Search Results (9)

Median Arcuate Ligament Syndrome, also known as Dunbar’s syndrome, is a rare condition caused by stenosis of the celiac artery (CAS) through the fibrous arch connecting the diaphragmatic branches. It manifests as postprandial abdominal pain, nausea, vomiting, weight loss and increased epigastric tenderness. The condition most commonly affects young females without coexisting vascular comorbidities. Diagnosis is difficult due to the non-specific symptoms, often overlapping with other gastrointestinal diseases. Standard investigations include duplex ultrasound, computed tomography angiography (CTA) and contrast-enhanced magnetic resonance imaging (CE-MRA). Treatment mainly consists of surgical release of the arch ligament, which can be performed by open, laparoscopic or robotic methods. Surgery is often supported by celiac truncal stenting for residual stenosis, which significantly improves vascular flow. Alternative approaches include visceral plexus blocks and novel hybrid techniques, such as a combination of ligament release and endovascular treatment of the celiac trunk. In severe cases, vascular by-passes are recommended. The aim of this paper is to discuss the clinical manifestations, diagnostic possibilities, therapeutic options and directions for further research on MALS from the perspective of a vascular surgeon. It emphasizes the need for a multidisciplinary approach, including collaboration between the surgeon, radiologist, gastroenterologist and psychologist, which enables comprehensive disease management and improved quality of life for patients. In addition, the need for further development of diagnostic and therapeutic methods for early diagnosis and effective treatment was pointed out. Full article

Background: The pathophysiology of median arcuate ligament syndrome (MALS) is poorly understood. The diagnostic process remains inadequately standardized, with an absence of precise criteria to guide therapeutic management. Methods: We studied consecutive subjects referred to the Department of Angiology at the University Hospital of Zurich over the past 17 years due to suspected MALS. We focused on (1) the imaging criteria that led to diagnosis, notably the results of color duplex ultrasound and the consistency with different imaging tests; (2) the clinical consequences focusing on symptom resolution. Results: We included 33 subjects; in 8 subjects (24.2%), the diagnosis of MALS was retained. The median expiration peak systolic velocity (PSV) on ultrasound was 3.05 (Q1; 2.1–Q3; 3.3). To confirm the sonographic results, either a CT or MRI was performed on all patients, with consistent findings confirming a significant stenosis. Seven patients underwent surgery, all involving arcuate ligament release. Four procedures were laparoscopic, one was via laparotomy, and two were robot-assisted. Additionally, two patients required angioplasty with stenting as a secondary intervention. Only two (28.6%) of the seven operated patients experienced a relief of symptoms. None experienced a relief of symptoms following secondary angioplasty, despite stent patency. The prevalence of psychiatric disorders was comparable between patients with retained and rejected diagnoses, 38% and 36%, respectively. Conclusions: Our study confirmed sonography and CT/MRI consistency. However, most patients with MALS did not benefit from invasive treatment. The majority (83%) of patients without MALS were diagnosed with alternative conditions, mainly functional disorders. Full article

Background: Sutton–Kadir syndrome describes a rare pathology that commonly includes an aneurysm of the inferior pancreaticoduodenal artery in combination with a celiac trunk stenosis or occlusion, often caused by median arcuate ligament compression. Several therapeutic approaches exist including open surgical, endovascular, and hybrid treatments. Other combinations of visceral artery aneurysms and upstream stenoses exist but the cumulative body of evidence on these combinations is weak due to their rarity. Methods: A retrospective analysis of patient data from a single center was carried out. Electronic patient records were filtered for keywords including “visceral aneurysm”, “Sutton–Kadir”, and “median arcuate ligament”. Imaging studies were re-examined by two blinded vascular surgeons with a third vascular surgeon as a referee in case of diverging results. Results: Sixteen patients had a visceral artery aneurysm with an upstream stenosis. All cases had a celiac trunk obstruction while one patient also had a concomitant superior mesenteric artery stenosis. Both median arcuate ligament compression and atherosclerotic lesions were identified. The location of the aneurysms varied even though the inferior pancreaticoduodenal artery was most frequently affected. A classification system based on the different combinations of stenoses and aneurysms is presented and introduced as a new pathologic entity: visceral artery aneurysm in the presence of upstream stenosis (VAPUS). Conclusions: The concomitant presence of visceral artery aneurysms, especially in the pancreaticoduodenal arteries, and blood flow impairment of the celiac axis or superior mesenteric artery is a rare pathology. The proposed VAPUS classification system offers an accessible and transparent route to the precise localization of the affected vessels. Full article

Median arcuate ligament syndrome (MALS) is an uncommon condition characterized by the compression of the celiac trunk by the median arcuate ligament. Due to the anatomical proximity to the foregut, MALS has significant implications in hepato-pancreato-biliary (HPB) surgery. It can pose complications in pancreatoduodenectomy and orthotopic liver transplantation, where the collateral arterial supply from the superior mesenteric artery is often disrupted. The estimated prevalence of MALS in HPB surgery is approximately 10%. Overall, there is consensus for a cautious approach to MALS when embarking on complex foregut surgery, with a low threshold for intraoperative median arcuate ligament release or hepatic artery reconstruction. The role of endovascular intervention in the management of MALS prior to HPB surgery continues to evolve, but more evidence is required to establish its efficacy. Recognizing the existing literature gap concerning optimal management in this population, we describe our tertiary center experience as a clinical algorithm to facilitate decision-making. Research question: What is the significance and management of median arcuate ligament syndrome in patients undergoing hepato-pancreato-biliary surgery? Full article

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. Full article

Dunbar syndrome is caused by the compression of the celiac artery by the median arcuate ligament (MAL) and the diaphragmatic crura, presenting with abdominal pain. The treatment is surgical, with the aim of dissecting and separating the diaphragmatic crura from the celiac axis, restoring blood flow in the celiac trunk. We propose the use of transesophageal echocardiography to measure the peak systolic velocity in the celiac trunk and to evaluate, in real time, the efficacy of the surgery during MAL resection. Two clinical cases with practical implications are reported. In one of these, the ultrasound exam was useful in providing an intraoperative confirmation of the outcome of the resection. Full article

Median arcuate ligament syndrome (MALS), otherwise called celiac artery compression syndrome (CACS), is an uncommon disorder that results from an anatomical compression of the celiac axis and/or celiac ganglion by the MAL. Patients typically present with abdominal pain of unknown etiology exacerbated by eating along with nausea, vomiting, and weight loss. MALS is a diagnosis of exclusion that should be considered in patients with severe upper abdominal pain, which does not correlate with the objective findings. The cardinal feature which is elicited in the diagnosis of MALS relies on imaging studies of the celiac artery, demonstrating narrowing during expiration. The definitive treatment is the median arcuate ligament’s surgical release to achieve surgical decompression of the celiac plexus by division of the MAL. This article describes our experience with this entity, focusing on symptom presentation, diagnostic challenges, and management, including long-term follow-up in four cases. Full article

Median arcuate ligament syndrome (MALS) is a rarely diagnosed condition resulting from compression of the celiac trunk (CT) by the median arcuate ligament (MAL) of the diaphragm. Ischemia due to reduced blood flow through the CT and/or neuropathic pain resulting from celiac ganglion compression may result in a range of gastrointestinal symptoms, including nausea, postprandial discomfort, and weight loss. However, the mechanism of compression and its anatomical correlates have been incompletely delineated. It has been hypothesized that CT angle of origination may be more acute in individuals with MALS. Here, frequency of anatomical variation in the MAL and CT were assessed in 35 cadaveric subjects (17M/18F), including the vertebral level of origin of CT and superior mesenteric artery (SMA), the distance between CT and MAL and SMA, the angles of origination of CT and SMA, the diameter at the CT base, and MAL/CT overlap. Females exhibited significantly higher rates of inferred MAL/CT overlap than males. Significant correlations were revealed between MAL/CT overlap and angles of origination of the CT and SMA. Vertebral level of origin of the CT in individuals with MAL/CT overlap was not significantly more superior than in those without. This study also revealed a significant relationship between MAL/CT overlap and angle of origination of the CT, which has clinical implications for understanding the anatomy associated with MALS. Full article

Median arcuate ligament compression (MALC) potentially causes arterial complications in orthotopic liver transplantation (OLT). Here we aimed to investigate the incidence of MALC and its impact on clinical outcome after OLT. In addition, we performed an international survey among 52 European liver transplant centers to explore local protocols on the management of these patients. Data of 286 consecutive OLT recipients from a prospective database were analyzed retrospectively (05/2010-07/2017). Preoperative computed-tomography images were evaluated. Celiac axis stenosis due to MALC was found in 34 patients (12%). Intrinsic stenosis was present in 16 (6%) patients. Twenty-six patients (77%) with MALC underwent standard arterial revascularization with median arcuate ligament (MAL)-division. Patients treated for MALC had comparable baseline data and no difference was found in early- and long-term outcome compared to the rest of our cohort. Our survey found heterogeneous strategies regarding diagnosis and treatment of MALC. Only 29% of the centers reported the division of MAL in these patients as routine procedure. Even though there is no consensus on diagnosis and management of MALC among European centers, a surgical division of MAL is feasible and safe and should be considered in OLT recipients with MALC. Full article