Hello all! I recently joined this group after learning this could be a possible part of my diagnostic and symptom constellation. I have hEDS and CCI with two prior cervical fusions front and back c4-6 and c3-6. I will need an upper cervical fusion however my neurosurgeon in New York referred me to Costantino after noting the possibility of jugular vein compression based on imaging and symptoms not improved while in my neck brace. I actually live in Colorado and attempted to get into Hepworth for months before this was on my radar for sinus issues but gave up due to the non-responsiveness of his office as it sounds like many of you have dealt with. Additionally, while I know he comes highly recommended by patients my neurologist at CU strongly advised against seeing him. But with my neurosurgeon right next to Costantino this worked out well other than travel .
I just got my CTV last week and Iām impatiently waiting results and my follow up. Iāve been told based on other imaging that the right side is the issue and I can see on the attached image C1 clearly pushing into the right (dominant) jugular. Which has me wondering is there a normal physiological version of this (insert nervous laughter) or is this more akin to compression. My C1 is unstable (confirmed with numerous dynamic imaging studies) and C0-1 so itās not stable in there either especially above a very solid fusion. Rotation and flexion makes it worse. But Iām lucky the fusion is so solid! I wish I could see whatās happening where my styloid is. My neurosurgeon has measured other images and I have a more narrow space between styloid and transverse process in the right side.
Any thoughts on this while I impatiently wait? Wish I knew what was going on here other than my fusion Front and back.
Are you able to open up the original imaging and find the axial view? If you go to the axial view and go to C1 I can see if your styloids are involved. Iāve attached my own imaging to help you know what to look for!
@mountangurl attached is your imaging with annotations. It appears your right ijv is pancaked against C1. But it looks like your right styloid is fairly distant from the ijv, at least in these particular axial slices. Your left side seems okay. Important to note that the vagus nerve runs between C1 and the ijv, so compression of the ijv almost inevitably means some compression of the vagus nerve.
Now that you know whatās what, you can follow the full length of your ijvs and styloids and see if compression is happening anywhere else!
Thank you! So it sounds like from some reading Iāve done as well that the C1 transverse process can be the main culprit. Is that a different type of diagnosis/treatment? Iām going to go dig more into these scans!
@mountangurl - The treatment is the same i.e. when the styloid is at play, it gets resected first to see if the IJV is then able to open sufficiently, if not and C1 is also at play, then the transverse process of C1 is shaved to some extent to make more space. In some cases, there are other soft tissue sources of compression either in the same area or lower down i.e. scar, muscle, fascia, nerve, other vascular or lymph nodes. Sometimes these need to be moved/removed, too, in order for full decompression to occur. Normally the styloid is also shortened even if C1 is more at fault for compression as elongated styloids/calcified stylohyoid ligaments can wreak havoc w/ some of the cranial nerves that āliveā in the neck or exit the skull near where the styloid is.
Dr. Costantino will be a fine & helpful doctor to help you with this. Iām sorry about your frustrating journey w/ Dr. Hepworthās ofc. Too many of us have been thereā¦He is a great doctor though.
I would say that your right IJV shows some flattening in your images but it still looks fairly large in terms of its potential for flow. An IR venogram with manometry would be able to determine how much pressure is being built up across that stenosis if any.
I looked up what that procedure does- so itās part of scanning for IIH? Interestingly, I have the opposite of that with spontaneous intracranial hypotension and brain sag with a spinal Csf leak. My last fibrin glue patch helped more than blood patches for a time. But Iām overall not doing well since. EDS has to complicate everythingā¦
The others have been helpful with info & imaging, so canāt add any more, but it does look as if the C1 is more of the culprit, & obviously as your C1 is unstable that doesnāt help! Weāve had quite a few discussions about CCI , and also about C1 shaves- some doctors think it might make instability worse, others say that it shouldnāt make any differenceā¦
Re the intracranial hypotension, weāve had quite a few members who have had intracranial hypertension because of IJV compression, then if thereās a weakness somewhere the increased pressure can cause a CSF leak, which can then cause hypotension. It then can cause a cycle between high pressures, leaks, low pressure, healing, high pressure againā¦Iām sorry that youāre not doing well now, sending you a hug
@mountangurl Regarding Orthostatic (better flat) Headaches/Pressures: Iāve already been the spinal CSF leak route with no leaks found on MRIs, CTM and DSMs at Cedars-Sinai. During that time, @Isaiah_40_31 helped me discover my bilateral, elongated styloids/IJV compressions on imaging. I had my right side surgery with Dr. Hepworth in December. Still symptomatic, he is doing my left side next week. One thing that gave me much hope regarding the better flat HAs is this paper by Dr. Fargen. If my upright HAs donāt resolve after surgery 2, then further options for me are to look into CCI and possibly go to Dr. Callen for another spinal CSF leak workup (since they are so hard to find, as you probably know). Anyway, check out this paper, as it explains how IJV compression can actually cause better-flat HAs. Best wishes and prayers to you!
Screw Hepworthā¦ Call UNC (University of North Carolina) for Dr. Trevor Hackman. He did the styloidectomy on me and did a FANTASTIC job. 984-974-6484.
Unfortunately, Dr. Hackman doesnāt deal with IJV compression when itās predominantly caused by C1. If itās only the styloid at fault, Dr. Hackman is a great resource.
Iām glad youāve had good results from your surgery w/ Dr. Hackman, @HappyBear.
Oh wow thank you that is very helpful! Iāll read it. Callen is my doc at the csf leak clinic. I do have positive imaging for a spinal leak (mri and myelogram) and each brain mri my BERN score for brain sag measurements is unchanged. I had a good response to a fibrin glue patch even though it wasnāt 100% or last. Itās all so complicated. Iāve read about spikey leaky syndrome too and sounds like something that makes a lot of sense.
I need my follow up to come sooner, Iām too impatient but really the severity of symptoms makes the hurry up and wait so hard. Everyone here Iām sure deals with that. Sigh.
A CSF leak is no easy thing to manage around, some medicos can, and often do, minimise the effects. But for some people they really can send your whole system for a spin. I donāt have Eagles, but Iāve had to deal with both intracranial hyper- and hypo- tension, neither is nice. But the hypo drained so much fluid I ended up with slit ventricle syndrome, where the walls of the ventricles (the CSF reservoir) were touching each other, and the symptoms were horrible bad, just awful.
Itās not āimpatienceā, you have a need, thatās different. The medico suggested a āWait and Watchā approach for me. My response, āWait for what? Things to get worse? My head to implode?ā in my view it felt like my face and my eyeballs were being sucked into my skull already, so waiting? WHY? Turns out Iām not a very nice patient, not a very āpatientā patient at all. When Iām symptomatic I tend to have a VERY short fuse, to then have a medico give me the āOhh you poor thingā¦ ā¦Youāll be OKā¦ā speech, ohh, that lights my fuse every time.
Is another patch possible? Even if itās just to manage symptoms. Some medicos are of the opinion if the āfixā didnāt work the first time, why do it again? For some the āpatchā doesnāt stick at all, but if youāve had partial success previously, is it an option? Some leaks are not easily accessible, so options can be limited.
It will be interesting to hear Dr Callens opinions.
@mountangurl - Iād suggest seeing Dr. Hepworth as he refers some of his patients to Dr. Callen, but he may be booking into Jan. next year. If you want to be seen sooner, Dr. Nakaji in Scottsdale, AZ, might have openings sooner.
ā¢Dr. Edward Hepworth, Denver Sinus Care, 3150 E 3rd Ave, Denver, CO 80206 (720) 899-9489, FAX (720) 953-5151, email: info@denversinuscare.com
Thank you, that is true. I do have my 4th patch scheduled late October. Since the fibrin helped another could do more. Now I wait for the Costantino follow up and see where I go from there with him and then neurosurgery. I do think my worst issues come from the upper cervical instability.
I did try Hepworths office again today and their system has changed with a new email for consults!
Iāll have to call Dr. Hepworthās ofc to see if we need to update their email information. Thank you for sharing your experience when you called their ofc today, @mountangurl.
Itās a constellation but significantly severe neck pain and spasms, suboccipital and occipital pain/burning/neuralgia, Trigeminal neuralgia, severe orthostatic intolerance. Stabbing head pains. Head pressure in the front and also back/pulling. Iām unstable and fall, severe fatigue and brain fog, tongue goes numbā¦Iām missing things lol.
Update on Hepworths office. After emailing I just got a call today and will see his PA in less than two weeks. She scheduled me with him for November.
@mountangurl - Thank you for the update about your contact w/ Dr. Hepworthās ofc. Thatās a pretty incredible turnaround time for them. I think they really are working at improving their front office/patient contact system. I did add Kristenās email to Dr. Hepworthās listing on our Doctors List. She is his office manager.
