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Achalasia

  • Disorder of the esophagus that makes it hard to swallow food and liquids
  • Symptoms include difficulty swallowing, regurgitation of food and liquids, chest pain
  • Treatment includes medications, Botox injections, and minimally invasive surgical procedures
  • Involves thoracic surgery, gastroenterology, gastrointestinal motility program, advanced endoscopy program

Overview

If you are having a hard time swallowing food or beverages, one possible cause is a condition known as achalasia, a disorder of the esophagus, the muscular tube that transports food and liquid from the mouth to the stomach. In people with this condition, swallowing doesn’t fully empty the esophagus so food and liquids remain. This leads to a range of symptoms, including difficulty swallowing, chest pain, and the regurgitation—or spitting up—of undigested food and drinks. Over time, achalasia can progress, making swallowing increasingly difficult.

While achalasia can occur in people of any age, it is most commonly diagnosed in people between the ages of 25 and 60. Worldwide, it affects an estimated 1 out of every 100,000 people.

Fortunately, several treatments are available for achalasia, including medications, Botox injections, and minimally invasive surgical procedures.

What is achalasia?

When a person swallows food or fluid, it passes into the esophagus where muscles contract to push it down toward the stomach. When food reaches the end of the esophagus, it encounters the lower esophageal sphincter (LES), a muscular ring (or valve) that relaxes, or opens, to allow food to pass into the stomach and then contracts to keep stomach contents from refluxing into the esophagus. 

In achalasia, the LES does not open. As a result, some food and fluid remain in the esophagus. At the same time, the muscles involved in pushing food down the esophagus fail to contract properly. As a result, the movement of food through the esophagus toward the stomach is impaired. 

What causes achalasia?

Achalasia is caused by damage to or loss of the nerve cells that help regulate muscle contractions in the esophagus and the LES muscle.

Scientists do not yet know what causes these nerve cells to degenerate.

What are the symptoms of achalasia?

Symptoms of achalasia may include:

  • Difficulty swallowing liquids and solids
  • Regurgitation of undigested food or liquid
  • Chest pain
  • Cough, especially at night
  • Significant weight loss
  • Heartburn

People with achalasia are at increased risk for aspiration, because food or liquid may be retained in the esophagus and may come upwards, entering into the lungs. This can lead to lung infections, including pneumonia.

How is achalasia diagnosed?

To diagnose achalasia, your doctor will review your medical history and conduct a physical exam. He or she will ask about symptoms, including when they began and whether they have progressed over time.

A diagnosis of achalasia requires additional tests. These may include one or more of the following:

  • Barium swallow test (or barium esophagram). In this test, the patient swallows a thick, chalky liquid that contains barium. X-rays are taken as the barium moves through the esophagus and upper gastrointestinal tract. The barium helps some internal structures appear more clearly in the X-ray images and allows doctors to see the size of the esophagus, as well as the contractions of its muscles—and how well it empties.
  • Esophageal manometry (or esophageal motility study). This test is used to assess the contractions and relaxations of the muscles in the esophagus, including the LES. In this procedure, a doctor inserts a thin tube equipped with pressure sensors through the nose and guides it into the esophagus and stomach. While the tube is in place, the patient swallows several sips of water. The sensors measure muscle contractions and pressure as the water moves down the esophagus.
  • Upper endoscopy or EGD (esophagogastroduodenoscopy). In this procedure, a doctor inserts an endoscope—a thin, flexible tube equipped with a camera and light—into the mouth and down into the esophagus, stomach, and small intestine. The camera and light allow doctors to see inside the esophagus.

How is achalasia treated?

While there is no cure for achalasia, treatments are available that can help relieve symptoms by improving the passage of food and liquid from the esophagus into the stomach.

Treatments may include:

  • Heller myotomy. In this surgical procedure, a surgeon makes small cuts in the muscles of the LES (myotomy means “making a cut in a muscle”). This allows food and liquid to more easily pass into the stomach. Usually, this type of surgery can be done using a minimally invasive procedure called laparoscopy, which involves small incisions in the abdomen. In some cases, a Heller myotomy requires open surgery. A Heller myotomy can increase the risk of gastroesophageal acid reflux (GERD), which occurs when stomach acid flows up into the esophagus. To minimize reflux, surgeons often perform a procedure called fundoplication, in which a portion of the upper stomach is wrapped around the esophagus. This prevents stomach acid from entering the esophagus.
  • Peroral endoscopy myotomy (POEM). Similar to the Heller myotomy, in POEM, incisions are made in the muscle of the LES to allow easier passage of food and liquid from the esophagus into the stomach. The difference is that in POEM, the procedure is conducted using an endoscope that is passed through the mouth and down into the esophagus. This minimally invasive procedure does not involve any incisions in the skin and is performed using special techniques during an upper endoscopy. It may also increase the risk for gastroesophageal reflux.
  • Balloon (or pneumatic) dilation. This procedure is used to widen, or dilate, the LES, which can help relieve symptoms. A doctor uses an endoscope and a guide wire to insert a balloon across the LES. He or she then inflates it to stretch the LES muscle. This procedure may need to be repeated periodically to maintain relaxation of the LES.
  • Botulinum toxin (Botox). Botulinum toxin is injected into the LES muscle during an endoscopy. The toxin relaxes the sphincter so that food and liquid can pass into the stomach. Its effects are temporary and often last between six months to a year (or longer). Repeat injections may be an option for some patients.
  • Medications. Calcium channel blockers or nitrates can help the LES muscle relax and thereby improve the passage of food and liquid from the esophagus into the stomach. These drugs are not very effective and often used temporarily, while the patient waits for other treatment options.
  • Esophagectomy. Rarely, with long-standing untreated achalasia, severe widening of the esophagus may develop, resulting in severe symptoms. This is known as end-stage achalasia. In these cases, surgical removal of the esophagus, or esophagectomy, may be necessary. A portion of the patient’s stomach replaces the esophagus. This procedure is rarely performed for achalasia today.

What is the outlook for people with achalasia?

Once it develops, achalasia is a lifelong condition. Several types of treatment are available, however, that can relieve symptoms and help prevent serious complications, such as aspiration of food or liquid into the lungs.

Achalasia is also a progressive disorder and symptoms may worsen over time. In some cases, even after initial treatment, the condition may recur, and additional or alternative therapies may be necessary.

Some treatments for achalasia may cause gastroesophageal reflux, and achalasia may increase the risk for esophageal cancer. Because of this, it is important that people with achalasia get the condition treated and see their doctor for regular checkups and monitoring.

What makes Yale Medicine unique in its approach to achalasia?

“The Yale Esophageal Motility Program has extensive experience in the diagnosis and treatment of esophageal disease, including achalasia,” says Yale Medicine gastroenterologist Harry Aslanian, MD. “We work as a multidisciplinary team to provide state-of-the-art diagnosis and therapy, which can markedly improve swallowing in patients with achalasia.”