Finding the Cause of Persistent Inexplicable Epigastric Pain
MALS is a congenital anatomic anomaly, meaning it is a structural aberrance present at the time of birth. In MALS patients, the diaphragm is too low, causing the median arcuate ligament to compress the celiac artery. This compression also affects the nerves of the celiac ganglion, a bundle of sensory nerves located in the same area. The pressure on the artery may cause a change in blood flow, while the pressure on the nerves causes them to become inflamed and send pain signals to the brain. Over time, the inflammation increases, and the nerves are chronically activated. Patients are often unable to eat due to pain/nausea and many lose weight.
SIGNS AND SYMPTOMS Pain in MALS patients is located between the ribs and below the sternum. Pain can also radiate into either the flank or back. Pushing on this area will increase the pain. Additional symptoms of MALS may include:
• Pain after eating • Fatigue after eating • Nausea/vomiting • Weight loss • Constipation/diarrhea
DIAGNOSIS Most doctors don’t know about it, so MALS is underdiagnosed. Differential diagnoses for epigastric pain include gastroparesis, eating disorders, IBS, or gallbladder issues.
Because MALS patients present with gastrointestinal symptoms, they often go through a battery of GI tests, including EGDs, gastric emptying tests, and colonoscopies. Often, these tests show no abnormality, so many patients are referred for psychological evaluations or to eating disorder clinics because doctors can find no physical cause for the pain. The anatomy for MALS can be seen on an abdominal CT scan. A mesenteric duplex or MRA can also be used, although these are less specific tests. Other diagnostic criteria required for diagnosis include physical exam findings consistent with inflamed celiac ganglion, and celiac ganglion block to rule in neurogenic cause of pain.
VASCULAR VS. NEUROLOGIC Because the median arcuate ligament is compressing both the celiac artery and the celiac ganglion nerves, there is debate as to whether the cause of pain is ischemic (caused by blood flow) or neurogenic (caused by the nerves).
Many doctors believe MALS is a vascular issue— however, this does not explain the pain patients feel, nor does it address those patients who continue to suffer despite having adequate blood flow through the celiac artery.
Those who believe MALS is a neurologic issue focus on the celiac ganglion as the main cause of a patient’s symptoms. Because of the pressure on the nerves, they have become inflamed beyond the point of repair. These inflamed nerves must be addressed to resolve the patient’s disabling pain.
MALS was first diagnosed and reported in the 1940s, and between 1963 and 2012, a total of less than 500 surgeries were performed. This means MALS is still a nascent diagnosis, and treatment continues to evolve. Because of this, there is often a great deal of variation between surgeons and the procedures they perform. It is therefore crucial for the patient to research those who treat MALS to find an experienced doctor and treatment plan that is right for them.
MALS can only be treated with surgery, either open or laparoscopic. In a laparoscopic procedure, the median arcuate ligament is released, which removes pressure from the artery. However, the celiac ganglion cannot be removed during a laparoscopic procedure, so if the nerves are inflamed and causing pain, they will continue to do so even after the pressure has been removed.
In an open procedure, the surgeon resects the median arcuate ligament and removes the celiac plexus nerves. Complete resection of the inflamed nerves has been shown to help reduce or completely eliminate the pain in over 90% of patients.
Richard C. Hsu, MD, PhD, FACS, RPVI
Dr. Richard C. Hsu is a Board-certified general and vascular surgeon, whose academic credentials include a PhD in Neuroscience from Yale University, surgical training at Stanford and Harvard Universities, and research positions at the National Institutes of Health and the John Hopkins University School of Medicine. His areas of interest include open and endovascular management of complex arterial aneurysms, peripheral arterial occlusive disease, extracranial cerebrovascular disease, venous occlusive disease, and venous hypertension. Dr. Hsu is recognized as a leader in the management of median arcuate ligament syndrome (MALS).
Timothy C. Stewart, PA-C is a native of New Zealand and is a current member of the American Academy of Physician Assistants. He attended the University of Southern California Keck School of Medicine, where he obtained his Master of Physician Assistant Practice, and he completed a Surgical residency at Norwalk Hospital through the Yale School of Medicine. His extensive treatment knowledge includes vascular surgery and endovascular care.
Founded in 2001 to deliver expert vascular medical and surgical care to the communities of Connecticut and Rhode Island, The Vascular Experts is now one of the largest groups of Board-certified vascular surgeons in the country. For questions, or to schedule an appointment with Dr. Hsu, contact The Vascular Experts office located in Danbury, CT at 860-661-1383.
TESTS NECESSARY TO DIAGNOSE MALS
• Abdominal CT with Intravenous Contrast
• Physical Exam
• Celiac Ganglion Block
TRAVELING FOR TREATMENT
At The Vascular Experts, it is our goal to make the patient experience as convenient and stress-free as possible. Our dedicated staff are prepared with information on various options for travel, accommodations and points of interest for any long-distance patients. Below, you will find further details on the various discounted rates, extended-stay options, local airports and other information that may be useful for planning your treatment with us. Please feel free to contact our office should you have any additional concerns regarding your travel at 844-482-7285.
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