Median arcuate ligament syndrome: case presentation and video-illustrated laparoscopic management.
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ABSTRACT:
SUBMITTER: El Asmar A
PROVIDER: S-EPMC5134203 | biostudies-literature | 2016 Dec
REPOSITORIES: biostudies-literature
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Median arcuate ligament syndrome: case presentation and video-illustrated laparoscopic management.
El Asmar Antoine A El Rassi Ziad Z
Clinical case reports 20161111 12
Laparoscopic release of the median arcuate ligament, to relieve symptomatic compression of the celiac trunk, is the ideal approach when performed in a specialized center, by a surgeon with the adequate experience in advanced laparoscopic surgery. ...[more]
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Project description:OBJECTIVE:Optimal diagnosis and management of median arcuate ligament (MAL) syndrome (MALS) remains unclear in contemporary practice. The advent and evolution of laparoscopic and endovascular techniques has redirected management toward a less invasive therapeutic algorithm. This study examined our contemporary outcomes of patients treated for MALS. METHODS:All patients treated for MALS at Dartmouth-Hitchcock Medical Center from 2000 to 2013 were retrospectively reviewed. Demographics and comorbidities were recorded. Freedom from symptoms and freedom from reintervention were the primary end points. Return to work or school was assessed. Follow-up by clinic visits and telephone allowed quantitative comparisons among the patients. RESULTS:During the study interval, 21 patients (24% male), with a median age of 42 years, were treated for MALS. All patients complained of abdominal pain in the presence of a celiac stenosis, 16 (76%) also reported weight loss at the time of presentation, and 57% had a concomitant psychiatric history. Diagnostic imaging most commonly used included duplex ultrasound (81%), computed tomography angiography (66%), angiography (57%), and magnetic resonance angiography (5%). Fourteen patients (67%) underwent multiple diagnostic studies. All patients underwent initial laparoscopic MAL release. Seven patients (33%) underwent subsequent celiac stent placement in the setting of recurrent or unresolved symptoms with persistent celiac stenosis at a mean interval of 49 days. Two patients required surgical bypass after an endovascular intervention failed. The 6-month freedom from symptoms was 75% and freedom from reintervention was 64%. Eighteen patients (81%) reported early symptom improvement and weight gain, and 66% were able to return to work. CONCLUSIONS:A multidisciplinary treatment approach using initial laparoscopic release and subsequent stent placement and bypass surgery provides symptom improvement in most patients treated for MALS. The potential placebo effect, however, remains uncertain. A significant minority of patients will require reintervention, justifying longitudinal surveillance and prudent patient selection. Patients can anticipate functional recovery, weight gain, and return to work with treatment.
| S-EPMC5292272 | biostudies-literature
Project description:Objectives: Median arcuate ligament syndrome is a complex disorder potentially caused by variation in the position of the median arcuate ligament. Symptomology involves chronic abdominal pain, nausea, and malnourishment. Pain management modalities and short-term outcomes for patients undergoing operative surgery for median arcuate ligament syndrome have yet to be fully evaluated. Our hospital implemented a pain management consultation program in 2017 focused on perioperative pain management. The objective of this study is to assess if the introduction of a pain management consultation program concurrent with median arcuate ligament syndrome surgery impacts patient outcomes and post-operative pain management strategies in these patients. Methods: De-identified data was collected retrospectively from our hospital’s electronic medical records system, identifying median arcuate ligament syndrome patients and using International Classification of Diseases (ICD) and Current Procedural Terminology (CPT) codes from September 2017 to August 2021. Patients were grouped into the “consultation” cohort if they had scheduled and attended a pre-operative pain consultation. Pre-operative and discharge medications, pain scores, and demographics were collected to evaluate if the initiative impacted outcomes. Results: Median arcuate ligament syndrome patients who had a pre-operative pain management consultation had higher rates of pre-operative opioid (35.5%; p = 0.01) and non-opioid use (60.7%; p < 0.001). Patients without a pre-operative consultation that did not use opioids pre-operatively were more likely to be discharged on one or more opioids. Differences were also found for psychiatric medication at discharge (p < 0.001) with patients receiving pain consultation indicating higher percentages of use. Conclusion: Special consideration on prescribing pain medication should be part of discharge planning for median arcuate ligament syndrome patients. Addition of a pain management consultation can aid in these decisions.
| S-EPMC10226036 | biostudies-literature
Project description:Median arcuate ligament syndrome (MALS) is a rarely diagnosed condition resulting from compression of the celiac trunk (CT) by the median arcuate ligament (MAL) of the diaphragm. Ischemia due to reduced blood flow through the CT and/or neuropathic pain resulting from celiac ganglion compression may result in a range of gastrointestinal symptoms, including nausea, postprandial discomfort, and weight loss. However, the mechanism of compression and its anatomical correlates have been incompletely delineated. It has been hypothesized that CT angle of origination may be more acute in individuals with MALS. Here, frequency of anatomical variation in the MAL and CT were assessed in 35 cadaveric subjects (17M/18F), including the vertebral level of origin of CT and superior mesenteric artery (SMA), the distance between CT and MAL and SMA, the angles of origination of CT and SMA, the diameter at the CT base, and MAL/CT overlap. Females exhibited significantly higher rates of inferred MAL/CT overlap than males. Significant correlations were revealed between MAL/CT overlap and angles of origination of the CT and SMA. Vertebral level of origin of the CT in individuals with MAL/CT overlap was not significantly more superior than in those without. This study also revealed a significant relationship between MAL/CT overlap and angle of origination of the CT, which has clinical implications for understanding the anatomy associated with MALS.
| S-EPMC7168915 | biostudies-literature
Project description:Background/aimsMedian 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.MethodsWe 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.ResultsWhen 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.ConclusionsWe 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.
| S-EPMC10083118 | biostudies-literature
Project description:Background Chronic mesenteric ischemia (CMI) due to either atherosclerosis of the mesenteric arteries or median arcuate ligament syndrome (MALS) is an underdiagnosed entity. The etiology of MALS and its existence have been debated and questioned. We aimed to identify plasma biomarkers indicating mesenteric ischemia in patients with CMI and MALS. Methods Plasma α-glutathione S-transferase (α-GST), intestinal fatty acid-binding protein (I-FABP), citrulline, and ischemia modified albumin (IMA) were analyzed in fifty-eight patients with CMI (Group A, n=44) and MALS (Group B, n=14) before and after revascularization. The plasma levels of these potential biomarkers were compared with those of healthy individuals (Group C, n=16). Group comparison was performed with the Mann–Whitney U-test. Cross-tabulation and its derivatives were obtained. Receiver operating characteristic (ROC) curves and area under the curve (AUC) were calculated. Results Plasma levels of α-GST were significantly raised in the patients with CMI (7.8 ng/mL, p<0.001) and MALS (8.4 ng/mL, p<0.001), as compared with the control Group C (3.3 ng/mL). The threshold for normal median plasma α-GST levels of 4 ng/mL yielded a sensitivity of 93% and 86%, specificity of 86% and 88%, respectively, for the diagnosis of CMI due to atherosclerosis and MALS. AUC of ROC curves was 0.96 (p<0.0001) for CMI and 0.85 (p<0.002) for MALS. The patient groups did not differ from the healthy controls in any other biomarkers. Conclusion Plasma α-GST levels are elevated in CMI and MALS patients. Elevated plasma levels of α-GST suggest ischemia as the etiology of MALS.
| S-EPMC9315055 | biostudies-literature
Project description: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.
| S-EPMC10731603 | biostudies-literature
Project description: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.
| S-EPMC10824967 | biostudies-literature
Project description:Celiac artery compression syndrome (CACS) is a disease caused by celiac artery compression by the median arcuate ligament (MAL), resulting in intestinal ischemic symptoms. However, a clear method for the invasive treatment of CACS has not yet been established because of limited treatment indications. In particular, only a few reports of endovascular therapy (EVT) using stents as the initial invasive treatment are available. Here, we report a case where EVT was performed using a stent in the celiac artery, resulting in good outcomes. A 59-year-old male patient presented to our hospital with postprandial abdominal pain and was diagnosed with MAL-induced CACS since the abdominal contrast computed tomography examination showed stenosis of a celiac artery origin. He was aware of the abdominal pain symptoms; therefore, we decided to treat CACS with EVT as an invasive treatment. A bare metal stent was placed in the celiac artery, whose lumen was well dilated using intravascular ultrasound. Consequently, he no longer felt abdominal pain and had good stent patency after 15 months. Minimally invasive EVT may be considered the first-line treatment for CACS.Learning objectiveThe efficacy of endovascular therapy (EVT) using stents for the invasive treatment of celiac artery compression syndrome (CACS) resulting from the compression of the median arcuate ligament has not yet been established. Specifically, the efficacy of EVT using stents for CACS is unknown. We can safely perform EVT with stent placement using intravascular ultrasound for maintaining long-term patency. Therefore, minimally invasive EVT may be considered the first-line treatment for CACS.
| S-EPMC10477046 | biostudies-literature
Project description:Median arcuate ligament syndrome and superior mesenteric artery syndrome are well-known abdominal compression syndromes, the coexistence of which is rarely described in literature. In addition, due to the common pathogenesis, anterior nutcracker syndrome may occur simultaneously to superior mesenteric artery syndrome. To our knowledge, this is the first case reporting combination of these 3 syndromes detected with ultrasound, Computed Tomography and upper gastrointestinal fluoroscopic exam. A 69-year-old man came to our attention for rapid weight loss, postprandial epigastric pain and recurrent vomiting for at least 6 months. Doppler ultrasound showed both celiac artery and left renal vein stenosis with simultaneous left varicocele. Computed tomography showed a reduction of aortomesenteric space causing both left renal vein and duodenal stenosis, this latter confirmed by upper gastrointestinal fluoroscopic exam. The diagnosis of these three vascular compression syndromes (MALS, SMAS, and anterior NCS) has been formulated, based on clinical and imaging findings. We assumed that the postprandial crises caused by median arcuate ligament syndrome may induce a reduction of meals consumption and progressive weight loss which can be a cause of anterior nutcracker syndrome and superior mesenteric artery syndrome onset. Doppler ultrasound, in expert hands, allows to accurately diagnosing these syndromes which are often underestimated. Failure to recognize it and inadequate treatment could have serious consequences for patients' health.
| S-EPMC8026914 | biostudies-literature
Project description:Purpose:Physiological tests may aid in diagnosing median arcuate ligament syndrome (MALS). MALS is a symptomatic compression of the celiac artery causing symptoms similar to chronic mesenteric ischemia (CMI) of atherosclerotic etiology. Simultaneous use of visible light spectroscopy (VLS) and laser doppler flowmetry (LDF) during upper endoscopy may detect microcirculatory changes in these patients. Patients and Methods:In a single-center, prospective comparative cohort, 25 patients were evaluated for MALS. Patients with a consensus diagnosis of MALS (n=15) underwent a gastroscopy assisted, transmucosal microcirculatory assessment with LDF and VLS. Results were compared to individuals with normal intestinal circulation (n=38) evaluated with duplex ultrasonography, and to patients with chronic mesenteric ischemia (n=32). Treatment response was evaluated clinically at 1, 3, 6, and 12 months, and with ultrasound, VLS and LDF at three months. Health-related quality of life (QoL) was assessed with Euroqol (EQ-5D-5L), preoperatively, and 12 months postoperatively. Results:Preoperative mean transmucosal oxygen saturation was significantly lower in patients with MALS (SO2 76±6), as compared to healthy individuals (SO2 81±4), p=0.02. An overall significant improvement in SO2 after surgical decompression of the celiac artery was found (SO2 81±3.7, p=0.05). Eleven (92%) patients with clinical improvement after laparoscopic decompression had a definitive diagnosis of MALS. Median follow-up was 18 months (4-24 months). Four of the five dimensions investigated with EQ-5D-5L improved. Conclusion:VLS detected a significantly lower baseline transmucosal SO2 in patients with MALS as compared to control subjects with normal intestinal circulation. An improvement in SO2 after laparoscopic decompression was found, supporting a possible ischemic etiology in our patient population.
| S-EPMC7493022 | biostudies-literature