Gastroenterology· Topic 5.1 Reflux and dysphagia· 5.1.6

Distal esophageal spasm and hypercontractile esophagus

Distal esophageal spasm and hypercontractile (jackhammer) esophagus are spastic esophageal motility disorders that cause angina-like chest pain and intermittent dysphagia to solids and liquids, while ineffective esophageal motility is their weak, hypotonic counterpart. Diagnosis requires exclusion of cardiac disease, upper endoscopy to exclude structural disease, and high-resolution manometry classified by the Chicago classification; treatment escalates from acid suppression and smooth-muscle relaxants to botulinum toxin and peroral endoscopic myotomy.

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What this article covers

  • Overview

    Definition of distal esophageal spasm (formerly diffuse esophageal spasm): a motility disorder in which normal peristalsis is periodically interrupted by premature, rapid, simultaneous…

  • Epidemiology

    A leading esophageal cause of noncardiac chest pain: GERD is the most common cause of noncardiac chest pain, and esophageal motility disorders, particularly the hypercontractile disorders, are the next consideration.

  • Risk factors

    Acid reflux: reflux can precipitate esophageal spasm in some patients, the reason any reflux symptoms are treated first.

  • Pathophysiology

    Loss of inhibitory innervation first: impaired inhibitory (nitric oxide) innervation within the esophageal myenteric plexus removes the normal descending inhibition that makes contractions propagate in sequence.

  • Clinical features

    Intermittent retrosternal chest pain, the hallmark: angina-like, sudden and severe, unrelated to exertion, beginning seconds after a swallow; pain is always present in the spastic disorders.

  • Differential diagnosis

    Acute coronary syndrome or stable angina: exertional or ischemic pain with ECG changes, elevated troponin, or abnormal stress testing; must be excluded first (chest pain overview).

  • Diagnosis

    The diagnosis is established by high-resolution esophageal manometry graded by the Chicago classification, after cardiac and structural causes are excluded: Distal esophageal spasm: premature…

  • Management

    First, reassure once cardiac disease is excluded and remove triggers: reassurance that the heart is normal may be all that is needed; advise avoiding very hot or cold liquids and carbonated beverages and addressing stress.

  • Complications

    Missed myocardial ischemia: the most dangerous error, because spasm pain and angina share character and nitrate response.

  • Prognosis

    Most patients are managed medically: symptoms often respond to nitrates, calcium channel blockers, or low-dose antidepressants, and reassurance alone suffices for some.

  • Prevention

    No primary prevention exists: the cause of impaired inhibitory innervation is unknown.

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