Possible jugular compression, CSF leak, intercranial hypertension. Advice?

[jrodefeld]

A have a few updates to share with everyone:

I received my recommendation letter from Dr Hui finally. I expected to receive it within a week but I was told he was dealing with a lot of surgeries and other issues so it was delayed.

Here’s what it said:

Observations:

You’ve now underwent a cerebral venogram/angiogram with Dr. McDougall. After reviewing the images, it confirms that you have impingement of your jugular veins most pronounced in the RIJV, predominantly related to residual Cl compression.

Post operative scarring and re-stenosis may also contribute to continued R IJV Cl stenosis.

Recommendations:

As such, given your symptoms, I believe that decompression would be of benefit to you. The residual R IJV stenosis is the most severe, and I would address that first. The targets for treatments could either be the residual styloid stump, or the soft tissue component of the narrowing. Open surgical revision of the styloid may help alternatively a stent may be feasible given that much of the styloid has been removed previously.

The R Transverse sinus is also static - which may be associated with the brain fog and discomfort you are experiencing. The flow in the R transverse sinus should improve once the R IJV is opened.

Conclusion:

I would consider either stenting of the R IJV C1 level or surgical revision of the C1 styloid/transverse process to further augment your cerebral venous drainage, resulting in reduced drainage of your right brain.

Hopefully, this will improve your head pain/pressure and brain fog and other related symptoms.

I am inquiring whether Dr. McDougall would be willing to do a stent. Revision of the C1 may be pursued with Dr. Hepworth, Dr. Nakaji or Dr. Lawton.

It feels really good to get validation for this and hopefully I’ll be having surgery to resolve this soon.

My gut tells me I should wait for Dr Hepworth, but part of me wants to have anyone (who’s qualified) do the operation who can schedule me the soonest.

I did have the Cisternogram testing to check for a CSF leak in my sinuses. They did both a Cisternogram test AND placed pledgets in my nose and left them there for more than 24 hours.

They did NOT find a leak.

At this point I probably have to conclude that I don’t have a leak (in my sinuses or skull base) and the earlier test suggesting I did was mistakenly interpreted.

I still feel I have a leak somewhere given my very strong positional headaches. I feel so bad after being up for a couple of hours and many symptoms seem to improve after lying down for a little while.

It could be I have a spinal leak and not a nasal or skull base leak?

I have a suspicious sacral tarlov cyst that may warrant closer examination.

Anyway, what the sinus doctor at OHSU did find was chronic sinusitis and a severely deviated septum. He gave me some strong medicine and I’m supposed to do a nasal irrigation procedure a couple of times a day for a few months.

Hopefully that helps, but I have a suspicion that the jugular compression could be related to the sinus issues since I probably have impaired lymphatic drainage especially on the right side of my face.

This is probably another reason to wait to see Dr Hepworth.

[Jules]

That’s good you have confirmation now! It’s a personal decision as to whether you go with whoever can do the surgery (they’re all respected surgeons…) or whether you go with your gut feeling & wait for Dr Hepworth, whoever you decide to have do the surgery it sounds as if a decompression would maybe be worth considering before seeing if a stent’s needed? The stenting has mixed opinions from what I’ve heard/ read…

[Isaiah_40_31]

I second @Jules comment about the stent. There have actually been cases where a patient had a stent placed without having decompression done first & the sources of IJV compression bent the stent & caused it to become worthless. Since stents aren’t removable, or not easily so, it may be best to have decompression first to see which symptoms resolve.

[jrodefeld]

Yes, I agree completely.

I would like to avoid having a stent unless absolutely necessary so it certainly seems wise to have a decompression surgery to see if the vein is able to open up and stay open and only pursue a stent if I need to once I recover from surgery.

[jrodefeld]

Still waiting for my appointment with Dr Hepworth which is coming up the first week in August, so under three months to go.

In the meantime, I’m working with my primary doctor and a few specialists here in Oregon to hopefully help me manage my symptoms while I wait.

Today I saw a vascular doctor to discuss all of this. Since I have a history of Thoracic Outlet Syndrome and some residual arm pain, they did an ultrasound exam of both arms with provocative maneuvers.

As far as that goes, everything looked pretty good.

I showed him the venography images from Dr McDougall as well as Dr Hui’s letter explaining the cerebral venous congestion and expressed my concerns about it.

He urged me to avoid a stent if I can, which is something I completely agree with.

I did ask his opinion about doing a trial of blood thinners to see if they improve my symptoms. I thought I would have to do some convincing, but to my surprise he agreed that it made sense right away.

I mentioned that Dr Hepworth often prescribes Plavix, but he didn’t agree with that. He said that Plavix is usually prescribed for arterial problems, and instead prescribed me a trial of Xarelto, which is a traditional anticoagulant for venous clots and obstructions.

In fact, I’m not even sure why Dr Hepworth uses Plavix instead of Xarelto or Eliquis?

Since I have read studies that people with this condition also respond favorably to venous anticoagulants, I agreed to give it a try.

Has anyone had favorable results from Xarelto for jugular vein compression and cerebral venous congestion?

The doctor did suggest I have someone do a balloon angioplasty to try to open up the vein and see if that alleviated my symptoms.

Is there any reason not to try to temporarily open up the vein this way as a diagnostic tool when there is suspected bony compression?

[Isaiah_40_31]

@jrodefeld - Good to hear from you & thank you for the update.

@jrodefeld

I mentioned that Dr Hepworth often prescribes Plavix, but he didn’t agree with that. He said that Plavix is usually prescribed for arterial problems, and instead prescribed me a trial of Xarelto

Dr. Hepworth Rxed Plavix several years ago but for the last year + has his patience take Brilinta which is designed for heart attack survivors. If a person has problems with the Brilinta he changes them over to Xarelto so you’re on a medication Dr. Hepworth is currently suggesting for his patients. We have members who’ve had great results w/ Brilinta & some who have not i.e. it made their symptoms worse. I don’t know if any went on to take Xarelto instead but I expect it should be helpful. You can let us know. It can take 2-4 weeks for significant results though if it makes you feel worse over the first to second week, it would be good to stop taking it & consult your doctor.

@jrodefeld

The doctor did suggest I have someone do a balloon angioplasty to try to open up the vein and see if that alleviated my symptoms.

Is there any reason not to try to temporarily open up the vein this way as a diagnostic tool when there is suspected bony compression?

The reason not to have your vein ballooned while it’s compressed by bony structures is that it will simply recompress as long as C1 & your styloid are pressing on it. The source of compression needs to be removed before ballooning will help. The hope, however, is that ballooning won’t be necessary once the sources of compression are alleviated.

I’m also in your camp regarding stents & feel that IJV stents near the skull are to be avoided if possible.

[Jules]

I hope that the medication helps you! I agree with @Isaiah_40_31 that it would seem a little pointless doing a balloon angioplasty while there’s still significant compression; presumably there’s some risk with that procedure so personally I’d avoid anything invasive like that even if it’s minimal…just my personal opinion, not a medical one!

[jrodefeld]

I was looking through this forum and came across a very interesting conversation about Ponticulus Posticus, otherwise known as arcuate foramen, and it’s potential relationship to vertebral artery compression or Bow Hunter Syndrome.

I have symptoms that seem to correspond to vertebral artery compression and thought that perhaps an unusually formed Atlas could be the link between jugular compression and potentially vertebral artery compression.

I took some images of my Atlas from 3D Slicer. I’m not too good at reading these, but could anyone tell me if I have the anatomy for ponticulus posticus?

I’m not sure if these images show the right angles to make the determination.

As much as I’d hate to have another rare disorder, I’d prefer to know what’s going on obviously.

Thanks!

[Isaiah_40_31]

In the top picture, your C1 vertebra does look abnormally shaped, but I don’t know enough about the arcuate foramen ligament to comment. There is good information on the internet about Ponticulus Posticus, & as you’ve seen, there are people on our forum who know a little about it.

[jrodefeld]

I’ve been doing additional research trying to see if I may have any of the other (related) conditions people seem to have when they have Eagle syndrome/jugular compression.

I was looking at my lumbar spine MRI I had last year. It does show a tarlov cyst at S2 in my sacrum, which may be responsible for nerve compression and possibly be a site of CSF leakage.

However, it appears to my untrained eye that I may have tethered cord syndrome. Does anyone know what I should be looking for to verify this?

Regarding the ponticulus posticus, it may be an irrelevant anomoly that has no clinical significance in my case. I had a CTA of my vertebral arteries and everything looked good. I also had a carotid and vertebral artery ultrasound, with some provocative maneuvers. I haven’t heard back yet, but I assume if they found something really wrong they would have contacted me by now.

A little over six weeks to go before my visit with Dr Hepworth and I am counting down the days. Hopefully he can get me on the surgery schedule pretty quickly.

[Isaiah_40_31]

There’s a tethered cord discussion going on in this thread. It might be helpful. I don’t know what tethered cord looks like on scans so can’t comment on the one you posted.

Dr Fargen

Most surgeons and radiologists don’t know what to look for. Here is my MRI and the views needed to evaluate. The arrow in the right image was drawn by Dr Petra Klinge who performed my dithering surgery. I have a subscription to RadiAnt so I could do a cursory review for you to see if he looks like me. What are his genetics? I am West Bohemian on my father’s side which seems to show the ehlers danlos. I have met other Northern European mutts who have similar symptoms and histories. Lol, I guess w…

[jrodefeld]

Thanks. I posted in that other thread. I read that one of the characteristics of this is “low lying Conus Medullaris”, which I believe is the dark part in the spinal canal. Apparently, it is supposed to end around L1 or so, but in my case it looks like it’s nearly down to L5.

Like you, I’m not an expert at reading these scans so I could be mistaken.