Esophageal achalasia

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Esophageal achalasia is a very rare swallowing disorder that involves an abnormality in the esophagus, or feeding tube. At the bottom of the esophagus is a muscle called the esophageal sphincter that is supposed to relax when we swallow so that food can continue on to the stomach. In esophageal achalasia, the muscle is no longer able to relax, so food just sits in the esophagus instead of moving down.

The Pediatric Gastroenterologist and Pediatric Surgeons offer the most compressive and cutting-edge care for kids with Achalasia.  We have one of the most experienced teams of specialists in the United States.  To learn more about how that leads to great care for kids with Achalasia please read this patient story.

What causes esophageal achalasia?

It’s not completely clear, but researchers think that it could be related to a virus or an abnormal reaction of the immune system.

How often does esophageal achalasia occur?

This disorder is much more common in adults. The annual incidence rate among children is 0.1 per 100,000, or one in a million.

How does esophageal achalasia develop?

Some children are born with esophageal achalasia, though it can occur at any age. If untreated, the condition can get progressively worse, causing more and more difficulty with swallowing.

What are the symptoms of esophageal achalasia?

Children with esophageal achalasia might feel like they’re unable to complete a swallow or feel like food is getting stuck in their throat. A patient usually starts experiencing symptoms with difficult-to-swallow foods, like steak or chips, and the condition gets progressively worse until it is difficult to even drink.

Other symptoms can include:

  • Chest pains
  • Reflux
  • Aspiration (inhaling food into the lungs)
  • Vomiting undigested food
  • Discomfort when lying down (gravity helps with swallowing, and a child with esophageal achalasia may cough, vomit or feel a choking sensation while lying down if food is stuck in the esophagus)

Who is at risk of developing this condition?

There is no way to prevent esophageal achalasia. In very rare cases, this condition could be inherited.

Why is esophageal achalasia a concern?

If untreated, this condition will get progressively worse until a child basically loses his or her ability to eat and drink normally. This can lead to significant weight loss and malnutrition. With infants, esophageal achalasia can cause choking, chest infections and refusal to eat.

How is esophageal achalasia diagnosed/evaluated?

The first step is a barium swallow test (also known as an esophagram) that allows doctors to examine the esophagus using X-rays. In patients with esophageal achalasia, the lower part of the esophagus may be enlarged at the top and narrower at the bottom, resembling a bird’s beak.

Doctors will need to confirm the diagnosis with an esophageal manometry test, which measures pressure inside the esophagus to show how the muscles are working and how well they’re able to relax.

An upper endoscopy (also known as an EGD) can examine the shape of the esophagus. If esophageal achalasia has progressed without treatment, the esophagus can be very stretched out, and the muscle at the bottom of the esophagus can be so tight that it might be difficult for the camera to move through during an endoscopic procedure.

What is the treatment for esophageal achalasia?

Achalasia can be treated in a few different ways.

  1. A gastroenterologist can use an endoscope, or a long camera to look inside the esophagus and stretch the muscle at the bottom with a special balloon to allow food to pass through.  While this treatment is less invasive and can provide immediate relief, symptoms often reoccur, and more definitive surgical treatment is necessary.
  2.  A surgeon may need to do a Heller myotomy, which involves cutting the esophageal muscle so that it relaxes. There are two common approaches to this surgery, and we offer both types of surgery.  One of the most common is a Laparoscopic Heller Myotomy. This is performed by making several small incisions (<1cm in size) in the stomach areas and using a camera through those incisions to perform the cut on the esophageal muscle (myotomy). Most patients with this type of surgery spend one night in the hospital and are able to resume normal activities within 1-2 weeks. The second approach is to use a special endoscope and instruments passed through the mouth that allows us to cut the esophagus muscle (myotomy) without any additional incisions in the stomach area. Most patients spend one night in the hospital and are able to resume normal activities within a few days. The type of operation performed depends on a variety of factors that your Surgeons and Gastroenterologist will talk with you about.

What happens after treatment?

After treatment your child’s doctor will continue to monitor your child’s symptoms and growth for improvement. Reflux can be a common complication following treatment. In very young infants and toddlers, esophageal achalasia can lead to not wanting to eat that continues even after the problem is fixed, and those patients may need significant interventions from speech pathologists or other feeding experts to overcome their fear of eating.

When should you contact a physician?

Talk to your pediatrician if your child is vomiting with every meal, having trouble swallowing, or experiencing chest pain, reoccurring chest colds or pneumonia.

What is the long-term outlook for esophageal achalasia?

If treated, the long-term outlook is very promising. Both endoscopic treatment and surgery are highly effective, and after treatment, 70 to 90 percent of patients experience excellent outcomes. More than 90 percent see long-term improvement.

How do I live with esophageal achalasia?

If your child continues to have problems eating, he or she might require ongoing treatment with Children’s speech pathologists and feeding specialists. Your child might need to adopt some minor eating changes, such as chewing more or drinking with every swallow. Patients routinely report a lasting improvement in their ability to swallow and enjoy life after treatment.

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