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Diffuse Esophageal Spasm

(Corkscrew Esophagus)

By

Kristle Lee Lynch

, MD, Perelman School of Medicine at The University of Pennsylvania

Last full review/revision Sep 2020| Content last modified Sep 2020
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Symptomatic diffuse esophageal spasm is part of a spectrum of motility disorders characterized variously by nonpropulsive contractions and hyperdynamic contractions, sometimes in conjunction with elevated lower esophageal sphincter pressure. Symptoms are chest pain and sometimes dysphagia. Diagnosis is by barium swallow or manometry. Treatment is difficult but includes nitrates, calcium channel blockers, botulinum toxin injection, surgical or endoscopic myotomy, and antireflux therapy.

Abnormalities in esophageal motility correlate poorly with patient symptoms; similar abnormalities may cause different or no symptoms in different people. Furthermore, neither symptoms nor abnormal contractions are definitively associated with histopathologic abnormalities of the esophagus.

Symptoms and Signs

Sometimes, diffuse esophageal spasm is asymptomatic and is found incidentally.

When symptomatic, diffuse esophageal spasm typically causes substernal chest pain with dysphagia for both liquids and solids. Very hot or cold liquids may aggravate the pain. Over many years, this disorder may evolve into achalasia (with impaired esophageal peristalsis and a lack of lower esophageal sphincter relaxation during swallowing).

Esophageal spasms can cause severe pain without dysphagia. This pain is often described as a substernal squeezing pain and may occur in association with exercise. Such pain may be similar to angina pectoris, and patients often present to the emergency department concerned they are having a heart attack.

Diagnosis

  • Coronary ischemia ruled out

  • Barium swallow

  • Esophageal manometry

Alternative diagnoses include coronary ischemia, which always needs to be excluded by appropriate testing (eg, ECG, cardiac markers, stress testing—see diagnosis of acute coronary syndromes). Definitive confirmation of an esophageal origin for symptoms is difficult.

Barium swallow may show poor progression of a bolus and disordered, simultaneous contractions or tertiary contractions. Severe spasms may mimic the radiographic appearance of diverticula but vary in size and position.

Esophageal manometry provides the most specific description of the spasms. At least 20% of test swallows must have a short distal latency (< 4.5 seconds) to meet manometric criteria for diffuse esophageal spasm. However, spasms may not occur during testing.

Esophageal scintigraphy and provocative tests with drugs (eg, edrophonium chloride 10 mg IV) have not proved helpful.

Treatment

  • Calcium channel blockers

  • Botulinum toxin injection

  • Sometimes surgical or endoscopic myotomy

Esophageal spasms are often difficult to treat, and controlled studies of treatment methods are lacking. Anticholinergics, tricyclic antidepressants, nitroglycerin, and long-acting nitrates have had limited success. Calcium channel blockers given orally (eg, verapamil 80 mg 3 times a day, nifedipine 10 mg 3 times a day) may be useful.

Rarely, a trial of injecting botulinum toxin type A into the lower esophageal sphincter is done.

Medical management is usually sufficient, but surgical or peroral endoscopic myotomy along the full length of the esophagus has been tried in severe cases.

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NOTE: This is the Professional Version. CONSUMERS: Click here for the Consumer Version
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