Symptomatic Diffuse Esophageal Spasm
(Spastic Pseudodiverticulosis; Rosary Bead or Corkscrew Esophagus)
(See also Overview of Esophageal and Swallowing Disorders.)
Symptomatic diffuse esophageal spasm is part of a spectrum of motility disorders characterized variously by nonpropulsive contractions, hyperdynamic contractions, or 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, 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.
Diffuse esophageal spasm typically causes substernal chest pain with dysphagia for both liquids and solids. The pain may waken the patient from sleep. Very hot or cold liquids may aggravate the pain. Over many years, this disorder may evolve into achalasia.
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 indistinguishable from angina pectoris.
Alternative diagnoses include coronary ischemia, which may need 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 sec) 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.
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 tid, nifedipine 10 mg tid) may be useful, as may injection of botulinum toxin type A into the lower esophageal sphincter.
Medical management is usually sufficient, but surgical myotomy along the full length of the esophagus has been tried in intractable cases.