Annals of Vascular Surgery
Volume 23, Issue 6 , Pages 778-784, November 2009

Management of Median Arcuate Ligament Syndrome: A New Paradigm

  • Andrew J. Duffy

      Affiliations

    • Yale School of Medicine, New Haven, CT
    • Corresponding Author InformationCorrespondence to: Andrew J. Duffy, MD, Section of Gastrointestinal Surgery, Yale School of Medicine, 40 Temple Street, Suite 7B, New Haven, CT 06510, USA
    • ,
  • Lucian Panait

      Affiliations

    • Saint Mary's Hospital, Waterbury, CT
    • ,
  • Dan Eisenberg

      Affiliations

    • Yale School of Medicine, New Haven, CT
    • ,
  • Robert L. Bell

      Affiliations

    • Yale School of Medicine, New Haven, CT
    • ,
  • Kurt E. Roberts

      Affiliations

    • Yale School of Medicine, New Haven, CT
    • ,
  • Bauer Sumpio

      Affiliations

    • Yale School of Medicine, New Haven, CT

published online 06 January 2009.

Article Outline

Median arcuate ligament (MAL) syndrome is an anatomic and clinical entity characterized by extrinsic compression on the celiac axis, which leads to postprandial epigastric pain, vomiting, and weight loss. Although characterized a few decades ago, the existence of this syndrome is still challenged by several authors. We reviewed the management of MAL syndrome, with special emphasis on the minimally invasive approaches. We also report the first case of successful combination of minimally invasive surgery and endovascular therapy in the treatment of this syndrome. A PubMed search was carried out to identify articles in English from 1963 to 2008 using the keywords “median arcuate ligament syndrome” and “celiac artery compression syndrome.” Additional articles were identified by a manual search of the references from the key articles. All clinical and experimental studies that contained material applicable to the topic were considered. Classic treatment of the condition is represented by open MAL release. However, permanent changes in the celiac artery wall lead to poor long-term outcomes, and associated complex vascular procedures have been employed. Laparoscopic treatment of MAL syndrome was reported in five cases. All patients had resolution of symptoms, but long-term follow-up is not available. Laparoscopic release of arcuate ligament syndrome with intraoperative duplex ultrasound may be used in patients with symptoms suggestive of the diagnosis. In patients with persistent celiac flow abnormalities noted on duplex ultrasound or postoperative imaging, celiac angioplasty and stenting are advocated. If this option is not available or does not relieve symptoms, vascular reconstruction should be employed.

 

Median arcuate ligament (MAL) syndrome (also known as celiac artery compression syndrome or Dunbar's syndrome) is an anatomic and clinical entity characterized by extrinsic compression on the celiac axis, which leads to postprandial epigastric pain, nausea or vomiting, and weight loss. The compression is believed to be caused by the MAL in patients with abnormally low insertion of the diaphragm or abnormally high origin of the celiac artery from the aorta. Fibers of the celiac ganglion may also contribute to the compression. Although characterized several decades ago, the existence of this syndrome is still challenged by several authors.

One of the first anatomic descriptions dates back to 1917, when during cadaveric dissections the origin of the celiac artery was noted to be sometimes overlapped by the diaphragm.1 Subsequent anatomic studies described constrictive effects on the celiac artery by the diaphragm fibers. Harjola2 was the first to report the clinical significance of the celiac artery compression in vivo. His 1963 article describes a 57-year-old male with postprandial abdominal pain and the presence of an epigastric bruit who was found to have compression of the celiac artery by a fibrotic celiac ganglion. Operative removal of the ganglion resulted in the disappearance of bruit and resolution of the symptoms. In 1967 Dunbar et al.3 published a case series in which similar symptoms were due to extrinsic compression of the celiac artery by fibers of the MAL. Operative division of these fibers resulted in clinical improvement.

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Case Report 

Our patient was a 43-year-old female who was evaluated by her primary care physician and a gastroenterologist for recurrent episodes of severe abdominal pain over the course of 3 months. The pain was postprandial, and the patient had an associated 20 lb weight loss during this period. A series of diagnostic studies were conducted, looking for more common causes of abdominal pain. These included abdominal ultrasound, computed tomographic (CT) scan of the abdomen, upper endoscopy, and hepatic 2,6-dimethyliminodiacetic acid (HIDA) scan, which were all within normal limits. Subsequently, a vascular surgery consultation was requested for identification of possible causes of mesenteric ischemia.

The new studies included magnetic resonance angiography (MRA) of the abdomen, which showed some extrinsic compression on the celiac trunk, just distal to its take-off from the aorta (Fig. 1). In order to define this further, the patient underwent aortic angiography with pressure gradient measurement across the celiac trunk (Fig. 2). There was a 20 mm Hg difference in expiration between the systolic blood pressure in the aorta at the level of the celiac artery and the celiac artery itself. A diagnosis of MAL syndrome was made based on the patient's history and clinical findings, the radiological studies with pressure measurement, and the absence of other pathology.

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  • Fig. 1 

    MRA of the abdomen, showing stenosis of the celiac artery distal to its take-off from the aorta. Ao, aorta; CA, celiac artery. Arrow indicates level of stenosis.

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  • Fig. 2 

    Lateral aortic angiogram showing celiac artery stenosis with poststenotic dilation. The superior mesenteric artery is of normal caliber. Ao, aorta; CA, celiac artery; SMA, superior mesenteric artery. Arrow indicates level of stenosis.

The patient was offered and consented to laparoscopic release of MAL syndrome. The procedure was performed under general anesthesia. Five 5 mm ports were inserted in a similar manner to a Nissen fundoplication. The right diaphragmatic crus was identified and divided until the anterior surface of the aorta was visualized. The dissection was continued inferiorly along the aorta until the origin of the celiac trunk was identified. The fibrous bands of the MAL and the elements of the celiac plexus were divided circumferentially (Fig. 3). The artery was completely skeletonized, releasing the extrinsic impingement of the fibers on the vessel (Fig. 4).

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  • Fig. 3 

    Intraoperative image showing the divided fibers of the MAL and the three terminal branches of the celiac artery. Ao, aorta; CHA, common hepatic artery; LGA, left gastric artery; SA, splenic artery; MAL, median arcuate ligament.

  • View full-size image.
  • Fig. 4 

    Intraoperative image showing the origin of the celiac artery freed from the diaphragmatic fiber compression. CA, celiac artery; CHA, common hepatic artery; LGA, left gastric artery.

The operative time was 218 min, and the blood loss was minimal. The patient was discharged home on the first postoperative day. She was able to tolerate food at this time and was symptom-free.

At 4-week follow-up the patient had partial recurrence of the abdominal pain. An aortic angiogram was performed and revealed residual mild proximal celiac artery narrowing. Pressure gradient measurement demonstrated a significant decrease in the blood pressure in the distal celiac artery compared with the proximal celiac artery and aorta. The lesion was treated with balloon angioplasty without stenting. At 10-month follow-up the patient had complete resolution of symptoms and regained weight.

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Discussion 

The MAL of the diaphragm is formed by fibrous bands connecting the left and right crus and bordering the aortic hiatus anteriorly. Its shape, consistency, and location vary in different individuals; it sometimes compresses the superior aspect of the celiac artery, especially when this originates high from the aorta. In vivo studies have demonstrated variations in the degree of compression with the breathing pattern, related to an inferior displacement of the aorta and an anterior displacement of the MAL on inspiration.4 The origins of the celiac axis and MAL therefore move apart on inspiration and nearer on expiration. Histologically, sustained compression on the celiac artery may lead to a series of changes in all vascular layers, including intimal hyperplasia, proliferation of elastic fibers in the media, and disorganization of the adventitia.5 This explains why operative procedures, including release of the MAL, do not relieve symptoms in all cases and further interventions like angioplasty or splanchnic revascularization may be needed.

The clinical presentation in MAL syndrome varies, and the diagnosis is often one of exclusion. Most patients are young females, with a thin body habitus. The pain is located in the upper abdomen and worsens after meals or exercise or when leaning forward. Relief may be obtained in the knee–chest position. The mechanism of pain is not completely understood. Different theories exist, but the most accepted one is that increased blood demand through a compressed celiac artery leads to foregut ischemia and subsequent pain. Another theory is that midgut ischemia causes the pain through a steal syndrome; blood from the superior mesenteric artery territory is diverted through collaterals to compensate for inefficient delivery through a stenosed celiac artery. Other, less accepted theories suggest that the pain is caused directly by chronic irritation of the celiac plexus or indirectly by overstimulation of the celiac plexus with subsequent splanchnic vasoconstriction and ischemia.6, 7

In addition to pain, patients may experience nausea, vomiting, bloating, and diarrhea. Weight loss is also a common feature and is often related to “food fear,” or fear of pain triggered by eating. The physical examination may elicit epigastric tenderness or may detect a thrill or bruit in the same area.

Diagnosis of MAL syndrome is often difficult. Other causes of chronic abdominal pain must be ruled out, and patients usually undergo extensive work-ups, including abdominal ultrasound, CT scan, and endoscopy. If these diagnostic studies and procedures fail to find any other pathology and MAL syndrome is suspected based on the clinical findings, the work-up can be tailored toward this entity. Duplex abdominal ultrasound may be used as an initial screening test.8 Findings that suggest MAL syndrome are elevated peak systemic velocities on expiration, which normalize with inspiration9 and standing erect;10 abnormal images of celiac artery and superior mesenteric artery origins; and reverse flow in the hepatic artery.6 However, the diagnosis must be confirmed by other imaging modalities.

Lateral aortic angiography is the gold standard for the diagnosis of MAL syndrome. Typical features include asymmetric focal narrowing of the proximal celiac axis, with poststenotic dilation. The narrowing is variable within the breathing cycle, being accentuated on expiration and relieved on inspiration. This is secondary to a physiological anterior and inferior displacement of the aorta (and therefore the celiac artery origin) during inspiration. Anterior–posterior views may show increased collateral vessels in the celiac artery distribution territory.

Other, less invasive imagining modalities like CT angiography and MRA also may be helpful. CT angiography, especially if combined with three-dimensional reconstruction, can demonstrate all characteristic aspects seen with conventional angiography. In addition, CT angiography offers information about the relationship of the celiac artery with the diaphragm and allows visualization of the compressed artery from various angles.11, 12 However, the study is not dynamic and does not offer information about changes during the breathing cycle. The same limitation is true about MRA.

Gastric exercise tonometry is a less conventional diagnostic approach, which has been used to identify gastric ischemia as a result of MAL syndrome by measuring the intraluminal gastric pCO2. In some limited studies, this modality yielded similar results to angiography and was used for diagnosis and follow-up after surgical treatment of MAL syndrome.13, 14

Preoperative percutaneous celiac ganglion block was suggested for identification of patients who would benefit from MAL release.15, 16 The rationale for this modality is based on the theory that MAL syndrome symptoms are related to compression and stimulation of the celiac ganglion and plexus. Temporary celiac ganglion block relieves abdominal pain in some patients and may help in selecting surgical candidates for MAL release.

Treatment of MAL syndrome is aimed at restoring normal blood flow in the celiac axis and eliminating neural irritation produced by the celiac ganglion fibers. The first published cases achieved this by removing the constrictive fibers of the celiac plexus2 and dividing the MAL.3 Larger series followed, but the long-term results did not prove satisfactory. Some authors even questioned the existence of MAL syndrome as a clinical entity and suggested that the same results would be obtained with nonoperative interventions.17

One of the largest series of patients treated was published in 1985 by Reilly et al.18 The authors defined patient criteria that correlate with a successful operation, including the presence of postprandial pain, age between 40 and 60, female sex, weight loss >20 lb, and an angiogram with poststenotic dilatation or increased collateral flow. Their long-term follow-up results suggested that MAL release alone is not sufficient in treating the condition and additional therapy is needed. This was represented by either graduated celiac dilatation or celiac reconstruction, with no significant differences in outcome between the two. Different types of vascular reconstruction include (1) patch angioplasty of the celiac artery, (2) aortoceliac bypass with saphenous vein or Dacron graft, and (3) aortic reimplantation of the celiac artery. Various treatment algorithms were recommended. An earlier one was based on the intraoperative findings at the time of laparotomy: If the celiac artery appeared stenosed or a thrill was palpated, the authors recommended vascular reconstruction in addition to releasing the MAL.19

Currently, more objective ways of assessing the celiac blood flow before and after MAL release are available: either measurement of pressure gradient in the aorta and celiac (or splenic) artery via angiography probe or intraoperative Doppler ultrasound use for velocity measurement in the celiac artery. We consider a pressure gradient of 10 mm Hg between the aorta and celiac artery to be significant for the presence of celiac artery stenosis. If the blood flow seems compromised by either one of these methods, the patient may benefit from endoluminal therapy like balloon angioplasty with or without stent deployment in the celiac artery, before being subjected to an operation with increased morbidity like vascular reconstruction.

Angioplasty alone was attempted by some authors but did not prove successful in the treatment of MAL syndrome. Cina and Safar20 reported a case in which percutaneous transluminal angioplasty was attempted for the treatment of MAL syndrome. The patient's symptoms did not improve, and subsequent MAL release and vascular reconstruction were performed. A similar outcome was encountered in the reports of Delis et al.21 and Wang et al.,22 in which multiple attempts at endovascular therapy with or without stenting were unsuccessful in the treatment of MAL syndrome. Finally, Matsumoto et al.23 published a case series in which MAL compression was involved in both immediate and delayed failure of the transluminal approach. The poor success rate of endovascular therapy alone in the treatment of MAL syndrome is believed to be related to the sustained compression of the diaphragmatic fibers on the celiac artery, with extrinsic luminal narrowing, leading to permanent changes in the vessel wall.19

This report represents the first description of minimally invasive combined modality therapy (laparoscopic surgery and endovascular therapy) in the treatment of MAL syndrome. This is an effective therapeutic method, with short hospital stay and minimal morbidity.

Laparoscopy for the treatment of MAL syndrome was first employed in 2000.24 Four more case reports followed, as described in Table I.25, 26, 27, 28 There is heterogeneity in the studies used for diagnosis and follow-up: All authors performed laparoscopic release of MAL, and some confirmed improved flow in the celiac artery with either intraoperative ultrasound or postoperative aortography, CT angiogram, or Doppler ultrasound. Follow-up was short in all cases, and all patients reported resolution of symptoms.

Table I. Laparoscopy for the treatment of MAL syndrome
SourcePatient age, sexDiagnosisAssociated modalitiesPostoperative studiesFollow-upOutcome
Roayaie et al.2443, FMRAIntraoperative ultrasoundNone3 months, MRAResolution
Dordoni et al.2745, FMRA, aortography, Doppler ultrasoundNoneAortography6 months, Doppler ultrasound, MRAResolution
Carbonell et al.2622, MAortographyIntraoperative ultrasoundCT angiography7 monthsResolution
Baldassarre et al.2538, MCT angiographyNoneNone3 months, CT angiographyResolution
Jaik et al.2823, FDoppler ultrasound, CT angiographyRoboticsDoppler ultrasound6 weeksResolution
Duffy et al. (present report)43, FMRA, aortographyAngioplastyAortography10 monthsResolution

Based on the literature and our experience, we believe that laparoscopy is a useful tool in the treatment of MAL syndrome, once adequate diagnosis is established. The operation should divide the fibrous bands of the MAL, including the celiac ganglion fibers around the celiac artery, leaving the celiac trunk completely free circumferentially. Adequate exposure is achieved by division of the gastrohepatic ligament and identification of the celiac artery branches. The posterior peritoneum is opened and the two diaphragmatic crura are exposed. The fibrous bands of the MAL of the diaphragm are divided longitudinally, allowing visualization of the anterior surface of the aorta and exposure of the celiac trunk. We prefer to apply vessel loops around the celiac artery branches in order to facilitate distal traction and circumferential exposure of the trunk. Direct grasping of the celiac artery or its branches should be avoided. These are friable vessels, and direct manipulation could lead to bleeding or vessel wall injury. Careful dissection is performed at this level in order to remove all fibrous and ganglionic tissue surrounding the artery. These fibers can be tightly attached to the artery and may bleed if ripped. We perform this dissection with very careful and judicious use of the hook cautery on a 20-Watt setting. Complete circumferential dissection of the celiac artery and release of all the impinging fibers from its origin at the aorta until division of the major branches is the most tedious and critical portion of the procedure.

However, laparoscopy alone may not be sufficient if permanent changes in the celiac artery wall are present. Therefore, if adequate flow is not confirmed with intraoperative ultrasound or postoperative imaging studies and the patient is symptomatic, he or she may benefit from adjunct intraluminal therapy. For patients with ongoing symptoms and hemodynamic abnormalities, vascular reconstruction can be used if the above treatments fail. Figure 5 outlines an algorithm for diagnosis and management of MAL syndrome.

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References 

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PII: S0890-5096(08)00422-6

doi:10.1016/j.avsg.2008.11.005

Annals of Vascular Surgery
Volume 23, Issue 6 , Pages 778-784, November 2009

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