Vascular Compressions {Part 2 - Treatment}

If you haven't read Part 1 or know what vascular compressions like MALS, SMAS, NCS, & MTS are, please read it first. If you are curious about how I was diagnosed and what symptoms I have, you can also read that here. I've held off making a post about treatment for the vascular compressions until I actually had a treatment plan and because there is just so much information, research is constantly changing, and I keep learning more and more that I didn't know beforehand.

So what can you do if you have them? What is treatment or options?

The controversy and why the news of the doctor I hoped would help me is no longer an option was so heartbreaking is that him and a German doctor look and do surgeries on the compressions as a whole instead of each one as a separate problem. They believe more compressions develop when the previous ones aren't treated. If you fix the compressions and blood flow at the top of the aorta (see above picture) with the MALS or SMAS, then the compressions below a lot of times will fix themselves without further surgeries. This is why I originally held off on jumping to do a stent or auto transplant for the MTS and NCS. What I have also found from others' experiences is that they might do a kidney auto transplant and get relief for a certain amount of time, but then the pain returns or even before that, the digestive pain begins, leading to a MALS or SMAS diagnosis and needing another major surgery. On the other hand, you don't know if someone who has multiple compressions with EDS just has the problems so bad that each one needs to be fixed anyway and that it will be an ongoing upkeep of our faulty veins and arteries.

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It is so difficult to know the right thing to do for a person who does their research and talks to others. Those who do not, unfortunately end up with 3 or 4 surgeries that may have not fixed or made things worse because the doctors who treated them didn't even understand. The biggest fear for all of us is that something gets missed or overlooked because it happens, it happens a lot. The problem also is that we can't just wait it out or decide not to do surgery. Those risks outweigh any other risks of surgery because of organ failure, stroke, blood clots. A big topic in our support groups is the damage to the organs, for example non alcoholic fatty liver like I have, and possible pancreatitis or kidney infections that we are looking into. The picture above shows how the organs and blood flow are affected with these conditions.

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Ok let's get down to specific treatment for MALS & SMAS. There's usually two options - open or laparoscopic. A lot depends on the doctor and their preference. Laparascopic is obviously an easier recovery, but there is some that say it's harder to see doing it that way, which can be easier to miss cutting enough of the nerves or Median Articulate Ligament and can reattach leading to another surgery. Another dispute among surgeons in the US is that some believe it's only neurogenic (nerves) and others believe that it's vascular or ischemic (blood flow) that's causing the pain and symptoms. That is another way that someone may have surgery only releasing the ligament, but need the nerves, ligament, and artery all trimmed or addressed so they will then need further surgery. A lot of doctors also require a celiac plexis block to see if a patient will get relief as a diagnostic tool before they agree to do surgery for MALS. For SMAS, the most popular surgeries are a duodenojejunostomy or SMA transposition, which you can read about both in further detail here.

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For Nutcracker Syndrome (NCS) there have been misled attempts to do a stent in the renal vein, which has caused a lot of people issues and death. When you start to research or join the Facebook groups for this syndrome, you usually learn that you will avoid this treatment at all costs because of horror stories. My cardiologist actually told me when he diagnosed me never to do this. Some doctors have been testing the water with doing an external PTFE graft that is sewed in, which is more popular and successful in Germany and China. The other successful surgery, although the most invasive, is the kidney auto transplant. The surgeon will take one of your kidney's (I believe it is usually the left) and attach and place it in your pelvis. Like the MALS & SMAS surgery, some doctors perform laparoscopic and others perform open. A surgery choice that is most drastic, but also gaining popularity because it has given relief to patients has been nephrectomy, just getting rid of a kidney completely.

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Now with a serious open surgery to fix these compressions there are risks in itself - bleeding, organ perforation, bowel obstructions, hernias, and because of EDS, we are even more likely to form scar tissue or have veins and arteries collapse that need a stent to stay open. I've heard a doctor describe the insides of EDS patients like cottage cheese. So even if the compressions are fixed correctly after the surgery, and with no fault of the surgeon or patient, our bodies may not heal or adapt like we hope and need further surgery.


If you have stayed tune until this part you may ask "Why did you now decide to stent your MTS?"

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Because I finally found a vascular surgeon who
a) I finally trusted
b) his knowledge of EDS & POTS
c) his experience with actual patients (a lot of college students who he gave them their life back), and
d) told me we needed to get blood flowing again with my iliac vein and to do it soon before I did develop a blood clot, which is when most of the time his patients ended up coming to see him after it was too late.

*If you were like my husband who knew how anti-stent I actually was before this appointment, you would be pretty curious how he was able to change my mind.

Besides just having peace from God about it (which I'll share in a future post), he also had no problem doing an angiogram to check blood flow of the NCS, SMAS, and MALS next if I was still having pain after the stent, but felt that it would be best to try least invasive surgery first. Especially if it gives me pain relief in the meantime. Even after reaching out to other friends who have EDS and the multiple compressions and surgeries, they agreed they would've still done this surgery first. You hate for anyone else to have these vascular compressions or have to go through any of this, but I'm very thankful for the ones that have gone before me and want to share their experiences and expertise to help others.