Median arcuate ligament syndrome

BackgroundMedian arcuate ligament syndrome (MALS) describes the clinical presentation associated with direct compression of the celiac artery by the median arcuate ligament. The poorly understood pathophysiologic mechanism, variable symptom severity, and unpredictable response to treatment make MALS a controversial diagnosis.MethodsThis review summarizes the literature pertaining to the pathophysiologic mechanism, presentation, diagnosis, and management of MALS. A suggested diagnostic workup and treatment algorithm are presented.ResultsIndividuals with MALS present with signs and symptoms of foregut ischemia, including exercise-induced or postprandial epigastric pain, nausea, vomiting, and weight loss. Consideration of MALS in patients' diagnostic workup is typically delayed. Currently, no group consensus agreement as to the diagnostic criteria for MALS exists; duplex ultrasound, angiography, and gastric exercise tonometry are used in different combinations and with varying diagnostic values throughout the literature. Surgical management involves decompression of the median arcuate ligament's constriction of the celiac artery; robotic, laparoscopic, endoscopic retroperitoneal, and open surgical intervention can provide effective symptom relief, but long-term follow-up data (>5 years) are lacking. Patients treated nonoperatively appear to have worse outcomes.ConclusionsMALS is an important clinical entity with significant impact on affected individuals. Presenting symptoms, patient demographics, and radiologic signs are generally consistent, as is the short-to medium-term (<5 years) response to surgical intervention. Future prospective studies should directly compare long-term symptomatic and quality of life outcomes after nonoperative management with outcomes after open, laparoscopic, endoscopic retroperitoneal, and robotic celiac artery decompression to enable the development of evidence-based guidelines for the management of MALS.     

Laparoscopic decompression as treatment for median arcuate ligament syndrome

Median arcuate ligament syndrome (MALS) is a rare disorder due to coeliac trunk compression by the median arcuate ligament, resulting in coeliac artery stenosis characterised by chronic, recurrent abdominal pain. Patients with MALS are often middle-aged females presenting with a triad of postprandial epigastric pain, weight loss and abdominal bruit. It is a diagnosis of exclusion and confirmed by computed tomography or magnetic resonance imaging. Laparoscopic or open surgical decompression are the only treatment options in MALS. We present two cases of MALS treated by laparoscopic decompression as well as a literature review on this treatment.     

Pediatric median arcuate ligament syndrome: first report of familial pattern and transperitoneal laparoscopic release

Median arcuate ligament syndrome (MALS) is a rare disorder resulting from luminal narrowing of the celiac artery by the insertion of the diaphragmatic muscle fibers. Surgical management entails division of the median arcuate ligament with or without celiac artery reconstruction. We are presenting an interesting case of a 16-year-old girl with postprandial abdominal pain and weight loss. Her mother also had MALS treated via open celiotomy with complete median arcuate ligament division and patch angioplasty of the celiac artery owing to persistent stenosis at our institution. After a diagnosis of MALS was confirmed in our patient, a transperitoneal laparoscopic release of the median arcuate ligament with skeletonization of the celiac artery and branch vessels was performed. The postoperative course was uneventful, and she was dismissed on postoperative day 2. She remains asymptomatic at 12-months follow-up. This represents the first report of a transperitoneal laparoscopic approach to MALS in an adolescent and the first report of a familial/generational component to MALS.     

Seconds from disaster: lessons learned from laparoscopic release of the median arcuate ligament

Median arcuate ligament syndrome (MALS) is a rare entity that manifests as abdominal pain, nausea, vomiting, and diarrhea. The median arcuate ligament is a fibrous band that connects the crura of the diaphragm. In some people, the ligament is positioned in a way that compresses the celiac axis, which in a subset of individuals causes the symptoms associated with MALS. Surgical release of the ligament can relieve these symptoms. After viewing a video that described the laparoscopic median arcuate ligament release technique at the 2006 SAGES meeting and reviewing the online video, we report our experience with two cases and discuss the lessons learned in performing the procedure within a training program. We also discuss the extent to which surgical resident participation contributes to intraoperative complications during a new and complex surgery. Electronic supplementary material  The online version of this article (doi:) contains supplementary material, which is available to authorized users.     

Laparoscopic management of the median arcuate ligament syndrome

Background: The median arcuate ligament syndrome (MALS) is an infrequent cause of abdominal pain. This diagnosis is made after exclusion of other more common causes of upper abdominal symptoms. Mesenteric duplex and a computerized tomography mesenteric angiography demonstrate dynamic compression of the coeliac axis during expiration. Methods: Retrospective analysis of presenting symptoms, preoperative findings and postoperative outcomes. Results: Five consecutive patients who underwent laparoscopic division of the median arcuate ligament over a 4‐year period (2006–2010) are presented. This procedure was associated with low morbidity and complete relief of symptoms in all patients. Conclusion: A minimally invasive procedure is the treatment of choice in selected patients with MALS.     

Histoplasmosis as a possible cause of retroperitoneal fibrosis and median arcuate ligament syndrome: A case report

INTRODUCTION

Median arcuate ligament syndrome (MALS), a condition of poorly understood etiology, is caused by compression of the celiac artery by fibers of the median arcuate ligament.

PRESENTATION OF CASE

A 46-year-old man with chronic abdominal pain and weight loss was diagnosed with MALS and admitted for surgery. During surgery, extensive retroperitoneal fibrosis around the celiac artery and adjacent aorta was noted. Large necrotizing granulomas and budding yeast, both indicators of histoplasmosis, were found on pathologic evaluation of retroperitoneal tissue removed during surgery.

DISCUSSION

Histoplasma capsulatum may cause pulmonary fibrosis and fibrosing mediastinitis, and the organism may disseminate to reach various internal organs in the immunocompromised individual. Retroperitoneal histoplasmosis has been demonstrated in immunocompromised individuals in the past without retroperitoneal fibrosis. Our patient may have had chronic histoplasma infection of his retroperitoneal lymph nodes, triggering fibrosis in the area surrounding the adjacent celiac artery and abdominal aorta, thus leading to symptomatic MALS.

CONCLUSION

Histoplasma capsulatum, an organism that has demonstrated its capability to cause fibrosis elsewhere in the body, may play a causative role in MALS in our patient, given the finding of retroperitoneal fibrosis in conjunction with retroperitoneal lymph node histoplasma.     

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

The median arcuate ligament syndrome (MALS) is recognized as a rare clinical entity, characterized by chronic post-prandial abdominal pain, nausea, vomiting, and unintentional weight loss. Due to its vague symptomatology, it is mainly regarded as a diagnosis of exclusion. Patients can often be misdiagnosed for several years before a correct diagnosis is established, also due to a medical team’s clinical suspicion. We present a case series of two patients who suffered from MALS and were treated successfully. The first patient is a 32-year-old woman, presenting with post-prandial abdominal pain and weight loss that have lasted for the past ten years. The second patient, a 50-year-old woman, presented with similar symptomatology, with the symptoms lasting for the last five years. Both cases were treated by laparoscopic division of the median arcuate ligament fibers, which alleviated extrinsic pressure from the celiac artery. Previous cases of MALS were retrieved from PubMed, to assemble a better diagnostic algorithm and propose a treatment method of choice. The literature review suggests an angiography with a respiratory variation protocol as the diagnostic modality of choice, along with the laparoscopic division of the median arcuate ligament fibers as the proposed treatment of choice.     

Intraoperative measurement of pressure gradient in median arcuate ligament syndrome as a rationale for radical surgical approach

Background: Median arcuate ligament syndrome (MALS) describes clinical symptoms in patients with stenosis of the celiac artery due to external compression by the ligament. There is an ongoing debate, whether sole release of the median arcuate ligament warrants long-term relief of the symptoms.

Materials and methods: Eight patients diagnosed with MALS underwent open surgical treatment beginning with the release of the ligament. Systemic pressure and pressure in the left gastric artery were measured before and after division of the median arcuate ligament and release of the celiac artery. In patients with persistent gradient above 15?mm?Hg after the release a PTFE bypass was performed.

Results: After the release, the pressure gradient decreased from 66?±?19 to 48?±?14?mm?Hg (p?=?.001) and therefore in all patients either an aorto-celiac bypass (n?=?6) or aorto-hepatic bypass (n?=?2) was created. Consequently, the gradient decreased to 7?±?2?mm?Hg (p?=?.0001). One month postoperatively, three patients were free of symptoms and the rest reported relief of symptoms.

Conclusions: Release of the celiac artery resulted in insufficient decrease of pressure gradient, which was achieved by bypassing the segment with favorable mid-term outcome. We believe that the effect of the release should always be assessed to decide on subsequent treatment.     

Factors associated with successful median arcuate ligament release in an international,multi-institutional cohort

ObjectivePrior research on median arcuate ligament syndrome has been limited to institutional case series, making the optimal approach to median arcuate ligament release (MALR) and resulting outcomes unclear. In the present study, we compared the outcomes of different approaches to MALR and determined the predictors of long-term treatment failure.MethodsThe Vascular Low Frequency Disease Consortium is an international, multi-institutional research consortium. Data on open, laparoscopic, and robotic MALR performed from 2000 to 2020 were gathered. The primary outcome was treatment failure, defined as no improvement in median arcuate ligament syndrome symptoms after MALR or symptom recurrence between MALR and the last clinical follow-up.ResultsFor 516 patients treated at 24 institutions, open, laparoscopic, and robotic MALR had been performed in 227 (44.0%), 235 (45.5%), and 54 (10.5%) patients, respectively. Perioperative complications (ileus, cardiac, and wound complications; readmissions; unplanned procedures) occurred in 19.2% (open, 30.0%; laparoscopic, 8.9%; robotic, 18.5%; P < .001). The median follow-up was 1.59 years (interquartile range, 0.38-4.35 years). For the 488 patients with follow-up data available, 287 (58.8%) had had full relief, 119 (24.4%) had had partial relief, and 82 (16.8%) had derived no benefit from MALR. The 1- and 3-year freedom from treatment failure for the overall cohort was 63.8% (95% confidence interval [CI], 59.0%-68.3%) and 51.9% (95% CI, 46.1%-57.3%), respectively. The factors associated with an increased hazard of treatment failure on multivariable analysis included robotic MALR (hazard ratio [HR], 1.73; 95% CI, 1.16-2.59; P = .007), a history of gastroparesis (HR, 1.83; 95% CI, 1.09-3.09; P = .023), abdominal cancer (HR, 10.3; 95% CI, 3.06-34.6; P < .001), dysphagia and/or odynophagia (HR, 2.44; 95% CI, 1.27-4.69; P = .008), no relief from a celiac plexus block (HR, 2.18; 95% CI, 1.00-4.72; P = .049), and an increasing number of preoperative pain locations (HR, 1.12 per location; 95% CI, 1.00-1.25; P = .042). The factors associated with a lower hazard included increasing age (HR, 0.99 per increasing year; 95% CI, 0.98-1.0; P = .012) and an increasing number of preoperative diagnostic gastrointestinal studies (HR, 0.84 per study; 95% CI, 0.74-0.96; P = .012) Open and laparoscopic MALR resulted in similar long-term freedom from treatment failure. No radiographic parameters were associated with differences in treatment failure.ConclusionsNo difference was found in long-term failure after open vs laparoscopic MALR; however, open release was associated with higher perioperative morbidity. These results support the use of a preoperative celiac plexus block to aid in patient selection. Operative candidates for MALR should be counseled regarding the factors associated with treatment failure and the relatively high overall rate of treatment failure.     

Median arcuate ligament syndrome in the pediatric population

Objectives

Median arcuate ligament syndrome (MALS) is a vascular compression syndrome with symptoms that overlap chronic functional abdominal pain (CFAP). We report our experience treating MALS in a pediatric cohort previously diagnosed with CFAP.

Patients and Methods

We prospectively evaluated 46 pediatric (< 21 years of age) patients diagnosed with MALS at a tertiary care referral center from 2008 to 2012. All patients had previously been diagnosed with CFAP. Patients were evaluated for celiac artery compression by duplex ultrasound and diagnosis was confirmed by computed tomography. Quality of life (QOL) was determined by pre- and postsurgical administration of PedsQL™ questionnaire. The patients underwent laparoscopic release of the median arcuate ligament overlying the celiac artery which included surgical neurolysis. We examined the hemodynamic changes in parameters of the celiac artery and perioperative QOL outcomes to determine correlation.

Results

All patients had studies suggestive of MALS on duplex and computed tomography; 91% (n = 42) positive for MALS were females. All patients underwent a technically satisfactory laparoscopic surgical release resulting in a significant improvement in blood flow through the celiac artery. There were no deaths and a total of 9 complications, 8 requiring a secondary procedure; 33 patients were administered QOL surveys. 18 patients completed the survey with 15 (83%) patients reporting overall improvement in the QOL. Overall, 31/46 patients (67%) reported improvement of symptoms since the time of surgery.

Conclusions

MALS was found to be more common in pediatric females than males. Laparoscopic release of the celiac artery can be performed safely in the pediatric population. Surgical release of the artery and resultant neurolysis resulted in significant improvement in the blood flow, symptoms, and overall QOL in this cohort. The overall improvement in QOL outcome measures after surgery leads us to conclude that MALS might be earlier diagnosed and possibly treated in patients with CFAP. We recommend a multidisciplinary team approach to care for these complex patients.     

Multimedia article. Laparoscopic management of median arcuate ligament syndrome

Median arcuate ligament syndrome is a rare disorder resulting from luminal narrowing of the celiac artery by the insertion of the diaphragmatic muscle fibers or fibrous bands of the celiac nervous plexus. The syndrome is characterized by weight loss, postprandial abdominal pain, nausea, vomiting, and an epigastric bruit. Surgical management entails complete division of the median arcuate ligament. The video demonstrates the laparoscopic release of the median arcuate ligament in a patient with median arcuate ligament syndrome. The patient is a 22-year-old male with a 6-month history of epigastric abdominal pain, nausea, vomiting, a 140-lb. weight loss, and an epigastric bruit on physical exam. Aortography demonstrated a >or=90% extrinsic compression of the celiac artery. A full laparoscopic skeletonization of the celiac artery and branch vessels was performed. Intraoperative duplex U/S demonstrated flow rate reduction after the median arcuate ligament release. A postoperative CT angiogram demonstrated no residual stenosis. The patient was discharged on postoperative day 3 and remained asymptomatic after 7 months of follow-up. Laparoscopic release of the median arcuate ligament is a novel approach to the management of celiac artery compression syndrome. The role of minimally invasive techniques to manage median arcuate ligament syndrome is evolving but they appear to be a safe alternative to open surgery.     

Inability of conventional imaging findings to predict response to laparoscopic release of the median arcuate ligament in patients with celiac artery compression

Objective

The objective of this study was to identify duplex ultrasound (DUS) or computed tomography angiography (CTA) imaging findings that can predict clinical response to laparoscopic release of the median arcuate ligament (MAL) in patients with celiac artery compression.

Laparoscopic treatment of Dunbar syndrome: A case report

BackgroundDunbar syndrome is a rare disorder due to external compression of the celiac trunk by the median arcuate ligament. The symptoms include abdominal pain, nausea and vomiting. Laparoscopic treatment was suggested as a safe procedure. The aim of this study is to present a case of Dunbar syndrome underwent laparoscopic treatment.CasepresentationA 17-year-old female 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. The decompression of the celiac trunk by the median arcuate ligament was performed. Postoperative course was uneventful and the patient was discharged on the 5th postoperativeConclusionsLaparoscopic division of the arcuate ligament in patients with Dunbar syndrome is feasible and safe. This procedure can be performed uneventful in order to restore quality of life of the patient.     

Laparoscopic versus robot-assisted surgery for median arcuate ligament syndrome

Background

Median arcuate ligament syndrome (MALS) is an uncommon disorder characterized by postprandial abdominal pain, weight loss, and vomiting related to the compression of the celiac artery by the median arcuate ligament. This syndrome has been classically treated with an open surgical approach. More recently, laparoscopic and robotic approaches have been used. We present our outcomes with laparoscopic and robot-assisted treatment of MALS.

Methods

We performed a retrospective review of all patients treated for MALS from March 2006 to August 2012 at a single institution.

Results

A total of 16 patients with MALS were treated: 12 patients via a laparoscopic approach and 4 patients via a robot-assisted approach. Patient characteristics and comorbidities were similar between groups. We experienced no intraoperative or perioperative conversions, complications, or deaths. The mean operative time for the laparoscopic approach was significantly shorter than for the robotic approach (101.7 vs. 145.8 min; P = 0.02). However, we found no significant difference in length of hospital stay (1.7 vs. 1.3 days, P = 0.23). The mean length of follow-up for laparoscopically treated patients was 22.2 months and for robotically treated patients it was 20 months. Eight patients (67 %) in the laparoscopic group and two patients (50 %) in the robotic group had full resolution of their abdominal pain. Three patients in the laparoscopic group and two patients in the robotic group ceased chronic narcotic use after surgery.

Conclusions

Both laparoscopic and robotic approaches to MALS treatment can be performed with minimal morbidity and mortality. The laparoscopic approach was associated with a significantly shorter operative time. While innovative, the true advantages to robot-assisted MALS surgery are yet to be seen.     

Laparoscopic Surgery for Coeliac Artery Compression Syndrome: Current Management and Technical Aspects

Objectives

The study aims to assess the feasibility and midterm outcome of trans-peritoneal laparoscopy for coeliac artery compression syndrome (CACS).

Design

Retrospective chart review involving four European vascular surgery departments and two surgical teams.

Materials and methods

charts for patients who underwent laparoscopy for symptomatic CACS between December 2003 and November 2009 were reviewed. Preoperative computed tomography (CT) angiography and postoperative duplex scan and/or CT angiography were performed.

Results

Eleven consecutive patients (nine women) with a median age of 52 years (interquartile range: 42.5-59 years) underwent trans-peritoneal laparoscopy for CACS. All patients had a history of postprandial abdominal pain; weight loss exceeded 10% of the body mass in eight cases. Preoperative CT angiography revealed coeliac trunk stenosis >70% in all cases. One patient had additional aortitis and inferior mesenteric artery occlusion, while another patient presented with an occluded superior mesenteric artery. Two conversions occurred (one difficult dissection and one aorto-hepatic bypass needed for incomplete release of CACS). The median blood loss was 195 ml (range: 50-900 ml) and median operative time was 80 min (interquartile range: 65-162.5 years). Symptoms improved immediately in 10/11 patients (no residual stenosis) while one remained unchanged despite a residual stenosis treated by a percutaneous angioplasty. Symptoms reappeared in one patient due to coeliac axis occlusion. The mean follow-up period was 35 ± 23 months (range: 12-78 months).

Conclusion

Our study demonstrates that trans-peritoneal laparoscopy for treating median arcuate ligament syndrome is safe and feasible. Additional patients and a longer follow-up are needed for long-term assessment of this laparoscopic technique.     

Outcome predictors in median arcuate ligament syndrome

Background

Median arcuate ligament syndrome (MALS) is a condition characterized by chronic abdominal symptoms associated with median arcuate ligament compression of the celiac artery. The selection of patients is difficult in the management of MALS. This study aimed to identify factors that predict outcomes of surgical and nonoperative treatment in these patients.