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 lower esophageal sphincter (LES) 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 can occur at any age. But it's more common in adults. It's rare in children.

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 problems. 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. These are common symptoms of achalasia:

• Vomiting undigested food

• Having trouble swallowing

• Losing weight

• Not gaining weight

• Feeling pain or burning in the chest

• Coughing a lot after eating

• Having 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. It has a light and camera on it. It helps the provider look at the esophagus and take tissue samples, if needed.

• 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 and LES 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. It 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 surgery eases symptoms. It is safe with long-lasting results for children.

• Fundoplication. This surgery is often done together with an esophagomyotomy. It's 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 occur. This can cause pneumonia and other breathing problems. Adults with achalasia are at greater risk for esophageal cancer. This disease 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 weeks 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 food 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. The LES also gets narrow and tight.

• Food and liquids don’t pass into the stomach correctly. 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

Before you agree to the test or the procedure for your child, make sure you know:

• The name of the test or procedure

• The reason your child is having the test or procedure

• What results to expect and what they mean

• The risks and benefits of the test or procedure

• When and where your child is to have the test or procedure

• Who will do the procedure and what that person’s qualifications are

• What would happen if your child did not have the test or procedure

• Any alternative tests or procedures to think about

• When and how you will get the results

• Who to call after the test or procedure if you have questions or your child has problems

• How much you will have to pay for the test or procedure