Median arcuate ligament syndrome: Vascular surgical therapy and follow-up of 18 patients

Department of Vascular Surgery and Kidney Transplantation, University Hospital, Heinrich-Heine-University, Düsseldorf, Germany.
Langenbeck s Archives of Surgery (Impact Factor: 2.19). 07/2009; 394(6):1085-92. DOI: 10.1007/s00423-009-0509-5
Source: PubMed


The median arcuate ligament syndrome (MALS) or celiac artery compression syndrome is a rare vascular disorder caused by an extrinsic compression of the celiac artery from the median arcuate ligament, prominent fibrous bands, and ganglionic periaortic tissue. Clinical symptoms are postprandial abdominal pain, nausea, vomiting, unintentional weight loss, and sometimes, abdominal pain during body exercise caused by an intermittent visceral ischemia. The aim of this study was to evaluate the operative management of patients with MALS in our institution, especially in consideration of various vascular reconstructive techniques.
Between June 2000 and January 2009, a total of 341 patients were treated in our department for vascular pathologies of the visceral arteries (225 chronic visceral ischaemia, 84 acute visceral ischaemia, and 14 visceral artery aneurysms). In a retrospective study of 18 patients with MALS, the records, clinical symptoms, diagnostic evaluation, and surgical procedures were compiled. This was completed by a reassessment for a follow-up.
A MALS was diagnosed in 15 female (83.3%) and three male (16.7%) patients. The mean patient age was 46.2 years (range 20-68 years). The diagnosis of MALS was based on a radiological analysis in all patients by a digitally subtracted angiogram, but duplex ultrasound was used lately more frequently to study the influence of respiration on the stenotic degree of the celiac trunk. All 18 patients were treated with open surgery in an elective situation. Due to the local and specific pathology of the celiac trunk with a fixed stricture or stenosis, out of 18 cases beside decompression, 11 (primary, seven; secondary, four patients) further procedures were performed on the celiac artery (aorto-celiac vein interposition n = 6, aorto-hepatic vein interposition n = 1, resection of the celiac artery and end-to-end anastomosis n = 2, patchplasty of the celiac artery with vein n = 1, and transaortic removal of a stent of the celiac artery n = 1) Follow-up was obtained in 15 patients (83.33%) with a mean duration after surgery of about three and a half years (40.68 months, range from 2 to 102 months). Eleven of the 15 patients (73.33%) were completely free of abdominal symptoms, and nine of them had gained between 3 and 10 kg in weight after surgery. The weight of two patients remained stable. Of the 11 patients with a successful outcome in the follow-up, six of them had undergone decompression solely. In the other five patients, vascular co-procedures on the celiac trunk had been performed.
The MALS is a rare vascular disorder caused by an extrinsic compression of the celiac artery and induces upper abdominal, mostly, postprandial pain. A definite diagnosis of MALS can be achieved by lateral aortography of the visceral aorta and its branches during inspiration and expiration. Open surgical therapy is a safe and reliable procedure with no mortality and low morbidity. As to the local and specific pathology of the celiac trunk after decompression with fixed stricture or stenosis, further vascular procedures are necessary. The long-time follow-up seemed adequate. The laparoscopic approach reduces the procedure of decompression only, something which seemed inadequate for most cases. Endovascular treatment with percutaneous transluminal angioplasty and insertion of a stent does not solve the underlying problem of extrinsic compression of the celiac trunk and often requires open procedures during the long-term course. Due to the low incidence of MALS, no guidelines will do justice to all the patients sufficiently, and the choice of treatment must depend on the specific clinical situation for each patient.

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    • "The categorizations for this condition are (1) intrinsic stenosis (concentric type) due to atherosclerotic disease which is the most common cause, being the cause for the stenosis in 87% of cases in western countries, (2) extrinsic stenosis (eccentric type) due to compression by the medial arcuate ligament or periarterial ganglionic tissue, and (3) other rare causes including neoplastic disease, acute or chronic dissection, external compression by an inflamed pancreas, or vascular injury [7]. Although it rarely leads to symptomatology, the management of symptomatic patients includes (1) endovascular treatment with balloon dilatation with or without stenting [12, 13], (2) arterial bypass grafting or arterial reimplantation [14, 15], and (3) medial arcuate ligament and periarterial tissue transection in cases of extrinsic pressure [16]. "
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    ABSTRACT: Duodenal metastasis from renal cell carcinoma is rare, and even rarer is a massive gastrointestinal bleeding from such tumours. Coeliac occlusive disease, although rarely symptomatic, can lead to ischaemic changes with anastomotic dehiscence and leaks when a patient undergoes pancreatoduodenectomy. A 41-year-old man with known metastasis to the adrenal glands and the second part of the duodenum close to the ampulla of Vater from clear cell renal cell carcinoma was admitted to our department due to massive gastrointestinal bleeding from the duodenal metastasis. Endoscopic control of the bleed was not possible, while the bleeding vessel embolization was able to control the haemorrhage only temporarily. An angiography during the embolization demonstrated the presence of stenosis of the coeliac artery and also hypertrophic inferior pancreaticoduodenal arteries supplying the proper hepatic artery via the gastroduodenal artery (GDA). The patient underwent emergency pancreatoduodenectomy with preservation of the gastroduodenal artery. The patient had an uneventful recovery and did not experience further bleeding. Also the blood flow to the liver was compromised as shown by the normal liver function tests (LFTs) postoperatively. To the best of our knowledge, this is the first report of a preservation of the GDA during an emergency pancreatoduodenectomy.
    Full-text · Article · Aug 2014
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    • "A laparoscopic approach may also be used to achieve celiac artery decompression. Endovascular methods such as percutaneous transluminal angioplasty have been used in patients who have failed open and/or laparoscopic intervention (6, 13, 14). "
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    ABSTRACT: Median arcuate ligament syndrome is an anatomic and clinical entity characterized by dynamic compression of the proximal celiac artery by the median arcuate ligament, which leads to postprandial epigastric pain, vomiting, and weight loss. These symptoms are usually nonspecific and are easily misdiagnosed as functional dyspepsia, peptic ulcer disease, or gastropathy. In this report, we presented a 72-year-old male patient with celiac artery compression syndrome causing recurrent abdominal pain associated with gastric ulcer and iron deficiency anemia. This association is relatively uncommon and therefore not well determined. In addition, we reported the CT angiography findings and three-dimensional reconstructions of this rare case.
    Full-text · Article · Jul 2014 · Korean journal of radiology: official journal of the Korean Radiological Society
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    • "Moreover, some authors suggested additional arterial reconstruction of the entrapped celiac artery by primary reanastomosis, interposition grafting, or bypass to offer better outcome [25] [26]. The average rate of being symptom-free is around 70–80% after successful surgery based on long-term followup [26]. Although the success rates of open surgery on vascular patency are excellent, "
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    ABSTRACT: Celiac artery compression syndrome (CACS) or median arcuate ligament (MAL) syndrome is a rare vascular disease. The clinical manifestations of CACS include the triad of postprandial pain, vomiting, and weight loss. The pathogenesis of CACS is the external compression of celiac artery by the MAL or celiac ganglion. Moreover, some authors also reported the compression with different etiologies, such as neoplasms of pancreatic head, adjacent duodenal carcinoma, vascular aneurysms, aortic dissection, or sarcoidosis. In the literature, most cases of CACS were reported from Western countries. In contrast, this disease was seldom reported in Oriental countries or regions, including Taiwan. Superior mesenteric artery syndrome (SMAS) is also a rare disease characterized by compression of the third portion of the duodenum by the SMA. The clinical features of SMAS are postprandial pain, vomiting, and weight loss. To date, there are no guidelines to ensure the proper treatment of patients with CACS because of its low incidence. Thus, tailored therapy for patients with CACS remains a challenge as well as the prediction of clinical response and prognosis. The aim of our present study was to investigate the clinical features, the association with SMAS, treatments, and outcomes of patients with CACS in a single institution in Taiwan.
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