National MALS Foundation Pediatric Portal
First things first, take a deep breath! When you find out you have MALS, people tend to go on a scavenger hunt for information which can be extremely overwhelming. It can also be frustrating since there isn’t a lot of accurate information on the internet. The purpose of this portal is to help make learning a little easier and for the information to be from evidence based research and testimonials. When it comes to testing for MALS, surgeries and developing coping skills for living with a serious medical illness, we want to be able to provide as much help as possible. We hope that these Q&As will be able to ease your mind about everything that comes with a MALS diagnosis. This part of the website is made specifically for pediatric patients and their parents. Many MALS patients are under 18, but there's not much information when it comes to how best to treat them. Research studies are mostly done with adults, making it hard to tell if those statistics even apply to someone under 18.
Even though age is in your favor when it comes to healing, it's also worth addressing the impact of the physical, social and emotional aspects of your MALS journey. It can be confusing, as well as, make you feel sad, lonely and even angry as you see your peers living symptom free lives. MALS not only involves physical things that happen with your body, but can also impact you socially and emotionally. This is completely normal and does not mean you have a mental health problem! MALS symptoms can be debilitating and trying to cope with all of that while trying to maintain a normal academic and social life can take a toll on you emotionally. It is definitely not easy living with MALS. It’s also not fair when you are not taken seriously by the doctors who are supposed to help you. You are not alone in this problem as this happens to adult MALS patients as well. We know that thinking about surgery as a young person and a parent can be overwhelming and we want to be able to make this process less stressful and less scary for both. We hope this information can provide peace of mind as well as filling in the gaps the doctors may have missed. Every MALS patient is unique as are their stories and we want to provide as much solace as we can as you navigate on your MALS journey.
MALS Frequently Asked Questions
What is Median Arcuate Ligament Syndrome (MALS)?
MALS is a condition that happens when the Median Arcuate Ligament pushes down on the celiac artery and sometimes the nerves around the artery. This makes it hard for blood to flow normally. This ligament attaches to your diaphragm which is a muscular structure in your upper belly that moves up and down when you breathe. MALS can lead to awful GI symptoms, nausea, and pain. Most patients have their symptoms increase after eating, drinking, and/or exercise. It’s not a simple tummy ache.
What is the Celiac Artery and what does it do?
How do I receive a MALS diagnosis?
MALS is usually a diagnosis of exclusion. That means it is important to rule out other, more likely causes. Most patients will need to go through a full set of gastrointestinal (GI) tests just to make sure your symptoms aren’t from another illness. These tests look at how your esophagus, stomach, gallbladder, pancreas, liver and intestines are functioning. Very rarely will MALS show up on GI testing, so don’t get discouraged if the tests all come back as “normal”. At this point, you should be sent for further testing to look for MALS. These MALS-specific tests may include a wand (Mesenteric duplex ultrasound) that is rolled around on your belly to see how fast the blood flows through the arteries or a special x-ray machine (CTA) with or without a dye that takes pictures to see if any arteries are squished, a special magnetic machine (MRA) can do this too. Sometimes, you may need to have a special test that numbs the celiac nerves around the artery. This is called a celiac plexus block and it is sometimes used as part of the diagnosis process and sometimes part of the pain management treatment, but not always. The doctors will look at all your test results to see if you have MALS. It's important that the tests are done using the correct protocols necessary and should be done by someone experienced in looking for MALS. It's also important that all tests are read or interpreted by someone who understands the complexity of MALS and who knows exactly what to look for in the results. If not, you may be told you do not have MALS, when in fact you might.
Can you have a GI illness and still have MALS?
It’s not that uncommon for a MALS patient to have more than just MALS, some people have another GI illness too. But there are also times when MALS patients get misdiagnosed with other illnesses because of their MALS symptoms. Gastroparesis (GP) is not that uncommon for MALS patients to have. Some doctors will diagnose MALS patients with Irritable Bowel Syndrome (IBS), Acid Reflux, Chronic Vomiting Syndrome (CVS) and Dumping Syndrome. These are all syndromes that are a diagnosis made by exclusion which is similar to how you get a MALS diagnosis. It’s important that you know that the symptoms you have that result in a GI diagnosis may or may not be directly related to MALS. We see both. Some patients have a (GI) diagnosis, but the symptoms are actually caused by MALS. In other cases, there is an independent (GI) condition not related to MALS. This is why it’s so important to have various GI testing prior to surgery including being tested for Small Intestinal Bacterial Overgrowth (SIBO), Cdiff (diarrhea due a bacteria and inflammation), celiac disease, fecal elastase stool and follow-up with your Gastroenterologist after surgery if you continue to have digestive issues.
Tests That are Used to Help Diagnose MALS
The most frequently used tests in getting a MALS diagnosis are the Mesenteric Duplex Ultrasound, a CTA or MRA and the Catheter Angiography through the groin or arm. Sometimes, doctors will also ask that you undergo a celiac plexus nerve block, if they think it will help. Just remember that a celiac plexus block is not exclusive to MALS. It is used to help with pain management in other conditions like pancreatitis and pancreatic cancer.
Mesenteric Duplex Ultrasound evaluates blood flow through the mesenteric arteries (celiac, superior and inferior) which supply blood to the digestive organs. Gas can make getting a good reading difficult because it blocks the blood flow image in the arteries. A gas relieving product may be recommended to take before the procedure. It is critical that this test be performed using inspiration and expiration to avoid false negatives.
CT angiography (CTA) scan is a special kind of CT x-ray exam that focuses on the blood vessels, using a contrast material to make them show up clearly in the images. CTA is used to examine blood vessels in areas including the abdomen. It is critical that this test be performed using inspiration and expiration to avoid false negatives.
Magnetic Resonance Angiography (MRA) or MRI scanner. Magnetic resonance imaging (MRI) is an advanced medical imaging technique that does not use x-ray or radiation. An MRA (a special type of MRI) is used to obtain detailed images of blood vessels and blood flow.
Catheter Abdominal Angiography with contrast dye. Angiography, angiogram, arteriogram are all terms used to identify a procedure that outlines blood vessels, usually arteries, in various areas in the body like the abdomen. A thin plastic tube, called a catheter is inserted into an artery through a small incision in the skin in the groin or arm. This tube is guided into the celiac artery. Contrast material is injected through the tube and images are captured. The doctor can also obtain pressure measurements which help determine how compressed or closed off the artery is and see how tortuous the blood flow is in that area compared to surrounding vessels. This test can determine if the artery is compressed and needs intervention.
Celiac Plexus Block can be used to assist with a MALS diagnosis and or for providing pain relief. The abdomen has many nerves that run through this area. There are also times when people have an unsuccessful block but still have success with surgery. This has recently been added to the MALS world and some doctors do not require or use it as a diagnostic test.
MALS Testing and Treatment Questions
Why not just skip to vascular testing?
Honestly, we wish it was that simple. MALS mimics a lot of other conditions and it is important to make sure MALS is the cause of your symptoms. MALS surgery isn’t something to take lightly. It can take some patients up to a year to fully recover from surgery. So it’s best to make sure you don’t have something else that could be treated without surgery.
How do you treat MALS?
What kind of surgery do you have to treat MALS?
So there are mainly 3 types of surgery to treat MALS: Open, Laparoscopic or Robotic. Open surgery is one incision several inches long that starts below the sternum. Sometimes nerves will be stripped or removed depending on what the surgeon feels is best for your situation. Laparoscopic surgery is almost the same thing except for the fact that you have usually four - six smaller incisions that the surgeon uses to insert surgical tools and a camera. Robotic is the newer surgical type which seems to have good results as well. Patients find significant success with all three types of surgical approaches. Choosing the type of surgery depends on numerous factors and is best discussed with your surgeon and clinical care team. Recovery is quicker for Robotic and Lap than it is for Open, but it really depends on the person. You just have to decide which surgery is best for your needs and to be at peace
What to expect after surgery?
Surgery is not a quick fix. As much as we’d love to instantly feel better after surgery that usually is not the case. You have to take things slow. Yes you are younger and that will help with healing but you still have to give your body time. When your body starts to readjust its blood flow you may have days you have the symptoms you did prior to surgery. It takes your body a while to fully level out. If you have had the compression for a long time it may take a few months to achieve healthy blood flow. If after healing from surgery, you continue to have symptoms, please consult your surgeon and Care Team. There may be additional treatments available to you. We hope that your surgeon will follow up with you at regular intervals during the first year at a minimum, but don't hesitate to go see them if you have concerns.
What is the next step if surgery doesn’t resolve my symptoms?
It is not uncommon for MALS patients to require more than one surgery. That doesn’t always mean your surgeon did something wrong in the surgery. Sometimes surgeons also want to wait and see if the celiac will bounce back on its own before doing further intervention. There are a few things that can’t be done until the ligament is released like stenting. If you need a stent placed this has to be done AFTER the release. It can be incredibly dangerous to have this done before or during the ligament release surgery. Sometimes the celiac will require a bypass or even a reconstruction after the release because it is unable to stay open on its own. Although the nerves are usually taken care of during the first surgery there may be a need for further intervention.
My surgeon wants me to see a therapist, does that mean they think it’s all in my head?
Sadly when MALS patients are going through the process of diagnosis they will likely have at least one doctor tell them it’s all in their head. When your surgeon asks you to see a therapist that is not what they mean. There have been studies done that say if a patient can talk about their concerns prior to surgery it can help their recovery. There are also techniques that therapists can teach you to help cope with the surgical pain and coping skills to manage chronic pain and the emotional pain that comes from not being able to participate in the activities that you wish to. This is not meant to get rid of the pain but to help you get through it. Some surgeons require you to go through this as part of their treatment plan while others do not. If you are uncomfortable going to a therapist make sure to voice that to your surgeon. Most will not force the issue.
How does MALS affect your mental health?
Having any type of chronic illness can affect your mental health. It can be even worse if you go years without a diagnosis. You not only stop trusting the medical community but you start to not trust yourself. There may even be times where you question if the symptoms are really just something you created. All the tests keep coming back normal yet you can feel that something is wrong. It can be incredibly hard to go through, even more so for a kid. This is the time you want to just be able to go out with your friends and be normal. But that isn’t always an option. You struggle to maintain the illusion of health while you feel like your body is attacking itself. We as humans use food as a way to connect to each other. We use food in almost all of our social activities. Then you have to decide if you want to eat and make yourself sick or not eat and have people judge you because of it. It starts to feel like its not worth going out because either way you are at a loss. Which can lead to isolating yourself from friends and family. Sadly sometimes people won’t believe you are sick even when you finally get diagnosed. Which can be incredibly hurtful at the time. This is why there are support groups for patients with MALS. Sometimes we lack the support of those around us in our real life so we find that support online.
What are breathing protocols?
Are there illnesses that are common for MALS patients to have?
MALS patients are all so different when it comes to our specific MALS symptoms. Oddly enough there are a few other illnesses and even surgeries we seem to commonly share. Just because fellow patients happen to have these illnesses doesn’t mean that every MALS patient does. Some people may have MALS but are otherwise healthy.
Some of the conditions seen in some MALS patients are:
Postural Orthostatic Tachycardia Syndrome (POTS)
Ehlers Danlos Syndrome (EDS)
Mast Cell Activation Syndrome (MCAS)
It’s also common for MALS patients to have their gallbladder removed sometimes unnecessarily.
There are also a few that aren’t as common but also appear in our fellow MALS patients:
Superior Mesenteric Artery Syndrome (SMAS)
Nutcracker Syndrome (NCS)
Pelvic Congestion Syndrome (PCS)
May Thurner Syndrome (MTS)
How do I connect with fellow MALS patients and caregivers?
There are a few support groups for MALS patients specifically. They can mainly be found on Facebook MALSPals. There is also a MALS forum on Mayo Clinic Connect Our community is small but it is incredibly compassionate and caring. It can be an incredible experience to connect with people who are going through the same thing you are. Most support groups have both the MALS patients and their caregivers. So you can hear about the MALS experience from each side. These groups were created to help us find each other. They have also become a safe haven for us to share our journeys with each other. They aren’t always happy stories but it does help to be able to share and read about fellow experiences.