Updated: 10/28/2019

Diffuse Esophageal Spasm

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Snapshot
  • A 50-year-old woman with a history of acid reflux presents to her primary care physician for intermittent non-exertional chest pain. The pain is not associated with exercise or shortness of breath. She also reports that for the past few months, she has experienced the sensation of food stuck in her throat. She had been worried about her heart, and so she recently had an extensive work-up for coronary artery disease with her cardiologist, all of which were negative. Suspicious for an esophageal etiology for her chest pain, her physician orders an upper gastrointestinal barium swallow study, which shows a corkscrew-like esophagus. She is started on a calcium channel blocker.
Introduction
  • Overview
    • diffuse esophageal spasms are non-peristaltic contractions of the esophagus that are simultaneous or uncoordinated
  • Epidemiology
    • incidence
      • likely underdiagnosed due to vague symptoms
    • demographs
      • rare in children
      • more common in women
  • Pathogenesis
    • causes are unclear
    • often precipitated by ingestion of hot or cold liquids
    • possibly due to aberrant neurological signaling
  • Associated conditions
    • gastroesophageal reflux disease
    • depression and anxiety
    • neuromuscular or neurodegenerative disorders
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 study
    • findings
      • "corkscrew” or “rosary bead esophagus” only during a spasm
      • not specific to diffuse esophageal spasm
Studies
  • 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
  • Invasive studies
    • endoscopy
      • findings
        • normal
    • esophageal manometry
      • indications
        • diagnostic
      • findings
        • shows normal lower esophageal sphincter and normal-amplitude, simultaneous contracts after a swallow
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
  • Medical
    • calcium channel blockers
      • indication
        • reduces severity of spasm
    • nitrates
      • indication
        • reduces severity of spasm
    • proton pump inhibitor
      • indication
        • treats associated reflux
    • tricyclic antidepressant
      • indications
        • treats any associated mood disorder
        • second-line as this has increased side effect profile
  • Surgical
    • botulinum toxin (endoscopic injection)
      • indication
        • refractory to medical therapy
    • myotomy
      • indications
        • refractory to all the above
        • indicated for severe and incapacitating symptoms
Complications
  • Lack of response to treatment

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Questions (3)
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(M1.GI.13.59) A 64-year-old Caucasian male presents to the cardiologist complaining of chest pain. He describes the pain as spontaneous and radiating to his back, ears, and neck. He denies dyspnea on exertion. The patient is referred for an upper GI barium swallow, shown in image A. Which of the following would you most expect to find during further workup of this patient?

QID: 101116
FIGURES:
1

Abnormal electrocardiogram

2%

(1/62)

2

Abnormal coronary angiogram

3%

(2/62)

3

Abnormal esophageal biopsy

8%

(5/62)

4

Abnormal pulmonary function tests

2%

(1/62)

5

Abnormal esophageal manometry

84%

(52/62)

M 2 E

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Evidence (4)
EXPERT COMMENTS (2)
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