Achalasia in Children

What is achalasia in a child?

Achalasia is a rare disease that makes it hard to swallow foods and liquids. In achalasia, there is a problem with the tube that carries food from the mouth to the stomach (esophagus). The muscles that make the esophagus contract and push food down to the stomach don’t work well. The muscle contractions get weak.

The LES (lower esophageal sphincter) also doesn’t work well. The LES is the muscle at the bottom of the esophagus, where it joins the stomach. With achalasia, the LES gets narrow and tight. Food does not pass into the stomach as it should. Over time, food and liquid can collect in the esophagus.

Achalasia happens more often in adults. It is rare in children.

It affects males and females equally. In some cases, there may be a family history of achalasia.

What causes achalasia in a child?

Achalasia happens because of problems with the nerve cells in the esophageal muscles. Experts don’t know what causes these abnormalities. In children, achalasia is often linked with other conditions. These include adrenal glucocorticoid deficiency, Allgrove syndrome, and Down syndrome.

What are the symptoms of achalasia in a child?

Symptoms often start slowly and get worse over time. They may look like symptoms of other disorders. Make sure your child sees their healthcare provider for a diagnosis. The following are common symptoms of achalasia:

  • Vomiting undigested food
  • Having trouble swallowing
  • Losing weight
  • Not gaining weight
  • Pain or a burning feeling in the chest
  • Coughing a lot after eating
  • Bad breath

How is achalasia diagnosed in a child?

Your child's healthcare provider may suspect achalasia if symptoms have lasted for a few weeks and are getting worse. To make a diagnosis, the provider will look into the esophagus and do special tests. These tests take pictures and measure pressures inside the esophagus while a child is swallowing. These tests include:

  • X-ray. A chest X-ray may show widening and fluid in the esophagus.
  • Endoscopy. A flexible tube (endoscope) is passed into the esophagus.
  • Barium swallow (esophogram). Pictures may be taken while the child swallows a thick liquid that shows up on X-rays.
  • Manometry. Pressures may be measured inside the esophagus as the child swallows sips of water.

How is achalasia treated in a child?

Achalasia often gets worse if it is not treated. Medicines are not very effective. Surgery is almost always recommended. Types of surgery include:

  • Balloon dilation. A special type of balloon is passed through an endoscope into the esophagus. The balloon is inflated. This stretches the tight LES muscles where the esophagus narrows as it meets the stomach. This surgery does not work as well in children as it does in adults. It often does not give long-term relief.
  • Esophagomyotomy. This surgery is the best treatment for children. It may be done as an open (traditional) surgery. Or it may be done using a laparoscope (laparoscopic Heller myotomy). For laparoscopy, the healthcare provider makes several small cuts (incisions) to put instruments into the chest (or elsewhere) for surgery. The surgeon cuts the LES muscles in the tight area at the end of the esophagus. This lets food pass through to the stomach. In most cases, this eases symptoms. It is a safe procedure with long-lasting results for children.
  • Fundoplication. This surgery is often done together with an esophagomyotomy. This is an antireflux surgery. A part of the upper stomach is wrapped around the lower esophagus. This stops acid reflux from the stomach from flowing back into the esophagus after the LES has been cut.

What are possible complications of achalasia in a child?

Achalasia will not get better without treatment. If untreated, weight loss will continue. Vomiting food and breathing water into the lungs (aspiration) can also result. This can cause pneumonia and other breathing problems. Adults with achalasia are at greater risk for esophageal cancer. This has not been reported in children.

Online Medical Reviewer: Liora C Adler MD

Online Medical Reviewer: Rita Sather RN

Online Medical Reviewer: Raymond Kent Turley BSN MSN RN

Date Last Reviewed: 12/1/2020

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