A little about my story

Hey everyone! Really glad that they have this website set up, as this is one of the weirdest diseases out there! I’m 32 years old with two little kids so this whole thing has been very difficult.

My story starts 3 years ago when I was trying to go to bed one night and my body just had a burning jolting feeling down it. I felt like I was struggling to breathe, had real bad chest pain, some real funny feelings in my throat and neck, and felt like my body was just on fire. Had no idea what was going on. This happened three additional nights in a row and I ended up in the ER. Of course they didn’t find anything but I started seeing specialists from there. Went to a good 30 doctors before looking into a CSF leak.

My symptoms progressed to where I had real bad headaches went upright especially, neck pain, heart palpitations, difficulty breathing, choking sensation in my neck, and digestive issues. I met with the doctors at Duke University about a CSF leak. They found multiple CSF Venus fistulas which drain my CSF through veins down my spine. Those are obviously not supposed to be there. Typically those are because of IIH and ultimately they determined I must be in high pressure, not low pressure like I initially thought.

They did a CTV and realized that both of my jugular veins were extremely compressed at my C1 level. Left side was 80% compressed, all my right side was around 60% compressed. I went to see Dr fragen here in North Carolina and got my pressure testing done. They found high pressure gradients in my left side which they immediately deemed was the cause of my symptoms. Also it was noted that I have around 4 cm long styloids. Not super long by any means, but long enough to cause weird symptoms of course.

After that, I went out and saw Dr Hepworth out in Colorado to discuss getting a styloidectomy done. Meeting with him went great and really learned a lot from him. The only thing I was really frustrated about was it would take 7 months to get me in for surgery. I was in a lot of pain and couldn’t wait that time.

Dr Fargen had been training with him for some time and said he was going to begin doing the surgeries. I volunteered to be the first in order to get on the schedule earlier than I would have otherwise. I went in for surgery on October 12th of this year. I am currently about 7 weeks out post op. He was able to shave down my styloid pretty close to the skull base, but he did leave a little piece, about 1.2 cm, of styloid in my neck because he couldn’t get to it. Really hoping that’s not going to cause problems in the future.

Since my surgery, I have had worse headaches than before. I had around six out of 10 headaches normally, and now my headaches are around 9 out of 10. A few days after my surgery I went back to meet with Dr Fargen and got another CTV done to see why my headaches might be worse. Now my left jugular vein is 100% closed up starting around my C1 joint. I’m assuming that the swelling has caused things to push up on my jugular vein and what not. I’m going again in mid-December for another CTV.

Another thing that happened is my accessory nerve was stretched out during the surgery for much longer since it was his first time. I left shoulder now is in pain and I have difficulties raising it past my head. My left arm is drooping really bad, and my shoulder blade “winging” and not functioning properly. Hoping that time will heal it up just fine. I will be doing physical therapy here shortly as well and hoping that’ll help.

Overall, my symptoms currently are headaches on the left side mainly, and a little bit on the right, next stiffness, something in my throat feeling, first bite syndrome, ear pain, and sometimes get some throat pain. Really has me down because of how many people experience immediate relief of their symptoms after their surgery. I definitely did not have that relief. Hoping it’ll come with time.

Next steps with me or I might look to do a provision surgery with Dr Hepworth if things don’t heal up. I feel like Dr Fargen did a great job in the surgery, but I think he was so focused on the styloid that he might have missed other things that could be causing the compression inside my neck. He also did not do the ballooning that a lot of doctors will do after the surgery to open up the vein. It has me worried and I want to make sure that it was done correctly. Thinking I might have just should have waited until Hepworth was available. I’m still feeling hopeful that things will heal up but only time will tell.

Would be curious to know if anybody else has had similar symptoms to me. Glad to have found you all here and hoping for a better life for everyone on this page! This has been the longest 3 years of my life and I just really want my life back like most of you do! Also would love to know everyone’s success stories with jugular vein compression. TIA!

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First I really sympathize with you and hope that your headache and pressure subsides with time. Sometimes experience is what makes the difference in surgeries so since Dr. Fargen was new to styloidectomy procedure, it is likely that he avoided taking too much of the styloid for the fear of facial nerve damage which is close to where the Styloid originates. It is also well documented that swelling will collapse the weak wall of the IJV for at least 2 to 3 months at times. It can sometimes occlude all together after neck dissection surgeries as the study below has found but the good news is, once the swelling subsides, the IJV does spring back in around 3 months timeframe.

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The study Link : (PDF) Internal jugular vein patency after lateral neck dissection: A prospective study

As far as the Spinal accessory nerve stretching is concerned, it is also known to improve or resolve altogether after few months as another study has found. In the study below, 7 patient has spinal accessory dysfunction after rescue styloidectomy, 4 have the symptoms resolved and 3 improved ( see the study below)

All patients with shoulder weakness/accessory nerve compression derived benefit from the procedure, this sometimes apparent immediately postoperatively, at other times emerging gradually over weeks or months (Table 3). In cases where styloidectomy was performed for stent compromise (Table 4), further endovascular procedures (angioplasty) were usually undertaken to attempt to re-establish a normal stent configuration or adequate vascular lumen, with limited success. These procedures are ongoing.

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Source Study : https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5357221/

I hope this gives you a piece of mind while you wait for an improvement of your symptoms. I am 4 weeks and few days from Styoidectomy surgery on my left side and I still have all the symptoms prior to my surgery (IIH symptoms; heachache, tinnittus, facial pressure…etc). Even sometimes more frequent than before surgery so I am hanging in here waiting things to improve in the coming weeks.

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@KoolDude those two patients with occluded IJV gave me hope!!

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Just want to clarify something here. This study shows that compressed spinal accessory nerve can recover after the Styloid was removed. So theoretically, the nerve can also recover from stretching as well is what I meant here.

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This is extremely helpful! Thank you very much. Can I ask you to help me understand the table for IJV compression? Not sure I understood that part fully. Is it saying that only a low amount of people have full occlusion?

As for the accessory nerve stuff, that makes perfect sense to me on how it can heal. I am really hopeful that is will heal up in due time.

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@Millcf - I just have to say, you’re one brave person! Allowing a surgeon to do his first styloidectomy on you.

I have read about Dr. Fargen’s reputation on here & am thrilled he’s venturing into the ES surgical arena. Thank you for your fearlessness in allowing him to start with you! I’m sure he’ll become very proficient with more experience.

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Well the table is summary of the study. It is saying that patients who go through neck dissection surgery (which Styloidectomy is part of) nearly half (46%) had their IJV narrowed after surgery and only 6% had their IJV occluded after the surgery with the remaining 50% having their IJV not affected by the surgery (neither narrowed nor occluded - fully patent). 3 months after the surgery (last column), 90% had their IJV fully open (patent) and only 10% had their IJV remained narrowed with 0% occluded.

Bottom line is nearly half of patients going through neck dissection surgery, will have some narrowing on their IJV on the operated side but most of them reopen fully after 3 months post operation with only minority remaining narrowed. It is also important to note that none remained occluded (closed) after 3 months. So if your new symptoms are due to swelling, chances of recovery is very good (90%) but if it is due to other reasons (like the remaining Styloid or C1…etc) then this study has no impact on those and IJV might remain collapsed but you said it only became 100% closed (occluded) after the surgery so the chances of it reopening is great as 0% remained closed after 3 months in the study.

I hope I clarified it. Wishing you peedy recovery.

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Also if you want to see what pre/post operation IJV narrowing looks like, an excellent comparison done by our friend @M_UK (shown in the link below). Although his surgery was a combination of Styloidectomy & C1 shaving.

A picture is worth thousand words as the old adage goes.

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Interesting research. I have found after both my surgeries that swelling & fluid in early stages have meant difficult post op symptoms fir a number of weeks. This second time I am doing better than first time as learnt about/using lymph drainage to move fluid. Hope you all start to feel better soon. In your shoes I would book Dr with experience so in his queue. You can cancel nearer the time if not needed (sure others would take the slot). D

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This is great information too. And that makes sense now looking at the chart again. My biggest concern is that my compression won’t be completely gone after the swelling and everything completely dies down. These are new symptoms though so I hope they’ll at least go back to where they were beforehand. Much more bearable beforehand. I really do also fear that my remaining styloid bone in there might be causing issues. Specifically I’m having more throat issues than normal so I really think I might have to have a revision surgery unfortunately.

And I’m really wondering about C1 shaves and how realistic they are to do. I have heard lots of horror stories about those and stents in the jugular. Two things I want to avoid if I can. But I’d also like to get back to normal life if I can!

So hard to know what the true problem is through all of this. I guess time will tell though! Thanks again for your feedback and the research in pulling out articles here. Still learning how to search and find things that I’m looking for. Thanks!

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I’m booked with Dr Hepworth in March already. Hoping he’s still kept my date because I’m most likely going to need it. The other doctor I looked into was Dr cognetti in Philadelphia. Not sure if he would be a good option but I need to look a little bit more into him as well. He would be much closer, but doctor Hepworth definitely has the most experience

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@Millcf - Dr. Hackman in Chapel hill, NC, has helped a number of our members who have VES. He is also the only doctor on our list currently willing to do revision & bilateral ES surgeries. He’d be even closer for you.

@Ddmarie, @JPB & @vdm all had surgery done by Dr. Hackman w/in the last month or so. You can private message any of them to ask questions.

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Hi! Absolutely please feel free to reach out to me with any questions you may have on Dr Hackman. He would be a lot closer to you and I completely trust that he knows what he’s doing, as does the team he has with him in the operating room.

Edited for a word I misspelled

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Hi, I will comment on C1 topic you mention. I had my C1 trimmed on both sides during 2022 (but am based in UK). If C1 is a significant factor in JV compression, then provided you find the right surgeon who will take a conservative approach and just trim the amount needed I would reassure you that C1 trim is ok. I have a small scar behind bottom of ear and I did get Some swelling post op but far less than with styloid ENT neck operations. What you want to avoid is aggressive surgery that removes too much/all of C1 as I think that can cause instability if major muscles attached to C1 lose the bone they anchor to. I did need to pay attention after each surgery to check I was using neck muscles to keep head supported in right place (pushing upwards on operation side) but my body soon learnt & adapted to the slight change to C1.

I agree we all hope to avoid some surgeries but for me symptoms were so bad that it became my only way forward. Hope you find solutions/help you need soon. This journey is tough. D

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There are very few doctors in the US who will even consider a C-1 shave but we know of at least one who carved a channel in the TP of C-1 to make more room for the IJV & another who gently scoots the IJV over & attaches it nearby to hold it away from C-1. These procedures seem to have been successful in helping stop IJV compression.

I definitely will have to. Does Hackman also do the decompression of the IJV as well? I’m really hoping to get someone who will be Able to find what is slamming my IJV against my C1.

Do you know what doctors do the later and connect it away from the C1?

Hi @Isaiah_40_31, what are the names of each of those surgeons please? I’m interested in the different c1 shave techniques

Dr. Peter Nakaji, 755 East McDowell Rd., Phoenix, AZ, 602-521-3201, Works w/ vascular ES, has also done a research paper about shaving C1 process as well as styloidectomy
https://doctors.bannerhealth.com/provider/Peter+Nakaji/1103456y_source=1_MTQ4NjU5MDktNzE1LWxvY2F0aW9uLndlYnNpdGU%3D

Yes, I am here for you if you want to reach out. I am interacting regularly with Dr. Hackman’s office in light of some lingering symptoms that are requiring treatment.

I start PT on Monday and am at my 30 day mark for post-op from Dr. Hackman. Oddly, my scars started rashing out - red, and in some cases pustules, on the incision area.

Pain - still around a 5-6 on most days if I am not taking some ibuprofen, and is mostly in the ears, jaw and neck. First bite remains a remarkably interesting experience (that is my positive way to describe it) and can put me to my knees but I prepare for it now and ease my way into a meal.

JPB

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