Celiac artery compression syndrome (also referred to as celiac axis syndrome, median arcuate ligament syndrome, and Dunbar syndrome) is defined as chronic, recurrent abdominal pain related to compression of the celiac artery by the median arcuate ligament. It is an uncommon disorder that is characterized clinically by the triad of postprandial abdominal pain, weight loss, and sometimes an abdominal bruit. The diagnosis is often one of exclusion, given the nonspecific symptoms which overlap with other forms of chronic intestinal ischemia. Treatment may involve surgical decompression of the celiac access; however, patient selection can be challenging because success cannot be guaranteed.
The etiology, clinical features, diagnosis, and treatment of celiac artery compression syndrome will be reviewed here. An overview discussing other etiologies of acute mesenteric and chronic mesenteric ischemia is presented elsewhere. (See "Acute mesenteric ischemia".)
ETIOLOGY AND PATHOPHYSIOLOGY
Celiac artery compression syndrome was initially described in the 1960s and is defined as abdominal pain related to compression of the celiac artery by the median arcuate ligament [1-4]. The syndrome is also referred to as celiac axis syndrome, median arcuate ligament syndrome, and Dunbar syndrome.
The median arcuate ligament is a fibrous arch that traverses the aorta just cephalad to the origin of the celiac artery, and bridges the crura of the diaphragm (figure 1). Adjacent to the median arcuate ligament is the celiac plexus (or ganglion), which originates from preganglionic splanchnic nerves, somatic branches from the phrenic and vagus nerves, parasympathetic preganglionic nerves, and sympathetic postganglionic fibers .
The etiology is incompletely understood and the pathophysiology may be related to both ischemic and neuropathic mechanisms. In spite of many case reports where the diagnosis appeared to be clear cut, and symptoms have been relieved following surgical decompression of the celiac artery, the diagnosis (and existence) of celiac artery compression syndrome has been a source of controversy [6-11]. In celiac artery compression syndrome, although flow may be reduced in the celiac artery, the superior mesenteric artery (SMA) and the inferior mesenteric artery are widely patent, and should be capable of providing ample perfusion to the bowel (figure 2). As a result, many have suggested that the symptoms may not be related to blood flow but rather to involvement of the celiac plexus, which lies in the vicinity of the arcuate ligament. Others have associated celiac artery compression with delayed gastric emptying, which can also be related to nerve dysfunction, suggesting another possible source for symptoms .