Median Arcuate Ligament Syndrome
At the time I started seeing my POTS specialist, he had been investigating Median Arcuate Ligament Syndrome (MALS) as a possible cause of POTS due to vagus nerve inflammation. MALS is when the diaphragm hangs too low or the celiac artery is too high coming off the abdominal aorta, and it compresses the celiac artery. The only solution to resolve MALS is surgery. Sometimes, a person can change their diet or bypass the stomach with a G-tube to help with symptoms if they wish to avoid surgery.
My doctor had an ultrasound in the office and a trained sonographer who knew the testing protocol. I had never heard of MALS in my life; however, little did I know just how perfectly my GI (gastrointestinal) symptoms matched it.
I was the kid who was constantly in the nurse’s office at school, especially after lunch or breakfast, complaining of stomach pain. I was given Sprite, crackers and a place to lay down for about 15 minutes. This continued throughout elementary school. I was even given a colonoscopy around 7 years old, but it was, of course, normal. In middle school, the nurses weren’t as accommodating, but by that time, I had learned not to eat big meals. Most of my meals were chips, coke, or the school pizza. Certain breakfasts always made me late to school. I learned in my 20s that some of this was lactose intolerance, but even after cutting out milk, I still had a lot of nausea and this tightness around my upper abdomen and chest. Sometimes, I would go to the hospital thinking I was having heart problems, but everything would come back normal.
When I was 20 years old, they did a HIDA scan to check my gallbladder. It showed a reduced ejection fracture, and I was having a lot of pain in the upper right abdomen. They did a cholecystectomy to take it out and found it to be very sludgy. The surgeon said this was very likely the cause of my ills, and it was good we got it out. However, recovery took weeks. I was in a lot of pain. It didn’t seem to fix much over time and now I had dumping syndrome (lots of Imodium and a medicine called Welchol were needed for several years). My primary doctor said it was probably IBS (Irritable Bowel Syndrome – one of the many misdiagnosis given to MALS patients).
Not long after my surgery, I started having reactive hypoglycemia. If I had a coke on an empty stomach for example, I would crash. One time I was driving, left my cell phone at home, and got extremely dizzy. I pulled over and waved out the window, but no one saw me. I managed to exit the freeway and asked for help, but they said I seemed drunk. Someone finally (though reluctantly) called 911 for me. My blood sugar was 56. It took some glucose to bring it up, but eventually it did. I learned to recognize my symptoms before it got that low from that point forward, but there were still several times I needed assistance getting sugar to bring it up.
Finally, 15 years after that gallbladder surgery, I got my answers. My ultrasound revealed a celiac artery velocity of 550, where anything over 200 is considered positive. That means that my blood was trying to get passed that arterial compression with a lot of force. The velocity of the hepatic artery was also severely reduced (since it branches off the celiac artery, a reduced velocity shows a compression of the celiac artery). A CTA confirmed severe stenosis; however, my doctor said it was up to me whether to get treatment, as the outcomes were not guaranteed. I found a support group on FB called “MALS Pals” and was able to locate a vascular surgeon who specialized in MALS. It is definitely something you want to have done by someone with a lot of experience. After surgery and recovery (which took about a year for my stomach to work properly), I no longer have that epigastric pain with referred chest pain, gastroparesis (confirmed by a normal emptying scan), ulcers, or reactive hypoglycemia.
I share this story to highlight something that is not well known about MALS. It can severely reduce blood flow to the stomach, liver and the gallbladder. These organs are frequently not taken into account when assessing for the syndrome, but when MALS goes untreated, it can lead to disease of the gallbladder, liver, stomach and sometimes the pancreas. Think fatty liver, reactive hypoglycemia, stomach or intestinal ulcers, gastroparesis, pancreatitis and even diabetes (luckily, I had no pancreatic involvement). And maybe even cancer of these organs? It is definitely something that needs more research. It’s estimated that nearly 35% of the population shows evidence of MALS on CTs, yet it is not communicated. MALS should not be dismissed as an incidental finding just because someone isn’t displaying the traditional symptoms. There is just not enough known about it yet. Having the surgery is not 100% guaranteed to solve all the pain and GI issues, but it can definitely help prevent other co-morbidities.
MALS and other vascular compressions (SMAS, NCS, MTS) are more common in the EDS population due to our loose connective tissue. You do not have to be a certain body size (I was actually overweight when diagnosed with MALS) or be a certain gender or age. The classic sign is epigastric pain with tightness that wraps around to the back (even if intermittent or barely noticeable), nausea and early satiety. Please reach out to a MALS support group to find a knowledgable doctor for diagnostic testing.
If you have any questions about MALS, please check out the National MALS Foundation at https://malsfoundation.org or on social media. The MALS Awareness page on FB is also a great resource.
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