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 between the aorta and the superior mesenteric artery. This results 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. Some studies report the incidence of superior mesenteric artery syndrome to be 0.1-0.3%.^[2]Approximately 0.013-0.78% of barium upper GI studies evaluating for superior mesenteric artery syndrome support the diagnosis.^{[3, 4]}

Despite the fact that about 400 cases are described in the English language literature, many have doubted the existence of SMA 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, anorexia, and trauma. It 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 SMA 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, and compression of the duodenum due to peritoneal adhesions.^{[5, 6]}

Frequency of SMA Syndrome

United States

The precise incidence of this entity is unknown. In a review of the literature, approximately 0.013-0.78% of the findings from upper GI tract barium studies support a diagnosis of superior mesenteric artery syndrome.

SMA Syndrome Mortality/Morbidity

Delay in the diagnosis of SMA syndrome can result in malnutrition, dehydration, electrolyte abnormalities, gastric pneumatosis and portal venous gas, formation of an obstructing duodenal bezoar, hypovolemia secondary to massive GI hemorrhage, and even death secondary to gastric perforation.^{[7, 8, 9, 10]}

Race

No racial differences have been identified.

Sex

More females are affected by SMA 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

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

Clinical Presentation

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Media Gallery

Prompt gastric emptying of residual contrast is present within the stomach into the second portion of the duodenum. Additional contrast was hand injected through the transpyloric feeding tube to further distend the second portion of the duodenum. The second portion of the duodenum is dilated with very slow transit across the spine into the jejunum. This occurred despite difference in position of the patient. The high-grade partial obstruction at the distal second portion of the duodenum is due to superior mesenteric artery (SMA) syndrome.

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Author

Frederick Merrill Karrer, MD, FACS Professor of Surgery and Pediatrics, Head, Division of Pediatric Surgery, University of Colorado School of Medicine; The Dr David R and Kiku Akers Chair in Pediatric Surgery, Surgical Director, Pediatric Transplantation, The Children’s Hospital of Colorado

Frederick Merrill Karrer, MD, FACS is a member of the following medical societies: American Academy of Pediatrics, American Association for the Study of Liver Diseases, American College of Surgeons, American Pediatric Surgical Association, American Society of Transplant Surgeons, Denver Academy of Surgery, International Pediatric Transplant Association, Pacific Association of Pediatric Surgery, Transplantation Society, Western Surgical Association, Western Trauma Association

Disclosure: Nothing to disclose.

Coauthor(s)

Stephanie A Jones, DO Resident Physician, Department of Pediatric Surgery, University of Colorado School of Medicine

Stephanie A Jones, DO is a member of the following medical societies: American College of Surgeons, American Medical Association

Disclosure: Received salary from University of Colorado Denver for employment.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

B UK Li, MD Professor of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Medical College of Wisconsin; Attending Gastroenterologist, Director, Cyclic Vomiting Program, Children’s Hospital of Wisconsin

B UK Li, MD is a member of the following medical societies: Alpha Omega Alpha, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

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, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching. for: Abbott Nutritional, Abbvie, speakers' bureau.

Additional Contributors

Jorge H Vargas, MD Professor of Pediatrics and Clinical Professor of Pediatric Gastroenterology, University of California, Los Angeles, David Geffen School of Medicine; 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, Latin American Society of Pediatric Gastroenterology, Hepatology & Nutrition, American Society for Gastrointestinal Endoscopy, American Society for Parenteral and Enteral Nutrition, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Avinash Shetty, MD, and Ivor D Hill, MB, ChB, DCH, FCP, MD, to the original writing and development of this article.