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Achalasia in Children

Achalasia is a rare disease that causes difficulty swallowing foods and liquids. The tube that connects the back of the throat to the stomach is the esophagus. In achalasia, the muscles that cause the esophagus to contract and push food down toward the stomach don’t work properly. The contractions become weak and not coordinated, and the end of the esophagus where it passes into the stomach develops tightness and narrowing.

Illustration of adolescent digestive tract
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Achalasia effects men and women equally. In some cases, there may be a family history of achalasia. Although doctors know that achalasia is the result of abnormalities of the nerve cells that supply the esophageal muscles, the cause of these abnormalities is not known.

Symptoms of achalasia in children

Symptoms usually develop gradually and get worse over time:

  • Vomiting undigested food

  • Difficulty swallowing

  • Loss of weight and failure to gain weight

  • Pain or burning in the chest

  • Coughing spells after eating

  • Bad breath

Diagnosing achalasia in children

Doctors may suspect achalasia if a child's symptoms have lasted for several months and are getting worse. The best way to make a diagnosis is to look into the esophagus and to do special types of tests that 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 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.

Treating achalasia in children

Achalasia usually continues to get worse if it is not treated. Because there are no drugs that help, surgery is almost always recommended.

One type of surgery is called balloon dilation. A special type of balloon is passed through an endoscope and inflated to stretch the tight muscles where the esophagus narrows as it meets the stomach. This procedure does not work as well in children as it does in adults, however, and it usually does not result in long-term relief of achalasia.

The best treatment for children is a procedure known as an esophagomyotomy. This surgery may be done through a laparoscope or as an open procedure. It allows the surgeon to cut the muscles in the tight junction at the end of the esophagus. The procedure is a laparoscopic Heller myotomy. This lets food pass through to the stomach and relieves symptoms of achalasia about 95% of the time. The procedure is considered safe with long-lasting results for children.

Many surgeons will complement this procedure with an anti-reflux procedure known as a fundoplication. In this procedure, a portion of the upper stomach is wrapped around the lower esophagus to prevent acid reflux from the stomach into the esophagus after the lower esophageal sphincter has been cut.

If your child has difficulty swallowing or other symptoms of achalasia, especially if the symptoms have been present for several months and seem to be getting worse, talk to your child's pediatrician. Achalasia is a rare disease in children, but once a diagnosis is made, surgical treatment is usually quite effective.