If you had a choice....?

[bombsh3ll]

I am not at the point of making this decision yet, & am not asking for advice but am very interested to hear what other people would choose;

A small but rapidly growing cohort of people with severe dysautonomia, most of whom have diagnosed or suspected EDS, have experienced dramatic improvement & in some cases complete cure, by undergoing craniocervical fusion surgery. The skull is lifted up, then fused to the neck in a rigid position, & the neck is also fused. There are variations on this theme but the procedure in my particular case would remove all movement in my neck, permanently. 

There is no guarantee the operation would help. Obviously this procedure is only performed where craniocervical instability is diagnosed, it is not done in the absence of this. (I have been diagnosed with cranial settling, which is a form of craniocervical instability). 

The mortality rate from the operation is 0-0.6% per the most recent research. 

Once I have definitively ruled out a spinal CSF leak as the cause in my case, this surgery is the next (& last as far as I can see for now) option to explore. 

So.. what would you do - permanently sacrifice ALL movement in your neck to have a chance (but no guarantee it would work) at being able to live again, or stay miserable syncopal & supine & not risk your neck?

B xxx

[haugr]

I wouldn't.  Too many non-gaurantees for such a permanent decision IMHO.

[yogini]

2 hours ago, bombsh3ll said:

I am not at the point of making this decision yet, & am not asking for advice but am very interested to hear what other people would choose;

A small but rapidly growing cohort of people with severe dysautonomia, most of whom have diagnosed or suspected EDS, have experienced dramatic improvement & in some cases complete cure, by undergoing craniocervical fusion surgery. The skull is lifted up, then fused to the neck in a rigid position, & the neck is also fused. There are variations on this theme but the procedure in my particular case would remove all movement in my neck, permanently. 

There is no guarantee the operation would help. Obviously this procedure is only performed where craniocervical instability is diagnosed, it is not done in the absence of this. (I have been diagnosed with cranial settling, which is a form of craniocervical instability). 

The mortality rate from the operation is 0-0.6% per the most recent research. 

Once I have definitively ruled out a spinal CSF leak as the cause in my case, this surgery is the next (& last as far as I can see for now) option to explore. 

So.. what would you do - permanently sacrifice ALL movement in your neck to have a chance (but no guarantee it would work) at being able to live again, or stay miserable syncopal & supine & not risk your neck?

B xxx

A risky surgery with neck damage and no guarantee for improvement doesn't seem like a great option. If other people are starting to try the surgery, it might be better to let them be the guinea pigs.  Not sure how long you've had dysautonomia but most people do improve over time.  It takes trial and error - sometimes years of trying different things - but surgery isn't your only option.

[Pistol]

@bombsh3ll - this is a decision solely between you and your doctor. It is an individual choice that cannot be made for you - but only by you. 

[lieze]

Have you tried the neck brace and did it help?

if I knew I could live a normal life just no head movement I would probably do it

[bombsh3ll]

2 hours ago, lieze said:

Have you tried the neck brace and did it help?

if I knew I could live a normal life just no head movement I would probably do it

I have tried a brace & it didn't help, but it would not really be expected to with vertical instability only, as the braces provide no upwards traction.

I have the option of trying a halo brace, but my surgeon doesn't feel they give the best idea of postsurgical results (although some do).

I would do it in a heartbeat too if I could know it would restore normal or reasonable cerebral blood flow & orthostatic ability!

It has been 5 years for me now, on the severe end & medication refractory, & I am inclined to agree with the consensus of the leading experts that severe adult onset dysautonomia seldom spontaneously resolves without identification & treatment of an underlying cause (where this is possible).

I think if a CSF leak can be definitively ruled out I will pursue this further. It is great to hear others' take on it, as I really value hearing different perspectives & there may be pluses & minuses that I hadn't thought of.

B xxx

[Guest KiminOrlando]

I was given this option and declined surgery for this reason. I can still drive but would lose it with surgery. There are people on here that had surgery with no relief of symptoms. Recovery/rehab was brutal. I used all this to decide that it probably wasn't a good risk/reward situation for me.

[toomanyproblems]

IIRC your onset of symptoms was related to a specific incident (blowing up a ballon?), and that subsequent to extreme valsalva during childbirth that you felt was involved. Why would those incidents be related to cranial settling, assuming that would cause a more prolonged course? 

I have EDS and it *looks* like my neck is sitting on my skull in such a way that this could be an issue. I had an MRI to detect Chiari but it was lying down, not upright, and they did not note it in the report. Even so it really looks hit or miss as to whether this surgery actually helps. Even if they had found it, with my own presentation and the present lack of knowledge on this problem, I don't think I could do it.

I feel for you with this choice and wish you the best in your decision.    

[bombsh3ll]

20 hours ago, KiminOrlando said:

I was given this option and declined surgery for this reason. I can still drive but would lose it with surgery. There are people on here that had surgery with no relief of symptoms. Recovery/rehab was brutal. I used all this to decide that it probably wasn't a good risk/reward situation for me.

Thanks Kim that is useful to hear. I cannot drive anyway due to syncope/presyncope, but can totally understand that being a concern if you can. 

 

18 hours ago, toomanyproblems said:

IIRC your onset of symptoms was related to a specific incident (blowing up a ballon?), and that subsequent to extreme valsalva during childbirth that you felt was involved. Why would those incidents be related to cranial settling, assuming that would cause a more prolonged course? 

Those are my questions exactly! - I still feel that a spinal CSF leak is the likelier culprit (as valsalvas are a recognized cause of leaks, & EDS is another risk factor) which is why I am continuing to pursue this avenue first although my MRI contrast was negative. Childbirth x3 didn't bring it on for me, it was just blowing up that dratted balloon, but it was a slightly different type of expiratory straining which is hard to describe as opposed to birthing (where the uterine contractions do most of the job).  The searing orthostatic headaches which most people on here don't seem to have also point towards this, as do the numbers of people on forums with diagnosed leaks who have/had lightheadedness, fainting & haemodynamic instability as part of their symptom complex. Many of them have been diagnosed with POTS and like me have had no response to POTS treatments. Stanford's Dr Carroll's youtube video on this is also very informative. 

I also do not see how that could have caused cranial settling, but there remains the possibility that it tipped something that was there but asymptomatic at that time into becoming symptomatic - or perhaps I would have started fainting soon afterwards anyway, maybe it was always on the cards.

The cranial settling is the only positive finding I have been diagnosed with to date (after very many tests) that is known to cause dysautonomia (via cervicomedullary syndrome) & is one for which there exists a treatment that has been shown to cure or substantially improve it in SOME patients (but by no means all). So for me, if I can eliminate a CSF leak & am left with only one diagnosis that is known to cause it, which has a treatment, I would probably go for it than sit there & do nothing when there is something that could potentially help. 

The symptoms of CSF leak and craniocervical instability are remarkably similar, & I really believe one of these two differentials could be it for me. 

B xxx

[bombsh3ll]

21 hours ago, KiminOrlando said:

I used all this to decide that it probably wasn't a good risk/reward situation for me.

PS Kim were you ever offered a trial of invasive traction (halo brace or Gardner Wells tongs) to help give an idea of whether surgery would benefit you?

B xxx

[Guest KiminOrlando]

No. I was given a regular neck collar that made things worse because it was too tall and kept my head in a position that put pressure on my brain stem. My muscles started getting weaker and I realized it was a bad thing to wear it.

[bombsh3ll]

3 minutes ago, KiminOrlando said:

No. I was given a regular neck collar that made things worse because it was too tall and kept my head in a position that put pressure on my brain stem. My muscles started getting weaker and I realized it was a bad thing to wear it.

I tried the popular Aspen Vista which is adjustable, and it didn't help me either - I understand collars are only really helpful for those with rotational and/or horizontal instability, not vertical. 

B xxx

[DizzyGirls]

I hadn't even read your post, but had asked my daughter your very question just the other day.  She is fused from C3-T4.  There was a possibility that she would come out of her surgery fused from C0-T4.  Thankfully, though, she had two amazing surgeons that didn't feel it was necessary at that point to restrict all movement in her neck.  They did say that it might be necessary later, but I know that they would only do it if her quality of life were so severely impacted (as it was before her surgery) that it would warrant it.  She suffers from dysautonomia, has EDS, too, but her most limiting symptom in all of this is her vertigo.  She actually just started wearing her Aspen Vista collar again for very limited amounts of time during the day to see if it would help.  It does, to a degree. But, in discussing the fact that the brace helps (a pretty good indicator that her neck is slipping) and asking her would she want to be completely fused, she said "no".  The reason for her is that she can adjust her brace to precisely the right spot that gives her the most relief, once you are fused, you don't have that option.  So, for now, that is her personal opinion on it. 

Best wishes in making the right decision for you.  Always remember everyBODY is different.  What is good for someone else, might not be an option for you and vice versa.

[bombsh3ll]

13 hours ago, DizzyGirls said:

She is fused from C3-T4. 

Thanks for replying, it is great to hear the experiences of someone who has actually dealt with this. I can understand her not wanting the upper segment done as this is where most of the neck's range of movement lies. Can I ask what benefits she gets from wearing the collar? The adjustable positioning being helpful makes sense - I think there is a common perception even among some surgeons that there is one optimal position for the neck to be in, which cannot really be the case as like other joints it is designed to move. 

One thing that concerns me about the fusion surgery is if I had it & it doesn't work & I am still fainting afterwards, what about the risk of damaging/breaking it, especially in the early months when the bones are fusing? Was syncope/seizures and protecting the fusion an issue for your daughter & if so how did you manage this?

Regarding the level of fusion though, what I have observed from reading published cases & speaking directly to a number of patients it has helped, is that only fusions skull downwards (ie occipitocervical) to various levels appears to cure or improve orthostatic intolerance/syncope, presumably as this is the level of the brainstem. I have not seen anyone recover whose fusion began lower than the skull (although obviously it can needed & helpful for other instability related problems pain/neuro etc).

I hope your daughter manages to find some relief from her vertigo.

B xxx

 

[Pistol]

12 hours ago, bombsh3ll said:

Regarding the level of fusion though, what I have observed from reading published cases & speaking directly to a number of patients it has helped, is that only fusions skull downwards (ie occipitocervical) to various levels appears to cure or improve orthostatic intolerance/syncope, presumably as this is the level of the brainstem. I have not seen anyone recover whose fusion began lower than the skull (although obviously it can needed & helpful for other instability related problems pain/neuro etc).

What does your physician tell you about these concerns?

[bombsh3ll]

16 hours ago, Pistol said:

What does your physician tell you about these concerns?

The neurosurgeon that I have seen was very honest about both the potential for, and degree of, improvement being impossible to predict in advance of surgery, however he only offers surgery where he believes it has the potential to improve someone's quality of life. He does however specialize in disorders of the craniocervical junction, particularly among patients with connective tissue disorders (ie atraumatic acquired instability) and the majority of those he performs this surgery on have symptoms of cervicomedullary syndrome - predominantly dysautonomia, some have additional neurological symptoms. 

We did not discuss my concerns about continuing to faint with a fusion if the surgery was not successful at my appointment, as I have not yet decided on the surgery but would definitely be raising this beforehand if I did. Obviously if there was effective medical treatment people would not be having surgery in the first place, so it must be an issue for those in whom the surgery doesn't work.

Some patients have much more marked radiological instability than I do, and also have seizures and/or breathing problems, so in their case the decision is more clear cut, it is a difficult one! I certainly intend to pursue exclusion of a spinal CSF leak to the nth degree before deciding on this route, as treatment of this is easier, less expensive & doesn't result in any long term loss of movement, & also my mode of illness onset (Valsalva) fits far more with this - although EDS is a risk factor for both spinal CSF leaks and CCI, and there are certainly patients who have experienced both. 

B xxx  

[Guest KiminOrlando]

For me, I have a slight Chiari, but they decided it wasn't impacting CSF and wasn't worth fixing. I also have the instability that the Neurosurgeon thought was worth fixing. He was planning to go in from the back to do the fusion because he believes it is a better result than going in from the front. I would have had the zipper scar, but my hair would cover it, so it wasn't a big deal. My neuro is Dr. Sunil Patel out of the Medical University of South Carolina. I think he publishes on PubMed if you want to read his stuff. He supposedly specializes in this.

[bombsh3ll]

13 hours ago, KiminOrlando said:

For me, I have a slight Chiari, but they decided it wasn't impacting CSF and wasn't worth fixing.

That's smart - many people have had innocent Chiaris operated on but didn't get better without coexisting instability being addressed - Drs Bolognese & Henderson term these the "complex Chiaris" where there is CCI as well, & I read a paper about the Chiari being what gets them in to see the neurosurgeon, but isn't actually the problem, then the CCI is discovered (if they are lucky). 

There have also been reports of patients with the appearance of a Chiari which is actually the result of a spinal fluid leak & the brain is sinking downwards. In these cases, when the leak is found & sealed, the "Chiari" has resolved. I do not have one, but my cerebellar tonsils are in the foramen magnum on upright scan. 

13 hours ago, KiminOrlando said:

I would have had the zipper scar, but my hair would cover it, so it wasn't a big deal.

I could probably cover it too but frankly I wouldn't care if my head looked like a second backside if it got me better . 

13 hours ago, KiminOrlando said:

My neuro is Dr. Sunil Patel out of the Medical University of South Carolina. I think he publishes on PubMed if you want to read his stuff. He supposedly specializes in this.

I think I have come across some of his material, he sounds good. What level did he suggest fusing you to?

B xxx

[Guest KiminOrlando]

You're right. The Chiari is what got me in his office, but when he listened to me and looked at the supine images, he sent me for the standing MRI with flexion. The local radiologist said they were inconclusive, but he read them and said he saw CCI and thought he could fuse only C2 & 3, but said he only goes in once and if he thought I needed more he would do it then or he wouldn't do the surgery. At that point it became a risk of not being able to drive. I'm not married and no real public transportation where I live. Not being able to drive would be catastrophic to my quality of life. Also, I have an autoimmune component to this little drama, so it was not clear that it would significantly help my situation. They still think mine is more autoimmune driven, possibly EDS III. I faint for several days after any anesthesia, and PT would start the next day. I just envisioned all kinds of problems since he isn't a dysautonomia doctor and lacks that understanding. 

[DizzyGirls]

@bombsh3ll - The collar helps give her muscles a break from the stress of keeping the upper half of her body upright.  Right now it's an Aspen Vista.  Her back is heavy and solid.  She wore a neck brace, not a cervical one, but a more cervicothoracic for several months after her surgery.  It's called a Minerva.  It has a front section and buckles and a back section with a ton of straps and buckles including one that wraps around your head.    You asked about fainting?  I have a story.....  The first time riding in a care without the brace, we went to SF to see her neurosurgeon.  On the way there, we were in a car accident.  I have never been so scared in all my life.  She screamed bloody murder from the pain, but thankfully we were heading for xrays anyway, and her neurosurgeon said everything was just fine.  Whew!!!!!  Her muscles were so rock hard from the spasms that she just couldn't wear her brace any more, so that's why she didn't have it on.  That's another thing you need to consider, too, the muscle spasms.  They are intense and can cause a lot of problems that you wouldn't think of as well.  

@KiminOrlando - What you said about anesthesia is sooooo true!  My daughter faints for several days after anesthesia, too.  And you are right, PT comes in the next day.  My daughter had a team full of very savvy neurosurgeons, anesthesia team was hand picked due to her dysautonomia, you name it, all the precautions had been taken.  Except for one.  PT.  They came in, I warned them that she would faint, to be ready.  But no, they weren't ready.  I think I warned them 5 times that she was going to faint, my daughter warned them too.  So, she fainted and they dropped her!  I kid you not!  I was so mad!!!!  They heave hoed her onto her bed and she blacked out more from the pain.  15 people came running in, tilted her upside down, blamed it on her pain meds, and gave her a dose of Narcan.  Thankfully, we were blessed with Dr. Grant, one of our wonderful neurosurgeons, and he immediately came in and gave the PT people a piece of his mind and that they had better be more careful with his POTsie patients! 

The moral of these stories is that things don't often go like they are supposed to.  Be well!

[bombsh3ll]

19 hours ago, KiminOrlando said:

saw CCI and thought he could fuse only C2 & 3, but said he only goes in once and if he thought I needed more he would do it then or he wouldn't do the surgery. At that point it became a risk of not being able to drive. I'm not married and no real public transportation where I live. Not being able to drive would be catastrophic to my quality of life. Also, I have an autoimmune component to this little drama,

I totally get why you made that decision. Single level fusions in EDS often end up exacerbating problems above & below & further surgeries later on are common. Also that level is too low to have any bearing on any brainstem pathology. 

13 hours ago, DizzyGirls said:

You asked about fainting?  I have a story.....  The first time riding in a care without the brace, we went to SF to see her neurosurgeon.  On the way there, we were in a car accident.  I have never been so scared in all my life.

That must have been really scary, I am glad her fusion was ok. Frustrating that the PT didn't listen post-op. The team in Barcelona where I would have surgery have a lot of dysautonomia patients so hopefully they would be prepared. How did you deal with fainting in daily life whilst it was healing? That is what worries me, if it didn't work I could knock it all out of place before it healed if I went down & hit my head or neck. 

B xxx 

[DizzyGirls]

My daughter takes Florinef.  We've had to change her dosing as of late, but when she can tell her bp is dropping (starts getting really lightheaded) then she takes a half a pill.  She was taking 1 0.125mg pill in the morning and that worked for several years, but lately it's been too much.  So, we've been doing half in the morning and as needed in the afternoon/evening.  When up and around, she used a walker to have something to hold on to.  I'm always with her, tho.  Do you have someone that lives with you that is a good help and could keep an eye on you for the first several days home.  Keep in mind, these are pretty big surgeries.  My daughter was at Stanford for 10 days and then at an acute rehab center closer to home for another 7.  If your surgery is as big as my daughter's, I'd definitely recommend the acute rehab as they taught her how to walk with her new "stack of bricks" on her shoulders.  It's heavy and really throws your balance off.  I'm glad you have some place lined up already that has experience in dysautonomia.  We found that the neurosurgeon who organized her surgery and did a fair amount of it knew more about dysautonomia than some of the others because he is a peds neurosurgeon.  He deals with kids, and who shows up with POTS?  Teens!  

Best wishes to you and I hope you find peace in whatever decision you make! 

[bombsh3ll]

Thanks. I would be in the hospital the first 10 days or so then a disabled adapted apartment with nurses on site for a couple of weeks after that. It is not so much being alone that bothers me, more the impact of landing on the fusion if I was still a fainting risk, although of course having someone with me would be helpful. 

The standard advice of "lie down when you feel faint" doesn't really help me, as I would spend my entire life supine, so what little I do, I do presyncopal.  

I will speak to the surgeon about this of course, but if there was medication to stop fainting nobody would be having the surgery, so I am not sure what his advice will be.

B xxx

[DizzyGirls]

@bombsh3ll- Just remembered this... one of my daughter's neurosurgeons hand picked the anesthesiology team because of her POTS.  I'm sure you've tried Florinef and Midodrine... they don't help you?   We asked our cardiologist about my daughter's bp, as her heart rate seems to be more under control.  He said that controlling the heart rate is much easier than controlling the bp.  You can bring bp up or bring it down, but the stability part is what is frustrating, he said.

[bombsh3ll]

12 minutes ago, DizzyGirls said:

one of my daughter's neurosurgeons hand picked the anesthesiology team because of her POTS.

That is fortunate & probably made the world of difference. I am lucky in that regard as the hospital in Barcelona have many patients with our issues.

I have tried both of those meds (and a number of others) but they didn't help me. My BP and HR are generally textbook normal, so wouldn't benefit from being manipulated. I believe I have low stroke volume as a result of hypovolaemia, hence low cardiac output. That's more difficult to prove, and to treat. Or, it could just be that the inflow/outflow to the brain is compressed as a result of spinal fluid leak or cranial settling. 

Did she ever faint post op, & was the fusion OK? I am guessing soft landings & possibly the brace are important there!

B xxx