Hello, my daughter has just been diagnosed with bilateral jugular vein compression. She has been wheelchair bound for the last several yrs. Her other diagnosis are POTS, MCAS, hEDS, tethered cord, MALS, nutcracker and possibly Maythurner Syndromes, IIH ect.. Dr Henderson and Dr Middleton both suggested that a lumbar shunt would be a better idea than trying to address the jugular compressions because of her hEDS. There’s possibly of creating a major CCI issue by removing styloids. Any thoughts or advice for us?
I’m so sorry that your daughter is going through all of this, that’s awful for her 
We have had quite a few discussions about Eagles and CCI / EDS , and the answer is even doctors disagree! The C1 processes are often involved in IJV compression as well, & some members have had these shave to make room for the IJVs, and there’s been debates about whether this can worsen instability too.
So some doctors believe that the styloids and stylo-hyoid ligaments can calcify & elongate to stabilise the neck if the ligaments are weak through EDS or another reason, but there’s also a thought that the styloids themselves can cause unnatural head positions , which can then possibly weaken muscles and so make the neck less stable?
We’ve had members with instability who don’t notice any difference after surgery, and some who feel it’s worse, so I’m afraid we can’t give you a definite answer. I guess that if you’re able to get several opinions from the doctors who know most about vascular ES (like Dr Hepworth in CO, Dr Nakaji in AZ, Dr Costantino in NY & Dr Cognetti in PA) then this might help.
Buth there’s fors & againsts, as putting in a lumbar shunt can be carry some risks, can get blocked, and isn’t solving the problem of IJV compression?
Maybe @Mod_support Merl might be able to give you info about lumbar shunts having had IH?
Any surgery I guess with the MCAS is going to be difficult, it’s a real dilemma for you both, sending you a hug & praying that you find some answers 
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Thank you for the hug, prayers and support! It means so much! I would really wish to hear from Merle, as all this has far reaching affects.
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Hey @Rosa ,
I’m Merl, apologies for not responding earlier. I didn’t see the notification until today. Your daughter has a few contributing factors which, when taken individually, could be challenging. But when taken as a whole (the whole person) can be a huge mountain to navigate.
As I’m sure you are aware, there are a few different types of shunts from brain shunts such as VP, LP and VA. There are a few variables to establish which type they elect to use, for example using a (V) ventricular type shunt when the drainage issue is further down the spine may be deemed inappropriate. This needs to be assessed by your medical team.
A shunt alters the intercranial pressure (the pressure inside of the skull) and some people can adjust to the change fairly well, but for some of us those changes can throw everything out of whack and it takes time for both the patient and their body to adjust and acclimatise to this new normal. Some medicos like to use an adjustable valve to regulate the pressures, some medicos prefer to use a fixed pressure shunt. It’s not that one is better than the other, but if the intracranial pressures fluctuate, a variable pressure valve is often used to assist in regulating the pressures. Personally, I’ve had both a fixed pressure valve and a variable and had differing issues with both, so I couldn’t say which is better.
Hope it helps
Merl from the Modsupport Team
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Hey, that’s ok! We can’t be watching our screens all the time. The info is really interesting. Dr Henderson is proposing a lumbar shunt. With an adjustable valve but I don’t know if it automatically adjusts itself according to the pressure or if it needs to be adjusted wi5h a magnet. I am inclined to think he was talking about one that needs adjusted. This is all so scary. There’s too many things. MCAS, hEDS ect…Dr Hepworth shook his head when our friends questioned him about placement of lumbar shunt. Dr Henderson shakes his head when we suggest messing with her neck. Two reputable drs…HELP!
One thing in her favor is that her flexion extension MRI did not show any major instability in the cervical spine. But I wonder if the congestion in her neck is holding everything together? If we remove it, will there be CCI after all? But by placing a shunt, you are not relieving that all important vagus nerve.
So glad for this forum to bounce ideas around and pick people’s brains. Even if no one can give definite answers, we don’t feel so alone?
Our hEDS, MCAS, POTS friend had a VP and suffered a plug every several months. Finally, after her last revision, she suffered tremendously until meningitis was discovered as well as a bad infection in her tummy. She was in ICU for several weeks with an external drain until finally they discharged her minus a shunt. Now her pressure is again building and we know what misery that entails. They are considering a placement on the other side of her head as it’s too soon to place one where infection was present. This does not bolster our courage concerning shunt placement.
Our other MCAS, POTS, hED friend is ending up with multiple fusion surgeries because of severe spinal instability. That is miserable and costly as well.
All and any thoughts are appreciated. Your shunt experience would be interesting to us, however much you’re comfortable sharing. I know you’re probably busy, too.
Blessings,
Charlene (mom)
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I’m glad you find it helpful to share stories on here, even if as you say we might not have answers…I find it so frustrating not to be able to give people definite answers as there’s that need to help, I guess all everyone can do is to make an informed choice and go with their instinct and whichever doctor they feel most comfortable with…hugs for you & your daughter, and praying for wisdom with your decision
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Hey Charlene,
I was hoping others with shunt experiences may contribute, but I must agree with Jules
Every situation is different and I am yet to hear 2 exactly the same scenarios on this journey. As I’m often saying to others “…2 people can have exactly the same surgery/ies but have vastly differing outcomes…”. Some people can bounce back really well, almost like nothing even happened. But for some there can be some issues. My personal situation is NOT a good example and the outcome has not been ‘as advertised’. And for these reasons you should always consult your medical team. They know your son’s medical history and would be taking his personal circumstances into account when making an assessment.
For me, initially, the plan was ‘Operate, fix, back to normal’ only that didn’t happen for me. The surgery, a craniotomy, unleashed a plethora of side effects which sent my whole system for a spin. The medicos were telling me the symptoms were normal ‘post op recovery pain’ but instead of getting better, I was getting worse. After 4weeks they decided to place the shunt. This did reduce the cranial pressures, but added to the weird sensory side effects ie visual distortions audio changes, problems with temperature regulation etc. Then I had issue with blockages, valve issues, shunt fractures. Some issues could be dealt with easily, others not so easy. I’ve now required 6 neurosurgeries and been told I’ll have more to come. Last year it was suggested I have another craniotomy because “Well, it might help” MIGHT!!! I declined. If it was a matter of life vs death, I’d have no choice, but to operate for a chance at ‘might help’, No. I’m not prepared to go through that again. I have a working device in my head now, why mess with it?
And that’s my choice. I know without the shunt I’d be dead, but the simple journey of ‘Operate, fix, back to normal’ wasn’t quite that simple for me.
Merl from the Modsupport Team
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Thanks for your time and all to answer my questions! Now I am curious Merl, if you had a Chairi decompression or maybe had your styloids removed? Your journey must have been extremely frustrating! I am sorry you had this experience! This is something we wish to avoid if we could! Sometimes I wish we could just wait two more yrs to do anything, in case there would be more data, case reports, statistics, ect. But it doesn’t seem like a good idea to do that either!
Rosa
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Hey @Rosa ,
My situation is a little different I must admit. I have a little nasty growing in my head which is putting pressure on the aqueduct causing the fluid to build up (hydrocephalus), hence the need for the shunt. So, technically, it’s not chairi, but rather, in my case, aqueductal stenosis.
Some medicos prefer a ‘Wait’n’watch’ approach. Some patients can have a partial blockage or a positional restriction ie when they laydown, the brain shifts within the skull restricting the natural drainage. Some patients can have an issue with the over-production of CSF (cerebral spinal fluid) or a lack of absorption of the fluid. This can be why Wait’n’watch is often suggested, so a ‘cause vs effect’ can be established. Neurosurgery is not something to be taken lightly and would not be suggested unless it was deemed necessary. I doubt very much that ‘more data, case reports, statistics, ect.’ would change the dr’s suggested approach. You could ask for a 2nd opinion (which is something I did. In fact I got a 2nd, 3rd, 4th and 5th opinion) but the reality can often be the same outcome. We produce around a pint (600ml) of CSF each day. Within the skull there needs to be a balance of brain tissue, blood and CSF. If one is out of balance, it doesn’t take long for those symptoms to overwhelm our bodily systems.
Hope it helps
Merl from the Modsupport Team
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