Background: The median arcuate ligament is a normal anatomical feature of the aortic hiatus, attached to the posteromedial aspect of the diaphragm. Median arcuate ligament syndrome (MALS), also known as celiac artery compression syndrome (CACS), is a presumed rare congenital defect thought to cause compression of the celiac trunk, in addition to possible impingement of the celiac plexus. Symptomatic patients, depending on age and stage of disease complain of post-prandial abdominal pain, post-exertional abdominal pain, nausea, vomiting, and weight loss. Due to rarity of the disease in conjunction with vague physical complaints and unclear etiology, a universal method for surgical screening has not been developed and predictors of surgical outcome are not widely understood. This systematic review addresses how providers can determine surgical candidacy based upon outcome predictors and symptomatology.
Methods: A search was conducted using Google Scholar, MEDLINE via PubMed, ScienceDirect, and CINAHL via Ebsco. Keywords included were median arcuate ligament syndrome, celiac artery compression syndrome, predict*, and outcomes. Studies were assessed for quality using GRADE criteria.
Results: This systematic review includes 3 studies that discuss predictive models for screening MALS surgical candidates. One study evaluated the surgical outcomes of MAL decompression in 42 patients using SF-36 to evaluate symptom improvement and resolution, and it was deduced that age and baseline celiac artery expiratory velocity served as predictors of positive surgical outcome. Another study evaluated 67 patients instead using SF-12 and the Visick score as symptom questionnaires. This study found that pre-operative post-exertional abdominal pain suggested a positive surgical outcome, and vomiting and unprovoked pain were associated with negative surgical outcomes. A prospective study was lastly analyzed. This study, which included 135 patients, determined that the development and grade at which collateral circulation was formed within the abdomen correlated with increasingly negative surgical outcomes.
Conclusion: Surgical decompression of the celiac artery is often successful, despite pre-operational predictors that might suggest incomplete symptom resolution. Predictors of positive surgical outcomes include post-exertional pain, low age of diagnosis, and reduced development of collateral circulation. Predictors of negative surgical outcome include vomiting, unprovoked pain, and increased formation of collateral circulation.
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