Update - Consultations with Hepworth, Costantino, & Nakaji + Discouraged

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Hello!

I recently have been fortunate enough to have had consultations with Dr. Hepworth, Dr. Costantino, and Dr. Nakaji. It did take quite awhile and bit of effort to make it happen, but I am glad to have had the opportunity to meet with each of them.

Overall though, after meeting with each of them, I am feeling fairly confused and discouraged. The combined summary is basically Hepworth and Costantino feel strongly about moving forward with surgery and Dr. Nakaji declined surgery and thinks I should explore other options first. I’ll go in a little more detail if that’s okay and maybe y’all can help me out, offer encouragement, or just commiserate with me.

HEPWORTH: Visit with Dr. Hepworth included review of CTA and Doppler Ultrasound and he concluded that in regard to the CTA: left jugular vein is absent, right jugular vein is diminutive, right transverse sigmoid sinus with an arachnoid granulation. He also noted a strong possibility of tethered cord, and low-lying cerebellar tonsils.

In regard to the Jugular Ultrasound: Right: Base of Neck 150.3 cm/s ; SCM 187.6 cm/s ; Carotid Bulb 26.8 cm/s ; C1 31.5 cm/s ; Foramen 48.0 cm/s Left: Base of Neck 75.5 cm/s ; SCM 83.2 cm/s ; Carotid Bulb 0 cm/s ; C1 0 cm/s ; Foramen 0 cm/s

I’m not sure if any of those numbers make sense to any of you, but it sounds like the right flow velocity was elevated to some extent, and on the left they could not obtain measurements at certain points. He went ahead and got me on the surgery schedule later this year, so it seems like he feels pretty confident about the issue. His notes say, “Recommend a right styloidectomy with jugular vein decompression first…Discussed there is a chance of doing a left styloidectomy/venous decompression in the future.” He was the doctor who prescribed me Brilinta (blood thinner) which I feel has worked better than I originally thought (when I went off it, I noticed more brain fog, worse headaches, increased visual snow), so I asked to stay on it.

COSTANTINO: I really liked Dr. Costantino, I felt he was straightforward but also compassionate. He reviewed the CTA and Doppler Ultrasound and said that the right side was significantly compressed by the styloid process and the left vein was hypoplastic. I don’t have his notes, but he said my case was a “no brainer.” I had had the dynamic cerebral venogram done the day before, so I didn’t have Dr. Mehta’s notes yet, but I let Dr. Costantino know Dr. Mehta’s thoughts. He seemed hesitant about the results because I was under sedation and he would have preferred a balloon venogram (or something like that). He said that he wouldn’t have even ordered the cerebral venogram though (done by Dr. Mehta) because he felt like the imaging I had sent him, along with the symptoms I was describing, were already convincing enough. Dr. Costantino felt the Brilinta was a pretty risky drug to be on because it is a blood thinner and said I should really consider the benefits vs. the risk.

Dr. Nakaji/Mehta: I really pushed to have the cerebral venogram done and to have a consult with Dr. Nakaji because I want to make sure I’m making the wisest decision regarding treatment, and also Dr. Nakaji is familiar with another condition I had called Chiari Malformation. I thought he would be an ideal person to speak with.

The cerebral venogram/catheter venogram went fine and it wasn’t too uncomfortable. They gave me enough sedation that I could acknowledge them when they asked me to turn my head, but otherwise I was pretty much unaware of them fiddling around. Except, I think when they put in more contrast or something, I would feel these rushes or waves of burning in my head that did not feel good (enough to where I had scrunch my face :laughing: and I usually don’t have to do that unless it’s a severe headache). I’m not sure if that is normal, but I figured it must be because it is a fairly extensive procedure.

Anyways! I felt sure that the results would be agreeable to Dr. H. and Dr. C.’s opinions. However, my results from Dr. Mehta are:

“Dynamic cerebral venogram performed with Dr. Mehta shows - the left jugular vein is thrombosed, with the right one open and only with minor stenosis and no significant gradient across the jugular vein including with provocative head positioning.

While there is stenosis of the right internal jugular vein which is dominant at C1-C2, there is no significant pressure gradient in neutral position. With head flexion there is a pressure gradient of 2mm Hg from C1- C4.”

Because of that, Dr. Nakaji’s consultation resulted in the opinion of: “We discussed that although there appears to be a focal point of compression on CTV, it is not severe and there is no pressure gradient on venogram. As such, it is unlikely that she would benefit from jugular venous decompression.” And then he recommended I look into other headache therapies (which unfortunately I have, but I listened to him because he is an expert and I take his opinion seriously). Dr. Nakaji also felt that there was probably no need for me to stay on Brilinta.

ULTIMATELY, all of the doctors seem to agree my left vein is not very functional, but have differing opinions on the right vein. But honestly, I’m not sure what to think if the pressure gradient aren’t high or indicating an issue? Regarding the left one being out, I know some people function on only one and that is not necessarily dangerous in itself and they are healthy and okay? I’m wondering if maybe I’m just one of those people who has a harder time than others for some reason, or maybe like Dr. Hepworth discussed, it could be a combination of issues working together. I’m not really sure. Or what is IJV compression isn’t my issue at all?

My plan is now to send of Dr. Costantino the results from Dr. Mehta’s testing and hear his thoughts. He wanted to meet again, so I’d like to go ahead and try to get that appointment scheduled. I already have an appointment scheduled with Dr. Hepworth in August.

Anyways, I left Dr. Nakaji’s consultation feeling appreciative but also discouraged. Wondering if anyone has any help or guidance, or encouragement. Thank you!

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Here are some lectures that should be helpful.

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I appreciate it - thank you!!

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@Kit_Cat - I’m sorry the appointments left you in confusion rather than providing clarity. I think your plan to see both Drs. Hepworth & Costantino again to discuss your new imaging results & get another set of opinions is good & may help you have a better idea of what is your best next step. Did you discuss your Chiari Malformation with the two of them as well at the time of your consults?

I know I’m not a doctor, but I also know from being on this forum for 11 years now that we are not created the same. Your situation may indeed be bad enough that the IJV needs to be decompressed, especially in light of the fact you only have 1 functional IJV. Your brain needs all the vein space it can get to drain the deoxygenated blood. I think surgery could help you.

On this subject, we have another member in a similar situation. I have examined her CT scan closely, & even though Drs. Hepworth & Costantino both dismissed it as being too hypoplastic to decompress, it appears to be pretty normal below the hypoplastic area, as does yours based on the US, which says to me there may be compression closing off the area that’s been deemed hypoplastic, & if that’s removed, the vein might open up & be able to help drain the brain. Maybe I’m just an optimist, but it seems if normal tissue can be seen below abnormal, there may be some way to restore the abnormal to make it more normal.

I’m interested to hear the info your second round of consults gives you.

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I don’t know about the pressures so can’t comment on that…but I can certainly commiserate with you on the differing opinions! It’s so hard to get consultations with the most experienced doctors, we always suggest second or third opinions if possible, but now it’s left you more confused… If you’ve got only one decent IJV & then that’s being compressed, even a small amount of compression I would’ve thought would make you quite symptomatic? And surely the fact that brilinta has improved your symptoms surely indicates that the compression is affecting you? But that’s just my opinion, not a medical one…

I guess you’ll have to wait & see what Dr Hepworth & Dr Costantino both suggest given the new testing, and take it from there? I wish we could offer you more suggestions, but sending you a hug & will pray that you’re guided to the right decision :hugs: :folded_hands:

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@Kit_Cat did the doctors inform you where the ijv compression is occurring? I assume it’s happening at the level of C1 since we are on the topic of ES? Based on the velocity readings you posted, I wonder if your right SCM is compressing your ijv in addition to styloid/C1.The velocity seems high in the SCM area, but could just be continued effect from C1 level. Do you have access to your CT imaging? If so, can you go to axial view and upload a screenshot of the area of C1? I can help you identify the ijvs and whatnot and we can follow them down to see if your SCM is involved! Mine are involved so I know what to look for. Here’s an axial image of my C1 to help you know where to look.

Screenshot_2025-06-01-19-27-49-96_40deb401b9ffe8e1df2f1cc5ba480b12
Screenshot_2025-06-01-19-27-49-96_40deb401b9ffe8e1df2f1cc5ba480b121080Ă—2376 147 KB

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@Kit_Cat I’m sorry there isn’t a clear path forward. The venogram is highly suggestive that decompressing the IJV would not give you relief as there is essentially no pressure gradient. I think a gradient of at least 5mm Hg or even 8 demonstrates that the stenosis is causing the symptoms. Dr. Amans measure pressure gradients at 10 sites starting at C1 all the way up to the superior sagittal sinus. My pressure gradients varied but were as low as 8 mm Hg and as high as 20 mm Hg. Of course there is always the possibility that the sedation you had lowered your pressure gradient and you ended up with a false negative. But assuming the gradient measurement is true, it does suggest that this particular surgery won’t give you the symptom relief you hope for.

Oh, your question about the burning in your head when the contract is released, that does occur and often suggests migraines. I wonder if it would be useful to have a consult with Dr. Cha or that neurologist that has been on both the webinars who is based in NY. I think her last name starts with an R. (Ruhoy)

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Hi @TML thanks for your response! I think they said the compression is at the C1, so I think you are correct. I have uploaded an image that I think is in the correct area - let me know if I am off though! It is image 187 of 313 of my CT imaging. Thanks for your help! I greatly appreciate it!

CT 187
CT 1871092Ă—1128 72.3 KB

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Hi @Chrickychricky , thanks so much for your response! That is what is sounds like regarding the pressure gradients and I definitely don’t want to have an unnecessary surgery. Thanks for commenting on the burning sensation! Yes, Dr. Nakaji gave me a referral to a Dr. Jill Rao (I think). She is in Arizona though. I am uploading my gradients below - none of mine were anywhere close to 20mm Hg and were more between 7 and 11 mm Hg.

Pressure Gradients
Pressure Gradients670Ă—434 62.7 KB

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Hi @Jules , thanks for your response! That’s what I would think regarding the right IJV - that I would experience some symptoms even if it was slightly compressed. I also thought because of the Brilinta working that that might indicate the compression was impacting me. I am considering going off it again for maybe a week or two to see what happens. So, all of it is very conflicting. I have my experience and then different professional perspectives and in various ways they conflict with one another to some extent. I appreciate the hug :people_hugging: and I’m crossing my fingers I’ll get some clarity at my next appointments also!

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Hi @Isaiah_40_31 , thanks for the response! I did discuss having Chiari Malformation with each of them - I have had decompression surgery for it though (2009, 2012), but it seems like the cerebellum has dropped a bit again (not to the extent it was pre-surgery), but enough to possibly cause CSF fluid issues. Dr. Hepworth seemed to put the most emphasis on how it could be affecting my overall system.

I also feel quite certain that one of the primary issues causing my symptoms is an IJV issue. It was kind of interesting when I was reflecting on my consultations, both Dr. Hepworth and Dr. Costantino reviewed my imaging with me from the axial point of view, but Dr. Nakaji only reviewed my imaging from the sagittal view. He made it sound like if you were to look at something from the side that looks very thin and then view it in a different angle and it turns out its wider than you originally thought (such as like a notebook or something), that’s what the results of his testing showed. So it might look like I have compression from one angle but it is actually wide enough for me to have adequate blood flow. Dr. Hepworth kind of described my right IJV as being “ribbon”-like, so kind of similar, but indicates I had very minimal adequate blood flow, so I’ll be very glad to hear his and Dr. Costantino’s thoughts after they review the testing results.

I appreciate your optimism! I really feel like I am experiencing some type of cerebral vascular issue, but for some reason one “no” feels so much louder than having two people say “yes.” Thanks again and I’ll update once I have more information!

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Food for thought.

If someone has a connective tissue disorder the pressure readings may be inaccurate because the reference range is based on a normal range not for someone with a connective tissue disorder. The pressures would be considered low to normal but not for the individual with a CT disorder. Their blood vessels would be more compliant (stretchy). Thus not registering a high pressure for a normal individual but may be high for an individual with a connective tissue disorder because their vessels are more elastic and do not exhibit a high pressure compared to a normal individual.

Some people say that a high percent (25-40%) individuals with Eagles syndrome fall into some spectrum of connective tissue disorder. There may be a correlation with Chiari and Tethered Cord Syndrome individuals and the connective tissue disorder spectrum also.

With that in mind the, basing a diagnosis on normal pressures could be incorrect for a individual that falls into a spectrum of the connective tissue disorder.

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Yes, thanks so much for sending me this webinar - it was so informative and also validating! Hopefully something similar is happening in my case. Dr. Hepworth thought I fit the profile of having a connective tissue disorder and felt my scans indicated that also (somehow?), and also felt I should look in POTS, so it could be a possibility.

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So around the 37:15 minute mark or so when Dr. Hui is speaking about a compliant system, is he saying that because individuals with connective tissues disorders have a more compliant system, their numbers would appear more “normal” (between 2-4 mm hG) even if they are still symptomatic (maybe even elevated for them), but it’s because they are comparing their numbers against data from the general population vs EDS/connective tissue population, which needs to be looked at on a different/relevant scale? (Sorry if that’s confusing!)

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Exactly, you got it. I really like that webinar. It is excellent, but it is long.

So there are a few things that could have caused the lower pressures (not detecting a high pressure gradient) 1. you were sedated, 2. you were probably lying down (some pressure gradient may only be detected if standing vs lying down due to the hemodynamics of the venous system), and 3. the possibility of a connective tissue disorder. It could be an accurate reading. Nevertheless relying on the pressure gradient and not looking at the whole picture is wrong in my opinion. A pressure gradient that is detected is just a tool to validate everything else. It is not the sole evidence needed for a vascular ES diagnosis. ( again just my opinion) I think the a main reason for the pressure gradients is to determine when to stent or not on some stenotic vessels. (Could be totally wrong on that) There are a lot of variables with ES IJV compression and gradients from just standing vs lying, movement of head in a certain direction while standing vs sitting vs lying down. I feel the pressure in my head change with movements. Hope this makes sense. Oh well, enough for now.

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Yes, thanks so much for sharing! I agree that I need to keep an open mind about my results, including that they might just be accurate. With that said, I also agree with you that’s it important to look at the whole picture rather than just relying on one test. Thank you again!

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@Rex the connective tissue disorder & pressure gradients being different makes sense, that’s a good point :+1:

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@Kit_Cat I’m not really sure how to read the pressure numbers you listed. On my report there are 2 numbers for each area measured the first is the venous pressure and the second the guide catheter venous pressure. For example: Torcular 18/0, so a pressure gradient of 18. This must be so frustrating for you. I’m so sorry it isn’t straight forward.

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@Chrickychricky Oh, how interesting! I did not see anything like that on my report. That’s okay though. It is frustrating and mostly discouraging, but I also feel like I know so much more than I did a year ago or even six months ago, so I feel like I’ve made a lot of progress overall and hopefully there’s more to come. Also, I want to make sure I’m making the most informed decision, so I’ll just keep exploring until I find a more concrete answer. I appreciate it though and everyone’s support!

I also meant to mention it on my main post, but there’s no way I would even be where I am right now without the support, knowledge, encouragement, and information I’ve received and found through this forum! So I appreciate everyone! @Isaiah_40_31 @Jules @Rex @TML @BirdsOfSore @Chrickychricky + everyone else who has helped me along the way!

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@Kit_Cat -
We’re so happy to be here helping you. This site has seen some masterful teamwork by our members over the years & I’m glad it’s continuing to be present here.

We’re with you for however long you want/need us.

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