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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.

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]
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]
Anorexia
  • Patient also reported anorexia and unprovoked weight loss of 8Kg over 3 months. Physical examination was normal. Other gastrointestinal pathologies were ruled out.[ncbi.nlm.nih.gov]
  • 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.The pain can be in the left or right side, but usually where the ribs meet.[en.wikipedia.org]
  • Randomized clinical trial comparing family-based treatment with adolescent-focused individual therapy for adolescents with anorexia nervosa.[healio.com]
  • […] the autonomic nerve supply to the upper gut can leads to gastroparesis, a finding which is seen to improve after surgical dissection of celiac ganglia with subsequent regularization of gastric myoelectric rhythm and appears to be responsible for the anorexia[pubs.sciepub.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]
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]
  • H 2018 22 Fish hook coeliac: Median arcuate ligament syndrome. ( 30309144 ) Xiang H...Ridley LJ 2018 23 Laparoscopic treatment of median arcuate ligament syndrome in a 16-year-old male. ( 30336385 ) Fujiwara Y...Ueno T 2018 24 Median Arcuate Ligament[malacards.org]
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 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]
Epigastric Pain
  • The median arcuate ligament syndrome is an unusual disease associated with postprandial epigastric pain, and the optimal treatment of this syndrome remains to be established.[ncbi.nlm.nih.gov]
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]
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]

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

  • 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]

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]
  • 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]
  • 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]

Epidemiology

  • 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]
  • 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]

Prevention

  • However, whether revascularization of the celiac artery can prevent the aneurysm formation is unknown.[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

  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: 2019-07-11 20:04