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.
- Topic reviewThe full article, organized the way the exam asks it.
- ABIM-format MCQsVignettes with an explanation for every wrong answer.
- Key factsThe numbers and thresholds worth memorizing, up front.
- AI tutorMedBrain answers the question you're stuck on.
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.
Read the whole article
Subscribing to Internal Medicine opens every article, the full question bank and MedBrain, on the web, iPhone and Android.
Related articles
- Achalasia
Achalasia is a primary esophageal motility disorder from myenteric plexus degeneration that causes failure of lower esophageal sphincter relaxation and aperistalsis, producing dysphagia to solids and liquids with regurgitation. Diagnosis requires endoscopy to exclude pseudoachalasia followed by esophageal manometry, and definitive treatment is pneumatic dilation, laparoscopic Heller myotomy, or POEM.
- Gastroesophageal reflux disease (GERD)
GERD is retrograde reflux of gastric contents into the esophagus causing heartburn and regurgitation, diagnosed clinically and confirmed by response to an empiric PPI trial, with endoscopy reserved for alarm features or refractory symptoms. Management proceeds from lifestyle measures through an 8-week once-daily PPI trial, dose escalation or agent switch for partial response, and fundoplication for medication-refractory or regurgitation-predominant disease.
- Vasospastic (Prinzmetal) angina
Vasospastic (Prinzmetal) angina is angina from transient epicardial coronary artery spasm, causing rest chest pain with transient ST elevation, typically at night or in the early morning. Diagnosis rests on documenting spasm or reversible ST changes, and treatment is calcium channel blockers and nitrates while strictly avoiding non-selective beta-blockers and triggers.
- Chest pain: an overview
Chest pain is a symptom, not a diagnosis, spanning cardiac, vascular, pulmonary, gastrointestinal, musculoskeletal, and psychiatric causes. The essential task is separating four do-not-miss diagnoses, acute coronary syndrome, aortic dissection, pulmonary embolism, and tension pneumothorax, from benign mimics with a structured history, an ECG within 10 minutes, and serial troponin, treating suspected acute coronary syndrome empirically with aspirin and nitroglycerin while the workup proceeds.
- Eosinophilic esophagitis
Eosinophilic esophagitis is a chronic, immune/antigen-mediated disease of the esophagus that presents with solid-food dysphagia and food impaction in young atopic men, diagnosed by endoscopic biopsy showing 15 or more eosinophils per high-power field after excluding other causes. Management follows the three D's: dietary elimination, drug therapy with a PPI and/or swallowed topical corticosteroids, and dilation for fibrostenotic strictures.
- Systemic sclerosis
Systemic sclerosis is a multiorgan disease of vascular injury and progressive fibrosis in which diffuse cutaneous disease extends skin thickening proximal to the elbows and knees and drives early visceral fibrosis. Diagnosis rests on the 2013 ACR/EULAR classification criteria and an antibody split (anti-Scl-70, anticentromere, anti-RNA polymerase III), and treatment is organ-specific: mycophenolate or nintedanib for interstitial lung disease, calcium channel blockers for Raynaud phenomenon, and captopril for scleroderma renal crisis.
- Dysphagia (oral and esophageal)
Dysphagia is difficulty swallowing, and the history alone localizes it to oropharyngeal disease, marked by choking or nasal regurgitation at the start of a swallow, or esophageal disease, marked by food sticking seconds later. Esophageal dysphagia with solids alone points to a mechanical obstruction worked up first with upper endoscopy, while dysphagia for solids and liquids together points to a motility disorder worked up with barium esophagram and manometry.
- Esophageal stricture
Esophageal stricture is a fixed luminal narrowing that produces progressive dysphagia to solids, most often a benign peptic stricture from chronic reflux but always requiring endoscopy with biopsy to exclude malignancy. Management is endoscopic dilation of the narrowed segment plus treatment of the underlying cause, with stenting or resection reserved for malignant obstruction.