CELIAC ARTERY COMPRESSION SYNDROMES

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In 1963 Harjola 15 described a case of chronic abdominal pain in a young woman that he attributed to mesenteric ischemia, which in turn was caused by extrinsic compression of the celiac artery (CA). The CA compression syndrome (also known as median arcuate ligament [MAL] compression syndrome, celiac band syndrome, and Dunbar's syndrome) has generated debate among surgeons ever since. All agree that if it does exist, the syndrome is rare, and thus knowledge about it will continue to depend on case reports and small institutional series. Even those who are convinced that extrinsic compression of the celiac axis by ligamentous and neural structures can be a cause of abdominal pain admit that the pathophysiology of the pain is obscure because stenosis of a single mesenteric trunk, it is commonly held, should not result in visceral ischemia. No clear diagnostic criteria have been developed, and no imaging modality is both sensitive and specific for predicting response to treatment. Nevertheless, over the last 30 years many surgeons have cared for patients who fit the unusual clinical profile of a young patient with chronic mesenteric ischemia and have successfully relieved long-standing pain with a safe and relatively simple operation. Unfortunately, because of the continuing disagreement that characterizes the literature of CA compression syndrome, effective treatment is probably denied to some patients who indeed suffer from it. The bias of the author of this article, based on personal experience of successful treatment of several patients, is that a strong case can be made for the existence of the syndrome. Accordingly, this review addresses the problems of pathophysiology and diagnosis that continue to create doubt.

Section snippets

HISTORY

Harjola's report 15 of the first operative case in which the celiac axis was released from compression by division of a “fibrotic celiac ganglion” was followed by a number of similar reports in the mid to late 1960s. In a 1965 review of their experience with several varieties of chronic mesenteric ischemia, Dunbar and his colleagues 2 at Ohio State University reported on 15 patients in whom division of the median arcuate ligament (not the celiac ganglion), releasing the CA from extrinsic

LATER SERIES

In 1974, Evans, 10 updating the Ohio State experience, largely fulfilled Szilagyi's prediction when he reported disappointing late follow-up on what was at the time the largest number of patients undergoing operation for the syndrome at one institution. After 3 to 11 years, 50% of 44 patients had persistent symptoms, which compared unfavorably with a smaller group with the same diagnosis treated nonsurgically: 9 of 12 of these were pain free and had later been given additional diagnoses of

CLINICAL HISTORY

Celiac compression syndromes affect females more often than males, in a ratio of nearly 4 to 1. Age at presentation is typically between 20 and 50, but extremes of age from 13 to 78 have been reported. An asthenic body habitus has been described by a minority of authors. The cardinal symptom is chronic abdominal pain, usually gradual in onset and beginning months or even years prior to presentation. Few authors have noted in their patients a reliable pattern of pain that is recognizable to

PHYSICAL EXAMINATION

Several authors have observed that a slender physique with a narrow subcostal angle25 is commonly present, but this is certainly not essential to the diagnosis. The hallmark of the physical examination is an epigastric bruit, which in most cases, but not all, becomes louder during expiration. Unfortunately, like the cardinal symptom of pain, this too is a nonspecific finding because examinations of normal, symptom-free individuals detect epigastric bruits in 6.5% to 30%.17 Whether variation

DIAGNOSTIC TESTS

CA compression syndrome is, in practice, very much a “diagnosis of exclusion.” Accordingly, the entire lengthy differential diagnosis of chronic abdominal pain has usually been considered and thoroughly investigated before this diagnosis is entertained. This is entirely appropriate. The aforementioned incidence of CA compression in normal, asymptomatic individuals, indicated both by epigastric bruit and by arteriographic demonstration of CA impingement, is high. It only makes sense (and has

CASE SELECTION

The most challenging aspect of CA compression syndrome is correctly identifying the patient who will benefit from surgical correction. The cardinal symptom (pain), the critical physical finding (bruit), and the “definitive” diagnostic finding (CA compression) are each to some degree nonspecific, so the “chance concurrence…of these phenomena from time to time, without…cause and effect relationship” might be expected. Of course, the same can be said for the concurrence of abdominal pain and

SURGICAL THERAPY

Clearly, the most difficult step in the surgical treatment of CA compression syndrome is the decision whether to operate. A frank preoperative discussion with the patient that acknowledges the complexity of this decision is always worthwhile. The typical young, otherwise healthy patient with debilitating pain is usually more than willing to accept a possible lack of efficacy in exchange for the greater possibility of pain relief, especially because a laparotomy offers additional diagnostic

ANATOMIC BASIS FOR CA COMPRESSION

In development, the CA originates in the cervical region and descends caudally. In 85% of cadaver dissections its anterior origin from the aorta occurs between the upper third of the eleventh thoracic vertebra and the upper third of the twelfth.4 In general, a more proximal CA origin occurs in females than in males, which is consistent with the female predominance of the CA compression syndrome. A wide variation also exists in the length (8 to 40 mm) and caliber (7 to 20 mm) of the CA.28

A 1917

SUMMARY

Compression of the visceral arteries can produce true mesenteric ischemia, but the syndrome is rare. The syndrome is caused by unfavorable anatomic relationships at the aortic hiatus among the CA, the SMA, and overlying structures, particularly the diaphragmatic crura. These anatomic relationships, in contrast to the syndrome they sometimes produce, are relatively common, which makes the detection of CA compression only a prerequisite to the diagnosis of the clinical entity. The diagnosis of CA

References (41)

  • D.A. Bull et al.

    Hepatic ischemia, caused by celiac axis compression, complicating pancreaticoduodenectomy

    Ann Surg

    (1993)
  • E.W. Cauldwell et al.

    The visceral branches of the abdominal aorta: Topographical relationships

    Am J Anat

    (1943)
  • J.H. Curl et al.

    Median arcuate ligament compression of the celiac and superior mesenteric arteries

    Ann Surg

    (1971)
  • M.E. Debakey et al.

    The “borrowing-lending” hemodynamic phenomenon (hemometakinesia) and its therapeutic application in peripheral vascular disturbances

    Ann Surg

    (1947)
  • J.D. Dunbar et al.

    Compression of the celiac trunk and abdominal angina

    Am J Roentgenol

    (1965)
  • A.J. Edwards et al.

    Coeliac axis compression syndrome

    Proc R Soc Med

    (1969)
  • A.J. Edwards et al.

    Experience with coeliac axis compression syndrome

    Br Med J

    (1970)
  • W.E. Evans

    Long-term evaluation of the celiac band syndrome

    Surgery

    (1974)
  • J.G. Fortner et al.

    Median arcuate ligament obstruction of celiac axis and pancreatic cancer

    Ann Surg

    (1982)
  • R.H. Geelkerken et al.

    Coeliac artery compression syndrome: The effect of decompression

    Br J Surg

    (1990)
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      2020, Journal of Vascular Surgery
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      Chronic compression by the MAL can lead to hyperplastic intimal changes of the celiac artery. Histologic studies have demonstrated an abundance of smooth muscle proliferation, abnormal elastic fibers, and disorganized medial and adventitial layers that may advance to cause complete arterial occlusion in patients with MALS.6 Downstream of the stenosis, poststenotic dilation and celiac artery aneurysms can complicate MALS further.

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    Address reprint requests to Fritz R. Bech, MD Section of Vascular Surgery Dartmouth-Hitchcock Medical Center One Medical Center Drive Lebanon, NH 03756

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