Celiac artery compression syndrome
- Sherry Scovell, MD, FACS
Sherry Scovell, MD, FACS
- Assistant Professor in Surgery
- Massachusetts General Hospital
- Allen Hamdan, MD
Allen Hamdan, MD
- Clinical Director of Vascular and Endovascular Surgery
- Associate Professor
- Harvard Medical School
- Section Editors
- John F Eidt, MD
John F Eidt, MD
- Section Editor — Vascular Surgery
- Professor of Surgery
- University of South Carolina School of Medicine Greenville
- Joseph L Mills, Sr, MD
Joseph L Mills, Sr, MD
- Section Editor — Vascular Surgery
- Professor of Surgery
- University of Arizona Health Sciences Center
Celiac artery compression syndrome (also referred to as celiac axis syndrome, median arcuate ligament syndrome, and Dunbar syndrome) is defined as chronic, recurrent abdominal pain related to compression of the celiac artery by the median arcuate ligament. It is an uncommon disorder that is characterized clinically by the triad of postprandial abdominal pain, weight loss, and sometimes an abdominal bruit. The diagnosis is often one of exclusion, given the nonspecific symptoms which overlap with other forms of chronic intestinal ischemia. Treatment may involve surgical decompression of the celiac access; however, patient selection can be challenging because success cannot be guaranteed.
The etiology, clinical features, diagnosis, and treatment of celiac artery compression syndrome will be reviewed here. An overview discussing other etiologies of acute mesenteric and chronic mesenteric ischemia is presented elsewhere. (See "Overview of intestinal ischemia in adults".)
ETIOLOGY AND PATHOPHYSIOLOGY
Celiac artery compression syndrome was initially described in the 1960s and is defined as abdominal pain related to compression of the celiac artery by the median arcuate ligament [1-4]. The syndrome is also referred to as celiac axis syndrome, median arcuate ligament syndrome, and Dunbar syndrome.
The median arcuate ligament is a fibrous arch that traverses the aorta just cephalad to the origin of the celiac artery, and bridges the crura of the diaphragm (figure 1). Adjacent to the median arcuate ligament is the celiac plexus (or ganglion), which originates from preganglionic splanchnic nerves, somatic branches from the phrenic and vagus nerves, parasympathetic preganglionic nerves, and sympathetic postganglionic fibers .
The etiology is incompletely understood and the pathophysiology may be related to both ischemic and neuropathic mechanisms. In spite of many case reports where the diagnosis appeared to be clear cut, and symptoms have been relieved following surgical decompression of the celiac artery, the diagnosis (and existence) of celiac artery compression syndrome has been a source of controversy [6-11]. In celiac artery compression syndrome, although flow may be reduced in the celiac artery, the superior mesenteric artery (SMA) and the inferior mesenteric artery are widely patent, and should be capable of providing ample perfusion to the bowel (figure 2). It has been suggested that pain may be mediated by the celiac plexus. Others have associated celiac artery compression with delayed gastric emptying, which can also be related to nerve dysfunction, suggesting another possible source for symptoms .
- Dunbar JD, Molnar W, Beman FF, Marable SA. Compression of the celiac trunk and abdominal angina. Am J Roentgenol Radium Ther Nucl Med 1965; 95:731.
- Carey JP, Stemmer EA, Connolly JE. Median arcuate ligament syndrome. Experimental and clinical observations. Arch Surg 1969; 99:441.
- Marable SA, Kaplan MF, Beman FM, Molnar W. Celiac compression syndrome. Am J Surg 1968; 115:97.
- HARJOLA PT. A RARE OBSTRUCTION OF THE COELIAC ARTERY. REPORT OF A CASE. Ann Chir Gynaecol Fenn 1963; 52:547.
- Jimenez JC, Harlander-Locke M, Dutson EP. Open and laparoscopic treatment of median arcuate ligament syndrome. J Vasc Surg 2012; 56:869.
- Stanley JC, Fry WJ. Median arcuate ligament syndrome. Arch Surg 1971; 103:252.
- Bobadilla JL. Mesenteric ischemia. Surg Clin North Am 2013; 93:925.
- Szilagyi DE, Rian RL, Elliott JP, Smith RF. The celiac artery compression syndrome: does it exist? Surgery 1972; 72:849.
- Gloviczki P, Duncan AA. Treatment of celiac artery compression syndrome: does it really exist? Perspect Vasc Surg Endovasc Ther 2007; 19:259.
- Kernohan RM, Barros D'Sa AA, Cranley B, Johnston HM. Further evidence supporting the existence of the celiac artery compression syndrome. Arch Surg 1985; 120:1072.
- Plate G, Eklöf B, Vang J. The celiac compression syndrome: myth or reality? Acta Chir Scand 1981; 147:201.
- Balaban DH, Chen J, Lin Z, et al. Median arcuate ligament syndrome: a possible cause of idiopathic gastroparesis. Am J Gastroenterol 1997; 92:519.
- Schweizer P, Berger S, Schweizer M, et al. Arcuate ligament vascular compression syndrome in infants and children. J Pediatr Surg 2005; 40:1616.
- Said SM, Zarroug AE, Gloviczki P, Shields RC. Pediatric median arcuate ligament syndrome: first report of familial pattern and transperitoneal laparoscopic release. J Pediatr Surg 2010; 45:e17.
- Aschenbach R, Basche S, Vogl TJ. Compression of the celiac trunk caused by median arcuate ligament in children and adolescent subjects: evaluation with contrast-enhanced MR angiography and comparison with Doppler US evaluation. J Vasc Interv Radiol 2011; 22:556.
- Mak GZ, Speaker C, Anderson K, et al. Median arcuate ligament syndrome in the pediatric population. J Pediatr Surg 2013; 48:2261.
- Bech F, Loesberg A, Rosenblum J, et al. Median arcuate ligament compression syndrome in monozygotic twins. J Vasc Surg 1994; 19:934.
- Okten RS, Kucukay F, Tola M, et al. Is celiac artery compression syndrome genetically inherited?: a case series from a family and review of the literature. Eur J Radiol 2012; 81:1089.
- Schreiber JP, Angle JF, Matsumoto AH, et al. Acute visceral ischemia occurring subsequent to blunt abdominal trauma: potential culpability of median arcuate ligament compression. J Trauma 1998; 45:404.
- Sanchez AM, Alfieri S, Caprino P, et al. Temporary medium arcuate ligament syndrome after pancreatoduodenectomy. Am Surg 2013; 79:E58.
- Shukla PJ, Barreto SG, Kulkarni A, et al. Vascular anomalies encountered during pancreatoduodenectomy: do they influence outcomes? Ann Surg Oncol 2010; 17:186.
- Farma JM, Hoffman JP. Nonneoplastic celiac axis occlusion in patients undergoing pancreaticoduodenectomy. Am J Surg 2007; 193:341.
- Sugae T, Fujii T, Kodera Y, et al. Classification of the celiac axis stenosis owing to median arcuate ligament compression, based on severity of the stenosis with subsequent proposals for management during pancreatoduodenectomy. Surgery 2012; 151:543.
- Lindner HH, Kemprud E. A clinicoanatomical study of the arcuate ligament of the diaphragm. Arch Surg 1971; 103:600.
- DERRICK JR, POLLARD HS, MOORE RM. The pattern of arteriosclerotic narrowing of the celiac and superior mesenteric arteries. Ann Surg 1959; 149:684.
- Alehan D, Dogan OF. Pediatric surgical image. A rare case: celiac artery compression syndrome in an asymptomatic child. J Pediatr Surg 2004; 39:645.
- Kazan V, Qu W, Al-Natour M, et al. Celiac artery compression syndrome: a radiological finding without clinical symptoms? Vascular 2013.
- Park CM, Chung JW, Kim HB, et al. Celiac axis stenosis: incidence and etiologies in asymptomatic individuals. Korean J Radiol 2001; 2:8.
- Reilly LM, Ammar AD, Stoney RJ, Ehrenfeld WK. Late results following operative repair for celiac artery compression syndrome. J Vasc Surg 1985; 2:79.
- Watson WC, Sadikali F. Celiac axis compression: experience with 20 patients and a critical appraisal of the syndrome. Ann Intern Med 1977; 86:278.
- Sultan S, Hynes N, Elsafty N, Tawfick W. Eight years experience in the management of median arcuate ligament syndrome by decompression, celiac ganglion sympathectomy, and selective revascularization. Vasc Endovascular Surg 2013; 47:614.
- Williams S, Gillespie P, Little JM. Celiac axis compression syndrome: factors predicting a favorable outcome. Surgery 1985; 98:879.
- Kopecky KK, Stine SB, Dalsing MC, Gottlieb K. Median arcuate ligament syndrome with multivessel involvement: diagnosis with spiral CT angiography. Abdom Imaging 1997; 22:318.
- Horton KM, Talamini MA, Fishman EK. Median arcuate ligament syndrome: evaluation with CT angiography. Radiographics 2005; 25:1177.
- Saglam M, Sildiroglu HO, Incedayi M, et al. A variant of the median arcuate ligament syndrome: are sagittal images enough for diagnosis? Wien Klin Wochenschr 2013; 125:220.
- Manghat NE, Mitchell G, Hay CS, Wells IP. The median arcuate ligament syndrome revisited by CT angiography and the use of ECG gating--a single centre case series and literature review. Br J Radiol 2008; 81:735.
- Jimenez JC, Rafidi F, Morris L. True celiac artery aneurysm secondary to median arcuate ligament syndrome. Vasc Endovascular Surg 2011; 45:288.
- Wolfman D, Bluth EI, Sossaman J. Median arcuate ligament syndrome. J Ultrasound Med 2003; 22:1377.
- Erden A, Yurdakul M, Cumhur T. Marked increase in flow velocities during deep expiration: A duplex Doppler sign of celiac artery compression syndrome. Cardiovasc Intervent Radiol 1999; 22:331.
- Ozel A, Toksoy G, Ozdogan O, et al. Ultrasonographic diagnosis of median arcuate ligament syndrome: a report of two cases. Med Ultrason 2012; 14:154.
- Kalapatapu VR, Murray BW, Palm-Cruz K, et al. Definitive test to diagnose median arcuate ligament syndrome: injection of vasodilator during angiography. Vasc Endovascular Surg 2009; 43:46.
- Mensink PB, van Petersen AS, Kolkman JJ, et al. Gastric exercise tonometry: the key investigation in patients with suspected celiac artery compression syndrome. J Vasc Surg 2006; 44:277.
- Duffy AJ, Panait L, Eisenberg D, et al. Management of median arcuate ligament syndrome: a new paradigm. Ann Vasc Surg 2009; 23:778.
- Takach TJ, Livesay JJ, Reul GJ Jr, Cooley DA. Celiac compression syndrome: tailored therapy based on intraoperative findings. J Am Coll Surg 1996; 183:606.
- Kohn GP, Bitar RS, Farber MA, et al. Treatment options and outcomes for celiac artery compression syndrome. Surg Innov 2011; 18:338.
- Grotemeyer D, Duran M, Iskandar F, et al. Median arcuate ligament syndrome: vascular surgical therapy and follow-up of 18 patients. Langenbecks Arch Surg 2009; 394:1085.
- Ghosn PB, Rabbat AG, Trudel J, et al. Celiac compression syndrome. Can J Surg 1982; 25:377.
- Mihas AA, Laws HL, Jander HP. Surgical treatment of the celiac axis compression syndrome. Am J Surg 1977; 133:688.
- Kokotsakis JN, Lambidis CD, Lioulias AG, et al. Celiac artery compression syndrome. Cardiovasc Surg 2000; 8:219.
- Daskalakis MK. Celiac axis compression syndrome. Int Surg 1982; 67:442.
- Delis KT, Gloviczki P, Altuwaijri M, McKusick MA. Median arcuate ligament syndrome: open celiac artery reconstruction and ligament division after endovascular failure. J Vasc Surg 2007; 46:799.
- Roayaie S, Jossart G, Gitlitz D, et al. Laparoscopic release of celiac artery compression syndrome facilitated by laparoscopic ultrasound scanning to confirm restoration of flow. J Vasc Surg 2000; 32:814.
- Relles D, Moudgill N, Rao A, et al. Robotic-assisted median arcuate ligament release. J Vasc Surg 2012; 56:500.
- Carbonell AM, Kercher KW, Heniford BT, Matthews BD. Multimedia article. Laparoscopic management of median arcuate ligament syndrome. Surg Endosc 2005; 19:729.
- Jaik NP, Stawicki SP, Weger NS, Lukaszczyk JJ. Celiac artery compression syndrome: successful utilization of robotic-assisted laparoscopic approach. J Gastrointestin Liver Dis 2007; 16:93.
- Do MV, Smith TA, Bazan HA, et al. Laparoscopic versus robot-assisted surgery for median arcuate ligament syndrome. Surg Endosc 2013; 27:4060.
- van Petersen AS, Vriens BH, Huisman AB, et al. Retroperitoneal endoscopic release in the management of celiac artery compression syndrome. J Vasc Surg 2009; 50:140.
- Baccari P, Civilini E, Dordoni L, et al. Celiac artery compression syndrome managed by laparoscopy. J Vasc Surg 2009; 50:134.
- Roseborough GS. Laparoscopic management of celiac artery compression syndrome. J Vasc Surg 2009; 50:124.
- El-Hayek KM, Titus J, Bui A, et al. Laparoscopic median arcuate ligament release: are we improving symptoms? J Am Coll Surg 2013; 216:272.
- Tulloch AW, Jimenez JC, Lawrence PF, et al. Laparoscopic versus open celiac ganglionectomy in patients with median arcuate ligament syndrome. J Vasc Surg 2010; 52:1283.
- Vaziri K, Hungness ES, Pearson EG, Soper NJ. Laparoscopic treatment of celiac artery compression syndrome: case series and review of current treatment modalities. J Gastrointest Surg 2009; 13:293.
- Duncan AA. Median arcuate ligament syndrome. Curr Treat Options Cardiovasc Med 2008; 10:112.
- Berard X, Cau J, Déglise S, et al. Laparoscopic surgery for coeliac artery compression syndrome: current management and technical aspects. Eur J Vasc Endovasc Surg 2012; 43:38.
- A-Cienfuegos J, Rotellar F, Valentí V, et al. The celiac axis compression syndrome (CACS): critical review in the laparoscopic era. Rev Esp Enferm Dig 2010; 102:193.
- Geelkerken RH, van Bockel JH, de Roos WK, Hermans J. Coeliac artery compression syndrome: the effect of decompression. Br J Surg 1990; 77:807.
- Palmer OP, Tedesco M, Casey K, et al. Hybrid treatment of celiac artery compression (median arcuate ligament) syndrome. Dig Dis Sci 2012; 57:1782.
- Lai EC. Vascular resection and reconstruction at pancreatico-duodenectomy: technical issues. Hepatobiliary Pancreat Dis Int 2012; 11:234.
- Lord RS, Tracy GD. Coeliac artery compression. Br J Surg 1980; 67:590.
- Muqeetadnan M, Amer S, Rahman A, et al. Celiac artery compression syndrome. Case Rep Gastrointest Med 2013; 2013:934052.
- Evans WE. Long-term evaluation of the celiac band syndrome. Surgery 1974; 76:867.
- Rogers DM, Thompson JE, Garrett WV, et al. Mesenteric vascular problems. A 26-year experience. Ann Surg 1982; 195:554.