What Is It?

Achalasia is an uncommon disorder of the smooth muscle of the esophagus, the muscular tube that extends from the mouth to the stomach. Normally, coordinated contractions of this smooth muscle, known as peristaltic waves, carry food into the stomach. Between the esophagus and stomach is a special muscle called the esophageal sphincter (LES) that surrounds the esophagus to keep it closed and prevent food and acid from splashing back up into the esophagus from the stomach. When you swallow, this sphincter relaxes and opens to allow food to pass into the stomach. When you swallow, nerves tell the muscles when to contract so that the contractions of the esophagus are coordinated with the relaxation and opening of the LES. In achalasia, the nerve cells in the lower two-thirds of the esophagus and LES are abnormal. This causes uncoordinated or weak peristaltic waves and causes the LES to remain closed, making it difficult for food to pass from the esophagus into the stomach.

The cause of achalasia is unknown. Studies show that there is degeneration and damage of the nerves that control the muscle contractions of the esophagus. The cause of the degeneration is unclear. There have been theories that a viral infection leads to nerve damage but these theories have not been proven. Another possibility is that the body’s immune system attacks and destroys the nerves. Approximately 2,000 new cases of achalasia are diagnosed each year in the United States.

Other conditions besides achalasia can cause the esophagus to function improperly, including diffuse esophageal spasm, polymyositis or dermatomyositis, hypothyroidism and scleroderma esophagus.

Most people with achalasia develop symptoms between the ages of 25 and 60, but the condition can occur in children. It does not run in families. The symptoms come on gradually and may take years to progress. Symptoms can include:

• Difficulty swallowing solid food (swallowing liquids is not affected in the early stages)
• Regurgitation or vomiting of undigested food
• Chest pain, discomfort, or fullness under the breastbone, especially following meals
• Coughing, especially at night or when lying down
• Difficulty swallowing solids and liquids (late in the illness)
• Weight loss (late in the illness)

Among the tests that may be needed to diagnose achalasia and rule out other conditions are the following:

• Esophagography (barium swallow) — You will swallow a thick liquid (barium) that can be seen on an X-ray. The test can show whether the esophagus is enlarged or dilated, and whether the barium is able to empty properly into the stomach. The study is generally painless, although some people experience the same discomfort they may have when swallowing foods or liquids.



• Endoscopy — Even if your medical history and the results of the barium swallow indicate you probably have achalasia, endoscopy usually is done to check for cancer, which could be keeping the LES closed, and other diseases such as infectious or inflammatory conditions associated with achalasia. This is an outpatient procedure. You will be sedated mildly as the doctor passes a flexible tube down your esophagus and looks at the lining of the esophagus and stomach. A piece of tissue (biopsy) may be taken to be examined under a microscope. One of the treatments for achalasia, balloon dilation, can be performed during endoscopy.



• Manometry — Manometry is a key test in diagnosing achalasia. A thin tube will be passed through your nose into your stomach, and pressure in your esophagus and at the LES will be recorded while you drink sips of water and the tube is slowly withdrawn. The pattern of pressure measurements can indicate whether a person has achalasia. Occasionally, a small injection of medication under the skin will be given to provoke the characteristic pressure readings of achalasia.

Achalasia generally progresses unless treated. After successful treatment, symptoms may still return five to 10 years later and require repeat treatments.

Since the cause of achalasia is unknown, there is no way to prevent it.

The choice of treatment method will depend on your general condition, your doctor’s expertise with various techniques, personal choice and prior treatments. Choices include:

• Pneumatic (balloon) dilation — This is widely thought to be the best initial treatment. In balloon dilation your doctor passes an endoscope, a flexible telescope, into your stomach while you are sedated, and then inflates a balloon at the level of the LES. The muscle fibers will be stretched, relieving the pressure that blocks food from passing easily into the stomach. Between 51 percent and 93 percent of people experience relief from their symptoms for several years following dilation. To maintain relief, repeat dilation may be necessary, or other treatments may be used. The chief risk of balloon dilation is a tear in the esophagus, which occurs in 2 percent to 3 percent of patients and requires emergency surgery.



• Surgery (Heller myotomy) — The LES also can be opened with surgery, called myotomy. In the past, surgery was reserved for those in whom balloon dilation was not successful. However, newer surgical techniques have led to improved outcomes with shorter hospital stays and lower risks, so the procedure is being used more often. Laparoscopic myotomy is performed with telescopic equipment inserted through small incisions in the abdomen. Most people have good to excellent results. Even with older forms of myotomy, benefits have been observed five years following surgery.



• Botulinum toxin — Tiny amounts of botulinum toxin are injected directly into the LES to paralyze and then relax the sphincter, allowing food to pass readily into the stomach. Botulinum (Botox) is expensive, however, and its effects are relatively short-lived. Only 32 percent of people receiving botulinum toxin do well 12 months later compared to 70 percent of those receiving pneumatic dilation.



• Other medications — Drugs that reduce LES pressure such as nifedipine (Adalat, Procardia) and nitrates (isosorbide or nitroglycerin, both sold under several brand names) may be useful. To be most effective, a tablet is dissolved under the tongue before meals. In studies, nifedipine benefited up to 75 percent of people, while 53 percent to 87 percent of people taking nitroglycerin or isosorbide tablets under the tongue improved.

You should call your doctor for an urgent evaluation if you experience any new chest pain, especially if it lasts for longer than five or 10 minutes, or if you cannot swallow liquids. If you experience unexplained weight loss, nighttime cough or pain, or difficulty in swallowing solid food, make an appointment to see your doctor for an evaluation.

Although there is no known cure for achalasia, several treatments can provide good to excellent relief from symptoms for a number of years. When treatment needs to be repeated, it can be as successful as initial treatment.

Diseases and Conditions Center

All ArmMed Media material is provided for information only and is neither advice nor a substitute for proper medical care. Consult a qualified healthcare professional who understands your particular history for individual concerns.