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Distal Esophageal Spasm

(Corkscrew Esophagus; Diffuse Esophageal Spasm)

By

Kristle Lee Lynch

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

Reviewed/Revised Feb 2024
View PATIENT EDUCATION

Symptomatic distal esophageal spasm (formerly called 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 of Distal Esophageal Spasm

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

When symptomatic, distal esophageal spasm typically causes substernal chest pain with dysphagia Dysphagia Dysphagia is difficulty swallowing. The condition results from impeded transport of liquids, solids, or both from the pharynx to the stomach. Dysphagia should not be confused with globus sensation... read more for both liquids and solids. Very hot or cold liquids may aggravate the pain. Over many years, this disorder rarely evolves into achalasia Achalasia Achalasia is a neurogenic esophageal motility disorder characterized by impaired esophageal peristalsis and a lack of lower esophageal sphincter relaxation during swallowing. Symptoms are slowly... read more 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 Angina Pectoris Angina pectoris is a clinical syndrome of precordial discomfort or pressure due to transient myocardial ischemia without infarction. It is typically precipitated by exertion or psychologic stress... read more , and patients often present to the emergency department concerned they are having a heart attack.

Diagnosis of Distal Esophageal Spasm

  • 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 biomarkers, stress testing—see diagnosis of acute coronary syndromes Diagnosis Acute coronary syndromes result from acute obstruction of a coronary artery. Consequences depend on degree and location of obstruction and range from unstable angina to non–ST-segment elevation... read more ). 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. Typically, barium swallow is done before manometry because it can be used to find other causes of symptoms and is less invasive.

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

Diagnosis reference

  • 1. Yadlapati R, Kahrilas PJ, Fox MR, et al: Esophageal motility disorders on high-resolution manometry: Chicago classification version 4.0. Neurogastroenterol Motil 33(1):e14058, 2021. doi: 10.1111/nmo.14058

Treatment of Distal Esophageal Spasm

  • 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. Oral calcium channel blockers may be useful. Anticholinergics, tricyclic antidepressants, nitroglycerin, and long-acting nitrates may also be tried but generally have limited success (1 Treatment references Symptomatic distal esophageal spasm (formerly called diffuse esophageal spasm) is part of a spectrum of motility disorders characterized variously by nonpropulsive contractions and hyperdynamic... read more ).

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

If medical management fails, a myotomy may be considered. A surgical or peroral endoscopic extended myotomy of the esophagus has been tried in severe cases (2 Treatment references Symptomatic distal esophageal spasm (formerly called diffuse esophageal spasm) is part of a spectrum of motility disorders characterized variously by nonpropulsive contractions and hyperdynamic... read more ).

Treatment references

Drugs Mentioned In This Article

Drug Name Select Trade
Deponit, GONITRO , Minitran, Nitrek, Nitro Bid, Nitrodisc, Nitro-Dur, Nitrogard , Nitrol, Nitrolingual, NitroMist , Nitronal, Nitroquick, Nitrostat, Nitrotab, Nitro-Time, RECTIV, Transdermal-NTG, Tridil
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NOTE: This is the Professional Version. CONSUMERS: View Consumer Version
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