Epidemiology & Risk Factors
Associated w/ anxiety, depression, somatic symptom disorder , and panic disorder.
Triggered by emotional stress , rapid eating, or ingestion of very cold/hot liquids .
Most common in middle-aged adults; slightly higher prevalence in females.
The primary defect in Distal Esophageal Spasm (DES) is the degeneration of inhibitory nitric oxide-producing postganglionic neurons in the esophageal myenteric plexus.
Clinical Features
Intermittent retrosternal chest pain :
Crushing, pressure-like pain that radiates to the back, jaw, or arms (mimics angina ).
Relieved by nitroglycerin or CCBs (due to smooth muscle relaxation, often causing diagnostic confusion with CAD ).
Intermittent dysphagia to both solids and liquids.Regurgitation and heartburn may co-occur.
Diagnosis
Initial Step : Rule out cardiac etiology (ECG, troponins) in any pt presenting with acute retrosternal chest pain .Initial Imaging : Barium esophagram (swallow) showing classic “corkscrew” or “rosary bead” appearance (due to simultaneous, non-peristaltic contractions).Confirmatory/Gold Standard : Esophageal manometry showing high-amplitude, simultaneous, non-peristaltic contractions (or shortened distal latency < 4.5 seconds on high-resolution manometry).Upper Endoscopy (EGD) : Typically normal; performed to rule out structural causes (malignancy, strictures, eosinophilic esophagitis ).
Differential Diagnostics
Angina Pectoris / Myocardial Infarction
Diff by exertional onset, ECG changes, (+) troponins, and absence of dysphagia.
Achalasia :
Diff by progressive dysphagia (solids and liquids), weight loss, “bird’s beak” deformity on barium swallow, and manometry showing aperistalsis + incomplete LES relaxation.
Hypercontractile (Jackhammer/Nutcracker) Esophagus :
Diff by high-amplitude, high-energy contractions that remain peristaltic (coordinated) on manometry.
GERD :
Diff by predominant heartburn/regurgitation, response to PPIs, and absence of characteristic manometric spasm features.
Management
First-line : Calcium channel blockers (e.g., diltiazem) to relax esophageal smooth muscle. Sublingual nitroglycerin or isosorbide dinitrate as-needed for acute chest pain episodes.Alternative First-line : Tricyclic antidepressants (TCAs) (e.g., imipramine) at low doses to reduce visceral hypersensitivity and chest pain.Second-line : Endoscopic botulinum toxin injection into the lower esophagus to decrease contraction amplitude.Refractory/Severe : Peroral endoscopic myotomy (POEM) or surgical myotomy (Heller myotomy).
Complications
Significant weight loss and malnutrition (due to fear of eating/odynophagia).
Epiphrenic diverticula (pulsion diverticula formed due to high intraesophageal pressures).
Aspiration.