Pancreaticoduodenectomy in Patients With Coeliac or Superior Mesenteric Artery Stenosis: A Review of the Literature.

Pancreaticoduodenectomy (Whipple's procedure) is a technically demanding operation performed for malignant and premalignant conditions of the pancreatic head, duodenum and bile duct. Awareness of the vascular anatomy, variations, and pathology of this area is essential to achieve safe surgery and good outcomes. The operation involves division of the gastroduodenal artery (GDA) which provides communication between the foregut and midgut blood supply. In patients with coeliac or superior mesenteric artery (SMA) stenosis, this can lead to reduced blood supply to the foregut or midgut organs, with consequent severe ischaemic complications leading to significant morbidity and mortality. Coeliac artery stenosis is caused by median arcuate ligament syndrome (MALS) in the majority of patients with atherosclerosis being the second most common cause. SMA stenosis is much less common and is caused in the majority of cases by atherosclerosis. A review of preoperative imaging and intraoperative gastroduodenal artery clamp test is important to identify cases that may need additional procedures to preserve the blood supply. In this paper, we present a literature review for studies reporting patients undergoing Whipple's operation with concomitant coeliac axis stenosis (CAS) or SMA stenosis. Analysis of causes of stenosis or occlusion, prevalence, risk factors, different management strategies and outcomes was conducted.

Post-surgical Median Arcuate Ligament Syndrome (MALS) Symptom Exacerbation Treated With Osteopathic Manipulative Treatment (OMT): A Case Report.

Median arcuate ligament syndrome (MALS, also known as celiac artery compression syndrome, celiac axis syndrome, celiac trunk compression syndrome, Dunbar syndrome, or Harjola-Marable syndrome) is a rare condition characterized by abdominal pain attributed to the compression of the celiac artery and celiac ganglia by the median arcuate ligament. Pain can occur post-prandially and may be accompanied by weight loss, nausea, or vomiting. Following angiographic diagnosis, current definitive treatment may include open or laparoscopic decompression surgery with celiac ganglion removal (if affected), which has been found to provide relief. In this case report, we outline a young female patient with a MALS diagnosis and subsequent surgery, but whose pain recurred in various stress-related instances even after surgical intervention. After a particular pain episode, osteopathic manipulative treatment (OMT) was applied, with a focus on restoring autonomic balance through the use of various gentle osteopathic treatment techniques. A significant reduction in pain was reported post-treatment, followed by complete pain resolution, indicating a great benefit to the incorporation of OMT into the treatment plan of MALS patients in future osteopathic practice.

Laparoscopic Treatment of a Symptomatic Young Woman With Median Arcuate Ligament Syndrome.

Median arcuate ligament syndrome (MALS), also known as Dunbar syndrome, celiac axis syndrome, or celiac artery compression syndrome, is caused by a band of tissue called the median arcuate ligament that compresses the celiac artery and sometimes the celiac plexus too. MALS does not always cause symptoms, but when symptoms occur, surgery is the treatment of choice. This case report focuses on the case of a 27-year-old woman presenting with postprandial episodes of abdominal pain and vomiting accompanied by loss of weight, which was found to be MALS.

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.

Celiac Artery Compression Syndrome in an Adolescent Male.

Celiac artery compression syndrome is not frequent in the pediatric population. The syndrome may entail long-standing abdominal pain, recurrent vomiting, bloating, weight loss, and an abdominal bruit, which in the case of our patient, was an incidental finding. Notably, patients may be asymptomatic.  Our patient is a 16-year-old male who presented with concerns about multiple, non-tender chest lymph nodes lasting for two weeks. He had also lost 80 lbs. over one year. On examination, however, an abdominal bruit was discovered, and a diagnostic workup was significant for celiac artery compression following a magnetic resonance angiography of the abdomen. Due to his significant weight loss and mediastinal lymphadenopathy, a chest computed tomography (CT) scan was done to rule out malignancy. The chest CT scan was reported as normal. Additionally, a renal duplex ultrasound was done to rule out renal artery stenosis, considering he had presented with elevated blood pressure; this was also unremarkable. Although this patient had a history of marijuana use, his assessment did not show marked dependence. Substance abuse and atherosclerotic vascular disease can be predisposing factors for celiac artery compression syndrome in older individuals. However, compression of the celiac trunk by the median arcuate ligament is a congenital anomaly more appreciated in younger age groups. The patient was referred to vascular surgery for possible median arcuate ligament release.

Recurrent aneurysmatic bleeding of pancreaticoduodenal aneurysm due to median arcuate ligament syndrome: a case report.

Median arcuate ligament syndrome (MALS) involves coeliac artery compression, causing a range of symptoms from chronic pain to life-threatening complications. This case features a 52-year-old patient with recurrent retroperitoneal bleeding from MALS-related inferior pancreaticoduodenal artery aneurysms (PDAAs). Emergency interventions, including surgical bleeding control, angioplasty, percutaneous drainage, and median arcuate ligament release, were conducted. The case highlights challenges in diagnosing and managing MALS-related PDAA, emphasizing the importance of early identification and tailored interventions based on clinical symptoms and imaging. Surgical intervention to release the ligament is the primary treatment, with considerations for prophylactic intervention in PDAA cases. Lack of established PDAA management protocols underscores the need for prompt intervention to prevent complications. In conclusion, this report stresses the association between MALS and PDAA, advocating for early identification and tailored management to mitigate complications.

Median Arcuate Ligament Syndrome (MALS) in Hepato-Pancreato-Biliary Surgery: A Narrative Review and Proposed Management Algorithm.

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?

Median arcuate ligament syndrome: A rare case report from Nepal.

INTRODUCTION: Median arcuate ligament syndrome (MALS) is a rare condition caused by the compression of the celiac trunk by the median arcuate ligament, leading to a typical symptom triad: postprandial abdominal pain, weight loss, nausea, and vomiting. CASE PRESENTATION: A 41-year-old female patient presented to our center with mild postprandial abdominal pain over the epigastric region, and bloating sensation. Ultrasonography of the abdomen showed multiple stones in the gall bladder lumen, and the computed tomography scan showed median arcuate ligament impingement along the proximal aspect of the celiac trunk causing moderate narrowing with post-stenotic dilation. Laparoscopic release of the median arcuate ligament with laparoscopic cholecystectomy was performed. DISCUSSION: The diagnosis of Median Arcuate Ligament Syndrome is based on the classical post-prandial symptoms and abdominal imaging technologies like Doppler ultrasonography, computed tomography angiography, or magnetic resonance angiography. Exclusion of other intestinal disorders should be considered before making the diagnosis. Celiac artery decompression through different means is the principle of treatment of this condition. CONCLUSION: The diagnosis of median arcuate ligament syndrome should be considered in patients with postprandial abdominal pain that does not have an established etiology. Celiac artery decompression by releasing the median arcuate ligament is the treatment.

Clinical outcomes after surgical decompression of median arcuate ligament syndrome-An observational study.

BACKGROUND AND OBJECTIVES: Median arcuate ligament syndrome is caused by compression and stenosis of the celiac artery. Incision of the median arcuate ligament improves persistent abdominal symptoms. The study aimed at evaluating the outcomes in patients who underwent median arcuate ligament syndrome decompression using a self-report questionnaire. METHODS: This single-center retrospective study included patients with median arcuate ligament syndrome who underwent decompression surgery between April 2021 and February 2023. The medical records were retrospectively reviewed. RESULTS: Ten patients were included in the study. Laparotomy and laparoscopic surgeries were performed in seven and three patients, respectively. The median operation time was 147 minutes. The median hospitalization period after the operation was seven days. The degrees of celiac artery stenosis before and after surgery were compared and the per cent diameter stenosis did not significantly improve; five of 10 patients (50%) had > 50% stenosis in the celiac artery after the operation. Compared to the baseline, the scores of upper gastrointestinal symptoms significantly improved during the six months' period (p < 0.001). Additionally, we evaluated the influence of post-operative per cent diameter stenosis and divided the patients into two groups (≥ 50% vs, < 50%). The scores of upper gastrointestinal (GI) symptoms in both groups improved significantly from baseline. However, the symptomatic improvement at six months in the post-operative per cent diameter stenosis < 50% group was significantly greater than that in the ≥ 50% group (p = 0.016). The scores of lower gastrointestinal symptoms did not change significantly during the six-month period. CONCLUSION: Decompression surgery for median arcuate ligament syndrome could improve upper gastrointestinal symptoms regardless of the post-operative per cent diameter stenosis.

Examining the impact of median arcuate ligament-induced celiac artery compression on target vessel patency, long-term survival and device integrity in fenestrated and branched endovascular repairs.

OBJECTIVE: To evaluate the impact of celiac artery (CA) compression by median arcuate ligament (MAL) on technical metrics and long-term CA patency in patients with complex aortic aneurysms undergoing fenestrated/branched endograft repairs (F/B-EVARs). METHODS: Single-center, retrospective review of patients undergoing fenestrated/branched endovascular aortic aneurysm repairs and requiring incorporation of the CA between 2013 and 2023. Patients were divided into two groups-those with (MAL+) and without (MAL-) CA compression-based on preoperative computed tomography angiography findings. MAL was classified in three grades (A, B, and C) based on the degree and length of stenosis. Patients with MAL grade A had ≤50% CA stenosis measuring ≤3 mm in length. Those with grade B had 50% to 80% CA stenosis measuring 3 to 8 mm long, whereas those with grade C had >80% stenosis measuring >8 mm in length. End points included device integrity, CA patency and technical success-defined as successful implantation of the fenestrated/branched device with perfusion of CA and no endoleak. RESULTS: One hundred and eighty patients with complex aortic aneurysms (pararenal, 128; thoracoabdominal, 52) required incorporation of the CA during fenestrated/branched endovascular aortic aneurysm repair. Majority (73%) were male, with a median age of 76 years (interquartile range [IQR], 69-81 years) and aneurysm size of 62 mm (IQR, 57-69 mm). Seventy-eight patients (43%) had MAL+ anatomy, including 33 patients with MAL grade A, 32 with grade B, and 13 with grade C compression. The median length of CA stenosis was 7.0 mm (IQR, 5.0-10.0 mm). CA was incorporated using fenestrations in 177 (98%) patients. Increased complexity led to failure in CA bridging stent placement in four MAL+ patients, but completion angiography showed CA perfusion and no endoleak, accounting for a technical success of 100%. MAL+ patients were more likely to require bare metal stenting in addition to covered stents (P = .004). Estimated blood loss, median operating room time, contrast volume, fluoroscopy dose and time were higher (P < .001) in MAL+ group. Thirty-day mortality was 3.3%, higher (5.1%) in MAL+ patients compared with MAL- patients (2.0 %). At a median follow-up of 770 days (IQR, 198-1525 days), endograft integrity was observed in all patients and CA events-kinking (n = 7), thrombosis (n = 1) and endoleak (n = 2) -occurred in 10 patients (5.6%). However, only two patients required reinterventions. MAL+ patients had overall lower long-term survival. CONCLUSIONS: CA compression by MAL is a predictor of increased procedural complexity during fenestrated/branched device implantation. However, technical success, long-term device integrity and CA patency are similar to that of patients with MAL- anatomy.

Median Arcuate Ligament Syndrome in Children: A Single-Center Experience.

BACKGROUND: Data on median arcuate ligament syndrome (MALS) in children are scant. It is postulated that MALS can cause chronic abdominal pain. It is unclear what percentage of children with this condition are symptomatic and what comorbidities are associated with this syndrome. METHODS: In this retrospective study, data on consecutive patients in a single center diagnosed coincidentally with MALS during routine echocardiogram were reviewed. Symptom burden, comorbidities, and the effect of anthropometric indices on MALS were investigated. Descriptive statistics and nonparametric tests were used to describe the findings and to compare variables with normal distribution. RESULTS: Between 2013 and 2020, there were 82 children, 55 females (67%), mean age 13.9 ± 3.2 years, with MALS and complete record. Mean velocity across the stenotic area was 2.6 ± 0.4 m/s. Forty-six patients (57%) had abdominal pain. Age, gender, weight, body mass index (BMI), and Doppler velocity had no statistically significant influence on symptom occurrence. Conversely, patients with joint hypermobility and symptoms of orthostatic intolerance were more likely to have abdominal pain from MALS. Of 24 patients with joint hypermobility, 18 patients had abdominal pain (p=0.027). Thirty-eight patients with orthostatic intolerance (OI) with MALS complained of abdominal pain vs 13 patients with OI and no abdominal pain (p=<0.0001). CONCLUSION: Nearly half of patients with MALS had abdominal pain. Age, gender, weight, and the degree of stenosis had no statistically significant influence on symptom occurrence. OI, specifically postural orthostatic tachycardia syndrome (POTS), and joint hypermobility on exam predicted a higher propensity for abdominal pain in patients with MALS.

Vascular compression syndromes in the abdomen and pelvis: a concise pictorial review.

Vascular compression syndromes are a diverse group of pathologies that can manifest asymptomatically and incidentally in otherwise healthy individuals or symptomatically with a spectrum of presentations. Due to their relative rarity, these syndromes are often poorly understood and overlooked. Early identification of these syndromes can have a significant impact on subsequent clinical management. This pictorial review provides a concise summary of seven vascular compression syndromes within the abdomen and pelvis including median arcuate ligament (MAL) syndrome, superior mesenteric artery (SMA) syndrome, nutcracker syndrome (NCS), May-Thurner syndrome (MTS), ureteropelvic junction obstruction (UPJO), vascular compression of the ureter, and portal biliopathy. The demographics, pathophysiology, predisposing factors, and expected treatment for each compression syndrome are reviewed. Salient imaging features of each entity are illustrated through imaging examples using multiple modalities including ultrasound, fluoroscopy, CT, and MRI.

Exploring celiac trunk parameters in median arcuate ligament syndrome: A CT study.

PURPOSE: To assess anatomical variations in the celiac trunk (Ct) in patients with Median Arcuate Ligament Syndrome (MALS) using computed tomography (CT). The primary objectives were to investigate the celiac trunk angle (CtA), origin level, length (CtL), and their relationships with the superior mesenteric artery (SMA) in MALS patients. Additionally, the study intended to evaluate gender differences in these parameters and explore correlations between variables. METHODS: Retrospectively, reports of abdominal CT scans taken between January 2018, and Sepmtember 2021, in the hospital image archive were screened vey two observers independently for MALS diagnosis. Parameters such as CtA, CtL, Ct-SMA distance, SMA angle (SMAA), and median arcuate ligament thickness (MALT) were measured. Statistical analyses were conducted using SPSS software. RESULTS: Among the 81 patients (25 females, 56 males), significant differences were observed in MALT between genders (p = 0.001). CtA showed a negative correlation with CtL and Ct-SMA (p < 0.001), and a positive correlation was found between CtL and Ct-SMA (p = 0.002). CtL was measured as 25 mm for the all group. Origin levels of Ct and SMA were evaluated in comparison to vertebral levels. Ct-SMA distance was relatively shorter (9.19 mm) compared to the literature. SMAA findings were consistent with normal population values. CONCLUSION: This study provided valuable insights into the anatomical parameters of the Ct ans SMA in MALS patients. Despite some differences compared to normal population parameters, no evidence supported the hypothesis of a superiorly placed Ct contributing to MALS.

Endovascular Treatment of Postpancreatectomy Hemorrhage from the Retroportal Artery with Compression of the Celiac Trunk by the Median Arcuate Ligament: A Case Report.

Retroportal artery is one of the communicating arteries between the hepatic artery and the superior mesenteric artery, but it is often a small artery and usually unrecognized. We report a 60-year-old man that was successfully treated for postpancreatectomy hemorrhage from the retroportal artery with compression of the celiac trunk by the median arcuate ligament. Following the pancreaticoduodenectomy, the bloody discharge was discovered through the drainage catheter. We underwent transcatheter arterial embolization for the bleeding from the retroportal artery associated with a postoperative pancreatic fistula. Additionally, because a stenosis of the common hepatic artery was discovered, we consequently installed a stent-graft on the common hepatic artery to prevent the liver failure due to decreased hepatic blood flow.

Laparoscopic management of median arcuate ligament syndrome: a video vignette.

Median arcuate ligament syndrome (MALS) is a rare condition and a diagnosis of exclusion. We present a 30-year-old man, who had postprandial upper abdominal pain and weight loss of 6 kg in 3 months. His gastroscopy and abdominal ultrasound results were both unremarkable. Computed tomographic angiography showed characteristic compression of the celiac artery by thickened median arcuate ligament causing a 'J' shaped course of artery with poststenotic dilatation and dilated branches of the celiac artery. The patient underwent laparoscopic release of the median arcuate ligament. The intraoperative blood loss was 20 mL and duration of the procedure was 140 minutes. The patient had an uneventful recovery and was discharged on postoperative day 2. The symptoms subsided 2 months following surgery and he started gaining weight. Laparoscopic division of the median arcuate ligament is a minimally invasive, safe, and effective method to decompress the celiac artery.

Laparoscopic treatment for median arcuate ligament syndrome in children: A case report.

In median arcuate ligament syndrome (MALS), the median arcuate ligament compresses the celiac trunk and surrounding nerves leading to chronic functional abdominal pain and vague gastrointestinal symptoms. MALS can be effectively treated by dividing the arcuate ligament through open surgery or laparoscopy. This is a rare vascular condition and mostly encountered in adult patients. We hereby report a case of a pediatric patient diagnosed with MALS and treated successfully by laparoscopic approach. An 11-year-old girl presented with severe abdominal cramps for 3 months, accompanied by nonbilious vomiting. Computed tomography (CT) angiography demonstrated clear images of celiac trunk compression suggesting MALS. Laparoscopic surgery to cut the ligament and decompress the celiac artery was performed. The patient was discharged on day 7 postoperative with no recurrence of symptoms after 12 months of follow-up. This report suggested the diagnostic value of CT scan, and the safety and the feasibility of laparoscopic surgical techniques to treat MALS in children.

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

INTRODUCTION: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

BACKGROUND: Median 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 PRESENTATION: A 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. CONCLUSIONS: Reports 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.

Characteristics and Trends in Median Arcuate Ligament Syndrome (MALS) Associated Visceral Artery Aneurysms: A Systematic Descriptive Review of the Literature.

Introduction: Median Arcuate Ligament Syndrome (MALS) is associated with true aneurysms, mainly of both the pancreaticoduodenal artery (PDA) and gastroduodenal artery (GDA). Although rare, their potential for rupture and adverse clinical outcomes warrants analysis. Prior studies suggest high rupture rates even for smaller aneurysms under 2 cm in this setting. We performed a systematic literature review, synthesising the evidence on visceral artery aneurysms related to MAL syndrome, with a focus on descriptive analyses of aneurysm size, presentation, rupture rates, and management. Methods: Literature search was performed using (Medline, EMBASE, Emcare and CINAHL). Inclusion criteria included true aneurysms secondary to MALS with or without rupture. The cases with pseudoaneurysms, concomitant pathologies eg, pancreatitis, conservatively managed aneurysms and articles with non-granular pooled data were excluded. Cases were assessed according to demographics, clinical presentation, aneurysm diameter, aneurysm rupture and management technique. Results: 39 articles describing 72 patients were identified. Aneurysm diameter in symptomatic patients was not significantly different from asymptomatic patients {21.0 and 22.3 mm respectively, P = .84}. Ruptured aneurysms were overall smaller than non-ruptured at presentation {12.3 mm v 30.8 mm respectively, P = .02}. Patients presented with abdominal pain (75.6%), nausea/vomiting (15.6%), hypotension (33.9%), shock (20.0%) and haemodynamic collapse (8.9%). 56.9% of all cases were managed with an endovascular approach, 19.4% were managed with an open surgical approach, and 23.6% were managed hybrid. Conclusion: This review suggests visceral artery aneurysms associated with median arcuate ligament rupture at variable sizes. Despite inability to clearly correlate size and rupture risk, our data supports prompt intervention irrespective of size, given the adverse outcomes. Further research is critically needed to clarify size thresholds or other predictors to guide management.

Pancreaticoduodenal Artery Aneurysm Complicated by Median Arcuate Ligament Syndrome.

PURPOSE: 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. CASE REPORT: 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. CONCLUSION: 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.