Median Arcuate Ligament Syndrome Research Articles

Eight Years Experience in the Management of Median Arcuate Ligament Syndrome by Decompression, Celiac Ganglion Sympathectomy, and Selective Revascularization

We aim to review an 8-year experience of median arcuate ligament syndrome (MALS) with chronic gastrointestinal ischemia (CGI) and evaluate clinical outcomes of arcuate ligament decompression, celiac sympathectomy, and selective revascularization. Between December 2002 and March 2012, of 25 patients referred with symptoms of CGI, 11 patients (10 women and 1 man) had clinical signs of abdominal angina and radiological evidence of MALS. Mean age was 50 ± 20.4 years. Median symptom duration was 34 months. All patients had median arcuate decompression and celiac sympathectomy. In all, 8 did not require revascularization, 2 had retrograde celiac and/or superior mesenteric artery (SMA) stenting, and 1 had SMA bypass. There was no mortality. The 30-day morbidity was 9%. Mean follow-up was 60 months. Eight patients noted complete relief of abdominal pain, and 1 reported some improvement. The MALS is not solely a vascular compression syndrome. The neurological component requires careful celiac plexus sympathectomy in addition to arcuate ligament decompression.

The Median Arcuate Ligament Syndrome: A Mimicker of Mesenteric Vasculitis

The median arcuate ligament syndrome is an uncommon condition characterized by the triad of postprandial abdominal pain, unintentional weight loss, and an epigastric bruit. This condition is diagnostically challenging and patients often undergo extensive laboratory, radiographic, and invasive evaluations before it is identified. Physicians should consider this syndrome in the differential diagnoses of chronic abdominal pain and mesenteric vasculitis. Once diagnosed, treatment is generally surgical with known predictors of favorable and unfavorable outcomes. Surgical candidates should be selected carefully. We describe the cases of two young active duty patients diagnosed with median arcuate ligament syndrome after suffering from chronic abdominal pain. Both were referred to our rheumatology department to evaluate for mesenteric vasculitis. Each had a different therapeutic outcome.

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.

Dunbar Syndrome: An Old Cause of a New Clinical Picture in an Older Adult

In January 2012, a 73-year-old woman was admitted to the Geriatrics Unit for investigation of a 2-month history of postprandial, diffuse, mainly epigastric, abdominal pain with anorexia and worsening general conditions. She reported undefined weight loss and progressive decrease in hemoglobin levels (~2 g/dL over the last year). Medical history included diabetes mellitus, hypertension, paroxysmal atrial fibrillation, and depressed mood. On examination, her body mass index was 18.7 kg/m2, and her physical examination was within normal limits. Blood tests showed hyposideremic anemia (hemoglobin 9.3 g/dL, mean corpuscular volume 76.0 fL, mean cell hemoglobin 24.2 pg, serum iron 21 μg/dL), moderate renal failure (serum creatinine 1.43 mg/dL, blood urea nitrogen 56 mg/dL, glomerular filtration rate 36 mL/min according to the Modification of Diet in Renal Disease equation); other findings were unremarkable. Gastroscopy revealed hyperemic and edematous mucosa with multiple shallow erosions; histology revealed mild chronic inflammation, without evidence of Helicobacter pylori. Treatment with intravenous iron and pantoprazole 40 mg twice a day was started. Because abdominal symptoms persisted, abdominal echography was performed; ultrasound examination revealed dyshomogeneous echogenicity of the pancreatic parenchyma, compatible with lipomatous involution, in the absence of obvious expansive alterations, and a normal choledochus. Secretin-enhanced magnetic resonance cholangiopancreatography evidenced fatty degeneration of the pancreas with multiple, diffuse cysts of a maximum size of 22 mm, mainly located within the tail, with cystic dilation of the secondary branches. Pancreatic enzyme replacement therapy for chronic pancreatitis was ineffective. Duplex ultrasound revealed stenosis of the celiac trunk exceeding 70%, which raised suspicion of angina abdominis. Computed tomography angiography confirmed severe stenosis of the proximal portion of the celiac axis secondary to compression by the median arcuate ligament with poststenotic dilatation (11 mm; Figure 1). All other vessels, including the superior and inferior mesenteric arteries, were normal. Considering these findings and symptoms, the diagnosis of Dunbar syndrome (deep crossing of the median arcuate ligament with ensuing compression of the proximal celiac axis), also known as median arcuate ligament syndrome or celiac artery compression syndrome, was established, and the woman was referred for laparoscopic treatment. She refused surgical intervention and started a diet of six easy-to-digest meals per day, with only mild improvement of symptoms. Diagnosis of this case was difficult because individuals with Dunbar syndrome are typically aged 20 to 40.1 In this woman, comorbid conditions might be relevant to explain the clinical picture. It is likely that anemia smoothed the beneficial effect of collateralization between the celiac axis and the superior mesenteric artery or that age-related changes in the dorsal spine curvature modulated the compression of the celiac trunk by the arcuate ligament. Progressive stiffening of the arcuate ligament might also play a pathogenetic role; diabetes mellitus and age are both known to promote the nonenzymatic glycosylation of collagen, with ensuing stiffening of tissues rich in low-turn-over proteins, especially fibrous tissues.2 Laparoscopic resection of the arcuate ligament has been substituted for surgical repair, variably consisting of decompression and reconstruction of the celiac axis,2 as the criterion standard remedy for Dunbar syndrome.3 Experience with elderly adults is limited, with only seven cases aged 70 and older in the five most recent series out of 102 individuals.1, 4-7 Age of 60 and older has been recognized as a risk factor for failure of the classical operative procedure,2 but it is unknown whether this might apply also to laparoscopic treatment. In this woman, the diet of six meals per day relieved symptoms only partially. It cannot be excluded that gastritis and pancreatic involution contributed to the clinical picture, but even though the definitive proof, based on the effect of laparoscopic repair, is lacking, the computed tomography angiography findings and the ineffectiveness of other medical therapies are highly consistent with the primary pathogenetic role of celiac artery compression by the arcuate ligament. This report suggests that atherosclerotic and embolic diseases should not be considered the only cause of intestinal ischemia in elderly adults; even a condition typical of younger people should be part of the diagnostic examination. Conflict of Interest: The editor in chief has reviewed the conflict of interest checklist provided by the authors and has determined that the authors have no financial or any other kind of personal conflicts with this paper. Author Contributions: Laudisio A., Rivera C.: data analyses. Laudisio A., Antonelli Incalzi R.: drafting of the manuscript. Laudisio A., Rivera C., Sansoni I., Antonelli Incalzi R.: study design. All authors: reading and approval of the final manuscript. Sponsor's Role: None.

Median arcuate ligament syndrome presenting as hemorrhagic shock

The major symptoms of median arcuate ligament syndrome, celiac axis stenosis, or occlusion compressed by the median arcuate ligament include eating-associated abdominal pain and weight loss. Because celiac stenosis increases retrograde collateral blood flow from the superior mesenteric artery to the celiac artery via the pancreaticoduodenal arcade, a pancreaticoduodenal artery aneurysm could occur at a low incidence rate. Rupture of the pancreaticoduodenal artery aneurysm and hemorrhagic shock are rare. In this report, we present 3 cases of patients who had been well with no abdominal symptoms until the day of admission, when they experienced sudden-onset intra-abdominal hemorrhage and shock. These 3 patients were admitted to the emergency department, and contrast-enhanced computed tomography and radiographic selective catheter angiography revealed intra-abdominal hemorrhage, stenosis of the celiac arteries, and dilated pancreaticoduodenal arcade. Case 1 demonstrated severe hemorrhagic shock, whereas case 2 demonstrated moderate shock. We treated ruptured pancreaticoduodenal artery aneurysms with coil embolization. Case 3 demonstrated complete celiac occlusion and moderate hemorrhagic shock, and no aneurysm was detected.

Median Arcuate Ligament Syndrome: Evolution of Management at a Regional Institution

Median Arcuate Ligament Syndrome: Evolution of Management at a Regional Institution

A variant of the median arcuate ligament syndrome: are sagittal images enough for diagnosis?

Median arcuate ligament syndrome (MALS), also known as celiac artery compression syndrome, is a rare cause of abdominal discomfort, postprandial pain and weight loss. Doppler ultrasonography (USG), computed tomography (CT), magnetic resonance imaging (MRI), and selective catheter angiography can be used to identify this abnormality. Classically, the "fish hook" appearance is seen on sagittal images. In our case, mild stenosis (20%) was seen at the origin of the celiac artery without typical "fish hook" appearance. Higher degree stenosis (55%) was demonstrated on axial images. We are presenting a rare case of a MALS caused by prominent crura.

A cadaveric study of the anatomical variation of the origins of the celiac trunk and the superior mesenteric artery

Gray's Anatomy states, "the celiac trunk is the first anterior branch of the abdominal aorta and arises just below the aortic hiatus. The superior mesenteric artery originates from the aorta c1.0 cm below the celiac trunk." (Standring, 2008a, Gray's Anatomy. 40th Ed. London: Churchill Livingstone Elsevier, p. 1073-1074). During dissection classes with medical students we found this not to be the case. We have re-evaluated the anatomy of the origins of the celiac trunk (CT) and superior mesenteric artery (SMA) and the relationship of the CT to the median arcuate ligament (MAL) in 99 cadavers. We have found the external distance between the CT and SMA to range from 0 to 20 mm (mean 3.4 mm, SD 5.17 mm), with the two in direct apposition in 57.6% (n = 99) of cases: a higher figure than previously documented. However, the internal distance between the CT and SMA ranged from 10 to 30 mm (mean 18.9 mm, SD 4.09 mm). There was no distance measurable between the MAL and the CT in 88 cadavers (92.6%, n = 95) and, of these, 32 (33.7%) showed evidence of compression or kinking of the CT. We suspect that the MAL is responsible for the approximation of the CT to the SMA in these cadavers, and that the high incidence of kinking of the CT (33.7% of cases) may have implications with regard to its role in MAL syndrome.

Celiacomesenteric Trunk as a Cause of Median Arcuate Ligament Syndrome

A 28-year-old man presented with a constellation of symptoms with 6 months of duration, most prominent being unexplained weight loss of 35 pounds and postprandial abdominal discomfort. Multiple specialist consultations, laboratory and instrumental studies did not yield a unifying diagnosis. The patient had been treated with multiple medications without improvements. On examination, an epigastric bruit that increased with expiration and decreased with inspiration raised suspicion for median arcuate ligament syndrome (MALS). Ultrasonography of the mesenteric arteries revealed a rare anatomical variant of abdominal vessels known as celiacomesenteric trunk (the celiac artery and superior mesenteric artery sharing a common origin from the aorta). There was also a median arcuate ligament impression on the celiac artery and marked respiratory variations of Doppler velocities in support of the diagnosis of MALS. This is a case report of celiacomesenteric trunk clinically associated with MALS.

Surgical treatment of Dunbar syndrome

Dunbar syndrome or celiac artery compression syndrome is an infrequently described clinical condition with poorly defined diagnostic criteria and an obscure pathophysiology. It is usually associated with an extrinsic compression upon the celiac axis near its takeoff from the aorta by fibrous diaphragmatic bands or sympathetic neural fibers. The authors report the case of a 70-year-old male patient presenting with nausea, epigastric pain, and weight loss. An aortography showed a compression of the celiac trunk. A preliminary attempt at percutaneous transluminal angioplasty and stenting proved unsuccessful. The patient became asymptomatic and his clinical condition improved after surgical release of the celiac trunk by partial section of the arcuate ligament of the diaphragm and with resection of the neural, fibrotic, and lymphatic tissues surrounding the aortic and visceral vessels. The purpose of this report is to discuss the indications and the therapeutic options of this syndrome.

Evaluation of Asymptomatic Patients with Median Arcuate Ligament Syndrome (Mals) Using Color Duplex Ultrasound and Computed Tomographic (Ct) Angiography

Median arcuate ligament syndrome is considered as rare syndrome manifested by variety of symptoms. Aim of the Work: To assess the presence of asymptomatic patients showing the typical color duplex ultrasound and CT angiographic features of MAL syndrome and the impact of these findings on the pathogenesis and management of the syndrome. Material and Methods: 200 asymptomatic patients were examined with color duplex ultrasound and further CT angiography was performed to all patients with color duplex findings of arcuate ligament syndrome. Results: 8 patients showed typical color duplex findings and CT angiographic features of MALS. Conclusion: The diagnosis of MAL syndrome shouldn't depend only on the imaging criteria and should be strongly linked to the unexplained patient symptoms after meticulous exclusion of other etiologies. The management shouldn’t depend only on relieving the arterial stenosis but also on dissection of peri arterial nerve plexus.

Median Arcuate Ligament Syndrome in a patient with Crohn's disease

INTRODUCTIONThe Median Arcuate Ligament Syndrome is a rare condition characterized by postprandial abdominal pain, bowel function disorder and weight loss. We report the first case to our knowledge of Crohn's disease and Median Arcuate Ligament Syndrome. PRESENTATION OF CASEThe patient was a 33 year-old female with a previous diagnosis of Crohn's disease. Acute postprandial abdominal pain affected the patient every day; she was, therefore, referred to US-Doppler and magnetic resonance angiography of the abdominal vessels and received a diagnosis of Median Arcuate Ligament Syndrome. Consequently, the patient was surgically treated, releasing the vascular compression. After the operation, she reported a complete relief from postprandial pain which was one of her major concerns. Subocclusive symptoms occurred after six months due to the inflammatory reactivation of the terminal ileitis. DISCUSSIONThe diagnosis of Median Arcuate Ligament Syndrome is mainly based on the exclusion of other intestinal disorders but it should be always confirmed using noninvasive tests such as US-Doppler, angio-CT or magnetic resonance angiography. CONCLUSIONThis case demonstrates that the Median Arcuate Ligament Syndrome could be the major cause of symptoms, even in presence of other abdominal disorders.

Diagnosis of Median Arcuate Ligament Syndrome on Multidetector Computed Tomography

The median arcuate ligament syndrome (MALS), also known as celiac artery compression syndrome, is a condition which develops secondary to an ischemia in gastrointestinal organs resulting from the compression of proximal celiac truncus by the median arcuate ligament of the diaphragm. It is characterized by postprandial abdominal pain or weight loss. The MALS is conventionally diagnosed by angiography; however, currently the condition can also be diagnosed with multidetector computed tomography (MDCT). In this paper, we aimed to present three cases that were diagnosed with median arcuate ligament syndrome on MDCT. J Med Cases. 2013;4(9):616-619 doi: http://dx.doi.org/10.4021/jmc1384w

Open surgical decompression of celiac axis compression by division of the median arcuate ligament

Median arcuate ligament syndrome is a rare cause of abdominal pain which results from compression of the celiac artery (CA) or rarely, the superior mesenteric artery by a ligament formed by the right and left crura of the diaphragm. We report a case of open surgical decompression of the CA by division of the median arcuate ligament for a 37-year-old female patient who had suffered from chronic postprandial epigastric pain and severe weight loss. We described clinical features, characteristic angiographic findings and details of the surgical procedure for the patient with this rare vascular problem.

Laparoscopic Median Arcuate Ligament Release: Are We Improving Symptoms?

Median arcuate ligament syndrome is a rare disorder characterized by postprandial abdominal pain, weight loss, and celiac stenosis. We report our experience using a laparoscopic approach for this uncommon diagnosis. This is an IRB-approved, prospectively collected retrospective analysis of patients treated with laparoscopic median arcuate ligament release at our institution. Data collected included patient demographics, preoperative symptoms, operative approach, and postoperative outcomes. Ultimately, patients were contacted to complete a postoperative survey aimed at assessing resolution of symptoms and overall satisfaction. A total of 15 patients underwent laparoscopic median arcuate ligament release from March 2007 to January 2012. Mean age was 34 years (range 17 to 68 years) and 93% were female. Mean preoperative celiac velocity was 380 cm/s (range 210 to 600 cm/s). Fourteen patients had laparoscopic median arcuate ligament release and 1 patient had robotic-assisted laparoscopic release. Mean operative time was 179 minutes (range 79 to 473 minutes) and there was 1 conversion to laparotomy. Twelve of 15 patients had a postoperative celiac axis ultrasonography. Celiac occlusion occurred in 2 patients (present in 1 patient preoperatively). In the remaining 10 there was a statistically significant decrease in celiac velocity to 215 cm/s (range 135 to 306 cm/s; p = 0.005). Survey response rate was 86% at a mean follow-up of 15.4 months (range 2.8 to 32.6 months), and all but 1 patient reported having resolution of pain. Laparoscopic release of the median arcuate ligament is a safe, feasible, and effective means of managing median arcuate ligament syndrome. Postoperative symptomatic relief is seen in the vast majority of patients undergoing this procedure.

Multislice bilgisayarlı tomografi ile median arkuat ligament sendromu tanısı: Olgu sunumu

Median arcuate ligament syndrome diagnosed with multisliced computerized tomography: A case report Median arcuate ligament syndrome occurs when median arcuate ligament pressures the root of the celiac artery and generally it reveals itself with the post-prandial pain. Because of the pressure there will be a significant tightness especially while expirium. Mesenteric ischaemia and low blood flow to gastro-intestinal system occurs related to the tightness and because of these we see the characteristic clinical symptoms of the syndrome. We present the patient whom we diagnosed with multislicde computerized tomograpy.

Single injection, inspiratory/expiratory high-pitch dual-source CT angiography for median arcuate ligament syndrome: Novel technique for a classic diagnosis

This article discusses a novel technique for dynamic imaging of median arcuate ligament syndrome utilizing low dose CT technology and a single contrast injection.

The many faces of Hereditary Hemorrhagic Telangiectasia: median arcuate ligament syndrome, arteriovenous malformations, and cerebral aneurysms

The many faces of Hereditary Hemorrhagic Telangiectasia: median arcuate ligament syndrome, arteriovenous malformations, and cerebral aneurysms

Open and laparoscopic treatment of median arcuate ligament syndrome

Median arcuate ligament syndrome (MALS) is a syndrome associated with chronic abdominal pain and radiographic evidence of celiac artery compression. We compared the evidence for both open and laparoscopic treatment of patients with MALS. We reviewed the English-language literature between 1963 and 2012. Presenting symptoms, clinical improvement, operative details, and intraoperative and postoperative complications were noted. A total of 400 patients underwent surgical (open and laparoscopic) treatment for MALS. Three hundred thirty-nine patients reported immediate postoperative symptom relief (85%). Late recurrence of symptoms was reported in 19 patients in the open group (6.8%) and seven patients in the laparoscopic group (5.7%). Eleven out of 121 patients (9.1%) in the laparoscopic group required open conversion secondary to bleeding. The available evidence demonstrates that both laparoscopic and open ligament release, celiac ganglionectomy, and celiac artery revascularization may provide sustained symptom relief in the majority of patients diagnosed with MALS. The role of arterial revascularization following ligament release remains unclear. The rate of open conversion with the laparoscopic approach is high, but no perioperative deaths have been reported.

Robotic-assisted median arcuate ligament release

Median arcuate ligament syndrome results from external compression of the celiac axis by attachments of the diaphragmatic crura. It has been treated with open or laparoscopic surgical decompression of the celiac axis with neurolysis. We describe our initial experience treating three patients using a robotic-assisted technique with median arcuate ligament release and celiac neurolysis. Average operative time was 2.2 hours. No intraoperative complications occurred. At an average of 11 months postoperative (14, 11, and 8 months), two patients continue with resolution of preoperative symptoms. Our experience affirms that further study using the robotic approach appears warranted.