Median Arcuate Ligament Syndrome (MALS)

Overview

Median arcuate ligament syndrome (MALS) is a condition where the arc-shaped band of tissue in the chest area, known as the median arcuate ligament, compresses the celiac artery, which sends blood to the upper abdomen. This compression can cause stomach pain and other symptoms such as chest pain, crushing or searing pain in the jaw, neck, shoulders, and arms, shortness of breath, cold sweats, dizziness, weakness, nausea, or vomiting. MALS can occur in anyone, including children, and its exact cause is not well understood. Surgery is often required to decompress the ligament and restore blood flow through the artery.

Understanding the disease

Symptoms

Symptoms of median arcuate ligament syndrome (MALS) include:

1. Pain in the upper middle stomach area, which may go away when leaning forward
2. Stomach pain after eating, exercising, or changing body position
3. Bloating
4. Diarrhea
5. Fear of eating food due to pain, leading to significant weight loss (usually greater than 20 pounds or 9.1 kilograms)
6. Nausea and vomiting These symptoms may develop due to a lack of blood flow through the celiac artery or compression on nerves in the area. It's important to note that MALS can cause chronic stomach pain, and symptoms typically start when people are in their 20s, although almost 20% of people diagnosed with MALS are under the age of 16. If you experience these symptoms, it's recommended to make an appointment with your healthcare provider.

Development

Median arcuate ligament syndrome (MALS) occurs when the arc-shaped band of tissue in the chest area (median arcuate ligament) presses on the artery that sends blood to the upper abdomen. The artery is called the celiac artery. MALS can cause stomach pain in some people. The location of the median arcuate ligament and celiac artery varies slightly from person to person. Typically, the ligament runs across the largest blood vessel in the body (aorta). It sits above the celiac artery. But sometimes the ligament or artery may be out of place, causing MALS. The ligament may also put pressure on the network of nerves surrounding the celiac artery (celiac plexus). MALS may occur in anyone, even children. Some people have developed MALS after pancreatic surgery and blunt injury to the upper stomach area.

Assessment and Diagnosis

Diagnosis

To diagnose median arcuate ligament syndrome (MALS), there's no specific test available. The diagnosis is typically made by a healthcare provider through a series of examinations, questions about symptoms and health history, and the use of imaging tests to rule out other causes of stomach pain. The provider may listen for a bruit, a sound indicating a blocked or narrowed blood vessel, when using a stethoscope on the upper stomach area. Tests like blood tests, magnetic resonance imaging (MRI), computed tomography (CT) of the abdomen, ultrasound, and arthroscopy may be used to diagnose MALS and rule out other conditions. Surgery, such as median arcuate ligament release or median arcuate ligament decompression, is the primary treatment option for MALS.

Management and Treatment

Complications

Median arcuate ligament syndrome (MALS) can lead to several complications, including:

1. Chronic pain: Individuals with MALS may experience chronic pain in the upper abdomen or back due to compression of the celiac trunk, a major artery that carries blood to the stomach, liver, and upper intestine.
2. Gastrointestinal issues: Compression of the celiac trunk can cause gastrointestinal symptoms such as bloating, abdominal cramping, and diarrhea.
3. Nutritional deficiencies: In severe cases, chronic pain and gastrointestinal issues can lead to malabsorption of nutrients, resulting in malnutrition and weight loss.
4. Intestinal ischemia: MALS can cause reduced blood flow to the small intestine, leading to a condition called intestinal ischemia, which can result in bowel necrosis (tissue death).
5. Infertility and sexual dysfunction: In rare cases, MALS can cause infertility or sexual dysfunction due to the compression of the nerves that control the reproductive organs.
6. Appendicitis: MALS may increase the risk of appendicitis, as the compression of the celiac trunk can cause inflammation and swelling of the appendix.
7. Acute vascular events: In rare cases, a rupture or tear in the celiac trunk can occur, leading to an acute vascular event that can be life-threatening. It is essential to seek medical attention if you suspect MALS or experience any of these complications.

Prevention

There isn't enough information available to determine if median arcuate ligament syndrome (MALS) can be prevented. The causes of MALS are not fully understood, and risk factors are unclear. However, if you are at risk for thoracic outlet compression, avoiding repetitive movements, lifting heavy objects, and maintaining a healthy weight may help prevent or relieve symptoms of thoracic outlet syndrome, which includes MALS.

Treatment

The primary treatment for median arcuate ligament syndrome (MALS) involves surgery to release (decompress) the ligament and restore blood flow through the artery. This surgical intervention is necessary to alleviate the compression on the celiac artery and associated symptoms.

Preparing for medical consultation

To prepare for an appointment for median arcuate ligament syndrome (MALS), you should:

1. Make an appointment with your health care provider if you have stomach pain that doesn't go away or symptoms of MALS. If MALS is found early, treatment may be more effective.
2. Be aware of any pre-appointment restrictions. Ask your health care provider if there's anything you need to do beforehand, such as avoiding food or drinks for a few hours before some blood or imaging tests.
3. Write down all your symptoms, including any that may seem unrelated to MALS.
4. Make a list of all medications, vitamins, or supplements that you're taking.
5. If possible, bring a family member or friend with you for support. They may remember something important that you missed or forgot.
6. Write down key personal information, including any family history of heart disease, stroke, high blood pressure, blood clots, and any major stresses or recent life changes.
7. Prepare a list of questions to ask your health care provider, such as: "What is likely causing my symptoms or condition?", "What are other possible causes for my symptoms or condition?", "What kinds of tests will I need?", "What's the most appropriate treatment?", and "What's an appropriate level of physical activity?" Remember to prioritize your questions from most to least important in case time runs out during the appointment. By being well-prepared, you can ensure a more productive and informative appointment.