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Updated: Dec 10 2021

Diffuse Esophageal Spasm

  • Snapshot
    • A 49-year-old woman with depression and anxiety presents to the emergency room for chest pain. She reports that she was feeling well apart from intermittent difficulty swallowing. After her smoothie today, she experienced acute onset of retrosternal chest pain that radiated to her neck. She is worried that she is having a heart attack.
  • Introduction
    • Clinical definition
      • non-peristaltic contractions of the esophagus that are simultaneous or uncoordinated
    • Associated conditions
      • gastroesophageal reflux disease
      • depression and anxiety
      • neuromuscular or neurodegenerative disorders
  • Epidemiology
    • Incidence
      • likely underdiagnosed due to vague symptoms
    • Demographs
      • rare in children
      • more common in women
  • etiology
    • Pathogenesis
      • causes are unclear
      • often precipitated by ingestion of hot or cold liquids
      • possibly due to aberrant neurological signaling
  • Presentation
    • Symptoms
      • chest pain (non-exertional) that may radiate to arm, back, neck, or jaw
      • dysphagia
      • globus
    • Physical exam
      • no specific findings
  • imaging
    • Upper gastrointestinal barium swallow
      • “corkscrew” or “rosary bead esophagus” only during a spasm
        • not specific to diffuse esophageal spasm
  • Studies
    • Diagnostic testing
      • diagnostic approach
        • can be difficult to distinguish from cardiac chest pain and cardiac work up such as electrocardiogram (EKG), stress test, and even coronary angiography is often indicated and normal
      • other studies
        • esophageal manometry
          • diagnostic
          • shows normal lower esophageal sphincter and normal-amplitude, simultaneous contracts after a swallow
        • endoscopy
          • normal
  • Differential
    • Achalasia
      • distinguishing factor
        • increased lower esophageal sphincter function
    • Nutcracker esophagus
      • distinguishing factor
        • coordinated but high amplitude contractions
    • Angina and acute coronary syndrome
      • distinguishing factors
        • cardiac risk factors
        • ↑ cardiac enzymes
        • ST changes on EKG
  • Treatment
    • First-line
      • calcium channel blockers and nitrates
        • reduce the severity of spasm
      • proton pump inhibitor
        • treat associated reflux
      • tricyclic antidepressant
        • treat associated mood disorder
    • Interventional
      • endoscopic injection of botulinum toxin
      • myotomy
        • indicated for severe and incapacitating symptoms refractory to other treatment
  • Complications
    • Lack of response to treatment
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