Median Arcuate Ligament Syndrome Research Articles

Median arcuate ligament syndrome complicated with gallbladder stones: A case report

BACKGROUND Median arcuate ligament syndrome (MALS) is a rare disease caused by compression of the celiac trunk artery by the median arcuate ligament (MAL). It can cause symptoms of postprandial abdominal pain, weight loss, and nausea and vomiting. CASE SUMMARY A 55-year-old woman was admitted due to abdominal pain, nausea and vomiting. On admission, the patient presented with epigastric pain that worsened after eating, without signs of peritoneal irritation. Computed tomography angiography of the upper abdomen showed compression of the proximal segment of the abdominal trunk, local luminal stenosis with angular “fishhook” changes, which changed significantly during forceful inspiration and expiration; gallbladder stones; and multiple cysts in the liver. Abdominal duplex ultrasonography showed that peak systolic velocity was 352 cm/s. After diagnosis of MALS was confirmed, an arch ligament release procedure was performed. MALS has no specific symptoms and can be misdiagnosed as other abdominal diseases. Awareness of MALS should be improved to avoid misdiagnosis. The commonly used treatment option is MAL release and resection of the peripheral ganglion of the celiac trunk artery. CONCLUSION The diagnosis and treatment of MALS must be individualized, and MAL release is effective and provides immediate symptomatic relief.

First description of a histopathologic grading system and relationship to outcomes after robotic median arcuate ligament release with celiac ganglionectomy and lymphadenectomy

BackgroundTwo dominating theories regarding median arcuate ligament syndrome include vascular and neurogenic etiologies from celiac artery and ganglion compression, respectively. Celiac ganglionectomy is not routine during surgery, and specimens are rarely excised; therefore, the extent of nerve involvement and histopathology are unknown. Our study aims to characterize histopathologic findings in median arcuate ligament syndrome, establish a histopathologic grading system, and correlate with clinical outcomes. MethodsRobotic median arcuate ligament release, celiac ganglionectomy, and lymphadenectomy were performed with specimens excised and stained using hematoxylin & eosin, trichrome, and S100. Neurofibrosis, adiposity, and reactive changes were described, a grading scale was developed, and results were analyzed with clinical outcomes. ResultsFifty-four patients were evaluated, of whom 36 met inclusion criteria (81% female, 34.9 [25.9–47.5] years, body mass index 23.5 [19.6–28.1] kg/m2). Histopathologic evaluation revealed fibrosis (hematoxylin & eosin and trichrome median score 1.5 [0–2.5]), reactive lymphadenopathy (89%), intraparenchymal nerves (31%), and lipogranulomas (31%). Greater fibrosis was associated with a lack of preoperative celiac plexus block relief (100% vs. 30%, P = .044) and lower postoperative celiac artery velocities (198 vs 323 cm/s, P = .02). Intraparenchymal nerves were associated with greater decreases in pre to postoperative velocities (161 vs 84 cm/s, P = .037). Symptoms improved in 28 patients (78%). ConclusionWe developed the first histopathologic grading system and identified unique findings of intraparenchymal nerves and lipogranulomas. Histopathologic abnormalities were associated with objective improvement and symptomatic relief postoperatively. These findings support nerve compression and inflammation as predominant contributors to median arcuate ligament syndrome pain, celiac ganglia resection to treat symptoms, and continued histopathologic analysis to better elucidate median arcuate ligament syndrome etiology.

Prognostic factors for the long term outcome after surgical celiac artery decompression in MALS

BackgroundThe median arcuate ligament syndrome (MALS) is a rare disease caused by compression of the celiac artery (ORPHA: 293208). Surgical treatment of MALS aims to restore normal celiac blood flow by laparoscopic celiac artery decompression. However, surgical success rates vary widely between patients, therefore adequate selection of patients is essential to improve surgical outcome. Symptoms of MALS might also overlap with other chronic multi-system disorders such as mast cell activation syndrome (MCAS). So far, no clinical or radiological parameter was found to be predictive of the postoperative outcome. We, therefore, aim to study preclinical parameters in one of the largest MALS cohorts with the focus to identify patients that would benefit from surgical MAL release.ResultsBy analyzing 20 MALS patients that underwent surgical celiac artery decompression, we found 60% of patients (12/20) had a postoperative relief of their symptoms and a simultaneous decrease of analgetic use. No demographic, radiologic or operative parameter was able to predict postoperative symptom relief. However, mast cell activation syndrome correlated significantly (p = 0.04) with persistent symptoms after the operation.ConclusionsOverall, laparoscopic MAL release can provide immediate symptomatic relief. Despite the missing predictive value of demographic and imaging data, our data show a correlation between persistent symptoms and a co-existing mast cell activation syndrome. This suggests that MCAS symptoms might be interpreted as MALS symptoms in the presence of celiac artery stenosis and therefore surgical treatment should be evaluated carefully. Overall, the selection of patients who are most likely to respond to surgical MAL release may best be accomplished by an interdisciplinary team of gastroenterologists, radiologists and surgeons.

Overview of Median Arcuate Ligament Syndrome: A Narrative Review.

Median arcuate ligament syndrome (MALS) is a rare disorder caused primarily by compression of the celiac trunk by the median arcuate ligament (MAL). This disorder typically results in patients presenting with bloating, weight loss, nausea, vomiting, and abdominal pain. The MALS diagnosis is one of exclusion, as the disorder has no specific diagnostic criteria. Imaging modalities are often utilized to assist in making the diagnosis, such as ultrasound, computed tomography angiography (CTA), and magnetic resonance angiography (MRA). These imaging modalities typically reveal a stenosed celiac artery with post-stenotic dilation in patients. This disorder is usually treated by dividing the MAL, thus relieving the compression of the celiac artery. The surgery may be done through either an open approach or a minimally invasive approach, which can be either laparoscopic or robot-assisted. Most patients respond well to this treatment, though certain factors that predict a poorer response to treatment include elderly age, a history of alcohol abuse, and psychiatric illness.

S4259 MAL-Eficent: Recurrent Median Arcuate Ligament Syndrome and a Robotic Approach to Resolution

S4259 MAL-Eficent: Recurrent Median Arcuate Ligament Syndrome and a Robotic Approach to Resolution

Celiac Plexus Blocks Prior to Median Arcuate Ligament Release Surgery: A Case Series

BACKGROUND: Median arcuate ligament release (MALR) surgery is the most common procedure used to treat median arcuate ligament syndrome (MALS). Estimates of the success rate of this surgery range from 70-80%. Factors that affect the success of this procedure are not well understood. CASE REPORT: Nine patients with abdominal pain and a vascular ultrasound study suggestive of MALS underwent a celiac plexus block. All 9 continued to surgery and experienced ≥ 50% pain reduction in the upper abdomen postoperatively. Eight of these 9 patients had a positive celiac plexus block. One of 9 patients with a negative prognostic block experienced ≥ 50% pain reduction in the upper abdomen after surgery. CONCLUSIONS: These data suggest that the accuracy of prognostic celiac plexus blocks in predicting successful MALR surgery is 89%. This can be compared to estimates of the success rate of surgery without prognostic blocks (70-80%). KEY WORDS: Chronic abdominal pain, median arcuate ligament release surgery, median arcuate ligament syndrome, nerve block prognostic value, upper abdomen

Obstructive jaundice as a rare complication of multiple pancreaticoduodenal artery aneurysms due to median arcuate ligament syndrome: a case report and review of the literature

BackgroundObstructive jaundice has various causes, and one of the rarest is pancreaticoduodenal artery aneurysm (PDAA), which is often associated with celiac axis stenosis caused by median arcuate ligament syndrome (MALS).Case presentationThe patient was a 77-year-old Azeri woman who presented with progressive jaundice, vague abdominal pain, and abdominal distension from 6 months ago. The intra- and extrahepatic bile ducts were dilated, the liver's margin was slightly irregular, and the echogenicity of the liver was mildly heterogeneous in the initial ultrasound exam. A huge cystic mass with peripheral calcification and compressive effect on the common bile duct (CBD) was also seen near the pancreatic head, which was connected to the superior mesenteric artery (SMA) and had internal turbulent blood flow on color Doppler ultrasound. According to the computed tomography angiography (CTA) findings, the huge mass of the pancreatic head was diagnosed as a true aneurysm of the pancreaticoduodenal artery caused by MALS. Two similar smaller aneurysms were also present at the huge aneurysm's superior margin. Due to impending rupture signs in the huge aneurysm, the severe compression effect of this aneurysm on CBD, and the patient's family will surgery was chosen for the patient to resect the aneurysms, but unfortunately, the patient died on the first day after the operation due to hemorrhagic shock.ConclusionIn unexpected obstructive jaundice due to a mass with vascular origin in the head of the pancreas, PDAA should be considered, and celiac trunk should be evaluated because the main reason for PDAA is celiac trunk stenosis or occlusion by atherosclerosis or MALS. The treatment method chosen (including transarterial embolization, open surgery, or combined method) depends on the patient's clinical status and radiological findings, but transarterial embolization would be safer and should be used as a first-line method.

Neurolysis with Celiac Plexus Block for the Treatment of Median Arcuate Ligament Syndrome

Neurolysis with Celiac Plexus Block for the Treatment of Median Arcuate Ligament Syndrome

Topic: Stomach Abstract ID: 73 Surgical Treatment of Median Arcuate Ligament Syndrome in a Series of Patients With Gastroparesis

Topic: Stomach Abstract ID: 73 Surgical Treatment of Median Arcuate Ligament Syndrome in a Series of Patients With Gastroparesis

Pancreaticoduodenal Artery Aneurysm Complicated by Median Arcuate Ligament Syndrome.

To highlight median arcuate ligament syndrome as a potential cause for celiac artery stenosis and pancreaticoduodenal artery aneurysm, and describe treatment options in this setting. A 63-year-old male presented with a pancreaticoduodenal artery aneurysm and concomitant celiac artery stenosis that was treated with celiac artery stenting and aneurysm coiling. He subsequently developed stent fracture and celiac artery occlusion secondary to previously unrecognized median arcuate ligament syndrome causing reperfusion of the aneurysm. This was treated with open median arcuate ligament release and aorta to common hepatic artery bypass with good clinical result and stable 20-month surveillance imaging. It is critical to recognize median arcuate ligament syndrome as a cause of celiac artery stenosis in the setting of pancreaticoduodenal artery aneurysm given the high risk of failure of endovascular stenting. Open aorto-hepatic artery bypass and endovascular aneurysm coiling should be the preferred approach in these patients.

Median Arcuate Ligament Syndrome: A Rare Cause of Abdominal Pain That is Difficult to Identify

Median Arcuate Ligament Syndrome: A Rare Cause of Abdominal Pain That is Difficult to Identify

Recurrent pancreatitis caused by previous coiling of a pancreaticoduodenal artery aneurysm in a patient with median arcuate ligament syndrome.

Recurrent pancreatitis caused by previous coiling of a pancreaticoduodenal artery aneurysm in a patient with median arcuate ligament syndrome.

Pancreaticoduodenectomy combined gastroduodenal collateral reconstruction and preservation due to median arcuate ligament syndrome: technical notes with two surgical cases report (with video)

IntroductionPancreaticoduodenectomy in patients with CA stenosis due to median arcuate ligament often required carefully collateral pathways management to avoid hepatic ischemic complications.Cases presentationCase 1: A 63-year-old man was referred to our department because of jaundice with distal common bile duct tumor. Pancreaticoduodenectomy with left posterior SMA first approach and circumferential lymphadenectomy was performed. Case 2: A 48-year-old man was referred to our department because of right-upper-quadrant abdominal pain with Vater tumor. Laparoscopic pancreaticoduodenectomy with left posterior SMA first approach and circumferential lymphadenectomy was performed. Postoperatively, in all two cases, three-dimensional reconstruction images showed developed collateral pathways around the pancreatic head, and the CA was stenosis in 75% and 70% due to MAL, respectively. Intraoperatively, in all two cases, we confirmed poor blood flow in the common hepatic artery (CHA) by palpation and observation. So that in the first case, we have decided to proceed a no-touch technique of GDA segmental resection en bloc with the tumor and reconstructed with an end-to-end GDA anastomosis; in the second cases, we have decided to proceed gastroduodenal collateral preservation. When preserving these collateral pathways, we confirmed that the PHA flow remained pulsatile as an indicator that the blood flow was adequate.ConclusionCeliac axis stenosis was a rare but difficult-to-managed condition associated with pancreaticoduodenectomy. Collateral pathways management depends on variety of collateral pathways.

Interventional Radiology for High-Flow Aneurysm of the Pancreaticoduodenal Arcades with Median Arcuate Ligament Syndrome: Review of 14 Patients.

Transarterial embolization (TAE) for high-flow pancreaticoduodenal artery (PDA) aneurysms in patients with celiac-trunk stenosis by the median arcuate ligament (MAL) has been found effective both after rupturing and to prevent rupture. The objective was to describe the TAE techniques used and their effectiveness in excluding PDA aneurysms due to MAL syndrome. This single-center retrospective study done at the Dijon-Bourgogne University Hospital included all patients treated by TAE in 2010-2022 for ruptured or unruptured high-flow PDA aneurysms caused by MAL syndrome. We identified 14 patients (7 women and 7 men; mean age, 64 years). Packing and trapping techniques were used alone or together. Occlusion was with microcoils, co-polymer, or cyanoacrylate glue, used separately or combined. Technical success was achieved in 13 (93%) patients. Clinical success was achieved in 12 (86%) patients. One major and two minor complications were recorded within the first 30 days. No complications occurred after 30 days. Follow-up ranged from 1 to 84 months. No cases of aneurysm recanalization have been recorded to date. TAE had high technical and clinical success rates in our patients with unruptured or ruptured PDA aneurysms due to MAL syndrome.

Median Arcuate Ligament Syndrome: A Case Report

Median Arcuate Ligament Syndrome (MALS) is a rare vascular compression disorder of the celiac artery with non-specific symptomatology that can mimic other, common abdominal diseases. We present one such case of MALS and review the relevant literature on this rare topic.

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

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

ID: 219777 Neuromodulation in a 10-Year-Old With Chronic Abdominal Pain: A Case Report

Chronic abdominal pain in the pediatric population is complex, and identifiable causes may be difficult to elucidate. Median arcuate ligament syndrome is a rare disease in which diagnosis has been controversial in the medical community. Patients typically present with chronic postprandial abdominal pain accompanied with nausea, vomiting and weight loss. It is a diagnosis of exclusion with unclear pathophysiology typically thought to have both a structural component via compression of the celiac artery by the median arcuate ligament, as well as a neuropathic component mediated by the celiac ganglia. Optimal treatment is also debated but generally involves surgical intervention to decompress the celiac artery with or without neurolysis of the celiac plexus. We present the case of a 10-year-old female with chronic abdominal pain refractory to aggressive medical and surgical treatments, who found relief with spinal cord stimulator implantation. Before evaluation in the pain clinic, the patient received extensive gastroenterological workup and treatments including arcuate ligament release with neurolysis of the celiac ganglion that provided relief for nearly two months. She had increasing abdominal pain since five years of age and required a feeding tube for adequate nutritional intake.

Trauma-Induced Median Arcuate Ligament Syndrome is an Under-Recognized Source of Chronic Abdominal Pain.

Median arcuate ligament syndrome (MALS) can be a debilitating condition resulting in epigastric pain, nausea, difficulty eating due to postprandial pain, weight loss, and malnutrition in otherwise healthy individuals. The pain is caused by the compression of the celiac artery and neural ganglia by the median arcuate ligament as it attaches from the spine to the diaphragm. Diagnostic imaging, either duplex or angiography, can show the abnormality however, vague symptoms can lead to a missed diagnosis. While MALS is a known anatomical variation in the population, to our knowledge, has not been identified to be caused by trauma. Here, we present 4 patients who developed MALS following abdominal or spinal trauma whom all required surgery to alleviate lifestyle-limiting pain.

Open Median Arcuate Ligament Release With Complete Celiac Plexus Neurolysis Provides Substantial Relief in Symptomatic Patients

Median arcuate ligament syndrome (MALS) is a clinical entity that has historically been met with significant equipoise on how to diagnose and treat the constellation of symptoms. Our objective was to describe distinct patient outcomes following open median arcuate ligament release and complete celiac plexus neurolysis in patients diagnosed with MALS. We performed a prospective analysis of patients referred for MALS at a single institution between February 2020 and January 2022. We assessed duplex ultrasound studies, abdominal computed tomography angiography results, celiac plexus nerve block procedures, and patient reported questionnaires. Patients who experienced symptomatic relief from celiac plexus nerve block were offered surgical release and neurolysis. Their preoperative and postoperative health and quality of life measures were assessed using the 36-item Short-Form Health Survey (RAND). There were 75 patients who were referred for MALS and 29 patients (39%) underwent surgery. Duplex ultrasound examination revealed a significant increase in celiac artery peak systolic velocity at end-expiration (211 cm/s to 276 cm/s), with only 55% having a J-hooked appearance of the celiac artery on computed tomography angiography. All patients underwent transgastric celiac plexus block with a combination of bupivacaine and triamcinolone. After nerve block, surgical patients had a mean relief of 207 hours (48-1008 hours) from their symptoms. Postoperatively, 16 patients completed the 36-item Short-Form Health Survey with significant improvement noted in each category of the questionnaire (Fig 1). Significant quality of life improvement can also be appreciated in Fig 2. Surgical patients had an average weight gain of 8.8 kg (range, 1.3-34 kg) at a mean follow-up of 8 months. There were no perioperative wound healing complications. Our data suggest that patients who are carefully selected for median arcuate ligament release and neurolysis have significant improvement in quality of life and benefit from surgery. Wide surgical neurolysis of the celiac plexus is critical and the same diagnostic imaging findings are not consistent across all patients. Celiac plexus neurolysis is required for accurate patient selection.Fig 2Averages in each domain of the 36-item Short-Form Health Survey.View Large Image Figure ViewerDownload Hi-res image Download (PPT)

Median Arcuate Ligament Syndrome: Comparing the Safety of Open and Laparoscopic Management in a Large Cohort

Open surgery has been the traditional approach for Median Arcuate Ligament Syndrome (MALS) management. However, there has been a recent rise in laparoscopic management for MALS. In this study we used a large-scale database to compare perioperative complications between open and laparoscopic approaches for MALS. Using the National Inpatient Sampling database, we identified all patients surgically treated for MALS between 2008 -2018 through conventional open and laparoscopic approaches. ICD 9 and 10 codes were used to identify patients and their specific surgical interventions. Statistical analyses were conducted to compare the perioperative complications between the two MALS surgical approaches, as well as and length of hospital stays and total charges. The complications include postoperative bleeding, accidental operative laceration/puncture, surgical wound infection, ileus, hemothorax/pneumothorax, and cardiac and respiratory complications. A total of 630 patients were identified: 487 (77.3%) patients underwent open surgery while 143 (22.7%) patients underwent laparoscopic decompression. The majority of the study population consisted of female patients (74.8%) with a mean age of 40.6 ± 19 years. Patients who underwent laparoscopic decompression had significantly less all-cause perioperative complications compared to their open surgery counterparts (0.7% vs. 9.9%; P = .001). Additionally, prolonged hospitalization was noted in the open group compared to the laparoscopic one (5.8 days vs. 3.5; P<.001, respectively) with a significantly higher mean of total hospital charges ($70,095.8 vs. 56,113.5; P =.016). Laparoscopic management of MALS has significantly less perioperative complications than open surgical decompression with shorter hospitalization and lower total charges. Given that, laparoscopic technique could be a safe option in treating select MALS patients.