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
The typical clinical triad of symptoms includes postprandial epigastric pain, weight loss and vomiting. [ncbi.nlm.nih.gov]
A 17-year-old girl with chronic postprandial abdominal pain and weight loss was referred to our clinic. [jiaps.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. [mayoclinic.pure.elsevier.com]
- Anemia
However, a few cases of a patient of MALS having both iron deficiency anemia and celiac disease or drug resistant gastric ulcer have been reported ( 8, 9 ). Our patient had iron deficiency anemia with gastric ulcer. [kjronline.org]
In this report, we presented a 72-year-old male patient with celiac artery compression syndrome causing recurrent abdominal pain associated with gastric ulcer and iron deficiency anemia. [ncbi.nlm.nih.gov]
Uncommon mild presentations are likely gastropathy, peptic ulcer disease and not specified anemia. In CACS, symptoms are nonspecific and misdiagnosis is easy to achieve. [omicsonline.org]
Clinical and radiologic review of uncommon cause of profound iron deficiency anemia: median arcuate ligament syndrome. Korean J Radiol. 2014;15:439-442. Arazinska A, Polguj M, Wojciechowski A, Trebinski L, Stefanczyk L. [vasculardiseasemanagement.com]
The first category is comprised of patients with chronic intermittent bleeding resulting in sideropenic anemia. Angiography is indicated here to reveal vascular pathology, but never contrast extravasation. [sircro.org]
- Fever
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]
She denied fevers, diarrhea, chest pain, shortness of breath, hematuria, and hematochezia. [vasculardiseasemanagement.com]
After RFA patients develop post-ablation syndrome with fever and flu-like symptoms as side effects of the procedure. Mortality rate is around 1% in most of series published in the literature. [sircro.org]
Gastrointestinal
- 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. [mayoclinic.pure.elsevier.com]
A 17-year-old girl with chronic severe abdominal pain and weight loss was referred to our clinic. Other causes of chronic abdominal pain were investigated and excluded. [jiaps.com]
We are presenting an interesting case of a 16-year-old girl with postprandial abdominal pain and weight loss. [ncbi.nlm.nih.gov]
The 3 patients with negative test were subsequently found to have other causes of their chronic abdominal pain. [savs.org]
Expiration is on the left and inspiration on the right Median arcuate ligament syndrome is usually suspected in patients with chronic abdominal pain. The pain is usually epigastric and related to food. This may result in fear of food. [angiologist.com]
- Nausea
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]
The median arcuate ligament syndrome (MALS) is characterized by abdominal pain, nausea, and vomiting attributed to compression of the celiac axis by a fibrous band (the median arcuate ligament) connecting the diaphragmatic crura. [ncbi.nlm.nih.gov]
Occasionally, nausea, vomiting, diarrhoea, eructation, flatulence. More frequent in young women. Compression of the celiac artery by extraluminal structures such as arcuate ligament was first detected by Benjamin Lipshutz (1878-1931) in 1917. [whonamedit.com]
- Diarrhea
[…] arcuate ligament syndrome (MALS) or Dunbar syndrome is a rare clinical entity characterized by celiac trunk compression by median arcuate ligament and variable gastrointestinal symptoms (postprandial epigastric pain, nausea, weight loss, anorexia and diarrhea [ncbi.nlm.nih.gov]
Importantly, vomiting and diarrhea are not commonly associated with this syndrome. Median arcuate ligament syndrome should be considered as part of the differential diagnosis of chronic atherosclerotic mesenteric ischemia. [angiologist.com]
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]
In 1963, it was described by Harjola as a clinical syndrome of postprandial pain invariably associated with a systolic bruit and other constitutional symptoms such as nausea, vomiting, occasional diarrhea and malabsorption. Bibliography P. T. [whonamedit.com]
- Abdominal Bruit
Median arcuate ligament syndrome (MALS), also known as celiac artery compression syndrome, is an uncommon condition classically characterized by chronic abdominal pain, weight loss, and abdominal bruit. [ncbi.nlm.nih.gov]
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]
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]
- 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]
Her symptoms persisted postoperatively, and over the following 6 months, extensive evaluation was undertaken, including an upper endoscopy, which showed mild esophagitis, and a solid-phase gastric emptying study, which showed markedly delayed gastric [gastroenterologyandhepatology.net]
Cardiovascular
- 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]
In addition, she was notably hypertensive throughout the hospital stay despite multiple antihypertensive medications. This prompted a renal artery duplex ultrasound to evaluate for renal artery stenosis as a secondary cause of hypertension. [gastroenterologyandhepatology.net]
The patient’s medical history included insulin depending diabetes, hypertension and dyslipidemia. She was not smoking or consuming alcohol. Abdominal examination was unremarkable, with no audible bruit, mass or hepatosplenomegaly. [latunisiemedicale.com]
Most often, you will have a patient with history of one or more of the following medical problems: diabetes, hypertension, gastroesophageal reflux disease, peptic ulcer disease, smoking, alcoholism, weight loss, vitamin deficiency, etc. [vasculardiseasemanagement.com]
- Thrombosis
MALS is a risk factor for hepatic artery thrombosis (HAT) in liver transplant recipients. A thin female complained about upper abdominal pain for two months. The patient was diagnosed as primary biliary cirrhosis. [ncbi.nlm.nih.gov]
This is the same kind for pain from a distended left kidney from nutcracker syndrome or a spleen from a splenic vein thrombosis. [vascsurg.me]
Outcome for Sympathetically Mediated Abdominal Pain in a Case of Suspected Median Arcuate Ligament Syndrome: A Case Report. ( 29634547 ) Sun Z....Dua A. 2018 16 Median arcuate ligament syndrome and arterial anastomotic bleeding inducing hepatic artery thrombosis [malacards.org]
Complications are classified in three groups: vascular (e.g. portal vein thrombosis, hepatic vein thrombosis, hepatic infarction and subcapsular hematoma), biliary eg. Bile duct stenosis, biloma, abscess and hemobilia), and extrahepatic (eg. [sircro.org]
- Chest Pain
I did not think the constant chest/epigastric pain had a vascular etiology. [vascsurg.me]
Sometimes upper stomach pain can be confused with chest pain. [mayoclinic.org]
Additional symptoms associated with the diagnosis, but frequently indicative of other medical problems include palpitations (hearing or feeling your own heartbeat), chest pain, diarrhea, constipation, and difficulty sleeping. < Previous section Next section [rarediseases.org]
She denied fevers, diarrhea, chest pain, shortness of breath, hematuria, and hematochezia. [vasculardiseasemanagement.com]
- Systolic Murmur
• Systolic murmur (in 15% of cases). • Nausea (in 29% of cases). • Lower thoracic and thoracolumbar pain (in 22% of cases). • Acidic reflux (in 17% of cases). • Weight loss (in 15% of cases). [slideshare.net]
Their symptoms included, among others, abdominal pain (71%), nausea (29%), thoracic pain (22%), heartburn (17%), weight loss (15%), vomiting (15%), systolic murmur (15%), postprandial accentuation of symptoms (15%), diarrhea (14%), respiratory discomfort [researchgate.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]
Skin
- Ulcer
These symptoms are usually nonspecific and are easily misdiagnosed as functional dyspepsia, peptic ulcer disease, or gastropathy. [ncbi.nlm.nih.gov]
However, a few cases of a patient of MALS having both iron deficiency anemia and celiac disease or drug resistant gastric ulcer have been reported ( 8, 9 ). Our patient had iron deficiency anemia with gastric ulcer. [kjronline.org]
Upon questioning, Mary states that she did visit a 'quick care' clinic about two months ago and they diagnosed her with a stomach ulcer, gave her a prescription and provided her with a list of foods to avoid. [study.com]
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. [symptoma.com]
Doctors have to rule out conditions such as hernia, peptic ulcer and other gastric pathology before finally getting a CT angiography to determine if an artery is being compressed. [indiamedtoday.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]
Psychiatrical
- Fear
A small subset of this population may present with MALS, an anatomic and clinical entity in which extrinsic compression of the celiac axis leads to postprandial epigastric pain, nausea or vomiting, and weight loss (often related to "food fear" or fear [kjronline.org]
This may result in fear of food. Weight loss is the norm. Importantly, vomiting and diarrhea are not commonly associated with this syndrome. [angiologist.com]
The pain can lead to a fear of eating and significant weight loss. The pain and related depression or anxiety can greatly impact quality of life. MALS symptoms may be vague and can mimic other conditions. [mayoclinic.org]
This was associated with diarrhea three to four times per day, and loss of body weight of 20 kilograms in the two years due to fear of eating and inappetance. No nausea nor vomiting were reported. [latunisiemedicale.com]
Weight loss is common secondary to food avoidance, with fear from pain triggered by eating. Epigastric tenderness and abdominal bruit may be found on examination. [gastroenterologyandhepatology.net]
Neurologic
- Irritability
The cause remains unknown but may be due to compression of the sensitive outer arterial wall layer by the muscle and ganglionic tissue, direct irritation or inflammation of sympathetic nerves, and possibly intermittent decrease in blood supply to the [med.uth.edu]
In some patients the symptoms can be devastating and can lead to erroneous diagnoses of an eating disorder, psychiatric conditions, or functional abdominal pain (e.g. irritable bowel syndrome, abdominal migraine). [rarediseases.org]
This finding indicates that the symptoms can be of neurogenic origin via irritation of the coeliac plexus. [slideshare.net]
Theories which explain the source of post prandial pain and other symptoms of the syndrome postulated mesenteric ischemia and or irritation of basal ganglia [ 10 ]. [pubs.sciepub.com]
The neurogenic pain is either via direct sympathetic pain fiber irritation or celiac plexus stimulation. Secondly, it is believed to be due to mesenteric ischemia. [mjdrdypu.org]
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
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 reduce symptoms [ncbi.nlm.nih.gov]
Vaziri K,Hungness ES, Pearson EG, Soper NJ (2009) Laparoscopic treatment of celiac artery compression syndrome: case series and review of current treatment modalities.J Gastrointest Surg 13: 293-298. [omicsonline.org]
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. [link.springer.com]
Prognosis
Treatment and prognosis Symptomatic patients are treated with surgical decompression. This is usually performed laparoscopically by dividing the median arcuate ligament. [radiopaedia.org]
Etiology
In this review article, we discuss briefly about the MALS in terms of etiology, diagnosis and its management including the role of interventional radiology. [ncbi.nlm.nih.gov]
Epidemiology
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]
Pathophysiology
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]
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 reduce symptoms [ncbi.nlm.nih.gov]
[…] uncertainty of those patients diagnosed, (iii) question the reliability of existing imaging techniques, with the additional use of multiphase electrocardiogram (ECG)-gated CT acquisition, and therefore (iv) augment the description of the currently recognized pathophysiology [bjr.birjournals.org]
Prevention
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]
References
- 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.