Buzz. My case history, findings and radiology images

Buzz · May 8, 2023, 7:03am

Hey everyone, it’s Buzz here.

I will aim to update this post with my case history, radiology images and videos, test results, findings, and timeline as I progress through my ES journey.

First off, I wanted to say hi and introduce myself.

I haven’t read all the posts on this forum yet, but I’ve been reading as much as possible over the past few months, absorbing all of the knowledge and advice that I can.

The people here are amazing - every question I’ve thought of, has been answered in another post. It’s been a long learning process, and I feel much more confident in my understanding of ES.

I’m so grateful for this community - I believe that it’s already saved me a ton of time, effort, and pain. I hope my own contributions can serve as thanks for this incredible resource.

It’s a pleasure to meet you all. Even though we haven’t met in person, I feel invested in your stories from reading through your years of suffering and success.

Reducing suffering is a great goal, and a community that helps each other to do that is a beautiful thing. I appreciate you all.

My own journey has been long and painful - decades of chronic symptoms. I’m a 40 yr old guy living in Sydney, Australia. I have a wonderful wife and family who have supported me through all of this beyond anything that I could have hoped for. I am truly blessed in that regard. Healthwise, life has been a struggle for decades. At this point I am debilitated and disabled to a significant degree. Thankfully, earlier this year, I made significant discoveries in my CT scans relating to abnormal TSPs, IJV stenosis and possible ES as the cause of my symptoms. I’ve gone from my lowest point in my life to feeling truly hopeful.

I’m getting a referral to see Dr Elliott here in Sydney, and it will probably be a few months until my first appointment with him.

I’ll start posting my case history, findings and radiology images below, and joining in the other discussions. I hope my story can one day become one of the many successes on this forum.

I have so many different radiology images from different scans, it’s hard to know what to upload.

I’ll start with a CT slice of both of my TSPs:

Here is my left IJV:

Here is a video that I created using the software Radiant, it includes 3D reconstructions of my CT scans:
https://www.youtube.com/embed/S3WEDUuq1Cc?vq=hd1080

Please feel free to share any comments or insights that you have about these.

I’ll upload more later.

Thank you!

vdm · May 8, 2023, 10:45am

G’day!

Elliott is great, I saw him in 2018. Had the nasal endoscopy and could see everything on a huge display, also got detailed explanation on every single chamber in my nose. He’s really nice, and knows his stuff. Back then he’d do only intraoral procedures and only shorten the bone, but now I’ve heard he goes externally and gets it all the way up to the melon.

Btw your IJV seems to be a bit compressed below the styloid too, perhaps by the ICA?

Jules · May 8, 2023, 2:01pm

Hi Buzz & welcome to the forum! So pleased that you’ve found the site helpful, it has been great for me & I doubt I’d have found the surgeon who did both my surgeries if it hadn’t been for the info here! I’m glad too that you have good support from your family
Those styloids look very long & pointy, not surprising you have symptoms, alongside the IJV compression…
I hope that you get on okay with Dr Elliott, & that he can help you. We’ve not had many members from Australia who’ve managed to get surgery, it seems to be really difficult…

Isaiah_40_31 · May 8, 2023, 10:41pm

@Buzz - I’m glad you’ve taken time to read on the forum. It is very helpful! Even though I think I’ve read all the posts over the years, I still run across info I’ve forgotten that comes in handy in the moment.

LOVE the video you made! What a great graphic! To me, it looks like your right SP is normal length but the stylohyoid ligament is calcified below it & a bit thick. The left is thick at the top & long. It looked to me like C-1 is playing a significant role in the IJV compression on the left & that may be a function of the asymmetry of your C-1.

I hope Dr. Elliott is supremely helpful & that you’re able to see him in less than a month. I don’t know if they keep cancellation lists in AU, but it’s always worth asking. Getting your name added to one can sometimes expedite your appt. date.

Leah · May 9, 2023, 1:52am

@Buzz I am new to this forum as well, a couple of weeks I think at this point.

And like you have found a wealth of information, after suffering for many years…like so many on this group. I am so sorry for your suffering as well.

I also feel hopeful as this is an answer to a giant puzzle that has been going on for years and years.

I read your information and had a view of your video, and oh my word… You need to go to the front of the line if that was possible!

It is evening here, and my brain gets foggy as the night goes on… The only thing that comes to mind (so far) that you may or may not have tried…but several folks had recommended is taking a blood thinner like Plavix. I am going to ask about that tomorrow myself. Believe it helped others with pain and intercranial pressure.

If other odds and ends fall out of my crowed brain, I will send it over to you.

I also have C1 Involvement… btw and there appear to be a good number of us here.

All the best )))

Buzz · May 9, 2023, 3:03am

@Isaiah_40_31 Thank you! I’ve enjoyed learning all of the features of the Radiant software. It’s amazing what it can do, and how much detail it can reveal by playing with the settings.

Yes, I am curious to find out if my segmented right TSP is a large calcified ligament or a fractured TSP. I suffered a facial / jaw injury, with teeth knocked out, just before my symptoms began at age 5.

I agree. I noticed that my left IJV looks stretched tight like a bowstring over my Transverse Process of C1. Here are some more 3D images from my CT:

There might be other factors that I’m not considering, but it does seem evident that the TP of my C1 is playing a pretty major role in my IJV compression?

The more I’m thinking about it, the more I feel like I might need to have my TPC1 resected along with the styloidectomy.

Thank you! At the very least, I’m hoping that he can arrange all of the necessary testing that I need to confirm the diagnosis.

If I do end up needing the C1 resected, then it seems probable that I’ll need to consider travelling internationally? Dr Costantino has been mentioned recently. He seems like a good option for TPC1 resection along with the styloidectomy. His practice of having a neurosurgeon present to complete the C1 resection seems smart.

Buzz · May 9, 2023, 3:09am

@vdm G’day!

Yes, I’ve heard nothing but good things about him. I’m excited for the appointment.

Are you talking about the left or right IJV? Did you spot that in the video or the images?

Thank you!

Buzz · May 9, 2023, 3:21am

@Jules Thank you! Yes, I owe them my life in so many ways. I can’t wait to get surgery so that I can hopefully stop being a burden to them and start enjoying life again.

Thanks. Yes, I’m ready to do whatever it takes to get the best possible outcome, even if I have to travel across the world to make it happen.

Buzz · May 9, 2023, 3:29am

Thank you @Leah !

Yes, based on the recommendations of this forum, I got a prescription for clopidogrel + aspirin (the brand name is Duocover in Australia), and also one for Olmesartan due to my hypertension. Fingers crossed that it helps

vdm · May 9, 2023, 3:32am

This one, the brighter one I suppose is the ICA, pushing on the IJV?

Buzz · May 9, 2023, 4:24am

@vdm I think I can see that spot in the 3d view of the left IJV isolated:

Here I have uploaded a video with the sagittal, axial and coronal views of my contrast CT scan:

It looks like it is the ICA that you are referring to.

vdm · May 9, 2023, 4:32am

I’ve heard on the grapevine that Dr Hepworth (Denver, CO) does some “untangling” sometimes, whatever that means, to make the jugulars free.

KoolDude · May 9, 2023, 3:51pm

Hi Buzz, first good job , you have done a great job in both rendering and learning on how to read these images. You pretty much know now what has been causing you all these sufferings. This is a road all too familiar with us so welcome to the Eagle family.

I am a bit like you in terms of anatomy (just opposite). I have dominant left Jugular Vein, you have right dominant jugular vein. My right is small by birth and your left is small by birth. Both IJVs are compressed in your case by C1 & Styloid (mine as well by C1) and I agree that removing styloid alone might not be enough although some got relief from bilateral styloidectomy despite C1 involvement. Your need to focus on your dominant right IJV for decompression first (removal of Stytloid and C1 trimming). Not a doctor but based on experience on numerous folks with longitudinal luminal defect on their IJV (vertical narrowing), your left IJV could potentially need stenting after the removal of the bony compression as it seems severely narrowed.

Just so you know, I went through Styloidectomy on the dominant left IJV but it is still collapsed by the C1. I think, in my case, it was the C1 that was pushing the IJV towards the Styloid area mimicking styloid compression only. So now I am shopping around for someone who can competently remove the C1 without any complications.

What are your current symptoms? Wish you best of luck with your search of surgeons.

Isaiah_40_31 · May 9, 2023, 11:38pm

@Buzz - Nice thorough replies to everyone! I don’t even know how to do what you did w/ this software & I’ve been here for 8+ years (that said, I don’t have a techie bone in my body )!!

I agree that it appears something will need to change w/ the left TP of C-1. It may not need total resection but just a good shave. Since your right is dominant, it may be worthwhile taking care of that one first unless your symptoms are dominantly left-sided.

Buzz · May 14, 2023, 1:58pm

@KoolDude Thank you so much! I’ve enjoyed learning the software. I hope that the images and video help in the appointments.

I’ve also purchased a resin 3D printer to print a model of my TSPs and IJVs from my cervical CT scan. I hope that the surgeon might find it useful.

I was overwhelmed by this comment. I really, really hope that’s true!

I would love to know more about why you say this. Do ENTs usually recommend operating on the less-affected IJV side first?

What makes you say that, as opposed to the narrowing being fully caused by the TSP/C1 compression?

I think I understand what you’re saying. Do you mean that the narrowing in the IJV continues beyond the compression site?

Here is a close-up of my left IJV:

I’m very sorry to hear that. I would also like to avoid that happening. Currently the combined surgery of Dr Costantino + his neurosurgeon seems a promising option.

I also wanted to say that thank you for your previous posts on this forum. I’ve bookmarked many of them as they’ve been very helpful learning about ES.

Buzz · May 14, 2023, 2:23pm

@Isaiah_40_31 Thank you!

That’s interesting! @KoolDude said the same thing. I’m very interested why you would recommend treating the side with the less severe compression first?

My symptoms are dominantly left-sided.

The shortness of breath and the intermittent pain in the left side of the neck are the two most severe symptoms.

Here is the full list:

My symptoms

Chronic

Vocal hoarseness, fatigue and tightness.
Shortness of breath, air hunger, breathing difficulty, airway sensitivity, irritation, dry cough, throat muscle tightness.
Headache, pressure sensation in head.
Insomnia, poor quality sleep.
Brain fog, poor memory.
Dizziness, lightheadedness, unsteadiness.
Feeling sick, fatigued, exhausted, malaise.
Congested nose and sinuses.
Tinnitus, sound hypersensitivity.
Eyes dry, sore, burning, fatigued.
Neck clicking, snapping when moving head.
Muscle pain and tightness in neck, head, jaw, shoulders, back, legs.
Reflux, nausea, constipation, diarrhoea.
Difficulty swallowing, aspiration.
Nocturnal bruxism.

Intermittent

Pain / burning / tingling sensations in my neck, throat, jaw, palate, sinuses, base of skull, left ear, shoulder, chest. Left side mainly. Lasts for hours. Unresponsive to analgesics.
Headache, pulsatile cranial pressure sensation, brain fog.
Shortness of breath, upper airway burning and irritation. Coughing.
Tachycardia, palpitations.
Faintness, unsteadiness, presyncope.
Dry eyes.

Triggers

Looking downwards, and to the left. Moving head laterally to the right.
Opening jaw wide, yawning, laughing, talking, swallowing.
Exertion.
Raised heart rate / blood pressure.
Lying horizontal.

Mitigation

Stretching my neck up and to the right.
Sleeping with head elevated and tilted to the right side.
Applying ice packs to the left side of my neck and head.

History

Symptoms began at age 5 after a face / jaw injury resulting in dental avulsion.
Worsening rapidly in the last decade.
Chronic symptoms now unbearable. Quality of life critically low. Limiting every aspect of life. Sleep is very bad. Mild exertion is unbearable. The only speaking I’ve done since February has been in doctors appointments, etc, and I suffer afterwards for many hours. I type and use sign language with my family. It’s miserable.

KoolDude · May 14, 2023, 4:35pm

It is all about the amount of blood that they drain which is proportional to their sizes. Your right IJV is dominant because it is bigger in diameter and as result drains more than the left IJV does. So if we assume (not scientific but to make the point) that your right drains 65% - 70% of your brain fluid (CSF + blood) and your left IJV drains 30% - 35%, then it is clear which pipe (IJV) you want to open up for optimal drainage to occur. It is also true in most cases, that the smaller IJV connects to smaller Sigmoid-Transverse Sinus once it enters the skull (Red arrow points where it enters the skull and joins to the Sigmoid Sinus). So the net-flow from the brain to the left IJV is minimal and that the narrowing is not confined to the IJV and compression area only but it is an overall narrowing by birth in most of the cases. So in your case, you will benefit more by opening up your dominant IJV first compared to opening the smaller left IJV ( You can do this after the dominant one). So in short, to get the most out of your first surgery, I would proceed with right IJV decompression (C1 atlas shaving & Styloid removal). This should, hopefully, resolve most of your symptoms except the left sided ones which I think are caused by that elongated styloid on the left but not outflow restriction since the amount it drains is relatively smaller compared to the right IJV.

Yes, you got it. I am not a doctor but based on all the research I read, your left IJV appears to have longitudinal luminal defect (meaning vertical narrowing - Area is pointed by Cyan Bars) and even if the compression was removed, that segment appears to be fibrotic (Read somewhere that due to a lengthy compression, veins lose their elasticity & become fibrotic) and could potentially need further intervention such as stenting.

I also noticed that your dominant right IJV has somewhat milder compression from sternocleidomastoid muscle (SCM) near the base of the neck ( Second image - Cyan Arrow - > SCM & Blue Arrow → right IJV). This one is minimal compared to the C1 & Styloid compression on it.

As far as your symptoms are concerned, I would say I have 90% of them. The only difference is your left sided ones because I do not have that elongated Styloid on your left side which could potential be messing with nerves such as Vagus nerve among other ones causing neck pain and dysautonomia (shortness of breath).

For mitigation, I might add to your list the following which I find helpful

MAGNESIUM L-THREONATE is special magnesium which helps with sleep and cognitive function (Research based benefits - crosses brain-blood barrier and enhances cognitive function and sleep)
Soft Collar Neck Brace to help keep your neck straight.
Stay away from high doses of Vitaman A (only if you take Multi-Vitamins). It is linked to aid rapid CSF production and since our drainage is impaired, it adds to the load in the brain.

I did talk about what help me here (I'm back - surgery with Dr Axon - #24 by KoolDude)

InkedAx Right IJV and SCM compression

KoolDude · May 14, 2023, 5:05pm

Typically, if you look at the 2 IJVs, it is obvious that the left one is smaller in diameter all the way from the brain to where it connects to brachiocephalic veins in the chest regardless of the compressed area. So it does appear to be by birth rather than induced one (I could be wrong but that is my case too so I assume yours to be similar)

Isaiah_40_31 · May 15, 2023, 1:39am

@Buzz,

Your symptoms are a mixture of those from cranial nerve irritation/damage & vascular compression. Knowing the functions of the 7 cranial nerves that can possibly be impacted by elongated styloids helps to demystify ES symptoms.

Based on the symptoms you’ve listed, I agree w/ @KoolDude that the vagus nerve is for sure one (digestive issues-reflux, nausea/diarrhea/constipation, cough, hoarseness/pain when talking, shortness of breath, etc., swallowing, palpitations/tachycardia, increase of symptoms from laughing, talking, swallowing, fatigue, burning sensation in chest); next I would implicate your facial nerve (dry eyes, etc. & hyperacusis), trigeminal nerve (congestion/sinus issues, tinnitus, muscle pain in jaw/face, ear pain), glossopharyngeal nerve (tightness in throat, difficulty swallowing/aspiration); spinal accessory nerve (pain & tightness in neck, shoulders, upper back - which can translate down the spine & possibly cause leg tightness/pain). Skull base pain is common but I’m not absolutely sure which nerve causes it - maybe vagus.

If I haven’t mentioned it, there’s a great series of YouTube videos called Two Minute Neuroscience w/ a video for each of the 12 cranial nerves. Watch the videos for - Facial, Trigeminal, Glossopharyngeal, Hypoglossal, Accessory, Vagus & Vestibulocochlear nerves.

Buzz · May 15, 2023, 10:45am

@KoolDude Thank you! I feel privileged to receive your detailed responses. That’s such useful information.

Do you know of a way to tell if an IJV is fibrotic in the CT or MRI images?

If the surgeon tests my IJV at the end of the surgery and it remains constricted, might they try to stent it immediately, or wait and see if it improves afterwards?

Yes, I suspect that might be the case. Especially since the pain often starts suddenly when I look down or to the left.

Your suggestion about operating on the dominant IJV side first has given me a lot to think about.

On the one hand, the IJV-compression-like symptoms are debilitating. But I believe that I could survive enduring an additional 6 months of brain fog, fatigue, terrible sleep, etc. On the other hand, the vagal-compression-like symptoms are unbearable. The suffocation sensation and pain episodes in my neck has become so severe I’m not sure that I could handle delaying it. I might be compelled to choose the side first that is most likely to reduce those two symptoms. I’ve got a lot more learning to do on the topic.

Is it possible that due to decades of compression, the whole IJV might have shrunken from lack of use? Once the compression is relieved, and the blood flow increased, could the circumference of the whole IJV increase over time?

Yes, I see what you mean. Thank you for the diagram! I will keep an eye on that section in the post-surgery scan. It might be important if that section remains compressed after the rest of the IJV is decompressed.