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 and Endovascular Surgery
- Professor of Surgery, Texas A&M Health Sciences Center - Dallas Campus
- Vice Chair of Vascular Surgical Services, Baylor Heart and Vascular Hospital at Dallas
- Joseph L Mills, Sr, MD
Joseph L Mills, Sr, MD
- Section Editor — Vascular and Endovascular Surgery
- Professor and Chief
- Division of Vascular Surgery and Endovascular Therapy
- Baylor College of Medicine
Celiac artery compression syndrome is defined as chronic, recurrent abdominal pain related to compression of the celiac artery by the median arcuate ligament. It is also referred to as celiac axis syndrome, median arcuate ligament syndrome, and Dunbar syndrome. 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 that overlap with other forms of chronic intestinal ischemia. Treatment involves surgical decompression of the celiac axis. Treatment success, which cannot be guaranteed, depends upon appropriate patient selection. Celiac artery decompression can be accomplished using an open or minimally invasive approach, which can either be a standard laparoscopic or a robotic-assisted approach. For some patients, celiac artery revascularization (open, endovascular) may be necessary.
The etiology, clinical features, diagnosis, and treatment of celiac artery compression syndrome will be reviewed here. An overview of other etiologies for acute mesenteric ischemia and chronic mesenteric ischemia is provided separately. (See "Overview of intestinal ischemia in adults" and "Chronic mesenteric ischemia".)
ETIOLOGY AND PATHOPHYSIOLOGY
The etiology and pathophysiology of celiac artery compression syndrome are incompletely understood but may be related to both ischemic and neuropathic mechanisms.
The anatomy of the celiac region (including the median arcuate ligament and its variations ) was described long before the relationship between abdominal symptoms and compression of the celiac artery by the median arcuate ligament was described clinically [2-6]. The syndrome is also referred to as celiac artery compression, celiac axis syndrome, median arcuate ligament syndrome, and Dunbar syndrome.
The median arcuate ligament is a fibrous arch that traverses the aorta and bridges the crura of the diaphragm (figure 1). Typically, the celiac axis branches from the abdominal aorta below the median arcuate ligament (between T11 and L1), but wide variation in the location of the celiac origin has been reported . A higher or lower origin of the celiac axis may be prone to compression. A subset of patients (approximately 10 percent) have an abnormally positioned median arcuate ligament that appears to compress an otherwise normally positioned celiac artery . Compression of the celiac artery by the median arcuate ligament is accentuated during expiration as the median arcuate ligament moves cranially and relieved during inspiration .
- Lipshutz B. A COMPOSITE STUDY OF THE COELIAC AXIS ARTERY. Ann Surg 1917; 65:159.
- Lindner HH, Kemprud E. A clinicoanatomical study of the arcuate ligament of the diaphragm. Arch Surg 1971; 103:600.
- 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.
- Loukas M, Pinyard J, Vaid S, et al. Clinical anatomy of celiac artery compression syndrome: a review. Clin Anat 2007; 20:612.
- Horton KM, Talamini MA, Fishman EK. Median arcuate ligament syndrome: evaluation with CT angiography. Radiographics 2005; 25:1177.
- Reuter SR, Bernstein EF. The anatomic basis for respiratory variation in median arcuate ligament compression of the celiac artery. Surgery 1973; 73:381.
- Park CM, Chung JW, Kim HB, et al. Celiac axis stenosis: incidence and etiologies in asymptomatic individuals. Korean J Radiol 2001; 2:8.
- Jimenez JC, Harlander-Locke M, Dutson EP. Open and laparoscopic treatment of median arcuate ligament syndrome. J Vasc Surg 2012; 56:869.
- Brandt LJ, Boley SJ. Celiac axis compression syndrome. A critical review. Am J Dig Dis 1978; 23:633.
- Bech FR. Celiac artery compression syndromes. Surg Clin North Am 1997; 77:409.
- Trinidad-Hernandez M, Keith P, Habib I, White JV. Reversible gastroparesis: functional documentation of celiac axis compression syndrome and postoperative improvement. Am Surg 2006; 72:339.
- Balaban DH, Chen J, Lin Z, et al. Median arcuate ligament syndrome: a possible cause of idiopathic gastroparesis. Am J Gastroenterol 1997; 92:519.
- Scholbach T. Celiac artery compression syndrome in children, adolescents, and young adults: clinical and color duplex sonographic features in a series of 59 cases. J Ultrasound Med 2006; 25:299.
- 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.
- DERRICK JR, POLLARD HS, MOORE RM. The pattern of arteriosclerotic narrowing of the celiac and superior mesenteric arteries. Ann Surg 1959; 149:684.
- Kazan V, Qu W, Al-Natour M, et al. Celiac artery compression syndrome: a radiological finding without clinical symptoms? Vascular 2013; 21:293.
- Kim EN, Lamb K, Relles D, et al. Median Arcuate Ligament Syndrome-Review of This Rare Disease. JAMA Surg 2016; 151:471.
- Reilly LM, Ammar AD, Stoney RJ, Ehrenfeld WK. Late results following operative repair for celiac artery compression syndrome. J Vasc Surg 1985; 2:79.
- Cusati DA, Noel AA, Gloviczki P, et al. Median arcuate ligament syndrome: a 20-year experience of surgical treatment. Presented at: 60th Annual Meeting of the Society for Vascular Surgery: June 1-4, 2006: Philadelphia, PA
- Williams S, Gillespie P, Little JM. Celiac axis compression syndrome: factors predicting a favorable outcome. Surgery 1985; 98:879.
- Gloviczki P, Duncan AA. Treatment of celiac artery compression syndrome: does it really exist? Perspect Vasc Surg Endovasc Ther 2007; 19:259.
- Duffy AJ, Panait L, Eisenberg D, et al. Management of median arcuate ligament syndrome: a new paradigm. Ann Vasc Surg 2009; 23:778.
- 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.
- Watson WC, Sadikali F. Celiac axis compression: experience with 20 patients and a critical appraisal of the syndrome. Ann Intern Med 1977; 86:278.
- 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.
- 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.
- Gruber H, Loizides A, Peer S, Gruber I. Ultrasound of the median arcuate ligament syndrome: a new approach to diagnosis. Med Ultrason 2012; 14:5.
- 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.
- Skeik N, Cooper LT, Duncan AA, Jabr FI. Median arcuate ligament syndrome: a nonvascular, vascular diagnosis. Vasc Endovascular Surg 2011; 45:433.
- 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.
- Lee MJ, Mueller PR, vanSonnenberg E, et al. CT-guided celiac ganglion block with alcohol. AJR Am J Roentgenol 1993; 161:633.
- Lee JM. CT-guided celiac plexus block for intractable abdominal pain. J Korean Med Sci 2000; 15:173.
- Duncan AA. Median arcuate ligament syndrome. Curr Treat Options Cardiovasc Med 2008; 10:112.
- 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.
- 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.
- Wang X, Impeduglia T, Dubin Z, Dardik H. Celiac revascularization as a requisite for treating the median arcuate ligament syndrome. Ann Vasc Surg 2008; 22:571.
- Cinà CS, Safar H. Successful treatment of recurrent celiac axis compression syndrome. A case report. Panminerva Med 2002; 44:69.
- Matsumoto AH, Tegtmeyer CJ, Fitzcharles EK, et al. Percutaneous transluminal angioplasty of visceral arterial stenoses: results and long-term clinical follow-up. J Vasc Interv Radiol 1995; 6:165.
- 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.
- 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.
- Kohn GP, Bitar RS, Farber MA, et al. Treatment options and outcomes for celiac artery compression syndrome. Surg Innov 2011; 18:338.
- Loffeld RJ, Overtoom HA, Rauwerda JA. The celiac axis compression syndrome. Report of 5 cases. Digestion 1995; 56:534.
- Thoolen SJ, van der Vliet WJ, Kent TS, et al. Technique and outcomes of robot-assisted median arcuate ligament release for celiac artery compression syndrome. J Vasc Surg 2015; 61:1278.
- Columbo JA, Trus T, Nolan B, et al. Contemporary management of median arcuate ligament syndrome provides early symptom improvement. J Vasc Surg 2015; 62:151.
- 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.
- 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.
- 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.
- ETIOLOGY AND PATHOPHYSIOLOGY
- CLINICAL FEATURES
- Abdominal pain
- Unintentional weight loss
- Other symptoms
- Physical examination
- Inspiratory and expiratory vascular imaging
- - Arteriography
- - Duplex ultrasound
- Physiologic testing
- - Gastric tonometry
- - Ganglion nerve block
- Differential diagnosis
- Patient selection
- Celiac artery decompression
- Approach and technique
- - Minimally invasive versus open decompression
- - Intraoperative vascular assessment
- Revascularization for persistent stenosis after decompression
- POSTOPERATIVE CARE AND FOLLOW-UP
- Early recurrence
- LONG-TERM OUTCOMES
- SUMMARY AND RECOMMENDATIONS