Newly diagnosed with eagles, symptoms for over a decade

[Krixen]

I have recently been diagnosed with eagles syndrome. I have calcified elongaged styloids on both sides, although my right side is longer. I had a CT angiogram as the final diagnosis by a thoracic outlet specialist, but it originally came up during a 360 dental scan (image attached)

My story is long and full of doctors. At age 16 I had spinal fusion done for scoliosis curvature, t4-t11. Since then my symptoms really started, with headaches late in the day.

After years, at age 25 after symptoms getting worse with more frequent headaches, I had the spinal hardware removed which made everything worse.

Every day I would have a BILATERAL headache. I could have pain on both sides at the same time but its almost always only on one side at a time. The headache is front/back of my neck, to my eye, and even down my shoulder to my arm.

Ive been down CSF leak route, thoracic outlet, every pain option possible (pain doctors say nothing left to try). I have not worked since the spinal removal and i have to remain sitting most of every day.

I have an appointment with an ENT skull surgeon at mass General hospital next week, his name is Doctor donald Annino, hopefully he will hande it.

As far as eagles is concerned, it sounds like a plausible reason for my pain. My pain does get worse when using my arms and turning my neck. Going to PT with neck movements causes me to be out of commission for days.

I also have Elongated c1 transverse processes on both sides that could be a contributor.

[Krixen]

[Jules]

New users can’t post images initially, it’s a technical protection issue, but you should be able to now- sorry!

And so sorry that you’ve been in pain for so long, and that you have spinal issues too, that’s just miserable to have had all that for so long…

If you’ve not read the section about common ES symptoms & possible explanations in the Newbies Guide Section it’s worth looking at, as there’s detailed info about causes of some of the pain etc. Headaches can be from Vascular ES- if the IJVs are compressed then the pressure can build inside the skull (which can then cause CSF leaks), you can get other veins swell at the back of the head to take over some of the blood flow , which can also cause pain. The pain in your eye could be caused by carotid artery compression or trigeminal neuralgia, which also causes face pain. Sleeping propped up if you don’t already might help. The spinal accessory nerve can be affected by ES too, and this can cause shoulder and arm pain, and weakness.

Dr Annino has done lots of successful surgeries for ES, but there are doctors more experienced with Vascular ES, which it sounds like you could have. And he doesn’t do C1 shaves, which could well be necessary … So the best doctors to see for that are Dr Costantino in NY, Dr Lui in NJ, Dr Cognetti in PA, and Dr Nakaji in AZ. When you see Dr Annino, you could ask if he’s willing to order more imaging for you- a CT with contrast is the best way to look at the blood vessels, ideally timed to show both the arteries and the veins. Then you could take it from there and see what this shows, then perhaps see if you could get a referral to one of the VES doctors?

Looking at your imaging, you have elongated styloids both sides I’d say, the right looks quite angled too, and possibly thick especially at the top. You can’t really see whether the C1 processes are that close to the styloids from these though. Also your hyoid bone processes looks really curved upwards, sometimes that can be an issue as well and can cause throat pain and swallowing issues, and also it can come into contact with the carotid artery. Might be worth asking Dr Annino about that too?

I hope that you’re able to get some treatment soon- you’ve obviously tried lots of pain relief, have you tried nerve pain medications? If it does look like you have IJV compression, some members have found anti-coagulants helpful with that.

[Krixen]

Hey thank you the reply! I have not tried many anti coagulants. Im mostly on medications to increase my blood pressure for POTS which include: droxidopa, mestinone, and fludrocortizone. Can those make it worse?

Also interesting about the CSF leak. Ive had 3 blood patches, and 1 5x embolization. In each case, i had some temporary relief. Even some days with no pain! Can this be described also?

Also when laying down, i do get relief but i have to be laying down for quite awhile. I know csf issues are usually resolved quickly but it can take a long time for mine to help. Is this an indicator as well?

[Jules]

Certainly CSF leaks can be caused by IJV compression- it’s called ‘spiky leaky syndrome’ :

Spiky-Leaky Syndrome | The EDS Clinic

A cycle of pressure building, causes a leak, then low pressure…

@Snapple2020 is very knowledgeable about POTS, we do have lots of members with both conditions, she might know about the medications, also @Isaiah_40_31 posted this link about vascular compressions, dysautonomia, POTS which might be interesting:

An Overview of Vascular Compression Syndromes and Associations with Autonomic Dysfunction: A Review - PubMed

Low intracranial pressure, say if you’ve had a leak, can usually be better laying down, normally high pressure is better being more upright, but we have had people say it’s not been like that for them! I would guess if you feel some relief after a patch, the CSF stabilises for a bit which presumably is when you feel well, but then as the pressure begins to build again (if you do have IJV compression), then you start to feel more unwell…

[Isaiah_40_31]

@Krixen - I’ve annotated a few of your images so you can see what @Jules mentioned about the greater horns of your hyoid being quite curved though I can’t tell if it’s both greater horns or only the left one.

You’ve also lost the lordotic cervical curve in your neck which brings the styloids closer to vascular & nerve tissues. The curve can be restored but it takes patience & time & doing gentle PT exercises. Here’s a link to information about that. Some members have found it helpful to begin working on curve restoration prior to surgery but others found it exacerbated symptoms so chose to wait till after surgical recovery to start.

List of my favourite resources on YouTube to learn anatomy

I’ll update this list if I remember/find more. These people are academics/practitioners/physiotherapists and so on, none of the “I tried this magnetic bracelet and it did miracles!” personae. Sam Webster https://www.youtube.com/channel/UCMRHZcgwdSE_9wbwcP8aRJw The Noted Anatomist The Noted Anatomist - YouTube Bob and Brad Physiotherapists https://www.youtube.com/user/physicaltherapyvideo Brian Abelson https://www.youtube.com/c/kinetichealthonline The Visible Body (not the person i…

[Krixen]

Thank you so much for doing this! i have some images/video from my CTA. i don’t know how to give you the actual raw DICOM, looks like I need doc permission? But i remember my doc showing me where the styloids were and hopefully this is helpful.

[Isaiah_40_31]

@Krixen - I’m not certain enough about what I think is your right styloid to mark it, however, I have marked your right hyoid greater horn which also is upturned at the tip but not nearly as much as the left side. I wish I could pause the video so I could get a better look at what it’s showing, but it’s dark enough & moving fast enough I can’t focus on anything in particular. Do you have any other images of your right side that aren’t so dark?

[Krixen]

Hello again, I am posting slower gifs (only way i know to share videos on here) and I pointed out the styloids on two frames. The thoracic outlet doctor pointed these out to me as the elongated styloids.

I am also attached a slow video and picture of the other side. I wish i had a better way to send these to you.

[Isaiah_40_31]

In this picture from the earlier set of images you posted, I’ve approximately marked your right styloid. It ends very close to your mandible & may even be touching it. We’ve had a couple of members who’ve a styloid actually fuse to the mandible. I can’t see yours well enough to make that call.

Even w/ the videos slowed down, I can’t get a still shot of anything that looks significant. I think the center one in the bottom row of those in your post above may show some IJV compression, but again, I can’t be sure.

When is your appointment w/ Dr. Annino? Have you had a CT scan or just the x-rays/MRI?

[Krixen]

The images here are from a CT angiogram. I dont have the DICOM links unfortunately, it seems like mass general only lets you pass those onto other doctors unless you know another way.

I have my meeting with Dr. Annino on Monday but we also called scheduling with Dr Constantino in New York and we are thinking that this is the right doctor for the job considering all that is going on here.

Do you know of a way I can send you the MP4 video? Perhaps a DM with a good drive link?

[Isaiah_40_31]

I’m glad you have another appt w/ Dr. Annino & Dr. Costantino is a good choice with the symptoms you have. You should also consider getting an opinion from Dr. Liu in NJ. I’ve answered your DM & annotated some images for you.

[lsheep]

@Krixen I just want you to know, you are entitled to copies of any medical records (including any tests) by law. Just wanted to pass that along .

[Krixen]

For anyone coming here later, i mistakenly claimed C1 was elongated but its actually C7. My mistake

[Krixen]

Hey all, got my DICOM images and put them into “microdicom” and 3D rendered. This shows just how long the styloids are. Interestingly, the one on my right looks to be broken? is that normal or is there likely something blocking the view?

[Isaiah_40_31]

@Krixen - It does look like your right styloid is broken. We’ve had members with broken styloids but infrequently. Elongated styloids can break from things as simple as laughing & coughing. Being broken doesn’t necessarily mean your symptoms will be worse. The broken section is held in place by the neck muscles so it’s basically not extra mobile. The left styloid has quite a downward curve & is very pointed. Are your symptoms worse in one side than the other?

The other thing I noticed is the greater horns of your hyoid bone look longer than normal. I’m sorry I’ve forgotten whether you have clicking & pain when swallowing? If so that could be a sign that they are contributing to your symptoms.

[Krixen]

Symptoms are bad on both sides but they switch sides. Almost never on the same side twice.

Symptoms are head, neck, shoulder, even arm pain.

Since my symotoms are always on one side at a time and it switches, i can sometimes not tell if one side is just worse and it makes the other side feel less in comparison.

Seemingly unusual symotoms, idk if ive found anyone like me before

[Jules]

We have had members who have pain both sides, or one and then the other ramps up…

[Krixen]

What about one whose it switches sides. I feel like this is the strangest symptom. It can switch multiple times a day and i know when its happening. But perhaps it is a comparison problem and its not switching, wish i could tell.

Dr annino at mgh seems to think this is a bigger clue for nTOS than it is eagles/neck compression

[Jules]

We’ve had members with cross-over symptoms; some have had symptoms just one side, which didn’t go when they had that side removed, but improved when they had their second side done, the side opposite where the pain was! So there are weird symptoms/ effects with ES which can’t always be explained… I can’t say whether your pain is from ES or from TOS, but ES is a strange beast!