Treatment Of Median Arcuate Ligament Syndrome Research Articles

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.

Prognostic factors for the long term outcome after surgical celiac artery decompression in MALS

BackgroundThe 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.ResultsBy 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.ConclusionsOverall, 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.

Minimally invasive approach & key aspects for the treatment of median arcuate ligament syndrome: case report

Background: Median arcuate ligament syndrome (MALS) is a rare condition resulting from the external compression of the celiac artery by an abnormally downward located media arcuate ligament (MAL). The aim of this video is to illustrate the key steps for minimally invasive approach for the surgical treatment of MALS.

Gabapentin as a Symptomatic Modifier in Median Arcuate Ligament Syndrome: A Case Report and Assessment of Treatment Modalities

Median arcuate ligament syndrome (MALS) is a rare constellation of neurogenic gastrointestinal (GI) symptoms resulting from compression of the celiac trunk and celiac plexus by the median arcuate ligament. MALS is characterized by nonspecific symptoms including nausea, vomiting, diarrhea, bloating, unintentional weight loss due to food aversion, and postprandial epigastric abdominal pain. We present a case of atypical, chronic MALS that confounded clinicians for over a decade and led to various misdiagnoses, including early-onset Parkinson’s disease. Of the constellation of symptoms that MALS may present with, postprandial epigastric pain is a classic symptom and increases the index of suspicion for the diagnosis; however, the absence of the classic symptom of postprandial epigastric pain and the predomination of nonspecific GI symptoms and syncope in our patient further clouded clinicians’ ability to diagnose MALS. Upon further investigation, we elucidated a link between gabapentin, which our patient was chronically prescribed, and its efficacy in decreasing neurogenic hypersensitivity in the GI tract. Our case and the implications of gabapentin use to decrease neurogenic pain from MALS represents a novel addition to the literature on MALS treatment modalities and elucidates new avenues for continued research in the use of gabapentin as a symptom-modifying agent in the nonoperative and preoperative treatment of MALS.

Latest Progresses in Surgical Treatment of Median Arcuate Ligament Syndrome

Median arcuate ligament syndrome(MALS)is compression of the celiac trunk by the median arcuate ligament.Median arcuate ligament release is the corner stone for the surgical treatment of MALS.Open surgery,laparoscopic surgery,and robot-assisted surgery have been developed,among which laparoscopic surgery has been proposed as the preferred approach in view of its minimal trauma and short hospital stay.Auxiliary celiac plexus neurolysis could further alleviate the patient's discomfort.Moreover,vascular reconstitution is of vital importance in the case of persistent stenosis in the celiac artery despite of median arcuate ligament decompression.Vascular reconstruction has satisfactory long-term patency rate,while endovascular treatment is less invasive.This article aims to summarize the consensuses and advances and shed light on the surgical treatment of MALS.

Chronic Non-specific Upper Abdominal Pain of Median Arcuate Ligament Syndrome: Laparoscopic Treatment

Median arcuate ligament syndrome (MALS) is caused by extrinsic compression of the celiac artery. It is characterized clinically by postprandial abdominal pain, nausea, vomiting, and weight loss, mimicking chronic mesenteric ischemia. Patients often undergo exhaustive but unremarkable diagnostic workup. Diagnosis is based on a combination of Doppler or angiographic studies of the celiac artery and clinical presentation. We report our 11-year experience (2008–19) of laparoscopic treatment of MALS. The objectives of this study were to review our technical experience and describe the long-term surgical outcomes. Out of 17 patients treated for MALS, 14 were female and with a median age of 36 years. All of them presented with abdominal pain. The length of symptoms on presentation was 10 months. Duplex ultrasonography indicated celiac trunk stenosis in each case, with an elevated peak velocity flow in the celiac trunk of 431 (± 80) cm/s. All underwent successful laparoscopic treatment with only one conversion to open. The operating time was 117 min (70–122) and intraoperative blood loss was < 50 ml in all but one converted to open. Length of stay was 3 to 6 days, with no postoperative complications or mortality. Median follow-up was 109.5 months (78–113.5). At this point, all patients remained symptom-free with only one patient who complained of occasional pain, not requiring any analgesics. Laparoscopic management of MALS is a safe and effective approach to achieve long-term improvement of symptoms after surgery.

Treatment of median arcuate ligament syndrome: outcome of laparoscopic approach.

ABSTRACT Background:Median arcuate ligament syndrome(MALS) is a rare condition thatmay cause significant clinical manifestations, including abdominal pain and weight loss. Its diagnosis may be difficult and very often delayed. The laparoscopic approach became the standard treatment of MALS. Aim:To assess the outcome of laparoscopic treatment in patients with MALS. Method:The data of sixpatients with MALS who were subjected to laparoscopic sectioning of the median arcuate ligament were retrospectively reviewed.The following data were evaluated: age, gender, clinical and diagnostic tests findings, ASA score, operative findings and complications, postoperative complications and mortality, hospital stay duration, and hospital readmission.The diagnosis of MALS was established by CT angiography and/or MR angiography. Results:There were four (66.7%) women and two (33.3%) men aged from 32 to 60 years. The main symptoms were epigastric pain (100%) and weight loss (66.7%). The findings of high-grade stenosis of the proximal celiac axis and poststenotic dilation confirmed on angiography confirmed the diagnosis in all patients. Surgical procedure was uneventful in all patients. The only postoperative complication was urinary retention that occurred in a male. At three-month follow-up, all patients were asymptomatic. Conclusion:Laparoscopic treatment of MALS is safe and effective in relieving the clinical manifestations of patients.

Robotic Surgery for Median Arcuate Ligament Syndrome.

Background:Compression of the celiac artery by the median arcuate ligament results in median arcuate ligament syndrome (MALS). Using a consecutive cohort of patients with MALS, this study evaluated the efficacy and safety of robotic median arcuate ligament release (MALR).Methods:A retrospective chart review was performed on patients who underwent robotic MALR from August 2012 to April 2018. Patient workup included history and physical examination, mesenteric Doppler ultrasound, and CT (Computed Tomography) scan. Outcomes included pain improvement, length of stay, operation duration, narcotic use, and postoperative complications.Results:Twenty-seven patients met inclusion criteria. Two thirds of the cohort were female and the mean age was 49 ± 15.5 years. Postprandial abdominal pain was the most common preoperative symptom (25/27, 93%). CT (Computed Tomography) was performed in 24 (89%), and celiac stenosis > 70% was observed in all. Operative duration was 95 minutes on average (range, 53–358 minutes), and in 24/27 (89%) patients, estimated blood loss was < 100 mL. Eighty-one percent of patients were discharged the day of surgery (22/27). Two cases were converted to open, with only one major complication occurring. At 30 or more days postoperation, 17 patients (68%) had full, 1 (4%) partial, and 1 (4%) no symptom resolution, 6 (24%) had symptom recurrence after initially having resolution. Fifty-six percent achieved narcotic liberation 9/16 (56%).Conclusions:Robotic MALR is a safe option for treatment of MALS with high response rates, early hospital discharge, and opportunity for narcotic liberation.

Pediatric Median Arcuate Ligament Syndrome: Surgical Outcomes and Quality of Life

The existence, etiology, diagnosis, and treatment of median arcuate ligament syndrome (MALS) have long been subjects of debate. To our knowledge, there have not been any studies assessing the effectiveness of surgical treatment in improving physical and psychological quality of life in pediatric patients. This is an Institutional Review Board-approved prospective study including all patients undergoing surgical treatment of MALS between 2009 and 2012 at our institution. Demographic information, presenting symptoms, radiological imaging, procedure duration, hospital length of stay, and perioperative complications were gathered for analysis. Patients and their parents were asked to complete the Child Health Questionnaire, a physical and psychological health survey, both within 1 week prior to and at least 3 months following their surgery. Six patients underwent laparoscopic release for MALS. The majority of patients were female (n=5 [83.3%]), with an average age of 15.7±1.5 years. Presenting symptoms lasted on average 16.5±12.7 months prior to treatment. Average pre- and postsurgical ultrasound celiac artery peak velocities with inspiration were 332.0±34.1 cm/second and 224.3±31.2 cm/second, respectively, with a statistically significant decrease of 107.67 cm/second (P=.03). The average follow-up period from time of surgery to time of quality of life survey completion was 13±11.3 months, with a range of 3-29 months. A significant improvement from pre- to postsurgical scores was observed in the physical functioning (P=.03), mental health (P=.03), and self-esteem categories (P=.03) of the child assessment. Similarly, there was a significant postsurgical improvement in all categories pertaining to the parent's quality of life (P=.03). Improvement was also seen in the parents' perception of their child's physical functioning (P=.03), bodily pain/discomfort (P=.03), mental health (P=.03), and general health perceptions (P=.03). No intraoperative or postoperative complications occurred. Our preliminary results demonstrate that laparoscopic median arcuate ligament release for MALS in the pediatric population is safe and effective and improves overall quality of life for the patients and their parents. In carefully selected patients, laparoscopic release for MALS without additional celiac artery reconstruction normalizes blood flow in the celiac artery and improves physical and psychosocial quality of life for the child and his or her parents.

Laparoscopic versus robot-assisted surgery for median arcuate ligament syndrome

Median arcuate ligament syndrome (MALS) is an uncommon disorder characterized by postprandial abdominal pain, weight loss, and vomiting related to the compression of the celiac artery by the median arcuate ligament. This syndrome has been classically treated with an open surgical approach. More recently, laparoscopic and robotic approaches have been used. We present our outcomes with laparoscopic and robot-assisted treatment of MALS. We performed a retrospective review of all patients treated for MALS from March 2006 to August 2012 at a single institution. A total of 16 patients with MALS were treated: 12 patients via a laparoscopic approach and 4 patients via a robot-assisted approach. Patient characteristics and comorbidities were similar between groups. We experienced no intraoperative or perioperative conversions, complications, or deaths. The mean operative time for the laparoscopic approach was significantly shorter than for the robotic approach (101.7 vs. 145.8 min; P = 0.02). However, we found no significant difference in length of hospital stay (1.7 vs. 1.3 days, P = 0.23). The mean length of follow-up for laparoscopically treated patients was 22.2 months and for robotically treated patients it was 20 months. Eight patients (67 %) in the laparoscopic group and two patients (50 %) in the robotic group had full resolution of their abdominal pain. Three patients in the laparoscopic group and two patients in the robotic group ceased chronic narcotic use after surgery. Both laparoscopic and robotic approaches to MALS treatment can be performed with minimal morbidity and mortality. The laparoscopic approach was associated with a significantly shorter operative time. While innovative, the true advantages to robot-assisted MALS surgery are yet to be seen.

“Re: Management of Median Arcuate Ligament Syndrome: A New Paradigm”

Median arcuate ligament (MAL) syndrome is an anatomic and clinical entity characterized by extrinsic compression on the celiac axis, which leads to postprandial epigastric pain, vomiting, and weight loss. Although characterized a few decades ago, the existence of this syndrome is still challenged by several authors. We reviewed the management of MAL syndrome, with special emphasis on the minimally invasive approaches. We also report the first case of successful combination of minimally invasive surgery and endovascular therapy in the treatment of this syndrome. A PubMed search was carried out to identify articles in English from 1963 to 2008 using the keywords "median arcuate ligament syndrome" and "celiac artery compression syndrome." Additional articles were identified by a manual search of the references from the key articles. All clinical and experimental studies that contained material applicable to the topic were considered. Classic treatment of the condition is represented by open MAL release. However, permanent changes in the celiac artery wall lead to poor long-term outcomes, and associated complex vascular procedures have been employed. Laparoscopic treatment of MAL syndrome was reported in five cases. All patients had resolution of symptoms, but long-term follow-up is not available. Laparoscopic release of arcuate ligament syndrome with intraoperative duplex ultrasound may be used in patients with symptoms suggestive of the diagnosis. In patients with persistent celiac flow abnormalities noted on duplex ultrasound or postoperative imaging, celiac angioplasty and stenting are advocated. If this option is not available or does not relieve symptoms, vascular reconstruction should be employed.