Worsening Headaches Post Surgery + Abnormally Small Veins Found

[Melodious21]

I finally had my surgery on 12/18/23 with Dr. Constantino. I was going to go with Dr. Hepworth and did travel to Denver for the initial visit. Dr. Hep thought I might have a small crack that could be a sinus CSF leak as well but he didn’t sound 100% on that. Dr. Constantino didn’t see any evidence of a CSF leak but was initially willing to test for it. However, when I got there he convinced me not to because it would involve a spinal puncture. He felt this would be a lot to recover from in addition to the surgery and I agreed with him since my experiences with getting a CT myelogram and blood patches caused me way more pain and problems. He said if I truly had a sinus CSF leak, I would have a lot of drainage from my nose, which I don’t. I also chose Dr. C because my family lives in NY so it was way easier for me to recover there. I’m glad I made that decision because my recovery has been brutal. I was able to stay with my family for 7 weeks and not deal with the stress of traveling back home to Oregon too soon. Dr. C has also been so kind and attentive. He was way easier to get in touch with than Dr. Hep and spent a lot more time talking to me (although I did really like Dr. Hep as well and realize he’s got a very busy practice). I do highly recommend Dr. C though. His partner who also participated in my surgery, Dr. Tobias (a neurosurgeon) is excellent as well.

I had elongated styloids on both sides as well as vascular compression on my internal jugular vein. It was equally bad on both sides but we decided to start with the left since my symptoms are generally worse on that side. After surgery they said the anatomy inside my neck was all smaller than it should be and is a genetic anomaly. Like having the anatomy of a 12 year old inside an adult’s body, which is pretty ironic given that I started having headaches at 13. They said my jugular vein and spinal accessory nerve were 1/4-1/3 of the size they should be. My styloid was compressing the jugular vein but that was only part of the problem given how tiny my veins/nerves are.

Apparently it explains all the compression I’ve had because there wasn’t enough space for everything. They did their best to take out and decompress what they could to allow the vein to widen and flow more freely, but they still couldn’t get it to a normal size. The original diameter of my jugular vein was around 1cm and they were able to widen it to 2cm but the average size is 3.5-4cm. So it sounded like the biggest problem was just that my veins/nerves are too small in general and there’s only so much they could do to try and fix that. I’m curious if anyone else has had this come up for them? Dr. C seemed pretty surprised by it and said he’d never seen it before. I also have Ehlers Danlos so maybe it’s related to that?

There’s a good chance they’ll have to do the right side at some point because it’s just as small and compressed, but they were really hoping I’d still get some relief with what they did on the left. I can’t imagine going through this again though. Recovery has been a nightmare. I’m still having a lot of nerve pain around the surgical area and my ear. I’m guessing because the nerve they had to sever to get access to the area is regenerating since I’m getting feeling back in my ear. That I can deal with though, what I can’t manage is that the headaches are worse than ever. They’re now 24/7 and way more intense than before. I’m completely debilitated by them and can barely be out of bed most days. I’ve lost my job and can’t drive anymore. I’ve never experienced so much agonizing pain in my life and that’s saying something as I’ve spent most of my life in pain. Has anyone else had this happen to them after surgery?

Dr. C thinks maybe I have craniocervical instability and that the surgery aggravated it. He’s wanting me to be evaluated for this but there doesn’t seem to be a lot of options for doctors who specialize in this.

I just don’t know where to go from here. It’s been almost 4 months since my surgery and the headaches are so unrelenting and so much worse. It’s to the point where I don’t know how much longer I can go on in this body, and I’m someone who really wants to be alive and find a way to keep going. I don’t know why the surgery made the headaches worse or how to get out of this terrible flare.

Dr. C doesn’t want to discuss doing the surgery on the other side until we figure out why the first one made things so much worse. I’m certainly not ready or wanting to do that yet either given how awful I feel. I’m just wondering if anyone else has had their headaches worsen after surgery and figured out why, or how you found relief? I’m also curious about my smaller veins and nerves and how that could be affecting me. I haven’t been able to find any info on this. Any insight, info or support anyone can offer would be greatly appreciated.

[Chan]

Hi, I hope that you do have some degree of stability in your symptoms as you await solutions, and that you have people around you for support.

My thoughts, and one possible solution resulting from a PubMed crawl

I haven’t had surgery, I do not have EDS, nor am I familiar with the condition aside from a glance at a search result and noting that with EDS, the connective tissues in blood vessels are not as “strong and elastic”.

Just regarding:

@Melodious21

I’m also curious about my smaller veins and nerves and how that could be affecting me. I haven’t been able to find any info on this.

I could dig through PubMed for information on EDS and how that affects vasculature for you, if reading from a screen is not a huge problem in your state.

The A&P text that I usually refer to first has an incomplete but simplified diagram of the venous outflow of the head & neck:

I don’t recall the specifics of what’s incomplete, I’d have to dig through for recall.

An image search for “venous drainage of the head, neck and brain” could be interesting.

Regarding EDS connection, there’s a phenomenon called “collateralization” in vascular science or whatever. Tissues and their cells that make up the arteries and veins are kind of specialized to be really good at finding a way out when things change or whatever. Scans could reveal where your veins have expanded to compensate, or show that your veins are having trouble expanding, or I suppose where/how the ligaments or whatever connections between your skull and atlas (as per my brief glance at a Wikipedia page on CCI) or whatever are interfering with your veins. Conceivably, some way to move or some way not to move or whatever might be inferrable from this. My thoughts were isometric neck exercises, to sort of get the veins to get their shit together in “use it or lose it” fashion, but obviously, I don’t have lived experience with EDS. “Are the headaches responsive to light cardio” generally, is what I would wonder, and I’d maybe check YouTube or whatever for “EDS headache exercises” and see what comes up. Obviously, being very judicious and perhaps consulting with someone nearby or here.

@Melodious21

spinal accessory nerve were 1/4-1/3 of the size

I missed this part in the above reply. This is a stumper for me off the cuff… in terms of why this is the case, before even getting into what that means. What I do know is that the nerves, even while innervating blood vessels, are themselves surrounded by like a plexus of microvasculature, the details of which elude me. I looked it up only very recently. In trying to understand how EDS relates to the smaller nerves, I would wonder whether the smaller nerve caliber is due to the smaller microvasculature exclusively, or if maybe the smaller microvasculature is unable to nourish any wider nerves, or if the nerve tissue itself consists of significant amounts of connective tissue which for whatever reason also is supposed to expand in some capacity perhaps by whatever microscopic amounts of fluid involved in nerve cell function??

EDIT:

Also, just supplementing with a bit of salient A&P 101: all the hundreds? thousands? of types of tissues (basically cell lattices) in the body are categorized into just four buckets: muscle tissue, nerve tissue, epithelial tissue, and connective tissue. I’m not sure that I’ve encountered a real good philosophical reason for the last bucket “connective tissue”, which like randomly includes platelets for whatever reason and such. So finding out what tissue specifically is affected by EDS would be worth a look, so as to narrow the scope of consideration.

EDIT:

Also, not sure what “not strong” means, but I would assume this means “liable to thrombosis” or tearing or whatever. “Not elastic” maybe means higher blood pressure or something, not being able to expand as well.

Maybe even a visit to the ER or something for any acute vascular damage. People who don’t complain much maybe tend to over-suffer…

EDIT:

If you happen to be into meditation, I’ve got lots to say about that, but that could get complicated… Maybe find some way to ‘activate the relaxation response’ or whatever for vascular dilation or whatever… Maybe like Tai Chi or Qi Gong, which recently had some results for hypertension, with a mediating mechanism unknown…

But yeah, I hope you get what you need…

EDIT:

A perusal of PubMed through the research paper posted recently about the connection between intracranial hypertension and EDS:

Using the similar articles feature:

Similar articles for PMID: 38269002 - Search Results - PubMed

Here’s a salient title from that list:

Connective tissue, Ehlers-Danlos syndrome(s), and head and cervical pain - PubMed

It’s from 2015 and the abstract begins with:

Ehlers-Danlos syndrome (EDS) is an umbrella term for a growing group of hereditary disorders of the connective tissue mainly manifesting with generalized joint hypermobility, skin hyperextensibility, and vascular and internal organ fragility. In contrast with other well known heritable connective tissue disorders with severe cardiovascular involvement (e.g., Marfan syndrome), most EDS patients share a nearly normal life span, but are severely limited by disabling features, such as pain, fatigue and headache.

Limited by institutional or ‘gray hat’ access.

Also, from the first EDS-IIH article, perusing the references, I saw the title “Can Elevated IGF‐1 levels among patients With Ehlers–Danlos syndrome cause idiopathic intracranial hypertension?”. It’s citation number 14, so searching the article for “(14)” I see that the reference is for the sentence:

While the physiology underlying the unique linkage between EDS and IIH is still widely unknown, abnormal levels of growth factors, such as IGF-1, have been proposed as one possible cause (14).

The abstract of the article says:

We offer for consideration a possible association between hypermobility syndrome seen in Ehlers–Danlos syndrome and risk of potential development of idiopathic intracranial hypertension – mediated primarily through the effects of insulin-like growth factor-1.

Again, not readily accessible, but through institutional or ‘gray hat’ means. The article is from 2013/2015. There were some slew of papers on IIH ever since, but given that the referring paper is from just this year, I guess it’s not completely invalidated.

The intro says:

Idiopathic intracranial hypertension (IIH) – formerly known as “pseudotumor cerebri” – is a neurological disorder characterized by an increase in intracranial pressure in the absence of a brain tumor or other secondary disease process.The pathophysiology of IIH is controversial: studies have identified the significance of growth hormone (GH) as well as insulin-like growth factor-1 (IGF-1) in mediating the increase in pressure – although the mechanism behind this process is not yet clearly understood. Here, we offer for consideration a possible association between hypermobility syndrome (HMS) seen in Ehlers–Danlos syndrome and risk of potential development of IIH – mediated primarily through the effects of IGF-1.

Notably, GH and IGF-1 are produced by the anterior pituitary gland and the “liver and other tissues”, respectively, according to the A&P text here:

The article continues with a case report:

In June 2010, a 36-year-old non-obese female (body mass index [BMI] 18.5) presented with symptoms of a generalized headache for 8 months. She described the headaches as throbbing, worse in the morning hours, disabling, and exacerbated by recumbency. Moreover, the patient reported that the headaches were similar in nature to those she had experienced as a teenager following a lumbar puncture (LP) performed to investigate visual obscurations following an auto accident. With the current headaches, the patient reported experiencing some nausea as well as occasional double vision. She denied having any sensitivity to light, sound, or smell; she also denied having any symptoms of lacrimation, rhinorrhea, or conjunctival injection associated with her headaches.

…

… In February 2011, we started therapy with subcutaneous octreotide, based on several reports from the medical literature. The patient had a dramatic clinical response to this treatment – with elimination of her visual symptoms and head pain. …

So it’s this one case of a patient that they were doing lumbar punctures on multiple times to alleviate symptoms, when they finally somehow incidentally figured out something that worked. Wikipedia says that octreotide is a growth hormone inhibitor. Maybe worth bringing up with a GP or something…?

A blood test to confirm elevated IGF-1, and an injection of octreotide if called for, basically. Obviously, close reading advised. I’m not at all a medical professional.

The discussion section talks about why it might have worked. I’ll save that and a discussion of the “similar” paper for tomorrow.

EDIT:

Specifically, there has been evidence suggesting that IGF-1 receptors present at the level of the choroid plexus – the site of CSF production – are excessively stimulated in high GH-IGF-1 states (as seen in cases of acromegaly or HMS) leading to excess CSF production and thus increased intracranial pressure.

The choroid plexus, last I looked it up, was somewhere in the ventricles. They’re responsible for CSF production at like some really slow rate. CSF is basically like filtered through ‘the blood-brain-barrier’, though I forget what the general distribution of that barrier tissue is like… How that all comes together is on my shortlist of things to look into…

eg:

https://www.researchgate.net/publication/374614201_Neurosurgical_implications_of_the_Jugular_Vein_Nutcracker

In any case, do note that I’d be quite happy for this comment to be deleted after whatever useful information is gleaned, perhaps more carefully presented.

[vdm]

Btw there is also a thing called “neovascularization”. You might want to read about it too.

[Isaiah_40_31]

@Melodious21 - I’m so, so sorry your situation is worse since your surgery & that you’re bedridden now. That’s really discouraging. Have you seen a pain management doctor to help with your headaches? Have you been put on a blood thinner to see if that helps relieve your pain to some degree? Does sleeping/lying down w/ your head elevated help at all?

Dr. Fraser Henderson in Washington, DC, is the CCI doctor/surgeon Dr. Hepworth sends his patients to when he notes CCI in them. Dr. Henderson is widely known throughout the US & has helped some of our members in the past. I don’t know of anyone who is currently seeing him. Dr. Fraser C. Henderson Sr. – Metropolitan Neurosurgery Group

I know how all consuming a terrible headache can be but one that goes on 24/7 day after day… We have had members who did not rally well after their first vascular decompression surgery, but when the second one was done, the difference was night & day. I feel a bit remiss suggesting that getting your other side done might make a difference since you’re still in so much pain after the first surgery, but it might help.

I’ve been told that the collateral veins that develop to bypass compressed IJVs can also cause problems/pain. Has Dr. Costantino said anything about your collaterals contributing to your current pain?

Do you know which type of EDS you have? I know hEDS is most common, but there are other varieties, & perhaps you have a variant that is, in part, the cause of your current situation?

Even with your headache pain, it’s good news that you’re getting the feeling back in the area around your ear. I remember how painful some of my nerve healing was post op as it took nearly a year for some of my symptoms to fade.

I’ll be praying that you’ll get answers & for your body to heal & the headaches to end. I wish I could do more to help you.

[Brandy]

I have seen both surgeons. Dr. Hepworth pointed out my left jugular vein is small and both are oblong and abnormal. I consulted with Dr. Henderson recently regarding CCI. He says, based on my imaging, I do not have it. He is ordering additional imaging however.

[Brandy]

I have EDS as well.

[jsisto]

I’m so sorry that you were having so much pain post-op. Can I ask how you are doing now? Are your headaches any better?

[Isaiah_40_31]

@Melodious21 - It’s been awhile since we’ve heard from you. Were you able to find out the reason your symptoms were worse post op? If not, I hope you’ve been able to see a pain specialist who was able to help you manage your headaches. Recently we’ve been suggesting to our members w/ unresolved post op symptoms/pain that they send their scans to mskneurology.com & request Kjetil Larsen to look at them to see if there’s something obscure the doctors/radiologists have missed. Kjetil Larsen is astute at seeing things others don’t. I hope you are doing much better so don’t need a follow-up opinion.