What is achalasia?

Achalasia is a rare disorder of the esophagus that makes swallowing difficult. This occurs because the nerves of the esophagus do not work. Children suffering from achalasia cannot sufficiently push food down toward the stomach because of a lack of nerves, an enlarged esophagus and the failure of the lower-esophageal muscle to relax and allow food to pass into the stomach. Children with achalasia can have complications like malnutrition, chest pain and respiratory difficulties. It is a progressive disease, which means it can get worse if not treated.

The causes of achalasia are not known.

What are the symptoms of achalasia?

The symptoms of achalasia can appear in infants, or they can gradually appear in childhood or adulthood. The most common symptoms are:

  • Difficulty swallowing liquids or solids
  • Feeling food is stuck in the chest
  • Chest pain
  • Regurgitation of undigested swallowed foods and liquids
  • Heartburn
  • Sensation of a lump in the throat
  • Hiccups
  • Weight loss
  • Asthma

How is achalasia diagnosed?

Achalasia is diagnosed with esophageal manometry.  During this test, a thin tube is placed through the nose or mouth into the esophagus to measure the pressure within the esophagus and the lower esophageal sphincter.

Other tests that may be used to diagnose this condition or to decide if an esophageal manometry is required include:

  • Chest X-rays: These simple exams can show distortion of the esophagus, which can indicate achalasia.
  • Barium swallow test:This procedure involves swallowing a substance called barium, a chalky liquid used to coat the inside of organs so that they will show up on an X-ray.
  • Endoscopy:This procedure involves the use of a thin and lighted tube placed through the nose or mouth into the esophagus to "see" inside the esophagus.

Read more about GI motility testing at Boston Children’s. 

What are the treatment options for achalasia?

Treating achalasia involves relieving the obstruction through either:

  • Esophageal pneumatic dilatation: A non-surgical therapy that passes a balloon into the esophagus to relax the lower esophageal sphincter and widens the opening where food enters the stomach. Your child will be lightly sedated for this procedure.
  • Heller myotomy: A minimally invasive surgical procedure to cut the lower esophageal sphincter, allowing food and liquids to pass to the stomach. This operation usually requires a one-day hospital stay.

Neither of these procedures cures achalasia, but they do provide most patients with long-term relief of their symptoms.