eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology

Superior Mesenteric Artery Syndrome: Differential Diagnoses & Workup

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

Differential Diagnoses

Other Problems to Be Considered

The differential diagnosis includes anorexia nervosa and bulimia. In addition, superior mesenteric artery (SMA) syndrome should be differentiated from other causes of megaduodenum, including diabetes mellitus, collagen vascular conditions, and chronic idiopathic intestinal pseudoobstruction.

Workup

Imaging Studies

  • The diagnosis of superior mesenteric artery (SMA) syndrome is difficult. Confirmation usually requires radiographic studies, such as an upper GI series, hypotonic duodenography, and CT scanning.
  • Upper GI study with barium reveals characteristic dilatation of the first and second parts of the duodenum, with an abrupt vertical or linear cutoff in the third part with normal mucosal folds.
  • Fluoroscopy demonstrates a to-and-fro motion of the barium in the dilated proximal portion of the duodenum. Other findings include delay of 4-6 hours in gastroduodenal transit and relief of the obstruction when the patient is in the left lateral decubitus position.
  • A Hayes maneuver (ie, pressure applied below the umbilicus in cephalad and dorsal direction), which elevates the root of small-bowel mesentery, may also relieve the obstruction.
  • In equivocal cases, hypotonic duodenography may depict the site of obstruction and dilation of the proximal duodenum, with antiperistaltic waves within the dilated portion.
  • CT scanning is useful in the diagnosis of superior mesenteric artery syndrome and can provide diagnostic information, including aorta-superior mesenteric artery distances and duodenal distension. Also, it can be used to assess intra-abdominal and retroperitoneal fat.
  • Upper GI endoscopy may be necessary to exclude mechanical causes of duodenal obstruction. However, the diagnosis of superior mesenteric artery syndrome may be missed with this study.
  • Abdominal ultrasonography may be helpful in measuring the angle of the superior mesenteric artery and the aortomesenteric distance.
  • Manometry may be used to differentiate between the possibility of a myopathic form of chronic intestinal pseudo-obstruction syndrome (hollow visceral myopathy) by demonstrating low-amplitude waves throughout the duodenum, and frequently the stomach, versus an irregular or absent phase III and no postprandial motility changes in a neuropathic form of chronic intestinal pseudoobstruction syndrome. The pattern of increased amplitude of propagated contractions and retrograde contractions should hint or suggest the mechanical obstruction in 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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