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Achalasia

What is achalasia?
The esophagus is the muscular tube that extends from the neck to the abdomen and connects the throat to the stomach.  Achalasia is a condition where the esophagus is unable to move food into the stomach. The lower esophageal sphincter (LES), a valve located at the end of the esophagus, stays closed during swallowing, resulting in the back up of food.


Who is affected by achalasia?

Achalasia develops in about 3,000 people in the United States each year. It is typically diagnosed in adults, but can occur in children as well. There is no particular race or ethnic group that is affected, and the condition does not run in families.

Is achalasia serious?
Slowly, over a number of years, people with achalasia experience an increasing difficulty in eating solid food and in drinking liquids. As their condition advances, achalasia can cause considerable weight loss and malnutrition.People with achalasia also have a small increase in the risk of developing esophageal cancer, particularly if the obstruction has been present for a long time. Your physician may recommend regular endoscopic screenings for the prevention and early detection of esophageal cancer.

What causes achalasia?
Why esophageal muscles fail to contract normally in people with motility disorders, including achalasia, is unknown. Researchers think it may be linked to a virus, and recent studies show achalasia is caused by nerve cells of the involuntary nervous system within the muscle layers of the esophagus.  They are attacked by the patient’s own immune system and slowly degenerate for reasons that are not currently understood.

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What are the symptoms of achalasia?
Achalasia is a persistent problem, causing symptoms lasting months or years. People who experience only a brief episode of symptoms, such as difficulty swallowing, typically do not have a true esophageal motility disorder. The main symptoms of achalasia include:
  • Difficulty swallowing (dysphagia)
  • Regurgitation of undigested bitter food
  • Chest pain
  • Heartburn

How is achalasia diagnosed?
Three tests are most commonly used to diagnose and evaluate a swallowing problem:

  • Barium swallow. The patient swallows a barium preparation (liquid or other form) and its movement through the esophagus is evaluated using X-ray.
  • Endoscopy. A flexible, narrow tube called an endoscope is passed into the esophagus and projects images of the inside of the esophagus onto a screen.
  • Manometry. This test measures the timing and strength of esophageal (pump) contractions and lower esophageal sphincter (valve) relaxation.

How is achalasia treated?
If left untreated, achalasia can be debilitating. People with the condition experience considerable weight loss that can result in malnutrition. Lung infections and pneumonia due to aspiration of food can result, particularly in the elderly.

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There are several successful treatments available for achalasia, including:

  • Surgery -- The traditional surgical approach since the early 20th century to treat achalasia has been the Heller myotomy. In this achalasia surgery operation, the muscles of the valve between the esophagus into the stomach are cut. Traditionally, the Heller myotomy is completed through an open incision in the abdomen or through an incision in the left side of the chest between the ribs. These types of incisions often required hospitalization of up to a week for adequate recovery.
    Today, achalasia can be treated successfully by laparoscopy. Using five small incisions, a Heller myotomy can be accomplished (called laparoscopic Heller myotomy). It has been shown that the addition of a partial fundoplication (Dor) minimizes reflux and protects the esophagus from damaging gastroesophageal reflux.  This operation usually requires one day of hospital stay and recovery is typically accelerated when compared to conventional achalasia surgery.
  • Balloon dilation - Occasionally, achalasia can be treated non-surgically with balloon (pneumatic) dilation. While the patient is under light sedation, the gastroenterologist inserts a specially designed balloon through the lower esophageal sphincter and inflates it. The balloon disrupts the esophageal muscle and widens the opening for food to enter the stomach. Some patients may have to undergo repeated dilation treatments in order to achieve symptom improvement, and the treatment may have to be repeated every few years to ensure long-term results. On average, this procedure offers a 75 percent chance of relieving symptoms for a period of years. Risk associated with the procedure, however, is potential perforation of the esophagus.
  • Medication -- Patients who are not appropriate candidates for balloon dilation or surgery may benefit from Botox (botulinum toxin) injections.  Botox is a protein made by the bacteria that cause botulism. When injected into muscles in very small quantities, Botox can relax spastic muscles. It works by preventing nerves from sending signals to the muscles that tell them to contract. A smaller percentage of patients (up to 35 percent) achieve good short-term results using Botox compared to balloon dilation. In addition, the injections must be repeated frequently in order to achieve symptom relief.Other medications, such as nifedipine and nitroglycerin, may help to relax spastic esophageal muscles. Patients who take nifedipine every day may experience satisfactory results for a couple of years.
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Your health care team will discuss the possible risks and benefits of each treatment option with you.

It must be remembered that treatment of achalasia does not fix the esophagus, it only attempts to improve esophageal emptying.  This, however, is at the risk of allowing the stomach to empty it contents into the esophagus (reflux). 

Regardless of the therapy, long-term follow up is necessary to assure that the esophagus can be preserved as a passive conduit. This requires adequate esophageal emptying after the treatment of choice and prevention of gastroesophageal reflux. The obstructed esophagus or one that is subjected to reflux will dilate and expand and may eventually need to be replaced.

References:

The Society for Thoracic Surgeons. "Achalasia." (2006)
http://www.sts.org/doc/4120
 
Rice TW, McKelvey AA, Richter JE, Baker ME, Vaezi MF, Feng J, Murthy SC, Mason DP, Blackstone EH. A physiologic clinical study of achalasia: should Dor fundoplication be added to Heller myotomy? J Thorac Cardiovasc Surg, 2005 Dec;130(6):1593-600.
 
Pasricha PJ. "Achalasia." www.UptoDate.com
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This information is provided for education purposes only and is not intended to replace the medical advice of your doctor or health care provider. While we strive to keep our website current, medical practices sometimes change quickly. Please consult your health care provider for advice about a specific medical condition.