Achalasia

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. This disorder happens more often in adults, and is rare in children.

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

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.

The symptoms of achalasia 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:

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

Your child's health care 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 (esophagram). 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.

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.

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

• Peroral endoscopic myotomy (POEM). This is the newest technique of surgery that treats esophageal achalasia in children. During the surgery only a single incision is done in the esophagus, and no incision in the abdomen. Children who have the POEM procedure reported less pain following Heller myotomy, faster recovery time and a shorter hospital stay. In addition, POEM can be performed at any point in treatment, even if other therapies or surgical interventions (including a Heller myotomy) have been previously performed. It can also be repeated if needed.
• 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). For laparoscopy, the healthcare provider makes several small cuts (incisions) to put instruments into the chest (or elsewhere) for surgery.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.