Achalasia

Definition: What Is Achalasia?

Achalasia is a disorder of the lower esophageal sphincter, which usually serves to seal between the esophagus and stomach to prevent stomach acid from entering the esophagus. The sphincter relaxes typically reflexively during swallowing to allow food to pass further into the stomach. If muscle relaxation is disturbed, food pulp can accumulate in the esophagus and cause discomfort.

Causes: How Does Achalasia Develop?

Achalasia often occurs without any identifiable cause. However, it is suspected that previous viral infections may have triggered an autoimmune reaction. The consequence is that nerve cells that usually have an inhibitory effect on the muscles of the lower esophagus perish.

Achalasia can also rarely result from esophageal cancer, which constricts the esophagus or destroys the nerve plexus in the esophagus that controls the esophagus muscles. Chagas disease, caused by parasites, can also cause symptoms resembling achalasia. It occurs mainly in Central and South America.

Achalasia Symptoms

Patients complain of difficulty swallowing (dysphagia) and often need to drink a lot of fluid after eating to swallow the food. After eating or while lying down, there may be reflux of food; this is called regurgitation, which is not to be confused with vomiting. There may be pressure behind the breastbone, and eating slowly will result in less severe symptoms. Stagnated food pulp may produce a breath odor, irritate the respiratory tract, and cause coughing.

The progression of symptoms may provide a clue. For example, suppose the symptoms develop gradually over months to years. In that case, this is more likely to indicate primary achalasia than esophageal cancer, leading to achalasia symptoms over weeks to months.

Diagnosis: How Is Achalasia Detected?

If the symptoms of achalasia occur, an endoscopy of the esophagus and, if necessary, of the stomach is performed. A camera tube is inserted into the esophagus, and samples are taken. Suppose no cause for the symptoms is found. In that case, a barium swallow test with an X-ray can make the remaining food in the esophagus visible (a typical image of achalasia is the champagne glass shape of the esophagus) and confirm the diagnosis.

With an accuracy of 90%, another diagnostic tool is manometry. It is a pressure measurement of the different sections of the esophagus. It can distinguish achalasia from other motor disorders of the esophagus (esophageal spasm, nutcracker esophagus).

Achalasia Therapy: Medication, Dilatation, or Surgery?

In the early stages, drug therapy with nifedipine, a calcium channel inhibitor, can reduce muscle tension and lower esophageal pressure. However, long-term results have not been good.

To treat achalasia, there is a possibility to dilate the narrowed muscle with a balloon probe (pneumatic dilatation). This method is successful in 90% of cases and can be repeated if necessary. If dilatation is not successful, a myotomy can be performed. This involves splitting the muscles of the lower esophageal sphincter longitudinally from the outside.

If dilatation or myotomy is not possible or desirable, Botox injection into the sphincter using an endoscope can decrease sphincter tightness. However, the effect lasts only six to twelve months and wears off with multiple applications.

Regular endoscopies must always follow achalasia therapy for control to detect esophageal cancer at an early stage if the risk is present.

What Are the Risks of Achalasia Patients?

The risk of achalasia is inflammation of the esophagus (esophagitis). It can develop because of the constant irritation caused by the remaining food. The consequence of the chronic inflammatory process is an increased risk of esophageal cancer. Another danger is that pent-up food pulp enters the respiratory tract and causes pneumonia (aspiration pneumonia). Suppose the achalasia was treated by balloon dilatation or myotomy. In that case, there is a 10% risk that the sphincter muscle can no longer fulfill its function of acid protection, and reflux disease (GERD) develops.