Jugular Compression, possible CSF leak & hypermobility

General
1

Hello,

I’ve been reading all your posts for many months now, and am very grateful for such an open, informative and supportive group. Thank you all for being here.

Like many of you, I’ve suffered from various and difficult to define issues for over a decade now, but finally made my way through the medical jungle and have been diagnosed by Dr. Hepworth with bilateral jugular compression due to fat and elongated styloids, and muscular entanglement on my left side. This was done via a CT scan (with contrast) and bilateral jugular ultrasound. I’ve been on his surgery schedule for a few months now, and just got lucky enough to be moved up to November 30th for a full left side styloidectomy. Dr. Hepworth said he’s 90% confident that if the left side surgery is successful that I won’t need a second surgery on my right side, even though I have elongated and thickened styloids compressing my jugular vein on both sides. He also said that 80% of his patients like me see at least a 50% improvement after surgery.

This week an appointment I scheduled over 8 months ago with Dr. Birlea, the head of the UC Health Neurology department, also came through and I did a video call with him. After reviewing my CT scan (with contrast) and discussing my symptoms, Dr. Birlea said he isn’t convinced that I need a styloidectomy, and that my issue may be partially a CSF leak (especially considering that I am hypermobile and my neck has straightened so there’s no curve anymore), and he recommended a full spine MRI with contrast, as well as seeing an optometrist to determine whether there’s any pressure behind my eyes. At the same time he confirmed that my symptoms don’t exactly track with typical CSF leak, nor with “typical” Eagles Syndrome. When I asked him if he thought the potential benefits of a styloidectomy outweigh the risk, he said he’s 50/50.

When we got into more detail, Dr. Birlea made it clear that if I do have a CSF leak, and they patch that leak, then my symptoms could get significantly worse due to the jugular compression. He thought that’s probably why Dr. Hepworth is recommending the styloidectomy prior to any further investigation of a possible CSF leak. Nevertheless, Dr. Birlea felt he can’t recommend surgery until more diagnostics for potential CSF leak are done.

I just had a short call with Dr. Hepworth’s PA, Alison Love and asked about this differing opinion, and she confirmed that their office regularly deals with CSF leaks, and are aware of that as a potential issue in my case. However, she stated that there was no point in worrying about a potential CSF leak at this point in time, because the jugular compression needs to be dealt with first. She also said that if they can fix the jugular compression issue, and I do have a CSF leak, there’s a good chance that my body would repair the CSF leak without intervention once normal blood flow is returned.

From what I’ve read here, it seems like I should trust Dr. Hepworth and go ahead with the surgery in a couple weeks, but I’d love to hear all your informed opinions as many of you seem to have dealt with competing opinions from different doctors in your journeys.

To give a little more context, the majority of my symptoms seem to align with the issues caused by jugular compression, but not all of them are very clear. My symptoms are:

Brain fog
Fatigue
Lightheadedness
Occasional Imbalance
Temporal pressure
Neck stiffness
Vision blackening with position change
Gastrointestinal issues
Word finding difficulty and memory problems
Heart beat audible and rapid in my ears with minor exertion
Occassional muscle weakness in legs/arms
Hypotension
Significant changes in blood pressure with position change (20 point drop between sitting and standing)
Crackling in my ears with jaw movement (not popping or snapping. Sounds like shrimp eating underwater)
Very slight tinnitus that comes and goes
Low grade constant headache
Exertion induced headaches
Severe menstrual headaches
Difficulty sleeping
Severe headaches that wake me from sleep, usually around 3am and are significantly worse laying down than sitting or standing.
Headaches that come on in the evening and worsen throughout the night
Severe cramping in calves as a precursor to nighttime headaches

Advil has always been somewhat effective at stopping most of my pain, but it leaves me groggy, exhausted and unfocused, especially when taken at night. It’s also started to cause some nausea and stomach issues as I’m taking it at least 2x a week at this point. Dr. Hepworth put me on blood thinners, which have made it so I occasionally get 2-4 good days before a severe headache event. A different neurologist prescribed nortriptyline, which has gotten rid of the radiating pain from the base of my skull forwards, but otherwise doesn’t seem to have much effect.

I’m certainly desperate for relief and a return to being a fully functioning human, but of course surgery is scary and has it’s own risks. So, my questions for all of you are:

  1. Is there any negative effect of not having styloids? (not including inherent risks of surgery)
  2. Does it track with your experience that jugular compression should be addressed prior to CSF leak?
  3. Has anyone else seen both Dr. Hepworth and Dr. Birlea? Any opinions on which to trust in this case?
  4. Does being hypermobile positively or negatively effect outcomes of a Styloidectomy?
  5. Should I bother with the full spine MRI with contrast prior to or after my Styloidectomy?
  6. Are any of the symptoms listed NOT possibly caused by jugular compression?
  7. Are any of the symptoms listed specifically related to CSF leak? (My preliminary research indicates the opposite, especially for the headaches that are worse laying down)

Thank you all for reading and I look forward to your replies.

2 Likes
2

@MsBearshark first, please welcome. Secondly I am really glad you found the best doctor to deal with this - Dr. Hepworth. Do not listen to any other doctor and proceed with surgery as the evidence based on imaging and ultrasound blood flow study indicated outflow obstruction by styloid compression of your jugular veins. I believe you will benefit from it.

CSF leaks can be caused by raised intercranial hypertension resulting from Jugular compression. The outflow obstruction creates a backup of blood + fluid back to the brain and if you happen to have a weak dura or skull bone somewhere, it leaks. So opening the Jugular veins, should in theory resolve any CSF leaks stemming from outflow obstruction. This was found in a number of studies but the study below was written by a world renown vascular surgeon in UK, Dr. Higgins. The study link is below the image, you can show this to Dr. Birlea. By the way, I do leak CSF from my nose from time to time and it has taste like metallic.

Here is a nice diagram illustrating the viscous cycle of CSF leak and IIH from the Study below.
image

Source Study : https://www.tandfonline.com/doi/pdf/10.1080/21641846.2021.1956223

Here is essence of the study regarding your case and I quote it below.

we have reported cases of spontaneous intracranial
hypotension caused by narrowing of the jugular veins at the same level, whose symptoms also resolved following treatment directed at the venous stenoses [31,32]. In these
cases, taking jugular venous obstruction as the underlying cause of intracranial hypotension, then a procedure (for example, jugular venous stenting) that would normally be
expected to reduce intracranial pressure, allowed the CSF leak to heal and intracranial
pressure to revert to normal.

3 Likes
3

Totally agree with @KoolDude- the increased intracranial pressure caused by the jugular compression has often caused CSF leaks for members, so the compression definitely needs addressing first, otherwise even if you hard one area patched you could still have further leaks. Dr Hepworth has repaired leaks during surgery as his PA says, & hopefully those who’ve had surgery with him can tell you about that.
I love your description of a shrimp eating underwater :joy: not had that symptom…
For your first question, the styloids are an anchor for quite a few ligaments, but it doesn’t seem to make much difference after surgery- with swallowing etc. There are risks of some nerves being affected- some members have had a lopsided smile, facial numbness or weakness, but this usually resolves with time. The accessory nerve can be affected too, which can cause arm weakness or pain. Nerves are usually monitored during surgery so the surgeon can see if they’re under stress.
We are seeing some members with CCI who have found this seem worse after surgery, you can search this in the discussions, your vascular symptoms are pretty intense though; personally I think when the styloids are causing vascular compression then there’s less choice about whether to have surgery…
I think that Dr Hepworth will ask for any imaging that he needs, Gadolinium is often used as a contrast medium with MRIs which can potentially have side effects, so unless there’s a suspicion of a spinal leak as well I wouldn’t worry about having the MRI before surgery- it’s something you can ask Dr Hepworth’s team though.
I think pretty much all your symptoms can be down to ES; you can use the search icon to look for any of them & there’s explanations of common symptoms in the Newbies Guide Section if you want to have a read. The vagus nerve can be irritated by the styloids which can cause fatigue, blood pressure fluctuations, GI issues. You’re right about headaches worse laying down being a symptom of IH, as well as pulsatile tinnitus, memory problems, vision issues, head pressure, lightheadedness… It might be an idea to see an optometrist though as Dr Birlea suggests just to check the pressure on the optic nerve, IH can cause that. Although removing the compression would be the sensible cure for that anyway. Not sure about the calf cramps how that fits in, hopefully someone with more knowledge can chip in about that!
My jugular compression & symptoms were less severe than yours, but mine resolved really well after surgery. It doesn’t always happen though as stenting can sometimes be needed as well, and both styloids removing. so be prepared for that.
Best wishes!

2 Likes
4

@MsBearshark - I can attest to the fact that, in general, the styloids don’t play a structurally significant role in the body. I had both of mine removed about 8 yrs ago & have been able to live my fully since then.

@Jules noted that some of our members who have ED/CCI/AAI have felt their necks’ hypermobility symptoms post op have worsened. The top CCI/AAI doctors in the US have stated that should not be the case as the styloids don’t play a role in cervical stability. We have speculated here that it’s possible that cervical instability itself is what caused the styloids to elongate &/or stylohyoid ligaments to calcify, thus for those w/ ED/CCI/AAI, the styloids may play a small role in cervical stability. This would explain a worsening of hypermobility symptoms after ES surgery. All that said, it is critical to take care of the IJV compression & potential CSF leak at this time as they are more significant problems. The ED symptoms can be addressed later should they become worse.

I also agree that the MRI should be put off till after ES surgery or indefinitely. There is info about gadolinium on our forum. It has had a very negative impact on some people. Gadolinium contrast

2 Likes
5

I LOVE the chart, @KoolDude! Thank you for sharing it. I’ve copied it & added it to my ES folder. It’s getting pretty full at this point. :joy:

2 Likes
6

I’ve wondered some of the same questions regarding CSF leaks. How are you doing now?

I had styloidectomy with Dr. Hepworth in January and now am trying to figure out the implications of this in presence of a possible CSF leak.

Dr.Creager, P.T. highly suspects a leak and I dread the possible journey of diagnosis and treatment.

It seems like maybe the old anatomy might have been holding some of that fluid in my head, and now it’s free to fall with gravity, maybe making some symptoms worse.

It’s very hard to tell, as I do feel more fatigued and more dysautonomic than before surgery, but I’m still recovering from surgery and a nasty stomach bug I got a month later.

7

@nnaeve I’m sorry to hear your styloidectomy did not cure all your symptoms. I’m in the same boat. I had a left side styloidectomy and ballooning of my IJV in December, with complete relief of symptoms for about 2 weeks, and then all my symptoms returned. I have documented IJV constriction on my right side at the styloid as well, so I always knew I might need a second styloidectomy. However, before going that route Dr. Hepworth has referred me to an interventional neuroradiologist for a catheter venogram, as my post surgery jugular ultrasound shows possible constriction in my IJV above the styloid in the transverse sinus region of my skull. I’m working my way towards getting that invasive diagnostic done, but it’s taking time to get referrals and appointments.

In the meantime the neurologist I’ve been seeing ordered a full spine MRI and suspects I have CSF leaks as well, but only found cysts (at every level of my spine) on the imaging. He is pushing for me to get a lumbar puncture, but I haven’t pursued that yet, as Dr. Hepworth has been consistent in saying that we must deal with the IJV compression first. If that is dealt with, any possible CSF leaks may heal on their own.

Have you seen Dr. Hepworth since your surgery? If not, schedule an appointment ASAP. Make sure you tell the scheduler that you’re post surgery and not doing well so that you can get in for an earlier appointment than his typical “next available”.

Have you had a post surgery jugular ultrasound done? If not, request a referral and get that going. It’s possible that your vein collapsed after surgery, or that you have a constriction somewhere else in the IJV.

From what I’ve learned going through this (and this is just my personal experience, not medical advice), if you had jugular vein compression at your styloid, there’s a high probability that you could have compression of the jugular vein at other locations as well. If that is the case, you’ll want to relieve all pressure in your veins before pursuing invasive testing for CSF leaks.

I wouldn’t recommend going forward with any invasive CSF leak test until you’ve seen Dr. Hepworth again and asked specifically about that. There is a non-invasive MRI procedure that can show CSF leaks in some cases, so you could go forward with that at this point if you’re concerned, but the results may be inconclusive.

Hang in there!

2 Likes
8

@MsBearshark - thank you for sharing great advice. We have at least one member who’s been dealing w/ unresolving CSF leaks (i.e. they get patched but patches don’t hold) & the reason was that IJV constriction still existed & needed to be resolved first. It can be a vicious cycle if the cause of the leak isn’t dealt with, & the leak just receives a “bandaid” which is likely not to stick.

@nnaeve - I’m sorry you’re still going the rounds w/ all this. Unfortunately, symptoms of vascular compression (i.e. CSF leaks among others) aren’t always straight forward to treat, as you’re learning. I hope Dr. Hepworth is able to get to the root of your recurrent symptoms quickly if you see him again.

1 Like