eMedicine Specialties > Gastroenterology > Esophagus

Achalasia

Author: Piero Marco Fisichella, MD, Assistant Professor of Surgery, Stritch School of Medicine, Loyola University; Director, Esophageal Motility Center, Loyola University Medical Center
Coauthor(s): Marco G Patti, MD, Professor of Surgery, Director, Center for Esophageal Diseases, University of Chicago Pritzker School of Medicine
Contributor Information and Disclosures

Updated: Dec 1, 2009

Introduction

Background

Sir Thomas Willis described achalasia in 1672. In 1881, von Mikulicz described the disease as a cardiospasm to indicate that the symptoms were due to a functional problem rather than a mechanical one. In 1929, Hurt and Rake realized that the disease was caused by a failure of the lower esophageal sphincter (LES) to relax. They coined the term achalasia, meaning failure to relax.

Achalasia is a primary esophageal motility disorder characterized by failure of a hypertensive LES to relax and the absence of esophageal peristalsis. These abnormalities cause a functional obstruction at the gastroesophageal junction. (See images below and Images 1-2.)

Barium swallow demonstrating the bird-beak appear...

Barium swallow demonstrating the bird-beak appearance of the lower esophagus, dilatation of the esophagus, and stasis of barium in the esophagus.

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Barium swallow demonstrating the bird-beak appear...

Barium swallow demonstrating the bird-beak appearance of the lower esophagus, dilatation of the esophagus, and stasis of barium in the esophagus.


Manometric evaluation of the esophagus in a patie...

Manometric evaluation of the esophagus in a patient with achalasia. Pertinent findings include absence of propulsive peristalsis in the body of the esophagus (note simultaneous contractions), elevated resting lower esophageal sphincter (LES) pressure, and the absence of LES relaxation.

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Manometric evaluation of the esophagus in a patie...

Manometric evaluation of the esophagus in a patient with achalasia. Pertinent findings include absence of propulsive peristalsis in the body of the esophagus (note simultaneous contractions), elevated resting lower esophageal sphincter (LES) pressure, and the absence of LES relaxation.


Pathophysiology

LES pressure and relaxation are regulated by excitatory (eg, acetylcholine, substance P) and inhibitory (eg, nitric oxide, vasoactive intestinal peptide) neurotransmitters. Persons with achalasia lack nonadrenergic, noncholinergic, inhibitory ganglion cells, causing an imbalance in excitatory and inhibitory neurotransmission. The result is a hypertensive nonrelaxed esophageal sphincter.

Frequency

United States

The incidence of achalasia is approximately 1 per 100,000 people per year.

International

Chagas disease may cause a similar disorder.

Sex

The male-to-female ratio of achalasia is 1:1.

Age

Achalasia typically occurs in adults aged 25-60 years. Fewer than 5% of cases occur in children.

Clinical

History

Achalasia is characterized by the following symptoms and signs1 :

  • Dysphagia (most common)
  • Regurgitation
  • Chest pain
  • Heartburn
  • Weight loss

Physical

Physical examination is noncontributory.

Causes

The cause of achalasia is unknown.

More on Achalasia

Overview: Achalasia
Differential Diagnoses & Workup: Achalasia
Treatment & Medication: Achalasia
Follow-up: Achalasia
Multimedia: Achalasia
References
Further Reading
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References

  1. Ferri LE, Cools-Lartigue J, Cao J, Miller L, Mayrand S, Fried GM, et al. Clinical predictors of achalasia. Dis Esophagus. Aug 28 2009;[Medline].

  2. Ayazi S, Crookes PF. High-resolution esophageal manometry: using technical advances for clinical advantages. J Gastrointest Surg. Sep 18 2009;[Medline].

  3. Kroupa R, Hep A, Dolina J, Valek V, Matyasova Z, Prokesova J, et al. Combined treatment of achalasia - botulinum toxin injection followed by pneumatic dilatation: long-term results. Dis Esophagus. Aug 28 2009;[Medline].

  4. Pastor AC, Mills J, Marcon MA, Himidan S, Kim PC. A single center 26-year experience with treatment of esophageal achalasia: is there an optimal method?. J Pediatr Surg. Jul 2009;44(7):1349-54. [Medline].

  5. Eckardt AJ, Eckardt VF. Current clinical approach to achalasia. World J Gastroenterol. Aug 28 2009;15(32):3969-75. [Medline]. [Full Text].

  6. Cowgill SM, Villadolid D, Boyle R, Al-Saadi S, Ross S, Rosemurgy AS 2nd. Laparoscopic Heller myotomy for achalasia: results after 10 years. Surg Endosc. Jun 24 2009;[Medline].

  7. Richards WO, Torquati A, Holzman MD, Khaitan L, Byrne D, Lutfi R, et al. Heller myotomy versus Heller myotomy with Dor fundoplication for achalasia: a prospective randomized double-blind clinical trial. Ann Surg. Sep 2004;240(3):405-12; discussion 412-5. [Medline].

  8. Torquati A, Lutfi R, Khaitan L, Sharp KW, Richards WO. Heller myotomy vs Heller myotomy plus Dor fundoplication: cost-utility analysis of a randomized trial. Surg Endosc. Mar 2006;20(3):389-93. [Medline].

  9. Sweet MP, Nipomnick I, Gasper WJ, Bagatelos K, Ostroff JW, Fisichella PM, et al. The outcome of laparoscopic Heller myotomy for achalasia is not influenced by the degree of esophageal dilatation. J Gastrointest Surg. Jan 2008;12(1):159-65. [Medline].

  10. Abid S, Champion G, Richter JE, McElvein R, Slaughter RL, Koehler RE. Treatment of achalasia: the best of both worlds. Am J Gastroenterol. Jul 1994;89(7):979-85. [Medline].

  11. Benini L, Sembenini C, Castellani G, Bardelli E, Brentegani MT, Giorgetti P, et al. Pathological esophageal acidification and pneumatic dilitation in achalasic patients. Too much or not enough?. Dig Dis Sci. Feb 1996;41(2):365-71. [Medline].

  12. Gelfond M, Rozen P, Gilat T. Isosorbide dinitrate and nifedipine treatment of achalasia: a clinical, manometric and radionuclide evaluation. Gastroenterology. Nov 1982;83(5):963-9. [Medline].

  13. Hunter JG, Trus TL, Branum GD, Waring JP. Laparoscopic Heller myotomy and fundoplication for achalasia. Ann Surg. Jun 1997;225(6):655-64; discussion 664-5. [Medline].

  14. Katz PO, Gilbert J, Castell DO. Pneumatic dilatation is effective long-term treatment for achalasia. Dig Dis Sci. Sep 1998;43(9):1973-7. [Medline].

  15. Moonka R, Patti MG, Feo CV, Arcerito M, De Pinto M, Horgan S, et al. Clinical presentation and evaluation of malignant pseudoachalasia. J Gastrointest Surg. Sep-Oct 1999;3(5):456-61. [Medline].

  16. Patti MG, Arcerito M, Tong J, De Pinto M, de Bellis M, Wang A, et al. Importance of preoperative and postoperative pH monitoring in patients with esophageal achalasia. J Gastrointest Surg. Nov-Dec 1997;1(6):505-10. [Medline].

  17. Patti MG, Feo CV, Arcerito M, De Pinto M, Tamburini A, Diener U, et al. Effects of previous treatment on results of laparoscopic Heller myotomy for achalasia. Dig Dis Sci. Nov 1999;44(11):2270-6. [Medline].

  18. Patti MG, Fisichella PM, Perretta S, Galvani C, Gorodner MV, Robinson T, et al. Impact of minimally invasive surgery on the treatment of esophageal achalasia: a decade of change. J Am Coll Surg. May 2003;196(5):698-703; discussion 703-5. [Medline].

  19. Patti MG, Pellegrini CA, Arcerito M, Tong J, Mulvihill SJ, Way LW. Comparison of medical and minimally invasive surgical therapy for primary esophageal motility disorders. Arch Surg. Jun 1995;130(6):609-15; discussion 615-6. [Medline].

  20. Smith CD, Stival A, Howell DL, Swafford V. Endoscopic therapy for achalasia before Heller myotomy results in worse outcomes than heller myotomy alone. Ann Surg. May 2006;243(5):579-84; discussion 584-6. [Medline].

  21. Spiess AE, Kahrilas PJ. Treating achalasia: from whalebone to laparoscope. JAMA. Aug 19 1998;280(7):638-42. [Medline].

  22. Stewart KC, Finley RJ, Clifton JC, Graham AJ, Storseth C, Inculet R. Thoracoscopic versus laparoscopic modified Heller Myotomy for achalasia: efficacy and safety in 87 patients. J Am Coll Surg. Aug 1999;189(2):164-9; discussion 169-70. [Medline].

  23. Vaezi MF, Richter JE, Wilcox CM, Schroeder PL, Birgisson S, Slaughter RL, et al. Botulinum toxin versus pneumatic dilatation in the treatment of achalasia: a randomised trial. Gut. Feb 1999;44(2):231-9. [Medline].

  24. Woltman TA, Oelschlager BK, Pellegrini CA. Surgical management of esophageal motility disorders. J Surg Res. Mar 2004;117(1):34-43. [Medline].

  25. Zaninotto G, Annese V, Costantini M, Del Genio A, Costantino M, Epifani M, et al. Randomized controlled trial of botulinum toxin versus laparoscopic heller myotomy for esophageal achalasia. Ann Surg. Mar 2004;239(3):364-70. [Medline].

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Further Reading

Clinical guidelines
Guidelines for oesophageal manometry and pH monitoring.
British Society of Gastroenterology - Medical Specialty Society. 2006 Nov. 11 pages. NGC:007150

Esophageal dilation.
American Society for Gastrointestinal Endoscopy - Medical Specialty Society. 2006 May. 6 pages. NGC:004974

Clinical trials
Outcome of Symptoms in Patients Undergoing a Heller Myotomy (Achalasia)

A Randomized Comparison of Laparoscopic Myotomy and Pneumatic Dilatation for Achalasia

Do Patients Who Have Had Surgery for Achalasia Suffer From Reflux


Related eMedicine topics
 Achalasia

Esophageal Motility Disorders

Swallowing Disorders

Esophageal Spasm

Gastroesophageal Reflux Disease


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Keywords

achalasia, fundoplication, myotomy, Heller myotomy, lower esophageal sphincter, esophageal sphincter, esophageal peristalsis, swallowing disorder, esophageal manometry, esophageal motility, gastroesophageal reflux, dysphagia, esophagus achalasia, esophageal achalasia, esophageal motility disorder

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Contributor Information and Disclosures

Author

Piero Marco Fisichella, MD, Assistant Professor of Surgery, Stritch School of Medicine, Loyola University; Director, Esophageal Motility Center, Loyola University Medical Center
Piero Marco Fisichella, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, Association for Academic Surgery, Society for Surgery of the Alimentary Tract, and Society of American Gastrointestinal and Endoscopic Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Marco G Patti, MD, Professor of Surgery, Director, Center for Esophageal Diseases, University of Chicago Pritzker School of Medicine
Marco G Patti, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Surgeons, American Gastroenterological Association, American Medical Association, American Surgical Association, Association for Academic Surgery, Pan-Pacific Surgical Association, Society for Surgery of the Alimentary Tract, Society of American Gastrointestinal and Endoscopic Surgeons, Southwestern Surgical Congress, and Western Surgical Association
Disclosure: Nothing to disclose.

Medical Editor

David Eric Bernstein, MD, Chief, Section of Hepatology, North Shore University Hospital, Director, Associate Professor, Department of Internal Medicine, Division of Hepatology, New York University School of Medicine
David Eric Bernstein, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

James L Achord, MD, Professor Emeritus, Department of Medicine, Division of Digestive Diseases, University of Mississippi School of Medicine
James L Achord, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Medical Association, American Society for Gastrointestinal Endoscopy, Mississippi State Medical Association, New York Academy of Sciences, Sigma Xi, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law Medicine and Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

 
 
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