Superior Mesenteric Artery Syndrome

Aka: Superior Mesenteric Artery Syndrome, Cast Syndrome, Wilkie Syndrome, Mesenteric Duodenal Compression Syndrome, Duodenal Occlusion by Superior Mesenteric Artery

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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.

VII. Imaging

  1. XRay Abdomen
  2. CT Abdomen and Pelvis
  3. Abdominal Ultrasound

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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