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.

• Weight Loss

  Signs and symptoms[edit] 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. [en.wikipedia.org]

• Abdominal Pain

  The major post-operative problem is either continued or return of the abdominal pain. [rarediseases.org]

  Abstract PURPOSE To assess the diagnostic potential and accuracy of CDS in the diagnosis and management of visceral artery stenosis in young patients with abdominal pain. METHOD 126 patients < 45 y with abdominal pain were examined by CDS. [unboundmedicine.com]

  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]

• Nausea

  Seek immediate medical help if your stomach pain occurs with: Bloody stools Fever Nausea and vomiting that doesn't go away Severe tenderness when you touch your belly area Swelling of the belly area Yellowing of the skin or whites of the eyes (jaundice [mayoclinic.org]

  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]

  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]

• 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]

  Symptoms of MALS include: Pain in the upper middle stomach area, which may go away when leaning forward Stomach pain after eating, exercising or changing body position Bloating Diarrhea Fear of eating food due to pain, leading to significant weight loss [mayoclinic.org]

  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]

  Occasionally, physical examination reveals an abdominal bruit in the mid-epigastrium.[2] Complications of MALS result from chronic compression of the celiac artery. [en.wikipedia.org]

• 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]

  Another postulation for MALS is the aberrant stomach electrical rhythm (e.g., tachycardia) caused by celiac ganglion compression, referred to as the gastroparesis component [1,3-10]. [cureus.com]

• Sweating

  […] the following symptoms: Pressure, fullness or tightness in your chest Crushing or searing pain that spreads to your jaw, neck, shoulders, and one or both arms Pain that lasts more than a few minutes or gets worse with activity Shortness of breath Cold sweats [mayoclinic.org]

  Figure 8 – Defibrosing of the coeliac plexus (patient standing)The effect of this technique is very strong and must be used with due consideration.If neurovegetative signs such as sweating occur, the force of the contact should bereduced. [slideshare.net]

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].

MALS diagnostic and therapeutic approach must be patient focused, bearing in mind the multiple clinical presentation and treatment options. Open surgical decompression of median arcuate ligament is the base of treatment. [ncbi.nlm.nih.gov]

Treatment and prognosis Symptomatic patients are treated with surgical decompression. This is usually performed laparoscopically by dividing the median arcuate ligament. [radiopaedia.org]

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]

In this study, predictors of favorable outcome included:[citation needed] 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) Epidemiology[edit] It is estimated [en.wikipedia.org]

However, a clear pathophysiological definition of MALS remains still obscure. [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. [mayoclinic.pure.elsevier.com]

However, whether revascularization of the celiac artery can prevent the aneurysm formation is unknown. [ncbi.nlm.nih.gov]

Prompt MALS release was succesful in order to prevent any major complications. The patient required a longer than usual postoperative stay in order to recover from bile leakage. [surgicalcasereports.springeropen.com]

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3. 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.
4. 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.
5. 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.
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7. 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.
8. Horton KM, Talamini MA, Fishman EK. Median arcuate ligament syndrome: evaluation with CT angiography. Radiographics. 2005;25(5):1177-1182.