty foods…etc. As far as the MRI goes, my hypertension shows as Partial Empty Sella and dilated optic nerve sheath with tortuous optic nerve. I can also see enlarged subarachnoid spaces on the frontal lobe which is indicative of CSF accumulation in the brain for some people and probably impacting my concentration and focus.

[KoolDude]

@DogLover One thing I would like to ask you is when they found brachiocephalic vein compression did they rule out any subclavian artery issues (I assume they did since you went through Catheter Angiogram). The reason why I am asking is, there little known condition called craniovascular hypertension thoracic outlet syndrome (TOS CVH) which causes increased hypertension in the brain. This is explained well in MSK Neurology site posted here (New paper associating vascular ES with IIH)

Source : New paper associating vascular ES with IIH

[DogLover]

It hasn’t really been looked for in detail as I’m convinced that they is a TOS element. TOS is one of the things I pursued in my journey trying to find out what the hell was going on. I’ve read this article before but I will re-read it thank you. I know some symptoms relate to lifting my arms and I have mentioned this to Dr Higgins. There is no treatment option for this yet as it is so new so I am really hoping that the styloid surgery gives me at least some relief.

For interest and for anyone looking for further causes for there symptoms, here is a snapshot of the braciocephalic You can see where the blood flow stops and also what looks like reflux in to anterior jugular (?) and collaterals

And another. You can watch the blood travel upwards as its injected. Not sure what vein on left of picture is. I can only think vertebral vein or external jugular

This one is shoulders shrugged which I had advised eased my symptoms. You can see improved flow and no collaterals or reflux.
.

[KoolDude]

@DogLover Yeah I can see the reflux and definitely that is contributing to the dilated collaterals. I was surprised at the severity of your dilated collaterals in axial images & 3D ones which did not correspond to the degree of compression of your Jugular Veins (which is moderate) so I thought based on the arm raising issue that you had, this might have something to do with blood back-up to the Vertebral arteries back to the brain and follow back to the collaterals causing secondary IIH hence the dilated collaterals as explained by KJETIL LARSEN in his article.

BTW, the images are in the venous phase, so we really do not see the subclavian arteries here but this issue is Compression of the distal subclavian artery called TOS CVH and I think it will be wise to talk to Dr. Higgins to investigate if there is any compression of the subclavian arteries as well. Since he has the Angiogram already, he can look again or order another non-invasive angiogram to see if the subclavian arteries are narrowed or occluded when you raise your arm. It might also be that brachiocephalic vein compression might be contributing your IIH more than the jugular Vein compression. At this point, it is just guess.

[DogLover]

Yes I think you’re right and I will ask him after the surgery. I’ve checked the subclavian arteries on my previous CT angiogram and they look fine but of course arms are not lifted and the results can be pretty dramatic when I lift my arms so I will pursue this. Thanks for you help. Have you considered lumbar puncture for your IH? My pressure was normal incidentally.

[KoolDude]

@DogLover No I did not as I noticed that I intermittently leak CSF in my nose and ears. This ironically keeps my CSF pressure down to a level that I predict will be normal if I do LP. The other reason is, my eye doctor could not find Papilledema which is good marker for extremely raised CSF.

Question, given the axial images you shared, I do not see Styloid compression on your Jugulars, seems more like C1 compression. Are they removing it because it is calcified (messing with Carotid/nerves) or because it is actually compressing your Jugular Vein against C1 but you did not share those slides here.

[DogLover]

I think it is wedging the jugular in against the transverse process. Also I have many symptoms that seem to fit with styloid, pain, nerve spasm etc

[KoolDude]

Ok…I see it is slightly compressing it against muscle in the vicinity of C1 but I am still baffled by the engorgement of your collaterals. I assume the surgery will be on the right then.

[DogLover]

Yes surgery on the right.

Yes me too on collaterals. I can only assume brachicocephalic but it is on both sides. I’m also worse upright. Maybe the vertebral veins are blocked. I’m very confused.

[KoolDude]

@DogLover do they know what caused brachiocephalic vein compression?. Based on the amount of dilated collaterals and other veins such as facial veins, I am beginning to think the central vein compression might be contributing more than IJVs which seem moderately narrowed by C1. I am not a doctor but my untrained logical brain based on the images shared makes me very suspicious that brachiocephalic vein compression is responsible a good portion of these venous engorgement through retrograde blood flow to the neck veins. So it would have been ideal, if they FIRST address the brachiocephalic vein compression through endovascular stent (assuming no bone or other extrinsic compression) which is less invasive. That way if a good portion of your symptoms disappear, you might decide whether to pursue Styloidectomy or not. Anyways, I know Dr. Higgins & Axon are both very reputable and experienced doctors so I do not doubt the soundness of their decision but you might want to discuss alternative strategies with them. I learned sometimes, even the best doctors overlook subtle issues that could have been addressed prior to other interventions since they are busy and do not focus individual cases too much. I also would like them to see axial images similar to the ones you posted here while doing the shrug thing that opens the brachiocephalic vein and establish if the massive collateral dilation goes away (I know they did this in catheter angiogram but was it evaluated at the brain level). This way they can also focus on the biggest offender be it brachiocephalic vein or IJVS.

Here is a comprehensive article about different types of TOS and how to detect it from MSK Neurology. You will need more time to read it but might be of help to you, just in case.

How to truly identify and treat thoracic outlet syndrome (TOS)

[DogLover]

Thanks so much for your thoughts @KoolDude; I really appreciate it. My logical brain which was an asset (I used to be a software developer, a computer programmer back in my day) is really not working these days!
Unfortunately there is no protocol to treat brachiocephalic vein compression at the moment; I’m not sure it’s even a recognised problem yet. I thought Vascular Eagle was rare! Maybe this isn’t rare, maybe just under diagnosed. I can’t find anything else online except a paper on transient amnesia, several on stenosis relating to fistula treatment for diabetes and one comprehensive paper on venous reflux -
Left brachiocephalic vein (1) is compressed to occlusion between the… | Download Scientific Diagram (researchgate.net).

I had a CT venogram with shoulders shrugged too, which strangely showed little difference and I have studied it extensively. I can only think it was a slightly different position.

Dr Higgins advised that stenting the brachiocephalic has a high risk of thrombosis in his experience and that treatment for this condition is a number of years away

I am hopeful that the op for the jugular will make some difference as this is the area all my discomfort and pressure is. Dr Higgins is fairly optimistic and certainly the ballooning of the jugular gave me noticeable but temporary relief. I also suspect that perhaps the compression of the jugular is much worse with head movement as I know tilting my head down definitely makes things worse and sometimes turning left and right even a fraction brings on the pulsatile tinnitus. I also think you were right that general inflammation of the carotid sheaf is exacerbating many of my symptoms and I think that a number of facial, neck, back muscles are involved, certainly the scalene. .

My theory is that it all stems from something structural, as I know my body is twisted and this is the one thing I can identify in my daughter, who is also showing symptoms worryingly. My atlas is rotated on the right I’ve noticed. Apparently a hereditary element is something Dr Higgins has already come across. My father had all of my symptoms, even the drooping eyelid, left leg issues, right hand tremor and he ended up bed bound. He was diagnosed with MS, as was his mother before him, but had no MRI, so it was a diagnosis of exclusion. Whether he was misdiagnosed or the conditions are related is a debate for another day. Either that or it’s a coincidence which seems unlikely.

My only hope with the brachiocephalic is to try and improve core strength and posture to try and lift the sternum off the vein. I also have an aberrant right subclavian which may make a difference but my impression from Dr Higgins was that he didn’t think so. This is the CT for interest.

Any thoughts? Yow would think if they could shave the atlas they could shave the sternum?

Sorry I have gone so much off topic. Maybe it will help someone else looking for answers.

BTW I wanted to let you know that my lumbar puncture seems to have cleared my head a little for the last few days! Hence why I’m back on this forum; don’t usually feel up to it.

Thanks again

[KoolDude]

Wow @DogLover I really admire your skills in both research and imaging (all self-thought I assume). You remind me of ME . I have the same background as you (not only in symptoms ) in that I am computer scientist with many years of software engineering and architecture. I also have degree in Physics so I am bit of science enthusiast. If I go on a tangent here a bit, many folks who are not into software engineering do not realize that we play a jack of all trades, master of none when it comes to many things. I think due to working with many different software industries, if you are developing software for genetics (GENOME software), for example, you will work with geneticists in order to develop it as they are the subject matter expert but learn a lot in the process same as other industries be it financial, scientific, medical…etc. So point being, you also master googling as you are better equipped with querying it more efficiently and finding items of interest as we understand how search engines parse queries. My 7 year old daughter suffered from a rare esophageal inflammatory disease called in short form EOE (Eosinophilic Esophagitis), after years of remaining undiagnosed, Google assisted me to diagnose her based on her symptoms and subsequent upper GI scope biopsy confirmed the disease. So while you are bit impaired due to ES/BVC, do not underestimate the competitive advantage of your computer skills.

Back to the topic at hand, I think the good news is we know with some degree of accuracy, based on the imaging and post LP symptom relief, that raised intercranial pressure is at least partially responsible for your symptoms and that both IJVS and Brachiocephalic Vein Compression (BVC) have role in this. What we do not know is how much from each of these compressions is contributing to the dilated veins. With the limited images you shared so far, I am leaning towards 40% from IJVS and 60% from BVC. There are 2 reasons why I am leaning towards BVC. One all the studies I have read thus far that contained severe to near-occlusion IJVS, produced similar number of dilated collaterals that you showed us in the images you shared but your IJVS do not seem severe or near-occlusion. Secondly, the image that you just shared with us shows pretty severe stenosis of BV compared to mine (I did compare them, see below). So now your whole facial & neck veins (front & back) are dilated and god knows how many nerves/nerve exists they are compressing producing all these weird symptoms.

I believe they should shave the atlas (I trust Dr Axon & Higgins to be expert in this) since it appears to be causing most of the compression based on the limited images you shared. As far as the sternum is concerned, I really do not know what the intervention would be as I have not done a lot of research in that area and as you noted only few studies exist. I can say with confidence though, that stenting, regardless of the type of vein, carries risk of clotting as there are a number of studies linking to it and one might need to be on blood thinners for long time once stented. In your BVC case, I would stay away from it as Higgins advised also noted in this study (Crushed stents in benign left
brachiocephalic vein stenoses) that without removing the extrinsic compression, it will be crushed. I wonder if they could nudge the Aorta Arch a bit somehow without shaving the sternum I guess that is not easy since they will have to open chest wall just like the open heart surgery. Looks to me the orientation of the Aorta Arch angle is compressing it against the sternum although your sternum also seems bit thicker (don’t know if it is due to magnification here).

Here is my chest CT (Red arrow pointing to BV and Cyan Arrow pointing to Aorta Arch). Second image Aorta is not visible.

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I think you have a good plan. If the surgery in March does not significantly reduce your symptoms, then BVC intervention is warranted. I know Higgins said it is years away but it is few millimeters away from total occlusion based on the image (perhaps, something to investigate when you stand up and raise your arms how that can dynamically interact with the sternum/Aorta arch movement). It could be that standing up and raising arms might be compressing it more hence all the symptoms. One hypothesis is, if we assume that IJV collapses significantly in standing up and BVC compression gets worse, then this is the double whammy that you so elegantly put that your whole body would be shot

Sorry for the long notes ( By now, everyone here knows, here comes KoolDude with his long rambling).
I am praying that your surgery goes well and that you have plan B if this does not work well. All in all things are looking good.

[Dontgiveup]

In regards to a styloidectomy/C1 shave, what are potential reasons why surgery would potentially not help? I understand that recovery is different for everyone and time plays an important part. I was talking to someone who was suffering pretty badly for weeks after her surgery but at around the 3 month mark, all of her symptoms had resolved.

But what would be the reason for the jugular vein remaining closed post surgery? Shouldn’t taking away the bony obstruction eventually resolve jugular compression?

I was talking to someone else who is a couple months out from surgery and is still struggling and considering a stent. I think more recovery time is needed, but is there a reason why after surgery, the jugular would collapse/remain closed?

[KoolDude]

@DogLover On close scrutiny, I hope it is just my eyes, but there is tiny streak of clot formation on where it is most compressed. You can see the tiny dark line demarcated by white line pointed by the Cyan Arrow below. Did they find any clot formation in CT Angio? I hope this is just my eyes or imaging artifacts.