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

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Abstract

Introduction

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.

Patients and 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.

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References

  1. Foertsch T, Koch A, Singer H, Lang W (2007) Celiac trunk compression syndrome requiring surgery in 3 adolescent patients. J Pediatr Surg 42:709–713. doi:10.1016/j.jpedsurg.2006.12.049

    Article  PubMed  Google Scholar 

  2. Lipshutz B (1917) A composite study of the coeliac artery axis artery. Ann Surg 65:159. doi:10.1097/00000658-191702000-00006

    Article  CAS  PubMed  Google Scholar 

  3. Harjola PT (1963) A rare abstruction of the coeliac artery. Report of a case. Ann Chir Gynaecol Fenn 52:547–550

    CAS  PubMed  Google Scholar 

  4. Dunbar JD, Molnar W, Beman FF, Marable SA (1965) Compression of the celiac trunk and abdominal angina. Am J Roentgenol Radium Ther Nucl Med 95:731–744

    CAS  PubMed  Google Scholar 

  5. Akatsu T, Hayashi S, Yamane T, Yoshii H, Kitajima M (2004) Emergency embolization of a ruptured pancreaticoduodenal artery aneurysm associated with the median arcuate ligament syndrome. J Gastroenterol Hepatol 19:482–483. doi:10.1111/j.1440-1746.2004.03385.x

    Article  PubMed  Google Scholar 

  6. Balaban DH, Chen J, Lin Z, Tribble CG, McCallum RW (1997) Median arcuate ligament syndrome: a possible cause of idiopathic gastroparesis. Am J Gastroenterol 92:519–523

    CAS  PubMed  Google Scholar 

  7. Carbonell AM, Kercher KW, Heniford BT, Matthews BD (2005) Multimedia article. Laparoscopic management of median arcuate ligament syndrome. Surg Endosc 19:729. doi:10.1007/s00464-004-6010-x

    Article  CAS  PubMed  Google Scholar 

  8. Cinà CS, Safar H (2002) Successful treatment of recurrent celiac axis compression syndrome. A case report. Panminerva Med 44:69–72

    PubMed  Google Scholar 

  9. Delis KT, Gloviczki P, Altuwaijri M, McKusick MA (2007) Median arcuate ligament syndrome: open celiac artery reconstruction and ligament division after endovascular failure. J Vasc Surg 46:799–802. doi:10.1016/j.jvs.2007.05.049

    Article  PubMed  Google Scholar 

  10. Dordoni L, Tshomba Y, Giacomelli M, Jannello AM, Chiesa R (2002) Celiac artery compression syndrome: successful laparoscopic treatment—a case report. Vasc Endovascular Surg 36:317–321. doi:10.1177/153857440203600411

    Article  CAS  PubMed  Google Scholar 

  11. Faries PL, Narula A, Veith FJ, Pomposelli FB Jr, Marsan BU, LoGerfo FW (2000) The use of gastric tonometry in the assessment of celiac artery compression syndrome. Ann Vasc Surg 14:20–23. doi:10.1007/s100169910004

    Article  CAS  PubMed  Google Scholar 

  12. Kernohan RM, Barros D’Sa AA, Cranley B, Johnston HM (1985) Further evidence supporting the existence of the celiac artery compression syndrome. Arch Surg 120:1072–1076

    CAS  PubMed  Google Scholar 

  13. Kokotsakis JN, Lambidis CD, Lioulias AG, Skouteli ET, Bastounis EA, Livesay JJ (2000) Celiac artery compression syndrome. Cardiovasc Surg 8:219–222. doi:10.1016/S0967-2109(00)00012-0

    Article  CAS  PubMed  Google Scholar 

  14. Kopecky KK, Stine SB, Dalsing MC, Gottlieb K (1997) Median arcuate ligament syndrome with multivessel involvement: diagnosis with spiral CT angiography. Abdom Imaging 22:318–320. doi:10.1007/s002619900199

    Article  CAS  PubMed  Google Scholar 

  15. Roayaie S, Jossart G, Gitlitz D, Lamparello P, Hollier L, Gagner M (2000) Laparoscopic release of celiac artery compression syndrome facilitated by laparoscopic ultrasound scanning to confirm restoration of flow. J Vasc Surg 32:814–817. doi:10.1067/mva.2000.107574

    Article  CAS  PubMed  Google Scholar 

  16. Wang X, Impeduglia T, Dubin Z, Dardik H (2008) Celiac revascularization as a requisite for treating the median arcuate ligament syndrome. Ann Vasc Surg 22:571–574. doi:10.1016/j.avsg.2008.02.007

    Article  PubMed  Google Scholar 

  17. Edwards AJ, Hamilton JD, Nichol WD, Taylor GW, Dawson AM (1970) Experience with coeliac axis compression syndrome. BMJ 7:342–345. doi:10.1136/bmj.1.5692.342

    Article  Google Scholar 

  18. Geelkerken RH, van Bockel JH, de Roos WK, Hermans J (1990) Coeliac artery compression syndrome: the effect of decompression. Br J Surg 77:807–809. doi:10.1002/bjs.1800770728

    Article  CAS  PubMed  Google Scholar 

  19. Gutnik LM (1984) Celiac artery compression syndrome. Am J Med 76:334–336. doi:10.1016/0002-9343(84)90797-6

    Article  CAS  PubMed  Google Scholar 

  20. Holland AJ, Ibach EG (1996) Long-term review of coeliac axis compression syndrome. Ann R Coll Surg Engl 78:470–472

    CAS  PubMed  Google Scholar 

  21. Jamieson CW (1986) Coeliac axis compression syndrome. Br Med J (Clin Res Ed) 293:159–160. doi:10.1136/bmj.293.6540.159

    Article  CAS  Google Scholar 

  22. Loffeld RJ, Overtoom HA, Rauwerda JA (1995) The celiac axis compression syndrome. Report of 5 cases. Digestion 56:534–537. doi:10.1159/000201288

    Article  CAS  PubMed  Google Scholar 

  23. Mihas AA, Laws HL, Jander HP (1977) Surgical treatment of the celiac axis compression syndrome. Am J Surg 133:688–691. doi:10.1016/0002-9610(77)90154-4

    Article  CAS  PubMed  Google Scholar 

  24. Plate G, Eklöf B, Vang J (1981) The celiac compression syndrome: myth or reality? Acta Chir Scand 147:201–203

    CAS  PubMed  Google Scholar 

  25. Takach TJ, Livesay JJ, Reul GJ Jr, Cooley DA (1997) Celiac compression syndrome: tailored therapy based on intraoperative findings. J Am Coll Surg 183:606–610

    Google Scholar 

  26. Trinidad-Hernandez M, Keith P, Habib I, White JV (2006) Reversible gastroparesis: functional documentation of celiac axis compression syndrome and postoperative improvement. Am Surg 72:339–344

    PubMed  Google Scholar 

  27. Watson WC, Sadikali F (1977) Celiac axis compression: experience with 20 patients and a critical appraisal of the syndrome. Ann Intern Med 86:278–284

    CAS  PubMed  Google Scholar 

  28. Williams S, Gillespie P, Little JM (1985) Celiac axis compression syndrome: factors predicting a favorable outcome. Surgery 98:879–887

    CAS  PubMed  Google Scholar 

  29. Wolfman D, Bluth EI, Sossaman J (2003) Median arcuate ligament syndrome. J Ultrasound Med 22:1377–1380

    PubMed  Google Scholar 

  30. Meves M, Beger HG (1973) Coeliac compression syndrome. A contribution on its etiology. Fortschr Geb Rontgenstr Nuklearmed 118:451–454

    Article  CAS  PubMed  Google Scholar 

  31. Beger HG, Meves M, Apitzsch D, Kraas E, Bittner R (1975) Diagnosis and operative treatment of coelic-artery compression. Dtsch Med Wochenschr 100:464–468

    Article  CAS  PubMed  Google Scholar 

  32. Beger HG, Apitzsch D, Meves M (1976) Compression of the celiac trunk. Langenbecks Arch Chir 341:253–269. doi:10.1007/BF01254580

    Article  CAS  PubMed  Google Scholar 

  33. Reilly LM, Ammar AD, Stoney RJ, Ehrenfeld WK (1985) Late results following operative repair for celiac artery compression syndrome. J Vasc Surg 2:79–91. doi:10.1067/mva.1985.avs0020079

    Article  CAS  PubMed  Google Scholar 

  34. Jarry J, Berard X, Ducasse E, Biscay D, Pailler A, Sassoust G, Midy D, Baste JC (2008) Laparoscopic management of median arcuate ligament syndrome. J Mal Vasc 33:30–34. doi:10.1016/j.jmv.2007.11.002

    Article  CAS  PubMed  Google Scholar 

  35. Grotemeyer D, Iskandar F, Voshege M, Blondin D, Pourhassan S, Grabitz K, Sandmann W (2009) Retrograde aortomesenteric loop-bypass behind the left renal pedicle (“French bypass”) in the treatment of acute and chronic mesenteric ischemia. Clinical experiences and long-term follow-up in 27 patients. Zentralbl Chir 134:1–7

    Article  Google Scholar 

  36. Horton KM, Talamini MA, Fishman EK (2005) Median arcuate ligament syndrome: evaluation with CT angiography. Radiographics 25:1177–1182. doi:10.1148/rg.255055001

    Article  PubMed  Google Scholar 

  37. Lee VS, Morgan JN, Tan AG, Pandharipande PV, Krinsky GA, Barker JA, Lo C, Weinreb JC (2003) Celiac artery compression by the median arcuate ligament: a pitfall of end-expiratory MR imaging. Radiology 228:437–442. doi:10.1148/radiol.2282020689

    Article  PubMed  Google Scholar 

  38. Schuler A, Dirks K, Claussnitzer R, Blank W, Braun B (1998) Ligamentumarcuatum syndrome: colordoppler ultrasound diagnosis in abdominal pain of unknown origin in young patients. Ultraschall Med 19:157–163. doi:10.1055/s-2007-1000482

    Article  CAS  PubMed  Google Scholar 

  39. Erden A, Yurdakul M, Cumhur T (1999) Marked increase in flow velocities during deep expiration: a duplex Doppler sign of celiac artery compression syndrome. Cardiovasc Interv Radiol 22:331–332. doi:10.1007/s002709900399

    Article  CAS  Google Scholar 

  40. Walter P (2005) Celiac trunk compression: angiographic phenomenon or cause of ischemic abdominal complaints? Zentralbl Chir 130:227–234. doi:10.1055/s-2005-836587

    Article  CAS  PubMed  Google Scholar 

  41. Mensink PB, van Petersen AS, Kolkman JJ, Otte JA, Huisman AB, Geelkerken RH (2006) Gastric exercise tonometry: the key investigation in patients with suspected celiac artery compression syndrome. J Vasc Surg 44:277–281. doi:10.1016/j.jvs.2006.03.038

    Article  PubMed  Google Scholar 

  42. Gloviczki P, Duncan AA (2007) Treatment of celiac artery compression syndrome: does it really exist? Perspect Vasc Surg Endovasc Ther 19:259–263. doi:10.1177/1531003507305263

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Vascular Surgery and Kidney Transplantation, University Hospital, Heinrich-Heine-University, Düsseldorf, Germany

    Dirk Grotemeyer, Mansur Duran, Franziska Iskandar, Kim Nguyen & Wilhelm Sandmann

  2. Institute of Radiology, University Hospital, Heinrich-Heine-University, Düsseldorf, Germany

    Dirk Blondin

  3. Klinik für Gefäßchirurgie und Nierentransplantation, Universitätsklinikum der Heinrich-Heine-Universität, Moorenstr. 5, 40225, Düsseldorf, Germany

    Dirk Grotemeyer

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  1. Dirk Grotemeyer
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  2. Mansur Duran
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  5. Kim Nguyen
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Correspondence to Dirk Grotemeyer.

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Dirk Grotemeyer and Mansur Duran contributed equally to this manuscript.

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Grotemeyer, D., Duran, M., Iskandar, F. et al. Median arcuate ligament syndrome: vascular surgical therapy and follow-up of 18 patients. Langenbecks Arch Surg 394, 1085–1092 (2009). https://doi.org/10.1007/s00423-009-0509-5

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