title: Median arcuate ligament syndrome: vascular surgical therapy and follow-up of 18 patients.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Vascular Surgery and Kidney Transplantation, University Hospital, Heinrich-Heine-University, Düsseldorf, Germany. grotemeyer@uni-duesseldorf.de
authors.forename: Dirk
authors.initials: D
authors.lastname: Grotemeyer
authors.forename: Mansur
authors.initials: M
authors.lastname: Duran
authors.forename: Franziska
authors.initials: F
authors.lastname: Iskandar
authors.forename: Dirk
authors.initials: D
authors.lastname: Blondin
authors.forename: Kim
authors.initials: K
authors.lastname: Nguyen
authors.forename: Wilhelm
authors.initials: W
authors.lastname: Sandmann
mesh_terms: D000328:Adult; D000368:Aged; D001157:Arterial Occlusive Diseases / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D002445:Celiac Artery; D019299:Decompression, Surgical; D003964:Diaphragm; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008022:Ligaments; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013577:Syndrome; D016896:Treatment Outcome; D055815:Young Adult