Median Arcuate Ligament Syndrome
Disease Details
Family Health Simplified
- Description
- Median arcuate ligament syndrome (MALS) is a condition caused by the compression of the celiac artery by the median arcuate ligament, leading to chronic abdominal pain and digestive issues.
- Type
- Median Arcuate Ligament Syndrome (MALS) is not typically associated with a specific type of genetic transmission. It is generally considered a rare, congenital anatomical abnormality rather than a hereditary genetic disorder.
- Signs And Symptoms
- Patients with MALS reportedly experience abdominal pain, particularly in the epigastrium, which may be associated with eating and which may result in anorexia and weight loss. The pain can be in the left or right side, but usually where the ribs meet. Other signs are persistent nausea, lassitude (especially after a heavy meal) and exercise intolerance. Diarrhea is a common symptom, some experience constipation. While some experience vomiting, not everyone does. Exercise or certain postures can aggravate the symptoms. Occasionally, physical examination reveals an abdominal bruit in the mid-epigastrium.Complications of MALS result from chronic compression of the celiac artery. They include gastroparesis and aneurysm of the superior and inferior pancreaticoduodenal arteries.
- Prognosis
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There are few studies of the long-term outcomes of patients treated for MALS. According to Duncan, the largest and more relevant late outcomes data come from a study of 51 patients who underwent open surgical treatment for MALS, 44 of whom were available for long-term follow-up at an average of nine years following therapy. The investigators reported that among patients who underwent celiac artery decompression and revascularization, 75% remained asymptomatic at follow-up. In this study, predictors of favorable outcome included:
Age from 40 to 60 years
Lack of psychiatric condition or alcohol use
Abdominal pain that was worse after meals
Weight loss greater than 20 lb (9.1 kg) - Onset
- Median arcuate ligament syndrome (MALS) typically presents in young adults, although symptoms can occasionally appear in adolescents. The exact age of onset can vary widely.
- Prevalence
- The prevalence of Median Arcuate Ligament Syndrome (MALS) is not well-defined and appears to be quite low. It is considered a rare condition, with many cases likely going undiagnosed due to its nonspecific symptoms and the potential for overlap with other gastrointestinal disorders. Exact prevalence rates are not well documented in the medical literature.
- Epidemiology
- It is estimated that in 10–24% of normal, asymptomatic individuals the median arcuate ligament crosses in front of (anterior to) the celiac artery, causing some degree of compression. Approximately 1% of these individuals exhibit severe compression associated with symptoms of MALS. The syndrome most commonly affects individuals between 20 and 40 years old, and is more common in women, particularly thin women.
- Intractability
- Median arcuate ligament syndrome (MALS) is not necessarily intractable. While it can be challenging to diagnose and manage, treatment options, including surgical intervention (e.g., decompression of the median arcuate ligament), have shown success in alleviating symptoms for many patients. Outcomes can vary, but many individuals experience significant relief after appropriate treatment.
- Disease Severity
- Disease severity for Median Arcuate Ligament Syndrome (MALS) varies among individuals. Some may experience mild discomfort, while others can suffer from significant pain and complications requiring surgical intervention. Symptoms often include abdominal pain, especially after eating, weight loss, and nausea.
- Healthcare Professionals
- Disease Ontology ID - DOID:9892
- Pathophysiology
- Median arcuate ligament syndrome (MALS) is a condition characterized by the compression of the celiac artery by the median arcuate ligament, a band of tissue that connects the diaphragmatic crura on either side of the aortic hiatus. The compression can lead to a reduction in blood flow and ischemia to the structures supplied by the celiac artery. This ischemia can result in abdominal pain, especially after eating, weight loss, and sometimes other gastrointestinal symptoms. Additionally, there might be involvement of the celiac ganglion, contributing to the associated pain.
- Carrier Status
- Median Arcuate Ligament Syndrome (MALS) is not a condition that involves carrier status; it is an anatomical problem where the median arcuate ligament compresses the celiac artery. It is not inherited or related to genetic carriers.
- Mechanism
- Several theories attempt to explain the origin of pain caused by compression of the celiac artery. One proposes that compression of the celiac artery causes ischemia, or decreased blood flow, to abdominal organs, leading to pain. Another hypothesizes that there is compression not only of the celiac artery but also of the celiac ganglia, and that pain results from compression of the latter.
- Treatment
- Decompression of the celiac artery is the general approach to treatment of MALS. The mainstay of treatment involves an open or laparoscopic surgery approaches to divide, or separate, the median arcuate ligament to relieve the compression of the celiac artery. This is combined with removal of the celiac ganglia and evaluation of blood flow through the celiac artery, for example by intraoperative duplex ultrasound. If blood flow is poor, celiac artery revascularization is usually attempted; methods of revascularization include aortoceliac bypass, patch angioplasty, and others.In recent, a laparoscopic approach used to achieve celiac artery decompression; however, should the celiac artery require revascularization, the procedure would require conversion to an open approach.Endovascular methods such as percutaneous transluminal angioplasty (PTA) have been used in patients who have failed open and/or laparoscopic intervention. PTA alone, without decompression of the celiac artery, may not be of benefit.
- Compassionate Use Treatment
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Median Arcuate Ligament Syndrome (MALS) is a condition where the median arcuate ligament compresses the celiac artery, causing various symptoms. Treatment options, including compassionate use treatments, off-label, and experimental treatments, are aimed at alleviating symptoms and improving the patient's quality of life. Some of these treatments include:
1. **Surgical Release**: The most common and effective treatment is the surgical release of the median arcuate ligament to decompress the celiac artery. This is often referred to as celiac artery decompression surgery.
2. **Celiac Ganglion Block**: An off-label, minimally invasive procedure that involves injecting anesthetic and corticosteroids near the celiac artery to relieve pain and improve symptoms. This can be diagnostic as well as therapeutic.
3. **Stenting**: Although not the first-line treatment due to variable success rates, some physicians may use endovascular stenting of the celiac artery as an off-label approach to maintain vessel patency after surgical decompression.
4. **Laparoscopic or Robotic Surgery**: Experimental approaches using minimally invasive laparoscopic or robotic surgery techniques for the release of the median arcuate ligament can result in reduced recovery times and less postoperative pain.
5. **Botox Injections**: Experimental use of Botox (botulinum toxin) injections around the celiac artery to reduce nerve irritation and pain has been explored in some cases with varying results.
These treatment options are generally tailored to the individual patient's needs and the severity of their symptoms, and it is crucial to discuss all potential treatment approaches with a healthcare provider who specializes in MALS. - Lifestyle Recommendations
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For Median Arcuate Ligament Syndrome (MALS), lifestyle recommendations might include:
- **Dietary Changes:** Eating smaller, more frequent meals may help alleviate symptoms by reducing the pressure on the abdominal blood vessels.
- **Physical Activity:** Gentle exercise can improve overall health, but activities that exacerbate symptoms should be avoided.
- **Stress Management:** Stress can aggravate symptoms, so techniques such as mindfulness, meditation, or yoga may be beneficial.
- **Hydration:** Staying well-hydrated can support overall vascular health.
It's important to consult a healthcare provider for a personalized plan. - Medication
- Median Arcuate Ligament Syndrome (MALS) typically does not have specific medications for its treatment, as it is primarily a vascular and mechanical condition. Management often involves surgical intervention, such as decompression of the celiac artery by releasing the median arcuate ligament. Pain management and other supportive therapies may be used to address symptoms, but these are adjunct treatments rather than curative solutions. Always consult a healthcare professional for personalized medical advice.
- Repurposable Drugs
- Median Arcuate Ligament Syndrome (MALS) is a rare condition in which the median arcuate ligament compresses the celiac artery, affecting blood flow and causing symptoms such as abdominal pain. Drug repurposing for MALS is not well-established, as treatment typically involves surgical intervention to relieve the compression. However, managing symptoms like pain might involve medications such as analgesics or antispasmodics, but these do not address the underlying cause. Always consult a healthcare professional for personalized advice.
- Metabolites
- For Median Arcuate Ligament Syndrome (MALS), there isn't a well-documented profile of specific metabolites directly associated with the condition. MALS primarily involves the compression of the celiac artery, leading to abdominal pain and other gastrointestinal symptoms. The diagnosis and understanding of MALS are more focused on imaging studies such as Doppler ultrasound, CT angiography, and the patient's clinical presentation rather than specific metabolic markers.
- Nutraceuticals
- There is no well-established evidence to support the use of nutraceuticals for the treatment of Median Arcuate Ligament Syndrome (MALS). MALS primarily involves the compression of the celiac artery and nearby structures, leading to symptoms such as abdominal pain and weight loss. The primary treatments for MALS typically involve surgical intervention rather than nutritional supplements.
- Peptides
- Median Arcuate Ligament Syndrome (MALS) is not typically treated or managed with peptides, which are short chains of amino acids. Treatment strategies for MALS generally focus on surgical intervention to relieve the compression of the celiac artery by the median arcuate ligament. Therefore, there is no relevant information or application of peptides in the context of this syndrome.