Last updated: July 29, 2023

Summarytoggle arrow icon

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.

Definitiontoggle arrow icon

Epidemiologytoggle arrow icon

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

Epidemiological data refers to the US, unless otherwise specified.

Etiologytoggle arrow icon

CHAgas disease may lead to secondary aCHAlasia.

Pathophysiologytoggle arrow icon

Clinical featurestoggle arrow icon

  • Dysphagia to solids and liquids; can be progressive; or paradoxical dysphagia (difficulty swallowing liquids, while solids are easily swallowed)
  • 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.

Diagnosticstoggle arrow icon

Differential diagnosestoggle arrow icon

Differential diagnoses of achalasia

Esophageal motility disorders

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

Normal esophagus


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
  • N/A
  • See “Treatment” in achalasia.

The differential diagnoses listed here are not exhaustive.

Treatmenttoggle arrow icon

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.

If a high surgical risk

Complicationstoggle arrow icon

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

Referencestoggle arrow icon

  1. Sadowski DC, Ackah F, Jiang B, Svenson LW. Achalasia: incidence, prevalence and survival. A population-based study.. Neurogastroenterol Motil. 2010; 22 (9): p.e256-61.doi: 10.1111/j.1365-2982.2010.01511.x . | Open in Read by QxMD
  2. Facco M, Brun P, Baesso I, et al. T cells in the myenteric plexus of achalasia patients show a skewed TCR repertoire and react to HSV-1 antigens.. Am J Gastroenterol. 2008; 103 (7): p.1598-609.doi: 10.1111/j.1572-0241.2008.01956.x . | Open in Read by QxMD
  3. Kahrilas PJ, Kishk SM, Helm JF, Dodds WJ, Harig JM, Hogan WJ. Comparison of pseudoachalasia and achalasia.. Am J Med. 1987; 82 (3): p.439-46.doi: 10.1016/0002-9343(87)90443-8 . | Open in Read by QxMD
  4. de Oliveira RB, Rezende Filho J, Dantas RO, Iazigi N. The spectrum of esophageal motor disorders in Chagas' disease.. Am J Gastroenterol. 1995; 90 (7): p.1119-24.
  5. Costigan DJ, Clouse RE. Achalasia-like esophagus from amyloidosis. Successful treatment with pneumatic bag dilatation.. Dig Dis Sci. 1983; 28 (8): p.763-5.doi: 10.1007/BF01312569 . | Open in Read by QxMD
  6. Foster PN, Stewart M, Lowe JS, Atkinson M. Achalasia like disorder of the oesophagus in von Recklinghausen's neurofibromatosis.. Gut. 1987; 28 (11): p.1522-6.doi: 10.1136/gut.28.11.1522 . | Open in Read by QxMD
  7. Dufresne CR, Jeyasingham K, Baker RR. Achalasia of the cardia associated with pulmonary sarcoidosis.. Surgery. 1983; 94 (1): p.32-5.
  8. Vaezi MF, Pandolfino JE, Vela MF. Diagnosis and Management of Achalasia. The American Journal of Gastroenterology. 2013; 108: p.1238–1249.doi: 10.1038/ajg.2013.196 . | Open in Read by QxMD
  9. Rohof WOA, Bredenoord AJ. Chicago Classification of Esophageal Motility Disorders: Lessons Learned. Curr Gastroenterol Rep. 2017; 19 (8).doi: 10.1007/s11894-017-0576-7 . | Open in Read by QxMD
  10. Clément M, Zhu WJ, Neshkova E, Bouin M. Jackhammer Esophagus: From Manometric Diagnosis to Clinical Presentation. Canadian Journal of Gastroenterology and Hepatology. 2019; 2019: p.1-7.doi: 10.1155/2019/5036160 . | Open in Read by QxMD
  11. Carlson DA, Ravi K, Kahrilas PJ, et al. Diagnosis of Esophageal Motility Disorders: Esophageal Pressure Topography vs. Conventional Line Tracing. Am J Gastroenterol. 2015; 110 (7): p.967-977.doi: 10.1038/ajg.2015.159 . | Open in Read by QxMD
  12. Khalaf M, Chowdhary S, Elias PS, Castell D. Distal Esophageal Spasm: A Review. Am J Med. 2018; 131 (9): p.1034-1040.doi: 10.1016/j.amjmed.2018.02.031 . | Open in Read by QxMD

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