Median Arcuate Ligament Compression Research Articles

Visceral Artery Aneurysms in the Presence of Upstream Stenoses.

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.

Median arcuate ligament compression syndrome with chest pain as the first symptom: a case report

Median arcuate ligament compression syndrome with chest pain as the first symptom: a case report

Sutton-Kadir Syndrome can be treated safely with endovascular embolisation alone.

Sutton-Kadir Syndrome (SKS) describes true inferior pancreaticoduodenal artery (IPDA) aneurysms in the setting of coeliac artery (CA) stenosis or occlusion. Although rare, SKS aneurysms can rupture and cause morbidity. Due to its rarity and lack of controlled treatment data, correct treatment for the CA lesion is currently unknown. Our aim was to assess if endovascular embolisation alone was safe and effective in treatment of SKS aneurysms, in emergent and elective settings. Secondary objectives were to describe presentation and imaging findings. A retrospective cohort study of patients treated at Sir Charles Gairdner Hospital between January 2014 and December 2021 was done. Data on presentation, diagnostics, aneurysm characteristics, CA lesion aetiology, treatment and outcomes were extracted from chart review. Twenty-four aneurysms in 14 patients were identified. Rupture was seen in 7/15 patients. Most aneurysms (22/24) were in the IPDA or one of its anterior or posterior branches. Median arcuate ligament (MAL) compression was identified in all. There was no difference in median (IQR) maximal transverse diameter between ruptured and non-ruptured aneurysms (6 mm (9), 12 mm (6), P = 0.18). Of ruptures, 6/7 had successful endovascular embolisation and 1/7 open surgical ligation. Of non-ruptures, 6/7 had successful endovascular embolisation, 1/7 open MAL division then endovascular CA stenting and aneurysm embolisation. No recurrences or new aneurysms were detected with computed tomography or magnetic resonance angiography over a median (IQR) follow-up period of 30 (10) months in 12 patients. Endovascular embolisation of SKS aneurysms without treatment of MAL compression is safe and effective in both the emergent and elective settings.

Laparoscopic median arcuate ligament section for median arcuate ligament compression syndrome initially detected as splenic infarction: a case report

BackgroundMedian arcuate ligament compression syndrome (MALS) causes upper abdominal pain and at times hemodynamic abnormalities in the pancreaticoduodenal region.Herein, we present a case of a 70 year-old man, initially diagnosed with splenic infarction and was successfully treated laparoscopically.Case presentationA 70-year-old man with abdominal pain admitted to our hospital. Abdominal-enhanced computed tomography revealed a poorly contrasted area in the spleen and stenosis at the root of the celiac artery. Arterial dilatation was observed around the pancreaticoduodenal arcade, however, no obvious aneurysm formation or arterial dissection was observed. Abdominal-enhanced magnetic resonance imaging indicated the disappearance of the flow void at the root of the celiac artery. The patient had no history of atrial fibrillation and was diagnosed with splenic infarction due to median arcuate ligament compression syndrome. We performed a laparoscopic median arcuate ligament section with five ports. Intraoperative ultrasonography showed a retrograde blood flow in the common hepatic artery and the celiac artery. After releasing the compression, the antegrade blood flow from the celiac artery to the splenic artery, and the common hepatic artery were visualized using intraoperative ultrasonography. The postoperative course of the patient was uneventful, and he was discharged on postoperative day 9. Postoperative computed tomography a month after surgery revealed no residual stenosis of the celiac artery or dilation of the pancreaticoduodenal arcade. Furthermore, the poorly contrasted area of the spleen improved.ConclusionsReports indicate that hemodynamic changes in the abdominal visceral arteries due to median arcuate ligament compression are related to the formation of pancreaticoduodenal aneurysms. In this case, median arcuate ligament compression syndrome caused splenic infarction by reducing blood flow to the splenic artery.

Celiac trunk stenosis by median arcuate ligament in orthotopic liver transplantation: a potential hidden foe to the biliary tree

BackgroundCeliac trunk compression by the median arcuate ligament (MAL) increases the risk of ischemic complications following gastrointestinal surgical procedures. Previous studies suggest increased risk of hepatic artery thrombosis (HAT) in orthotopic liver transplant (OLT) recipients. The aim of this study is to investigate the impact of untreated MAL compression (MAL-C) on biliary complications in OLT. MethodsContrast-enhanced imaging was used to classify celiac trunk stenosis by MAL-C. Medical records were reviewed to extract pre-transplant, transplant and post-transplant data. Patients were divided into two groups: no MAL compression (nMAL-C) and MAL-C. The primary endpoint was biliary complications. Secondary endpoints were HAT and graft survival. Results305 OLT were performed from 2010 to 2021, of which 219 were included for analysis: 185 (84.5%) patients without and 34 (15.5%) with MAL-C. The incidence of HAT was 5.9% in both groups. Biliary complications were more common in the MAL-C group (35.3% vs. 17.8%, p = 0.035). Graft survival was decreased in patients with MAL-C (p = 0.035). ConclusionsMAL-C of the celiac trunk was associated with increased risk of biliary complications and inferior graft survival in OLT patients. These findings highlight the importance of preoperative screening and treatment of MAL in this population.

Median Arcuate Ligament Compression Associated with Flow-Related Visceral Aneurysms

PurposeTo identify risk factors for rupture, and to determine outcomes of endovascular treatment of median arcuate ligament (MAL) compression–related visceral artery aneurysms (VAAs). Methods and MaterialsA retrospective review of patients who presented with MALC-related VAAs was performed from 1999 to 2021. A total of 21 patients (12 men) and 39 VAAs associated with MAL compression were encountered (mean age, 59 years). Imaging studies were reviewed for the number, morphology/size, and recurrence of aneurysms. Statistical analysis was performed to identify risk factors for rupture. ResultsTen patients presented with acute rupture, and 12 patients were symptomatic with nonspecific abdominal pain. Twenty-two aneurysms were fusiform in morphology and 17 aneurysms were saccular in morphology. Of the 14 aneurysms that presented with acute hemorrhage, 12 (86%) were fusiform in morphology (odds ratio, 9.0; P < .01). The mean aneurysm size was 1.3 cm, and the mean ruptured size was 0.6 cm. Thirty-one aneurysms were treated by endovascular techniques, and technical success was achieved in all cases. Fourteen patients were found to have an Arc of Buhler. No procedure-related adverse events occurred. No patient underwent surgical ligament release. The mean time of follow-up was 3.2 years, and no aneurysms recurred after endovascular treatment. ConclusionsMAL compression-associated VAAs are an important clinical entity that should be treated even at small sizes, particularly if they are fusiform in morphology. Endovascular therapy is safe and feasible and results in durable aneurysm exclusion.

Prevalence of celiac artery compression by median arcuate ligament in patients with splanchnic artery aneurysms/pseudoaneurysms submitted to endovascular embolization

PurposeTo study the association between median arcuate ligament compression (MALC) of celiac artery (CA) and splanchnic artery aneurysms/pseudoaneurysms (SAAPs) submitted to endovascular embolization.MethodsSingle center retrospective study of embolized SAAPs between 2010 and 2021, to evaluate the prevalence of MALC, and compare demographic data and clinical outcomes between patients with and without MALC. As a secondary objective, patient characteristics and outcomes were compared between patients with different causes of CA stenosis.ResultsMALC was found in 12.3% of 57 patients. SAAPs were more prevalent in the pancreaticoduodenal arcades (PDAs) in patients with MALC, compared to those without MALC (57.1% vs. 10%, P = .009). Patients with MALC had a greater proportion of aneurysms (71.4% vs. 24%, P = .020), as opposed to pseudoaneurysms. Rupture was the main indication for embolization in both groups (71.4% and 54% of patients with and without MALC, respectively). Embolization was successful in most cases (85.7% and 90%), with 5 immediate (28.6% and 6%) and 14 non-immediate (28.6% and 24%) post-procedure complications. Thirty and 90-day mortality rate were 0% in patients with MALC, and 14% and 24% in patients without MALC. Atherosclerosis was the only other cause of CA stenosis, in 3 cases.ConclusionsIn patients with SAAPs submitted to endovascular embolization, the prevalence of CA compression by MAL is not uncommon. The most frequent location for aneurysms in patients with MALC is in the PDAs. Endovascular management of SAAPs is very effective in patients with MALC, with low complications, even in ruptured aneurysms.Graphical

S3559 Acute Gastric Ischemia Caused by Median Arcuate Ligament Compression Syndrome

S3559 Acute Gastric Ischemia Caused by Median Arcuate Ligament Compression Syndrome

Abstract No. 74 Endovascular treatment of pancreaticoduodenal artery aneurysms associated with median arcuate ligament compression of the celiac artery

To review the feasibility, safety, and efficacy of endovascular embolization of pancreaticoduodenal artery aneurysms (PDAAs) associated with median arcuate ligament compression (MALC) of the celiac artery in the largest single-center cohort reported in the literature to date. Also, to provide recommendations for management and follow-up of PDAAs associated with MALC.

Abstract No. 77 Median arcuate ligament compression related pancreaticoduodenal arcade aneurysms: a 22-year single-center experience

Inferior pancreaticoduodenal artery aneurysms (IPDAA) comprise 2% of all splanchnic aneurysms and are clinically significant due to their high risk of rupture. While small series have suggested that IPDAAs may be associated with median arcuate ligament compression (MALC), there remains a paucity of data regarding the clinical presentation, imaging findings, endovascular treatment and long-term clinical outcomes of this entity. In this study, we present a 22 year single-center experience in the treatment of MALC-related IPDAA.

The Blood Regurgitation of Common Hepatic Artery Due to Median Arcuate Ligament Compression Syndrome Revealed by Doppler Echo—A Case Report

The Blood Regurgitation of Common Hepatic Artery Due to Median Arcuate Ligament Compression Syndrome Revealed by Doppler Echo—A Case Report

Combined treatment of an aortosplenic bypass followed by coil embolization in the treatment of pancreaticoduodenal artery aneurysms caused by median arcuate ligament compression: a report of two cases

BackgroundPancreaticoduodenal artery aneurysms (PDAAs) are rare visceral aneurysms, and prompt intervention/treatment of all PDAAs is recommended at the time of diagnosis to avoid rupture of aneurysms. Herein, we report two cases of PDAA caused by the median arcuate ligament syndrome, treated with surgical revascularization by aortosplenic bypass followed by coil embolization.Case presentationCase 1 A 54-year-old woman presented with a chief complaint of severe epigastralgia and was diagnosed with two large fusiform inferior PDAAs and celiac axis occlusion. To preserve the blood flow of the pancreatic head, duodenum, liver, and spleen, we performed elective surgery to release the MAL along with aortosplenic bypass. At 6 days postoperatively, transcatheter arterial embolization was performed. At the 8-year 6-month follow-up observation, no recurrent perfusion of the embolized PDAAs or rupture had occurred, including the non-embolized small PDAA, and the bypass graft had excellent patency.Case 2 A 39-year-old man who had been in good health was found to have a PDAA with celiac stenosis during a medical checkup. Computed tomography and superior mesenteric arteriography showed severe celiac axis stenosis and a markedly dilated pancreatic arcade with a large saccular PDAA. To preserve the blood flow of the pancreatic arcade, we performed elective surgery to release the MAL along with aortosplenic bypass. At 9 days postoperatively, transcatheter arterial embolization was performed. At the 6-year 7-month follow-up observation, no recurrent perfusion or rupture of the PDAA had occurred, and the bypass graft had excellent patency.ConclusionCombined treatment with bypass surgery and coil embolization can be an effective option for the treatment of PDAAs associated with celiac axis occlusion or severe stenosis.

Laparoscopic treatment for celiac artery stenosis caused by median arcuate ligament compression with Adachi V type vascular anomaly: a case report

BackgroundMedian arcuate ligament syndrome (MALS), which results from compression of the median arcuate ligament (MAL), is a rare cause of abdominal pain and weight loss. Treatment is dissection of the MAL; however, the laparoscopic procedure is not yet established and it involves the risk of major vascular injury, especially in cases with an anomaly.Case presentationA 47-year-old man was evaluated at the hospital for epigastric pain. Contrast computed tomography scan revealed stenosis of the celiac artery origin due to the MAL. An Adachi V type vascular anomaly was also observed. Laparoscopic treatment was performed to release pressure on the celiac artery. Laparoscopic ultrasonography was used to less invasively confirm the release of the MAL. Despite a concomitant Adachi V type vascular anomaly, surgery was safely performed using the laparoscopic magnification view and intraoperative ultrasonography. Follow-up ultrasonography confirmed the celiac artery stenosis has not recurred.ConclusionsA rare case of MALS with an Adachi V type vascular anomaly is presented and the laparoscopic treatment is detailed.

Median arcuate ligament (Dunbar) syndrome: Laparoscopic management and clinical outcomes of a single centre.

Background:Median arcuate ligament syndrome (MALS) is a condition characterised by chronic abdominal symptoms associated with median arcuate ligament (MAL) compression of the coeliac artery.Aim:In this observational study, we aimed to evaluate the outcomes of laparoscopic treatment in patients with MALS.Materials and Methods:The data of ten patients with MALS who were subjected to laparoscopic sectioning of the MAL were retrospectively reviewed. The following data were evaluated: age, gender, clinical and diagnostic test findings, American Society of Anaesthesiologists score, operative findings and complications and mortality, hospital stay duration and hospital readmission. The diagnosis of MALS was established by computed tomography (CT) angiography.Results:Six (60%) of ten patients with MALS were female and four (40%) were male. The mean age was 42.4 ± 12.3. The main symptoms were epigastric pain (100%) and weight loss (60%). CT angiography showed high-grade stenosis of the anterior wall of the proximal coeliac trunk and post-stenotic dilation caused by extrinsic compression of the MAL. Surgical procedure was uneventful in all patients. Operating time was 155.5 min (120–200) and intra-operative blood loss was 150 ml (100–250). Length of stay was 3.1 day (2–9), with no mortality. The post-operative complications developed in two female patients. One of them developed ileus and the other patient developed pulmonary thromboembolism. At 6-month follow-up, all patients were asymptomatic.Conclusion:Laparoscopic decompression is an effective treatment for MALS and can provide symptomatic relief. This method may be the preferred modality of treatment in view of its lack of morbidity and good results.

Effect of celiac axis compression on target vessel-related outcomes during fenestrated-branched endovascular aortic repair

ObjectiveTo report the effect of median arcuate ligament (MAL) compression on outcomes and technical aspects of celiac artery (CA) stenting during fenestrated-branched endovascular aneurysm repair for thoracoabdominal aortic aneurysms (TAAA) or pararenal aortic aneurysms. MethodsWe retrospectively reviewed the clinical and anatomic data on 300 consecutive patients enrolled in a prospective nonrandomized physician-sponsored investigational device exemption study from 2013 to 2018. From this group, 230 patients with CA incorporation by fenestration or directional branch were included. MAL compression was defined by preoperative computed tomography angiogram as a J-hook narrowing of the proximal CA at the level of the ligament; the shift angle between the downward and upward segments within the CA was measured. End points were technical success, rates of intraoperative or early (30-days) CA branch revision, and freedom from target vessel instability, defined by any death or rupture owing to target vessel complication, occlusion, or reintervention for stenosis, endoleak, or disconnection. ResultsCA incorporation was performed using fenestrations in 118 patients (51%) and directional branches in 112 (49%). MAL compression was present in 97 patients (42%), resulting in a stenosis of more than 50% in 48 (49%). MAL compression was more often present in patients with extent I to III TAAAs compared with extent IV TAAA-pararenal aortic aneurysms (56% vs 31%; P < .001). Technical success rate was 99%. Patients with MAL compression more often received a directional branch (65% vs 37%; P < .001), self-expanding bridging stent grafts (32% vs 16%; P = .007), adjunctive bare metal stents (46% vs 24%; P = .001), and coverage of the gastric artery (44% vs 22%; P < .001). An intraoperative (n = 6, 2.6%) or early (n = 1, 0.4%) revision of the CA branch was required in seven patients (3%) owing to dissection/occlusion (n = 2 [0.9%]), kinking/stenosis (n = 3 [1.3%]), stent dislodgement (n = 1 [0.4%]), or type IC endoleak (n = 1 [0.4%]). A shift angle of less than 120° was the most significant factor associated with CA branch revision (odds ratio, 10.9; 95% confidence interval, 2.3-88.9; P = .013). Freedom from CA branch instability was 97 ± 2% at 4 years, and this outcome was not associated with MAL compression (hazard ratio, 0.83; 95% confidence interval, 0.14-5.02; P = .588) or any other predictor. ConclusionsMAL compression was more common in extent I to III TAAAs, and related to additional challenges for CA stenting in fenestrated-branched endovascular aneurysm repair. This process may include bare metal stenting, gastric artery coverage, or early revision, especially in presence of an angulation of less than 120°. However, durable results can be achieved for CA incorporation despite these difficulties.

A Case Report of Stent Placement for Celiac Artery Stenosis Caused by Median Arcuate Ligament Compression Syndrome

A Case Report of Stent Placement for Celiac Artery Stenosis Caused by Median Arcuate Ligament Compression Syndrome

Pancreaticoduodenectomy following total occlusion of the superior mesenteric artery: a case report and literature review

BackgroundPatients with chronic occlusion of the celiac artery and superior mesenteric artery (SMA) are often asymptomatic, and occlusion may be caused by arteriosclerosis or median arcuate ligament compression. Pancreaticoduodenectomy (PD) is occasionally performed for patients with celiac artery occlusion; however, reports on patients with SMA occlusion are rare. We report a patient with cholangiocarcinoma and total atherosclerotic occlusion of the SMA without preoperative stenting or bypass.Case presentationA 73-year-old man suspected to have lower bile duct carcinoma was admitted to our hospital for further treatment. Three-dimensional computed tomography (3DCT) showed a common bile duct tumor and total occlusion of the SMA with collateral circulation of the gastroduodenal artery (GDA) and inferior mesenteric artery (IMA). We performed a PD. During the operation, we used test clamping of the GDA, which revealed no bowel ischemia. The postoperative course was uneventful, and the patient was discharged on postoperative day (POD) 30. 3DCT on POD 98 and POD 307 showed development of collateral circulation between the IMA and SMA.ConclusionHere, we report the case of a patient with total occlusion of the SMA who subsequently underwent PD. 3DCT was instrumental in gathering vascular collateral information and thus we conclude that the assessment of collateral circulation before surgery is important.

Median Arcuate Ligament Compression in Orthotopic Liver Transplantation: Results from a Single-Center Analysis and a European Survey Study.

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.

Pancreaticoduodenal artery aneurysm: Treatment outcomes of a rare disorder

Objective: Pancreaticoduodenal artery aneurysm is a rare vascular disorder that manifests with a clinical presentation ranging from asymptomatic cases to hemorrhagic shock due to aneurysm rupture. In this study, we aimed to evaluate the outcomes of patients treated with different methods for pancreaticoduodenal artery aneurysm. Material and Methods: A total of 5 patients who were treated and followed for pancreaticoduodenal artery aneurysm between January 2011 and January 2018 were enrolled in the study. Standard endovascular embolization and surgical resection were performed. The demographic findings and treatment outcomes were compared. Results: All patients were shown to have a pancreaticoduodenal artery aneurysm by radiological methods. The most common complaint was abdominal pain. The mean aneurysm size was 2.8 cm. Two patients had coeliac axis stenosis and occlusion, one had hypertension, one had Marfan syndrome, and one had chronic pancreatitis as the possible etiological cause. One patient with occlusion of the coeliac axis had thrombus, one patient with coeliac axis stenosis had median arcuate ligament compression. Three patients were treated with embolization, one with surgery, and one with low-molecular weight heparin. Conclusion: Different etiological factors and multiple vascular problems accompanying the disorder create difficulties for establishing a treatment algorithm. Due to the rare nature of the disease, studies available in the literature involve limited numbers of patients, as was also the case in our study. There appeared to be no significant differences between the treatment and follow-up duration among the different treatment methods we applied for our patients.

Celiac axis stenosis due to median arcuate ligament compression in a patient who underwent pancreatoduodenectomy; intraoperative assessment of hepatic arterial flow using Doppler ultrasonography: a case report

BackgroundCeliac axis stenosis due to compression by the median arcuate ligament has been reported in patients undergoing pancreaticoduodenectomy; it leads to the development of major collateral pathways that feed the hepatic artery. Dividing these important collaterals during pancreaticoduodenectomy can cause ischemic complications which may lead to a high mortality rate. To prevent these complications, it is necessary to assess intrahepatic arterial flow.Case presentationA 71-year-old Japanese man with anorexia was referred to us for the treatment of alcoholic chronic pancreatitis. Computed tomography revealed a pancreatic head tumor with a calculus, associated with the dilatation of the main pancreatic duct and intrahepatic bile duct. Three-dimensional imaging demonstrated focal narrowing in the proximal celiac axis due to median arcuate ligament compression and a prominent gastroduodenal artery that fed the common hepatic artery. The preoperative diagnosis was alcoholic chronic pancreatitis with common bile duct obstruction and celiac axis stenosis due to median arcuate ligament compression. Pancreaticoduodenectomy with median arcuate ligament release was scheduled. Before the division of the median arcuate ligament, the peak flow velocity and resistive index of his intrahepatic artery measured with Doppler ultrasonography decreased from 37.7 cm/second and 0.510, respectively, to 20.6 cm/second and 0.508 respectively, when his gastroduodenal artery was clamped. However, these values returned to baseline levels after the division of the median arcuate ligament. These findings suggested that pancreaticoduodenectomy could be performed safely. Our patient was discharged on postoperative day 17 without significant complications.ConclusionThe intraoperative quantitative evaluation of intrahepatic arterial blood flow using Doppler ultrasonography was useful in a patient who underwent pancreaticoduodenectomy, who had celiac axis stenosis due to compression by the median arcuate ligament.