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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.

Pleural Effusion
  • Complications consisted of chylous ascites ( n 1), wound dehiscence ( n 1), transient neurological disorders ( n 2), and pleural effusion ( n 1) (Table 3 ).[bmcsurg.biomedcentral.com]
Weight Loss
  • 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 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 typical clinical triad of symptoms includes postprandial epigastric pain, weight loss and vomiting.[ncbi.nlm.nih.gov]
  • Postprandial abdominal pain and weight loss are the main symptoms. Surgical treatment still remains the gold standard therapy. Here, we present the first case to our knowledge in which MAL syndrome accompanies Behcet's disease.[ncbi.nlm.nih.gov]
  • 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.[symptoma.com]
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]
  • All patients with renal artery entrapment had resistant hypertension. The MDCT showed the proximal narrowing caused by compression of median arcuate ligament .[journals.lww.com]
Collapse
  • Tipe III terdapat pemisahan komplit dari duodenum proksimal dengan duodenum distal yang collapsed. Lebih dari 50% dari pasien dengan atresia duodenal berkaitan dengan anomaly kongenital lain.[galaksimedika.wordpress.com]
Weight Gain
  • Patients can anticipate functional recovery, weight gain, and return to work with treatment.[ncbi.nlm.nih.gov]
Abdominal Pain
  • Exercise-related transient abdominal pain is a common entity in young athletes. An uncommon aetiology of this type of pain is median arcuate ligament syndrome.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
  • 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 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]
  • 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]
  • Other gastrointestinal symptoms that are common during prolonged or high-intensity exercise include nausea, diarrhoea and gastrointestinal bleeding (3, 4).[ncbi.nlm.nih.gov]
  • 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]
  • Patients with median arcuate ligament syndrome present with postprandial abdominal pain, nausea, vomiting, weight loss, and abdominal bruit.[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]
  • Occasionally, physical examination reveals an abdominal bruit in the mid-epigastrium . [1] Complications of MALS result from chronic compression of the celiac artery.[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]
  • bruit that varies with respiration may be audible in the mid-epigastric region.[hkmj.org]
Right Upper Quadrant Pain
  • Repeat CTA for right upper-quadrant pain and elevated lactic acid showed a significant distention of the common bile duct and mild intrahepatic biliary duct dilatation.[vasculardiseasemanagement.com]
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]
Tremor
  • Gejala hipoglikemia tersebut berupa: lemas, apatis, peka rangsang, tremor, pucat, berkeringat, syok, kejang sampai koma.[galaksimedika.wordpress.com]
Neglect
  • The functional disability inventory: measuring a neglected dimension of child health status. J Pediatr Psychol . 1991;16(1):39–58. doi:10.1093/jpepsy/16.1.39 [CrossRef] Claar RL, Walker LS.[healio.com]

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].

Pleural Effusion
  • Complications consisted of chylous ascites ( n 1), wound dehiscence ( n 1), transient neurological disorders ( n 2), and pleural effusion ( n 1) (Table 3 ).[bmcsurg.biomedcentral.com]

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]
  • Presenting symptoms lasted on average 16.5 12.7 months prior to treatment.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
  • This study aimed to identify factors that predict outcomes of surgical and nonoperative treatment in these patients. Patients referred with a possible diagnosis of MALS between 1998 and 2013 were identified retrospectively.[ncbi.nlm.nih.gov]

Prognosis

  • [2] Endovascular methods such as percutaneous transluminal angioplasty (PTA) have been used in patients who have failed open and/or laparoscopic intervention. [2] PTA alone, without decompression of the celiac artery, may not be of benefit. [2] [8] Prognosis[en.wikipedia.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]
  • 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]
  • 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]

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]
  • 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]

Epidemiology

  • Epidemiology [ edit ] 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. [1] [3] Approximately 1% of these individuals[en.wikipedia.org]
  • 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]
  • Due to the obscurity and under-diagnosis of the disease, the epidemiology remains poorly delineated and at-risk populations are not defined.[ashdin.com]
  • Berbagai studi epidemiologi telah dipublikasi terkait kasus trauma kepala.[galaksimedika.wordpress.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]
Sex distribution
Age distribution

Pathophysiology

  • However, a clear pathophysiological definition of MALS remains still obscure.[ncbi.nlm.nih.gov]
  • A discussion of the pathophysiology, literature review, and multispecialty treatment approach are presented.[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]
  • 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]

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]
  • When evaluating the celiac artery, it is important to include erect views to prevent misdiagnosis of celiac artery stenosis.[ncbi.nlm.nih.gov]
  • 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]
  • 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

Article

  1. 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.
  2. Lainez RA, Richardson WS. Median arcuate ligament syndrome: a case report. Ochsner J. 2013;13(4):561-564.
  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.
  6. 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.
  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.

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Last updated: 2018-06-21 18:13