Esophageal Achalasia

Motility is the movement and contraction of the muscles in the digestive tract needed to mix and move food and liquids through the body. Esophageal achalasia is a motility problem in the esophagus treated by the Gastrointestinal Motility Program at CHOC Children’s. Many motility problems can be challenging to diagnose, and therefore, challenging to treat. Our experts specialize in a comprehensive approach to diagnosing and treating motility and functional gastrointestinal disorders that gives patients and their families the answers and relief that they are seeking.

Learn more about the CHOC Children’s Gastrointestinal Motility Program.

Frequently Asked Questions about Esophageal Achalasia

What is esophageal achalasia?

Achalasia is a disorder that affects the esophagus, which is the swallowing tube that connects the back of the throat to the stomach. In a person with achalasia, the esophagus does not push food or liquid into the stomach like it should. In addition, the ring of muscle that circles the lower portion of the esophagus does not relax enough to let food and liquid pass through easily. In fact, achalasia means “failure to relax.”

Achalasia usually develops slowly, making it harder to swallow food and beverages over time. It is caused by loss of the nerve cells that control the swallowing muscles in the esophagus. Why these nerve cells degenerate is not known. Although achalasia has no cure, symptoms can be controlled with treatment.

What are the symptoms of achalasia?

Symptoms of achalasia develop gradually over time. As the esophagus becomes wider and weaker, symptoms can include:

  • Difficulty swallowing food, a condition called dysphagia
  • Food or liquid flowing back up into the throat, or regurgitation
  • Waking up at night from coughing or choking because of regurgitation
  • Heartburn
  • Chest pain or pressure
  • Difficulty burping or hiccups
  • Weight loss.

Who is at risk for developing achalasia?

Achalasia can develop at any age, but is more common in adults than in children. Scientists don’t know exactly why this loss of muscle control in the lower esophagus happens, but risk factors may include:

  • Genes the child is born with
  • A disordered immune system that attacks the nerve cells in the esophagus
  • The herpes simplex virus or other viral infections.

How is achalasia diagnosed?

If your child’s doctor suspects achalasia, he or she may order tests to confirm the diagnosis:

  • Endoscopy. This is an outpatient procedure during which a flexible telescope is passed through the mouth to examine the esophagus and the valve that opens into the stomach. This valve is called the lower esophageal sphincter (LES). Learn more about endoscopy.
  • Esophogram. This is a special type of X-ray that takes pictures of the esophagus while the patient swallows a thick contrast material called barium. Signs of achalasia that your child’s doctor looks for are widening of the esophagus, incomplete emptying and tightness of the LES. Learn more about X-ray.
  • Manometry. This is an outpatient procedure during which a pressure-measuring tube is passed through the patient’s nose into the esophagus. Pressure measurements are taken as the child swallow sips of water. This test may show weak and uncoordinated muscle contractions and pressure buildup at the LES.

How is achalasia treated?

No treatment can restore normal esophageal movement, but treatments can help relieve symptoms, open up the LES to improve emptying of the esophagus and prevent complications. Some patients may need repeat treatments. Common treatments include:

  • Pneumatic dilation. This is an outpatient procedure done under anesthesia. While the gastroenterologist looks into the esophagus through an endoscope, an air-filled balloon is passed through the valve between the esophagus and stomach and then inflated.
  • Botox injection. Botox is a medication that can paralyze muscles. Botox can be injected into the muscles that control the LES to relax the valve opening. This procedure is also done during endoscopy, but patients don’t need to be asleep. The results usually wear off in three months to one year, so the procedure may need to be repeated.
  • Surgery. Surgery to open the LES is called myotomy. During myotomy, the muscles of that valve are cut. This type of surgery usually provides long-term relief from achalasia symptoms.
  • Medications. Two commonly used medications to treat achalasia are calcium channel blockers and long-acting nitrates if surgery is not an option.

What are the complications from achalasia?

Although achalasia cannot be prevented, treatment can prevent long-term complications. Possible complications include:

  • Aspiration pneumonia. This type of pneumonia may be caused when food or liquids in the esophagus back up into the throat and are breathed into the lungs.
  • Esophageal perforation. This complication may occur if the walls of the esophagus become weak and distended. Perforation may also occur during treatment. Esophageal perforation may cause a life-threatening infection.
  • Esophageal cancer. People with achalasia are at higher risk for esophageal cancer.

It is important to call your child’s doctor if you have any questions about their medications or treatment. It is especially important to contact the doctor if the child has:

  • Increased difficulty swallowing
  • Worsening regurgitation, especially if they are waking up coughing or choking at night
  • Symptoms of infection such as chills or fever
  • Chest pain or difficulty breathing.

Living With Achalasia

Achalasia is a long-term disease. At CHOC Children’s we are by our patients’ sides every step of the way throughout childhood and into adulthood, and we help patients transition to an adult physician when the time is right.

It is important to learn as much as possible about achalasia so that you can help your child live their very best life possible. Children with achalasia will see their gastroenterologist on a regular basis, usually once or twice a year, even after symptoms have been controlled.

Children and young adults with achalasia can help reduce their symptoms of dysphagia or regurgitation by:

Long Live Childhood

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