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ACHALASIA

 

Achalasia is the condition in which the lower esophageal sphincter (LES), a valve that opens and closes to allow food into the stomach, loses its ability to relax and stays closed. The result is that food and liquid collect in the esophagus, sometimes causing regurgitation. Over time, the esophagus, or food tube, will dilate, which can lead to complications. The disorder is characterized by loss of the wave-like contraction of smooth muscles that forces food through the digestive tract, peristalsis. The condition can also include spasms of the LES, and is caused by a lack of nervous stimulation of the esophagus. If not treated, achalasia can lead to cancer of the esophagus in very rare cases. Sometimes the opposite can also occur; a cancer occurring in the esophagus can impair nervous function and lead to a "secondary" achalasia.  Signs of achalasia can include difficulty in swallowing both liquids and solid food, regurgitation of undigested food, weight loss, malodorous breath (halitosis), weight loss, excessive burping or belching, or heartburn or pains in the chest that may be unrelated to mealtimes or one's particular diet.

Diagnostic tests

Signs of a poorly functioning esophagus such as a dilated esophagus with a "bird's beak" sign, retained food in the esophagus, and disordered or poor esophageal muscle contractions can be seen on upper GI series with esophagram.  The diagnosis is also made by evaluating for and excluding other disorders by endoscopy (EGD). Esophageal manometry is a test that measures the strength and pattern of muscle contractions in the esophagus. Scleroderma, esophageal spasm and cancer of the esophagus can present with similar symptoms; these tests can help differentiate these other possibilities.

Treatment

Options for therapy for achalasia include stretching the lower esophageal sphincter muscles  using a pneumatic dilator. It is a stiff balloon that is expanded until it opens the LES by partially disrupting, or ripping, these muscles. This is usually done on an outpatient basis in a hospital under X-ray guidance and with a sedative, while you are conscious. The risk of perforation with this procedure has been cited from two to twelve percent.  If the dilator perforates the esophagus, intravenous antibiotics and/or surgery may be required to repair the injury. For some people with achalasia, however, even multiple dilation attempts are unsuccessful, and surgical myotomy, where the muscle is cut surgically, may be needed. This procedure, which requires general anesthesia and several days of hospitalization, may result in problems with gastroesophageal reflux, due to the fact that the lower esophageal sphincter is damaged by design during the procedure.  Advanced centers can now attempt the procedure laparoscopically, dramatically cutting down the recovery times involved.

One treatment uses a medication called botulinum toxin, or Botox(R), injected directly into the esophagus.  Botulinum toxin acts on the nerves that regulate the lower esophageal sphincter, causing the sphincter to relax. Although its long-term results are not yet known, botulinum toxin has proven effective for several months to over a year at a time. This treatment's main drawbacks include its short duration as well as the ability of repeated treatments to produce an undesired reaction called tachyphylaxis, where the patient pay experience unwanted effects from a repeat exposure to the compound.  Repeat treatment is often needed at regular intervals after the first.

The outlook for those who suffer with achalasia is good,  although more than one session of esophageal dilation may be needed over the course of several weeks to adequately lower LES pressure to prevent blockage of the passage of foods through the esophagus. Usually, dilatation brings a permanent cure. However, as noted, the effect of botulinum toxin may wear off after several months; the medication's long-term success is not established, and the problem can come back over time.  It is wise to discuss options available, their risks and benefits, thoroughly with your doctor.