ACHALASIA

Achalasia is a disorder of the esophagus, the tube that connects the mouth and stomach. With this disease, the esophagus has a decreased ability to move liquid and solids down to the stomach. This movement is known as peristalsis. Also the lower esophageal sphincter (the muscle between the esophagus and stomach) does not relax in response to swallowing. The causes of this disorder include damage to the nerves to the esophagus, parasitic infections, and hereditary factors. Achalasia may occur at any age but is more common as you grow older. The incidence of achalasia is 2 cases per 10,000 individuals.

Achalasia is detected by a barium swallow x-ray. On x-ray there is a narrowing of the lower portion of the esophagus and widening of the upper portion. A chest x-ray may be useful if it shows the esophagus is enlarged with air or fluid. Pressure measurements (manometry) may be done to prove the lack peristalsis and the increased pressure at the lower esophageal sphincter. Endoscopy, using a small light tube with a tiny video camera on the end, can be used to confirm a tight sphincter.

There is no cure for achalasia, but treatment can improve the symptoms in 60% to 85% of cases and help prevent complications.

The main symptom of achalasia is difficulty swallowing. Typically, problems with swallowing liquids occur first. It can progress to problems swallowing solid foods. When this occurs weight loss is not uncommon. Chest pain and pain on swallowing are not typical but can occur. Heartburn and belching are rare. In advanced cases, halitosis or bad breath can occur. Rarely does vomiting occur, but when it does it is caused by the overflow of food in the esophagus. Symptoms associated with the respiratory system such as coughing and wheezing can also occur.

Without treatment complications can arise. These include perforation (tearing) of the esophagus and regurgitation of acid or food from the stomach into the esophagus (gastroesophageal reflux disease [GERD]). Another complication is aspiration pneumonia. It can occur if the stomach contents are aspirated into the lungs. About 5% of patients with achalasia have a chance of developing esophageal cancer.

The goal of treatment is to reduce the pressure at the lower esophageal sphincter. This is done by dilating the sphincter with special weighted instruments or balloons that are inflated to dilate the sphincter. Even after dilation the esophagus will not have normal movement. The procedure may have to be repeated if symptoms reoccur. A rare complication of this procedure is esophageal perforation.

Medications such as long-acting nitrates or calcium channel blockers can also be used to lower the pressure at the lower esophageal sphincter. The medications usually are used in individuals who are unable to tolerate the dilation procedure.

Surgery to decrease the pressure in the lower sphincter (called an esophagomyotomy) may be indicated if other treatments fail.

• Seek medical advice.
• Eat and drink in an upright position.
• Eat and chew slowly.

• Avoid eating and drinking in a lying position.
• Avoid hot or cold liquids because they may make the condition worse.

• If you have persistent difficulty swallowing.
• If painful swallowing develops.
• If symptoms persist despite treatment for achalasia.
• If you are vomiting blood or other new symptoms develop.

National Digestive Diseases Information Clearinghouse
2 Information Way
Bethesda, MD 20892-3570
http://www.niddk.nih.gov
nddic@aerie.com



Esophageal achalasia. A, Early stage showing narrowing of lower esophagus. B, Advanced stage showing dilated middle esophagus. (From Phipps WJ, Cassmeyer VL, Sands JK, et al: Medical-surgical nursing: concepts and clinical practice, ed 5, St Louis, 1994, Mosby. Courtesy of Price SA, Wilson LM: Pathophysiology: clinical concepts of disease processes, ed 4, St Louis, 1992, Mosby. Used by permission.)

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