I’ve been dealing with daily headaches and head pressure for the past year, and was diagnosed with the jugular form of ES. Both of my jugular veins are severely compressed between the styloid and C1. An angiogram with manometry showed a pressure gradient above/below the styloid on each side (over 5mm on the worst side), as well as an elevated saggital sinus pressure in the mid-20’s.
I was planning to have a styloidectomy done, but now my neurologist and interventional radiologist are trying to convince me to instead consider an additional angiogram with manometry to measure pressures in the transverse sinus (which they didn’t do the first time), and place a stent if there is a pressure gradient >8mm. They seem convinced the compression by the styloid and C1 can’t be causing so much pressure in the saggital sinus, leading them to suspect a narrowing higher up, likely in the transverse sinus.
The research I’ve done suggests stenting the transverse sinus before styloidectomy is not the right approach. The jugular compression can actually elevate ICP in the skull and cause the walls of the transverse sinus to narrow — but it’s extrinsic stenosis (versus intrinsic, disease of the vein itself), which can be reversed by performing jugular decompression. By placing a stent, assuming there is stenosis, we would be treating the effect, rather than the cause.
I discussed my research with the interventional radiologist who did validate that most stenosis they treat with stenting is extrinsic, but patients tend to have good outcomes. He suggested styloidectomies were not very effective in the majority of cases, which as we all know is not the case according to published case studies and the experiences of patients on this forum.
As part of my diligence, I wanted to check to see if anyone here had transverse sinus stenosis that resolved on its own (without needing a stent) after jugular decompression. I’m also curious if anyone else has been pushed in the direction of stenting rather than styloidectomy. In my case, I think it comes down to the doctors not having a full appreciation for the jugular ES condition and the success rates of jugular decompression with the right surgeons. They seem to be pushing me down a path they know, but unfortunately it’s probably not the right solution for me and others with jugular compression. Not only does it fundamentally seem to be the wrong solution, stents are permanent and it is common for patients to need multiple due to re-stenosis. I imagine a scenario where the root cause (jugular compression) never gets fixed and a patient needing stent after stent as veins continue to stenose. This seems like a vicious cycle.
Hope everyone is having an above average day symptom wise. If not, there’s always tomorrow!
This is a very insightful post, @blue. We would agree with every premise you made about stents & about having a stent placed w/o treating the cause of compression first to see what outcome that produces especially in the sigmoid or transverse sinuses.
You are very wise to have done your homework prior to jumping into surgery. You are correct that your IR & neurologist aren’t well informed about the success that ES surgeries, especially those w/ IJV decompression, can produce when done by an experienced surgeon.
@blue Have you received a head and neck CT with contrast? This would allow us to determine all structures contributing to compressions in the entirety of the neck. Additionally, if elongated styloids are present, they should be examined in the context of their spacing from their carotids as well, particularly the ICAs. This can be missed in imaging other than CT because a styloid can be in contact with or in close proximity to a carotid and not causing compression (so it wouldn’t be flagged in ultrasound or angiogram). If vascular compression is present, and a stlyoid(s) is a suspected culprit, a head and neck CT with contrast is the absolute gold standard.
If you have happened to receive a head and neck CT with contrast, I would be happy to take a look.
Also, stents are less ideal if an IJV is being compressed by a styloid (and likely between a styloid and C1), because you can try to open a vein as much as you want, but it can only be so big when it is wedge between bony structures. I highly recommend IJV decompression over a stent in the context of ES. A stent can be helpful if an IJV is just naturally small in diameter, but not when there is compression by structures.
To clarify, the stenting they are proposing is higher up in the transverse sinus rather than in the IJV itself. To your point, that would be a bad idea due to the bony structures pinching the IJV. They agreed, also adding it’s a straight shot the heart from the IJV should the stent become dislodged.
I do have that imaging and would appreciate you taking a peek! Once I offload it from the CD, I’ll get it over to you. Thank you very much for offering.
Great!! You can put all the CT files into a folder on google drive then send me a link to the folder. You’ll no longer be anonymous to me but I’ll keep all your personal information private!
This is something that weighs on my mind quite a bit as I await surgery. I’ve been told stenosis is possible intracranially, mainly in one of my transverse/sigmoid sinus. Same info with gradient of 8 needing stenting.
I haven’t had any angiogram yet but was offered one by Dr. Kenneth Liu in 2022 when he diagnosed me with EDS and possible occult tethered cord. At the time I did not know I had Eagles Syndrome. While looking for surgeons for OTC locally, I researched EDS and found I also had Eagles. To me, it seems to make sense to treat the stenosis due to Eagles before trying to tackle anything intracranial due to the changes that can occur.
Here is a lecture by Dr. Liu on venous stenting…
This is something I hope to talk to Dr. Hepworth about at the end of the week, when I have my long awaited appointment.
@BirdsOfSore - Thank you for the link to the video. I’m so glad your appt w/ Dr. Hepworth is coming up soon. Please go prepared w/ a written list of questions you want to ask him so you don’t forget anything. A couple of members have recently mentioned their initial consults were a bit short so be your own best advocate if things seem rushed & press to have your questions answered as you have paid for your time with him.
Hi Blue, I suggest you watch the below webinar especially from 9:25 minutes. Michael Elliott ENT surgeon in Australia will explain why your interventional radiologist wants to do an additional angiogram with manometry to measure pressures in the transverse sinus. He explains, like your radiologist, that compression of the internal jugular veins by the styloid and C1 can’t cause increased pressure in the sagittal sinus as compensatory collateral venous drainage at the C1 level relieves most of the extra venous pressure. This is why your interventional radiologist wants to look for an intracranial cause; ie the transverse sinus.
As far as what procedure should be done first, Dr Nick Higgins neurointerventional radiologist UK (the inventor of cerebral stents) advises that for people who suffer from headaches and who don’t have tinnitus or papilledema then extracranial internal jugular decompression surgery is best done first and then if the headaches are not completely resolved, then placing a stent in the transverse sinus if there is a stenosis there. However, in these headache cases without papilledema, if they are diagnosed with an important pressure gradient in the transverse sinus, then he prefers to place a stent in the transverse sinus first before performing the extracranial decompression surgery of the internal jugular vein(s). See the below webinar from 1:10:44.
It’s a difficult decision for you, I don’t know much about the intracranial compressions…my gut feeling would be that there are risks with the angiogram itself, and the stenting, and maybe the styloidectomy is perhaps the least risky option, so would it be best to try that first & see what resolves? Especially if you have other symptoms of nerve pain, neck pain etc., but even with just the IJV compression it would seem perhaps that you’ll need this surgery at some point anyway, so maybe try it first? But there are some members who have (initially anyway, we don’t always know long term) not always done as well as we would expect post surgery with the compression removed, and that they maybe have other intracranial issues going on… then as I say, that’s without knowing about stenosis in the transverse or saggital sinus, it sounds like @BirdsOfSore has given you good info & an interesting link to watch…I think you’re being very wise and thorough researching this as much as you can…
@Emerald - Thank you, too, for sharing links with valuable & helpful information for @blue. It sounds like you’ve done a lot of solid research for yourself, too. Good work!!
Thank you, @Emerald for sharing both of these videos. In the second one with Dr. Higgins, he mentions that he would prioritize jugular decompression and only place a stent in the venous sinus if the patient’s headache symptoms did not improve. My impression is Dr. Hepworth disagreed, favoring the opposite approach, but I’m not sure I completely understood his response. @BirdsOfSore I’d really appreciate you sharing Dr. Hepworth’s perspective after your consult.
Sorry I’m a bit late but here is a summary of my visit with Dr. Hepworth.
Assessment and Plan
The following list includes any diagnoses that were discussed at your visit.
Assessment Note
Tyler’s Health Issues and Treatments
Edward and Tyler reviewed a CT scan of Tyler’s head, focusing on his vein anatomy and potential
issues. They discussed how Tyler’s left jugular vein appears smaller and more constricted than the
right, which Edward suggested could be contributing to cognitive issues and headaches. Edward also
pointed out a fluid collection in Tyler’s left nasal cavity, which he suspected could be due to a skull
fracture. They agreed to further investigate this finding.
CSF Leak and Venous Treatment
Edward explained to Tyler that his CT scan showed an old skull fracture from a 2012 concussion and
suggested the presence of a CSF leak in the roof of his nose, which could be treated as a sinus
infection for insurance purposes. Edward proposed addressing both the leak and the venous
insufficiency simultaneously to provide symptom stability, acknowledging the complexity of the issue.
Jugular Vein Stenosis Treatment Plan
Edward and Tyler discussed the results of a Doppler study showing irregular flow velocity in Tyler’s
left jugular vein, likely due to stenosis caused by the styloid and surrounding lymph nodes. Edward
suggested that Eagle syndrome surgery, specifically a styloidectomy, could help address the issue.
They also briefly touched on the possibility of checking for Lyme disease, though Tyler reported
negative tests from a few years ago. Edward proposed an action plan involving fixing the left jugular
vein through surgery and ensuring no distorted anatomy or inflammation is present.
Nasal Roof Leak Surgery Discussion
Edward explained the surgical procedure for fixing a leak in the roof of the nose, which involves
examining the bony defect and making a judgment call on whether to reconstruct the roof aggressively.
He mentioned that while some doctors may be reluctant to fix leaks that are not obviously singular
cause broken, he performs this operation 6-8 times a week with a success rate of less than 1% for
nerve injuries. Edward also discussed alternative treatments using blood thinners, diuretics, and
platelet inhibitors, but noted that these treatments can sometimes make leak symptoms worse.
Understanding Tyler’s Medical Condition
Edward discussed Tyler’s medical condition, explaining that symptoms of Eagle syndrome and CSF
leak have been present since at least 2013. He described how jugular vein compression and histamine
production can lead to various health issues over time. Edward advised against intracranial stenting
before surgery and explained that CT myelograms are not effective for detecting CSF leaks. He
suggested using MRI with prolonged T2 sequence to visualize the leak and sinus disease. Edward also
mentioned the possibility of EDS being a contributing factor to Tyler’s condition, but emphasized that
it does not condemn him to a lifetime of symptoms.
Recommendations
-CTV to assess intracranial and extracranial venous outflow and evaluate pressure gradients.
-MRI Cisternogram with <1 mm slice thickness to correlate with prior CT findings and evaluate for
possible CSF leak
-pursue sinus surgery as sinus disease was noted on all imaging + include CSF leak repair + left
jugular vein decompression
Follow-Up
Follow up via telehealth or in person with Dr. Hepworth.
Based on current findings, anticipate that surgical intervention may be recommended to address both
vascular and neurogenic components of compression.
Clinical Considerations / Impression
-Persistent left ethmoid sinus fluid pooling observed on all imaging; lack of response to antibiotics
suggests this is unlikely infectious.
-Findings suggest a transverse fracture of the posterior ethmoid roof (left)
-If the defect were to resolve with conservative or pharmacologic management, it would typically have
done so by now.
-suspect a CSF leak occurring concurrently with venous outflow obstruction.
-excess histamine contributing to the patient’s symptom cascade.
-POTS is likely secondary to impaired venous outflow due to jugular vein compression.
-history of pseudotethered spinal cord noted in consultation with Dr. Pedrefina.
***Intracranial venous stenting is not recommended at this time; venous outflow obstruction should be
addressed first.
