Median Arcuate Ligament Syndrome Research Articles

Plasma α-Glutathione S-Transferase in Patients with Chronic Mesenteric Ischemia and Median Arcuate Ligament Syndrome

BackgroundChronic mesenteric ischemia (CMI) due to either atherosclerosis of the mesenteric arteries or median arcuate ligament syndrome (MALS) is an underdiagnosed entity. The etiology of MALS and its existence have been debated and questioned. We aimed to identify plasma biomarkers indicating mesenteric ischemia in patients with CMI and MALS.MethodsPlasma α-glutathione S-transferase (α-GST), intestinal fatty acid-binding protein (I-FABP), citrulline, and ischemia modified albumin (IMA) were analyzed in fifty-eight patients with CMI (Group A, n=44) and MALS (Group B, n=14) before and after revascularization. The plasma levels of these potential biomarkers were compared with those of healthy individuals (Group C, n=16). Group comparison was performed with the Mann–Whitney U-test. Cross-tabulation and its derivatives were obtained. Receiver operating characteristic (ROC) curves and area under the curve (AUC) were calculated.ResultsPlasma levels of α-GST were significantly raised in the patients with CMI (7.8 ng/mL, p<0.001) and MALS (8.4 ng/mL, p<0.001), as compared with the control Group C (3.3 ng/mL). The threshold for normal median plasma α-GST levels of 4 ng/mL yielded a sensitivity of 93% and 86%, specificity of 86% and 88%, respectively, for the diagnosis of CMI due to atherosclerosis and MALS. AUC of ROC curves was 0.96 (p<0.0001) for CMI and 0.85 (p<0.002) for MALS. The patient groups did not differ from the healthy controls in any other biomarkers.ConclusionPlasma α-GST levels are elevated in CMI and MALS patients. Elevated plasma levels of α-GST suggest ischemia as the etiology of MALS.

Gabapentin as a Symptomatic Modifier in Median Arcuate Ligament Syndrome: A Case Report and Assessment of Treatment Modalities

Median arcuate ligament syndrome (MALS) is a rare constellation of neurogenic gastrointestinal (GI) symptoms resulting from compression of the celiac trunk and celiac plexus by the median arcuate ligament. MALS is characterized by nonspecific symptoms including nausea, vomiting, diarrhea, bloating, unintentional weight loss due to food aversion, and postprandial epigastric abdominal pain. We present a case of atypical, chronic MALS that confounded clinicians for over a decade and led to various misdiagnoses, including early-onset Parkinson’s disease. Of the constellation of symptoms that MALS may present with, postprandial epigastric pain is a classic symptom and increases the index of suspicion for the diagnosis; however, the absence of the classic symptom of postprandial epigastric pain and the predomination of nonspecific GI symptoms and syncope in our patient further clouded clinicians’ ability to diagnose MALS. Upon further investigation, we elucidated a link between gabapentin, which our patient was chronically prescribed, and its efficacy in decreasing neurogenic hypersensitivity in the GI tract. Our case and the implications of gabapentin use to decrease neurogenic pain from MALS represents a novel addition to the literature on MALS treatment modalities and elucidates new avenues for continued research in the use of gabapentin as a symptom-modifying agent in the nonoperative and preoperative treatment of MALS.

Median Arcuate Ligament Syndrome with Common Hepatic and Splenic Arteries Directly Originating from the Abdominal Aorta: A Case Report

Introduction: Knowledge of the unpaired abdominal vasculature is important for uncomplicated operations. The absent celiac trunk has previously been reported; however, developmental alterations can result in a variety of branching patterns of the downstream arteries from the abdominal aorta. Case Presentation: A 53-year-old female patient with lymphoma underwent abdominopelvic computed tomography (CT) for staging. She had a history of postprandial off-and-on abdominal pain since her youth. CT scan revealed an absent celiac trunk with common hepatic and splenic arteries directly originating from the abdominal aorta. Compression of the median arcuate ligament had narrowed and angulated the 2 branches. Conclusions: When the celiac trunk is absent, its main branches may separately arise from the abdominal aorta. Also, due to the displacement of these branches, their path and diameter can vary from the normal.

Imaging Signs of Median Arcuate Ligament Syndrome

Imaging Signs of Median Arcuate Ligament Syndrome

Median Arcuate Ligament Syndrome and Concomitant Autonomic Dysfunction Pathology—Case Reports and Penn Treatment Algorithm

Median arcuate ligament syndrome (MALS) causing compression of the celiac artery can be found as an incidental finding on imaging studies. Postural orthostatic tachycardia syndrome (POTS) is a syndrome of autonomic dysfunction that can be associated with the finding of MALS. The objective of this study was to determine whether celiac artery compression by MALS is contributory to the pathophysiology of POTS in such patients. We describe our series of three patients with MALS and POTS who had undergone provocative vasodilator angiography and subsequent surgical decompression. We reviewed their symptoms before and after surgery. The patients were predominantly women in their third decade of life. Symptoms of abdominal pain with associated nausea and weight loss typically lasted from 6 months to 2 years. All three patients with MALS and POTS had complete resolution of their symptoms of POTS after surgical decompression. Surgical decompression of the median arcuate ligament with careful neurolysis of the celiac plexus was associated with complete symptomatic relief of POTS in our three patients. A multidisciplinary approach involving primary care, gastroenterology, cardiology, psychiatry, general surgery, and vascular surgery is essential to providing an accurate diagnosis and treatment of this rare association of inherently challenging conditions.Fig 2View Large Image Figure ViewerDownload Hi-res image Download (PPT)

Outcomes of Median Arcuate Ligament Release in an International, Multi-Institutional Cohort

Median arcuate ligament syndrome (MALS) is an uncommon disease diagnosed and treated by several surgical specialties. Prior research is limited to institutional case series, making the optimal approach to median arcuate ligament release (MALR) unclear. The objective of this study is to compare the outcomes of approaches to MALR.

Median Arcuate Ligament Release with Celiac Ganglionectomy for Management of Chronic Disabling Abdominal Pain in Patients With and Without Celiac Artery Compression - A Case Series

The current accepted surgical treatment for median arcuate ligament syndrome (MALS) is by division or release of the MAL (MALR), but current treatment strategies are known to remain inconsistently effective with variable recurrence rates. Treatment algorithms typically rely on the demonstration of celiac artery compression before offering patients surgical intervention. We sought to decrease reliance on abnormal arterial imaging as inclusion criteria and to see whether ganglionectomy in patients without celiac compression would provide symptom relief.

Laparoscopic surgical treatment of median arcuate ligament syndrome with the retrograde division technique: a case report

Median arcuate ligament syndrome is a rare entity. This clinical condition develops by compression of the root of a celiac artery with the median arcuate ligament. The typical triad of this syndrome is the following; abdominal discomfort and pain, especially after a meal, and weight loss. In diagnosis, other causes should be ruled out and compression must be demonstrated by any type of imaging method. The main principle of treatment is cutting down the median arcuate ligament. A 54-year-old woman presented with untreatable recurrent abdominal pain and was diagnosed with median arcuate ligament syndrome by imaging with angiographic computed tomography. This patient was operated on. We performed laparoscopic division of median arcuate ligament with the retrograde surgical dissection technique. The patient was discharged from the hospital without any complaint on the third day after surgery. She was still symptom-free after 12 months. The laparoscopic retrograde dissection approach is a safe and feasible treatment modality for median arcuate ligament syndrome.

Surgical Treatment of Wilkie's Syndrome by Vascular Transposition.

IntroductionSuperior mesenteric artery syndrome (SMAS), also called mesenteric duodenal compression syndrome, Wilkieʼs syndrome, chronic duodenal ileus or cast syndrome, is a rare clinical condition defined as a compression of the third portion of the duodenum in between the SMA and abdominal aorta (AA), due to narrowing of the space between them. SMAS is primarily attributed to loss of the intervening mesenteric fat pad, leading to partial or complete duodenal obstruction. Its manifestations are complex and non-specific, including postprandial epigastric pain, nausea, vomiting, early satiety, weight loss and anorexia. SMAS may present as an acute syndrome, or it may have an insidious onset with chronic symptoms. SMAS mainly affects females between 10 and 40 years of age. This study aims to discuss the safety and efficacy of vascular decompression of the duodenum by infrarenal transposition of SMA.MethodsThis single-centre prospective clinical study analysed 37 patients with Wilkie’s syndrome who underwent infrarenal transposition of the SMA between January 2012 and December 2021. The indications for the surgery were severe weight loss, uncontrolled upper abdominal pain, vomiting and other gastrointestinal (GI) symptoms that were severely debilitating to patients’ daily lives, along with radiological findings such as aortomesenteric angle < 25°, aortomesenteric distance <8 mm and distention of proximal part of the duodenum and the stomach. Ten patients (27%) concurrently had Nutcracker syndrome and seven patients (18.9%) had Dunbar syndrome (median arcuate ligament syndrome). Three female patients (8.1%) had all three above-mentioned vascular compression syndromes, which were treated in the same surgery. One male patient (2.7%) was after a laparoscopic duodenojejunostomy with symptoms that relapsed three months postoperatively, which was cured after the infrarenal transposition of SMA.ResultsTechnical operative and clinical success were achieved in all patients. There were no cases of anastomotic failure, SMA thrombosis or intestinal ischemia. All of the patients are currently living symptom-free. One patient (2.7%), four days postoperatively, had a lymphocele formed in the retroperitoneum, which was successfully drained by a CT-guided percutaneous pigtail catheter. Another patient (2.7%) after three months of surgery needed a re-laparotomy for adhesive obstruction of the second part of the duodenum and was treated by adhesiolysis and omentoplasty. One patient (2.7%), 2-year postoperatively, had a proximal SMA stenosis up to 60% where drug-eluting balloon percutaneous transluminal angioplasty (DEB PTA) was performed successfully. Finally, the upper GI symptoms were resolved in all 37 patients (100%).ConclusionWilkie’s syndrome, although rare, is frequently late-diagnosed or underdiagnosed. In cases of failure of conservative therapy, infrarenal transposition of the SMA can be considered a safe and feasible surgical option with more physiologically favourable outcomes comparable to gastrointestinal bypasses, especially in patients concurrently suffering from Nutcracker syndrome. Simultaneously, it also restores physiologic duodenal passage of gastroduodenal content without the need of creating a digestive tract anastomosis. To our best knowledge, we have the highest number of SMA transposition surgeries performed in a single centre for the treatment of Wilkie’s syndrome.

A case report on median arcuate ligament syndrome: Need for a high index of suspicion

The Median Arcuate Ligament Syndrome (MALS) is a rare clinical entity occurring due to abnormally low insertion of the ligament across the origin of the coeliac axis resulting in varying degrees of celiac artery compression. Its symptoms mimic those of chronic mesenteric ischemia and its diagnosis requires a high index of clinical suspicion coupled with appropriate imaging. We report the case of a young male with pain abdomen, nausea, and vomiting in whom routine evaluation for pain abdomen was inconclusive. He was diagnosed with MALS after multi-modality imaging. The patient was suspected to have MALS by using ultrasound and color Doppler imaging. These findings were confirmed using Computed tomography angiography (CTA) of the abdomen. MALS should be considered as an important differential in patients with symptoms resembling post-prandial angina. A combination of a high index of suspicion with the use of appropriate imaging techniques can clinch this diagnosis. We describe the diagnostic challenges faced in this case with a brief review of the literature.

Laparoscopic treatment of symptomatic Dunbar syndrome: a case report.

Introduction and aim of the studyDunbar syndrome is a rare disorder due to the external compression of the celiac trunk by the median arcuate ligament. The symptoms include abdominal pain, nausea and vomiting with a significative reduction in patients' quality of life. Laparoscopy has proven to be a safe procedure. The aim of this study is to present a case of Dunbar syndrome undergoing laparoscopic surgery.Case presentationA 40-year-old male patient presented at Emergency Room with upper abdominal pain and dyspepsia, related to food intake. A selective arteriography of the celiac trunk revealed stenosis due to compression of the celiac artery.Clinical discussionLaparoscopic decompression of the celiac trunk by the median arcuate ligament was performed. Postoperative course was uneventful and the patient was discharged on the 5th postoperative day.ConclusionMinimally invasive surgical division of the arcuate ligament is feasible and safe and it can restore patients' quality of life.

Patient-Reported Experiences With Median Arcuate Ligament Syndrome: Resilience and Resources Required.

Median arcuate ligament syndrome (MALS) is a vascular condition that is treated surgically. MALS is a diagnosis of exclusion and therefore often requires that pediatric patients undergo an exhaustive number of tests and potential experiences with misdiagnosis before receiving an accurate diagnosis and treatment. The purpose of the current mixed-methods study was to assess the pediatric patient- and parent-reported experience of (a) coping with and managing symptoms associated with MALS and (b) the diagnostic, treatment, and recovery process for pediatric MALS. Nine postsurgical pediatric patients (M age = 20.11 ± 3.59; all <18 at time of surgery) and 6 parents participated in semistructured focus groups and completed self-report questionnaires about their experiences and current functioning. Four themes emerged: (a) the impact of MALS (physical and psychosocial), (b) uncertainties and feeling misunderstood, (c) extensive medical tests, and (d) mental health impacts, with an additional three themes related to specific querying (i.e., appreciation, recommendations, and interdisciplinary treatment team). With the exception of variable long-term physical impacts, patients and their parents reported fairly consistent experiences, and the content of these themes was consistent with self-reported quantitative data. The current study highlights the resilience and resources seemingly required for effective diagnosis and intervention for pediatric MALS. The need for increased knowledge about MALS, the inclusion of an interdisciplinary treatment team, and long-term follow-up for pediatric patients with MALS are implicated.

Chronic Abdominal Pain, an Overlooked Diagnosis of Median Arcuate Ligament Syndrome (MALS)

Median arcuate ligament syndrome (MALS) is a rare condition that is often overlooked as a result of its nonspecific symptoms. It is usually presented with nausea, bloating, abdominal postprandial pain, and weight loss. The diagnosis of MALS is usually delayed and made by excluding other causes for the symptoms. The diagnosis of this syndrome is based on clinical presentation and radiological findings on computer tomography angiography (CTA) or magnetic resonance angiography (MRA). Surgery is treatment of choice, with promising results from laparoscopic surgery.

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

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

Vascular compression syndromes: a pictorial review.

Vascular compression syndromes include a group of rare vascular changes due to extrinsic compression of veins or arteries by surrounding structures. These pathologies are often underestimated due to their rarity, clinicians’ poor level of knowledge, and the non-specificity of their symptoms. The best known are Eagle syndrome, thoracic outlet syndrome, nutcracker syndrome, May-Thurner syndrome, Dunbar syndrome, and popliteal entrapment syndrome. This work summarizes the main ultrasonographic characteristics, symptoms, and treatments of choice for these syndromes. Knowledge of these conditions’ characteristic signs is essential for the differential diagnosis. Failure to diagnose these rare diseases can expose patients to serious complications and risks to their health.

Severe Post-prandial Pain: A Case Report

Median arcuate ligament syndrome (MALS) is a rare cause of post-prandial abdominal pain due to compression of the celiac artery and celiac plexus. Associated symptoms include nausea, vomiting, diarrhea, and weight loss. The incidence of radiologic compression of the celiac axis is reported to be between 10% and 24%; however, symptomatic compression is noted to be found in about half of the population. MALS is considered a diagnosis of exclusion due to its tendency to present with nonspecific symptoms that mimic other common causes of abdominal pain. Radiologic evidence from angiography with breathing maneuvers is the gold standard for diagnosis. Surgical division of the median arcuate ligament to decompress the celiac artery is an effective treatment proving to provide up to 60-70% of symptomatic relief.

“MEDIAN ARCUATE LIGAMENT SYNDROME’’ -A RARE CASE REPORT

Background: The median arcuate ligament passes superior to the origin of the celiac artery and is a continuation of the posterior diaphragm that wraps over the aorta. If it lies too low on the aorta or if the origin of celiac artery is higher in location, the ligament may cause symptoms of abdominal pain related to compression of the celiac artery. Case Report: An abdominal ultrasound in a 20-year-old woman with longstanding abdominal pain after eating and Computed tomography angiography of the abdomen showed stenosis of the origin of the celiac artery and conrmed the diagnosis of median arcuate ligament syndrome. Laparoscopic release of the median arcuate ligament resulted in relief of the patient's symptoms. Conclusion: The diagnosis of median arcuate ligament syndrome should be considered in patients with postprandial abdominal pain that does not have a clearly established etiology.

A diagnostic workup and laparoscopic approach for median arcuate ligament syndrome.

BackgroundMedian arcuate ligament syndrome (MALS) is a rare disorder characterized by the compression of the coeliac trunk and plexus by fibrous arches of the median ligament. It commonly occurs in young women with postprandial epigastric pain, weight loss and nausea. We present a single surgeon experience on the diagnostic approach and management of MALS with a focus on laparoscopic surgery.MethodsA retrospective review of adult patients diagnosed with MALS during a 10‐year period (2011–2021) was conducted at Bankstown‐Lidcombe hospital in New South Wales, Australia.ResultsMALS was diagnosed in six patients (mean 46 years, range: 27–74 years old), all confirmed on mesenteric duplex ultrasound and computed tomography angiography. The most common presentations were women with post‐prandial pain, exercise induced pain and an average weight loss of 14.5 kg. The median interval from onset of symptoms to surgical referral was 10.5 months. The average BMI was 24.1 kg/m2 and most had a grade III American Society of Anaesthesiologist physical status. All patients underwent laparoscopic release of median arcuate ligament with one patient requiring endovascular stenting. The mean operative time was 119 minutes with two minor post‐operative complications, but no mortalities. The median hospital length of stay was 3.5 days with a median follow up of 3.5 years.ConclusionLaparoscopic median arcuate ligament release with endovascular support for selected cases provides sound clinical resolution of symptoms and long‐term results.

Median Arcuate Ligament Syndrome is Effectively Relieved with Minimally Invasive Surgery.

Median arcuate ligament (MAL) syndrome is a constellation of symptoms related to compression of the celiac artery trunk. Minimally invasive release of the ligament has been shown to improve these symptoms. This study describes one institution's experience with this procedure and reports on outcomes of minimally invasive release and patient quality of life. We performed a retrospective chart review of all patients who underwent minimally invasive release of the MAL at our institution. Patients were mailed a survey consisting of the 36-Item Short Form and Visick questionnaires. If surveys were not returned after one month, patients were called and asked to complete them over the phone. Demographic and pre- and postoperative data were collected and analyzed. Eleven patients underwent a laparoscopic MAL release from January 1, 2015 to January 31, 2020. Most patients, 73%, reported epigastric pain as their primary symptom for a median of 18 months. All cases were successfully completed laparoscopically, with only one intraoperative complication. Mean hospital length of stay was 1.4 d. At the time of survey completion, the mean weight change was 2.3 kg. Additional interventions for resolution of symptoms and celiac artery stenosis were required for two patients. Surveys were completed by eight patients. A mean Visick score of 1.8 showed resolution or improved symptoms for all patients. SF-36 scores were highest for physical functioning, emotional well-being, and social functioning health areas. Minimally invasive release of the MAL is a safe and effective surgery for patients suffering from MALS. Symptoms improved after adequate release of the ligament, with minimal morbidity and additional postoperative procedures needed.

Median arcuate ligament syndrome with predominantly respiratory manifestatios

Introduction. One of the causes of respiratory disorders in young people may be visceral circulation chronic vio-lation in median arcuate ligament syndrome (MALS). The aim of the study was to assess the clinical significance of respiratory disorders in establishing the MALS diagnosis in order to determine the necessary management tactics for patients with this pathology. Materials and methods.49 MALS patients were examined, the main symptoms being respiratory disorders, such as shortness of breath, difficult inhaling and/or exhaling, and others. The medi-an age was 24.7±0.9 years, females predominated (60%). The diagnosis of MALS was confirmed by clinical data, the results of transabdominal ultrasound duplex scanning of the abdominal aorta and its visceral branches using re-spiratory and orthostatic tests. Subsequent comparative analysis of the characteristic respiratory symptoms’ seve-rity before and after surgery (decompression of the celiac trunk) was performed. Research results. 10 main symp-toms of respiratory disorders were identified in patients under study, the frequency of each ranged from 31% to 50%, in most patients they were combined. Spirometry in these patients did not reveal any changes in both static lung volumes or airway patency. Bronchodilation revers-ibility tests were negative. Chest X-ray and/or computed tomography did not reveal any visible changes. During examination, hemodynamically significant compres-sion stenosis of the celiac trunk (CT) was observed in all patients. For all patients, auscultation of the abdominal cavity and detection of systolic murmur were performed. Confirmation of the diagnosis was based on ultrasound of the unpaired visceral arteries with color mapping, im-aging of the severity of CT stenosis, nature and speed of blood flow. An ultrasound study of the diaphragm func-tion revealed a direct statistically significant relationship between the diameter of the orifice of the CT and the am-plitude of the respiratory displacement of the diaphragm dome during quiet breathing (r=0.58), the amplitude of the respiratory displacement of the diaphragm dome during forced breathing (r=0.64), and the thickening frac-tion of the muscular part of the diaphragm, with quiet breathing (r=0.61) and thickening fraction of the muscu-lar part of the diaphragm during forced breathing (r=0.46, not significant, trend). The given correlation coefficients were estimated as a medium-strength relationship, i.e., the narrower the CT mouth, the worse the diaphragm ki-netics. In most patients, after surgical treatment, decom-pression of the celiac trunk, the clinical manifestations of respiratory disorders resolved, which may indicate their connection with diaphragmatic compression stenosis of the CT. Conclusion. The clinical manifestations of respi-ratory disorders in MALS, in most cases, are similar to the symptoms of bronchial asthma, which often led to the establishment of an erroneous diagnosis and ineffective-ness of the prescribed therapy. This theoretically and clin-ically important circumstance must be taken into account in the differential diagnosis of celiac trunk compression syndrome in combination with respiratory disorders. In the presence of hemodynamically significant compres-sion stenosis of the CT and respiratory disorders, it is ad-visable to perform surgical intervention