Median Arcuate Ligament Syndrome Research Articles

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

Dunbar (or median arcuate ligament) syndrome: A case series

Dunbar syndrome (DS) (or median arcuate ligament [MAL] syndrome) is a rare entity of the vascular compression syndrome, where there is focal proximal coeliac axis compression by MAL. It results in an insufficient supply of blood to the respective organs of the gastrointestinal tract. Multidetector computed tomography is a very convenient, noninvasive modality in diagnosing this condition and helpful in distinguishing it from other conditions, such as atherosclerotic disease. DS can further be treated disorder surgically by relieving the compression and sometimes may need vascular reconstruction. We present five cases of the DS.

Median arcuate ligament syndrome

Chronic abdominal pain has several aetiologies, and many a times, the aetiology is one of the exclusions. Median arcuate ligament syndrome (MALS) also called coeliac artery compression syndrome is a form of chronic mesenteric ischaemia, although rare is an important cause. A 20-year-old male presented with recurrent episodes of pain abdomen, with a history of multiple admissions for the same, before the surgery. His physical examination and laboratories were normal, but the computed tomography (CT) done initially gave a doubtful diagnosis of MALS. A CT angiogram was done, which showed a definite kinking of the coeliac axis. After all the other causes of abdominal pain were ruled out, the patient was taken up for surgery with a provisional diagnosis of MALS. Intraoperatively, there was fibrosis around the coeliac axis with kinking. The fibrosis was cleared, and pulsations of the artery were confirmed. Postoperatively, the patient had an uneventful recovery.

Robot Assisted Laparoscopy for Median Arcuate Ligament Syndrome Relief

ObjectiveTo evaluate the outcomes of robot assisted laparoscopic surgery for median arcuate ligament syndrome (MALS) relief.MethodsThis was a single centre and retrospective study, including all consecutive patients with symptomatic MALS treated with robot assisted laparoscopic surgery. Symptom relief and quality of life (QoL) were evaluated post-operatively. A comparison between the peak systolic velocity (PSV) of the coeliac artery (CA) measured pre-operatively and post-operatively was carried out.ResultsNine interventions were performed. No conversion to laparotomy was required. There was post-operative abdominal pain relief in eight patients and QoL was improved in seven patients. Post-operatively, the CA PSV decreased (175 (IQR 160 - 195) cm/s vs. 365 (IQR 350 - 419) cm/s; p < .001).ConclusionMALS relief with robot assisted laparoscopy is safe and provides satisfactory outcomes in terms of symptom relief and CA compression release.

Clinical Response to Celiac Plexus Block Confirms the Neurogenic Etiology of Median Arcuate Ligament Syndrome

Single center, retrospective review between January 1, 2017, and April 3, 2020. Ninety-six patients (female, 75; mean age, 27 years) underwent 103 computed tomography (CT)‒guided percutaneous celiac plexus block (CPB) procedures for a presumptive diagnosis of median arcuate ligament syndrome (MALS). Technical success was achieved in 102 of 103 cases. No major adverse events were reported. The prevalence of postprandial nausea and vomiting significantly decreased after treatment (38% to 12%;16% to 5%, respectively). Findings of celiac artery compression were documented on CT scan in only 27% of these selected patients. The postprandial pain score decreased in 84% of patients overall; no differences were noted between patients with and without celiac artery compression. In patients with a clinical diagnosis of MALS, a large majority reported pain relief and decreased gastrointestinal symptoms after CPB. Pain relief did not correlate with the presence of celiac arterial stenosis. These findings support neuropathy as the primary etiology of MALS and suggests that the absence of celiac stenosis should not be used as an exclusion criterion.

Two Decades of Experience With Chronic Mesenteric Ischaemia and Median Arcuate Ligament Syndrome in a Tertiary Referral Centre: A Parallel Longitudinal Comparative Study.

BackgroundChronic mesenteric ischaemia (CMI) and median arcuate ligament syndrome (MALS) have similar clinical presentations with surgical intervention as the mainstay of treatment. However, surgical response varies and is unpredictable. Therefore, we aim to evaluate the technical and clinical success rates of selective revascularisation in older patients with CMI and younger patients with MALS undergoing arcuate ligament decompression with celiac sympathectomy.MethodsWe conducted a retrospective single-centre longitudinal comparative study of all the patients who underwent surgery for symptoms of CMI and MALS from December 2002 to 2020 at our tertiary referral vascular centre. Our primary outcome was symptom-free survival post-intervention. The secondary outcomes were perioperative mortality, technical success, and all-cause mortality at 17 years.ResultsWe operated on 28 patients; 17 patients with CMI (revascularisations with bypass) and 11 with MALS (decompression and celiac sympathectomy). All (100%) patients had technical success. There was no perioperative mortality. All the MALS patients had symptom-free survival following the procedure throughout follow-up. In contrast, three patients with CMI complained of recurring abdominal pain even after one year of the surgery. However, there was no further weight loss and none of them required any intervention.ConclusionStratified management of CMI with revascularisation and open surgical decompression with celiac sympathectomy in MALS are effective treatments with favourable long-term outcomes.

A Case of Chronic Mesenteric Ischemia: Complete Revascularization Using Multiple Procedures.

Chronic mesenteric ischemia (CMI) involving occlusion and/or stenosis of multiple mesenteric arteries is rare. We report our experience with a 66-year-old man who presented with a more than 3 months history of abdominal pain and vomiting/diarrhea. A diagnosis of CMI due to occlusion of the superior mesenteric artery (SMA) and severe stenosis of the celiac artery by median arcuate ligament syndrome was made. Complete revascularization through iliac artery–SMA bypass grafting and arcuate ligament dissection assisted with staged-catheter intervention successfully alleviated the patient’s symptoms. The patient has maintained a normal daily diet for 6 months postoperatively.

Median arcuate ligament syndrome: A case report of a rare disease.

IntroductionMedian arcuate ligament syndrome (MALS) is a rare condition caused by the compression of the celiac trunk by the median arcuate ligament, which may trigger a typical symptom triad: postprandial abdominal pain, weight loss, nausea and vomiting.Clinical caseA 58-year-old male was admitted to the emergency department due to abdominal pain, vomiting, fever and diarrhea. On admission, the patient was tachycardic, had abdominal distension, no peritoneal irritation signs, and a serum lactate level of 5 mmol/L. The computed tomography (CT) scan showed gastric and intestinal pneumatosis associated with pneumoportia. Conservative treatment with intravenous fluids, antibiotics and low molecular weight heparin, was initiated. The reassessment CT scan showed resolution of the gastric and intestinal pneumatosis and a severe stenosis of the initial portion of the celiac trunk with associated angulation. After recovery from the acute episode, an abdominal duplex ultrasound and an aortoiliac arteriography in forced inspiration and expiration was performed, corroborating the diagnosis of MALS. A laparoscopic decompression of the celiac trunk was performed.DiscussionSymptoms of MALS closely mimic other abdominal disorders, and it should be considered in the differential diagnosis. Currently, there are no international guidelines on MALS diagnostic criteria. Treatment is focused on decompression of the median arcuate ligament constriction of the celiac artery, with or without celiac lymph node removal.ConclusionMALS diagnostic and therapeutic approach must be patient focused. Laparoscopic decompression is an effective treatment and can provide immediate symptomatic relief, associated with the benefits of the less invasive nature of the procedure.

Vascular abnormalities visualized by multislice computed tomography of the abdomen: accidental findings or immediate causes of pain syndrome? (topic review)

Abnormalities of the abdominal aorta branches may cause chronic mesenteric ischemia, progressive pathological changes of the internal organs in this setting, and chronic pain syndrome. The causes of chronic mesenteric ischemia may be divided into atherosclerotic and non-atherosclerotic. Atherosclerosis of the unpaired branches of the abdominal aorta involves stenosis or occlusion. Other causes include fibromuscular dysplasia, vasculitis (Takayasu, segmental mediolytic arteriopathy), and median arcuate ligament syndrome. These syndromes, the pathogenesis of some of which remains controversial, lead to nonspecific complaints such as abdominal pain, weight loss, and others. Digital subtraction angiography or duplex ultrasound may provide hemodynamic information in cases of vascular disease in this area. However, multislice spiral computed tomography is in many cases the first choice because it allows for a comprehensive assessment of the state of blood vessels and associated morphological changes of internal organs. Structural changes accompanying these syndromes can also occur in patients who are undergoing a medical examination for other reasons. However, these syndromes should not be diagnosed solely on the basis of imaging; instead, the findings should be compared with the clinical presentation, which implies collaboration of radiologists and clinicians.

Diagnosis of Median Arcuate Ligament Syndrome with Intravascular Ultrasound

Diagnosis of Median Arcuate Ligament Syndrome with Intravascular Ultrasound

Chronic Mesenteric Ischemia: Differential Vascularsurgical Therapy and Its Outcome in a Single-Center Observational Study

Aim: The aim of this study was to investigate short-/long-term vascularsurgical patency and the outcome in chronic mesenteric ischemia (CMI) depending on the mesenteric revascularization technique and reflecting real-world data. Methods: This retrospective single-center observational study registered all patients who had undergone open vascularsurgical reconstruction because of CMI at a tertiary German university hospital comparing 1-versus (vs.) 2-vessel as well as antegrade versus retrograde reconstructions. Results: In total, 35 patients were enrolled (mean [± SD] age, 64 ± 13 [range, 45–83] years; sex ratio [m:f], 16:19 [46:54]) over 12 years. Three patients with symptoms of mesenteric ischemia because of rare causes (radiation-induced and median arcuate ligament syndrome) have been excluded. While 51% of patients underwent 1-vessel reconstruction, 49% underwent 2-vessel reconstruction. There was a trend of (i) more perioperative complications in the 2-vessel group (88.2% vs. 55.6%, p = 0.06) and (ii) higher morbidity at 1 year in the 2-vessel versus 1-vessel group (57.1% and 42.9%, respectively; p = 0.466), while the morbidity of the 2-vessel versus 1-vessel group at 5 years (100% vs. 33.3%) was significantly different (p = 0.009). The mortality was greater in the 2-vessel versus 1-vessel group as it was significantly different in the early postoperative period (31.3% vs. 0, p = 0.016) and at 1 year (50% vs. 0, p = 0.005) and 5 years (100% vs. 11%, p = 0.003). Regarding overall survival, the 1-vessel group showed a significant superiority above the 2-vessel group (p = 0.004). Actually, there was no significant difference of early postoperative morbidity comparing the retrograde and antegrade group (p = 0.285) as well as at 1 year and 5 years (p = 0.715 and p = 0.620, respectively). In addition, there was no significantly different postoperative mortality in antegrade versus retrograde group at each time. Specific and general complication rates were 62.9% and 57.1%, respectively, resulting in an overall morbidity of 77.1% (mortality, 20%). Conclusion: The vascular surgeon should be prepared to perform various procedures of mesenteric reconstruction to tailor the operative strategy to the specific needs of the individual patient.

Robotic versus laparoscopic median arcuate ligament (MAL) release: a retrospective comparative study.

Multiple retrospective studies have demonstrated the safety and feasibility of laparoscopic median arcuate ligament division with celiac neurolysis for the definitive management of median arcuate ligament syndrome (MALS). This study queries the clinical equipoise of robotic (RMALR) versus laparoscopic MAL release (LMALR) at a high-volume center. A retrospective analysis of consecutive 26 RMALR and 24 LMALR between March 2018 and August 2019 by a single surgeon at a quaternary academic institution was completed. Primary endpoint was postoperative decrease in celiac trunk expiratory peak systolic velocities (PSVs) measured by mesenteric duplex ultrasonography. Secondary outcomes included reported improvement in MALS-related clinical symptoms, distribution of first assistant seniority level, and involvement of second assistants in RMALR versus LMALR. Mean operative times for LMALR and RMALR were 86 and 134min, respectively (p < 0.0001). There were no open conversions and mean length of hospital stay was 1day for both cohorts. Both groups provided an equally effective decrease in postoperative peak systolic velocities (PSVs) (LMALR p = 0.0011; RMALR p = 0.0022; LMALR vs. RMALR p = 0.7772). While RMALR had significantly higher reduction of chronic abdominal pain postoperatively, there were no significant differences in other postoperative symptom relief between groups. However, RMALR patients reported significant relief of postprandial abdominal pain (p < 0.0001) and chronic nausea (p = 0.0002). RMALR had significantly more junior first assistants (p = 0.0001) and less frequently required second assistants compared to LMALR (p = 0.0381). In this study comparing RMALR to LMALR, postoperative chronic abdominal pain relief was significantly less in the former while other outcomes were equivalent. In comparison with LMALR, RMALR cases were associated with more junior first assistants, fewer second assistants, and longer operative times. Both approaches are safe and feasible for well-selected patients in experienced centers.

Median arcuate ligament syndrome – Current state of management

Symptomatic celiac artery compression syndrome (CACS) or median arcuate ligament syndrome (MALS) is a controversial diagnosis that should be considered in patients with chronic abdominal pain of unknown etiology despite an extensive medical evaluation. Once suspected, patients should undergo mesenteric duplex ultrasound. Diagnosis is confirmed with elevated celiac artery velocities which normalize with deep inspiration followed by CT angiogram showing the typical “J-hook” conformation of the celiac artery. Patients should then undergo evaluation by a multi-disciplinary team to appropriately select and prepare patients for potential surgical treatment. Surgical options include release of the median arcuate ligament, with or without neurolysis of the celiac nerve plexus, and with or without concomitant revascularization procedures. Approaches can be open, laparoscopic, or robotic. Surgical treatment has an overall success rate of 70-80% with patients reporting improved abdominal pain and quality of life. Post-operatively, patients can have persistent or recurrent abdominal pain and should undergo re-evaluation for possible need for a revascularization procedure for stenosis of the celiac artery or celiac plexus block if the celiac artery flow is normalized. Additionally, some patients will have persistent pain consistent with functional gastrointestinal disorder (FGID) that will then require medical management. Psychiatric comorbidities have been identified as a predisposing factor that may predict poorer outcomes, and there are preliminary findings suggesting that patients with dysautonomia diagnoses may have worse outcomes as well.

Surgical treatment of abdominal compression syndromes: The significance of hypermobility-related disorders.

Case reports and systematic studies of the most common hypermobility-related disorders, hypermobile Ehlers-Danlos syndrome (hEDS), and hypermobility spectrum disorder (HSD) typically describe gastroenterological symptoms and complaints attributed to structural malfunction, autonomic dysfunction, or inflammation of the gastrointestinal tract. However, abdominal compression syndromes (CS) may also contribute to pain and dysfunction in these individuals and be the leading pathology given symptoms significantly reduce or cease after decompressive surgery. Arising not only in the abdomen and causing pain (median arcuate ligament syndrome [MALS] and superior mesenteric artery syndrome [SMAS]), CS also occur in the retroperitoneum and the pelvis (nutcracker syndrome and May-Thurner syndrome), these latter conditions causing chronic pelvic congestion syndrome (PCS). Here, we report primarily on our experience of the assessment and management of MALS and SMAS in a cohort of cases with a surprising prevalence of HSD and hEDS. To our knowledge, this is the first cohort report of its kind in hEDS, HSD, and CS. We recommend that CS are considered in hEDS and HSD individuals with gastrointestinal and other painful complaints within the "belt" area. These CS can be identified using functional ultrasound duplex examination in experienced hands, and in appropriate cases stabilizing surgery can substantially improve quality of life.

Targeting Coping to Improve Surgical Outcomes in Pediatric Patients With Median Arcuate Ligament Syndrome: Feasibility Study.

Background: Median arcuate ligament syndrome (MALS) is a vascular compression syndrome leading to postprandial epigastric pain, nausea, and weight loss; it can be treated surgically. While most patients report improved quality of life following surgical intervention, 30% continue to experience chronic abdominal pain. Pre-surgical diagnoses of depression and/or anxiety have been found to significantly predict post-surgical: quality of life, highest experience of pain, anxiety, and parent- and self-reported coping strategies. As such, increasing the coping strategies of pediatric patients with MALS may impact their post-surgical outcomes. The purpose of the current study was to: (1) implement a pre-operative cognitive behavioral therapy protocol with a focus on psychoeducation and coping strategies; and (2) determine feasibility of a pre-surgical intervention for this population.Method: Children (<18 years of age) with a diagnosis of MALS who were eligible for surgical intervention were invited to participate in a 7-week in-person or video-based pre-surgical cognitive behavioral therapy intervention. Psychiatric comorbidities were assessed at baseline and post-surgery; patient-reported distress, pain interference and intensity, health-related quality of life, and health status were assessed at four time points (baseline, week 4, week 7, and post-surgery). Descriptive analyses were used to characterize the sample, assess feasibility outcomes (i.e., attrition rates), and explore symptom-based outcomes across time.Results: Twelve pediatric patients (M age = 15.2 ± 1.7; 91.7% female) and their parents (91.7% mothers) participated. Feasibility metrics based on protocol completion were exceeded for engagement at the stages of consent (68.4% vs. goal of ≥50%), treatment initiation (92.3% vs. 85%), and treatment completion (84.6% vs. 75%). Out of the 12 participants, nine (75%) met criteria for at least one comorbid psychiatric diagnosis at baseline and nine (75%) elected to undergo MALS surgery after completing the intervention.Conclusion: The intervention implementation was feasible, despite chronic pain symptoms experienced by the sample, a high prevalence of psychiatric diagnoses, and an international pandemic, suggesting that it would be beneficial to further evaluate the efficacy of the intervention. Future research should include stakeholder input in the design, deployment, and evaluation of a pilot efficacy trial of pre-surgical cognitive behavioral therapy for pediatric patients with MALS.

Celiac Plexus Block: A Preoperative Procedure to Improve Surgical Outcomes in Median Arcuate Ligament Syndrome

Schneck, Matthew BS; Goudreau, Bernadette MD; Uribe, Celso II MD; Tracci, Margaret MD, FACS Author Information

Surgical Treatment of the Neurogenic Etiology of Median Arcuate Ligament Syndrome

Journal of the American College of Surgeons: November 2021 - Volume 233 - Issue 5 - p S324-S325 doi: 10.1016/j.jamcollsurg.2021.07.675

Laparoscopic treatment of rare median arcuate ligament syndrome - medium-term follow-up.

&lt;b&gt;Introduction:&lt;/b&gt; Median arcuate ligament syndrome (MALS) is a rare cause of chronic epigastric pain. The presentation might be unclear and non-specific. Diagnosing the syndrome requires interdisciplinary methods and specialists. Treatments consist of celiac axis release performed laparoscopically or robotically, and intraluminal stenting. &lt;/br&gt;&lt;/br&gt; &lt;b&gt;Aim:&lt;/b&gt; The aim of the study was to report the medium-term postoperative follow-up results for four patients with MALS. &lt;/br&gt;&lt;/br&gt; &lt;b&gt;Materials and methods:&lt;/b&gt; We performed 5 laparoscopic celiac axis releases in patients with MALS in our department in 2018. The study included 4 patients who were admitted 16-23 months after surgery for computed tomography angiography.&lt;/br&gt;&lt;/br&gt; &lt;b&gt; Results:&lt;/b&gt; Patients constituted 4 women aged 28-63 with a mean body mass index of 22.4 kg/m2. The diagnosis of MALS was confirmed by computed tomography angiography, which showed severe (&gt;70%) narrowing of the celiac axis. Patients underwent laparoscopic celiac axis release, and were all discharged on the first postoperative day with no postoperative complications. The patients' quality of life improved and their symptoms subsided completely. Follow-up computed tomography angiography confirmed full decompression of the celiac axis in all 4 patients, with no stenosis caused by scarification of the celiac axis.&lt;/br&gt;&lt;/br&gt; &lt;b&gt; Conclusion:&lt;/b&gt; Laparoscopy is a valuable and safe method to treat patients with MALS.

Standardized Approach to Median Arcuate Syndrome and Laparoscopic Release: An Institutional Experience

Median arcuate ligament syndrome is a rare disorder characterized by compression of the celiac axis and plexus that is both challenging to diagnose and treat. Recently, laparoscopic approaches to median arcuate ligament release (MALR) have been described, with promising outcomes, emphasizing the importance of ligament release, in conjunction with celiac plexus ganglionectomy for postoperative symptom relief. The purpose of the present study was to evaluate our institutional experience and outcomes with laparoscopic MALR using a standardized approach for the diagnostic workup, surgical technique, and perioperative management for patients with suspected median arcuate ligament syndrome.

911 Delayed Presentation of a Re-Bleed from A Traumatic Splenic Artery Rupture Following Endovascular Management in A Patient with Median Arcuate Ligament Syndrome

Abstract Endovascular management of splenic blunt trauma is widely accepted as a safe and effective alternative to open surgery in carefully selected patients. Following a radiologically successful intervention, patients are normally discharged with no follow up after 48-72 hours of haemodynamic stability and satisfactory serial haemoglobin levels. We present a case of a fit and well 24-year-old male patient who presented with abdominal pain, syncope and haemodynamic instability 14 days post successful splenic artery coil-embolization for splenic artery aneurysm rupture secondary to blunt trauma. After initial resuscitation, computed tomography angiography was performed and demonstrated active bleeding from the splenic artery aneurysm which was deemed likely to be a consequence of retrograde filling. The patient underwent successful emergency re-embolization using a combination of embolization coils and Onyx via a trans-splenic approach to eliminate retrograde flow. On further review of the imaging, it was incidentally noted there was evidence suggesting a diagnosis of median arcuate ligament syndrome, which may have predisposed the patient to splanchnic artery aneurysm formation. This case report highlights a potential limitation of endovascular management compared to open surgery and summarises the literature surrounding splenic artery anatomical variations and the implications of median arcuate ligament syndrome. A re-bleed following embolization is a hostile prospect with potentially catastrophic outcomes for patients if not recognised quickly. The authors propose that interval re-imaging should be considered following endovascular management of blunt splenic trauma.