Median Arcuate Ligament Syndrome (MALS) Research
MALS is considered an "ultra-rare" condition; therefore, there is a section on case studies as the majority of research in this area is via case studies. Please ensure to take the case study implications lightly as they often represent preliminary research. Learn more about how to read research HERE.
20 total studies as of March 2, 2022
For navigation, click on the topic you are interested in below:
Introduction
MALS: REVIEW, SYMPTOMS, AND VARIOUS TREATMENTS
Goodall, R., Langridge, B., Onida, S., Ellis, M., Lane, T., & Davies, A. H. (2020). Median arcuate ligament syndrome. Journal of Vascular Surgery, 71(6), 2170–2176. https://doi.org/10.1016/j.jvs.2019.11.012
This literature review of MALS discusses diagnostic delay, mechanisms, symptoms, diagnosis, and treatment. The primary symptoms include ischemia of the foregut, exercise-induced GI symptoms, postprandial pain, vomiting, weight loss, and nausea. They suggest that future studies should compare the quality of life and long-term outcomes of laparoscopic, open surgery, and nonoperative management to develop some standardized management.
Summary by Kimberly Czotter
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MALS DIAGNOSIS, COMPLICATIONS, AND SURGERY
Tracci, M. C. (2015). Median Arcuate Ligament Compression of the Mesenteric Vasculature. Techniques in Vascular and Interventional Radiology, 18(1), 43–50. https://doi.org/10.1053/j.tvir.2014.12.007
This article touches on MALS and symptoms, diagnostic process, potential complications (aneurysm), indications for surgery, the surgical procedures, surgical complications, follow up, and potential outcomes of surgery.
Summary by Kimberly Czotter
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MALS: A NEW PERSPECTIVE
Smereczyński, A., Kołaczyk, K., & Kiedrowicz, R. (2021). New perspective on median arcuate ligament syndrome. Case reports. Journal of Ultrasonography, 21(86), e234–e236. https://doi.org/10.15557/jou.2021.0037
The authors discuss the prevalence of celiac trunk stenosis - suggesting that 10-24% have this, with only 7% of this group having symptoms related to this compression which is diagnosed as MALS. The presumed lack of symptoms in most patients is theoretically due to collateral circulation, often involved the head of the pancreas (22-69.6% of patients). Accordingly, the celiac blood flow normalizes when patients are standing. This article presents 5 case studies to explore this phenomenon further, and shows imaging for clarity.
Summary by Kimberly Czotter
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CELIAC PLEXUS BLOCK FOR DIAGNOSIS
Barbon, D. A., Hsu, R., Noga, J., Lazzara, B., Miller, T., & Stainken, B. F. (2021). Clinical Response to Celiac Plexus Block Confirms the Neurogenic Etiology of Median Arcuate Ligament Syndrome. Journal of Vascular and Interventional Radiology, 32(7), 1081–1087. https://doi.org/10.1016/j.jvir.2021.04.003
This retrospective study evaluated 96 patients (75 f, 21 m) who had 103 celiac plexus blocks that were guided by CT scan. There was only one moderate adverse event, with success for 86 patients (84%) experiencing a decrease in postprandial pain (mean reduced from 6.3 to 0.9), vomiting (15.5% to 4.9%), and nausea (37.9% to 11.6%). The authors suggest that neuropathy is a primary MALS etiology as some patients did not have celiac artery compression, but yet, benefited from the celiac plexus block.
Summary by Kimberly Czotter
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MALS: DIAGNOSTIC DIFFICULTIES AND SURGICAL COMPLICATIONS
Rodriguez, J. H. (2021). Median arcuate ligament syndrome: A clinical dilemma. Cleveland Clinic Journal of Medicine, 88(3), 143–144. https://doi.org/10.3949/ccjm.88a.21001
Rodriguez (2021) discusses MALS and the difficulties with diagnosis and surgical complications. They suggest proceeding with a celiac plexus block before surgery as it may help predict the success of surgery.
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Life Quality
MALS QUALITY OF LIFE: WITH AND WITHOUT SURGERY
Ho, K. K. F., Walker, P., Smithers, B. M., Foster, W., Nathanson, L., O’Rourke, N., Shaw, I., & McGahan, T. (2017). Outcome predictors in median arcuate ligament syndrome. Journal of Vascular Surgery, 65(6), 1745–1752. https://doi.org/10.1016/j.jvs.2016.11.040
This retrospective review included patients with radiologically confirmed MALS were included (n = 67) of which 43 underwent surgery and 24 did not. Within 2 years of follow-up, 37% of patients were asymptomatic after surgery and 56% partially improved, where as 7% had no symptom changes. In the group that did not have surgery, only 1 person became asymptomatic, whereas 4 worsened, 7 partially improved, and 12 had no change. Their results suggested that patients with post-exertional pain were more likely to have positive surgical outcome compared to patients that vomited or had unproved pain.
Summary by Kimberly Czotter
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Potential Misdiagnoses
REVERSIBLE GASTROPARESIS
Trinidad-Hernandez, M., Keith, P., Habib, I., & White, J. V. (2006). Reversible Gastroparesis: Functional Documentation of Celiac Axis Compression Syndrome and Postoperative Improvement. The American Surgeon, 72(4), 339–344. https://doi.org/10.1177/000313480607200413
*Celiac Axis Compression Syndrome is another term for MALS*. This article details the difficulty in differentiating two similarly presenting conditions gastroparesis and MALS, while detailing 3 case studies: diagnosis (or lack of), surgery, and recovery. The authors then discuss different controversies surrounding the diagnostic criteria and treatment of MALS patients.
Summary by Kimberly Czotter
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Treatment Options for MALS (Surgery)
MALS DIAGNOSIS, COMPLICATIONS, AND SURGERY
Tracci, M. C. (2015). Median Arcuate Ligament Compression of the Mesenteric Vasculature. Techniques in Vascular and Interventional Radiology, 18(1), 43–50. https://doi.org/10.1053/j.tvir.2014.12.007
This article touches on MALS and symptoms, diagnostic process, potential complications (aneurysm), indications for surgery, the surgical procedures, surgical complications, follow up, and potential outcomes of surgery.
Summary by Kimberly Czotter
"
LAPAROSCOPIC SURGERY
Sun, Z., Zhang, D., Xu, G., & Zhang, N. (2019). Laparoscopic treatment of median arcuate ligament syndrome. Intractable & Rare Diseases Research, 8(2), 108–112. https://doi.org/10.5582/irdr.2019.01031
This article discusses the diagnostic process of MALS and the treatment through laparoscopic surgery, suggesting a "cure" rate of 80%. It also details the median arcuate ligament and its anatomy.
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MALS: DIAGNOSTIC DIFFICULTIES AND SURGICAL COMPLICATIONS
Rodriguez, J. H. (2021). Median arcuate ligament syndrome: A clinical dilemma. Cleveland Clinic Journal of Medicine, 88(3), 143–144. https://doi.org/10.3949/ccjm.88a.21001
Rodriguez (2021) discusses MALS and the difficulties with diagnosis and surgical complications. They suggest proceeding with a celiac plexus block before surgery as it may help predict the success of surgery.
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LAPAROSCOPIC SURGERY
Rege, S. A., Singh, A., & Dalvi, A. N. (2020). Chronic Non-specific Upper Abdominal Pain of Median Arcuate Ligament Syndrome: Laparoscopic Treatment. Indian Journal of Surgery, 83(1), 237–243. https://doi.org/10.1007/s12262-020-02355-z
The authors discuss their experience with laparoscopic surgery for 17 MALS patients (14 f, 3 m) that had abdominal pain for at least 10 months. They detail the preoperative workup, surgery, and results. Each patient had success with surgery, although, one required open surgery mid laparoscopic surgery and one had occasional pain after surgery with a follow-up between 78-113.5 months.
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LAPAROSCOPIC SURGERY REVIEW
San Norberto, E. M., Romero, A., Fidalgo-Domingos, L. A., García-Saiz, I., Taylor, J., & Vaquero, C. (2020). Laparoscopic treatment of median arcuate ligament syndrome: a systematic review. International Angiology, 38(6). https://doi.org/10.23736/s0392-9590.19.04161-0
As the majority of studies regarding MALS are case studies, the authors completed a review to gain a clear picture of laparoscopic surgery as a treatment intervention. They included 504 cases, finding that the key advantages of laparoscopic surgery compared to open surgery are as follows: shorter operative time (mean = 136 minutes), low rate converting to open surgery (4.2%), and reduced hospital stay (mean = 3.8 days). They suggest that laparoscopic surgery is preferred due to the aforementioned reasons, lack of morbidity, safety, and positive results.
Summary by Kimberly Czotter
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SURGERY AND POST-OPERATIVE IMPROVEMENTS
Trinidad-Hernandez, M., Keith, P., Habib, I., & White, J. V. (2006). Reversible Gastroparesis: Functional Documentation of Celiac Axis Compression Syndrome and Postoperative Improvement. The American Surgeon, 72(4), 339–344. https://doi.org/10.1177/000313480607200413
*Celiac Axis Compression Syndrome is another term for MALS*. This article details the difficulty in differentiating two similarly presenting conditions gastroparesis and MALS, while detailing 3 case studies: diagnosis (or lack of), surgery, and recovery. The authors then discuss different controversies surrounding the diagnostic criteria and treatment of MALS patients.
Summary by Kimberly Czotter
"
MALS: REVERSIBLE GASTROPARESIS AND POST-OPERATIVE OUTCOMES
Trinidad-Hernandez, M., Keith, P., Habib, I., & White, J. V. (2006). Reversible Gastroparesis: Functional Documentation of Celiac Axis Compression Syndrome and Postoperative Improvement. The American Surgeon, 72(4), 339–344. https://doi.org/10.1177/000313480607200413
*Celiac Axis Compression Syndrome is another term for MALS*. This article details the difficulty in differentiating two similarly presenting conditions gastroparesis and MALS, while detailing 3 case studies: diagnosis (or lack of), surgery, and recovery. The authors then discuss different controversies surrounding the diagnostic criteria and treatment of MALS patients.
Summary by Kimberly Czotter
"
CELIAC PLEXUS BLOCK AND NEUROGENIC MALS
Barbon, D. A., Hsu, R., Noga, J., Lazzara, B., Miller, T., & Stainken, B. F. (2021). Clinical Response to Celiac Plexus Block Confirms the Neurogenic Etiology of Median Arcuate Ligament Syndrome. Journal of Vascular and Interventional Radiology, 32(7), 1081–1087. https://doi.org/10.1016/j.jvir.2021.04.003
This retrospective study evaluated 96 patients (75 f, 21 m) who had 103 celiac plexus blocks that were guided by CT scan. There was only one moderate adverse event, with success for 86 patients (84%) experiencing a decrease in postprandial pain (mean reduced from 6.3 to 0.9), vomiting (15.5% to 4.9%), and nausea (37.9% to 11.6%). The authors suggest that neuropathy is a primary MALS etiology as some patients did not have celiac artery compression, but yet, benefited from the celiac plexus block.
Summary by Kimberly Czotter
"
ROBOTIC SURGERY FOR MALS
Fernstrum, C., Pryor, M., Wright, G. P., & Wolf, A. M. (2020). Robotic Surgery for Median Arcuate Ligament Syndrome. JSLS : Journal of the Society of Laparoscopic & Robotic Surgeons, 24(2), e2020.00014. https://doi.org/10.4293/jsls.2020.00014
This study discusses the safety and efficacy of robotic MAL release in 27 patients (18f, 9m) in regards to: length of stay, narcotic use, operation duration, pain improvement, and complications. In this patient cohort prior to surgery, 93% had postprandial abdominal pain and 70% had celiac stenosis. The operations lasted approximately 95 minutes (range 53-358), with 81% of patients discharged the day of surgery and after 30 days 17 patients had full symptoms resolution, with 6 experiencing symptom recurrence after this. Out of 27 surgeries, only two converted to open surgery with one major complication.
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MALS SURGERY AND FOLLOW UP
Grotemeyer, D., Duran, M., Iskandar, F., Blondin, D., Nguyen, K., & Sandmann, W. (2009). Median arcuate ligament syndrome: vascular surgical therapy and follow-up of 18 patients. Langenbeck’s Archives of Surgery, 394(6), 1085–1092. https://doi.org/10.1007/s00423-009-0509-5
This retrospective study evaluates 18 MALS patients (15 f, 3 m) and their surgical management (all open surgery) and clinical history. They found that 11 patients required further surgery than decompression, suggesting a benefit to open surgery compared to laparoscopic. In the 15 patients that received follow up, 11 remained symptom free with 6 of them receiving ONLY decompression.
Summary by Kimberly Czotter
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Celiac Plexus Block for Neurogenic MALS
CELIAC PLEXUS BLOCK AND NEUROGENIC MALS
Barbon, D. A., Hsu, R., Noga, J., Lazzara, B., Miller, T., & Stainken, B. F. (2021). Clinical Response to Celiac Plexus Block Confirms the Neurogenic Etiology of Median Arcuate Ligament Syndrome. Journal of Vascular and Interventional Radiology, 32(7), 1081–1087. https://doi.org/10.1016/j.jvir.2021.04.003
This retrospective study evaluated 96 patients (75 f, 21 m) who had 103 celiac plexus blocks that were guided by CT scan. There was only one moderate adverse event, with success for 86 patients (84%) experiencing a decrease in postprandial pain (mean reduced from 6.3 to 0.9), vomiting (15.5% to 4.9%), and nausea (37.9% to 11.6%). The authors suggest that neuropathy is a primary MALS etiology as some patients did not have celiac artery compression, but yet, benefited from the celiac plexus block.
Summary by Kimberly Czotter
"
Case Studies
MALS MIMICKING CROHN'S DISEASE
Becker, E., Mohammed, T., & Wysocki, J. (2021). Often Overlooked Diagnosis: Median Arcuate Ligament Syndrome as a Mimicker of Crohn’s Disease. ACG Case Reports Journal, 8(10), e00675. https://doi.org/10.14309/crj.0000000000000675
*THIS IS A CASE STUDY, not necessarily representative.* This case study of a 35-year-old man discusses how his MALS symptoms were incredibly similar to crohn's disease: weight loss, vomiting, diarrhea, abdominal pain, and more. MALS is incredibly rare and occurs primarily in women (4:1 ratio) that this condition is often a diagnosis of exclusion - leading to significant diagnostic delay. The authors discuss diagnostic criteria: angiography (change in celiac artery shape with breathing), mesenteric duplex ultrasound (velocity > 200 cm/s in celiac artery, end diastolic velocity > 55 cm/s), and gastric exercise tonometry. They suggest that if patients are not improving following treatment for another GI condition to follow up with a MALS workup due to such unspecific symptoms.
Summary by Kimberly Czotter
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MALS MIMICKING FUNCTIONAL ABDOMINAL PAIN
Scharf, M., Thomas, K. A., Sundaram, N., Ravi, S. J. K., & Aman, M. (2021). Median Arcuate Ligament Syndrome Masquerading as Functional Abdominal Pain Syndrome. Cureus. https://doi.org/10.7759/cureus.20573
*THIS IS A CASE STUDY, not necessarily representative.* This case study of a 68-year-old women details an extremely delayed diagnosis due to a misdiagnosis with "functional abdominal pain syndrome," chronic pain, nause, vomiting, depression, and reflux esophagitis. Due to MALS being rare, she suffered for over 20 years before undergoing diagnosis and surgery to relieve her symptoms. The authors discuss the importance of continuing to search for an underlying pathologic diagnosis when patients have continuous GI symptoms.
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