Diffuse esophageal spasm

Specialty: Gastrointestinal.

• Retrosternal pain of esophageal origin
• symptomatic corkscrew esophagus
• spastic esophageal motor disorder

Why it occurs

• Uncoordinated, simultaneous, large-amplitude esophageal contractions of idiopathic origin (primary motor disorder)
• Severe gastroesophageal reflux that triggers reflex muscle spasms of the esophageal smooth muscles
• Psychogenic disorders or panic attacks with vagal hyperactivity
• Distal obstruction of the gastroesophageal junction (initial achalasia or early stages with vigorous esophageal contractions).

Initial workup

High resolution esophageal manometry (study of choice demonstrating repetitive premature contractions with reduced distal latency and preserved lower sphincter relaxation) | Barium esophagram (classic "corkscrew esophagus" or "rosary beads" image) | Upper gastrointestinal endoscopy (to rule out severe esophagitis, stricture or esophageal neoplasia causing pseudospasm) | Complete ischemic heart disease exclusion protocol (ECG, serial troponins, echocardiogram).

Standard management

• Sublingual or long-acting nitrates — sublingual nitroglycerin 0.4 mg during acute spasm, or isosorbide dinitrate to relax smooth muscles by stimulation of guanylate cyclase
• Calcium channel blockers — nifedipine 10-30 mg three times a day or diltiazem 60-90 mg three times a day before meals to attenuate contractile amplitude
• Tricyclic antidepressants at low doses — imipramine 10-25 mg at night to modulate esophageal visceral pain and reduce central pain perception
• Endoscopic injection of botulinum toxin into the muscles of the esophageal body in severe refractory cases.