Median Arcuate Ligament Syndrome (MALS)

Hi Kari, sorry it took me so long to get back to you. I have been busy with doctors and researching what that heck is wrong with me!!!Ha Ha. I am so sorry you are struggling with the pain again. I never had the problem of the weight loss. For my first surgery the doctor even said he wouldn't have suspected MALS because I wasn't loosing weight. I had always been skinny, but I had stomach problems for as long as I can remember so I think I just got used to it and adjusted. The energy is a huge factor. I am sorry.
I am still struggling as well. At my last vascular follow up my celiac velocity was up. It was 340 or around that. My numbers have been higher. My sma however decreased. I am weird. Nothing with me is normal. My SMA velocities go up and down. My celiac always seems to go up until some intervention. He said we could do a balloon again, but I thought we should wait. I have so much going on trying to figure out all this dysautonomia stuff. I have a "POTS overlap, whatever that is. My primary, who is amazing, says definitely dysautonomia and the pots is secondary to that and some mast cell issues. The problem is I have so many things going on there is no clear diagnosis except for MALS. I go back the end of Nov. for another ultra sound. I am not trying to be negative, but I will be shocked if it is the same or better. I am not sure what to do then. That will be the fifth time going in. I am still on the iv hydration at home. I do 2 liters a day, but I am still dehydrated. Nothing like I was though. I can't drink water. It makes me nauseous and hurts my stomach.
My daughter is getting married in June in Jamaica. Since we have no family and she always wanted to get married on the beach, why not. I am so scared I wont feel well. My goal is to be better, or at least able to just do the flight. We booked it so I have a few days of rest before the wedding. It is not a big fancy thing, but I want to be 100% for her. She was so close to my parents and I know it's killing me and must be her that they won't be there, I have to be strong. So that is my motivation.
Anyway, sorry I am going on. I hope you start feeling better. Is it your artery narrowing again, or the nerves or you don't know? I'm sorry. You are such a kind and giving person, you shouldn't have to keep dealing with this. I will be sending you some positive healing vibes. Take care.
Jill

Hi All
I have suffered from MALs for over 25 years, I will post my story at a later date. With regards to pain relief it is important that doctors get it right. As you can imagine after 25 years I've been through pretty much every pain med there is. Everyone reacts differently to the different opioids on the market and must be careful which they choose to use. For me Tramadol Hydrochloride was a nightmare causing nausea, wooziness, hot flushes, confusion . . ., however MST Continuous at the right dose was good. At one stage my pain was such that I was on 90mg of MST twice daily; I was pretty much out of it for months until I got used to it. With such a high dose there were other side effects; morphine does cause your blood pressure to be lowered. Mine was so low sometimes (70/35 on many occasions) if I ate a snack I would pass out for 30 minutes to 3 hours. @astaingegerdm mentioned Gabapentin, which is great if used in conjunction with Amitriptyline and a low dose of MST. When I was on this combination there was one beneficial side effect, the gabapentin caused me to put on weight. This combination did help with only a few side effects that were easily tolerated. My MALs is at an advanced stage now and my pain specialist has put me on Transdermal Patches. Out of all the pain medication I have taken this has helped the most. I am on 30mg patches which last a whole week, so no taking tablets everyday and no chance of overdose. I also find it gets to the pain far better than tablets.

Tramadol - great for short term pain relief over a couple of hours. If your pain is constant then you may find the pain relief subsiding long before your next dose.
MST Continuous - slow release over 12 hours great for those who are at the constant pain stage.
Oramorph - a liquid pain relief used ideally for breakthrough pain, used in conjunction with MST
Gabapentin with Amitriptyline - provides good neural pain relief ideally used with low dose of MST
Transdermal patch (Butrans or Butec) great for long term pain relief and those who have issues keeping food down due to vomiting.

Depending on what stage your MALs is at will depend on which pain relief will work best. Currently there is very little research into MALs with consultants looking at the condition with blinkers on unable to even agree on how the pain is caused. Most cases of MALs are only taken seriously when the patient gets to the not eating stage and loses a vast amount of weight. It is far more risky to undertake open surgery when the patient is under weight. I hope this has helped.

Hi Jill
I am so sorry you're still having problems with MALs, it's funny you should mention you were always skinny so there was not much weight to lose. I was the same, all my life I was skinny with abdominal pain on and off; maybe it is something we are born with that afftects our matabolism? (I wonder how many other MALs sufferers were slim in their youth?) You mentioned that your velocity is up again and they suggested a balloon; I think you were quite right to hold off. I have read a lot of talk about stenosis of the artery, has the consultant mentioned any dialation. I have stenosis of the celiac artery, but it is also dialated at the other end. I only mention this because my consultant advised me not to fly, which meant I missed my older brothers wedding which was abroad. It is a question you should ask your consultant. Your consultant seems to focus on the stenosis and blood flow. Have they looked at your Celiac Plexus which is a bundle of nerves near your Celiac trunk. Has that been affected by the ligament compression? There may be scaring/scar tissue on the celiac plexus which may be the source of most of your pain. Finally I'm guessing that if you had an angioplasty in the past they also performed surgery to release the median arcuate ligament from compressing the artery. I am new to Mayo so I don't know what procedures or pain relief you have tried. I put a post up about meds so if you have any questions please ask. Keep writing and posting as there are so many people in your situation that need the information.
Take care,
Mark

I was skinny all my life! I consumed a lot of milk shakes as a child to gain weight but that didn’t work.

Hi Kari
Over here in the UK I was told by my pain specialist that they are actively trying to reduce the amount of morphine they are prescribing to patients. It is good and bad, good because I think morphine is over prescribed by consultants that don't look deeply enough into a patients illness. I mentioned in a post that I was once on 90mg of MST twice daily which in hindsight was far too higher dose. It got that way because like you said I became tolerant and had to move up a dose. Why I say bad is because before I was diagnosed with MALs a couple of months ago, my pain specialist said the final stage is to put me on a spinal cord stimulator. He really pushed for me to sign up to this. I did my research and talked to people who had it fitted. If I went ahead that would be the end of investigating what was causing the pain and there were certain scans I could not have if I had the stimulator fitted. I was adamant that I wasn't going to give up finding what was causing this horrific pain so declined the stimulator until I had exhausted all avenues. I'm glad I did otherwise I wouldn't have had the angiogram which showed up the MALs. I know the spinal cord stimulator has helped many people especially those with nerve pain. The problem I have over here in the UK is MALs is even less known, it's taken 2 months for me just to get a consultation with a vascular surgeon. I am hazarding a guess that he knows nothing about MALs. I see him in 2 weeks.

I asked Jill the same question if the consultant has looked at the Celiac Plexus. If like me you have pain when you breathe in and out then (and this is my hypothesis) maybe your suffering from the hammer anvil action. The celiac trunk is trapped in between the median acuate ligament and the celiac plexus. The ligament acting as the hammer when you breathe and the plexus acting as the anvil. I am thinking after a long period of time the celiac plexus becomes damaged too causing scaring/scar tissue on the plexus nerve bundle. Amongst the research they believe that if you get relief from a celiac plexus block then you would be a good candidate for surgery to remove the celiac plexus nerve bundle which may offer permanent pain relief. I believe that MALs sufferers get pain from different areas, pain after eating maybe from lack of blood supply because of compression. Pain on breathing as mention before from hammer anvil action. Constant deep pain could be from the stenosis of the artery itself. That's why I say pain relief should be tailored to the stage you are at. I will post about food too and what has helped me to eat again and not lose weight. There is something you could try yourself which may tell you if it is the nerve bundle. Try a tens machine, if you get some pain relief then you may need to talk to your primary about investigating your celiac plexus.

Thanks for the reply, for me it was fast food. No matter how much I ate I remained skinny.

Hello, my name is Shawnee. I live in Houston, TX and was recently diagnosed with Celiac Artery Compression Syndrome after 2 CT scans, 2 Ultrasounds, and a lot of abdominal pain/nausea. Of course, I’m one of the “out of the norm” patients whose compression occurs when I inhale (not exhale). I’m 44 years old and I’ve been dealing with this for over a year which all started with GERD. The pain and nausea didn’t occur until just 4 months ago. I see Dr. Galvani here in Houston at the Baylor College of Medicine. He suggests robotic surgery to cut the ligament and remove some of the nerves. Has anyone else had this done? I was hoping to get a second opinion but having a hard time finding a doctor who is familiar with this particular medical condition. All information, suggestions, advice would be greatly appreciated. I requested to join the MALS PALS FB group today. Thank you all in advance for your help and support.

Hi Shawnee
Welcome to Mayo. From my research the procedure your Dr recommended has worked for many in the past. Is the doctor doing the surgery a vascular surgeon? May I ask if you have got to the stage of not eating yet. If you are lucky and they caught the compression early having the surgery may be beneficial. I feel the longer the condition goes un-diagnosed the worse the symptoms get. in many of the posts you will find they can not eat as the pain gets worse leading to them losing weight rapidly. I have my surgery consultation in 2 weeks, here are the questions I will be asking that may be relevant for you too:
1) What part of the celiac trunk has been compressed?
2) What is the mean systolic velocity of the celiac artery?
3) Have you as a surgeon dealt with celiac compression before.
4) If so what is your success rate? (how many patients are pain free?)
5) Will it be necessary to have an angioplasty or stent in the artery?

I am hoping your compression is minor and you don't need the angioplasty or stenting. If the answers to the questions are satisfactory then I wouldn't leave it too long to make your decision. The longer you leave it the worse you will get. I wish you all the best, please keep us informed.

Mark

Welcome, @ukmalsman. Mayo Clinic Connect is all about meeting other members who experience things similar to yourself, share information, and encourage one another – I'm so glad you've joined this group and shared some incredible insights.

It's interesting that you note, "Currently there is very little research into MALs with consultants looking at the condition with blinkers on unable to even agree on how the pain is caused." I agree – the diagnosis of MALS has been the subject of much debate.There are several factors (about the lack of clarity) that I thought I'd mention.

Because the cause of MALS is not clear and symptoms may persist even after surgical treatment, its diagnosis is considered controversial. The challenge lies in the fact that it mimics other GI symptoms. Therefore, MALS is considered to be a diagnosis of exclusion – every other possibility that might be causing pain must be ruled out before making a clinical diagnosis.

In MALS, the medial arcuate ligament compresses the celiac artery, reducing blood flow to the stomach/intestines; surgery, where the ligament is cut away from the artery, doesn’t always get rid of the pain and symptoms. Many surgeons reason that if compression of this artery was causing the pain and other symptoms, then eliminating the compression should get rid of it – which may not be the case.
Although, I've learned that it's become less challenging to treat, since surgical intervention now includes removing the celiac ganglion, which is the mass of nerves in that area (some call it the “solar plexus”) because pressure on the nerves may be causing the abdominal pain. https://pdfs.semanticscholar.org/d6b6/4ec69d1cd3ef5f02036362f960d3b0be0181.pdf

That being said, I'd also like to mention these very encouraging published studies that show the tremendous advancements taking place (imaging techniques, laparoscopic surgery), which would help in establishing diagnoses of this condition:
- Contemporary management of median arcuate ligament syndrome provides early symptom improvement https://bit.ly/2Rrfop4
- A predictive model for patients with median arcuate ligament syndrome https://bit.ly/2pDJQzN

@ukmalsman, if there is one thing I've learned from the members of Connect, including you, is that you should never give up until you find the right physician or the right treatment that can help you in the best possible way. I'm certain @kariulrich will join in soon, and I look forward to seeing more posts from you.

Hi Kanaaz

Thank you for the welcome and thank you for the links to studies, I look forward to reading them. I agree with what you say, but hope that consultants don't leave looking for MALs until the very last moment. It is the very fact that MALs is considered to be a diagnosis of exclusion and every other possibility that might be causing the pain must be ruled out that worries me. 10 years ago my symptoms became chronic, for the first 3 years I could barely leave the house and I looked like a skeleton. They went by that very premise and started ruling out other things. Over the 10 years I had numerous operations i.e they took out a perfectly healthy appendix hoping it would solve the problem; it didn't. In fact I got peritonitis which nearly ended my life. Then they took out my gall bladder hoping it would solve the problem. They also performed exploratory laparotomy and found nothing. After so many operations I ended up with Adhesions which they had to open me up for twice. Even up to a few months ago they were ready to discharge me to the pain specialist for a spinal cord stimulator, but I refused to give up. It was after talking to an consultant immunologist about my symptoms he recommended the angiogram which showed the compression. The CT report stated that i was not eligible for stenting or angioplasty. I can't help wondering if like @shawbing they had seen it at the early stage 10 years ago a minor operation to release the artery from the ligament would have provided long lasting or total pain relief. My concern is if consultants continue with that line of thinking they will keep catching patient at an advanced stage where major damage has been done by the ligament and possibly the lack of blood supply. Making any surgery useless further validifying their notion that MALs is a diagnosis of exclusion; a cycle that must be stopped. They should be looking at what symptoms should be present for them to immediately do an angiogram to look at the celiac trunk. In my view ruling out everything else first is being reactive rather than proactive. Just because they don't know the cause doesn't mean it is not a real illness.

Mark