EDS, CCI, POTS… Eagle’s syndrome?

Good morning!

I’m so happy to have found this wonderful group, it’s been a long journey…

I’ve been diagnosed with hEDS, CCI and POTS over the past decade or so, but I had never heard of styloids or calcified ligaments. Dr Patel at MUSC and another doctor diagnosed CCI and retroflexed odontoid but didn’t mentioned Eagles. I had an MRI of the neck with contrast last week but not a CT with contrast although I do have a CBCT from a couple years ago from a dentist. I’m am attaching some images. Please share your experiences opinions, and I have asked my primary doctor for a referral to Dr Hackman since I am blessed to be in NC.

Thanks for your input :relaxed:




Hi NCardinal,

Welcome to our forum! from your images, I’d say you do have ES because your right styloid is long & very pointy at the tip. Your left styloid, though not long, is very thick. Thickness alone can cause problems as it narrows the space that the nerves & vascular tissues can occupy in that area. For the moment, I’m going to assume your symptoms are mostly on the right. Is that correct?

We have a number of members w/ EDS, CCI & POTS. They seem to go together w/ ES for some people. Our member @Snapple2020 has posted very helpful information for people suffering from these other health challenges so you should find interesting reading material here.

I’m glad you’re planning to see Dr. Hackman. He is a great surgeon & has helped many of our current members.

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Good morning Isaiah_40_31,

Thank you so much for your input, I didn’t realize the thickness of left styloid. Most of my neuro issues are when I turn to the right, but I have bilateral neck pain, and my vision issues seem to be in both eyes - it’s pressure/heaviness that occurs and makes it difficult to keep my eyes open. Not sure what that’s all about. I look forward to seeing Dr. Hackman, and I am so happy to have found this group. :smiley:

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Hi NCardinal! They’re not the longest styloids, but as Isaiah says the thickness can cause symptoms, as well as the angle they grow, & if they’re pointy. And if these are from a couple of years ago they could’ve grown more by now…
Have you read the section about common symptoms & possible explanations in the Newbies Guide Section? Head pressure & vision issues can be caused by vascular ES.
Glad that you’re able to see Dr Hackman!

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Hi Ncardinal,
It seems hEds and CCI run together. I have both and dealing with it for decades.Having had a couple whiplash accidents in my late teens, early twenties didn’t help with the CCI. I had my tonsils out at age 17. Removal of tonsils appears to be associated with alot of ES patients but not alot of data on it. I’m 65 now and had various bouts of pain flair periods in my neck and jaw and never knew about ES or hEds. I also have significant TMJ and osteoarthritis in my jaw which I have pretty much ignored most of my life. This all adds to neck tightness and pain. I have severe osteoarthritis in my elbows and had several hand surgeries for nerve compression by scar tissue there and elsewhere in my body - an hEds thing. Osteoarthritis seems to worsen and/or develop early in hEds. Had I known all this in my early years, I may have avoided some of the high impact sports and putting myself in position of overstretching my joints. I started having ES symptoms in 2015 and wasn’t diagnosed until 12/2019. I had ES surgery in April and Dec 2020. Ive now moved on to deal with my TMJ issues - I think caused my chronic neck instability. Ive gone thru multiple rounds the past 2 years of injections of botox, steroids and nerve blocks in my neck and jaw which seems to help. Had proliferon injections in my late 30’s. Alot of years of chiropractic and massage. Finding a good osteopath that is skilled in cranialsacral manipulation has been my go to’s.
My daughter has hEds and POTS. She is now 26 and been disabled from it since age 13. I just took her to my TMJ specialist and had a Cone Beam CT and had him pull it up so I could take a look at her styloids. For now they appear to be ok. The technician said elongated styloids dont seem to be a problem for patients. I educated him on Eagles syndrome.I am not sure how old you are. Recent data shows about 50% of POTS patients have hEDS. Most of the people I know with hEds have low blood pressure and have POTS/dysautonomia symptoms. My daughter has low blood volume which explains alot of her symptoms. Alot of long haul COVID people are being diagnosed with POTS. There has been some evidence to suggest POTS is triggered by a virus. In the case of long haul COVID, it does make one wonder. It seems some with ES have some dysautonomia symptoms assumed to be related to possible vagus nerve impingement?
Have you been evaluated for vascular POTS? There is also vascular related eds too. My daughter has a full heart workup to rule out vascular type eds and POTS. There is alot of cross over to all these medical conditions. Its a bit tough to sort it all out. Not many docs can handle the complex nature of it all.
It sounds like you are in good medical hands which is 75% of the battle.

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Wow, I’m sorry to hear that you and your daughter have been dealing with all this for a very long time. I’m 52 and have been dealing with spine issues since I was 35. I’ve had POTS for about 7 years now, after a lumbar fusion. Also have extreme abdominal distention, thought it was related to my kidney that falls into my pelvis when I stand up but I had surgery to correct (nephropexy) and the distention is still there. I am grateful the “bouncing” feeling in my abdomen is gone though. Thank you for sharing your experiences, and I wish you and your daughter improvements in your pain and disabilities. We must never give up hope of finding answers!

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Yes, it has been a long haul and it makes me sad sometimes because my daughter hasnt had friends really since the age of 13. She was taking some college classes which were hard for her to attend but it gave her some social connections. Since COVID though, we are online only. We have been dealing with it so long, its just something we have become used to. We have 2 good POTS specialists that manage her and I do deep dive searches online to see what new (if anything) is on the horizon. I think COVID will bring about some more $$$ for studies which is hopeful. I am certain a fix will come down the pike hopefully in my daughters lifetime. I never give up the hope it will.
There has been more and more data to support a theory that POTS is triggered by a virus (autoimmune), injury or in your case an assault on the body medically. Its pretty clear that COVID virus (or illness) can trigger it and I suspect may be connected to genetics. A recent study in Australia with a drug appears to turn back on what gets shut down that causes POTS to trigger. Too early in studies to determine if the theory holds water. hEds is genetic. Im keeping my eye on a genetic fix down the road and the advances in CRISPR.
I am wondering if your abdominal distention/kidney drop has to do with the hEds? I have a hiatal hernia that causes acid reflux problems and after my 1st kid at age 24, was told I was going to have prolapse issues (lax ligaments)…about 2 years ago, I had to have pelvic reconstruction and bladder suspension as it was all falling out. I just wish they could have done a tummy tuck at the same time. LOL

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I completely agree with you that COVID will bring $$ to research the correlation between POTS and viruses, injury or surgery. I hadn’t heard about the drug in Australia, very interesting and hopeful but I don’t know what CRISPR is?
The abdominal distention/nephroptosis is probably related to laxity in ligaments, I think there’s other organs that drop too but I can’t figure out how to have that diagnosed while standing up. I do have a barium swallow with small bowel follow through that looks like ptosis to me, but I don’t know anyone to confirm that since the radiologist here didn’t look at supine compared to upright imaging. Did your pelvic reconstruction and bladder suspension help? I had a rectocele repaired at the age of 45, but have a feeling there’s more to the story… I have had two tummy tucks without success, but that was before the nephropexy. Another surgeon suggested an abdominal mesh for support, but I don’t like the idea of having foreign object inside, I just had my breast implants removed en bloc 3 years ago. I have been able to work full time these past few months, which I hadn’t been able to do before the kidney surgery so I’m headed in the right direction. I hope you and your daughter are too!

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As for the bladder suspension success…I chose not to use the mesh also because of the bad press at the last minute. I had full pelvic reconstruction which included the rectocele and cystocele and suspension. Pretty much everything was dropping out of me. This was not a pleasant surgery and I had mismanaged pain issues after surgery. I was told my ligaments were in good shape to attach and hold it all up. This may depend on how stretched out your ligaments are and I imagine the severity of the hEds. I did chose to lose about 25 lbs prior to surgery as I had put on over the past 5 years which wasnt my normal weight anyways. I felt that I needed to do this to support a successful surgery. I had alot of stretch marks and sagging skin from have 5 pregnancies. I asked about a tummy tuck but that was a no go unless I wanted to pay for it out of pocket. I went to a new urogynolcologist who suggested I might need mesh at a future date. I have had a little more bladder leakage recently but I also have put on COVID weight. Personally I think staying on the thin side helps with this. The more weight you got on you, the more it will sag. After my bad experience with the surgery, I doubt I will go back in and get mesh unless the bladder leakage worsens. I would consider the mesh if the situation warranted it.

CRISPR is a gene editing system being used to modify genes to correct gene mutations. Its in its early days and somewhat controversial about safety but I do think it is the future of medicine.

Here is some info on the POTS drug: if you just google New POTS drugs, it will show up. My daughters POTS specialist really don’t have much comment on this just yet as it is a small study. Often these studies dont pan out when it is applied to a larger population. I do believe in the theory of genes getting switched on and off.

Drug discovered for Postural Orthostatic Tachycardia Syndrome (POTS)

A novel therapy, tested ex vivo , has been successful at correcting the dysfunctional body mechanism in Postural Orthostatic Tachycardia Syndrome (POTS).
Researchers have discovered how genes that protect against Postural Orthostatic Tachycardia Syndrome (POTS) become silent or ‘switched off’ and have identified a drug to switch them on again.

POTS is one of a group of disorders that have orthostatic intolerance as their primary symptom, in which a reduced volume of blood returns to the heart after the person stands up from a lying-down position. A main symptom in a third of POTS patients is fainting.

The researchers were from Monash University and led by Professor Sam El-Osta who said that while the gene implicated in POTS – norepinephrine transporter (NET) – had been known for several decades, past research had failed to find a genetic mutation responsible for the symptoms. This latest study revealed that the enzyme EZH2 is responsible for silencing this gene.

“We predicted that inhibiting this epigenetic condition could reactivate NET gene function and used a pharmacological drug called GSK-126 that could specifically inhibit EZH2 activity with dramatic results,” Professor Sam El-Osta said. “This is the first description of NET reactivation using a drug. The beauty is that it allows us to target very specifically the enzyme to reactivate gene function.”

Currently there is no specific pharmacological treatment available for POTS, which affects mainly people aged 18 to 50 years, 80 percent of whom are women. “We’re far from it now and clinical trials would need to be conducted, but the new drug could lead to better management of POTS,” he continued.

Professor El-Osta’s team conducted ex vivo studies isolating blood cells from patients with POTS who failed to respond to current treatments and cells from healthy donors. He said the sample group was relatively small: “Ideally, we’d like to see the work expanded in Australia and overseas and to have our findings validated.”

The study was published in the American Heart Association journal Circulation Research.

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