Achalasia

What is it?

Achalasia is an uncommon disorder of the esophagus. The disorder makes it difficult for food to pass from the esophagus into the stomach.

The esophagus is a muscular tube. It carries food from the mouth to the stomach. Normally, coordinated contractions of smooth muscle move food through the esophagus. These contractions are called peristaltic waves.

Between the esophagus and stomach is a muscle called the lower esophageal sphincter (LES). The sphincter surrounds the esophagus. It keeps the esophagus closed. This prevents food and acid from splashing back up into the esophagus from the stomach.

When you swallow, this sphincter relaxes. It opens to allow food to pass into the stomach. At the same time, nerves coordinate the contractions of the esophagus. This moves food into the stomach when the sphincter opens.

In achalasia, the nerve cells in the lower two-thirds of the esophagus and the sphincter are abnormal. This causes uncoordinated or weak peristaltic waves. It also causes the sphincter to remain closed.

The cause of achalasia is unknown. It does not run in families.

Most people with achalasia develop symptoms between the ages of 25 and 60.

Symptoms

The symptoms of achalasia come on gradually. They 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
• difficulty belching
• difficulty swallowing solids and liquids (late in the illness)
• weight loss (late in the illness).

Diagnosis

Tests will be done to diagnose achalasia. These tests will also look for other conditions that could be causing the symptoms.

Tests include:

• 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. It will also show whether the barium is able to empty properly into the stomach.

The study is generally painless. Some people with achalasia experience discomfort, similar to what they feel when swallowing foods or liquids.

• Endoscopy. Even if your medical history and barium swallow suggest achalasia, endoscopy usually is done. Endoscopy allows the doctor to see if some other problem might be causing the narrowed esophagus.

Endoscopy is an outpatient procedure. You will be sedated as the doctor passes a flexible tube down your esophagus. He or she will look at the lining of the esophagus and stomach. A piece of tissue (biopsy) may be taken to be examined under a microscope.

Balloon dilation, a treatment for achalasia, can be done during endoscopy.

• Manometry. Manometry is a key test in diagnosing achalasia. A thin tube will be passed through your nose into your stomach. Pressure in your esophagus and at the sphincter will be recorded while you drink sips of water. The tube will be slowly withdrawn. The pattern of pressure measurements can indicate whether a person has achalasia.

Expected duration

Achalasia generally worsens unless treated.

Even after successful treatment, symptoms may still return five to 10 years later. They may require repeat treatments.

Prevention

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

Treatment

The choice of treatment method will depend on

• your general condition
• your doctor's expertise with various techniques
• personal choice
• prior treatments.

Treatment options include:

• Pneumatic (balloon) dilation. This is widely thought to be the best nonsurgical treatment. Your doctor passes an endoscope into your stomach while you are sedated. He or she then inflates a balloon at the esophageal sphincter. The muscle fibers are stretched. This relieves the pressure that blocks food from passing easily into the stomach.

Most patients experience relief from their symptoms for several years following dilation. The procedure may have to be repeated. Other treatments also may be needed.

The chief risk of balloon dilation is a tear in the esophagus, which occurs in a small number of patients. This requires emergency surgery.

• Surgery (Heller myotomy). The esophageal sphincter can be opened with surgery, called myotomy. Newer surgical techniques have led to improved outcomes with shorter hospital stays and lower risks.

Myotomy can be done laparoscopically. This means telescopic equipment is inserted through small incisions in the abdomen. Most people have good to excellent results.

• Botulinum toxin. Tiny amounts of botulinum toxin are injected directly into the esophageal sphincter. This paralyzes and then relaxes the sphincter, allowing food to pass readily into the stomach.

• Other medications. Drugs can be taken to reduce pressure at the esophageal sphincter. They include nifedipine (Adalat, Procardia) and nitrates (isosorbide or nitroglycerin). Improvements with these medications are quite variable. They are seldom used as primary therapy today.

When to call a professional

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
• cannot swallow liquids.

Make an appointment to see your doctor for an evaluation if you experience

• unexplained weight loss
• nighttime cough or pain
• difficulty swallowing solid food.

Prognosis

There is no known cure for achalasia. But 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.

Additional info

American College of Gastroenterology (ACG)
http://www.acg.gi.org/

American Gastroenterological Association
https://gi.org/

Society of Thoracic Surgeons
https://www.sts.org/