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.
(See also Overview of Esophageal and Swallowing Disorders.)
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 for both liquids and solids. Very hot or cold liquids may aggravate the pain. Over many years, this disorder rarely evolves 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 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). 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 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 distal esophageal spasm. However, spasms may not occur during testing (1).
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
1).
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
1. Khalaf M, Chowdhary S, Elias PS, Castell D: Distal Esophageal Spasm: A Review. Am J Med 131(9):1034-1040, 2018. doi: 10.1016/j.amjmed.2018.02.031
2. Leconte M, Douard R, Gaudric M, et al: Functional results after extended myotomy for diffuse oesophageal spasm. Br J Surg 94(9):1113-1118, 2007. doi: 10.1002/bjs.5761
