What is Achalasia?

What is Achalasia?

Achalasia is a rare swallowing disorder that affects the esophagus. The esophagus is the hollow, muscular tube that moves food and liquid from your mouth into your stomach. A valve called the lower esophageal sphincter is at the bottom of the esophagus, which connects to the stomach. Achalasia occurs because the lower esophageal sphincter does not open properly.

Achalasia also causes the muscles in the esophagus to malfunction. Because of these two issues, food does not go into the stomach properly and can build up in the esophagus.

This disorder is rare, only affecting eight to 12 people per 100,000. It can occur at any age but is most commonly diagnosed between 25 and 60. Patients may have symptoms like difficulty swallowing food, food piling up, regurgitation, chest pain, and weight loss.

At NewYork-Presbyterian, we have a dedicated esophageal disorders center offering comprehensive diagnostic testing and advanced endoscopic and surgical treatments to relieve your achalasia symptoms — restoring your ability to eat and drink comfortably and enhancing the quality of your life.

Types of Achalasia

Types

All patients with achalasia have an abnormal lower esophageal sphincter (LES) that does not open to allow food out of the esophagus. The type of achalasia helps to indicate which treatment may be best. There are three types of achalasia:

  • Type I Achalasia: no contraction of the esophagus
  • Type II Achalasia: There are pan-pressurizations of the esophagus where the entire esophagus contracts at once.
  • Type III Achalasia: There are spastic contractions of the esophagus.

Signs & Symptoms of Achalasia

Symptoms

Achalasia symptoms develop slowly and can vary from patient to patient. The most common symptom is difficulty swallowing. Patients often feel that solid foods and liquids get stuck in the chest. Many people will start compensating by eating slowly, standing up or lifting their neck, or drinking carbonated beverages. People often do not seek help until symptoms advance.

Other achalasia symptoms are the result of abnormal contractions in the esophageal muscles. While symptoms of achalasia—such as chest pain and regurgitation—can mirror symptoms of gastroesophageal reflux disease (GERD), they are different.

Symptoms of achalasia can include:

What Causes Achalasia?

Causes

Achalasia occurs because of degeneration and inflammation of the nerve cells in the esophagus. This loss of nerves causes the lower esophageal sphincter to remain closed and the muscles of the esophagus not to contract normally. The exact cause of achalasia and this loss of nerves is unknown.

One theory is that achalasia is an autoimmune disease triggered by a virus. Another is that people with achalasia may have a genetic predisposition to develop the disease. This disease can also develop secondarily to other diseases, such as certain cancers or Chagas disease.

Risk Factors

Risk Factors

There are no direct causes of achalasia of the esophagus, and it is unclear what increases a person’s risk of developing the disease. However, there are a few factors involved:

  • Achalasia is equally common in men and women.
  • It can occur at any age but is most commonly diagnosed between the ages of 25 and 60.
  • It is possibly caused by viral infections in people with a genetic predisposition.
  • Chagas disease may be linked to secondary achalasia. Chagas disease is an infection caused by a parasite passed to people through the bite of an insect. It is mainly found in rural areas of Mexico and Central and South America.
  • Certain cancers can cause secondary achalasia, but this only accounts for 2-4% of all achalasia.

Complications

Complications

If achalasia is left untreated, the esophagus can get dilated as food builds up and is unable to drain into the stomach.

Most complications associated with achalasia are the result of food building up in the esophagus and ultimately being regurgitated. When the food is regurgitated, it can go into your lungs and cause an infection called aspiration pneumonia. This is more likely to happen when you are laying down.

Other complications include:

  • Weight loss and malnutrition
  • Aspiration pneumonia
  • Food impaction (food getting stuck in the esophagus and not being able to drain into the stomach)
  • Esophageal perforation (a hole in the esophagus)
  • Increased risk of esophageal cancer in patients with long-standing achalasia
Get Care

Trust NewYork-Presbyterian for Achalasia Care

Not all medical centers have clinicians with experience diagnosing and treating achalasia. Various treatments are available. At NewYork-Presbyterian, we are experienced in offering all achalasia therapies, including the latest approaches, and will match you with an expert in esophageal disorders. Call us today to make an appointment so you can start feeling better sooner.