Achalasia is a disorder of the esophagus that prevents normal swallowing.

Description

Achalasia affects the esophagus, the tube that carries swallowed food from the back of the throat down into the stomach. A ring of muscle called the lower esophageal sphincter encircles the esophagus just above the entrance to the stomach. This sphincter muscle is normally contracted to close the esophagus. When the sphincter is closed, the contents of the stomach cannot flow back into the esophagus. Backward flow of stomach contents (reflux) can irritate and inflame the esophagus, causing symptoms such as heartburn. The act of swallowing causes a wave of esophageal contraction called peristalsis. Peristalsis pushes food along the esophagus. Normally, peristalsis causes the esophageal sphincter to relax and allow food into the stomach. In achalasia, which means "failure to relax," the esophageal sphincter remains contracted. Normal peristalsis is interrupted and food cannot enter the stomach.

Causes and symptoms

Causes

Achalasia is caused by degeneration of the nerve cells that normally signal the brain to relax the esophageal sphincter. The ultimate cause of this degeneration is unknown. Autoimmune disease or hidden infection is suspected.

Symptoms

Dysphagia, or difficulty swallowing, is the most common symptom of achalasia. The person with achalasia usually has trouble swallowing both liquid and solid foods, often feeling that food "gets stuck" on the way down. The person has chest pain that is often mistaken for angina pectoris (cardiac pain). Heartburn and difficulty belching are common. Symptoms usually get steadily worse. Other symptoms may include nighttime cough or recurrent pneumonia caused by food passing into the lower airways.

Diagnosis

Diagnosis of achalasia begins with a careful medical history. The history should focus on the timing of symptoms and on eliminating other medical conditions that may cause similar symptoms. Tests used to diagnose achalasia include:

• Esophageal manometry. In this test, a thin tube is passed into the esophagus to measure the pressure exerted by the esophageal sphincter.
• X ray of the esophagus. Barium may be swallowed to act as a contrast agent. Barium reveals the outlines of the esophagus in greater detail and makes it easier to see its constriction at the sphincter.
• Endoscopy. In this test, a tube containing a lens and a light source is passed into the esophagus. Endoscopy is used to look directly at the surface of the esophagus. This test can also detect tumors that cause symptoms like those of achalasia. Cancer of the esophagus occurs as a complication of achalasia in 2-7% of patients.

Treatment

The first-line treatment for achalasia is balloon dilation. In this procedure, an inflatable membrane or balloon is passed down the esophagus to the sphincter and inflated to force the sphincter open. Dilation is effective in about 70% of patients.

Three other treatments are used for achalasia when balloon dilation is inappropriate or unacceptable.

• Botulinum toxin injection. Injected into the sphincter, botulinum toxin paralyzes the muscle and allows it to relax. Symptoms usually return within one to two years.
• Esophagomyotomy. This surgical procedure cuts the sphincter muscle to allow the esophagus to open. Esophagomyotomy is becoming more popular with the development of techniques allowing very small abdominal incisions.
• Drug therapy. Nifedipine, a calcium-channel blocker, reduces muscle contraction. Taken daily, this drug provides relief for about two-thirds of patients for as long as two years.

Prognosis

Most patients with achalasia can be treated effectively. Achalasia does not reduce life expectancy unless esophageal carcinoma develops.

Prevention

There is no known way to prevent achalasia.

Key Terms

Botulinum toxin

Any of a group of potent bacterial toxins or poisons produced by different strains of the bacterium . The toxins cause muscle paralysis.

Dysphagia

Difficulty in swallowing.

Endoscopy

A test in which a viewing device and a light source are introduced into the esophagus by means of a flexible tube. Endoscopy permits visual inspection of the esophagus for abnormalities.

Esophageal manometry

A test in which a thin tube is passed into the esophagus to measure the degree of pressure exerted by the muscles of the esophageal wall.

Esophageal sphincter

A circular band of muscle that closes the last few centimeters of the esophagus and prevents the backward flow of stomach contents.

Esophagomyotomy

A surgical incision through the muscular tissue of the esophagus.

Esophagus

The muscular tube that leads from the back of the throat to the entrance of the stomach.

Peristalsis

The coordinated, rhythmic wave of smooth muscle contraction that forces food through the digestive tract.

Reflux

An abnormal backward or return flow of a fluid.