Median arcuate ligament syndrome: vascular surgical therapy and follow-up of 18 patients.
ABSTRACT 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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ABSTRACT: Uterine leiomyomas are commonly reported to cause deep venous thrombosis and rarely arterial compression. A 48-year-old woman was transferred to our institution with acute right lower limb ischemia and tissue loss. She underwent urgent iliac thrombectomy and was subsequently found to have right common iliac artery compression by a large uterine leiomyoma. She underwent successful resection of the tumor followed by endovascular iliac stent placement. This case emphasizes the importance of preoperative imaging when possible in the setting of acute arterial ischemia to evaluate for sources of extrinsic compression. Management requires correction of the etiology of extrinsic compression.Vascular and Endovascular Surgery 02/2012; 46(1):80-4. DOI:10.1177/1538574411429866 · 0.77 Impact Factor
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ABSTRACT: Median arcuate ligament syndrome (MALS) is a rare disorder defined by compression and narrowing of the celiac artery by the median arcuate ligament. The increased blood flow through the pancreaticoduodenal arcade can lead to the aneurysmal formation withing the vessel. We report three cases of pancreaticoduodenal aneurysms (PDAA) in patients with MALS whose aneurysms were occluded, but celiac artery revascularization was not performed. CASE 1: Asymptomatic 61 year-old female with no past medical history was referred to vascular surgery for evaluation of a PDAA incidentally found on CT scan. The patient was taken for laparoscopic division of the median arcuate ligament, however, the release was incomplete. This was followed by endovascular coil embolization of the PDAA without celiac revascularization. The patient tolerated the procedure well with no complications and the one year follow-up shows no signs of aneurysm recurrence. CASE 2: 61 year-old male found to have an incidental PDAA on CT scan. The patient was taken for coil embolization without median arcuate ligament release. At the one year follow-up the patient continues to be asymptomatic with no recurrence. CASE 3: 56 year-old male who presented with a ruptured PDAA. He was taken immediately for coil embolization of the ruptured aneurysm. Post-operatively, the patient was identified to have MALS on CT scan. Due to his asymptomatic history and benign physical examination before the rupture, he was not taken for a ligament release or celiac revascularization. He continues to be asymptomatic at his follow-up.Annals of Vascular Surgery 06/2014; 29(1). DOI:10.1016/j.avsg.2014.05.020 · 1.03 Impact Factor