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Introduction: Median arcuate ligament syndrome (MALS) is an uncommon cause of chronic abdominal pain resulting from the compression of the celiac artery. It shares symptoms with chronic functional abdominal pain, a more common cause of pediatric chronic abdominal pain. Typically found in middle-aged females, MALS is a diagnosis of exclusion.Case Report: A 17-year-old male who presented to the emergency department with periumbilical pain for two months was subsequently diagnosed with MALS through computed tomography angiography. Further vascular and gastroenterology evaluations confirmed the diagnosis, ruling out other common causes of chronic abdominal pain. The patient received non-operative treatment in the form of endoscopic ultrasound celiac plexus block, with the possibility of surgical management if necessary.Conclusion: Median arcuate ligament syndrome is an uncommon cause of chronic abdominal pain that is difficult to differentiate from other causes, especially in pediatric patients. It should be considered in the patient whose previous workup was not conclusive and symptom management had failed. Management is multidisciplinary with non-operative management preferred initially. If there is no improvement, surgical management should be considered.

Background: Median arcuate ligament syndrome (MALS) poses a considerable challenge in terms of diagnosis due to its manifestation of diverse symptoms linked to constriction of the median arcuate ligament surrounding the celiac artery. The present study introduces an earlier diagnostic modality using ultrasound measurements of the flow velocity of the celiac artery during the inspiratory and expiratory phases, with the latter being higher than the former, to avoid prolonged follow-up of postprandial symptomatology. Case Report: A 46-year-old female patient presented with acute postprandial abdominal pain, which was alleviated by analgesic medication. The findings from the physical examination and laboratory tests were within normal limits. Further investigations were conducted due to persistent symptoms, revealing an elevation in celiac artery flow velocity during expiration on ultrasound. The diagnosis of median arcuate ligament syndrome (MALS) was confirmed through contrast-enhanced CT and angiography. Subsequently, the patient underwent laparoscopic release of the median arcuate ligament, leading to alleviation of symptoms at the 1-year follow-up assessment. Conclusions: Our case report highlights the importance of a dynamic imaging diagnostic strategy for MALS. When encountering challenging postprandial abdominal pain that is hard to diagnose, it could be crucial to utilize abdominal ultrasound to measure the flow velocity of the celiac artery. This approach may serve as a valuable screening method for identifying MALS and, subsequently, prompt the need for further diagnostic tests. [ABSTRACT FROM AUTHOR]

Median arcuate ligament syndrome (MALS) is rare syndrome and is diagnosis of exclusion. Though first robotic median arcuate ligament release (MALR) was described in 2007, there are only a few case reports (CR), case series (CS), and retrospective cohort studies (RCS) in the published literature. The purpose of this study was to assess the feasibility and safety of robotic-assisted MALR (r-MALR). PubMed, Embase, and Cochrane databases were searched (last search December 2023) for articles reporting r-MALR. All studies including CR, CS, RCS reporting technical feasibility (operating time and rate of conversions), safety (intra-operative complications, blood loss, post-operative complications), and outcome (length of stay [LoS]) were included (PROSPERO: CRD42024502792). A total of 23 studies (8-CR, 5-CS, 10-RCS) with total 290 patients were available in the literature. Except 1-RCS, all studies reported age and mean age for CR was 40.38 ± 17.7, 36.08 ± 15.12 for CS, 39.72 ± 7.35 years for RS; except 2-RCS, all studies reported gender distribution and there were 57 males and 214 females. Operating time: 3-CR, 4-CS, 9-RCS reported operating time, and mean time was 111 ± 54, 131.69 ± 7.51, 117.34 ± 35.03 min, respectively. Conversion rate: All studies reported data on conversion and only four (1.37%) cases were converted—one to laparoscopic approach, three to open approach (1—inadvertent celiac arteriotomy, 2—reasons not mentioned). Intraoperative complications: only 5-CR, 4-CS, and 9-RCS reported data on intra-operative complications, and there were only 6 complications (1.5%): 1—inadvertent celiac arteriotomy converted to open; 3—unnamed vascular injuries (2 managed robotically, 1 converted to open); 1—bleeding managed robotically; there were no other reported injuries. Estimated blood loss ranged from 5 to 30 ml. Post-operative complications: 5-CR, 4-CS, 8-RCS described post-operative complications in 21 (7%) patients. Twenty cases were grade I, one was grade IIIa, and all managed successfully. LoS stay: 2-CR, 4-CS, 10-RCS reported LoS and overall, it was 2 days. r-MALR is reasonable, technically feasible, safe, and has acceptable outcomes. In addition, robotic approach provided superior vision, improved dexterity, precise, and easy circumferential dissection. PROSPERO registration: The protocol was registered in the PROSPERO database (CRD42024502792). [ABSTRACT FROM AUTHOR]

Celio-mesenteric trunk (CMT) is a rare vascular variation of the ventral branches of the abdominal aorta that supply blood to the mesentery and the gut. This rare variation is seen in 2/100.000 population. The presence of this anomaly is associated with an increased risk of mesenteric ischemia in the case of proximal occlusion. Median arcuate ligament (MAL) syndrome is a controversial entity characterized by compression of the celiac axis by MAL causing post-prandial pain. We report the fourth case of MAL compression syndrome involving a CMT in the world

Median arcuate ligament (MAL) syndrome or celiac artery (CA) compression syndrome previously treated mainly by vascular surgeons using the open approach is now being increasingly performed by general surgeons with training in advanced laparoscopy. Although this approach has all the advantages of minimal access surgery, the procedure is fraught with serious complications like injury to major vascular structures during dissection. Vascular injury by far is the major cause of conversion to open procedure. Herein, we report a laparoscopic repair of iatrogenic CA injury by intra-corporeal suturing during MAL release. We also elaborate the causes, the preventive measures that can be applied to avoid such catastrophic occurrences in future. To the best of our knowledge, this is the first report of a laparoscopic repair of CA bleed during MAL release.

Background: Median arcuate ligament syndrome (MALS), also known as Dunbar syndrome or celiac artery (CA) compression syndrome, is a rare condition characterized by persistent post-meal or post-exercise abdominal discomfort, often more pronounced during expiration. Additional symptoms include nausea, vomiting, and weight loss. Aims and Objectives: To document the clinical presentation, diagnostic process, and treatment outcomes of seven patients diagnosed with MALS and to evaluate the effectiveness of surgical release of the median arcuate ligament (MAL) over a follow-up period of at least six months. Materials and Methods: The study included seven patients diagnosed with MALS from 2019 to 2021. Diagnosis was based on the presence of chronic abdominal pain and associated symptoms, absence of alternative diagnoses after various clinical assessments, and angiographic evidence of CA compression. The primary treatment involved surgical decompression of the CA by releasing the MAL through either open or laparoscopic techniques. Results: All seven patients underwent successful surgical release of the MAL. The patients were followed up for at least six months post-surgery. The study evaluated the persistence of symptoms and the need for additional interventions such as revascularization techniques. Conclusion: Surgical release of the MAL is the primary treatment for MALS, providing relief for many patients. However, long-term follow-up is essential as some patients may continue to experience symptoms post-surgery, necessitating further interventions.

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.

Background: Pancreaticoduodenectomy (PD) is considered a challenging surgery for resecting the gastroduodenal artery (GDA), right gastric artery (RGA), and lymph node tumors. In cases of pancreatic head cancer surgery, vascular anastomosis or right gastroepiploic artery (RGEA)/GDA preservation is necessary after postoperative gastric tube reconstruction for esophageal cancer. Therefore, we report for the first time an extremely rare case of PD in a patient with pancreatic head cancer and median arcuate ligament syndrome (MALS) after gastric tube reconstruction following esophageal cancer surgery, in which the entire pancreatic head arcade was preserved. Case presentation: The patient was a 76-year-old man who had undergone esophageal cancer surgery after sternal gastric tube reconstruction 7 years ago. He was referred to our hospital because of the suspicion of intraductal papillary mucinous carcinoma (IPMC) owing to an enlarged cystic lesion and a substantial component in the uncinate process of the pancreas. Preoperative three-dimensional computed (3D-CT) tomography angiography showed celiac axis stenosis and pancreatic head arcade dilation. The diagnosis was IPMC without evidence of invasion; therefore, gastric tube blood flow was maintained by preserving the GDA and RGEA. Due to MALS, the GDA blood flow was supplied through the pancreatic head arcade, necessitating its preservation. The GDA-RGEA, right gastroepiploic vein, and anterior superior pancreaticoduodenal artery were taped over the entire pancreatic head for preservation. The inferior pancreaticoduodenal artery (IPDA) was also taped on the dorsal pancreas and the posterior or anterior IPDA, which further bifurcates were taped to preserve them. Subsequently, PD was performed. Conclusion: We report a case of PD after gastric tube reconstruction for esophageal cancer with MALS, in which the pancreatic head arcade vessels were successfully preserved using 3D-CT to confirm the operation of the vessels. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Median arcuate ligament syndrome (MALS), also known as Dunbar syndrome or celiac artery (CA) compression syndrome, is a rare condition characterized by persistent post-meal or post-exercise abdominal discomfort, often more pronounced during expiration. Additional symptoms include nausea, vomiting, and weight loss. Aims and Objectives: To document the clinical presentation, diagnostic process, and treatment outcomes of seven patients diagnosed with MALS and to evaluate the effectiveness of surgical release of the median arcuate ligament (MAL) over a follow-up period of at least six months. Materials and Methods: The study included seven patients diagnosed with MALS from 2019 to 2021. Diagnosis was based on the presence of chronic abdominal pain and associated symptoms, absence of alternative diagnoses after various clinical assessments, and angiographic evidence of CA compression. The primary treatment involved surgical decompression of the CA by releasing the MAL through either open or laparoscopic techniques. Results: All seven patients underwent successful surgical release of the MAL. The patients were followed up for at least six months post-surgery. The study evaluated the persistence of symptoms and the need for additional interventions such as revascularization techniques. Conclusion: Surgical release of the MAL is the primary treatment for MALS, providing relief for many patients. However, long-term follow-up is essential as some patients may continue to experience symptoms post-surgery, necessitating further interventions. [ABSTRACT FROM AUTHOR]

Median arcuate ligament (MAL) syndrome or celiac artery (CA) compression syndrome previously treated mainly by vascular surgeons using the open approach is now being increasingly performed by general surgeons with training in advanced laparoscopy. Although this approach has all the advantages of minimal access surgery, the procedure is fraught with serious complications like injury to major vascular structures during dissection. Vascular injury by far is the major cause of conversion to open procedure. Herein, we report a laparoscopic repair of iatrogenic CA injury by intra-corporeal suturing during MAL release. We also elaborate the causes, the preventive measures that can be applied to avoid such catastrophic occurrences in future. To the best of our knowledge, this is the first report of a laparoscopic repair of CA bleed during MAL release. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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? [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Abstract Background Ectopic kidney and median arcuate ligament syndrome are both rare conditions. The clinical presentation and diagnosis of these conditions are not well studied. There are no reports on the combination of these two rare conditions. Case presentation We report a 24-year-old woman with fever, dysuria, urinary frequency and left flank pain for two days. The primary diagnoses in the clinic were left acute pyelonephritis and left hydronephrosis due to throbbing pain in the left costovertebral angle and pyuria. However, further computed tomography showed right ectopic pelvic kidney, left renal pelvis dilatation without definite ureteral lesion, good bilateral renal contrast enhancement, and compression of the celiac axis due to obstruction by the median arcuate ligament. Chronic abdominal symptoms were reported by the patient after repeat history taking. The patient’s condition was fully explained and discussed with her and her family, but they refused further therapy. After the acute pyelonephritis began improving, the patient was discharged for follow-up at our outpatient clinic. Conclusion We present an extremely rare case of a combination of two rare conditions: ectopic kidney and median arcuate ligament syndrome. No study to date has reported on the relationship between the two diseases. Given the rarity of the two conditions, no evidence or even a hypothesis exists to explain the possible etiology of their combination. More reports are required to enhance the understanding of these rare conditions.

Background: Chronic abdominal pain (CAP) is a medical condition resulting in enormous economic burden and healthcare utilization costs. One understudied source of CAP is the median arcuate ligament syndrome (MALS). MALS is often not diagnosed and treated for a variety of reasons, including the fact that MALS is highly comorbid with psychological symptoms and psychiatric disorders similar to CAP. To better inform future work on the study of MALS, we undertook a pilot study to estimate the economic impact and public health burden of this condition. We hypothesized that MALS imposes a significant public health burden. Methods: Pediatric and adult patients enrolled in a prospective study undergoing multidisciplinary evaluation and treatment for MALS at a tertiary care facility were invited to participate in a brief self-report survey, the Direct and Indirect Medical Care Impact of MALS Form, to capture health care resources including procedures, surgeries, health care visits, and absenteeism (school and work). To estimate costs from the Direct and Indirect Medical Care Impact of MALS Form, the medical care usage data self-reported by patients were converted to dollar value utilizing FSC-93 billing data and corresponding current procedural terminology (CPT) codes for procedures and provider visits one year prior to surgery and then following surgery. Descriptive analyses were conducted to characterize the sample in terms of demographics and reported absences from school and work. Results: One hundred and nineteen patients (mean age = 30.9 ± 13.0) completed the questionnaires, yielding a 57% response rate. 82.4% (n = 98) of the participants were female and 90.8% (n = 108) were non-Hispanic/Latine white. The mean and median surgical follow-up periods were 5.3 and 5.4 years, respectively. Overall, median cost of provider and ancillary healthcare provider visits for each patient was (US)$19,119 including the pre-operative and postoperative visits. The mean cost for providers alone was (US)$28,908. Wilcoxon signed-ranks tests indicated that the postoperative missed number of days of school were significantly lower than the pre-surgical number of missed school days (Z = -3.36, p = 0.001). Similarly, there were significantly less missed workdays following surgery than before for the entire sample (Z = -2.86, p = 0.004). Conclusion: Median arcuate ligament syndrome imposes a large economic burden on patients and the healthcare system. The current findings, although reflective of a homogenous population, are adding to a growing body of literature suggesting that healthcare disparities play a role in the low rates of diagnosis and treatment of MALS. [ABSTRACT FROM AUTHOR]

Median arcuate ligament syndrome (MALS) is a rare disorder caused by the compression of the celiac axis by the fibrous structure of the diaphragm called the median arcuate ligament. Patients with MALS are usually undiagnosed unless characteristic symptoms such as nausea and vomiting, postprandial pain, and weight loss are presented. We report a case of a 29-year-old patient diagnosed with MALS and secondary antiphospholipid syndrome (APS) that developed celiac trunk, common hepatic artery and splenic artery thrombosis. There is not enough information on MALS as a trigger of thrombosis in predisposed patients such as those with APS. However, the case gives rise to suspicion and highlights the diagnostic processes, especially for patients with APS presenting postprandial abdominal pain and weight loss. This review likewise aims at the importance of Doppler ultrasonography as a screening tool and computer tomography (CT) or magnetic resonance (MR) both in the angiography variant, especially to diagnose confirmation and underlying treatment options. [ABSTRACT FROM AUTHOR]

Rare vascular conditions frequently pose a diagnostic and therapeutic dilemma for health care providers. Several of these conditions have distinct relevance to females populations but, due to their infrequency, there has been little reported on the outcomes of rare vascular conditions specifically in females populations. We performed a literature review of a selection of three rare vascular conditions known to either disproportionately affect females (median arcuate ligament syndrome and fibromuscular dysplasia) or have unique manifestations in females populations (vascular Ehlers-Danlos syndrome). We performed a descriptive review of the literature focused on these three vascular conditions and identified aspects of the current available research describing sex-based differences in prevalence, any pathophysiology explaining the observed sex-based differences, and the contribution of sex to outcomes for each disease process. In addition, considerations for pregnant females with respect to each rare vascular disease process are discussed. [ABSTRACT FROM AUTHOR]

Background: Ectopic kidney and median arcuate ligament syndrome are both rare conditions. The clinical presentation and diagnosis of these conditions are not well studied. There are no reports on the combination of these two rare conditions. Case presentation: We report a 24-year-old woman with fever, dysuria, urinary frequency and left flank pain for two days. The primary diagnoses in the clinic were left acute pyelonephritis and left hydronephrosis due to throbbing pain in the left costovertebral angle and pyuria. However, further computed tomography showed right ectopic pelvic kidney, left renal pelvis dilatation without definite ureteral lesion, good bilateral renal contrast enhancement, and compression of the celiac axis due to obstruction by the median arcuate ligament. Chronic abdominal symptoms were reported by the patient after repeat history taking. The patient's condition was fully explained and discussed with her and her family, but they refused further therapy. After the acute pyelonephritis began improving, the patient was discharged for follow-up at our outpatient clinic. Conclusion: We present an extremely rare case of a combination of two rare conditions: ectopic kidney and median arcuate ligament syndrome. No study to date has reported on the relationship between the two diseases. Given the rarity of the two conditions, no evidence or even a hypothesis exists to explain the possible etiology of their combination. More reports are required to enhance the understanding of these rare conditions. [ABSTRACT FROM AUTHOR]

The objective of the study is to estimate the incidence of celiac artery compression (CAC) using computed tomography (CT) scans and to compare the results to the clinical symptoms of patients presenting for abdominal CT scan in a hospital. Contrast-enhanced CT abdomen scans of patients were reviewed between August 2022 and October 2022. If the celiac axis appeared to have a J-shaped or hooked morphology with stenosis, it was termed CAC. The clinical history of the patients was examined for information on food phobia, weight loss, and gastrointestinal symptoms (abdominal discomfort, nausea, vomiting, constipation, and diarrhea). Patients with CAC had a lower incidence of symptoms compared with those without CAC. However, CAC is not an uncommon CT finding in patients presenting for a CT scan. [ABSTRACT FROM AUTHOR]

Median arcuate ligament (MAL) syndrome (MALS) is a rare condition caused by compression of the celiac artery by the MAL. Symptoms include abdominal pain, nausea, and weight loss. Rarely, the MAL can compress both the celiac artery and the superior mesenteric artery (SMA). We describe the case of a young man with MALS involving the celiac artery and SMA. Laparoscopic release of the MAL was performed, and the patient had resolution of his symptoms at 6 months of follow-up. A review of the literature identified only six cases of MALS involving the SMA and celiac artery, making this a rare occurrence.

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

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

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

BackgroundChronic abdominal pain (CAP) is a medical condition resulting in enormous economic burden and healthcare utilization costs. One understudied source of CAP is the median arcuate ligament syndrome (MALS). MALS is often not diagnosed and treated for a variety of reasons, including the fact that MALS is highly comorbid with psychological symptoms and psychiatric disorders similar to CAP. To better inform future work on the study of MALS, we undertook a pilot study to estimate the economic impact and public health burden of this condition. We hypothesized that MALS imposes a significant public health burden.MethodsPediatric and adult patients enrolled in a prospective study undergoing multidisciplinary evaluation and treatment for MALS at a tertiary care facility were invited to participate in a brief self-report survey, the Direct and Indirect Medical Care Impact of MALS Form, to capture health care resources including procedures, surgeries, health care visits, and absenteeism (school and work). To estimate costs from the Direct and Indirect Medical Care Impact of MALS Form, the medical care usage data self-reported by patients were converted to dollar value utilizing FSC-93 billing data and corresponding current procedural terminology (CPT) codes for procedures and provider visits one year prior to surgery and then following surgery. Descriptive analyses were conducted to characterize the sample in terms of demographics and reported absences from school and work.ResultsOne hundred and nineteen patients (mean age = 30.9 ± 13.0) completed the questionnaires, yielding a 57% response rate. 82.4% (n = 98) of the participants were female and 90.8% (n = 108) were non-Hispanic/Latine white. The mean and median surgical follow-up periods were 5.3 and 5.4 years, respectively. Overall, median cost of provider and ancillary healthcare provider visits for each patient was (US)$19,119 including the pre-operative and post-operative visits. The mean cost for providers alone was (US)$28,908. Wilcoxon signed-ranks tests indicated that the postoperative missed number of days of school were significantly lower than the pre-surgical number of missed school days (Z = −3.36, p = 0.001). Similarly, there were significantly less missed work-days following surgery than before for the entire sample (Z = −2.86, p = 0.004).ConclusionMedian arcuate ligament syndrome imposes a large economic burden on patients and the healthcare system. The current findings, although reflective of a homogenous population, are adding to a growing body of literature suggesting that healthcare disparities play a role in the low rates of diagnosis and treatment of MALS.

Median arcuate ligament syndrome (MALS) is known to promote arterial collateral circulation development from mesenteric vessel compression and can lead to the development of visceral aneurysms. These aneurysms are often diagnosed at the time of rupture and pose a significant morality risk without appropriate intervention. A celiacomesenteric trunk is a rare anatomic variant in which the celiac artery and superior mesenteric artery share a common origin and has been postulated as a risk factor for developing MALS. In this report, we present a novel case of MALS in a patient with a celiacomesenteric trunk and a superior mesenteric artery aneurysm.

Objective: Unusual clinical course Background: Superior mesenteric artery (SMA) aneurysms account for about 5.5% of all visceral aneurysms, and are most commonly secondary to infectious causes or dissection. They tend to expand and rupture. Here, we present our successful diagnosis and treatment of a 41-year-old man with asymptomatic coeliac trunk stenosis, in whom the large aneurysm of the branch of the SMA developed in a very short time after conservative treatment of plastron appendicitis. Case Report: A 41-year-old man was diagnosed with plastron appendicitis during abdomen ultrasound (US) examination. Following 2 weeks of conservative treatment with intravenous antibiotic therapy, complete resolution of symptoms was obtained and confirmed in the computed tomography (CT) scan, and no other pathologies were diagnosed. Three weeks later, during the US examination, a 33-mm aneurysm of the branch of the SMA was diagnosed. The patient was admitted to the Vascular Surgery Department, where a critical stenosis of the coeliac trunk secondary to the compression by median arcuate ligament and a 33-mm true visceral aneurysm of one of the branches of the SMA were diagnosed. Successful treatment of the aneurysm was performed. Surgical decompression of the coeliac trunk and subsequent elective endovascular embolization of the SMA aneurysm with angioplasty of the coeliac trunk were performed. The postoperative period was uneventful and the patient was released from the hospital and remains asymptomatic. Conclusions: Visceral artery aneurysm can form very quickly. In some of the aneurysms, a combination of open surgical and endovascular methods should be performed. [ABSTRACT FROM AUTHOR]

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

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

Median arcuate ligament syndrome (MALS) is an uncommon disorder characterized by postprandial abdominal pain, vomiting, anorexia, and weight loss. The symptoms are due to the MAL compressing the celiac artery and irritating the celiac plexus/ganglion. We report the case of one patient who presented with chronic abdominal pain, bloating, vomiting, and weight loss. Computed tomography angiography led to the diagnosis of MALS. The patient was treated surgically by laparoscopic division of the MLA and excision of the celiac plexus. He was discharged on day two without complications. On one-month follow-up, he was symptom-free, eating well, and had gained weight. He is on regular follow-up. [ABSTRACT FROM AUTHOR]

Median arcuate ligament syndrome (MALS) is an uncommon disorder characterized by postprandial abdominal pain, vomiting, anorexia, and weight loss. The symptoms are due to the MAL compressing the celiac artery and irritating the celiac plexus/ganglion. We report the case of one patient who presented with chronic abdominal pain, bloating, vomiting, and weight loss. Computed tomography angiography led to the diagnosis of MALS. The patient was treated surgically by laparoscopic division of the MLA and excision of the celiac plexus. He was discharged on day two without complications. On one-month follow-up, he was symptom-free, eating well, and had gained weight. He is on regular follow-up.

Management of pancreaticoduodenal artery aneurysms (PDAAs) and gastroduodenal artery aneurysms (GDAAs) with concomitant celiac occlusion represents a challenging clinical scenario. Here, we describe a 62-year-old female with PDAA and GDAA complicated by celiac artery occlusion due to median arcuate ligament syndrome. We used a staged, minimally invasive approach consisting of: (1) a robotic median arcuate ligament release; (2) endovascular celiac artery stenting; and (3) visceral aneurysm coiling. The findings from this case report represent a novel treatment strategy for the management of PDAA/GDAA with celiac artery compression secondary to median arcuate ligament syndrome.

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

Pancreaticoduodenal artery aneurysms are rare visceral artery aneurysms that may be associated with stenosis of the coeliac trunk. We present a case of a 48-year-old woman with a symptomatic aneurysm of the inferior pancreaticoduodenal artery caused by occlusion of the coeliac trunk due to median arcuate ligament syndrome. The patient was treated with an aorto-hepatic bypass and excision of the aneurysm. We discuss the case and the complexities and controversies in the treatment of this disease. [ABSTRACT FROM AUTHOR]

Spontaneous retroperitoneal hematoma is rare and can cause duodenal obstruction. We report four cases of spontaneous retroperitoneal hematoma with duodenal obstruction, wherein endoscopic ultrasound was useful for diagnosis. The patients complained of vomiting with stable vital signs. Computed tomography, esophagogastroduodenoscopy, and endoscopic ultrasound findings were similar in all cases. Contrast-enhanced computed tomography revealed a low-density mass around the 2nd to 3rd part of the duodenum. Esophagogastroduodenoscopy showed an edematous, reddish, but non-neoplastic duodenal mucosa with stenosis of the lumen. Endoscopic ultrasound revealed a low-echoic mass around the duodenum and high-echoic floating matter suggesting debris and anechoic areas that indicated a liquid component. These findings suggested hematomas or abscesses. Although pseudoaneurysm of the pancreaticoduodenal artery was suspected in Case 3, we chose conservative treatment because the aneurysm was small. In Case 4, median arcuate ligament syndrome was suspected on angiography. No aneurysms or arteriovenous malformations were found; thus, endovascular embolization was not performed. The patients were treated conservatively and discharged within 3–5 weeks. English literature queries on spontaneous retroperitoneal hematoma with duodenal obstruction in MEDLINE revealed 21 cases in 18 studies. The clinical features of these patients and the present four cases have been discussed. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Median arcuate ligament syndrome (MALS) is a rare and debilitating condition that remains difficult to diagnose. Proper patient selection remains key to achieving favorable outcomes for those undergoing MALR. The robotic technique facilitates a minimally invasive MALR approach given the fine precision of the instrumentation and stability of visualization. Here we describe our management algorithm and clinical outcomes for a large series of robotic MALR patients. Methods: This retrospective cohort study analyzed adult patients who underwent robotic MALR performed by a single surgeon at a tertiary academic hospital from 2014 to 2021. The diagnosis of MALS was made using objective criteria from celiac artery duplex ultrasound with a peak systolic velocity of > 350 cm/s combined with a right upper quadrant abdominal ultrasound, esophagogastroduodenoscopy, and computer tomography or magnetic resonance angiography to exclude other diagnoses. Information on patient demographics, perioperative factors, and patient reported symptoms up to 1-year post-operatively were collected. Results: A total of 74 patients underwent robotic MALR during the study period. The mean age was 27.3 ± 7.9 years and the majority of patients were female (n = 60/74, 81.1%). The most common presenting symptom was post-prandial abdominal pain (n = 65/74, 87.7%). The mean operative time was 52.6 ± 18.1 min. There were no conversions to open surgery and minimal blood loss (mean = 13.9 ± 8.4 mL). At 3-months, 12% (n = 9/74) of patients had persistent abdominal pain and underwent additional imaging. 5 of these 9 patients had persistently elevated DUS expiratory PSV and were referred for angioplasty. 3 of these 5 referred patients had resolution of abdominal pain after angioplasty. At 1-year follow up, 90.3% (n = 56/62) continued to have no abdominal pain. Conclusions: Through this series, the largest set of minimally invasive (laparoscopic or robotic) MALR procedures published to date, we show that with strict adherence to a management algorithm, the robotic approach to MALR is safe and feasible, with good patient outcomes. [ABSTRACT FROM AUTHOR]

Median arcuate ligament syndrome is a clinical condition in which the median arcuate ligament causes compression and narrowing of the celiac artery. It has been reported that collateral pathways, which is developed by the decrease of blood flow from the celiac artery, facilitates the formation of aneurysms. Aneurysms around the pancreas in particular require aggressive therapeutic intervention, because a rupture can be fatal. We herein report two cases of pancreaticoduodenal aneurysms associated with median arcuate ligament syndrome treated by coil embolization and median arcuate ligament incision. Case 1 required a hybrid procedure in which median arcuate ligament incision and coil embolization were performed simultaneously. In Case 2, the median arcuate ligament incision was performed about 3 months after emergency endovascular hemostasis for hemorrhagic duodenal ulcer. In both cases, there were no major postoperative complications and no recurrence of aneurysm. Median arcuate ligament incision may be effective to prevent organ ischemia and aneurysm recurrence after coil embolization of intra-abdominal aneurysms associated with median arcuate ligament syndrome.

Background/Aims Median arcuate ligament syndrome (MALS) is known as chronic recurrent abdominal pain related to compression of the celiac artery by the median arcuate ligament. We aim to seek the specific mechanism of the pain by evaluating symptoms and radiological characteristics on abdominal CT scans. Methods We analyzed 35 patients who visited the emergency room for recurrent abdominal pain after cholecystectomy. We classified the characteristics of patients as 4 clinical components and 2 radiological components. We defined the sum of weighted clinical scores and weighted radiological scores as nutcracker ganglion abdominal pain syndrome (NCGAPS) scores. We categorized the patients into 3 groups classified by the degree of NCGAPS scores. The 3 patients with top-3 NCGAPS scores were recommended for CT angiography. Results When the suspicion was graded by NCGAPS scores, post stenotic dilatation was significantly different among all groups (P < 0.001). The clinical components of pain varied positional or respirational change and continuous pain were significantly different among all the groups (P < 0.01). NCGAPS scores can remarkably differentiate highly suspicious patients in comparison to simply combined scores. Only 1 patient in the highly suspicious group by NCGAPS scores took the CT angiography and was confirmed with NCGAPS. Conclusions We suggest renaming MALS as NCGAPS, nutcracker celiac ganglion abdominal pain syndrome, to better explain the mechanism of the recurrent abdominal pain. Further studies on the diagnostic cutoff of clinical and radiological scores of NCGAPS are needed not to miss the diagnosis of NCGAPS. [ABSTRACT FROM AUTHOR]

Introduction: Compression syndromes of the celiac artery (CAS) or superior mesenteric artery (SMAS) are rare conditions that are difficult to diagnose; optimal treatment remains complex, and symptoms often persist after surgery. We aim to review the literature on surgical treatment and postoperative outcome in CAS and SMAS syndrome. Methods: A systematic literature review of medical literature databases on the surgical treatment of CAS and SMAS syndrome was performed from 2000 to 2022. Articles were included according to PROSPERO guidelines. The primary endpoint was the failure-to-treat rate, defined as persistence of symptoms at first follow-up. Results: Twenty-three studies on CAS (n = 548) and 11 on SMAS (n = 168) undergoing surgery were included. Failure-to-treat rate was 28% for CAS and 21% for SMAS. Intraoperative blood loss was 95 ml (0–217) and 31 ml (21–50), respectively, and conversion rate was 4% in CAS patients and 0% for SMAS. Major postoperative morbidity was 2% for each group, and mortality was described in 0% of CAS and 0.4% of SMAS patients. Median length of stay was 3 days (1–12) for CAS and 5 days (1–10) for SMAS patients. Consequently, 47% of CAS and 5% of SMAS patients underwent subsequent interventions for persisting symptoms. Conclusion: Failure of surgical treatment was observed in up to every forth patient with a high rate of subsequent interventions. A thorough preoperative work-up with a careful patient selection is of paramount importance. Nevertheless, the surgical procedure was associated with a beneficial risk profile and can be performed minimally invasive. [ABSTRACT FROM AUTHOR]

Median Arcuate Ligament Syndrome (MALS) also known as Celiac Axis Compression Syndrome (CACS) or Dunbar syndrome which is a very rare condition with an incidence of about 2 cases per 100,000 patients caused by compression of the celiac trunk by the median arcuate ligament . The etiology of Dunbar's syndrome is not well known as it is rare and is difficult to diagnose and treat. An abdominal CECT in a 22-year-old woman with a 4 months history of postprandial abdominal pain and vomiting showed thickening of median arcuate ligament measuring 6mm causing narrowing of the proximal celiac trunk forming a hooked or a J-shaped appearance suggestive of median arcuate ligament syndrome or celiac artery compression syndrome. After thoroughly ruling out the other causes of the above mentioned symptoms, Laparotomy and release of the median arcuate ligament was done, which resulted in relief of the patient's symptoms.The diagnosis of median arcuate ligament syndrome should be considered in patients with postprandial abdominal pain that does not have a clearly established etiology and surgical decompression/release of median arcuate ligament is the mainstay of management. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]