Median Arcuate Ligament Syndrome
Celiac Artery Compression Syndrome
Median arcuate ligament syndrome (MALS) is a rare disease caused by a compression of the celiac artery by the ligament with nonspecific manifestations such as an abdominal pain and weight loss. Therefore, exclusion of other, more frequent diseases is an important part of the diagnosis. The main diagnostic tools are various imaging methods, such as angiography and computed tomography. The treatment is surgical, usually laparoscopic.
Presentation
The median arcuate ligament syndrome (MALS) is associated with symptoms caused by the pressure exerted by the ligament on the celiac artery and possibly on the celiac ganglion. The condition is also named celiac artery compression syndrome (CACS), celiac axis syndrome, or Dunbar syndrome [1]. The median arcuate ligament lies in the proximity of the aorta, close to the branch point of the celiac artery (celiac trunk), and if its position is lower than usual it may compress the celiac artery [2]. This compression may result in ischemia which gives rise to postprandial abdominal pain and other symptoms. However, vascular problems may not be the only explanation for the condition; the median arcuate ligament also compresses the celiac ganglion and thus neurogenic effects also contribute (or could be the main factor) in the etiology of the condition [3].
MALS is a rare condition that mainly affects relatively young women. The predominant symptoms are an abdominal pain in the right upper quadrant and epigastric region [4]. The abdominal pain is exacerbated by leaning forward, exercise, and food intake, and as a consequence, sitophobia accompanied by weight loss could ensue. More than 80% of patients also display epigastric bruits [5]. The bruits, signifying the compression of the celiac trunk, are worsened on expiration. Conversely, the symptoms are temporarily ameliorated when patients bring their knees close to the chest because this position lessens the compression of the celiac trunk. Other symptoms and signs include nausea and vomiting.
Entire Body System
Weight Loss
- Abstract Median arcuate ligament syndrome, a rarely reported condition, is characterized by postprandial abdominal pain, nausea, vomiting, and weight loss. Its cause is unclear.[ncbi.nlm.nih.gov]
- The typical clinical triad of symptoms includes postprandial epigastric pain, weight loss and vomiting.[ncbi.nlm.nih.gov]
- The syndrome is characterized by weight loss, postprandial abdominal pain, nausea, vomiting, and an epigastric bruit. Surgical management entails complete division of the median arcuate ligament.[ncbi.nlm.nih.gov]
- Sustained symptom relief occurred more often with a postprandial pain pattern (81% cure), age between 40 and 60 years (77%), and weight loss of 20 pounds or more (67%).[ncbi.nlm.nih.gov]
- Abstract Median arcuate ligament syndrome (MALS) is a rare entity characterized by extrinsic compression of the celiac artery and symptoms of postprandial epigastric pain, nausea, vomiting, and weight loss mimicking mesenteric ischemia.[ncbi.nlm.nih.gov]
Fishing
- Classically, the "fish hook" appearance is seen on sagittal images. In our case, mild stenosis (20 %) was seen at the origin of the celiac artery without typical "fish hook" appearance. Higher degree stenosis (55 %) was demonstrated on axial images.[ncbi.nlm.nih.gov]
Resistant Hypertension
- All patients with renal artery entrapment had resistant hypertension. The MDCT showed the proximal narrowing caused by compression of median arcuate ligament.[ncbi.nlm.nih.gov]
Gastrointestinal
Abdominal Pain
- KEYWORDS: Coeliac artery; exercise-related transient abdominal pain; median arcuate ligament syndrome; pain; stitch[ncbi.nlm.nih.gov]
- Abstract The median arcuate ligament syndrome is an uncommon condition characterized by the triad of postprandial abdominal pain, unintentional weight loss, and an epigastric bruit.[ncbi.nlm.nih.gov]
- METHODS: We present a case series of three athletes who presented with exercise-related transient abdominal pain and were ultimately diagnosed and treated for MALS.[ncbi.nlm.nih.gov]
- The character of the abdominal pain is often postprandial and associated with gradual weight loss from poor food intake, suggestive of chronic mesenteric ischemia.[ncbi.nlm.nih.gov]
- Abstract Median arcuate ligament syndrome (MALS) is a rare disorder associated with chronic, postprandial abdominal pain and radiographic evidence of celiac artery compression.[ncbi.nlm.nih.gov]
Nausea
- The syndrome is characterized by weight loss, postprandial abdominal pain, nausea, vomiting, and an epigastric bruit. Surgical management entails complete division of the median arcuate ligament.[ncbi.nlm.nih.gov]
- Abstract Median arcuate ligament syndrome (MALS) is a rare entity characterized by extrinsic compression of the celiac artery and symptoms of postprandial epigastric pain, nausea, vomiting, and weight loss mimicking mesenteric ischemia.[ncbi.nlm.nih.gov]
- The syndrome is characterized by weight loss, postprandial abdominal pain, nausea, vomiting, and an epigastric bruit [ 2 ]. Surgical management entails complete division of the median arcuate ligament [ 4 ].[doi.org]
- Other gastrointestinal symptoms that are common during prolonged or high-intensity exercise include nausea, diarrhoea and gastrointestinal bleeding (3, 4).[ncbi.nlm.nih.gov]
Diarrhea
- The initial presentation of MALS typically includes postprandial nausea, bloating, abdominal pain, and diarrhea, but in athletes, the initial presentation may be ETAP.[ncbi.nlm.nih.gov]
- Presentation can include chronic intermittent mild to severe abdominal pain, nausea, diarrhea, and significant weight loss. This syndrome most commonly affects young women (median age, 35 years).[radiologykey.com]
- The pain causes patients to avoid eating, which can lead to weight loss (often more than 20 pounds).Other associated symptoms may include: Nausea Diarrhea Vomiting Delayed gastric emptying (a delay in food moving from the stomach into the small intestine[my.clevelandclinic.org]
Abdominal Bruit
- Patients with MALS are often middle-aged females presenting with a triad of postprandial epigastric pain, weight loss and abdominal bruit. It is a diagnosis of exclusion and confirmed by computed tomography or magnetic resonance imaging.[ncbi.nlm.nih.gov]
- Patients with median arcuate ligament syndrome present with postprandial abdominal pain, nausea, vomiting, weight loss, and abdominal bruit.[ncbi.nlm.nih.gov]
- The abdominal pain may be related to meals, may be accompanied by weight loss, and may be associated with an abdominal bruit heard by a clinician.[en.wikipedia.org]
- The main symptoms are chronic abdominal pain that has lasted several months, abdominal pain after eating, weight loss, and sometimes an abdominal bruit, or the sound made by blood flowing through an obstruction.[bcm.edu]
- Abdominal bruit , which is an abnormal sound, or murmur, made by the attempted movement of blood flowing through a blood vessel that is either blocked or narrowed. This can be detected by a physician with a stethoscope placed on the upper abdomen.[study.com]
Cardiovascular
Tachycardia
- Six patients required re-admission for tachycardia, pancreatitis, or a segmental pulmonary embolus. All six pateints were treated non-operatively. At this time, no patient has required re-operative therapy for recurrent symptoms.[sages.org]
Hypotension
- Avoid thispain during the technique.During or just after the technique orthostatic hypotension can occur. Inform thepatient that this frequently occurring reaction will spontaneously resolve.5.2.3.[slideshare.net]
Workup
Because of the rarity of the condition and the unspecific nature of the associated symptoms, other, more frequent diseases with similar symptoms such as gallbladder diseases and peptic ulcer must first be excluded. Therefore, the patients usually undergo various imaging studies.
Angiography has been the gold standard for the diagnosis of MALS. This technique can reveal stenosis of the celiac trunk [6]. The resulting compression of the artery is increased on expiration and lowered during inspiration. However, a considerable percentage of healthy people also show marks of celiac artery compression during expiration. Therefore, it is important to view imaging results in light of the clinical observations. Sometimes, angiography also shows dilatation distal to the stenosis.
Duplex ultrasonography will detect an increased velocity of blood flow in the area of constriction. The extent of this increased velocity varies with inspiration and expiration: the maximum systolic flow velocity may be over twice as much during expiration as during inspiration [7].
Multidetector computed tomography (CT) scanners, coupled with three dimensional (3D) software for viewing images at different angles, have largely replaced conventional angiography. Optimal view of the stenosed celiac artery is afforded in the sagittal plane [2]. A finding specific for MALS is the hooked character of the stenosis which helps in differentiating MALS from other conditions causing a narrowing of blood vessels, such as atherosclerosis [2] [8].
Treatment
- Accepted treatment options are open release of median arcuate ligament, laparoscopic release of edian arcuate ligament, robot-assisted release of median arcuate ligament and open vascular treatment.[ncbi.nlm.nih.gov]
- CONCLUSION: Despite the existing controversy concerning pathophysiological mechanism, the clinical presentation and treatment modalities of patients with MAL syndrome, it is evident that careful selection and adequate surgical treatment may significantly[ncbi.nlm.nih.gov]
- Presenting symptoms lasted on average 16.5 12.7 months prior to treatment.[ncbi.nlm.nih.gov]
- Laparoscopic or open surgical decompression are the only treatment options in MALS. We present two cases of MALS treated by laparoscopic decompression as well as a literature review on this treatment.[ncbi.nlm.nih.gov]
- We also report the first case of successful combination of minimally invasive surgery and endovascular therapy in the treatment of this syndrome.[ncbi.nlm.nih.gov]
Prognosis
- Treatment and prognosis Symptomatic patients are treated with surgical decompression. This is usually performed laparoscopically by dividing the median arcuate ligament. vascular compression disorders Promoted articles (advertising)[radiopaedia.org]
Etiology
- The etiology of celiac occlusion is most commonly atherosclerotic disease. Occlusion or severe stenosis of the celiac artery secondary to a median arcuate ligament is less frequently encountered, and symptoms can be vague or completely lacking.[ncbi.nlm.nih.gov]
- Most occurrences are due to a "cramp" or "stitch," but an uncommon, and often overlooked, etiology of ETAP is median arcuate ligament syndrome (MALS).[ncbi.nlm.nih.gov]
- BACKGROUND: The existence, etiology, diagnosis, and treatment of median arcuate ligament syndrome (MALS) have long been subjects of debate.[ncbi.nlm.nih.gov]
- However, vascular problems may not be the only explanation for the condition; the median arcuate ligament also compresses the celiac ganglion and thus neurogenic effects also contribute (or could be the main factor) in the etiology of the condition.[symptoma.com]
- Based on the multiple theories of MALS etiology, it is unlikely that we know the true cause of this syndrome. In fact, there are many physicians who question the validity of the diagnosis of MALS.[ncbi.nlm.nih.gov]
Pathophysiology
- However, a clear pathophysiological definition of MALS remains still obscure.[ncbi.nlm.nih.gov]
- The controversy stems from an undefined pathophysiologic mechanism and the existence of celiac compression in asymptomatic patients.[ncbi.nlm.nih.gov]
- A discussion of the pathophysiology, literature review, and multispecialty treatment approach are presented.[ncbi.nlm.nih.gov]
- CONCLUSION: Despite the existing controversy concerning pathophysiological mechanism, the clinical presentation and treatment modalities of patients with MAL syndrome, it is evident that careful selection and adequate surgical treatment may significantly[ncbi.nlm.nih.gov]
- Proposed but unproven pathophysiological mechanisms include neurogenic pain from compression of the splanchnic nerve plexus and intermittent ischemia from compression of the celiac artery.[ncbi.nlm.nih.gov]
Prevention
- However, whether revascularization of the celiac artery can prevent the aneurysm formation is unknown.[ncbi.nlm.nih.gov]
- Despite a trial of preventative strategies, the patient's pain persisted, prompting surgical intervention. Following a laparoscopic median arcuate ligament release, the patient's symptoms resolved.[ncbi.nlm.nih.gov]
- CONCLUSIONS: When evaluating the celiac artery, it is important to include erect views to prevent misdiagnosis of celiac artery stenosis.[ncbi.nlm.nih.gov]
- CONCLUSION: ETAP is common in athletes and often resolves with preventative or conservative strategies. When ETAP persists despite these methods, alternative causes, including MALS, should be considered.[ncbi.nlm.nih.gov]
- The three-dimensional high definition view magnified the artery multi-fold to help me achieve precision that prevented collateral damage to healthy tissue, arteries and nerves,” says Dr.[indiamedtoday.com]
References
Article
- You JS, Cooper M, Nishida S, Matsuda E, Murariu D. Treatment of Median Arcuate Ligament Syndrome Via Traditional and Robotic Techniques. Hawaii J Med Public Health. 2013;72(8):279–281.
- Lainez RA, Richardson WS. Median arcuate ligament syndrome: a case report. Ochsner J. 2013;13(4):561-564.
- Balaban DH, Chen J, Lin Z, Tribble CG, McCallum RW. Median arcuate ligament syndrome: a possible cause of idiopathic gastroparesis. Am J Gastroenterol. 1997;92(3):519-523.
- Sproat IA, Pozniak MA, Kennell TW. US case of the day. Median arcuate ligament syndrome (celiac artery compression syndrome). Radiographics. 1993;13(6):1400-1402.
- A-Cienfuegos J, Rotellar F, Valentí V, et al. The celiac axis compression syndrome (CACS): critical review in the laparoscopic era. Rev Esp Enferm Dig. 2010;102(3):193-201.
- Duffy AJ, Panait L, Eisenberg D, Bell RL, Roberts KE, Sumpio B. Management of median arcuate ligament syndrome: a new paradigm. Ann Vasc Surg. 2009;23(6):778-784.
- Ozel A, Toksoy G, Ozdogan O, Mahmutoglu AS, Karpat Z. Ultrasonographic diagnosis of median arcuate ligament syndrome: a report of two cases. Med Ultrason. 2012;14(2):154-157.
- Horton KM, Talamini MA, Fishman EK. Median arcuate ligament syndrome: evaluation with CT angiography. Radiographics. 2005;25(5):1177-1182.