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Achalasia in Children

What is achalasia in children?

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. This disorder happens more often in adults. It is rare in children.

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

What causes achalasia?

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 children?

Symptoms often start slowly and get worse over time. They may look like symptoms of other disorders. Make sure your child sees his or her 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 in children diagnosed?

Your child's healthcare provider may suspect achalasia if symptoms have lasted for a few months 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 telescope (endoscope) is passed into the esophagus.
  • 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 in children treated?

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 using a laparoscope (laparoscopic Heller myotomy). Or it may be done as an open (traditional) 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 anti-reflux 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 the complications of achalasia in children?

Achalasia will not get better without treatment. If untreated, weight loss will continue. Breathing food and 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.

When should I call my child's healthcare provider?

Call your child's provider if your child has trouble swallowing or shows other symptoms of achalasia. It’s also important to call if your child has had symptoms for a few months and they are getting worse. Achalasia is rare in children. But once a diagnosis is made, surgery often helps.

Key points about achalasia in children

  • Achalasia is a rare disease that makes it hard to swallow foods and liquids.
  • This disorder happens more often in adults. It is rare in children.
  • The muscles of the food pipe (esophagus) get weak and don’t work well.
  • Food and liquids don’t pass into the stomach. They can collect in the esophagus.
  • Symptoms include vomiting after eating, and weight loss.
  • Once a diagnosis is made, surgery is often very effective.

Next steps

Tips to help you get the most from a visit to your child’s health care provider:
  • Before your visit, write down questions you want answered.
  • At the visit, write down the names of new medicines, treatments, or tests, and any new instructions your provider gives you for your child.
  • If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.

Online Medical Reviewer: Bundy, Marianne, MSN, RN, CEN
Online Medical Reviewer: MMI board-certified, academically affiliated clinician
Last Review Date: 07/15/2015
© 2000-2015 The StayWell Company, LLC. 780 Township Line Road, Yardley, PA 19067. All rights reserved. This information is not intended as a substitute for professional medical care. Always follow your healthcare professional's instructions.
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