Materiały źródłowe

• #1 Overview of Median Arcuate Ligament Syndrome: A Narrative Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC10629207/

  Median arcuate ligament syndrome (MALS) is a rare disorder caused primarily by compression of the celiac trunk by the median arcuate ligament (MAL). […] It is believed to be caused by the constriction of the celiac artery and the celiac plexus by the median arcuate ligament (MAL). […] When the MAL is found inferior to its typical insertion on the spine or when the celiac artery is superior to where it typically branches from the thoracic aorta, MALS may develop. […] Due to this abnormal anatomical structure, the celiac artery and the celiac plexus are compressed by the MAL. This compression may result in hyperplasia of the intimal wall of the celiac artery, thus causing stenosis of its lumen and ultimately resulting in ischemia of the abdominal organs. […] The symptoms of MALS are believed to be due to the abnormally low insertion of the MAL, which occurs because of errors in embryological development. This low insertion of the MAL results in compression of the celiac artery, causing a decrease in blood flow to the foregut.

• #2 Celiac Artery Compression Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470601/

  Celiac artery compression syndrome is also known as Dunbar syndrome or median arcuate ligament syndrome. It is a rare condition characterized by recurrent abdominal pain that results from compression of the celiac artery. Compression is due to the presence of a fibrous band of the diaphragm known as the median arcuate ligament. […] The etiology of celiac artery compression syndrome is not fully understood. Uncertainty arises because the anatomic compression of the celiac artery by the median arcuate ligament can also be seen in asymptomatic patients. […] The median arcuate ligament is a fibrous band that connects the two medial borders of the diaphragmatic crura, usually near the level of the 12th thoracic or first lumbar vertebra. Compression of the celiac artery can occur in two anatomic situations: an abnormally cephalad origin of the celiac artery or an abnormally caudad insertion of the diaphragm.

• #3 Diagnosis of Median Arcuate Ligament Syndrome on Multidetector Computed Tomography | Göya | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/1384/820

  The median arcuate ligament syndrome (MALS), also known as celiac artery compression syndrome, is a condition which develops secondary to an ischemia in gastrointestinal organs resulting from the compression of proximal celiac truncus by the median arcuate ligament of the diaphragm. […] The syndrome is concerned with the ischemia in gastrointestinal organs which develops secondary to the compression of proximal celiac truncus by the median arcuate ligament (MAL) of the diaphragm. […] In this syndrome, the compression occurs on the proximal part of the celiac artery (especially during the expiration) and thus leads to an abdominal angina and pain. […] The MALS was initially described by Harjola, in 1963. […] Although the syndrome was described in the 1960s, there is an ongoing debate as to whether celiac compression leads to the clinical picture or not.

• #3 Median Arcuate Ligament Syndrome (MALS) – The Operative Review Of Surgery

  https://operativereview.com/median_arcuate_ligament_syndrome/

  Median Arcuate Ligament Compresses Celiac Artery […] Causes: Abnormally High Origin of Celiac Artery (Most Common) […] Abnormally Low Median Arcuate Ligament (10%) […] Celiac Plexus Nerves Can Also Contribute to Compression.

• #4 Median arcuate ligament syndrome: A clinical dilemma | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/88/3/143

  Compression of the celiac artery was first described in 1917 by Lipshutz, but it took almost 50 years to understand any clinical implication. […] The triad of epigastric abdominal pain exacerbated by eating, weight loss, and celiac artery compression was initially described in 1963 by Harjola, leading to the first description of median arcuate ligament syndrome (MALS). […] To this day, controversy persists around the pathology of this syndrome, with some groups labeling it a vascular disease, while others consider it a neurogenic disease. […] This neurolysis is now believed to be the main technical aspect that results in symptom improvement, rather than revascularization. […] Our understanding of the disease has highlighted the role of the celiac plexus nerve fibers as the most relevant anatomic structure related to this syndrome.

• #4 MALS (Median Arcuate Ligament Syndrome): Symptoms & Diagnosis

  https://my.clevelandclinic.org/health/diseases/16635-median-arcuate-ligament-syndrome-mals

  Median arcuate ligament syndrome (MALS) refers to a condition that happens when the median arcuate ligament in your chest presses against your celiac artery and nearby nerves (celiac plexus). […] Normally, your median arcuate ligament sits above your celiac artery. In MALS, your ligament is lower than usual. When it presses on your celiac artery, the pressure keeps blood from flowing through your celiac artery. It may also put pressure on nearby nerves. […] Experts dont know the exact reason why it happens. Some researchers suggest people are born with a median arcuate ligament thats not in the right place. Another theory is MALS is a complication of abdominal or spinal surgery or abdominal trauma. […] Your healthcare provider may recommend median arcuate ligament release, which is surgery to remove or release your ligament so its not pressing on your celiac artery. This procedure restores blood flow through your celiac artery and removes pressure on nearby nerves. […] Surgery often eases symptoms. But research shows median arcuate ligament syndrome can come back after surgery.

• #5 Median Arcuate Ligament Syndrome | Top Diagnosis & MALS Surgery Near You – The Vascular Experts

  https://thevascularexperts.com/median-arcuate-ligament-syndrome/

  MALS is a congenital anatomic anomaly, meaning it is a structural aberrance present at the time of birth. In MALS patients, the diaphragm is too low, causing the median arcuate ligament to compress the celiac artery. This compression also affects the nerves of the celiac ganglion, a bundle of sensory nerves located in the same area. The pressure on the artery may cause a change in blood flow, while the pressure on the nerves causes them to become inflamed and send pain signals to the brain. […] Because the median arcuate ligament is compressing both the celiac artery and the celiac ganglion nerves, there is debate as to whether the cause of pain is ischemic (caused by blood flow) or neurogenic (caused by the nerves). […] Those who believe MALS is a neurologic issue focus on the celiac ganglion as the main cause of a patients symptoms. Because of the pressure on the nerves, they have become inflamed beyond the point of repair. These inflamed nerves must be addressed to resolve the patients disabling pain.

• #6 Median Arcuate Ligament Syndrome: Pathophysiology, Diagnosis, and Management – Clinical Tree

  https://clinicalpub.com/median-arcuate-ligament-syndrome-pathophysiology-diagnosis-and-management/

  Multiple theories exist regarding the pathophysiology of the epigastric pain associated with MALS. One theory hypothesizes that increased blood demand through a compressed celiac artery leads to foregut ischemia and pain. […] Another theory states that midgut ischemia induces abdominal pain through a vascular steal syndrome. […] Others suggest that the epigastric pain is caused by overstimulation of the celiac plexus with subsequent splanchnic vasoconstriction and ischemia. […] Some authors favor a neurogenic hypothesis for symptoms of MALS. Histologic analysis of the celiac plexus shows that the nerves consist of pain fibers and inhibitory motor fibers to the stomach. Thus, entrapment of the ganglion by the MAL may alter gastric myoelectrical activity and impair antral motility, and induce pain. Since the underlying pathophysiology of MALS is unknown, the diagnosis of MALS is difficult. Consequently, to enhance the benefit of patient selection and surgical intervention, the diagnosis of MALS needs to be reliable and accurate.

• #7 Diagnosis of Median Arcuate Ligament Syndrome on Multidetector Computed Tomography | Göya | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/1384/820

  The controversy arises from an undefined pathophysiological mechanism and the existence of celiac compression in asymptomatic patients. […] It has been reported that 13% – 50% of healthy individuals may exhibit angiographically different features of compression especially during expiration. […] There are two main theories used to explain the pathogenesis of the symptoms. The first one is the mesenteric ischemia arising from celiac artery compression. The second theory is the neurogenic stimulation that is caused by the compression of celiac ganglion and plexus.

• #8 Celiac Artery Compression Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK470601/

  The compression of the celiac artery by the median arcuate ligament is believed to cause intermittent mesenteric ischemia. However, this explanation alone may not completely explain the condition as there is usually a rich collateral network of mesenteric vessels between the celiac artery and the superior mesenteric artery. Therefore, there may be a role for underlying celiac nerve plexus dysfunction as well when considering the etiology of this condition. Nerve dysfunction may lead to abnormal splanchnic vasoconstriction, leading to ischemia. […] During expiration, there is compression of the celiac axis by the median arcuate ligament. During expiration, the diaphragm moves upwards, causing stretching of crura, which causes more compression of celiac axis. […] The celiac artery develops fibrotic changes in its wall due to the recurrent compression from the median arcuate ligament. […] Further studies are required to better define the underlying pathogenesis of celiac artery compression syndrome.

• #9 Median arcuate ligament syndrome: When to consider the diagnosis and management options

  https://www1.racgp.org.au/ajgp/2024/supplement-december/median-arcuate-ligament-syndrome

  It is thought that the compression of the coeliac artery can cause foregut pain and ischaemia during increased demand when patients eat. […] However, a prospective study in 2006 found that a positive gastric tonometry should be used to identify patients who would benefit from MALS release, suggesting that severe coeliac artery compression can result in ischaemic symptoms. […] Some postulate that a steal phenomenon occurs due to significant collateral circulation causing blood to be diverted away from the midgut to the foregut following meals, resulting in midgut ischaemia and pain. […] Further theories include that overstimulation of the coeliac plexus causes significant vasoconstriction, which results in ischaemic abdominal pain and the entrapment of the coeliac ganglion, altering gastric myoelectrical activity, impairing antral motility and causing neurogenic pain. […] The pathophysiology of MALS is unclear but is likely an interplay between coeliac artery and coeliac ganglion compression.

• #10 A Median Arcuate Ligament Syndrome Could Be Re-termed as a Nutcracker Celiac Ganglion Abdominal Pain Syndrome

  https://www.jnmjournal.org/journal/view.html?uid=1832&vmd=Full

  We suggest renaming MALS as NCGAPS, nutcracker celiac ganglion abdominal pain syndrome, to better explain the mechanism of the recurrent abdominal pain. […] If the mechanism of abdominal pain in MALS is decrease in blood flow due to celiac artery compression, there should be evidence of ischemic tissue damage, but we could not find any ischemic evidence in the liver or stomach in our patients according to liver function profiles, CT, or endoscopy. […] Therefore, the possibility of an ischemic mechanism due to vascular compression is extremely low. […] In conclusion, we suggest renaming the name of MALS as NCGAPS, nutcracker celiac ganglion abdominal pain syndrome, to better explain the mechanism of the recurrent abdominal pain and to raise awareness of the cause of the pain.

• #11 A Median Arcuate Ligament Syndrome Could Be Re-termed as a Nutcracker Celiac Ganglion Abdominal Pain Syndrome

  https://www.jnmjournal.org/view.html?uid=1832&vmd=Full

  Median arcuate ligament syndrome (MALS) is known as chronic recurrent abdominal pain related to compression of the celiac artery by the median arcuate ligament. […] Two hypotheses of MALS etiology are well known, one due to the decrease in intravascular blood flow due to compression of the celiac artery as the pathogenesis, and the other due to overestimation by the celiac ganglion. […] The mechanism of MALS is unclear and it is not included in the diagnostic category for abdominal pain, some case reports of MALS have been described with rare congenital diseases or gastroparesis. […] We thought that the characteristics of the pain in these patients are important and the mechanism of the pain is eventually the compression of the celiac trunk ganglion by medical arcuate ligament based on our case series.

• #12 Median Arcuate Ligament Syndrome a dilemma of a delayed diagnosis of a rare disease: A Case Report

  https://www.acquaintpublications.com/article/median_arcuate_ligament_syndrome_a_dilemma_of_a_delayed_diagnosis_of_a_rare_disease_a_case_report76

  Median arcuate ligament syndrome is a rare disorder where there is compression on the celiac trunk by the median arcuate ligament of the diaphragmatic crura. […] Pathophysiology of this disease is not well known, but it is hypothesized that it is due to one of either: decreased blood flow through celiac trunk due to compression, leading to foregut ischemia that leads to this periodic pain, but the issue is that it is a chronic disorder and development of collateral blood vessels usually inhibit progression to ischemia and pain. […] Another hypothesis is that two simultaneous disease processes in the forms of compression on the celiac trunk and overstimulation of the celiac ganglion led to irritation and sympathetic pain fibers, leading to splanchnic vasoconstriction and chronic ischemic changes.

• #13 Median Arcuate Ligament Syndrome Clinical Presentation, Pathophysiology, and Management: Description of Four Cases

  https://www.mdpi.com/2624-5647/3/1/5

  Median arcuate ligament syndrome (MALS), otherwise called celiac artery compression syndrome (CACS), is an uncommon disorder that results from an anatomical compression of the celiac axis and/or celiac ganglion by the MAL. […] The pathophysiology of MALS is related to both ischemic and neuropathic mechanisms due to the anatomic relationship of the MAL to the origin of the arterial celiac plexus and ganglion. […] Theories have been proposed to explain the altered anatomy of the median arcuate ligament, including a congenital anomaly. A traumatic origin has also been hypothesized, causing a change of the diaphragm anatomy, resulting in an alteration of the diaphragm’s aortic hiatus. […] The detection of an abnormal gastric electric rhythm in patients with MALS is suggestive of neurogenic pathogenesis.

• #14 Median Arcuate Ligament Syndrome – Practical Gastro

  https://practicalgastro.com/2015/02/05/median-arcuate-ligament-syndrome/

  It was first suggested that the compromise of blood supply by compression of the celiac artery would lead to post prandial ischemia and hence worse abdominal pain. However the SMA and IMA are widely patent in this entity and the stomach has extensive collateral blood flow from the gastric and epiploic arcades leading to the conclusion that ischemia is not the explanation for the abdominal pain and other associated symptoms. It was suggested later that pressure on the celiac plexus might be the main etiological factor in MALS and thus it could also explain the accompanying findings often present in addition to the abdominal pain, namely nausea, vomiting, gastroparesis, and gastric electrical dysrhythmias. […] Many theories have been proposed to explain this altered anatomy including congenital origin as it has been found in children and twins, as well as traumatic origin. The presentation in adults could be explained by a combination of a congenitally early take off origin of the celiac artery and/or an abnormally thickened MAL with or without accompanying arteriosclerosis in the vessel that may predispose to the narrowing finding on expiration.

• #15 Median arcuate ligament syndrome – UpToDate

  https://www.uptodate.com/contents/celiac-artery-compression-syndrome

  Median arcuate ligament syndrome (MALS) is defined as chronic, recurrent abdominal pain related to compression of the celiac artery by the median arcuate ligament. […] The etiology and pathophysiology of MALS are incompletely understood but may be related to both ischemic and neuropathic mechanisms. […] Compression of the celiac artery by the median arcuate ligament is accentuated during expiration as the median arcuate ligament moves cranially and relieved during inspiration.

• #16 Median Arcuate Ligament Syndrome Clinical Presentation, Pathophysiology, and Management: Description of Four Cases

  https://www.mdpi.com/2624-5647/3/1/5

  The major findings specific for the diagnosis are compression of the celiac artery with respiratory variations, post-compression dilation, and elevated velocities of blood flow. […] The compressive effect of the MAL on the celiac artery is relieved during inspiration since the aorta moves anteriorly and inferiorly. […] The gold standard for diagnosing MALS is lateral view aortic angiography, which shows more than 50% narrowing in the origin of the celiac artery, with or without distal dilation, with improvement during inspiration. […] The surgical approach of minimally invasive laparoscopic and robotic techniques focuses on ligating the ligament, and often times results in partial surgical disruption of the celiac plexus, thus overcoming the compression of the celiac ganglion from which the symptoms are emanating.

• #17 Median arcuate ligament syndrome: When to consider the diagnosis and management options

  https://www1.racgp.org.au/ajgp/2024/supplement-december/median-arcuate-ligament-syndrome

  Median arcuate ligament syndrome (MALS) occurs due to extrinsic compression of the coeliac plexus, leading to postprandial and exercise-induced epigastric pain, nausea, vomiting, food fear and weight loss. […] Median arcuate ligament syndrome (MALS) refers to the external compression of the coeliac plexus by the median arcuate ligament (MAL) with resulting symptoms. […] Compression alone is a common radiological finding in approximately 25% of the population; however, only 1% of the population have corresponding symptoms. […] Chronic compression by this ligament can lead to hyperplastic intimal changes of the coeliac artery. […] This might progress to cause stenosis or complete arterial occlusion, along with post-stenotic dilation and coeliac artery aneurysms. […] The pathophysiology of MALS is unclear, and multiple theories have been discussed in the literature.

• #18 Median arcuate ligament syndrome often poses a diagnostic challenge: A literature review with a scope of our own experience

  https://www.wjgnet.com/1948-9366/full/v15/i6/1048.htm

  The European Society for Vascular Surgery guidelines regarding diseases of the mesenteric arteries and veins, state that MALS is the most common cause of single vessel abdominal arterial stenosis. […] One common complication of MALS due to the increased flow speed through the celiac trunk is the subsequent development of collateral blood vessels. […] This vascular compression theory is accompanied by some other researchers who support that extrinsic pressure upon the celiac plexus from the median arcuate ligament may also play a role in the pathophysiology of the syndrome. […] The diagnostic modalities that are more commonly used include CT of the abdomen with IV contrast, magnetic resonance angiography (MRA), or Doppler ultrasound with a respiratory variation protocol. […] A very recent report from an expert panel on interventional radiology, regarding the topic of mesenteric ischemia, included MALS in their attempt to provide concise and evidence-based instructions for the diagnosis and treatment of mesenteric ischemia. […] The development of collateral vessels, which is a complication from the celiac artery stenosis, has been found to be a poor prognostic factor in patients with MALS.

• #19 Acute median arcuate ligament syndrome after pancreaticoduodenectomy | Surgical Case Reports | Full Text

  https://surgicalcasereports.springeropen.com/articles/10.1186/s40792-016-0242-6

  Median arcuate ligament syndrome (MALS) has been reported in 27.6 % of patients undergoing pancreaticoduodenectomy (PD). […] The mechanism of MALS has been well demonstrated. The median arcuate ligament is a tendinous band spanning the right and left diaphragmatic crura, anterior to the aorta. In MALS, the celiac artery (CA) is compressed by this fibrous band, causing extrinsic compression anteriorly and leading to partial or complete CA occlusion. […] Most of the reported cases of MALS have been diagnosed perioperatively, either by radiologic findings or by intraoperative digital palpation or Doppler sonography. […] Acute onset MALS during or after PD is very rare. […] MALS can develop acutely after PD in patients with normal hepatic artery flow during GDA clamping. […] There is no known explanation for this acute onset of MALS after PD in a patient with normal celiac anatomy.

• #20 Median arcuate ligament syndrome (MALS) – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/median-arcuate-ligament-syndrome-mals/symptoms-causes/syc-20505001

  Median arcuate ligament syndrome, also called MALS, happens when the band of tissue in the upper belly area presses on the artery that sends blood to the stomach, spleen and liver. This tissue is called the median arcuate ligament. The artery is called the celiac artery. […] But sometimes, the ligament or arteries may be out of place. The ligament may put pressure on the celiac artery and the network of surrounding nerves, called the celiac plexus. This pressure can cause the symptoms of MALS. The condition can cause serious stomach pain in some people. […] The exact cause of median arcuate ligament syndrome, also called MALS, is not known. […] Because the cause of MALS is poorly understood, the risk factors are unclear. Median arcuate ligament syndrome is more common in adults than in children. It also is more common among women than among men. […] Some people have developed median arcuate ligament syndrome after pancreatic surgery or blunt injury to the upper stomach area.

• #21 Median arcuate ligament syndrome (MALS)

  https://www.drpadclinic.com/median-arcuate-ligament-syndrome-mals

  Median arcuate ligament syndrome (MALS) develops when the artery that supplies blood to the upper abdomen is compressed by the arc-shaped band of tissue in the chest region. The celiac artery is the name of the vessel. […] Each person has a somewhat different position for the celiac artery and median arcuate ligament. Typically, the ligament crosses the bodys largest blood vessel (aorta) over the celiac artery, where it is located. But occasionally, the ligament or artery may be misaligned, leading to MALS. Moreover, the ligament may exert pressure on the celiac arterys surrounding nerve system (celiac plexus). […] The actual cause of median arcuate ligament syndrome is unknown to medical professionals. There is debate concerning the origins and diagnosis of MALS. […] The risk factors for MALS are uncertain because it is unclear what causes the illness. MALS has been observed in children, including twins, suggesting that genetics may be involved.

• #22 Median arcuate ligament syndrome (MALS) | Altru Health System

  https://www.altru.org/health-library/conditions/median-arcuate-ligament-syndrome-mals

  Median arcuate ligament syndrome, also called MALS, happens when the band of tissue in the upper belly area presses on the artery that sends blood to the stomach, spleen and liver. […] The exact cause of median arcuate ligament syndrome, also called MALS, is not known. […] Because the cause of MALS is poorly understood, the risk factors are unclear. […] Some people have developed median arcuate ligament syndrome after pancreatic surgery or blunt injury to the upper stomach area. […] A complication of median arcuate ligament syndrome, also called MALS, is long-term pain, especially after meals. […] Surgery is the only treatment for median arcuate ligament syndrome, also called MALS. […] During decompression surgery, the surgeon cuts away parts of the median arcuate ligament. This reduces pressure on the celiac artery and nerves.

• #23 Median Arcuate Ligament Syndrome (MALS): Laparoscopic

  https://www.laparoscopicsurgeons.com/blog/median-arcuate-ligament-syndrome-mals-signs-symptoms-causes.html

  The primary cause of MALS is the compression or entrapment of the celiac artery by the median arcuate ligament. However, several factors can contribute to the development of this condition: […] Some individuals may have a naturally lower or more prominent median arcuate ligament, which increases the likelihood of celiac artery compression. […] People with a thinner body type or a low percentage of body fat may be more prone to MALS due to the decreased cushioning around the abdominal structures. […] MALS is more commonly diagnosed in young to middle-aged adults, with a higher prevalence among women. […] Abdominal trauma or injury, such as from a car accident or sports-related incident, can lead to the development of MALS by altering the normal anatomy and positioning of the median arcuate ligament. […] Certain medical conditions, such as Marfan syndrome, Ehlers-Danlos syndrome, or fibromuscular dysplasia, may increase the risk of MALS due to their effects on connective tissue and vascular structure.

• #24 Acute median arcuate ligament syndrome after pancreaticoduodenectomy | Surgical Case Reports | Full Text

  https://surgicalcasereports.springeropen.com/articles/10.1186/s40792-016-0242-6

  We hypothesize that a very tight median arcuate ligament presented in this young patient in addition to lymph-node clearance around the celiac trunk might induce the stenosis. […] MALS is most often diagnosed preoperatively, and a small portion of patients are diagnosed intraoperatively. Unfortunately as in our patient, MALS can have an acute postoperative onset even if all preoperative and intraoperative evaluations are normal.

• #25 Median arcuate ligament syndrome (MALS)

  https://www.mymlc.com/health-information/diseases-and-conditions/m/median-arcuate-ligament-syndrome-mals/

  Median arcuate ligament syndrome (MALS) occurs when the arc-shaped band of tissue in the chest area (median arcuate ligament) presses on, or traps, the artery that supplies blood to the organs in your upper abdomen (celiac artery). […] The causes and diagnosis of MALS has been a subject of controversy. The signs and symptoms may be due to a lack of blood flow through the celiac artery, or compression on the nerves (neurological) of the celiac ganglion causing the pain. […] Because the cause of MALS is poorly understood, the risk factors for the syndrome are unclear. MALS has been seen in children, even twins, which might mean genetics plays a role. […] Some people have developed MALS after pancreatic surgery and blunt injury to the upper stomach area.

• #26 Triad of MALS, Celiac Artery Dissection, and Fibrillin-1 Gene Variance of Unknown Significance | ACS

  https://www.facs.org/for-medical-professionals/news-publications/journals/case-reviews/issues/v4n3/09-kirskey-triad-of-unknown-significance/

  Spontaneous isolated celiac artery dissection (SICAD) is a rare phenomenon that occurs without involvement of the aorta but may involve concurrent dissection of other visceral vessels. […] Theoretically, the median arcuate ligament may represent a fixed transition point for the origin of SICAD. […] This has led many case reports and series to specify whether a concurrent median arcuate ligament compression was associated with the presented cases of SICAD. […] In another series, 2 out of 12 patients presenting with SICAD had associated MALS. […] We believe the widespread nature of spontaneous visceral arterial dissection in this patient, which included not only the celiac artery and its immediate branches but also spontaneous dissection of the right renal artery, could be reflective of genetic predisposition to arteriopathy secondary to fibrillin-1 gene variance.

• #27 Psychiatric comorbidities of median arcuate ligament syndrome: Indications for intervention across the lifespan

  https://www.probiologists.com/article/psychiatric-comorbidities-of-median-arcuate-ligament-syndrome-indications-for-intervention-across-the-lifespan

  Median Arcuate Ligament Syndrome (MALS) is the terminology that describes the vascular compression of the celiac artery, which at times is associated with numerous gastrointestinal symptoms. […] Despite the often-striking symptom presentation of patients, significant hesitation in treating MALS is present, partially due to the lack of understanding of the pathophysiological mechanism of pain in this condition. […] A diathesis-stress model of psychopathology in the context of chronic pain suggests that pain and GI symptoms associated with MALS may trigger, or be triggered by, mental health concerns. […] Given the association of poorer clinical outcomes for adults and pediatric patients with MALS with psychiatric symptoms and disorders, evaluation and implementation of treatment for such disorders prior to surgery is crucial. […] As pain may contribute to the development and maintenance of psychiatric symptoms in this population, interventions aimed at addressing psychopathology must consider and account for the role of pain in the etiopathology.

• #28 Diagnosis of Median Arcuate Ligament Syndrome on Multidetector Computed Tomography | Göya | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/1384/820

  The controversy arises from an undefined pathophysiological mechanism and the existence of celiac compression in asymptomatic patients. […] It has been reported that 13% – 50% of healthy individuals may exhibit angiographically different features of compression especially during expiration. […] There are two main theories used to explain the pathogenesis of the symptoms. The first one is the mesenteric ischemia arising from celiac artery compression. The second theory is the neurogenic stimulation that is caused by the compression of celiac ganglion and plexus.

• #29 A Median Arcuate Ligament Syndrome Could Be Re-termed as a Nutcracker Celiac Ganglion Abdominal Pain Syndrome

  https://www.jnmjournal.org/view.html?uid=1832&vmd=Full

  We suggest renaming MALS as NCGAPS, nutcracker celiac ganglion abdominal pain syndrome, to better explain the mechanism of the recurrent abdominal pain. […] If the mechanism of abdominal pain in MALS is decrease in blood flow due to celiac artery compression, there should be evidence of ischemic tissue damage, but we could not find any ischemic evidence in the liver or stomach in our patients according to liver function profiles, CT, or endoscopy. […] Therefore, the possibility of an ischemic mechanism due to vascular compression is extremely low. […] If the patient does not respond to medication, then surgical revision of the median arcuate ligament with ganglionectomy should be considered. […] In conclusion, we suggest renaming the name of MALS as NCGAPS, nutcracker celiac ganglion abdominal pain syndrome, to better explain the mechanism of the recurrent abdominal pain and to raise awareness of the cause of the pain.

• #30 Median arcuate ligament syndrome: A clinical dilemma | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/88/3/143

  It is important for clinicians evaluating patients with abdominal pain to understand that an imaging finding of celiac artery compression is not diagnostic of MALS. […] This syndrome requires careful and meticulous evaluation to rule out more common pathology. […] Median arcuate ligament syndrome is not a vascular disease.

• #31 Triad of MALS, Celiac Artery Dissection, and Fibrillin-1 Gene Variance of Unknown Significance | ACS

  https://www.facs.org/for-medical-professionals/news-publications/journals/case-reviews/issues/v4n3/09-kirskey-triad-of-unknown-significance/

  Furthermore, the extensive nature of SICAD, in this case, was likely due to synergistic effects of fibrillin gene variance manifesting as a predisposing collagen vascular disorder and MAL compression of the celiac artery, a known risk factor for this pathology. […] Our protocol for surgical management for MALS includes appropriate imaging workup and celiac plexus nerve block to determine whether the physiologic absence of activity by nerve fibers compressed by the median arcuate ligament will result in the resolution of a patient 's pain-related symptoms. […] At this time, we believe that pain in the setting of MALS is secondary to neurogenic compression of the celiac plexus by the MAL; hypoperfusion is unlikely to be the etiology for pain in these patients. […] In appropriate cases of MALS, minimally invasive median arcuate ligament release should be offered as it may improve vascular complications and patient symptoms.

• #32 Median Arcuate Ligament Syndrome: From Diagnosis to Multidisciplinary Management—A Narrative Review

  https://www.mdpi.com/2813-2475/4/1/11

  Median Arcuate Ligament Syndrome (MALS) also known as Dunbar’s syndrome is one of the main factors leading to Celiac Artery Stenosis (CAS). MALS is defined as external compression of the celiac trunk and celiac plexus by the fibrous arch connecting the diaphragmatic crura, or MAL. Individuals with a higher position of celiac artery or a lower position of median arcuate ligament are predisposed to this condition. The characteristic presentation of a patient with MALS is that of a young female, without comorbidities of atherosclerotic phenotype. MALS may remain asymptomatic in up to 25% of affected patients, while, according to different article, between 15% and 50% of asymptomatic cases can still be identified. The most common associated symptoms include postprandial abdominal pain, nausea and vomiting, weight loss, bloating, abdominal bruit and increased epigastric tenderness. The presentation of clinical symptoms is due to hemodynamic disturbances in the CA, leading to malperfusion of visceral organs supplied by this vessel such as the spleen, pancreas, liver, stomach and proximal part of the duodenum. Symptoms are caused by vascular and neuropathic components. The vascular component is responsible for a decrease in blood flow due to compression of the CT. The neuropathic component, on the contrary, results in irritation of the celiac plexus and visceral pain. In addition, chronic compression of the CA by the MAL may promote the formation of aneurysms within the visceral arteries branching from the CA, such as aneurysms in the pancreatoduodenal artery arch. The aim of this review is to discuss the clinical and imaging features, therapeutic management and potential future research directions that may be useful in the early diagnosis and treatment of MALS from a vascular surgeon’s perspective. In addition, the review emphasizes, the need for a multidisciplinary approach involving the vascular surgeon, radiologist, gastroenterologist and psychologist to optimally manage patients with MALS.

• #33 UT Physicians | Median arcuate ligament syndrome (MALS)

  https://www.utphysicians.com/median-arcuate-ligament-syndrome/

  The standard treatment of MALS is the release of the celiac artery by open surgical or laparoscopic removal of portions of the median arcuate ligament and ganglionic tissue that surround or completely encase the celiac artery. Regardless of the specific approach, the operation requires meticulous release of the celiac artery by division of muscle fibers from the median arcuate ligament and surgical removal of the overlying lymphatic, ganglionic, and soft tissue. The term neurolysis is used to describe the removal of nerve and ganglionic tissue that surrounds the artery in the front, sides, and back. […] Endovascular approach is not recommended as a stand-alone therapy because the muscle and ligament cause persistent compression of the artery, which typically recoils back to its compressed state after the balloon angioplasty.

• #34 Median arcuate ligament syndrome (MALS) – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/median-arcuate-ligament-syndrome-mals/

  While you’re under general anesthesia, a surgeon divides the median arcuate ligament and network of nerves in the stomach area (celiac plexus). Doing so provides more room for the artery, restores blood flow and relieves pressure on the nerves. […] Several studies have shown that surgery to release the median arcuate ligament is safe, even in children with MALS. It often results in immediate pain relief and improves the person’s quality of life.

• #35 Median arcuate ligament syndrome (Dunbar syndrome) – Iqbal – Cardiovascular Diagnosis and Therapy

  https://cdt.amegroups.org/article/view/67298/html

  Median arcuate ligament syndrome (MALS) is a rare condition which is due to the compression of celiac trunk by low riding of fibrous attachments of median arcuate ligament and diaphragmatic crura. […] The higher origin of celiac artery or lower insertion of diaphragmatic crura are likely to lead to MALS. […] Compression can be due to thick, fibrous tissue or thin bands at or near the origin of celiac artery. […] The treatment of MALS aims at decompression of celiac artery to establish adequate blood flow and pain management by neurolysis. […] The role of intervention radiology is emerging in the treatment of MALS, though its outcomes are not promising. […] This may be due to the extrinsic compression of celiac artery causing intimal hyperplasia resulting luminal narrowing of artery. […] However, further definitive studies are needed to address the pathophysiology, better diagnose and devise minimally invasive treatment for this entity.

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