eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology

Superior Mesenteric Artery Syndrome

Author: Shervin Rabizadeh, MD, MBA, Pediatric Gastroenterologist, Cedars Sinai Medical Center; Instructor, Department of Pediatrics, University of California, Los Angeles, David Geffen School of Medicine
Contributor Information and Disclosures

Updated: Jun 18, 2009

Introduction

Background

Superior mesenteric artery (SMA) syndrome is an uncommon but well recognized clinical entity characterized by compression of the third, or transverse, portion of the duodenum against the aorta by the superior mesenteric artery, resulting in chronic, intermittent, or acute complete or partial duodenal obstruction.1 Superior mesenteric artery syndrome was first described in 1861 by Von Rokitansky, who proposed that its cause was obstruction of the third part of the duodenum as a result of arteriomesenteric compression.

Despite the fact that about 400 cases are described in the English language literature, many have doubted the existence of superior mesenteric artery syndrome as a real entity; indeed, some investigators have suggested that superior mesenteric artery syndrome is overdiagnosed because it is confused with other causes of megaduodenum. Nonetheless, the entity (also called cast syndrome) is a well-known complication of scoliosis surgery and often poses a diagnostic dilemma; its diagnosis is frequently one of exclusion.

Pathophysiology

The superior mesenteric artery usually forms an angle of approximately 45° (range, 38-56°) with the abdominal aorta, and the third part of the duodenum crosses caudal to the origin of the superior mesenteric artery, coursing between the superior mesenteric artery and aorta. Any factor that sharply narrows the aortomesenteric angle to approximately 6-25° can cause entrapment and compression of the third part of the duodenum as it passes between the superior mesenteric artery and aorta, resulting in superior mesenteric artery syndrome.

In addition, the aortomesenteric distance in superior mesenteric artery syndrome is decreased to 2-8 mm (normal is 10-20 mm). Alternatively, other causes implicated in superior mesenteric artery syndrome include high insertion of the duodenum at the ligament of Treitz, a low origin of the superior mesenteric artery syndrome, and compression of the duodenum due to peritoneal adhesions.2,3

Frequency

United States

The precise incidence of this entity is unknown. In a review of the literature, approximately 0.013-0.3% of the findings from upper GI tract barium studies support a diagnosis of superior mesenteric artery syndrome.

Mortality/Morbidity

Delay in the diagnosis of superior mesenteric artery syndrome can result in malnutrition, dehydration, electrolyte abnormalities, and even death.

Race

No racial differences have been identified.

Sex

More females are affected by superior mesenteric artery syndrome. In one large series of 75 patients with superior mesenteric artery syndrome, two thirds of the cases involved women, with an average age of 41 years; one third of cases involved men, with an average age of 38 years.

Age

The superior mesenteric artery syndrome usually occurs in older children and adolescents. In one report, 75% of the cases occurred in patients aged 10-30 years.

Clinical

History

The patient often presents with chronic upper abdominal symptoms such as epigastric pain, nausea, eructation, voluminous vomiting (bilious or partially digested food), postprandial discomfort, early satiety, and sometimes, subacute small bowel obstruction. Symptoms of superior mesenteric artery (SMA) syndrome often develop from 6-12 days after scoliosis surgery.

The symptoms are typically relieved when the patient is in the left lateral decubitus, prone, or knee-to-chest position, and they are often aggravated when the patient is in the supine position.

Physical

An asthenic habitus is noted in about 80% of cases. Abdominal examination may reveal a succussion splash. Peptic ulcer disease has been noted in 25-45% of the patients, and hyperchlorhydria has been noted in 50%. Patients can present with signs of subacute small bowel obstruction.

Causes

Important etiologic factors that may precipitate narrowing of the aortomesenteric angle and recurrent mechanical obstruction include the following:

  • Constitutional factors
    • Thin body build
    • Exaggerated lumbar lordosis
    • Visceroptosis and abdominal wall laxity
    • Depletion of the mesenteric fat caused by rapid severe weight loss due to catabolic states such as cancer, surgery, burns, or psychiatric problems
  • Severe injuries, such as head trauma, leading to prolonged bedrest
  • Dietary disorders
  • Spinal disease, deformity, or trauma: Use of body cast in the surgical treatment of scoliosis or vertebral fractures. Superior mesenteric artery syndrome cases after corrective spine surgery are due to the result of spinal elongation, which decreases the superior mesenteric/aortic angle. Postoperative weight loss is an important factor for development of superior mesenteric artery syndrome. Although use of Harrington rods for corrective surgery commonly used in the 1950s and 1960s was an important contributory factor for development of superior mesenteric artery syndrome, newer derotation/translation corrective techniques can also rarely be associated with this disease entity.4
  • Rapid linear growth without compensatory weight gain, particularly in adolescents
  • Anatomic anomalies, rarely
    • Abnormally high and fixed position of the ligament of Treitz with an upward displacement of the duodenum
    • Unusually low origin of the superior mesenteric artery
  • Unusual causes
    • Traumatic aneurysm of the superior mesenteric artery after a stab wound
    • Familial superior mesenteric artery syndrome
    • Recurrent superior mesenteric artery syndrome

More on Superior Mesenteric Artery Syndrome

Overview: Superior Mesenteric Artery Syndrome
Differential Diagnoses & Workup: Superior Mesenteric Artery Syndrome
Treatment & Medication: Superior Mesenteric Artery Syndrome
Follow-up: Superior Mesenteric Artery Syndrome
References
Further Reading
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References

  1. Gerasimidis T, George F. Superior Mesenteric Artery Syndrome. Wilkie Syndrome. Dig Surg. May 20 2009;26(3):213-214. [Medline].

  2. Merrett ND, Wilson RB, Cosman P, Biankin AV. Superior mesenteric artery syndrome: diagnosis and treatment strategies. J Gastrointest Surg. Feb 2009;13(2):287-92. [Medline].

  3. Kyslan K, Barla J, Kyslan K, Stanislayova M. [Superior mesenteric artery (SMAS/AMS) syndrome and its management]. Rozhl Chir. May 2008;87(5):255-8. [Medline].

  4. Ha CD, Alvear DT, Leber DC. Duodenal derotation as an effective treatment of superior mesenteric artery syndrome: a thirty-three year experience. Am Surg. Jul 2008;74(7):644-53. [Medline].

  5. Morris TC, Devitt PG, Thompson SK. Laparoscopic Duodenojejunostomy for Superior Mesenteric Artery Syndrome-How I Do It. J Gastrointest Surg. Apr 9 2009;[Medline].

  6. Jo JB, Song KY, Park CH. Laparoscopic duodenojejunostomy for superior mesenteric artery syndrome: report of a case. Surg Laparosc Endosc Percutan Tech. Apr 2008;18(2):213-5. [Medline].

  7. Altiok H, Lubicky JP, DeWald CJ, Herman JE. The superior mesenteric artery syndrome in patients with spinal deformity. Spine. Oct 1 2005;30(19):2164-70. [Medline].

  8. Altman DH, Puranik SR. Superior mesenteric artery syndrome in children. Am J Roentgenol Radium Ther Nucl Med. May 1973;118(1):104-8. [Medline].

  9. Baltazar U, Dunn J, Floresguerra C. Superior mesenteric artery syndrome: an uncommon cause of intestinal obstruction. South Med J. Jun 2000;93(6):606-8. [Medline].

  10. Barnes JB, Lee M. Superior mesenteric artery syndrome in an intravenous drug abuser after rapid weight loss. South Med J. Mar 1996;89(3):331-4. [Medline].

  11. Crowther MAA, Webb PJ, Eyre-Brook IA. Superior mesenteric artery syndrome following surgery for scoliosis. Spine. 2002;27:E528-33.

  12. Hutchinson DT, Bassett GS. Superior mesenteric artery syndrome in pediatric orthopedic patients. Clin Orthop. Jan 1990;(250):250-7. [Medline].

  13. Massoud WZ. Laparoscopic management of superior mesenteric artery syndrome. Int Surg. Oct-Dec 1995;80(4):322-7. [Medline].

  14. Munns SW, Morrissy RT, Golladay ES. Hyperalimentation for superior mesenteric-artery (cast) syndrome following correction of spinal deformity. J Bone Joint Surg Am. Oct 1984;66(8):1175-7. [Medline].

  15. Ortiz C, Cleveland RH, Blickman JG. Familial superior mesenteric artery syndrome. Pediatr Radiol. 1990;20(8):588-9. [Medline].

  16. Raissi B, Taylor BM, Taves DH. Recurrent superior mesenteric artery (Wilkie's) syndrome: a case report. Can J Surg. Oct 1996;39(5):410-6. [Medline].

  17. Rokitansky C. Handbuch der Pathologischen Anotomie. Vienna: Branmiller and Siedel. 1842.

  18. Santer R, Young C, Rossi T. Computed tomography in superior mesenteric artery syndrome. Pediatr Radiol. 1991;21(2):154-5. [Medline].

  19. Shah MA, Albright MB, Vogt MT, Moreland MS. Superior mesenteric artery syndrome in scoliosis surgery: weight percentile for height as an indicator of risk. J Pediatr Orthop. Sep-Oct 2003;23(5):665-8. [Medline].

  20. Shapiro G, Green DW, Fatica NS, Boachie-Adjei O. Medical complications in scoliosis surgery. Curr Opin Pediatr. Feb 2001;13(1):36-41. [Medline].

  21. Shetty AK, Schmidt-Sommerfeld E, Haymon ML. Radiological case of the month. Superior mesenteric artery syndrome. Arch Pediatr Adolesc Med. Mar 1999;153(3):303-4. [Medline].

  22. Vitale MG, Higgs GB, Liebling MS, et al. Superior mesenteric artery syndrome after segmental instrumentation: a biomechanical analysis. Am J Orthop. Aug 1999;28(8):461-7. [Medline].

  23. Wilkie DPD. Chronic duodenal ileus. Am J Med Sci. 1927;173:643-9.

  24. Ylinen P, Kinnunen J, Hockerstedt K. Superior mesenteric artery syndrome. A follow-up study of 16 operated patients. J Clin Gastroenterol. Aug 1989;11(4):386-91. [Medline].

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Further Reading

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Keywords

superior mesenteric artery syndrome, SMA syndrome, cast syndrome, Wilkie's syndrome, Wilkie syndrome, arteriomesenteric duodenal compression syndrome, chronic duodenal ileus, scoliosis, megaduodenum, small bowel obstruction, peptic ulcer disease, diagnosis, treatment

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Contributor Information and Disclosures

Author

Shervin Rabizadeh, MD, MBA, Pediatric Gastroenterologist, Cedars Sinai Medical Center; Instructor, Department of Pediatrics, University of California, Los Angeles, David Geffen School of Medicine
Shervin Rabizadeh, MD, MBA is a member of the following medical societies: American Academy of Pediatrics, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology, Hepatology and Nutrition
Disclosure: Nothing to disclose.

Medical Editor

Jorge H Vargas, MD, Professor of Pediatrics and Clinical Professor of Pediatric Gastroenterology, David Geffen School of Medicine, University of California at Los Angeles; Consulting Physician, Department of Pediatrics, University of California at Los Angeles Health System
Jorge H Vargas, MD is a member of the following medical societies: American Liver Foundation, American Society for Gastrointestinal Endoscopy, American Society for Parenteral and Enteral Nutrition, Latin American Society of Pediatric Gastroenterology, Hepatology & Nutrition, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

B UK Li, MD, Professor of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Director, Pediatric Fellowships and Gastroenterology Fellowship, Medical Director, Functional Gastrointestinal Disorders and Cyclic Vomiting Program, Medical College of Wisconsin; Attending Gastroenterologist, Children's Hospital of Wisconsin
B UK Li, MD is a member of the following medical societies: Alpha Omega Alpha, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

CME Editor

Steven M Schwarz, MD, FAAP, FACN, AGAF, Professor of Pediatrics, Children's Hospital at Downstate, SUNY-Downstate Medical Center
Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research
Disclosure: TAP Pharmaceuticals Honoraria Speaking and teaching; Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor

Chief Editor

Carmen Cuffari, MD, Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine
Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

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