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Achalasia

Last updated: June 7, 2021

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Achalasia is a failure of the lower esophageal sphincter (LES) to relax that is caused by the degeneration of inhibitory neurons within the esophageal wall. It is classified as either primary (idiopathic) or secondary (in the context of another disease). In patients with achalasia, the chief complaint is dysphagia to both solids and liquids, although regurgitation, retrosternal pain, and weight loss may also occur. Upper endoscopy, esophageal barium swallow, and esophageal manometry play complementary roles in the diagnosis of achalasia. While upper endoscopy and/or esophageal barium swallow are often obtained initially, manometry usually confirms the diagnosis, and upper endoscopy is indicated to rule out a malignant underlying cause. In good surgical candidates, achalasia is usually treated with pneumatic dilation or myotomy. In most other cases, an injection of botulinum toxin is attempted. If these measures fail to provide relief, medical therapy (e.g., nifedipine) is indicated.

  • Rare disorder (∼ 1.6/100,000 individuals) [1]
  • Most commonly occurs in middle-aged individuals

Epidemiological data refers to the US, unless otherwise specified.

CHAgas disease may lead to secondary aCHAlasia.

  • Dysphagia to solids and liquids; can be progressive or paradoxical
  • Regurgitation
  • Retrosternal pain and cramps
  • Weight loss

Achalasia typically manifests with progressive dysphagia to solids and liquids while esophageal obstruction manifests with dysphagia to solids only.

Differential diagnoses of achalasia

Esophageal motility disorders

Esophageal motility disorders [9][10][11]
Characteristics

Normal esophagus

Achalasia

Diffuse esophageal spasm (distal esophageal spasm/corkscrew esophagus/rosary bead esophagus)

Hypercontractile esophagus (jackhammer esophagus)
Clinical features
  • None

Lower esophageal sphincter (LES) pressure and relaxation

  • Normal
  • LES pressure: high (failure to relax)
  • LES relaxation: incomplete/absent
  • Normal
  • Normal

Contraction waves

  • Progressive (toward the LES)
  • Simultaneous, nonprogressive

  • Simultaneous, nonprogressive, repetitive
  • Nonperistaltic contractions
  • Vigorous propagative contractions

Esophageal barium swallow

  • Normal
  • Typically normal

Esophageal manometry

  • Normal (40–100 mm Hg)
  • High LES resting pressure
  • High esophageal body pressure
  • Low peristaltic contraction pressure
  • Simultaneous multi-peak premature contractions
  • ≥ 10% of swallows have simultaneous contractions with mean amplitude ≥ 30 mm Hg. [12]
  • Intermittent normal peristalsis
  • N/a
High-resolution esophageal manometry [9]
  • Distal contractile integral (DCI) < 5000 mm Hg/sec/cm
  • Distal latency (DL) ≥ 4.5 seconds
  • DCI < 5000 mm Hg/sec/cm
  • DL < 4.5 seconds in at least 20% of swallows
  • DCl > 8000 mm Hg/sec/cm
Treatment
  • N/A
  • See “Treatment” in achalasia.

The differential diagnoses listed here are not exhaustive.

If a low surgical risk [8]

The preferred treatment often depends on the surgeon and the patient's situation. However, attempting pneumatic dilation before myotomy is gaining popularity because it is less invasive and the time of recovery is faster. This approach is already more popular in Europe.

  • Pneumatic dilation
    • Endoscope-guided graded dilation of the LES that tears the surrounding muscle fibers with the help of a balloon
    • The success rate at one month is ∼ 85%; perforation risk is ∼ 2%.
  • LES myotomy (Heller myotomy): a surgical procedure in which the lower esophageal sphincter is incised longitudinally to re-enable passage of food or liquids to the stomach.

If a high surgical risk

We list the most important complications. The selection is not exhaustive.

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