Chiari Malformation and EDS

So many of us with Ehlers Danlos Syndrome have also been diagnosed with Chiari Malformation, brain settling, tethered cord, etc. As a newly diagnosed Chiarian (for lack of a better word) I was wondering who else is out there? I have met many of you via facebook and thought this would be a great way to meet others and offer support to each other. I am a patient of Dr. Fraser Henderson and trust him completely. He has spoken at the EDNF conference and will be performing my surgery when the time comes. Hope this small note finds everyone well. Looking forward to hearing from all of you!!!

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I'm out here, and also waiting for Dr. Henderson to do my surgery. He's one of the few who understands the significant spinal issues that go along with EDS.

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I have EDS III and have been dealing with severe neckpain, headaches, etc. as a result of craniocervical hypermobility. I have done every treatment known to man and nothing has worked. My MRI's have been negative, my flex/ext films show no subluxing, however, my most recent MRI now shows swelling in the occipitoatlantoaxial joints and C1 has begun fusing to my occiput. The surgeon who discovered this believes that this irritation, etc. is the result of the increased motion between these joints. Finally, something has shown up on MRI, and I have a surgeon who does believe that there is hypermobility there even though there is no physical movement of the vertebrae with flex/ext.

I have made an appt. to see Dr. Henderson, even though he is so far away from me. The surgeon at my hospital mentioned injections of steroids/lidocaine to the joints for diagnostic purposes as well as for pain relief and he also discussed possible fusion. I am a physical therapist, who is unfortunately on disability now - too much pain, too many narcotics making it difficult for me to stay awake and make it through the day. I hate the pain meds even though they do help some and I have a wonderful pain doc. who has since become very interested in pain associated with EDS. He even joined a focus group after meeting me. I want my life back, to some degree - I want to work and be able to function without having to take pain meds around the clock. I have already lost ~50% of my neck motion so I'm not really going to miss much as a result of the fusion.

Has anybody had this type of fusion for craniocervical instability? I also worry that if I ever receive a blow to the head/neck, that I may suffer severe spinal cord damage. One of my doctors today rec. that I wear my Aspen collar when I drive. I do have extra mirrors, and again, I've already lost a lot of motion already so I don't think I'll have much of a problem.

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If you don't mind, can you tell me what your symptoms are and specifics about your pain? All my problems seem to come from my neck and I can't seem to get help with it. Thank you.

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Sorry it's taken me awhile to respond. I basically have deep neck pain at the base of my skull that increases with the slightest of movements: such as when riding in the car and hitting a bump or pot hole. If I am in bed sleeping, I will often wake up in pain when my husband just gets into the bed. The shaking of the bed just from him getting into it is painful to the point that I will wake up. I will have pain just putting lotion on my face = I don't seem to tolerate any resistance applied to my head, even with my own hand! I can't stabilize my head.

When the pain is particularly bad, I develop pain the goes up into my head and behind my eyes. I get earaches and I feel like something is crawling in my ears at times. My balance is off and I get dizzy occ. Hope this helps you

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AlyGail & BelieverEM:

I have made an appointment to see Dr. Henderson in May. After years of suffering I have figured out how to stabilize my neck pretty well on my own, and I am not sure that it would be worth it for me to have surgery at this time. But I need someone to look at my neck and tell me what is going on and what I can expect. I also have dural ectasia.

I am curious to know what surgery he is planning for you. What does he do, exactly? Fusions? When you saw him, did he order fresh MRIs, and if so, where did you do them? How many days did you have to stay in DC?

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Do you have dural ectasia in your cervical spine? I don't believe that I have chiari, but I do have other problems, including scoliosis and dural ectasia in my cervical spine. I consider surgery to be an absolute last resort. What do you do to stabilize your neck?

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I am also interested in what surgery he has planned for you. Surgery is a last resort, it's just difficult to know/admit if you are really there. I'm on pain meds all the time, and I need to lay down and/or take a nap everyday. This keeps the pain more manageable. Resting my neck helps overall. I took my son and his friend to the museum today (we were out for ~5 hours) and I needed to lay down when I got home.

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I don't know if I have dural ectasia in my cervical spine or not. I know I have it in my lumbar spine, but I discovered this somewhat accidentally. I had been seeing a spine doc locally for injections, etc., and he never mentioned it. I don't know if he even knows what it is. Then a neurologist at Mayo pointed it out on an old MRI I had brought in with me, but he didn't tell me what it was called, and he said he thought it was protective (!). I think he meant that it was cushioning the herniated discs. Once I did a little research and figured out what it was called I demanded that my geneticist at Mayo, who is also a neurologist, look at the MRI and tell me whether I had it or not. When she saw it she practically rushed me to cardiology for an echo. She also ran a genetic test for Marfan, which was negative. Mayo did a cervical MRI five years ago and no one mentioned it. I have since had other cervical MRIs done locally, but I don't think anyone who knows anything about dural ectasia ever looked at them. It has been very frustrating. In addition to what I think of as mechanical neck pain, which seems to come from moving my head around too much or raising my arms, I have also been having these bizarre crises that appear to be triggered by vasodilation (from heat or exertion, mainly). It's a long story, but I suspect this has something to do with the dural ectasia, because in warm weather I can hardly walk. I also get a sensation of pressure all the way up my spine, and if I can't stop it I go into a tremendous migraine. Luckily I discovered that it was much less likely to happen at higher altitude, so I am moving to New Mexico. Is this from the dural ectasia? Sometimes I take acetazolamide during a crisis if I am in New York, and it sometimes cuts it short. Acetazolamide is used to treat dural ectasia (among other things). Of course, I had to figure that out myself, from Wikipedia.

Do you know much about dural ectasia?

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I have it in my cervical spine, and it is wearing the vertebrae away, though I am unclear about how dangerous this is or will become. Basically, as I understand it, dural ectasia is a weakening of the dura--the membrane that surrounds the spinal cord and brain. Spinal fluid pulses up and down the spinal canal and in and out of the brain constantly. Normal people have a strong membrane that holds this fluid in--like a garden hose. We have less of a garden hose and more of a balloon. This is an exaggeration, of course. But, a thin dura allows the bones of the vertebrae to be eroded. A thin dura can also leak.

Dural ectasia in the lumbar spine is very common in Marfan, which is why they tested you for that. Mine was picked up by a competent radiologist, who didn't call it dural ectasia--he described it, and I called him to verify that was what he meant. He told me that he didn't use the term because no one was familiar with it (except me, the patient!). My neurologist had never even heard of dural ectasia--as the radiologist predicted--and became very defensive when I questioned him about it. While I know I have it in the c-spine, I don't know if I have it in the l-spine. Another radiologist did not see it there, but I don't know how competent he was. It is unusual to have it in the c-spine and NOT have it lower down.

I have neck pain constantly, and have had it for many years. Yes, moving my head makes it worse, like you. When it gets really bad, it goes up over my head and my eyes tear, down my arms, etc. My peripheral neuropathy gets worse in the warm weather--feet feel like they are asleep, but I don't get "crises." Things come on pretty gradually, and each new pain and symptom becomes my new normal.

Swimming helps.

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Yes, your description matches what I have learned about dural ectasia, mostly from reading Marfan literature. Has anyone ever proposed that you get a dural reduction? I have seen papers where they have surgically reduced the dural space to relieve headaches, but I don't know what the long term effects of this would be. Are you sensitive to changes in barometric pressure? I am noticing that even when it isn't hot, I don't feel well when wet weather sets in. More than the usual achiness, I get trembly and thirsty and disoriented, with numbness and tingling down my arms and legs, and my legs feel sluggish and unwieldy. I have difficulty after flying as well.

I avoid moving my head around much and wear a rigid cervical collar in a car or train. I don't drive. I also avoid using my arms much, especially raising them above my head. I try not to look up, which makes me sick. I wonder if this will get worse or just stay pretty much the same. I am hoping Dr. Henderson can tell me.

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I haven't followed up on it. I don't have a good neurologist, and was really not inclined to go to the chiari institute. I have family, friends, & doctors on LI who strongly advised me against going there. I am also terrified of surgery :) I'm afraid to open up the whole can of worms that a surgical eval would bring. I have been to see a spine surgeon who really didn't know anything about EDS, and he thought that surgery was unnecessary (which was what I wanted to hear). Of course, then he told me that the pedicles on my spine were "razor thin" in spots from the erosion. So where does that leave me?

My husband says that wet weather makes me worse, and, yes, plane travel is awful! Part of that is the dehydration, I think, which is never good for Potsy people. I do drive, but I don't like to drive far--just having my arms in front of my to hold the steering wheel is difficult as is turning my head. But, where I live, not driving is not really an option.

I also fear for the future. So, I just avoid thinking about it, for the most part, which is probably NOT the best strategy!

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P.S. Good luck with Dr Henderson!

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Hi all,

Where is Dr. Henderson? I have been to The Chiari Institute and while they definitely have administrative problems, I trust the neurosurgeons and they are well versed in EDS as well - referring patients to Dr. Francomano for same.

I'm wanting to get a second opinion, though, as to my surgery candidacy. Over the past few years my "anatomy" has remained relatively unchanged, but I am definitely have more problems because of the Chiari, Basilar Invagination, Cranial Settling, and Tethered Cord.

Thanks for any help anyone can lend!

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fyi, when I called Dr. Francomano's office last fall asking for suggestions on east coast re: chiari or cranial instability I was given the phone number for Chiari Institute by her office...

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Dr. Henderson is located in Bethesda, MD, ~1-2 miles from NIH. Check out the web: I think the address is not exactly correct, but if you enter this, you should be able to find more info about him. I am scheduled to see him May 4. I'm from Vermont. I hear he is excellent. Best of luck to you!!

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My daughter had chiari surgery and the cervical fusion May 2007. Dr. Bolognese and Dr. Milhorat of the Chiari Institute did the surgery. It changed her life. She went from being homebound to getting her GED and is now a college student. She still has POTS and has to watch her activity level but she is pain, nausea and dizziness free. 49% of the Institutes surgeries are redo's from other surgeons. I completely trust them. They do an extremely thorough set of diagnostics. My daughter's POTS doctor was very impressed with the level of detail. She ended up being in a car accident in October and had to go back to Dr. Bolognese to remove her herniated C6 disc and fuse her spine to the previous C1 to C5 fusion. Dr. B told us that people having the fusion now are only fused to C3 not C5 like my daughter was. Hope this helps

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I, too, am a "Chiarian". I was diagnosed with CM in April 2008. I have not had the surgery for this as my neurosurgeon thinks the chances of it bringing any relief to my headaches are about 20%. Too risky at this time. I was just very recently diagnosed with EDS. Type III for now, possible IV (waiting on the results from the U of WA for this). My NS is looking towards referrring me to Dr. Bolognese at the Chiari Institute because of this. I have a very long neck and it is not very stable. I had a discectomy and fusion at C6-C7 about two years ago due to a ruptured disk that was pressing against my spine. My NS hopes that TCI will not call for a craniocervical fusion as I would not have any mobility in my neck after that due to the previous fusion.

I'm so tired of doctor appointments. It took ten years to get the CM diagnosis. Now I learn that I have EDS. Oh yes, I also have a Tarlov cyst at T1-2. I think this is the cause of my left arm numbness and pain. My doctors are telling me this is not the reason; however I beg to differ. I am having another nerve conduction study done hopefully next week to learn more (hopefully). I have epilepsy as well. Occipital neuralgia too. I could go on, but that would belong under a different topic of discussion!

Sorry for my rambling! In answer to your question, yes, there are many of us Chiarians out here who also have EDS. Maybe there is a support group for CM/EDS-ers out there somewhere...


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Zumbro and EllyMae:

Is this what yours looked like? es/IS_case444.cfm

I would love to see the images if you can figure out a way to post them.

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I was dx with Chari Malformation before I was dx with EDS vascular type with POTS. I had many nero surgeries 15 last yr. Before I was dx with Chari I had a spinal fluid problem my CSF fluid was 5x higher than it supposed 2b. I had shunt after shunt. Then i had chari nerve decompression in Dec 2 days b4 Christmas. It's a hard horrible surgery.I woke up in more pain than I had ever had in my entire life. I do remember screaming in ICU. My blood pressure rose 2 stroke level. I was low my entire life. i could not move my right hand, and I still have trouble with my hand. With EDS we are slow healer, This surgery turned my life upside down. And my body is just starting to heal. I am still some days in bed due to pain and BP problems. Headaches, and vomiting are still a problem. Please research this and talk to an EDS doc not a surgeon. Thank God I am here and was finally dx with EDS or my nero surgeon would still b cutting on me to find out why I am and was so ill with pain so bad my Bp rose. Also it made me gain weight. I am still very bloated and swollen. The weight gain was hard on me first my hair was gone, then I blew up gaining over 60ibs in less than 6mo. EVeryone is different I dont want scare you. I just wish some one would have told me what bad things might happen. Again my body had been through alot. Just get a non sugeron opion. Good luck God bless.

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So I have EDS type III and trying to find a Neuro to even consider checking into chiari has been impossible!!! The Neuro that I saw said there is not correlation between chiari & EDS. I left his office and had a melt down cause I feel NOBODY will take me serious!! I have a lot of the symptoms but one thing is my headaches come and go. Also, I have never been woken up from a horrible headache. But if my headache is bad, I don't want to move at all. The headache always starts at the base of my skull and radiates to behind ear to temple and then really bad eye pain. I get sensitivity to light, sound and sometimes feel like I'm dreaming. My gag reflex comes and goes. I wish I could find a dr here in Houston that knows EDS & Chiari. If you were me, do you think traveling to Chiari Institute would be something worth while or not. The Neuro also said if I had chiari they would of found it before I turned 38 years old. Frustrated!!!

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