History

The patient often presents with chronic upper abdominal symptoms such as abdominal 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. These maneuvers are thought to reduce the small bowel mesenteric tension at the aortomesenteric angle.^[11]

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, trauma, or psychiatric problems
Severe injuries, such as head trauma, leading to prolonged bedrest
Dietary disorders
- Anorexia nervosa
- Malabsorption
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.^[12]
Rapid linear growth without compensatory weight gain, particularly in adolescents: Adolescents with low body mass index (< 18 kg/m²) may be at higher risk for developing superior mesenteric artery syndrome after spinal fusion for scoliosis than patients with a higher body mass index.^[13]
Anatomic anomalies (rare)
- 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
- Abdominal aortic aneurysms and mycotic aortic aneurysms^[14]
- Familial superior mesenteric artery syndrome
- Recurrent superior mesenteric artery syndrome
- Idiopathic neonatal superior mesenteric artery syndrome^{[15, 16]}

Differential Diagnoses

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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.

of 1

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.

Sections Superior Mesenteric Artery (SMA) Syndrome
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Superior Mesenteric Artery (SMA) Syndrome Clinical Presentation

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