Celiac artery compression syndrome

INTRODUCTION — Celiac artery compression syndrome is defined as chronic, recurrent abdominal pain related to compression of the celiac artery by the median arcuate ligament. It is also referred to as celiac axis syndrome, median arcuate ligament syndrome, and Dunbar syndrome. 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 that overlap with other forms of chronic intestinal ischemia. Treatment involves surgical decompression of the celiac axis. Treatment success, which cannot be guaranteed, depends upon appropriate patient selection. Celiac artery decompression can be accomplished using an open or minimally invasive approach, which can either be a standard laparoscopic or a robotic-assisted approach. For some patients, celiac artery revascularization (open, endovascular) may be necessary.

The etiology, clinical features, diagnosis, and treatment of celiac artery compression syndrome are reviewed here. An overview of other etiologies for acute mesenteric ischemia and chronic mesenteric ischemia is provided separately. (See "Overview of intestinal ischemia in adults" and "Chronic mesenteric ischemia".)

ETIOLOGY AND PATHOPHYSIOLOGY — The etiology and pathophysiology of celiac artery compression syndrome are incompletely understood but may be related to both ischemic and neuropathic mechanisms.

The anatomy of the celiac region (including the median arcuate ligament and its variations [1]) was described long before the relationship between abdominal symptoms and compression of the celiac artery by the median arcuate ligament was described clinically [2-6]. The syndrome is also referred to as celiac artery compression, celiac axis syndrome, median arcuate ligament syndrome, and Dunbar syndrome.

The median arcuate ligament is a fibrous arch that traverses the aorta and bridges the crura of the diaphragm (figure 1). Typically, the celiac axis branches from the abdominal aorta (figure 2) below the median arcuate ligament (between T11 and L1), but wide variation in the location of the celiac origin has been reported [7]. A higher or lower origin of the celiac axis may be prone to compression. A subset of patients (approximately 10 percent) has an abnormally positioned median arcuate ligament that appears to compress an otherwise normally positioned celiac artery [8]. Compression of the celiac artery by the median arcuate ligament is accentuated during expiration as the median arcuate ligament moves cranially and relieved during inspiration [9].

Regardless of whether there is reduced flow in the celiac artery, perfusion to the bowel should be adequate in the presence of a widely patent superior mesenteric artery (SMA) and the inferior mesenteric artery (IMA), given the collateral circulation to the intestines (figure 3). The high prevalence of individuals with radiographic evidence of celiac compression but without symptoms supports this assertion. In one review of 400 celiac artery angiograms performed at the time of chemoembolization of hepatic tumors, 7.3 percent of the patients had significant celiac stenosis, defined as greater than 50 percent stenosis and greater than a 10 mmHg pressure gradient (one-half due to median arcuate ligament compression), but none were symptomatic [10].

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. These nerves may also contribute to celiac artery compression [11,12]. Pain associated with celiac artery compression syndrome may be mediated by the celiac plexus, although this is controversial and the evidence is limited [13,14]. Celiac artery compression can also be associated with delayed gastric emptying, which may be due to nerve dysfunction [15,16].

The diagnosis has been made in children and in monozygotic twins, suggesting a possible genetic basis [17-23].

An acquired transient form of the syndrome has also been reported following blunt abdominal trauma and pancreatic surgical procedures, such as pancreaticoduodenectomy likely related to local trauma [24-28].

EPIDEMIOLOGY — The celiac artery is compressed by the median arcuate ligament in up to one third of autopsy subjects [2,29]. However, the incidence of clinical symptoms attributable to this syndrome is much lower than the finding of celiac artery compression on imaging studies. Based upon diagnostic studies using computed tomography (CT) of the abdomen or arteriography, the incidence of asymptomatic celiac artery stenosis appears to be approximately 7 percent, approximately one half of which might be attributable to compression by the median arcuate ligament [10,30].

Celiac artery compression syndrome is more prevalent in females compared with males (4:1 ratio) and is more common in individuals between the ages of 40 and 60 years with a thin body habitus [31]. In a large series, 39 of 51 patients were females, and the mean age was 47 years [32]. However, it can occur in pediatric populations [33-35].

CLINICAL FEATURES — Patients with celiac artery compression syndrome report a widely variable degree of postprandial abdominal pain most often located in the epigastric region. Some patients have symptoms of delayed gastric emptying [15,16,36]. Weight loss, nausea, vomiting, and diarrhea are also reported.

Abdominal pain — Abdominal pain, most typically occurring after meals, is the most common presenting symptom and was present in 94 percent of patients in one early review [37] and in 80 percent of patients in a systematic review [11]. Abdominal pain triggered by exercise has also been reported [37]. The pain can also occur at rest and can be intermittent or constant [3,32,38].

Abdominal pain may be positional, relieved by leaning forward or drawing the knees to the chest [31,39,40].

Unintentional weight loss — Unintentional weight loss (>20 pounds) is reported in approximately one half of patients [3,11,32,38].

Other symptoms — Abdominal pain may be accompanied by a variable degree of nausea, vomiting, or diarrhea. In one series of 36 patients, bloating occurred in 39 percent and nausea/vomiting in 56 percent [37]. In a larger series, nausea occurred in only 9.7 percent and diarrhea in 7.5 percent [11].

Physical examination — Physical examination is usually normal. Weight loss may be evident, and epigastric tenderness may be present. On auscultation of the abdomen, an epigastric bruit that is louder with expiration may be heard but is often not present. In one review, 35 percent of patients had an abdominal bruit [11]. However, neither epigastric tenderness nor epigastric bruits is specific to celiac artery compression syndrome.

DIAGNOSIS — Celiac artery compression syndrome may be suspected in middle-aged (40 to 60 years) female patients based upon a clinical triad of postprandial abdominal pain, weight loss (>20 pounds), and sometimes an abdominal bruit [3,32,38,41,42]. However, the diagnosis requires vascular imaging to confirm compression of the celiac artery by the median arcuate ligament, preferably using respiratory maneuvers (algorithm 1). For some patients, other imaging may be needed to exclude another identifiable source for the patient's symptoms. (See 'Differential diagnosis' below.)

Approach — Because of its rarity and lack of specificity of symptoms and signs, celiac artery compression syndrome is low on the differential diagnosis for chronic abdominal pain. An imaging diagnosis of celiac artery compression may be quite unexpected, or the diagnosis may be one of exclusion only after an extensive workup. Upon review of the patient's history, the patient may have undergone extensive evaluation, including endoscopy or imaging studies or even surgery in an effort to identify the etiology for their pain [32]. In one case series, the average duration of symptoms was 34 months before a diagnosis was made [41].

Cross-sectional abdominal imaging (computed tomographic [CT] or magnetic resonance [MR] imaging) is frequently obtained initially to evaluate abdominal pain or other symptoms. In the absence of other obvious findings suggesting a source of abdominal pain, findings consistent with celiac artery compression include celiac artery stenosis with or without poststenotic dilation or splanchnic artery aneurysm (algorithm 1) [8,20,36,43-49]. In a review of asymptomatic patients with celiac artery compression followed over a seven-year period, 9 of 32 (24 percent) developed splanchnic artery aneurysm, one of whom ruptured [48]. The pancreaticoduodenal arcades and dorsal pancreatic artery were the most common sites for development of aneurysms (56.3 percent), which were significantly more common in patients with a collateral circulation compared with those without collateral circulations (47 versus 5 percent). In another series of 23 patients, the degree of celiac stenosis positively correlated with the presence of collateral circulation [50].

For patients in whom celiac artery compression is suspected based upon cross-sectional imaging, positive findings on advanced vascular imaging (typically CT or MR angiography with three-dimensional reconstruction (image 1)) combined with a representative duplex study with respiratory maneuvers establishes the diagnosis. Three-dimensional reconstruction provides multiple views by which to see arterial compression from various angles and the relationship to surrounding structures. Collateral vessels (image 2) may or may not be seen on CT or MR angiography. In one review of 23 patients, 16 (70 percent) had significant arterial collateral circulation [51]. Although not usually needed, catheter-based arteriography with inspiratory and expiratory views may provide additional information if the diagnosis is still in question. (See 'Arteriography' below.)

Duplex ultrasound may show elevated systolic velocities (table 1) at the origin of the celiac artery indicative of stenosis, which are increased during expiration and normalized with inspiration [52-54]. (See 'Duplex ultrasound' below.)

Based upon findings on abdominal imaging studies with respect to the clinical presentation, further evaluation is as follows:

●No further evaluation is needed for those with positive study for celiac artery compression (or stenosis) in a patient who does not complain of abdominal pain (ie, incidental diagnosis), provided the superior mesenteric artery is patent and there is evidence of collateral flow. The patient should be counseled regarding symptoms of celiac artery compression syndrome and advised to follow up if postprandial abdominal symptoms occur.

●We suggest inspiratory and expiratory vascular imaging for the clinical scenarios listed below. Inspiratory and expiratory views are most easily accomplished using duplex ultrasound or catheter-based arteriography [55]. (See 'Inspiratory and expiratory vascular imaging' below.)

•The abdominal imaging study is positive for celiac stenosis in a patient strongly suspected of having the disease based upon the clinical presentation.

•The abdominal imaging study is negative for celiac stenosis in a patient suspected of having the disease.

•The abdominal imaging study is positive for celiac stenosis in a patient with abdominal pain who was not suspected of having celiac artery compression (ie, a surprise diagnosis). In this case, the finding may be a coincidence given the high prevalence of asymptomatic celiac artery stenosis. It is important to review the patient's complaints for consistency with this syndrome.

Other provocative physiologic studies, including gastric tonometry and ganglion nerve block, may help confirm the diagnosis in patients with atypical clinical features or indeterminate findings on imaging. These may also be helpful for predicting clinical success following celiac artery decompression. (See 'Physiologic testing' below.)

Inspiratory and expiratory vascular imaging

Arteriography — Patients with symptoms consistent with celiac artery compression, no other obvious etiology for their abdominal pain, and a suggestion of celiac artery compression on routine imaging studies (eg, cross-sectional abdominal imaging, duplex ultrasound imaging) should undergo inspiratory and expiratory arteriography to confirm the diagnosis. In a systematic review comparing the outcomes of open with laparoscopic treatment, inspiratory and expiratory arteriography was the most common imaging modality used to definitively diagnose celiac artery compression [11]. Many clinicians will proceed with treatment based upon the results of advanced imaging studies (CT or MR angiography) due to their high sensitivity for detecting arterial stenosis, given the difficulty in obtaining respiratory views with CT or MR angiography [56]. With CT angiography, one can choose imaging with either inspiration or expiration; obtaining the other view would require another bolus of contrast. Comparatively speaking, obtaining such views is less cumbersome for MR angiography. An alternative approach is to combine the results of CT or MR angiography without respiratory maneuvers and ultrasound with respiratory maneuvers, rather than subject the patient to catheter-based arteriography.

Catheter-based conventional aortography will confirm the narrowed or obstructed lumen of the celiac axis and can evaluate flow dynamics as well as the collateral circulation, but it only indirectly images the source of external compression (unlike CT or MR angiography or duplex ultrasonography). Celiac artery compression syndrome is inferred by differences in end-inspiratory versus end-expiratory imaging. During expiration, the diaphragm moves cranially, stretching the crura, and exacerbating celiac artery compression if it is present (image 3A-B). Conversely, during inspiration, the diaphragm moves in a caudal direction and the crura become lax, relieving any compression. A widely patent celiac artery without any evidence for compression with expiration excludes a diagnosis of celiac artery compression syndrome.

Other findings on arteriography that suggest celiac artery stenosis include a poststenotic dilation, which is a common physiologic response to chronic arterial stenosis; measurement of a pressure gradient across the celiac artery origin; and retrograde filling of the celiac artery via a dilated gastroduodenal artery.

One report described a novel technique in which the superior mesenteric artery was selectively cannulated and a vasodilator drug injected during angiography [57]. A positive test was defined as reproduction of symptoms and loss of collateral filling of the celiac territory. The study involved only eight patients, four of whom had a positive test. Of these four, three had resolution of their symptoms following surgical decompression.

Duplex ultrasound — Inspiratory and expiratory imaging can also be performed using duplex ultrasound. The changes seen on ultrasound with respiratory maneuvers are often more dramatic compared with those identified on arteriography. Ultrasound has the advantage of being noninvasive; however, a disadvantage of ultrasound, particularly for this condition, is operator dependence.

Findings that are diagnostic for celiac artery compression include visible external compression of the artery with expiration, increased flow velocities (>200 cm/second for the celiac artery, >275 cm/second for the superior mesenteric artery) (table 1), and poststenotic dilation (image 4) [8,17,52,54,58-60].

In one review in which duplex ultrasonography was used in 364 patients, sensitivity was 83 percent and specificity was 100 percent for a diagnosis of celiac artery compression syndrome using peak systolic velocity of ≥350 cm/second, a 210 percent change in pulse volume amplitude with inspiration and expiration, and a celiac artery deflection angle of 50° [55].

Physiologic testing

Gastric tonometry — Gastric tonometry has been used as an adjunctive physiologic test to identify gastric ischemia in patients with suspected celiac artery compression syndrome [61,62]. It is a sensitive test for differentiating celiac artery compression syndrome from other confounding diagnoses, but it is not widely available and is rarely necessary to establish the diagnosis [63].

Measurements are taken before, during, and after exercise. Criteria for a positive test include:

●Gastric arterial PaCO₂ difference >0.8 kPa

●Arterial lactate level <72 mg/dL (multiply by 0.11 to convert to millimoles per liter)

●An increase in gastric PaCO₂ levels after exercise

A positive gastric tonometry study may predict clinical success after celiac artery decompression. In a small study of 29 patients diagnosed with celiac artery compression aided by gastric exercise tonometry, at a mean follow-up of 39 months, repeat tonometry after treatment demonstrated normal findings in all patients who had relief of their symptoms but in only one of four patients who had persistent symptoms [63].

Ganglion nerve block — Percutaneous celiac ganglion block can also be used as a provocative physiologic test. The rationale for ganglion nerve block is that symptoms of celiac artery compression syndrome may be the result of inflammation and compression of the nerve fibers of the celiac plexus. (See 'Etiology and pathophysiology' above.)

The procedure involves CT-guided percutaneous injection of the celiac ganglion using local anesthetic agents (eg, lidocaine, bupivacaine) for short-term relief of pain. If successful, ethanol can be used for permanent nerve blockade [64,65].

A positive response to celiac ganglion nerve block may also identify patients who will respond well to surgical treatment [39,66]. In one small study, long-term pain relief was seen in nine patients who had good response to preoperative celiac ganglion block [39].

Differential diagnosis — The clinical presentation of celiac artery compression syndrome is nonspecific, and the symptoms and signs overlap with other forms of chronic intestinal ischemia and other causes of abdominal pain. Among the causes of abdominal pain, postprandial abdominal pain can be due to cholecystitis, pancreatitis, gastroesophageal reflux disease, chronic intestinal ischemia, and gastric outlet obstruction. The differential diagnosis of abdominal pain is discussed in detail elsewhere. (See "Causes of abdominal pain in adults".)

Chronic intestinal ischemia can be related to peripheral artery disease (PAD) or mesenteric vasculitis. These patients present with postprandial abdominal pain, weight loss, and abdominal pain. However, chronic mesenteric ischemia typically occurs in older individuals with risk factors for cardiovascular disease (eg, smoking, hypertension) and often with other clinical manifestations of PAD (eg, claudication). (See "Chronic mesenteric ischemia" and "Overview of gastrointestinal manifestations of vasculitis".)

Patients with gastric outlet obstruction may have postprandial abdominal pain and weight loss; however, vomiting occurs in nearly all patients, and upper gastrointestinal imaging studies will note the obstruction. (See "Gastric outlet obstruction in adults", section on 'Clinical manifestations'.)

Although delayed gastric emptying has been reported in patients with celiac artery compression syndrome and patients may complain of postprandial fullness, vomiting is not prominent. The younger patient with postprandial abdominal and gastric outlet obstruction may have superior mesenteric artery syndrome. (See "Superior mesenteric artery syndrome".)

TREATMENT

Patient selection — Celiac artery decompression is indicated only for symptomatic patients with confirmed celiac artery compression on inspiratory and expiratory vascular imaging studies. Asymptomatic patients should be counseled regarding symptoms of celiac artery compression syndrome and advised to follow up if postprandial abdominal symptoms occur. (See 'Diagnosis' above.)

The key to successful outcomes for the treatment of celiac artery compression syndrome is appropriate patient selection. However, data to guide the identification of patients are limited [32,38,39,42,67,68]. The following factors were associated with a favorable outcome after surgery in a review of 51 patients [32]:

●Postprandial pain pattern

●Age between 40 and 60 years

●Weight loss of 20 pounds or more

Less success was associated with:

●Atypical pain patterns with periods of remission

●Age greater than 60 years

●A history of a psychiatric disorder or alcohol abuse

●Weight loss of less than 20 pounds

In a review of 67 patients, of which 64 percent were treated surgically, postexertional pain predicted improvement after surgery [69]. Patients who presented with vomiting and unprovoked pain were not likely to respond to surgery.

Although these features can provide some guidance, the quality of these data is low given the small size of the study and other factors.

Before consideration for celiac artery decompression, preoperative psychologic assessment and management of any comorbid conditions is a necessary step. In a prospective study of 51 adult patients, the preoperative assessment identified concurrent psychiatric diagnosis in 28 percent, the presence of which predicted a significantly lower postoperative quality of life [70]. Similarly, in children, comorbid psychological conditions are common and continue following treatment of celiac artery compression syndrome [71].

Celiac artery decompression — The treatment of symptomatic celiac artery compression syndrome aims to restore celiac blood flow by relieving the extrinsic compression on the vessel (algorithm 1) [11,40,72-76]. Division of the fibers of the celiac ganglion (ie, neurolysis, celiac ganglionectomy) is performed concurrently by many surgeons to address the potential neuropathic component of the disease. Aggressive resection of the median arcuate ligament and nerve fibers, rather than simple division, may inhibit reformation. Laparoscopic and robotic-assisted laparoscopic approaches to celiac artery decompression have been reported [11,66,77-89]. The outcomes of treatment are based primarily on institutional reviews with small numbers of patients, given the rarity of the syndrome. The heterogeneity of patient symptoms and treatment regimen makes it difficult to determine the best treatment with certainty. Regardless of the approach used, celiac artery decompression does not always relieve symptoms [90]. (See 'Long-term outcomes' below.)

Approach and technique — Based on the available information, we approach celiac artery decompression in the following manner:

●Median arcuate ligament release should be the primary surgical treatment. We also perform ganglionectomy during the index operation [72-76]. Although percutaneous transluminal angioplasty (PTA) is a useful adjunct for persistent stenosis following surgical celiac artery decompression, it should not be used as a sole treatment. When used without celiac artery decompression, patient outcomes are poor, likely due to the sustained extrinsic pressure exerted on the celiac artery [76,91-94]. (See 'Revascularization for persistent stenosis after decompression' below.)

●An open or minimally invasive approach can be used for celiac decompression. We suggest a laparoscopic approach (standard or robotic assisted), rather than open surgery. Immediate relief of symptoms is improved for minimally invasive approaches, which may be related to a better operative view and more complete circumferential release and neurectomy. (See 'Minimally invasive versus open decompression' below.)

●Following decompression, assessment of celiac artery flow can be performed intraoperatively or postoperatively. For robotic-assisted procedures, intraoperative assessment may not be possible. (See 'Intraoperative vascular assessment' below.)

●For persistent stenosis or recurrent symptoms, any of the following may be used: ganglionectomy, if not already performed; percutaneous revascularization (eg, celiac artery angioplasty/stenting); or surgical revascularization (ie, interposition or bypass grafting) [95,96]. (See 'Revascularization for persistent stenosis after decompression' below.)

To perform celiac artery decompression:

●Laparoscopic celiac artery compression syndrome involves the use of four to five port sites placed into the upper abdomen (figure 4). Following mobilization of the supraceliac aorta, the median arcuate ligament and ganglionic fibers are resected to skeletonize the celiac vessel [84,87,97]. Robotic-assisted celiac artery decompression is our preferred method because of the ease of visualization and the ability to obtain a 360° complete release.

●The traditional open surgical approach involves an upper midline laparotomy to access the proximal aorta through the gastrohepatic ligament [3,6]. The diaphragmatic crura are mobilized away from the celiac artery by incising them for approximately 5 cm cephalad to expose up to 4 cm of aorta. Celiac artery decompression may be accompanied by reconstruction or bypass of the stenotic arterial segment [13,98].

Minimally invasive versus open decompression — Based upon observational studies, we suggest a minimally invasive approach (conventional laparoscopic, robotic-assisted) for celiac artery decompression. Minimally invasive decompression of the celiac artery has increasingly become accepted as the standard surgical approach.

A systematic review identified 20 observational studies that included 400 patients treated using a laparoscopic (n = 121) or open (n = 279) approach for celiac artery decompression [11]. A higher proportion of patients in the laparoscopic group had immediate relief of their symptoms compared with the open group (96 versus 78 percent). Conversion from a laparoscopic to an open approach was needed in 9.1 percent related to bleeding. Late recurrence of symptoms was similar for the laparoscopic and open approaches at 5.7 and 6.8 percent, respectively. Among patients initially treated laparoscopically, 14 percent required subsequent celiac artery revascularization (endovascular or open). Comparing several small published series on laparoscopic decompression performed via a transabdominal approach with one large series performed via a retroperitoneal approach, better results were obtained using a retroperitoneal approach [82].

Advantages of a minimally invasive approach for the treatment of celiac artery compression syndrome include smaller incisions, improved view of the surgical field, decreased pain and postoperative morbidity (eg, ileus, blood loss, adhesive bowel obstruction), and shorter recovery time [99]. Disadvantages include difficulty controlling hemorrhage, potential for incomplete decompression, and increased risk of injury to the abdominal aorta if laparoscopic dissection is difficult. In addition, fixed stenosis of the celiac artery may necessitate conversion for open bypass or adjunctive endovascular angioplasty or stenting. In one case series, a higher proportion of patients (26 percent; 4 of 15) compared with the review above required conversion to open surgery, all for intraoperative bleeding; only one required a blood transfusion [84].

Robotic-assisted laparoscopic surgery may help overcome some of these disadvantages by providing optical enhancements (increased magnification of structures, three-dimensional view) and operator-based improvements (tremor elimination, added degrees of motion, and scaled operator movements) [100]. This may enhance the view of the dissection at the base of the celiac trunk [78,80,100]. As with any robotic surgery, limitations of this modality include longer operating time, additional training for the surgeon, and increased cost. Although limited studies suggest that robotic-assisted treatment of celiac artery compression syndrome is effective, further study is warranted. (See "Robot-assisted laparoscopy".)

Intraoperative vascular assessment — Confirmation of the completeness of celiac artery release can be obtained by visual inspection or by directly assessing celiac flow to determine whether there is a need for additional decompression or other treatments [32,98]. Either arteriography or duplex ultrasound can be used. With a minimally invasive approach, intraoperative ultrasound using a laparoscopic probe is the most practical.

Ultrasound verifies adequate decompression by directly imaging the vessel and by demonstrating the return of celiac artery velocities to normal levels. In one study that used laparoscopic ultrasound, subjective improvement of symptoms was reported in 14 of 15 patients (93 percent) at a mean follow-up period of 44.2 months [84]. However, resolution of the symptoms has been reported both with and without the use of intraoperative ultrasound [77,79,84,87].

Another option is intraoperative aortography with anteroposterior and lateral views obtained before and after decompression, particularly with the availability of a hybrid operating room. Arteriography may be facilitated by positioning an aortic catheter prior to the decompression procedure.

Revascularization for persistent stenosis after decompression — A decision for celiac artery decompression combined with vascular reconstruction depends on the intraoperative status of the celiac artery after release of the compressive fibers. The rationale for vascular reconstruction is based upon the known histologic changes that can occur in the celiac artery related to chronic compression. These include intimal hyperplasia and abnormalities in the smooth muscle and adventitia [13].

The need for arterial reconstruction is suggested by persistently elevated velocities on ultrasound or persistent stenosis on arteriography after celiac artery decompression and also by persistent arterial deformation, persistent thrill, or pressure gradient in the celiac artery in spite of what appears to be successful decompression [91,94,99]. Although celiac artery decompression alone appears to provide relief in most series [11], the largest surgical series showed that successful revascularization of the celiac artery, including correcting any intrinsic stenosis that may persist after decompression, is important for reducing late symptom recurrence [32].

●Open celiac artery revascularization – One option while performing minimally invasive celiac artery decompression is conversion to an open approach with open celiac artery revascularization to restore adequate flow. However, open celiac artery reconstruction appears to be more commonly performed during procedures that have used an open surgical approach initially [11]. Options for open revascularization include patch angioplasty of the celiac artery, reimplantation of the celiac artery onto the aorta (with or without interposition grafting), and aortoceliac bypass of the stenotic segment using a reversed saphenous vein conduit or polyester (eg, Dacron) graft. (See "Chronic mesenteric ischemia", section on 'Revascularization'.)

●Celiac angioplasty – Although not a successful first-line therapy, angioplasty/stenting has a role in the treatment of residual stenosis after celiac artery decompression and in those with recurrent symptoms [58,101]. (See 'Approach' above and 'Early recurrence' below.)

When resources are available (eg, hybrid operating room), minimally invasive celiac artery decompression can be combined with intraoperative angioplasty/stenting, or angioplasty/stenting can be performed postoperatively. In one small series of 15 patients treated laparoscopically, 4 underwent angioplasty [84]. Symptoms improved in three of these patients; the remaining patients achieved symptom relief only after a celiac artery bypass. In another series, 14 of 16 patients with celiac artery compression syndrome treated laparoscopically remained asymptomatic over a mean follow-up of 28 months [83]. Two required further intervention for relief of symptoms, including angioplasty and stenting of the celiac artery in one and aortoceliac bypass in the other.

Complications — Complications depend upon the nature of the treatment. In the above-mentioned systematic review of 400 cases [11]:

●Following laparoscopic decompression, open conversion was needed in 9 percent of patients due to bleeding, which was serious in some cases; no deaths were reported. Other complications included pneumothorax, pancreatitis, and gastroparesis. (See "Complications of laparoscopic surgery".)

●Following open vascular reconstruction, the incidence of perioperative complications was 6.5 percent; no deaths were reported. Complications included thrombosed bypass graft, stroke, pancreatitis, and splenic infarction.

POSTOPERATIVE CARE AND FOLLOW-UP — Patients are typically hospitalized for two to three days postoperatively following celiac artery decompression. A diet can be initiated immediately and advanced as tolerated. For patients with significant weight loss preoperatively, nutritional consultation is advised.

Patients follow up postoperatively in approximately one month to check any incisions, monitor their dietary progress, and evaluate for any recurrent symptoms. Duplex scanning is performed to confirm normalization of celiac artery velocities.

Early recurrence — When appropriately selected, the majority of patients treated with open or laparoscopic celiac artery decompression have immediate postoperative pain relief (78 and 96 percent, respectively, in the systematic review discussed above [11]).

For patients with symptoms that have not fully resolved or for early recurrence of symptoms, reevaluation is necessary. Treatment options include arterial stenting (if not performed) or arterial bypass. (See 'Revascularization for persistent stenosis after decompression' above.)

In a review of 21 patients, seven underwent celiac stent placement for recurrent or unresolved symptoms after laparoscopic celiac artery decompression [101]. Two patients required surgical bypass after an endovascular intervention failed. At the six-month follow-up, 75 percent of the patients were free from symptoms, and 64 percent were free of additional intervention.

In a study of a robotic-assisted approach, median arcuate ligament release was completed successfully in all nine patients. All patients improved symptomatically at the 25 week median follow-up [100]. Three patients experienced complete resolution on postoperative celiac duplex ultrasound imaging, and six patients showed an improved but persistent stenosis (peak systolic velocity >200 cm/second) compared with preoperative velocities. No patients required additional treatment.

LONG-TERM OUTCOMES — Long-term outcomes following open surgical decompression vary widely, partly due to the difference in patient selection with a variety of abdominal complaints as well as other medical (and often psychiatric) problems [38]. Some patients will not have long-lasting symptom relief [32,72,74,75,102-104].

Long-term follow-up is available predominantly for open surgical techniques; however, in contemporary series, minimally invasive techniques appear to be comparable, with late symptomatic recurrence rates of approximately 10 percent [11,32,105,106]. In the systematic review, symptom recurrence occurred in 19 of 280 patients (6.8 percent) in the open group and 7 of 123 patients in the laparoscopic group (5.7 percent) [11].

In the largest single series of 51 patients, at an average follow-up of nine years, 8 of 15 patients (53 percent) treated with celiac decompression alone and 22 of 29 patients (76 percent) treated by celiac artery decompression plus some form of revascularization remained asymptomatic [32]. Late follow-up arteriograms (18 studies) showed a widely patent celiac artery in 70 percent of asymptomatic patients but a stenosed or occluded celiac axis in 75 percent of symptomatic patients.

In a later series, 18 patients underwent open decompression of the celiac artery, 11 of whom required additional revascularization [107]. Follow-up was obtained in 15 patients with a mean duration after surgery of approximately 3.5 years. Eleven of the 15 patients were asymptomatic, and 9 of them had gained weight.

SUMMARY AND RECOMMENDATIONS

●Celiac artery compression syndrome – Celiac artery compression syndrome is defined as recurrent abdominal pain related to compression of the celiac artery by the median arcuate ligament (figure 1). It is an uncommon disorder that is also referred to as celiac axis syndrome, median arcuate ligament syndrome, and Dunbar syndrome. The symptoms may be related to ischemic and possibly also neuropathic mechanisms. (See 'Introduction' above and 'Etiology and pathophysiology' above.)

●Clinical features – Celiac artery compression syndrome is characterized clinically by the triad of postprandial abdominal pain, weight loss, and sometimes an abdominal bruit. It is four times more prevalent in females compared with males, typically in the fourth decade. It can also occur in pediatric populations. (See 'Epidemiology' above and 'Clinical features' above.)

●Diagnosis – Celiac artery compression syndrome may be suspected based upon clinical features; however, a definitive diagnosis requires vascular imaging, which may be supplemented by other tests. (See 'Diagnosis' above.)

•Vascular imaging – Vascular imaging can confirm compression of the celiac artery by the median arcuate ligament and is preferably performed with respiratory maneuvers (algorithm 1). During expiration, the diaphragm moves cranially, stretching the crura, and exacerbating celiac artery compression. Conversely, during inspiration, the diaphragm moves in a caudal direction and the crura become lax, relieving any compression. (See 'Inspiratory and expiratory vascular imaging' above.)

•Physiologic tests – Physiologic tests (gastric tonometry, ganglion nerve block) can help confirm the diagnosis in patients with atypical clinical features or indeterminate findings on imaging. These may also be helpful for predicting clinical success following celiac artery decompression. (See 'Physiologic testing' above.)

●Patient selection – The key to successful outcomes is the careful selection of patients for treatment. When appropriately selected, most patients treated with open or laparoscopic celiac artery decompression have immediate postoperative pain relief. Late symptomatic recurrence rates are 5 to 10 percent. Patients with an incidental diagnosis and either no symptoms or symptoms that are not related to the imaging findings should not be treated. Prior to consideration for celiac artery decompression, preoperative medical and psychological assessment is a necessary step. (See 'Patient selection' above and 'Long-term outcomes' above.)

●Treatment – Treatment of symptomatic patients with celiac artery compression confirmed on inspiratory and expiratory imaging involves dividing the median arcuate ligament and usually also the fibers of the celiac ganglion (ie, neurolysis). (See 'Celiac artery decompression' above.)

•Initial approach – For appropriately selected patients with confirmed celiac artery compression syndrome, we suggest an initial laparoscopic approach (standard or robotic assisted), rather than open surgery (Grade 2C). Immediate relief of symptoms is improved for minimally invasive approaches, which may be related to a better operative view and more complete circumferential release and neurectomy. Following celiac artery decompression, assessment of celiac artery flow can be performed intraoperatively or postoperatively. (See 'Approach and technique' above.)

•Persistent or recurrent symptoms – For patients with persistent stenosis or recurrent symptoms, any of the following may be used: ganglionectomy, if not already performed; percutaneous revascularization (eg, celiac artery angioplasty/stenting); or surgical revascularization (ie, interposition or bypass grafting). (See 'Revascularization for persistent stenosis after decompression' above and 'Early recurrence' above.)