Achalasia

(Esophageal Achalasia)

Definition

Achalasia is a relatively rare disorder of the smooth muscle of the esophagus. The esophagus is a muscular tube that carries food and liquids from the mouth to the stomach. Achalasia makes it difficult for food and liquid to pass into the stomach from the esophagus.

Causes

There is a muscle called the lower esophageal sphincter (LES). This is where the esophagus meets the stomach. When not swallowing, the LES remains closed to keep food, liquid, and stomach acid from moving back into the esophageal tube. When swallowing, nerve signals tell muscles to contract to push food down the esophagus (an action called peristalsis). This allows the LES to open.
In people with achalasia, the nerve cells in the lower esophageal tube and the LES do not work correctly. This results in:
  • Missing peristaltic (muscular) activity
  • Failure of the LES to open completely
While achalasia is associated with the loss of nerve cells in the esophagus, the cause of this process is unknown.

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition. Because the exact cause of achalasia is unknown, risk factors are unknown.

Symptoms

Symptoms of achalasia can occur between the ages of 25 and 60. Symptoms rarely develop in children. Symptoms tend to be mild at first, and then grow worse over months or years. The main symptom is difficulty swallowing solids and, as the disorder progresses, liquids. As many as 70% to 97% of patients with achalasia have difficulty swallowing both solids and liquids.
Other symptoms may include:
  • Discomfort or pain in the chest , especially after meals
  • Coughing, especially when lying down
  • Heartburn
  • Weight loss (as the disorder progresses)
  • Vomiting or regurgitating food or liquids. This may occur during sleep. This can cause a person to inhale food particles or liquid, which can lead to aspiration pneumonia and other respiratory infections.
Heartburn / Reflux Disease
Gastroesophageal Reflux
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Diagnosis

The doctor will ask about your symptoms and medical history and perform a physical exam. Tests may include:
  • Manometry—A tube is inserted down the throat to test the pressure in the esophagus and the stomach when swallowing.
  • Esophagram—X-rays are taken of the esophagus while you swallow barium (a thick liquid that lights up on x-rays).
  • Upper gastrointestinal endoscopy (esophagoscopy)—The esophagus is viewed directly through a fiberoptic tube to look for other causes of the symptoms.

Treatment

The goal of treatment is to make it easier for the LES to open. Treatment may include:

Pneumatic Dilation

This treatment stretches the LES muscle. A thin tube is inserted into the throat. At the end of this tube is an uninflated balloon. When the tube reaches the LES muscle, the balloon is inflated. Pneumatic dilation has a high success rate. It is the primary treatment in most patients, although the procedure may need to be repeated.

Surgery

Small incisions are made in the LES to help it relax. This is called Heller myotomy surgery. This can usually be performed via laparoscopy, making it a relatively minor surgical procedure. Because the LES is partially cut, about 15% of patients experience gastroesophageal reflux symptoms (ie, acid reflux) after this surgery.

Botulinum Toxin

Tiny amounts of botulinum toxin, type A, are injected into the LES. Botulinum causes the LES to relax, which makes it easier to open. Since the effect is temporary, repeat injections are almost always needed. The response decreases with successive injections.

Medication

Certain medications may help those that can not tolerate surgery and do not have success with botulinum therapy. The symptom relief is temporary.
Certain medications may help those that can not tolerate surgery and do not have success with botulinum therapy. The symptom relief is temporary.

Prevention

There are no guidelines to prevent achalasia because the cause is usually not known.

RESOURCES

American Academy of Family Physicians http://www.familydoctor.org

American College of Gastroenterology http://www.acg.gi.org/

American Gastroenterological Association http://www.gastro.org/

CANADIAN RESOURCES

The Canadian Association of Gastroenterology http://www.cag-acg.org/

Health Canada http://www.hc-sc.gc.ca/index-eng.php/

References

Achalasia. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated August 7, 2012. Accessed October 26, 2012.

Achalasia and esophageal motility disorders. The Society of Thoracic Surgeons website. Available at: http://www.sts.org/patient-information/esophageal-surgery/achalasia-and-esophageal-motility-disorders . Accessed October 26, 2012.

Vaezi MF, Pandolfino JE, Vela MF. Diagnosis and management of achalasia. Am J Gastro. 2013;108:1238-1249.

2/13/2014 DynaMed's Systematic Literature Surveillance http://www.ebscohost.com/dynamed: Vaezi M, Pandolfino J, et al. ACG Clinical Guideline: Diagnosis and Management of Achalasia. Am J Gastroenterol. online publication, 23 July 2013.

Revision Information

  • Reviewer: Daus Mahnke, MD
  • Review Date: 09/2013 -
  • Update Date: 02/13/2014 -