What is Median Arcuate Ligament Syndrome (MALS)?
What is Median Arcuate Ligament Syndrome? By the end of this video, you'll not only know that, but also how MALS is related to Postural Orthostatic, Tachycardia Syndrome and Hypermobile Ehlers-Danlos syndrome.
Watch the full video at the link below, or read on for the full transcript.
If you're a neurodivergent or a spoonie and are looking to master yourself and your time in a compassionate way, this is the place for you to be subscribe to my channel and hit the bell to be notified whenever I post a new video. I have MALS, POTS, and EDS. So let's dive in, shall we?
By the way, I've listed all of my research sources in the description box below. So the first thing we need to understand, and I've got my notes here 'cause this is not gonna work in a teleprompter situation, as I have already found out, we need to understand what the Median Arcuate Ligament itself is. It's something that connects the left and right sides of the diaphragm and it is a piece of connective tissue, which is very relevant for those of us with connective tissue disorders like EDS.
"The Ligament is formed at the base of the diaphragm where the left and right diaphragmatic crura join near the 12th thoracic vertebra. This fibrous arch forms the anterior aspect of the aortic hiatus." And there are two really important pieces of biology around there. And one of them is the celiac artery and the other is the celiac plexus.
The celiac artery is one of the largest arteries in the body and it supplies blood to most of the gut. The celiac plexus is the largest autonomic nerve in the body. It's part of the sympathetic nervous system, which is responsible for the fight or flight response. It innervates most of the digestive tract and this can not only cause pain, but possibly also the autonomic phenomenon often associated with MALS. And we're gonna get to symptoms in a little bit.
Simply stimulating the celiac ganglion activates the sympathetic nervous system. So a lot of us in this space know, for example, the vagus nerve in our neck is something that we can attempt to superficially stimulate in order to activate the rest and digest response in our body. The celiac plexus is the, the opposite of that. It's the fight or flight response. And we can also get in MALS sympathetic over discharge because of overstimulating the celiac plexus.
So now that we understand what the Median Arcuate Ligament is, we can talk about the Syndrome. So Median Arcuate Ligament Syndrome is where, "the Median Arcuate Ligament encroaches on the celiac artery and the celiac ganglia." It's a very rare condition and there are a couple of ways mechanically that encroachment can happen. Either the Ligament itself is lower, that it should be, or the celiac artery and ganglia are higher. Either way, they have come to meet each other in a compressed fashion that is no bueno.
Another thing that's important to keep in mind is that because the Median Arcuate Ligament is part of what attaches both sides of the diaphragm, this Ligament also moves with the movement of our diaphragm. So when we take in a breath and our diaphragm descends, the Ligament descends. When we exhale and our diaphragm moves upward, the Ligament moves upward and often it is the exhalation that increases the compression of the artery and the ganglia.
One of the notes that I have here, a direct quote, due to individual variations of topographic relationships of the participating structures, a spectrum exists from slight compression to complete constriction of the celiac artery. There's also some microscopic pathology that can occur. So there can be fibrosis of the Median Arcuate Ligament fibers. There can be an enlargement of the celiac nerve plexus after being compressed and basically aggravated over a long period of time. And there can also be a web of scar tissue within the wall of the celiac artery. Again, because of this ongoing compression and aggravation.
So what are the causes of Median Arcuate Ligament Syndrome? There are three basic categories. One is congenital, it's just how you're born. That's how your body was built. It can also be developmental. So for example, with growth spurts, connective tissue disorders and lordosis, I think mine was puberty onset. So for me it was developmental. The other thing that can happen is just an alteration of anatomy, either from abdominal or spinal surgery or abdominal trauma.
So now let's talk about symptoms. I, the symptoms are awful. So first off, you get persistent abdominal symptoms, which can include pain after eating, epigastric pain or tenderness, early satiety, indigestion, nausea, vomiting, constipation and or diarrhea. Why not have both? Gastroparesis, It can cause weight loss and or low body weight because sometimes if someone is unable to eat from pain, they eat less, therefore they lose weight. But that isn't true for everyone. You do not have to be at a low weight to get diagnosed for MALS, I am not at a low weight, but I have MALS.
And then you can also have autonomic symptoms from MALS because of how it affects the celiac plexus. So you can have pulse changes, AKA, the your heartbeat, you can have blood pressure issues. There's also something called a bruit, which is like a heart murmur. It's, it's what's happening in a heart murmur, but it's happening somewhere other than the heart. And it's essentially turbulent flow of blood through a narrowed blood vessel. And so that's what's happening when the celiac artery gets compressed. The blood makes a very particular sound going through that compressed artery and that's called a bruit. And it said that this epigastric bruit is heard in about 50% of MALS patients. So you don't have to have it in order to be diagnosed with MALS.
Another thing is exercise intolerance. In addition to POTS and EDS being comorbidities, you can also have hyper adrenergic POTS be a comorbidity.
I'm curious, before watching this video, had you ever heard of MALS before? Let me know in the comments below.
So we've talked about what the Median Arcuate Ligament is, what the Syndrome is and the symptoms. Now let's talk a little bit about how you go about diagnosing MALS. So usually there's a detailed history and physical exam laboratory work to rule out other causes of gastrointestinal symptoms. You have a abdominal color doppler flow velocity test, abdominal CT angiogram at end expiration. So like I mentioned earlier, because of how the diaphragm affects the Median Arcuate Ligament imaging may only show compression of the artery and ganglia at end exhalation.
This, this was my experience because I have had all of these symptoms since I was 14 in the year 2000. If anyone wants to date me as an elder millennial, I was diagnosed with other things or I was told, it was in my head. I got diagnosed with IBS, I got diagnosed with gastroparesis, I got told, it was all in my head.
I got told I just didn't wanna go to school. I loved school, boogers? Anyways, MALS is missed often because standard testing from GI doctors and cardiologists don't look at this Ligament artery or ganglia. It's like this black hole in the the field or specialty around abdominal pain. And it's really unfortunate and that's why it gets missed a lot. Doctors can think patients are faking it because gastrointestinal studies come up negative. That was me. And it's also possible to have incorrectly read imaging and also incorrectly done imaging. So for example, the exhalation in versus out is important when doing some imaging studies for MALS.
Another thing, and this is for those who have the POTS comorbidity like me, I was diagnosed with POTS in 2015 and I tried everything under the sun for standard treatment for POTS and I have treatment resistant POTS. It wasn't until I learned about MALS that I'm like, "oh, this might be why my POTS is treatment resistant. Because I have an actual mechanical issue where I have a Ligament compressing a really important artery and a really important nerve." One of the sources listed in the description below I, I just love how he shared that "the idea that POTS can have a lot of different root causes and we as a medical community can't get tired of looking for them." And I just really appreciated that and agree.
The way my diagnosis worked. I started, it was a simple like ultrasound of the celiac plexus area, but that came up negative. But I was determined, I was so certain that I had MALS that I kept going and they ended up ordering a CT with contrast with end expiration. And the picture that came outta that was like textbook for MALS according to my doctor. Then the next step after that, at least for me was do something I haven't mentioned yet, which is called a celiac plexus block.
And that is where if you wanna look it up, look it up. I don't wanna trigger anyone 'cause this was actually a very traumatic experience for me. I had two of them. The first one did not go well, but it's where they stick long needles into your back on either side of your spine and numb the celiac plexus. And if numbing, it eliminates your pain. That is further confirmation that you have MALS. My second one that was done, it went great. It went like it was supposed to and I was shocked at how the pain was gone. It was all gone. I could eat without pain and other things, but the numbing agent only lasted seven hours. So it's not a permanent treatment solution. It's more of like a will surgery actually help and that's what the celiac plexus block does. It helps determine whether or not surgery will help.
So now let's talk about the treatment. 'cause I just started talking about the surgery. So treatment is a surgical release of the Median Arcuate Ligament, which can not only resolve abdominal pain and digestive issues, but also autonomic dysfunction. More often than not these days it is done robotically, laparoscopically. There are still some doctors who do it as a full open surgery, which is, you know, slicey slice. You open, open, you like a book, do the surgery. But most specialists who do this surgery are doing it with a robot. So it's not even them inside you. It's a fancy robot that they're driving from a chair next to you. It's very fancy. It's also something you can look up if you wanna see those things. I have linked a surgery video below, but again, you know it's a surgery, so don't watch that. If you're not, just save yourself. But what's cool about doing it robotically is it's a lot easier to recover from 'cause the incisions into your abdomen. I think there's maybe three of them and they're really small, and so you have a lot less to recover from and you start feeling better right away.
I wanted to also include two studies that I found. Both of them, thanks to one of the surgeons I was trying to get the surgery with, and I have not had the surgery yet. My next video will be talking about that story. I'm gonna have my editor put up the abstracts from these two studies in some cards next to my head, so you can read those, but they're also linked in the description box below.
“Median Arcuate Ligament Syndrome (MALS) is a rare entity characterized by severe post-prandial epigastric pain, nausea, vomiting, and/or weight loss. Symptoms have been attributed to vascular compression (celiac artery compression syndrome, CACS), but it remains controversial whether they could be secondary to neural compression. Literature review identified rare description of pathologic findings in surgery journals. The clinicopathologic findings of four MALS patients who underwent robotic or laparoscopic surgery in our hospital are described. All our patients were female with a median age of 32.5 (range 25-55 years), and a median BMI of 23.5 kg/m(2). They presented with chronic often post-prandial abdominal pain (4/4), nausea (3/4), emesis (2/4), anorexia (1/4), and weight loss (1/4). Two patients had a history of Crohn's disease. At intraoperative exploration, the celiac artery and adjacent nerves and ganglia were encased and partially compressed by fibrotic tissue in each patient. In each case laparoscopic excision of fibrotic tissue, celiac plexus and ligament division and was performed; celiac plexus nerve block was also performed in one patient. After surgical intervention, symptoms improved in three of the patients whose specimens show periganglionic and perineural fibrosis with proliferation of small nerve fibers. Our findings support neurogenic compression as a contributing factor in the development of pain and other MALS symptoms, and favor the use of MALS rather than CACS as diagnostic terminology. To further study the pathogenesis of this unusual syndrome, surgeons should submit all tissues excised during MALS procedures for histopathologic examination.”
Median Arcuate Ligament Syndrome and Its Associated Conditions
“The risk factors and associated conditions of median arcuate ligament syndrome (MALS) have not been well characterized in the literature. In this study, we aim to investigate the presentation and outcomes of MALS patients with an emphasis on the prevalence of other uncommon disorders. To this end, data of patients with MALS who underwent surgery between 2013 and 2018 were collected and compiled into a retrospective database and analyzed. Eleven patients were identified. Seven of these eleven patients underwent diagnostics to evaluate gastric emptying. Five of these seven patients (71.4%) had radiographic evidence of delayed gastric emptying. Four of the eleven patients (36.4%) were found to have anatomic abnormalities of their visceral vasculature. Two of the eleven patients (18.2%) were found to have connective tissue disorders, both with Ehlers-Danlos syndrome. Three of the eleven (27.3%) had a diagnosis of postural orthostatic tachycardia syndrome. This is the first case series reporting on an association between MALS and delayed gastric emptying. We also explored the relationship between MALS and visceral vascular abnormalities, Ehlers-Danlos syndrome, and postural orthostatic tachycardia syndrome. It is notable that these conditions are more prevalent in the MALS population than in the general population, suggesting a possible pathophysiologic relationship.”
There you have it, a crash course in Median Arcuate Ligament Syndrome. If you're wanting to learn even more about this rare condition, I highly recommend checking out all of the sources I've listed in the description box below. There you'll also find links to my videos about POTS and EDS specifically.
Meanwhile, if you're looking to master your time, get organized and offer yourself radical compassion as a neurodivergent spoonie, check out my signature course, The Action Navigator, at this link.
If you liked this video, hit that like button and subscribe and be sure to share it with your friends. I'll be back soon with another video. See you then. Bye.
Blooper: I'm done. I can stop. (making silly mouth sounds because we did it!) (Off camera: alright) I don't know. Help me.
RESEARCH SOURCES:
Research Update: POTS & Median Arcuate Ligament Syndrome - Dr. Hassan Abdallah
Median Arcuate Ligament Syndrome in POTS - Dr. Hasan Abdallah
Median Arcuate Ligament Syndrome (MALS) Los Angeles, CA | Dr. Danny Shouhed
Robotic Release of Median Arcuate Ligament
Median Arcuate Ligament Syndrome and Its Associated Conditions