[Diagnosis and treatment of Dunbar syndrome]. / Diagnostik und Therapie des Dunbar-Syndroms.

The rare Dunbar syndrome or medial arcuate ligament syndrome (MALS) is defined as compression of the celiac trunk and/or ganglion by the medial arcuate ligament. It is often diagnosed after patients have suffered for a long time and is characterized by intermittent food-related pain, nausea, and unexplained weight loss. After exclusion of other causes of the above symptoms by gastroscopy, colonoscopy, CT, or MRI, the gold standard for diagnosis is dynamic color-coded duplex sonography, which may be supplemented by CT or MR angiography. The treatment of choice is a laparoscopic division of the arcuate ligament at the celiac trunk, although percutaneous transluminal angioplasty (PTA) with stent implantation may be performed in cases of postoperative persistence of symptoms or recurrent stenosis. Since symptoms persist postoperatively in up to 50% of cases, strict indication and complete diagnosis in designated centers are of great importance for successful treatment.

A fatal case of massive hemobilia caused by invasive pancreatic cancer with median arcuate ligament syndrome: A case report.

INTRODUCTION: In median arcuate ligament syndrome (MALS), the celiac artery is compressed, causing an arcade to develop in the pancreatic head, leading to ischemic symptoms and aneurysms. PATIENT CONCERNS: The patient was diagnosed with borderline resectable pancreatic cancer (PC) and MALS. Endoscopic biliary drainage with a covered metal stent (CMS) was performed for the obstructive jaundice. After the jaundice improved, a modified FOLFIRINOX regimen was initiated. Several days later, cardiopulmonary arrest occurred after hematemesis occurred. Cardiopulmonary resuscitation was performed, his blood pressure stabilized, and emergent upper endoscopy was performed. The CMS was dislodged and active bleeding was observed in the papillae. The CMS was replaced, and temporary hemostasis was achieved. Contrast-enhanced computed tomography revealed a diagnosis of extravasation from the posterior superior pancreaticoduodenal artery (PSPDA) into the biliary tract. Transcatheter arterial embolization was performed. However, the patient was subsequently diagnosed with hypoxic encephalopathy and died on day 14 of hospitalization. DIAGNOSIS: Biliary hemorrhage due to invasion of pancreatic cancer from the PSPDA associated with MALS. INTERVENTION: None. OUTCOMES: Biliary hemorrhage from the PSPDA was fatal in the patient with invasive PC with MALS. LESSONS: Since MALS associated with PC is not a rare disease, the purpose of this study was to keep in mind the possibility of fatal biliary hemorrhage.

Atypical case of coexistent vascular compression syndromes: median arcuate ligament syndrome and nutcracker syndrome.

Vascular compression syndromes, including median arcuate ligament syndrome (MALS) and nutcracker syndrome (NCS), are poorly understood and frequently delayed diagnoses. This case describes a young adult female presenting with chronic vomiting, abdominal pain and weight loss, with dependence on nasogastric feeding. Subsequent to her gastrointestinal symptoms, she developed haematuria and orthostatic intolerance. Investigations confirmed NCS and possible MALS, with superadded gastroparesis and bradygastria. Under the joint care of general and vascular surgeons, she underwent a gastrojejunostomy and panelled renocaval bypass which led to partial resolution of her symptoms. It is hypothesised that gastroparesis may have been caused by MALS via a neurogenic mechanism, or coexistent compression of the duodenum by the superior mesenteric artery. This case highlights the difficulty in diagnosis of vascular compression syndromes, the overlap between the conditions and the potential for multiple coexistent conditions which complicate diagnosis and lead to increased lead-time and morbidity for patients.

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.

Robotic Approach for Median Arcuate Ligament Relief: A Case Report and Literature Review.

Introduction: There are very few reported cases of robotic surgery for median arcuate ligament syndrome. This clinical condition develops when the root of the celiac trunk is compressed by the median arcuate ligament of the diaphragm. The symptoms that typically accompany this syndrome are: discomfort and pain in the upper abdominal region, particularly after eating, and weight loss. During the diagnostic process, it is important to rule out other potential causes and demonstrate compression using any imaging technique available. Transecting the median arcuate ligament is the primary focus of the surgical treatment. We report a case of robotic MAL release, focusing on the particular aspects of the surgical technique. A literature review was also performed on the topic of robotic approach for MALS. Clinical case: A 25-year-old woman presented with sudden onset severe upper abdominal pain after physical activity and eating. She was then diagnosed with median arcuate ligament syndrome by imagistic means with computer tomography, doppler ultrasound, and angiographic computed tomography. After conservative management and careful planning, we performed robotic division of median arcuate ligament. The patient was discharged from the hospital without any complaint on the second day after surgery. Subsequent imaging studies revealed no residual celiac axis stenosis. Conclusion: The robotic approach is a safe and feasible treatment modality for median arcuate ligament syndrome.

Minimally Invasive Surgery for Median Arcuate Ligament Syndrome and Celiac Artery Stenosis.

Median arcuate ligament syndrome (MALS) is a pathology commonly reported in educational literature, although in reality it is scarcely seen. Herein, we present the case of a 48-year-old female patient who presented with nausea, vomiting, and unintentional weight loss. After thorough work up of her symptoms through a variety of different modalities, MALS was confirmed and she underwent surgical release via a minimally invasive approach. The authors of this article feel that this case is important to present due to paucity of reported cases in the literature. In addition, this patient was exceptionally unique to report as we selected to perform a slight adaptation of a minimally invasive approach, while there are multiple different treatment techniques and management plans that have previously been described in a variety of different literatures and require further discussion.

Systematic Review of the Efficacy of Treatment for Median Arcuate Ligament Syndrome.

OBJECTIVE: Since the first description of the median arcuate ligament syndrome (MALS), the existence for the syndrome and the efficacy of treatment for it have been questioned. METHODS: A systematic review conforming to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) statement was conducted, with a broader view on treatment for MALS including any kind of coeliac artery release, coeliac plexus resection, and coeliac plexus blockage, irrespective of age. Online databases were used to identify papers published between 1963 and July 2021. The inclusion criteria were abdominal symptoms, proof of MALS on imaging, and articles reporting at least three patients. Primary outcomes were symptom relief and quality of life (QoL). RESULTS: Thirty-eight studies describing 880 adult patients and six studies describing 195 paediatric patients were included. The majority of the adult studies reported symptom relief of more than 70% from three to 228 months after treatment. Two adult studies showed an improved QoL after treatment. Half of the paediatric studies reported symptom relief of more than 70% from six to 62 months after laparoscopic coeliac artery release, and four studies reported an improved QoL. Thirty-five (92%) adult studies and five (83%) paediatric studies scored a high or unclear risk of bias for the majority of the Quality Assessment of Diagnostic Accuracy Studies 2 (QUADAS-2) items. The meaning of coeliac plexus resection or blockage could not be substantiated. CONCLUSION: This systematic review suggests a sustainable symptom relief of more than 70% after treatment for MALS in the majority of adult and paediatric studies; however, owing to the heterogeneity of the inclusion criteria and outcome parameters, the risk of bias was high and a formal meta-analysis could not be performed. To improve care for patients with MALS the next steps would be to deal with reporting standards, outcome definitions, and consensus descriptions of the intervention(s), after which an appropriate randomised controlled trial should be performed.

Duodenopancreatectomy for PDAC Associated with MALS: A Case Report.

Introduction: Pancreatic ductal adenocarcinoma (PDAC) is an aggressive form of pancreatic malignancy which occurs in over 90% of such cases. Pancreaticoduodenectomy (PD) is used with a curative purpose for localized PDAC. Case presentation: A 68-year-old woman presented to our service through a transfer from another service, to be investigated and treated for a head of the pancreas tumor in a tertiary referral hospital. After a complete clinical and paraclinical evaluation, the patient was diagnosed with a PDAC and also with a median arcuate ligament syndrome (MALS). The surgical treatment was considered adequate, therefore, the patient underwent a PD with transmesocolic hepaticojejunostomy, pancreaticogastric anastomosis, precolic end-to-side gastrojejunostomy, Witzel jejunostomy and with the help of the cardiovascular surgery team from the Heart Institute, Cluj- Napoca, an aortohepatic bypass using saphenous vein graft was performed. Conclusion: Bypass was essential because the blood flow in the proper hepatic artery was not restored after sectioning the median arcuate ligament and clamping the gastroduodenal artery. The patient had a favorable outcome. The particularity of the present case consists of the complete occlusion of the celiac trunk by MALS and the total vascularization of the supramesocolic organs due to the superior mesenteric artery through the gastroduodenal artery.

DUNBAR SYNDROME: CLINICAL MANIFESTATION IN ADULTS, DIAGNOSTIC PROBLEMS (CASE REPORT).

In this case we used CBC and biochemical tests, ECG, ultrasound of the abdominal cavity and heart, CT scan with and without IV contrast. Women with complains on recurrent severe abdominal pain in epigastric region and right part of the abdomen radiating to the back, nausea, vomiting. Making laboratory and instrumental tests for confirmation of the diagnosis. Surgical treatment of DS was performed and after one year of the follow up there were no complications. DS may mimic other medical conditions such as gallbladder diseases, gastritis/peptic ulcer, appendicitis, colorectal malignancy, hepatitis, atherosclerotic diseases etc. That is why DS is a diagnosis of exclusion. This case illustrates pathway to find correct diagnosis and improve management tactic.

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

BACKGROUND: Median 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. METHODS: A 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. RESULTS: MALS 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. CONCLUSION: Laparoscopic median arcuate ligament release with endovascular support for selected cases provides sound clinical resolution of symptoms and long-term results.

Clinico-pathologic findings in patients with median arcuate ligament syndrome (celiac artery compression syndrome).

Median Arcuate Ligament Syndrome (MALS) is a rare entity characterized by severe post-prandial epigastric pain, nausea, vomiting, and/or weight loss. Symptoms have been attributed to vascular compression (celiac artery compression syndrome, CACS), but it remains controversial whether they could be secondary to neural compression. Literature review identified rare description of pathologic findings in surgery journals. The clinico-pathologic findings of four MALS patients who underwent robotic or laparoscopic surgery in our hospital are described. All our patients were female with a median age of 32.5 (range 25-55 years), and a median BMI of 23.5 kg/m2. They presented with chronic often post-prandial abdominal pain (4/4), nausea (3/4), emesis (2/4), anorexia (1/4), and weight loss (1/4). Two patients had a history of Crohn's disease. At intraoperative exploration, the celiac artery and adjacent nerves and ganglia were encased and partially compressed by fibrotic tissue in each patient. In each case laparoscopic excision of fibrotic tissue, celiac plexus and ligament division and was performed; celiac plexus nerve block was also performed in one patient. After surgical intervention, symptoms improved in three of the patients whose specimens show periganglionic and perineural fibrosis with proliferation of small nerve fibers. Our findings support neurogenic compression as a contributing factor in the development of pain and other MALS symptoms, and favor the use of MALS rather than CACS as diagnostic terminology. To further study the pathogenesis of this unusual syndrome, surgeons should submit all tissues excised during MALS procedures for histopathologic examination.

Median arcuate ligament syndrome in a patient with frontal lobe syndrome.

A boy aged 19 years presented to emergency room with severe postprandial upper abdominal pain and recent significant weight loss, with history of decompressive craniotomy for post-traumatic frontal lobe haemorrhage. CT scan revealed an acute indentation of coeliac artery with high-grade stenosis and post-stenotic dilatation, diagnostic of median arcuate ligament syndrome (MALS). MALS, a diagnosis of exclusion, is identified using patient's accurate symptomatic description. Exclusion of other causes of abdominal angina in a patient with frontal lobe syndrome was a challenging job, as they lack critical decision-making ability. Hence, the decision to proceed with the complex laparoscopic procedure was made by the patient's parents and the surgeon, with the patient's consent. Laparoscopic release of the median arcuate ligament resulted in relief of the patient symptoms much to the relief of his parents and the surgeon.

Perioperative Microcirculatory Changes Detected with Gastroscopy Assisted Laser Doppler Flowmetry and Visible Light Spectroscopy in Patients with Median Arcuate Ligament Syndrome.

PURPOSE: Physiological tests may aid in diagnosing median arcuate ligament syndrome (MALS). MALS is a symptomatic compression of the celiac artery causing symptoms similar to chronic mesenteric ischemia (CMI) of atherosclerotic etiology. Simultaneous use of visible light spectroscopy (VLS) and laser doppler flowmetry (LDF) during upper endoscopy may detect microcirculatory changes in these patients. PATIENTS AND METHODS: In a single-center, prospective comparative cohort, 25 patients were evaluated for MALS. Patients with a consensus diagnosis of MALS (n=15) underwent a gastroscopy assisted, transmucosal microcirculatory assessment with LDF and VLS. Results were compared to individuals with normal intestinal circulation (n=38) evaluated with duplex ultrasonography, and to patients with chronic mesenteric ischemia (n=32). Treatment response was evaluated clinically at 1, 3, 6, and 12 months, and with ultrasound, VLS and LDF at three months. Health-related quality of life (QoL) was assessed with Euroqol (EQ-5D-5L), preoperatively, and 12 months postoperatively. RESULTS: Preoperative mean transmucosal oxygen saturation was significantly lower in patients with MALS (SO2 76±6), as compared to healthy individuals (SO2 81±4), p=0.02. An overall significant improvement in SO2 after surgical decompression of the celiac artery was found (SO2 81±3.7, p=0.05). Eleven (92%) patients with clinical improvement after laparoscopic decompression had a definitive diagnosis of MALS. Median follow-up was 18 months (4-24 months). Four of the five dimensions investigated with EQ-5D-5L improved. CONCLUSION: VLS detected a significantly lower baseline transmucosal SO2 in patients with MALS as compared to control subjects with normal intestinal circulation. An improvement in SO2 after laparoscopic decompression was found, supporting a possible ischemic etiology in our patient population.

Combined Treatment of Multiple Splanchnic Artery Aneurysms Secondary to Median Arcuate Ligament Syndrome: A Case Study and Review of the Literature.

BACKGROUND Median arcuate ligament syndrome (MALS) is a rare and often misdiagnosed condition affecting about 0.4% of the population, typically ages 20-50 years old, and more frequently females. Caused by the compression of the celiac artery and adjacent nervous structures by the median arcuate ligament, it is typically manifested by postprandial abdominal pain, nausea or vomiting, and loss of weight. This condition also results in compensatory increased blood flow in peripancreatic arcades, facilitating formation of true aneurysms of the visceral vessels. CASE REPORT A 45-year-old woman with hypertension and left inferior renal pole cysts was referred to our department due to chronic, recurrent postprandial abdominal pains, nausea, and weight loss of approximately 15 kg in 1 year. A computed tomography (CT) scan demonstrated complete occlusion of the celiac trunk, significant stenosis of the superior mesenteric artery, and multiple aneurysms up to 17 mm in collateral circulatory vessels. Surgical decompression of the median arcuate ligament was performed and venous bypass was implanted between the aorta and the common hepatic artery, resulting in restoration of proper blood in the visceral circulation. Subsequently, 2 endovascular embolizations of visceral aneurysms were successfully performed. In the 48-month follow-up period, there was resolution of symptoms and no aneurysm formation was observed. CONCLUSIONS Endovascular methods should be the treatment of choice in patients with splanchnic artery aneurysms. However, in patients with multiple aneurysms secondary to MALS, arterial reconstruction may be considered prior to performing an endovascular procedure to restore physiological blood flow in the visceral circulation.

Assessment of celiac artery compression using color-coded duplex sonography.

BACKGROUND: Median arcuate ligament syndrome (MALS) is a rare condition due to compression of the celiac artery (CA) by an anatomically abnormal median arcuate ligament. With ultrasonography (US) as first-line diagnostic modality in patients with unclear abdominal pain, there is limited data on its diagnostic performance in MALS. OBJECTIVE: To investigate the value of CA peak systolic velocity (PSV) in the workup of patients with suspected MALS. METHODS: Patients with diagnosis of MALS between 2009 and 2019 were referred by Department of Visceral Surgery after clinical and gastroenterological workup. Diagnosis was confirmed by surgery or further cross-sectional imaging. B-mode US findings and PSV in the CA during various respiratory states were compared between patients with a final MALS diagnosis and patients not meeting the diagnostic criteria. RESULTS: Patients with proven MALS (n = 10) had higher median CA PSV during normal inspiratory breath-hold (239 [IQR, 159-327] vs. 138 [IQR, 116-152] cm/s; p < #x003C;< #x200A;0.001), and expiratory breath-hold (287 [IQR, 191-412] vs. 133 [IQR, 115-194] cm/s; p < #x003C;< #x200A;0.001) compared to patients without MALS (n = 26). CA PSV in both inspiratory breath-hold (AUC 0.88, 95% CI 0.77-1.00) and expiratory breath-hold (AUC 0.89, 95% CI 0.78-1.00) was of diagnostic value for confirming MALS. The best diagnostic performance (100% sensitivity, 80% specificity) was found for the combination of CA PSVexpiration + 2.4 · PSVinspiration > 550 cm/s . CONCLUSIONS: Since results on optimal cutoff values are inconsistent, a combination of CA PSVs during breathing maneuvers may help to diagnose or rule out MALS.

Median Arcuate Ligament Syndrome and Its Associated Conditions.

The risk factors and associated conditions of median arcuate ligament syndrome (MALS) have not been well characterized in the literature. In this study, we aim to investigate the presentation and outcomes of MALS patients with an emphasis on the prevalence of other uncommon disorders. To this end, data of patients with MALS who underwent surgery between 2013 and 2018 were collected and compiled into a retrospective database and analyzed. Eleven patients were identified. Seven of these eleven patients underwent diagnostics to evaluate gastric emptying. Five of these seven patients (71.4%) had radiographic evidence of delayed gastric emptying. Four of the eleven patients (36.4%) were found to have anatomic abnormalities of their visceral vasculature. Two of the eleven patients (18.2%) were found to have connective tissue disorders, both with Ehlers-Danlos syndrome. Three of the eleven (27.3%) had a diagnosis of postural orthostatic tachycardia syndrome. This is the first case series reporting on an association between MALS and delayed gastric emptying. We also explored the relationship between MALS and visceral vascular abnormalities, Ehlers-Danlos syndrome, and postural orthostatic tachycardia syndrome. It is notable that these conditions are more prevalent in the MALS population than in the general population, suggesting a possible pathophysiologic relationship.

Laparoscopic treatment of median arcuate ligament syndrome: a systematic review.

INTRODUCTION: Median arcuate ligament syndrome (MALS) is an uncommon condition caused by the extrinsic compression of the celiac trunk (CT) and celiac ganglion, secondary to an anatomical abnormality of the median arcuate ligament fibers. It is characterized by postprandial epigastric pain, chronic abdominal pain, weight loss, nausea and vomiting. MALS is typically diagnosed after the exclusion of other, more common conditions; however, a variety of imaging and diagnostic modalities, including Duplex ultrasonography, computed tomography angiography, magnetic resonance angiography, gastric tonometry and angiography can suggest findings consistent with MALS. EVIDENCE ACQUISITION: Laparoscopic approach has been proposed as the option of choice for the treatment of these patients. A systematic review of the international literature regarding this modality of treatment is presented here. EVIDENCE SYNTHESIS: The review included 504 cases, and several principles are suggested to improve the management. The advantages of this minimally invasive technique include short operative time (136.0 minutes, range 70-242), shorter hospital stay (3.8 days, range 0.5-7) and low rate of conversion to open surgery (4.2%). CONCLUSIONS: Various treatment modalities are available to decompress the celiac trunk. Although open surgery has been traditionally accepted as the gold standard, laparoscopic division of the MALS has proven equal results. Others have described different treatment modalities, including bypass surgery and endovascular procedures (angioplasty or stent placement). Laparoscopic approaches to correct MALS are feasible and safe. It may be the preferred modality of treatment in view of its lack of morbidity, shorter hospital stay and good results.

Laparoscopic decompression of a stricture of the celiac artery caused by the median arcuate ligament in a gastric cancer patient: A case of report.

Stricture of the celiac artery caused by the median arcuate ligament induces abdominal ischemic symptoms and aneurysm near the pancreatic head. However, the need to treat asymptomatic patients is unclear. We safely performed surgical decompression of a stricture of the celiac artery by MAL in an asymptomatic patient at the same time as gastrectomy for gastric cancer. After surgery, the stricture of the celiac artery had disappeared as demonstrated by CT scan and 3-D CT angiography.

The impact of psychiatric comorbidities on patient-reported surgical outcomes in adults treated for the median arcuate ligament syndrome.

OBJECTIVE: Median arcuate ligament syndrome (MALS) is an often overlooked, surgically correctable condition that mimics functional chronic abdominal pain. Patient-reported surgical outcomes are unpredictable in MALS. The objective of this study was to define the psychiatric comorbidities in a cohort of adults undergoing surgery for MALS and to determine whether these comorbidities are predictive of patient-reported quality of life (QOL) outcomes. METHODS: A prospective observational trial was conducted between April 1, 2010, and December 31, 2015, at a single tertiary care hospital. Adults with a diagnosis of chronic abdominal pain in the setting of celiac artery compression were enrolled in a prospective Institutional Review Board-approved observational trial. Patients completed psychological assessments before surgery for MALS and at 6 months after surgery. The primary outcome was patient-reported health-related QOL (young adult version of the Pediatric Quality of Life Inventory). RESULTS: A total of 51 patients (80% female; n = 41) with a mean age of 30.5 (±12.4) years were enrolled. Surgery significantly improved celiac artery hemodynamics in the entire cohort (P < .0001) as well as overall QOL (67.8 ± 14.6 [before surgery] vs 80.3 ± 13.7 [after surgery]; P < .001). Psychiatric diagnoses were common in this cohort, with 14 of 51 (28%) patients meeting criteria for a psychiatric diagnosis. There were no differences in the number of patients with psychiatric diagnoses between presurgical and postsurgical evaluations (14 [28%] vs 13 [26%]; P = .8). Exploratory analyses suggest that having a psychiatric diagnosis at the presurgical evaluation may predict significantly lower postsurgical QOL (R2 = 0.009; P = .01). CONCLUSIONS: Surgery improves patient-reported QOL in adults treated for MALS. Psychiatric diagnoses are common in adults with MALS and predict worse patient-reported QOL outcomes.