AUTHOR AND EDITOR INFORMATION

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
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INTRODUCTION

Section 2 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
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Background

Although Schnitzler first described the clinical picture of postprandial clinical pain in 1901, the syndrome of postprandial abdominal angina generally is attributed to Baccelli or Goodman (1918). In 1936, Dunphy recognized that this syndrome was a precursor of fatal intestinal necrosis; however, not until 1957 did Mikkelsen propose surgical treatment of occlusive mesenteric vascular disease. Shaw and Maynard reported the first transarterial thromboendarterectomy of the superior mesenteric artery (SMA) in 1958, followed in rapid succession by Mikkelsen and Zarro in 1959. Numerous technical refinements followed.

Abdominal angina is a highly descriptive term for the postprandial pain that occurs in individuals with sufficient mesenteric vascular occlusive disease such that blood flow cannot increase enough to meet visceral demands. The mechanism is believed to be similar to the angina pectoris that occurs in individuals with coronary artery disease or the intermittent claudication that accompanies peripheral vascular disease.

Pathophysiology

The most common cause of abdominal angina is atherosclerotic vascular disease. The occlusive process commonly involves the ostia and the proximal few centimeters of the mesenteric vessels. Aortoiliac occlusive disease frequently coexists and may be the cause of the ostial lesions.

The 3 arteries supplying the gut are the celiac, superior mesenteric, and inferior mesenteric (see Media files 1-2). Unless significant stenoses or actual occlusion of 2 of the 3 vessels is present, efficient collateral circulation between the celiac and superior mesenteric arteries (ie, the pancreaticoduodenal arcades) and the superior and inferior mesenteric arteries (ie, the meandering mesenteric artery) ensures that blood flow to the gut generally is adequate. The internal iliac arteries also may be an important source of collateral hindgut and midgut perfusion in the presence of inferior mesenteric artery occlusion. 

SMA occlusion almost invariably is observed in patients with symptomatic occlusive mesenteric ischemia. Theories suggest that, because the SMA provides vascularity to the foregut, midgut, and hindgut, collaterals cannot sufficiently compensate for occlusion of this central artery.

Within 15 minutes of eating, duplex Doppler studies can show increased blood flow in the celiac and superior mesenteric vessels in healthy volunteers. Patients with abdominal angina are unable to sufficiently increase flow in the mesenteric vessels, and ischemic pain results. Affected individuals learn to associate food with pain, and thus, they develop a fear of eating. Weight loss may be significant.

Median arcuate ligament syndrome is thought to be a syndrome of abdominal pain caused by compression of the celiac trunk by the median arcuate ligament and, perhaps, by dense encasement by periarterial neural tissue. Described in 1965 by Dunbar and colleagues, compression of the celiac artery is thought to cause intimal fibrosis that leads to luminal stenosis and impaired splanchnic blood flow. This would result in symptoms similar to those of atherosclerotic mesenteric ischemia, which nearly always is caused by at least 2 major visceral artery occlusive lesions. In patients with median arcuate ligament syndrome, symptoms may be a result of compression of a single visceral artery in the absence of adequate collaterals; mesenteric steal or neurogenic mechanisms also have been proposed as causes. Symptoms have been reported to be provoked by exercise in isolated cases.

Definitive corroboration of any of these explanations is lacking, hence the controversial nature of the condition. Further discussion of this topic exceeds the scope of this article, but interested readers may refer to related references in the bibliography.

Frequency

International

The syndrome is extremely rare, and the true incidence is unknown.

Race

No data are available regarding the relative incidence among different races.

Sex

In contrast to the usual male predilection of atherosclerotic vascular disease, in most series, females outnumber males by approximately 3 to 1.

Age

The mean age of affected individuals is slightly older than 60 years. Median arcuate ligament syndrome (see Pathophysiology above) has been reported in young individuals.

CLINICAL

Section 3 of 11  

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History

• The hallmark of this condition is disabling midepigastric or central abdominal pain that develops 10-15 minutes after eating.
• • The pain gradually increases in intensity, reaches a plateau, and then slowly decreases in intensity several hours after eating.
  • Initially, this pain pattern develops only after large meals, but as the disease progresses, even small meals may be poorly tolerated.
• Some patients have associated motility disturbances such as diarrhea or constipation, bloating, or vomiting.
• The pain is poorly localized and described as cramplike or a dull ache. Occasionally, a patient may have constant or intermittent pain that occurs without a clear temporal relationship to eating.
• Soon, patients associate eating with pain and develop a characteristic fear of food (ie, sitophobia) or food-avoidance behavior. In several clinical series, reported weight loss averages 15-25 lb.
• The constellation of abdominal pain, weight loss, and an average age of 60 years commonly leads to a presumed diagnosis of malignancy and a protracted workup. Because none of the usual contrast studies or endoscopies performed in the course of a workup for malignancy are diagnostic, considerable delay in diagnosis typically results. In several series, the reported delay averages 16-18 months.
• If patients have multiple risk factors for atherosclerotic occlusive disease, a heightened clinical suspicion for this diagnosis shortens the typical delay in diagnosis.
• A history of peripheral vascular disease is common. As with other vasculopathies, individuals who smoke predominate in all series.
• Although diabetes occurs in all series, it is uncommon in patients with this syndrome (in contrast to most other vascular problems).
• Occasionally, a patient presents with a duodenal or gastric ulcer (which may be Helicobacter pylori negative) or with ischemic colitis.
• Ischemic pancreatitis also may occur and is associated with epigastric pain. Laboratory studies reveal mildly elevated amylase and lipase. Steatorrhea may be observed.

Physical

• Physical examination reveals stigmata of weight loss. The abdomen typically is scaphoid and soft, even during an episode of pain.
• In one series, approximately 10% of patients had positive test results for guaiac.
• Abdominal bruit is present in 60-90% of patients, but this is common in many elderly persons who are not affected by this syndrome.
• Signs of peripheral vascular disease, particularly aortoiliac occlusive disease, may be present.

Causes

• Smoking is an associated risk factor. In most series, approximately 75-80% of patients smoke.

DIFFERENTIALS

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Other Problems to be Considered

Many patients initially are assumed to have cancer. The constellation of weight loss, abdominal pain, and an age of older than 60 years leads to a workup with GI contrast studies, CT scans, and other diagnostic tests related to malignancy, none of which are diagnostic for chronic mesenteric vascular disease.

WORKUP

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Lab Studies

• No laboratory tests are diagnostic.
• Patients with ischemic pancreatitis may have elevated amylase or lipase levels.

Imaging Studies

• Biplane aortography still is the criterion standard test. Because the vessels emerge from the anterior wall of the aorta, the ostia are visualized only on a lateral view (see Media file 3). 
• A meandering mesenteric artery is another clue (see Media file 4). In addition to demonstrating the level of stenosis or occlusion of the mesenteric vessels, angiography findings also help plan the operative approach by delineating the anatomy of the supraceliac and infrarenal abdominal aorta.
• Hydrating the patient well before angiography is extremely important, not only to avoid renal toxicity but also because visceral infarction may be precipitated by the injection of contrast.
• Duplex ultrasound examination is emerging as a useful screening modality. It currently is most useful in patients in whom the diagnosis is suspected. If the duplex examination is positive, angiography is performed.
• Indices that are studied include the following:
• • Peak systolic flow is increased if a stenosis is present.
  • When an increase is expected, a change in flow velocities occurs in response to feeding, unless a flow-limiting stenosis is present.
  • Perform a spectral analysis of Doppler frequencies.
• Magnetic resonance angiography has been used as an alternative to aortography in patients who have contrast sensitivity or who are at risk for contrast-related renal dysfunction. It is emerging as an excellent diagnostic modality due to its ability to delineate the anatomy. It also is a useful experimental tool for studying metabolic parameters.

Other Tests

• Because most of these patients have generalized vascular disease, performing a cardiovascular evaluation prior to surgery is prudent.

TREATMENT

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Medical Care

No effective medical therapy exists. Percutaneous transluminal angioplasty (PTA) may be an alternative therapy for selected patients.

• The role of PTA (with or without stenting) as an alternative to surgery is currently under investigation.
• The use of PTA has been reported for severe stenoses, but follow-up data are limited. It may not be feasible if the ostial stenosis is caused by a calcified aortic plaque. In one series of 25 patients, 24 were treated successfully. Of the patients with recurrent stenoses, which developed in 4 patients, 2 were treated angiographically and 2 were treated surgically.
• Reperfusion syndrome does not appear to occur after angiographic dilatation. Stenting may increase the durability of this procedure, but, as yet, long-term patency rates have not been described for this relatively new form of treatment.

Surgical Care

Mesenteric revascularization relieves the symptoms and may prevent intestinal infarction.

• Generally, at least 2 of the 3 vessels must be revascularized. Sometimes, all 3 are corrected. In one series, recurrent symptoms developed in 50% of patients in whom only 1 vessel was revascularized. Also, several perioperative deaths occurred from intestinal infarction.
• Several surgical options are available as follows:
• • The first is transarterial endarterectomy, which also was the first type of procedure developed. The artery is dissected, and proximal and distal control is achieved. A longitudinal arteriotomy is made, and the plaque is removed from the visceral vessel. The artery is closed, with or without a patch.
  • The next procedure developed was the retrograde bypass (see Media file 5). In this procedure, a graft is brought from a convenient nondiseased (ie, healthy) origin on the distal aorta or even the iliac vessels. It is routed retrograde and anastomosed to a healthy segment distal to the occlusion or stenosis in the visceral vessel. The major advantage of this procedure is ease of dissection; however, in some series, the more indirect route and the length of the graft (ie, with susceptibility to kinking) have resulted in an increased risk of graft occlusion.
  • Antegrade bypass probably is the most common procedure performed today. Typically, the proximal trunk of a small-caliber bifurcated prosthetic graft is anastomosed to the supraceliac aorta, and the distal limbs are sewn to the celiac artery and the SMA, just beyond the stenotic segments (see Media files 6-8). The conduit may be the autogenous saphenous vein or it may be a prosthetic graft. Prosthetic graft alternatives are ePTFE or Dacron. Although some favor prosthetics over vein grafts and vice-versa, no clear patency advantages exist for either. Most surgeons concur that a vein is the material of choice in a contaminated field.
  • Trapdoor aortotomy is an alternative used in some centers. The aorta is exposed, and vascular control is obtained. A flap is developed that includes the orifices of all the visceral vessels. Endarterectomy is performed, and the flap is sutured back in place (see Media file 9). The difficulties associated with working with a diseased aorta and concerns about complicating future aortic surgery have resulted in the failure of this method to attain widespread adoption.
• Intraoperative duplex ultrasound examination is performed to confirm the technical adequacy of the revascularization (see Media file 10). 
• Controversies in surgical treatment include the following:
• • Antegrade bypass versus transaortic endarterectomy
  • The role of duplex ultrasound in follow-up
  • The role of magnetic resonance imaging in follow-up
  • The management of asymptomatic occlusion detected at follow-up
  • The best material for bypass, ie, vein or prosthetic graft
• The specific approach for surgical reconstruction, ie, bypass versus endarterectomy, depends on the location and number of stenoses, previous surgeries, patient comorbidities, and local operative conditions. Because patency and morbidity and mortality rates are similar for both, the authors prefer to individualize each patient, applying the technique best suited to the circumstances. Follow-up of patients with duplex ultrasound is performed yearly for the first several years. Asymptomatic occlusions are followed expectantly. Most patients succumb to other atherosclerotic comorbidities before developing symptomatic restenoses or occlusions. However, if a restenosis is identified, it is treated based on the same criteria as the original lesion.
• Whether to use a vein of a prosthetic graft usually is addressed in the same manner as the choice of the surgical approach. Local individual patient characteristics dictate the choice of conduit material. Often, synthetic material is chosen for ease of use and availability. However, in the face of a contaminated field, a vein is clearly preferred.

Diet

• Although most of these patients are cachectic, preoperative central venous nutrition has not been shown to decrease complications and is employed only selectively.
• No particular dietary restrictions are associated with the surgery. After adequate surgical correction, patients may resume the usual diet for their particular underlying medical condition (if any).

MEDICATION

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No effective medical therapy exists.

FOLLOW-UP

Section 8 of 11  

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• Follow-up
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Further Inpatient Care

• Intraoperative considerations
• • Cardiac monitoring with transesophageal echo or invasive monitoring may be needed.
  • Intraoperative duplex ultrasound examination of the reconstruction is important.
• Postoperative care and complications
• • Most patients require monitoring in an intensive care unit. Postoperative ileus is common.
  • In addition to the usual cardiac problems traditionally associated with major vascular repairs, major postoperative complications include bleeding and coagulopathy, pulmonary insufficiency, and hepatic and renal failure.
  • In multiple studies, a picture similar to multiorgan dysfunction occurs in a small but significant minority of patients. Reperfusion injury has been hypothesized to trigger this cascade of events. Immediate pronounced hepatocellular dysfunction has been noted as an early event in some of these cases.
  • In one series, coronary artery disease and chronic renal insufficiency prior to surgery were associated with postoperative complications.

Further Outpatient Care

• Both duplex ultrasonography and magnetic resonance imaging have been used for follow-up, but, because the proper management of an asymptomatic occlusion of a reconstruction is unknown, this generally is not recommended.

Complications

• See Further Inpatient Care.

Prognosis

• Currently, more than 700 cases in the literature describe good early clinical results after surgical revascularization, and this treatment remains the criterion standard.
• • Reocclusion is more prevalent in males than in females (in contrast to the female predominance noted at initial presentation).
  • Several series have demonstrated that 86-96% of patients remain asymptomatic at 5 and 10 years, with similar graft patency rates.

Patient Education

• Patients should be counseled to stop smoking.

MISCELLANEOUS

Section 9 of 11  

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Medical/Legal Pitfalls

• Because most of these patients have generalized vascular disease, failure to perform a cardiovascular evaluation before surgery could be a medicolegal pitfall.

MULTIMEDIA

Section 10 of 11  

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Media file 1:  The superior mesenteric artery and inferior mesenteric artery share collateral circulation near the splenic flexure of the colon (shown in red). When dilated, this vessel is termed the meandering mesenteric artery (see Media file 4). As seen on an angiogram, this is a sign of chronic mesenteric ischemia.
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Media file 2:  The pancreaticoduodenal arcades (shown in red) are collateral pathways between the celiac artery and the superior mesenteric artery.
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Media file 3:  A lateral aortogram shows abrupt cutoffs at the origin of the visceral vessels and a tapered occlusion of the distal aorta. Because these vessels originate from the anterior surface of the aorta, stenoses and occlusions are not observed clearly on standard anteroposterior views.
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Media file 4:  This arteriogram illustrates a meandering mesenteric artery. This dilated tortuous vessel corresponds to the small collateral near the splenic flexure seen in Media file 1. The appearance of a meandering mesenteric artery such as this one supports the diagnosis of chronic mesenteric ischemia.
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Media type:  X-RAY

Media file 5:  This operative photograph shows a completed retrograde bypass to the superior mesenteric artery using ePTFE graft material. Photograph courtesy of Jamal Hoballah, MD, University of Iowa College of Medicine.
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Media file 6:  The celiac artery is exposed at its origin in preparation for antegrade bypass.
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Media file 7:  The superior mesenteric artery and several branches are exposed for antegrade bypass.
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Media file 8:  This photo shows an antegrade bypass from the aorta to the superior mesenteric artery and the celiac artery (superior mesenteric artery anastomosis is shown) using a Dacron graft.
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Media type:  Photo

Media file 9:  This diagram shows the possible incision for a trapdoor aortotomy. Plaque at the orifices of the visceral vessels is removed after the trapdoor incision is lifted. When a satisfactory endarterectomy has been achieved, the trapdoor is sutured shut.
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Media file 10:  This completion duplex ultrasound study shows excellent flow at the distal anastomosis.
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Media file 11:  Upper gastrointestinal series (barium swallow) shows an ulcer.
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REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
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• Follow-up
• Miscellaneous
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• References

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• Cognet F, Ben Salem D, Dranssart M, Cercueil JP, Weiller M, Tatou E, et al. Chronic mesenteric ischemia: imaging and percutaneous treatment. Radiographics. Jul-Aug 2002;22(4):863-79; discussion 879-80. [Medline].
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• Maspes F, Mazzetti di Pietralata G, Gandini R, Innocenzi L, Lupattelli L, Barzi F, et al. Percutaneous transluminal angioplasty in the treatment of chronic mesenteric ischemia: results and 3 years of follow-up in 23 patients. Abdom Imaging. Jul-Aug 1998;23(4):358-63. [Medline].
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• Park WM, Cherry KJ Jr, Chua HK, Clark RC, Jenkins G, Harmsen WS, et al. Current results of open revascularization for chronic mesenteric ischemia: a standard for comparison. J Vasc Surg. May 2002;35(5):853-9. [Medline].
• Pereles FS, Baskaran V. Abdominal magnetic resonance angiography: principles and practical applications. Top Magn Reson Imaging. Oct 2001;12(5):317-26. [Medline].
• Reilly LM, Ammar AD, Stoney RJ, Ehrenfeld WK. Late results following operative repair for celiac artery compression syndrome. J Vasc Surg. Jan 1985;2(1):79-91. [Medline].
• van Bockel JH, Geelkerken RH, Wasser MN. Chronic splanchnic ischaemia. Best Pract Res Clin Gastroenterol. Feb 2001;15(1):99-119. [Medline].
• van Wanroij JL, van Petersen AS, Huisman AB, Mensink PB, Gerrits DG, Kolkman JJ, et al. Endovascular treatment of chronic splanchnic syndrome. Eur J Vasc Endovasc Surg. Aug 2004;28(2):193-200. [Medline].
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• Wolf YG, Verstandig A, Sasson T, Eidelman L, Anner H, Berlatzky Y. Mesenteric bypass for chronic mesenteric ischaemia. Cardiovasc Surg. Feb 1998;6(1):34-41. [Medline].
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Article Last Updated: Sep 6, 2007