II. Pathophysiology

  1. Third section of Duodenum constricted between superior Mesenteric Artery and abdominal aorta

III. Epidemiology

  1. Rare

IV. Risk Factors

  1. Genetic predisposition (more common in the twin of an affected patient)
  2. Corrective spinal surgery for Scoliosis
  3. Slender patients with acute weight loss (loss of mesenteric fat pad)
    1. Anorexia Nervosa
    2. Gastroenteritis

V. Findings: Proximal Small Bowel Obstruction

  1. Bilious Emesis
  2. Upper Abdominal Pain
  3. Early satiety
  4. Weight loss

VI. Associated Conditions

  1. Celiac Axis Compression Syndrome.

VIII. Complications

  1. Small Bowel Obstruction related complications
    1. Gastric or Intestinal Pneumatosis
    2. Intestinal Perforation
    3. Duodenal Bezoar
    4. Portal Vein Gas
  2. Malnutrition and Electrolyte disturbance
  3. Aspiration Pneumonia

IX. Management

  1. Manage Small Bowel Obstruction
  2. Supportive care (hydration, Electrolyte replacement)
  3. Treat underlying causes
  4. Surgical interventions
    1. Duodenum repositioning to right of SMA (Strong’s procedure)
    2. Gastrojejunostomy (duodenal resection)
    3. Duodenojejunostomy

X. Resources

  1. Superior Mesenteric Artery Syndrome (NIH Rare Diseases)
    1. https://rarediseases.info.nih.gov/diseases/7712/superior-mesenteric-artery-syndrome

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Related Studies

Ontology: Superior Mesenteric Artery Syndrome (C0038828)

Definition (NCI) A very rare syndrome characterized by compression of the third portion of the duodenum against the aorta. The compression is caused by the superior mesenteric artery. It results in complete or partial duodenal obstruction. Signs and symptoms include nausea, vomiting, abdominal pain and distention, failure to gain weight, and weight loss.
Definition (MSH) DUODENAL OBSTRUCTION by the superior mesenteric artery (MESENTERIC ARTERY, SUPERIOR) which travels in the root of the MESENTERY and crosses over the DUODENUM. The syndrome is characterized by the dilated proximal duodenum and STOMACH, bloating, ABDOMINAL CRAMPS, and VOMITING. Often it is observed in patient with body casts after spinal surgery.
Concepts Disease or Syndrome (T047)
MSH D013478
SnomedCT 24988007, 197006009
English Cast Syndrome, Duodenal Compression Syndrome, Mesenteric, Mesenteric Duodenal Compression Syndrome, Syndrome, Cast, Superior mesenteric A.syndrome, SMAS - Sup mesent artery syndr, Superior Mesenteric Artery Syndrome [Disease/Finding], Wilkie Syndrome, Syndrome, Wilkie, cast syndrome, Superior mesenteric artery syndrome, SMAS - Superior mesenteric artery syndrome, Superior mesenteric artery syndrome (disorder), a.mesenterica superior; syndrome, mesenteric; vascular insufficiency syndrome, syndrome; mesenteric vascular insufficiency, syndrome; superior mesenteric artery, Superior Mesenteric Artery Syndrome
Swedish Arteria mesenterica superior-syndrom
Japanese ジョウチョウカンマクドウミャクショウコウグン, キャスト症候群, 躯幹ギプス症候群, Cast症候群, 上腸間膜動脈症候群, SMA症候群, 上腸間膜動脈性十二指腸閉塞, 腸間膜動脈性十二指腸イレウス, 腸間膜動脈性十二指腸狭窄, 腸間膜動脈性十二指腸閉塞, 腸間膜十二指腸圧迫症候群, 腸間膜性イレウス症候群, 十二指腸圧迫症候群-腸間膜
Czech syndrom arteria mesenterica superior, Syndrom arteria mesenterica superior
Finnish Ylemmän suolilievevaltimon oireyhtymä
Russian BRYZHEECHNOI VERKHNEI ARTERII SINDROM, BRYZHEIKI 12-TIPERSTNOI KISHKI SDAVLENIIA SINDROM, БРЫЖЕЕЧНОЙ ВЕРХНЕЙ АРТЕРИИ СИНДРОМ, БРЫЖЕЙКИ 12-ТИПЕРСТНОЙ КИШКИ СДАВЛЕНИЯ СИНДРОМ
Italian Sindrome del compasso aorto-mesenterico, Sindrome da compressione duodenale dell'arteria mesenterica, Sindrome di Wilkie, Sindrome dell'arteria mesenterica superiore
French SAMS, Syndrome de l'artère mésentérique supérieure, Syndrome de Wilkie, Syndrome du corset plâtré
Polish Zespół tętnicy krezkowej górnej
Hungarian Arteria mesenterica superior syndroma
Norwegian Arteria mesenterica superior-syndrom, Casts syndrom
Dutch a.mesenterica superior; syndroom, mesenteriaal; vasculair insufficiëntie syndroom, syndroom; arteria mesenterica superior, syndroom; mesenteriaal vasculaire insufficiëntie, arteria mesenterica superior syndroom, Arteria-mesenterica-superiorsyndroom, Duodenaal compressiesyndroom, mesenterisch, Mesenterisch duodenaal compressiesyndroom, Syndroom, arteria-mesenterica-superior-
Spanish síndrome de la arteria mesentérica superior (trastorno), síndrome de la arteria mesentérica superior, Síndrome de la arteria mesentérica superior, Síndrome de Compresión Duodenal Mesentérica, Síndrome de la Arteria Mesentérica Superior
Portuguese Síndrome da artéria superior mesentérica, Síndrome da Artéria Mesentérica Superior, Síndrome de Compressão Duodenal pela Artéria Mesentérica
German Arteria-mesenterica-superior-Kompressionssyndrom, Duodenumkompressionssyndrom, mesenteriales, Mesenterialarterien-Syndrom, oberes, Mesenteriales Duodenumkompressionssyndrom