Achalasia

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Introduction:

Achalasia is an esophageal disorder in which the contraction of esophageal muscles are no longer able to efficiently force food through to the stomach. The symptoms produced can vary, but may include: difficulty swallowing food and liquid; heartburn, chest pain that can mimic heart attack, regurgitation, coughing, and night time choking. The main medical treatments are: Heller's Myotomy to cut muscle, Dilatation to stretch muscle and Botox injections to relax muscle.

Achalasia is also known as: achalasia cardiae, cardiospasm, dyssynergia esophagus, esophageal achalasia, esophageal aperistalsis and megaesophagus.

Details:

Types Achalasia can be secondary or pseudo achalasia, in which case it is a symptom produced by another disorder. Achalasia can be primary, meaning it is not a symptom of another disorder. Primary achalasia is also called idiopathic achalasia, meaning there is no known cause. Primary achalasia is rare with only one new case per 100,000 per year. Treating secondary achalasia is sometimes done by treating the condition it is a symptom of. Treating secondary achalasia as a symptom is often the same as treating primary achalasia.

The muscles of the esophagus are unable to effectively push the contents of the esophagus through a valve, called the lower esophageal sphincter (LES), into the stomach. In secondary achalasia this can be caused by damage to the controlling nerves caused by something such as cancer or a response to Chagas' disease. In pseudo achalasia it could be due to the pressure of a tumor or a change in the muscle tissue due to scleroderma. In primary achalasia there is damage to the controlling nerves by a yet undetermined cause. Suspected causes are viral and autoimmune and inflammation due to an immune system response to an unknown stimulus.

The Problem Normally, after a swallow a wave of muscle contractions in the esophagus, called peristalsis, moves food much like a person would empty a tube of paste. When the contractions approach the valve above the stomach, (the LES), the valve relaxes from its normally constricted state and the food is able to pass through. In achalasia these contractions and the relaxing of the valve are either too weak, non-existent, or uncoordinated. The food is weakly pushed against a valve that is too tight to let it through. The food can become trapped in the esophagus and in some cases remain for days. In time with the esophageal muscles weak and the food pressing against the wall of the esophagus the esophagus may become dilated and curved.

Symptoms Achalasia can cause choking, aspiration and regurgitation (much like vomiting but only from the esophagus and not the stomach). It can also cause heartburn and pain that can be mistaken for heartburn or heart attack. The symptoms of achalasia are often worsened by stress or may become better or worse for a time for no apparent reason. Some people will progress very fast, while others will take years before even seeking medical help. When help is sought it sometimes takes years for a proper diagnosis. This is not because tests could not confirm a diagnosis but because many doctors do not test for achalasia and instead proceed with an eating disorder diagnosis or one of GERD.

Diagnosis Often the first diagnostic test to indicate achalasia is a barium swallow. It is a type of X-ray in which the patient is given a barium drink. The barium shows on the X-rays providing a view of the swallowing process and the shape of the esophagus. Typical finding of achalasia are: a dilated esophagus, narrowing where the esophagus meets the stomach, described as a "bird's beak," and failed or slow exit of the barium from the esophagus. The barium swallow may be followed with an endoscopy, which uses a small camera inserted through the mouth while the patient is under sedation. The endoscopist checks for signs of damage, cancer and how hard it is to pass the endoscope through the lower esophageal sphincter. A manometry is often done to confirm the suspected diagnosis. The manometry uses pressure sensors to test the constrictions and relaxations of the muscles.

Treatments There is no known cure for primary achalasia, though there are reports by people who seem to have been cured. At this time little is done to improve peristalsis beyond medications, such as Reglan. The main treatments focus on reducing the pressure of the LES. Often this is done by a surgery called Heller's myotomy (or Heller myotomy). In this surgery the muscle of, and near, the LES is cut to weaken it. Another treatment uses devices to stretch the LES and in that way weaken it. Stretching the the LES is called dilatation. See:Pneumatic dilatation for achalasia. Botox can also be injected into the LES to cause it to relax more. Botox injections are always temporary and may not work at all in some people. Medications and alternative treatments are sometimes used, but generally with limited success.

None of the treatments work for all people. In some cases they can lead to problems with acid reflux and GERD. Most patients eventually find treatment they consider successful. Over time more treatments may or may not be need. If the above treatments fail a few patients will need an esophagectomy to remove most of the esophagus. One of the most important factors for successful treatment is to have a doctor that has treated many people with achalasia. These doctors are rare and often travel is needed to reach them.

Etymology The word "achalasia" is from Greek “a”- not (failure or absence) +“chalasis” - loose (relaxed) = failure of relaxation. In the case of esophageal achalasia it refers to the failure of the LES to relax. Some dictionaries indicate that the "a" should be pronounced long while others say to pronounce it short. The word "achalasia" is used in the names of other disorders such as "anal achalasia"

International equivalents of "achalasia" are: acalasia, acalassia, achalasie, achalazja, achalazie, ackalasia, ahalazija, ahalaziya, akalasi, akalasia, akalazya, aхалазия, αχαλασία, chalasia, cardiospasmus, kardiospazm, kардиоспазм, megaaesophagus, and vélindislokakrampi.