Median Arcuate Ligament Syndrome Research Articles

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

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. 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. Ten patients were included in the study. Laparotomy and laparoscopic surgeries were performed in seven and three patients, respectively. The median operation time was 147minutes. The median hospitalization period after the operation was sevendays. The degrees of celiac artery stenosis before and after surgery were compared and the percent 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 sixmonths'period (p < 0.001). Additionally, we evaluated the influence of post-operative percent 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 percent 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. Decompression surgery for median arcuate ligament syndrome could improve upper gastrointestinal symptoms regardless of the post-operative percent diameter stenosis.

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.

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?

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

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. 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. 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 25mm for the all group. Origin levels of Ct and SMA were evaluated in comparison to vertebral levels. Ct-SMA distance was relatively shorter (9.19mm) compared to the literature. SMAA findings were consistent with normal population values. 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.

Presentation and management of true aneurysms of the pancreaticoduodenal arcade with concomitant celiac artery stenosis using the endovascular approach

True aneurysms of the pancreaticoduodenal artery (PDA) arcade are rare but require intervention due to the high risk of rupture. Historically, these aneurysms have been managed with open surgical methods. In this study, we describe a contemporary series of aneurysms treated using a modern approach that includes endovascular and hybrid techniques. All the patients with aneurysms of the PDA arcade in an institutional database were identified between 2008 and 2022. Patients with history of pancreatic resection were excluded. Data on demographics, presenting symptoms, imaging findings, operative approach, and outcomes were collected and reviewed. There were nine patients diagnosed with a PDA aneurysm, and all nine underwent endovascular intervention. Most were men (n = 5; 55.6%) and White (n = 7; 77.8%) and had American Society of Anesthesiologists class II or III. The median aneurysm size was 21 mm (range, 6-42 mm), and five (55.5%) were symptomatic. Of the five symptomatic cases, two presented with rupture and were treated urgently. The median time to intervention for the nonurgent cases was 30 days. All but one patient had concomitant celiac artery stenosis and two of the eight cases (25%) were due to extrinsic compression from median arcuate ligament syndrome. Both patients underwent median arcuate ligament syndrome release before endovascular intervention. Another patient required open surgical bypass before endovascular repair from the supraceliac aorta to hepatic artery using a Dacron graft to maintain hepatic perfusion. Among the eight patients with celiac axis stenosis, five (62.5%) required celiac stent placement within the same operation. Coil embolization of the aneurysm was used for all except for two patients (n = 7 of 9; 77.8%), with one patient receiving embolic plugs and another receiving an 8 × 38-mm balloon-expandable covered stent for aneurysm exclusion. The median operating room time was 134 minutes. All repairs were technically successful without any intraoperative or postoperative complications. The mean follow-up was 30 months. There was no morbidity, mortality, or unplanned secondary reinterventions within 6 months after aneurysm repair. Stent patency and aneurysm size remained stable at 2 years of follow-up. True pancreaticoduodenal artery arcade aneurysms can be safely and effectively treated using endovascular and hybrid techniques. Because many of these aneurysms have concomitant celiac artery stenosis, the use of endovascular technology allows for simultaneous treatment of both the aneurysm and the stenosis with exceptional results.

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

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. 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. 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). 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.

A radio-anatomical study of median arcuate ligament syndrome: unveiling the morphology and morphometry of median arcuate ligament, celiac trunk, and superior mesenteric artery.

In the context of medical literature, a significant lacuna exists in understanding median arcuate ligament syndrome (MALS). While clinical aspects are well documented, literature lacks a robust exploration of the anatomical relationship between the celiac trunk and the median arcuate ligament (MAL). Morphometric parameters, including the vertebral level of MAL origin, MAL thickness, celiac trunk (CeT) origin level, diameter, and distances between CeT/Superior Mesenteric Artery (SMA) and the MAL center were observed on 250 CT angiograms. Cadavers (n = 11) were dissected to examine the same parameters and histo-morphological examination of MAL tissue was done. Radiological findings established average MAL thickness of 7.79 ± 2.58mm. The celiac trunk typically originated at T12. The average distance between the celiac trunk and the MAL center was 1.32 ± 2.04mm. The angle of the celiac trunk to the abdominal aorta was primarily obtuse. The average celiac trunk diameter was 5.53 ± 1.33mm. Histological examinations revealed a diverse MAL composition, indicating variable mechanical properties. This study provides comprehensive morphometric data on the anatomical relationship between the MAL and the celiac trunk. In contrast to available literature which says the average MAL thickness of > 4mm is an indicator of increased thickness, we observed much higher average thickness in the studied population. The findings contribute to a better understanding of normal anatomical variations which can serve as reference values for accurate radiological diagnosis of MALS. The histological examination revealed the heterogeneous nature of the MAL tissue composition, suggesting variable mechanical properties and functions in different regions.

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 Median Arcuate Ligament Release: Surgical Technique and Clinical Outcomes.

Median arcuate ligament syndrome (MALS) is characterized by a constellation of symptoms related to the compression of the celiac artery trunk. Laparoscopic release of the ligament has demonstrated its effectiveness in alleviating these symptoms while showing lower postoperative complication rates, reduced hospital stays, and improved clinical outcomes. This study describes a single institution's experience with this procedure and reports on the preoperative assessment, surgical technique, and clinical outcomes of patients with MALS. We performed a retrospective chart review of all patients who underwent a primary laparoscopic MAL release (MALR) at a single high-volume academic institution from June 2021 to July 2023. Patient demographics, preoperative assessment, postoperative complications, and resolution of preoperative symptoms data were collected. A total of 30 patients underwent laparoscopic MALR, with 76.7% being female and a mean age of 33.4±16.3 years. The most common presenting symptom was postprandial epigastric pain (100%), followed by abdominal pain and nausea (83.3%), among others. The preoperative evaluation for all patients included a duplex mesenteric doppler and CT angiogram during inspiration and expiration and 3D reconstruction. Successful laparoscopic decompression of the celiac artery was achieved in 96.6% of cases, with only one conversion to an open procedure. There was only one reported early (<30d postoperatively) complication with no subsequent late complications or mortality. None of the patients required reintervention or reoperation. Only 1 patient required postoperative celiac plexus/splanchnic block injection to alleviate pain. MALS can be effectively and safely managed using a laparoscopic approach when performed by an experienced minimally invasive surgeon. Further studies with longer follow-ups are needed to confirm the long-term effectiveness of this technique.

Covered versus bare-metal stenting of the mesenteric arteries in patients with chronic mesenteric ischaemia (CoBaGI): a multicentre, patient-blinded and investigator-blinded, randomised controlled trial

Mesenteric artery stenting with a bare-metal stent is the current treatment for atherosclerotic chronic mesenteric ischaemia. Long-term patency of bare-metal stents is unsatisfactory due to in-stent intimal hyperplasia. Use of covered stents might improve long-term patency. We aimed to compare the patency of covered stents and bare-metal stents in patients with chronic mesenteric ischaemia. We conducted a multicentre, patient-blinded and investigator-blinded, randomised controlled trial including patients with chronic mesenteric ischaemia undergoing mesenteric artery stenting. Six centres in the Netherlands participated in this study, including two national chronic mesenteric ischaemia expert centres. Patients aged 18 years or older were eligible for inclusion when an endovascular mesenteric artery revascularisation was scheduled and a consensus diagnosis of chronic mesenteric ischaemia was made by a multidisciplinary team of gastroenterologists, interventional radiologists, and vascular surgeons. Exclusion criteria were stenosis length of 25 mm or greater, stenosis caused by median arcuate ligament syndrome or vasculitis, contraindication for CT angiography, or previous target vessel revascularisation. Digital 1:1 block randomisation with block sizes of four or six and stratification by inclusion centre was used to allocate patients to undergo stenting with bare-metal stents or covered stents at the start of the procedure. Patients, physicians performing follow-up, investigators, and radiologists were masked to treatment allocation. Interventionalists performing the procedure were not masked. The primary study outcome was the primary patency of covered stents and bare-metal stents at 24 months of follow-up, evaluated in the modified intention-to-treat population, in which stents with missing data for the outcome were excluded. Loss of primary patency was defined as the performance of a re-intervention to preserve patency, or 75% or greater luminal surface area reduction of the target vessel. CT angiography was performed at 6 months, 12 months, and 24 months post intervention to assess patency. The study is registered with ClinicalTrials.gov (NCT02428582) and is complete. Between April 6, 2015, and March 11, 2019, 158 eligible patients underwent mesenteric artery stenting procedures, of whom 94 patients (with 128 stents) provided consent and were included in the study. 47 patients (62 stents) were assigned to the covered stents group (median age 69·0 years [IQR 63·0-76·5], 28 [60%] female) and 47 patients (66 stents) were assigned to the bare-metal stents group (median age 70·0 years [63·5-76·5], 33 [70%] female). At 24 months, the primary patency of covered stents (42 [81%] of 52 stents) was superior to that of bare-metal stents (26 [49%] of 53; odds ratio [OR] 4·4 [95% CI 1·8-10·5]; p<0·0001). A procedure-related adverse event occurred in 17 (36%) of 47 patients in the covered stents group versus nine (19%) of 47 in the bare-metal stent group (OR 2·4 [95% CI 0·9-6·3]; p=0·065). Most adverse events were related to the access site, including haematoma (five [11%] in the covered stents group vs six [13%] in the bare-metal stents group), pseudoaneurysm (five [11%] vs two [4%]), radial artery thrombosis (one [2%] vs none), and intravascular closure device (none vs one [2%]). Six (13%) patients in the covered stent group versus one (2%) in the bare-metal stent group had procedure-related adverse events not related to the access site, including stent luxation (three [6%] vs none), major bleeding (two (4%) vs none), mesenteric artery perforation (one [2%] vs one [2%]), mesenteric artery dissection (one [2%] vs one [2%]), and death (one [2%] vs none). The findings of this trial support the use of covered stents for mesenteric artery stenting in patients with chronic mesenteric ischaemia. Atrium Maquet Getinge Group.

Median Arcuate Ligament Syndrome in 17-year-old Male with Abdominal Pain: Case Report.

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. 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. 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.

Median arcuate ligament syndrome: a cost analysis to determine the economic burden of a rarely diagnosed disease.

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. 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. 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 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). 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.

Laparoscopic repair of iatrogenic celiac artery injury during median arcuate ligament release: A retrospection and tips at troubleshooting.

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.

Clinical Outcomes after Median Arcuate Ligament Release in Patients Responsive to Celiac Plexus Block

PurposeTo determine if symptom relief with celiac plexus block (CPB) is associated with favorable clinical outcomes after median arcuate ligament release (MALR) surgery. Materials and MethodsA retrospective review was performed from January 2000 to December 2021. Fifty-seven patients (42 women, 15 men; mean age, 43 years [range, 18–84 years]) with clinical and radiographic features suggestive of median arcuate ligament syndrome (MALS) underwent computed tomography (CT)–guided percutaneous CPB for suspected MALS. Clinical outcomes of CPB and MALR surgery were correlated. Adverse events were classified according to the Society of Interventional Radiology (SIR) guidelines. ResultsCT-guided percutaneous CPB was successfully performed in all 57 (100%) patients with suspected MALS. A cohort of 38 (67%) patients showed clinical improvement with CPB. A subset of 28 (74%) patients in this group subsequently underwent open MALR surgery; 27 (96%) responders to CPB showed favorable clinical outcomes with surgery. There was 1 (4%) CPB-related mild adverse event. There were no moderate, severe, or life-threatening adverse events. ConclusionsPatients who responded to CPB were selected to undergo surgery, and 96% of them improved after surgery.

Fibromuscular dysplasia and median arcuate ligament syndrome, infrequent and simultaneous

Fibromuscular dysplasia and median arcuate ligament syndrome, infrequent and simultaneous

Unusual Cause and Treatment of Uncontrolled Hypertension

Median arcuate ligament syndrome (MALS) is a rare benign condition typically affecting young females. It usually presents with chronic abdominal pain, nausea, vomiting and weight loss. They are usually diagnosed incidentally on MDCT angiography. We report a rare adult male of MALS which presented with a recurrent hypertensive urgency without any abdominal pain leading to an extensive workup and incidental diagnosis of MALS which was successfully treated by angioplasty with stenting of celiac trunk without any complication.

Diagnosis and treatment of Dunbar syndrome

The rare Dunbar syndrome or medial arcuate ligament syndrome (MALS) is defined as compression of the celiac trunk and/or ganglion by the medial arcuate ligament. It is often diagnosed after patients have suffered for along time and is characterized by intermittent food-related pain, nausea, and unexplained weight loss. After exclusion of other causes of the above symptoms by gastroscopy, colonoscopy, CT, or MRI, the gold standard for diagnosis is dynamic color-coded duplex sonography, which may be supplemented by CT or MR angiography. The treatment of choice is alaparoscopic division of the arcuate ligament at the celiac trunk, although percutaneous transluminal angioplasty (PTA) with stent implantation may be performed in cases of postoperative persistence of symptoms or recurrent stenosis. Since symptoms persist postoperatively in up to 50% of cases, strict indication and complete diagnosis in designated centers are of great importance for successful treatment.

A rare case of a combination of ectopic kidney and medial arcuate ligament syndrome: a case report

BackgroundEctopic 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 presentationWe 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.ConclusionWe 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.

Median arcuate ligament syndrome complicated with gallbladder stones: A case report

BACKGROUND Median arcuate ligament syndrome (MALS) is a rare disease caused by compression of the celiac trunk artery by the median arcuate ligament (MAL). It can cause symptoms of postprandial abdominal pain, weight loss, and nausea and vomiting. CASE SUMMARY A 55-year-old woman was admitted due to abdominal pain, nausea and vomiting. On admission, the patient presented with epigastric pain that worsened after eating, without signs of peritoneal irritation. Computed tomography angiography of the upper abdomen showed compression of the proximal segment of the abdominal trunk, local luminal stenosis with angular “fishhook” changes, which changed significantly during forceful inspiration and expiration; gallbladder stones; and multiple cysts in the liver. Abdominal duplex ultrasonography showed that peak systolic velocity was 352 cm/s. After diagnosis of MALS was confirmed, an arch ligament release procedure was performed. MALS has no specific symptoms and can be misdiagnosed as other abdominal diseases. Awareness of MALS should be improved to avoid misdiagnosis. The commonly used treatment option is MAL release and resection of the peripheral ganglion of the celiac trunk artery. CONCLUSION The diagnosis and treatment of MALS must be individualized, and MAL release is effective and provides immediate symptomatic relief.

First description of a histopathologic grading system and relationship to outcomes after robotic median arcuate ligament release with celiac ganglionectomy and lymphadenectomy

BackgroundTwo dominating theories regarding median arcuate ligament syndrome include vascular and neurogenic etiologies from celiac artery and ganglion compression, respectively. Celiac ganglionectomy is not routine during surgery, and specimens are rarely excised; therefore, the extent of nerve involvement and histopathology are unknown. Our study aims to characterize histopathologic findings in median arcuate ligament syndrome, establish a histopathologic grading system, and correlate with clinical outcomes. MethodsRobotic median arcuate ligament release, celiac ganglionectomy, and lymphadenectomy were performed with specimens excised and stained using hematoxylin & eosin, trichrome, and S100. Neurofibrosis, adiposity, and reactive changes were described, a grading scale was developed, and results were analyzed with clinical outcomes. ResultsFifty-four patients were evaluated, of whom 36 met inclusion criteria (81% female, 34.9 [25.9–47.5] years, body mass index 23.5 [19.6–28.1] kg/m2). Histopathologic evaluation revealed fibrosis (hematoxylin & eosin and trichrome median score 1.5 [0–2.5]), reactive lymphadenopathy (89%), intraparenchymal nerves (31%), and lipogranulomas (31%). Greater fibrosis was associated with a lack of preoperative celiac plexus block relief (100% vs. 30%, P = .044) and lower postoperative celiac artery velocities (198 vs 323 cm/s, P = .02). Intraparenchymal nerves were associated with greater decreases in pre to postoperative velocities (161 vs 84 cm/s, P = .037). Symptoms improved in 28 patients (78%). ConclusionWe developed the first histopathologic grading system and identified unique findings of intraparenchymal nerves and lipogranulomas. Histopathologic abnormalities were associated with objective improvement and symptomatic relief postoperatively. These findings support nerve compression and inflammation as predominant contributors to median arcuate ligament syndrome pain, celiac ganglia resection to treat symptoms, and continued histopathologic analysis to better elucidate median arcuate ligament syndrome etiology.