Advice on Surgeon Selection after Hui Consult

[dreamliner]

Hi all, had my consultation with Dr. Hui a few weeks ago & was happy to finally get some straightforward answers. I was offered surgery next year as part of the Hawaii quarterly meeting of Hepworth/Theler/Zhang, but that would require substantial travel.

To Summarize: 26 year old M, symptoms since age 21.

Hui: Imaging & Tests sufficient to confidently recommend IJV decompression. Mod-Sev Bi-lateral IJV compression from C1 Transverse Process & Styloid. Right IJV Dominant. Possible CSF Leak, Dysautonomia for 4+ years, possible Vagus Nerve Compression. Cranial CSF Leak repair in 2023 due to leak from duraplasty, which was placed in 2020 during Chiari Decompression.

Main Symptoms: SIH (low pressure) headache, dysautonomia, chronic fatigue, nausea, tinnitus, brain fog, MCAS.

I’ll throw some images below if anyone wants to see. My primary problem right now is I am unsure which of the surgeons to go with given the procedures I’ve had in the past. I’ve read through many posts on Hepworth, Costantino, Lo, & Nakaji, however I was wondering if anyone else had thoughts or had a similar situation. I have consulted with Costantino x2, Lo x2, Patsalides x3.

Costantino has supported surgery from beginning, & Q collar trial bolstered his position. I have read many good things about him, however my main concern is what appears to be relatively limited knowledge on co-morbidities, as well as components of the surgery outside of direct IJV involvement (such as vagus nerve, CSF leak, etc.)

Lo has always called me “as challenging as it gets,” & has been very reluctant to offer surgery, as he believes I am in low pressure, & that IJV decompression will make my SIH symptoms worse. I do however feel like he understands more of the bigger picture.

Patsalides has agreed with Lo in large, however he believed I should be offered surgery because “there wasn’t anything else for me to do” after we tried yet another myelogram in late 2023, which failed to show a leak. He said I’m looking at a 10% chance of getting better from the IJV procedure.

I reviewing with Dr. Hui, I went over how everything started as a Chiari diagnosis in 2019. I underwent two Chiari decompressions at Johns Hopkins, with the second involving a synthetic duraplasty. He believes what was, at that time, undiagnosed IJV stenosis, provided a constant high pressure force that eventually found a defect in my duraplasty seal, therefore leading to a CSF leak from my surgical site, which was eventually treated in spring 2023.

My leak never showed up on countless MRIs + 5 myelograms at top institutions. I was offered exploratory surgery, & only then did my surgeon find the leak, which was very small in size. Post-op my headache immediately changed to one of high pressure, & I thought I was experiencing RIH following leak closure. However that only lasted ~four weeks, until I went back into low pressure symptom presentation. Our theory is that RIH from leak closure + underlying high pressure from IJV stenosis caused an accelerated ICP increase, leading to either a rupture of the recent surgical site, or creation of a leak elsewhere.

He believes that normalizing CSF dynamics some by undergoing IJV decompression could allow my suspected CSF leak to heal, which I would tend to agree with.

If you made it this far I sincerely appreciate it, & my last questions would be: How did you decide on a surgeon if you had a rather complex case? If you had dysautonomia symptoms, did that at all change which IJV side was operated on? If you knew you usually scarred heavily, is there anything you did pre/post op to lower the risk of post-op IJV scar compression?

Costantino & Lo are very close to me, & travel causes considerable MCAS flares which I would assume are not good for prep or recovery. However I do want to make an informed decision, & would consider meeting with Hepworth if there could be a substantial difference in treatment. Thanks a lot everyone!

[JugularEagle]

We can’t possibly know all the factors that you have to consider. However, I will tell you what I would do in your shoes. I would jump through all the hoops I had to in order to try to get that surgery with the quarterly meeting team. Even if that meant I had to stay longer to recuperate.

But your situation may prevent that. Maybe I can take your place

Good Luck with whatever decision you make. You have a lot of life to live. I hope someone can straighten out your situation.

[Isaiah_40_31]

One of the first things I noticed in your images is how ramrod straight your cervical vertebrae are, @dreamliner. You’ve completely lost your lordotic cervical curve which puts the nerves & vascular tissues in your neck at greater risk of contact w/ & irritation from your styloids. I don’t know much about Chiari & perhaps this is a function of your having Chiari, but it is a problem we see among many of our members, & it’s a correctable problem, however, it takes time & consistency in doing he appropriate exercises. In our fast paced computer & cell phone driven world, many of us have taken to spending our days looking down. Over the years our once happily curved necks straighten out & then problems begin. This situation can result in military neck or forward head posture. Yours looks more like military neck to me. We have a lot of information on our forum on this topic & there are many YouTube videos which also address it. Since you have IJV compression + comorbidities, it would be best for you to see an experienced PT vs trying to fix this on your own. In the end, regaining some lordotic curve, could help reduce your symptoms.

Dr. Hepworth is quite astute in noting comorbidities or possible commodities that the patients themselves aren’t aware of so he is a good choice for you in that arena & would be knowledgeable about the other things you’re struggling with beside ES & IJV compression. His office is frustrating to work with because of the inconsistency of calls being answered & vm/emails being returned. As long as you’re willing to be patient as you try contacting his ofc, I think you’d find a consult with him satisfying & informative.

I’m sorry I didn’t really address the questions you ask. I know some of our members who’ve seen him have more complex cases. Hopefully some of them will reply to your post.

[dreamliner]

That’s very kind, thank you for your thoughts! I was thinking of perhaps going down the route of trying to get a Telehealth with Dr. Hepworth before I made a decision of scheduling with the Hawaii group, however that can be a bit tricky with his office. I wish we were all a bit more streamlined so things could be a bit more straightforward with decision making, but I guess we just do the best we can in the meantime. Best wishes to you as well!

[dreamliner]

That’s a great observation, funny enough I always wondered if that’s what I was seeing on imaging all these years…but no one else has ever commented on it much. I was actually in college & working as a flight instructor when all these symptoms started. I do wonder if that had anything to do with it…we spend plenty of time looking down at our instruments/iPads/etc, & that plus regular electronics use could certainly contribute. I remember in the webinar that someone mentioned: “Our bodies can compensate until they can’t.” Maybe that was the last straw that started making me feel some of those pre-existing anatomical things? Hard to say for sure but certainly a possibility.

Thank you also for the vote of confidence in Dr. H, I feel like I’ve had so many invasive procedures at this point that I really want to make sure I’ve exhausted my consult options before making a decision. I may have “run” into other procedures a bit too quickly once a doctor or two recommended them, but with the varying success rate & tough recovery of this one, I want to make sure I’m being as thorough as possible. Appreciate your help & thoughts!

[jrodefeld]

I second the recommendation to see Dr Hepworth. I’m having surgery with him on November 11 and it’s great to go into a surgery with complete confidence.

One thing I haven’t had explained is what rationale there is to consider going to Hawaii for surgery with Hepworth/Theler/Zhang, as opposed to just Hepworth himself in Denver?

As I understand it, one purpose for Dr Hepworth to go to Hawaii quarterly is to help train other surgeons in his techniques which is incredibly important no doubt. But is there an advantage for the patient?

What role do Theler and Zhang have in these surgeries versus what Dr Hepworth does himself?

[dreamliner]

That’s a great question, & thank you for your thoughts! In your case, do you have more of a styloid or C1 involvement, or is it split between the two?

From when I talked to Dr. Hui a few weeks ago, it seems like Hepworth on his own is a bit more reluctant to involve something like a C1 shave, & will usually opt for another technique such as IJV relocation. Apparently he will touch C1 if he feels like it is necessary, but Dr. Hui said the goal of the Hawaii group would be to make a C1 shave standard procedure for those who need it.

I’m assuming Dr. Zhang as a neurosurgeon would take responsibility for that within the group of surgeons they have there? It seemed like Dr. Hui was planning on using data from these more “standardized” Hawaii procedures to perhaps identify & solidify the best surgical techniques going forward. At least that’s what I gathered from the appointment

[jrodefeld]

Okay, that makes sense.

In my case I think it’s mostly styloid, but maybe some C1 involvement. In my case I think it might even be more muscular (digastric, omohyoid) or scar tissue contributing.

[JugularEagle]

Did they tell you what it takes to be selected to be operated by the group? Like it can’t just be an “ordinary” bilateral vascular Eagles with C1 compression. Does it have to be one of the horrible/complicated/ dire cases?

[dreamliner]

That’s a good question, he didn’t spell out anything in particular but he did say they try & prioritize younger people when they can, I guess along the lines of trying to get them “into the world” if they haven’t had the chance yet. Perhaps also if he feels that there are multiple other co-morbidities (such as a CSF leak, MCAS, dysautonomia, etc.) that stem from the IJV, that group would try & help those people first? Just speculating there but that’s what it seemed like. Although I would assume that maybe once it gets flowing a bit more, they will be able to select a bit wider of a patient population.

[JugularEagle]

You would seem to fit into their research surgery category. I would encourage you to seriously consider them as well as the others that are available as you make your decision. Travel to Hawaii is tough. There is a concern of that travel after the surgery. Costs in Hawaii are much higher.
Did they offer any kind of extra discount for cost as being part of this special group. There are definitely people here that seem to qualify and have difficulty with cost.

These surgeries are the ones that end up in research papers.

[Isaiah_40_31]

Dr Hepworth has recently begun doing C1 shaving more frequently. I take that to mean that he’s either feeling more comfortable with the outcome of C1 shaves vs moving the IJV &/or that he’s getting more practiced doing them so is more at ease when he does them this does them more often. I’m sure he would never do a C1 shave unless it’s needed. .

[Chrickychricky]

In my own decision making process regarding surgeons, I chose someone who not only has experience performing the surgery but who is also actively trying to understand the disorder and develop a best practices model for people like us. I think you are in great hands in Hawaii and certainly Dr. Hepworth has a lot of experience with complicated cases.

[Jules]

It’s a difficult decision and I totally agree that you want to get this right! Members have had successful surgeries with all the doctors (I can’t recall reading much about Dr Lo, can’t see anything in past discussions but I know his name has been mentioned!), they’re all very skilled, but I agree with the others that if you have complicated medical conditions then it sounds as if wither the Hawaii team or Dr Hepworth would work best to support you in that. I would be a bit daunted at having surgery with a doctor who isn’t keen on doing the surgery, or doing it as a last resort, whereas others will hopefully work with you to address anything else which crops up, for example Dr Hepworth has fixed CSF leaks for members when needed.
I don’t know much about MCAS, but some other members have had this and have taken steps to help them during their hospital stays, you could use the search function for that & hopefully others will chip in.
Re the scar tissue, it’s asked quite a bit & it would be good to get an answer from one of the experienced members about how to help that. External scars are best massaged with some sort of oil, Mederma, Bio oil etc. once the incision has healed. Some members have had cold laser therapy for scar tissue but this needs to be done by a knowledgeable practitioner to avoid the thyroid gland.
Best wishes with your decision!

[dreamliner]

That’s a great point to be aware of those things that come with a stay in Hawaii. I have not heard of any sort of financial aid or discount for this program – I think it would be the same type of situation as if you had it elsewhere. I still have great insurance from my parents, so I can factor in some reimbursement from my company for travel/lodging/etc., but yes the costs are still high.

I wouldn’t anticipate any sort of big complications after the surgery, however I wonder if that could play a more prominent role in decision making. I am 12ish hours by plane to Hawaii & 4 to Denver. I can be about 2/3 hours from Dr. Costantino by car, so I obviously do have to factor that in a tad. I have contacted Dr. Hui’s office about a referral to Dr. Hepworth (on top of another question I sent last week), but I suspect they move a tad slowly as well.

[dreamliner]

Absolutely, it seems like Dr. Costantino has established his own standardization between himself & Dr. Tobias, but he doesn’t seem to be connected much to the “usual” group of people that we discuss in here. I got the impression he wasn’t too much of a fan of other places’ techniques, as he seemed pretty confident in what he’s currently doing. I do question some of his understanding/explanations about things outside of “just” the direct IJV compression.

I’m trying to find people with similar stories & line up which doctor they went with/how they did, but it’s amazing how different we can all be within a relatively niche disorder.

[dreamliner]

Thanks for the insight Jules! I totally agree, I think even a bit of reluctance on the surgeon’s part would make it difficult to roll into surgery with them. I’ll certainly search for some other MCAS posts – I was traveling recently with friends & it was such a striking difference between my “less stressed” life at home. I don’t have the typical symptom presentation, but even without a flare, my plasma histamine levels are 10-15x higher than average.

It might even be worth a consult with Dr. Hepworth just to talk about my CSF leak theory alone – I suspect I am still leaking from my sub-occipital surgical site. I know he has spotted stuff in the past that has eluded many others, so perhaps I should pause my decision until I know whether I can consult with him or not.

I might search up more on the scarring as well – I’m actually more concerned about internal scarring. I mean I have pretty raised scars externally, but I’m more concerned about potential scarring that could compromise the decompression of the IJV, which I’ve heard supposedly can happen. Appreciate your thoughts, thanks!

[JugularEagle]

I would not a flight straight from Hawaii to Indiana after my surgery. You were a flight instructor so you know the problems that can happen in the air. Blood Clots, etc. You are going to likely feel bad afterwards for a bit.

Consider it a vacation. One or two weeks in Hawaii . Then book some kind of flight that allows a layover for a day or two in California.

This is one way to deal with the travel. In theory it would work. However, you see how some of these people feel after surgery. It is rough. You could also price out first class tickets for the ride home. I know for me where ever I have my surgery I am pricing in return first class into my budget because I don’t want to be dealing with the cheap seats and all the jostling and lack of room, etc.I am much older than you and know recovery is going to be an issue.

Hard decision to make but the more complicated the case the more a person needs the best that they can afford and that insurance will allow.

I was impressed with the way Nakaji handled the poster’s recent operation. He quickly adapted to the nerve monitoring and changed his plan. Time will tell if it solves the problems for the poster.

[Isaiah_40_31]

@dreamliner - I know a patient of Dr. Hepworth’s who’d had problems with scar tissue build up causing IJV compression. When Dr. Hepworth did his surgery last year, he removed the problematic scar tissue & wrapped the IJV w/ some sheep tissue to prevent future scarring from causing a problem. I can’t remember what the material was he used, but that’s to say, there are ways of dealing with situations where excess scarring is a concern.

[JugularEagle]

It is hearing these nitty gritty details of how a surgeon deals with complications while in surgery that helps future people decide on who to use.