Median arcuate ligament syndrome (MALS) is a rare gastroenterological illness that arises from the compression of the celiac trunk by the fibrous arch known as the median arcuate ligament, which connects the muscular tendon of the diaphragm to the vertebral column. It is hypothesized that this syndrome arises due to the inadequate caudal migration of the celiac trunk during embryogenesis, although the exact pathophysiology behind this disease process remains unclear. While MALS is classically associated with a triad of post-prandial pain, weight loss, and epigastric bruit, the triad is often incomplete due to variations in vascular structures with collateral circulation from adjacent vessels. When symptoms are present, they can be vague and often characterized as unexplained nausea, vomiting, diarrhea, or flatulence. Frequently, MALS is identified incidentally upon imaging of the abdomen in response to these nonspecific complaints. We present the case of a patient suffering from MALS in which a rare anatomic variant of the celiac trunk was identified.

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).

Abdominopelvic vascular compression syndromes (VCS) refer to conditions where blood vessels in the abdomen or pelvis are compressed by nearby structures, leading to various symptoms and complications. These conditions include superior mesenteric artery syndrome (SMAS), nutcracker syndrome (NCS), May-Thurner syndrome (MTS), and median arcuate ligament syndrome (MALS). Each syndrome is characterized by specific compressions of blood vessels, resulting in symptoms such as pain, nausea, vomiting, weight loss, leg swelling, and other related issues. Ehlers-Danlos syndrome (EDS), characterized by hyperelasticity, altered collagen, and mobility of the viscera, has been associated with VCS, although the exact prevalence is unknown. We report a case of a patient with EDS who presented with multiple VCS, including NCS, MTS, SMAS, and MALS.

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.
METHODS: 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.
CONCLUSIONS: 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.

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.

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.

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.

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.

BACKGROUND: 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.
METHODS: 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.
CONCLUSIONS: 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.
CONCLUSIONS: 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.

OBJECTIVE: 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.
CONCLUSIONS: Decompression surgery for median arcuate ligament syndrome could improve upper gastrointestinal symptoms regardless of the post-operative per cent diameter stenosis.