Hi, I’ll put some screen grabs of my CTA below. Report said it was normal of course but they were looking at my arteries, not veins. MRV brain also shows a filling defect at the junction of the sigmoid and transverse sinus on my right side (that is my more symptomatic side and the side that looks more compressed to me). MRI brain shows white matter hyperintensities (not MS) that are worse on the right. Does this compression look significant? Do my styloids look elongated? Is there any way to tell from imaging if there is vagus nerve involvement? My symptoms have been progressive over the last two years and they are:
severe, unrelenting headache (that started as mild and episodic 2 years ago with zero prior history of headache)
Severe brain fog/cognitive dysfunction
Intoxicated/hungover/out of it feeling
Severe fatigue (diagnosed with narcolepsy 1yr after onset of symptoms)
Worsened ME/CFS (was mild, now moderate-severe)
Slower-than-usual resting heart rate
Low blood pressure
Episodic POTS (diagnosed by cardiologist)
Constant PVCs and PACs (thousands per day)
Constant severe pain in right ear, neck, jaw, teeth, lymph nodes etc despite negative workups for any conditions that could be affecting those parts
Neck ‘fullness,’ pressure, and tenderness on right side
Severe head pressure that worsens when lying flat despite negative workup for iih - must sleep elevated
Dizziness
Presyncope
Gastroparesis/loss of appetite
Constipation
Intestinal methanogen overgrowth
MCAS diagnosed 1yr after onset of symptoms
Difficulty swallowing
Frequent hiccups
Tinnitus
Occasional pulsatile tinnitus when lying down despite normal CTA
Phantosmia (smelling smoke, blood, etc)
Constant pain in upper stomach/diaphragm
Worsening endometriosis
Amenorrhea
Severe dry eyes
Extremely pale face
Weight loss and muscle atrophy
Diagnosed with hEDS though likely have always had it. It seems to be worsening though
I have been checked for and treated for almost everything that could be causing the above symptoms and I just keep getting worse. I see Dr Costantino next week for all of this - is there anything in particular that I should ask him or mention to him? What do you see in my images? Could vagus nerve involvement be causing all of the systemic issues I’m having?
This has been so hard on me as I’m sure it has been on all of you as well. I had to move home from a city I loved because I can no longer work or drive. I had to quit my three jobs and extremely active hobbies (bouldering, performing in a band, roller skating, strength training, etc) and am now almost fully housebound except for doctor’s appointments. Has anyone been this bad and fully recovered after surgery? I miss my old life and I want to feel like myself again, but above all else I just want the constant pain and brain fog to stop. I want to at least be somewhat independent again. Is it too optimistic to think that decompression surgery will get me to that point? Thanks in advance.
It looks to me like both your IJVs are compressed significantly. You can see in the first picture you posted how significantly that IJV is squashed, but I’m couldn’t tell if that’s your right or left IJV. I’ve annotated the second picture where both IJVs look pretty flattened.
I can see your styloids in the last movie you posted, but since I can’t pause it, I can’t annotate the image to show you your styloid. That said, it sounds like you know what you’re looking at as both look very long.
I’m glad you have an appt. w/ Dr. Costantino coming up soon. We’ve had members with terribly debilitating symptoms caused by IJV compression who have had decent recoveries from their styloidectomies & IJV decompressions, however, those w/ hEDS often have other compressions below that neck that also need to be dealt with so it’s an ongoing process that can require several additional surgeries.
The other compressions can be Thoracic Outlet Syndrome (TOS), May Thurner Syndrome, Median Arcuate Ligament Syndrome (MALS), & Nutcracker Syndrome (NCS). With your symptoms being as severe as they are, I’m sorry to say I wouldn’t be surprised if you have other things going on besides elongated styloids & stenosis of your IJVs.
Thank you so much for the response! I do suspect other compressions as well based on my systemic symptoms. My pelvis and abdomen are full of scar tissue from endometriosis and other surgeries, so I suspect that scar tissue may be contributing to some compressions. I also have a lot of pain in my right clavicle and shoulder - I’m wondering whether I could have TOS or if it’s just referred pain from my neck. If anyone has recommendations for doctors who can diagnose other compressions in the northeastern or Midwestern US, I’d love to hear them!
Wow, your IJV looks incredibly compressed in the first image you’ve posted! It’s not surprising that you’re feeling so rough…The vagus nerve won’t show on imaging, but as it lies inside the carotid sheath with the IJV and carotid artery, given that severe compression it could well be involved as well. We had a really interesting discussion about the effects of vagus nerve compression in this discussion which you might find helpful: Successful Surgery for severe Vagus Nerve Compression - General / Eagle Syndrome Stories - Living with Eagle @Chrickychricky has posted an interesting link about POTS which might be helpful: The Science Behind the Symptoms is a great podcast. Attaching a link to an episode on POTS - General - Living with Eagle
And @Snapple2020 mentioned Dr Hacker in MO as being very knowledgeable about pelvic compressions I believe… @Snapple2020 is pretty expert about EDS so hopefully she can give you info about doctors if you need it!
Obviously you have alot going on, we’ve had discussions about MCAS which you could search for, & have you been tested for Sjogrens Syndrome? As you mentioned dry eyes?
Thank you so much for all of the information and tips! Looking forward to diving into all of this. It’s interesting that the vagus nerve is within the carotid sheath - I have had 2 ENTs diagnose me with carotidynia (pain and inflammation of the carotid artery) which was the reason for the CTA. Fortunately no dissections or anything with the carotid, but I’m almost certain that I have vagus nerve involvement now that you mention that, because my right carotid hurts all the time. I have read that the right branch of the vagus nerve has more influence on heart rate and also influences bile production which is interesting as I’ve had my gallbladder removed due to issues with that.
I have had the blood test for Sjogren’s which was negative - I know that a cheek biopsy can sometimes diagnose it too, but I don’t have dry mouth so my rheumatologist hasn’t wanted to do one. I do wonder if treating for Sjogren’s could help though - it has gotten to the point where I can no longer wear contacts, have to use prescription eyedrops throughout the day, and have to use eye ointment at night to keep them from drying out:(
@carina - I’ve added an annotation to the sagittal plane image you posted. It’s the internal carotid artery (ICA) which I saw but didn’t think it was that important till you mentioned carotidinia. The way it’s wrapping around your IJV could possibly also be causing IJV compression lower down in certain head positions, & the way it wraps behind the IJV puts it in a possible position to suffer compression itself. I’m glad you don’t have signs of dissection though even irritation can cause blood clots to form. Make sure you bring up your carotidinia diagnosis w/ Dr. Costantino when you see him. I believe that’s a pretty critical piece of the puzzle of what’s causing your symptoms.
Thanks so much for taking the time to do this! I will mention it to him for sure. I do take low-dose aspirin daily as it helps my head pressure, so hopefully that will work as a preventative for clots until I can get this taken care of. Clots are a real fear of mine, but I try to remind myself that if I was going to have one, I probably would have by now - I have a lot of risk factors, have been on meds that greatly increase the risk, and have had major surgeries, but fortunately have never had an issue with them
I’m very glad that you’ve had no clots so far in spite of being high risk for them. The aspirin seems to be doing a good job of keeping you safe so far which is good news! I hope & pray that continues.
I had a negative blood test for SjS too, I have a very dry mouth & eyes, in the end I felt it wasn’t worth having a lip biopsy as I didn’t like the idea of the immunosuppressant meds anyway, & I’d heard the biopsy can take a while to heal, so I manage for now, unless things worsened…but that was 10 years ago so I don’t know if meds have changed…
Sounds like you have plenty to discuss with Dr C, hope the appointment goes well!
I think our symptoms match up pretty good. I saw Dr. Farm and I had an angiogram venogram and lumbar puncture in 2024. I have confirmed total occlusion on the left side and severely stenosis on the right side internal jugular veins. I also have sigmoid sinus compression. Before I saw him in 2023 I was diagnosed with May Thurner which is an iliac vein compression. I got a stent for that in the fall of 2023 and it was a huge improvement for me. I was no longer bedbound. I was 57 at the time when I saw Dr. Fargen. I had surgery with him in August 2024, and when I saw him the next time for a postop follow up to get my stent, I learned he did not decompress me. He did not even remove my style. He just did a C1 shave, and he was as surprise as I was so with that being said, I’m glad you’re seeing Constantino. I hear he’s great. I had already made an appointment in Denver with Dr Hepworth and I’ll be seeing him for surgery in May. I do think that if you follow up and get the recommended surgery for jugular decompression especially if you’re using Constantino I think you’re gonna make a great recovery you do need to think about other compressions though Dr Hepworth suggested I go back and see the Doctor Who diagnosed me with iliac compression and have a repeat CTA to see if I have any other abdominal compressions. I have since ruled those out. I’m currently looking into the compression in my shoulder area of thoracic outlet so having ADS means you’re more likely to have compressions like this. My EDS is myopathic EDS and I learned that from a genetic test that I had I think you’re on the right track and I bet you’re gonna be pleasantly surprised when you recover that you’ll be able to go back to doing the things you enjoy doing. It’s gonna take a while though that’s the hard part and if you have other compressions, you may need to get some of those addressed whichever ones are most symptomatic man. There’s no way we can track it down every little thing but if we figure out what’s given us the most grief in my case, my head and focus on that I think that’ll go along way ways towards getting me totally back to where I was before all of this started in 2022
Thanks for the info and I’m glad the iliac stent helped! Sorry to hear that you’re needing a revision the jugular vein compression, but I’ve heard good things about Hepworth as well! I was between him and Costantino but ultimately chose Dr Costantino since I’ve heard that he resects more of the C1 and soft tissues which I think is what I need. I also like that he does one side at a time and uses a drain. I will definitely be looking into pelvic compressions also. I think I may have vascular TOS as well, as I have a lot of pain under my right clavicle and pressure in my right arm. Glad that you’ve seen some improvement and are continuing to get things taken care of. I appreciate the reassurance and kind words
@Kmoore464 - What’s your surgery date w/ Dr. Hepworth in May? @Jules & I like to put our members’ surgery dates on our calendars so we can pray for them especially on surgery day.
I’m sorry you’re having to do round 2 of decompression surgery. Dr. Hepworth is a great surgeon. I am hopeful you’ll notice a big difference after your surgery.
@Kmoore464 I’m sorry that you’re needing revision surgery as well as all the other vascular compressions you have, I hope that this surgery is more helpful, like @Isaiah_40_31 says, let us know the date
Sounds like we are all the same boat! Im glad to hear @Kmoore your stenting helped for MTS. Ill be looking at doing the same and get a game plan to deal with Nutcrackers and get more definitive answer on the MALS. Im waiting on vascular surgeon review all my scans and records. The radiologist report says I don’t meet criteria for MALS which is 70% stenosis and nothing more. Thanks to Isaiah, I took a closer look at the velocities and from what I can gather as a lay person, I have about 50% occlusion. Thats 50% more than I want to have.
Im starting at the neck and working my way down. Dr. H office suggest I may have about 25% improvement with each decompression?
Thats sucks you need to go in for revision. I am doing the same because first ES surgeon did not look at all that should have. @Carina have you considered MCAS as a possible source of your dry eyes? I dont know how old you are but a recent podcast I saw of a MCAS expert (Dr. Afrin) suggested hormone replacement may help with those type of things? I have a good friend struggling with dry eyes and she has tried about every treatment known to man and spent a huge amount of money out of pocket on treatment, none of which have helped her much. She is miserable.
You really did have alot of symptoms that could be associated with vascular compressions @carina elsewhere in your body. I had a laproscopy about year ago to try and determine my serve pain and the surgeon found adhesions everywhere, some of which he released and also a spigalian hernia which was repaired but was not the source of my left sided flank pain. As it turns out, that pain is likely Nutcracker, another vascular compression.
I dont think I deserve the compliment that I am an expert EDS person as what I have learned in the past few months has really taught me how much I don’t know. Especially about the vascular compressions. I always thought that since I dint have vascular EDS, I didnt have to worry about these type of issues but they are now finding that is not the case with hEDS. I think there is finally some acceleration in data coming out and some new frontiers finally opening up.
There is a free EDS research Symposium that is free to attend online I just found and Im sure it will be enlightening: you need to register to get the links
Looks like a great source of EDS information, @Snapple2020. Thank you for the link! Again, I’m sorry for what you may have ahead of you but am hopeful that each surgery is a step closer to you feeling great again!
I didn’t think about MCAS causing dry eyes - I do take 3 meds for it (I’ve tried four) that help with a lot of the symptoms, but the dry eyes are still relentless. I haven’t tried hormone replacement therapy though - my hormones are so out of whack, so that probably would help. I’m not sure what type of specialist to talk to about that though - I recently moved, so I no longer have access to the MCAS specialist who diagnosed me, and I get my meds filled by a functional medicine doctor. I can try asking my gyn/endometriosis specialist when I see her. I’ve seen two endocrinologists and they were not helpful:(
I’ve had two laparoscopies over the years to remove adhesions and fluid from my pelvis, but they’ve never mentioned anything about vascular compressions. I looked back at some of my imaging, and it looks to me like I may have MALS (though I’m obviously not an expert), and I certainly have a lot of symptoms of MALS and SMAS. I’ll have to ask some of my specialists if they know anyone who can help with that.
I think your approach of working from the neck down is a good one - I will probably do the same! I already have a vascular specialist in mind who can evaluate me for TOS, then I’ll go after treatment for MALS if I have it. Once I get my endometriosis more under control then I will look into pelvic compressions. Thanks for all of the info and sharing the symposium - I’ll check it out!
Ask your functional med doctor about HRT. I didn’t even know I needed it until I started seeing a functional med doctor. Hormone replacement changed my life in a very positive way. Your OBGYN would also be a possibility. You want to try to get bio-identical hormones that are compounded just for what your body needs not something that only comes in particular dosages. Getting hormones specifically compounded is more expensive but soooo worth it.
