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

  A young female powerlifter presented with a 3-month history of postprandial epigastric pain and involuntary weight loss following the start of a vigorous weightlifting program. [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]

• 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]

• Abdominal Pain

  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]

  The predominant symptoms are an abdominal pain in the right upper quadrant and epigastric region. [symptoma.com]

• 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]

  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]

• 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]

  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]

• 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]

• Delayed Gastric Emptying

  Alterations in blood flow and ganglion compression are both associated with delayed gastric emptying, another physiological correlate of the clinical syndrome. [mayoclinic.pure.elsevier.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]

• Biliary Colic

  A diagnosis of biliary colic was made, and she underwent cholecystectomy. [gastroenterologyandhepatology.net]

• 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]

• Sweating

  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]

The most reliable treatment comprises open surgical treatment with division of the MAL, removal of surrounding celiac ganglion, evaluation of the celiac artery with pressure measurements or ultrasound, and celiac artery reconstruction if indicated. [doi.org]

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]

Iatrogenic trauma comprises wide range of injuries with various prognosis. Results of catheter therapy depend on patient´s primary disease and on his or her age. [sircro.org]

I was wondering what her long term prognosis will be. After surgery will she resume normal life again? Though I understand this is a congenital dise … read more Dr. [justanswer.com]

Rumination syndrome in children and adolescents: diagnosis, treatment, and prognosis. Pediatrics. 2003;111(1):158–162. doi:10.1542/peds.111.1.158 [CrossRef] Caplan A, Lambrette P, Joly J, Bouin M, Boivin M, Rasquin A. [healio.com]

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]

Table 1: Common etiologies of abdominal pain and weight loss. Table 2: Etiology of abdominal pain by gender. [ashdin.com]

Due to the obscurity and under-diagnosis of the disease, the epidemiology remains poorly delineated and at-risk populations are not defined. [ashdin.com]

The epidemiology of chronic pain in children and adolescents revisited: a systematic review. Pain. 2011;152(12):2729–2738. doi:10.1016/j.pain.2011.07.016 [CrossRef] Shelby GD, Shirkey KC, Sherman AL, et al. [healio.com]

Both types of GIB can be distinct clinically and also in epidemiology, prognosis, management and outcome. The hemorrhage could be acute, chronic, obvious or occult. Acute upper GIB is best investigated and treated with endoscopy. [sircro.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.
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8. Horton KM, Talamini MA, Fishman EK. Median arcuate ligament syndrome: evaluation with CT angiography. Radiographics. 2005;25(5):1177-1182.