About esophageal aperistalsis

What is esophageal aperistalsis?

Achalasia is a rare disorder of the esophagus, the tube that carries food from the throat to the stomach. It is characterized by enlargement of the esophagus, impaired ability to push food down toward the stomach (peristalsis), and failure of the ring-shaped muscle at the bottom of the esophagus, the lower esophageal sphincter (LES), to relax. It is the contraction and relaxation of the sphincter that moves food through the tube.



What are the symptoms for esophageal aperistalsis?

Achalasia symptoms generally appear gradually and worsen over time. Signs and symptoms may include:

  • Inability to swallow (dysphagia), which may feel like food or drink is stuck in your throat
  • Regurgitating food or saliva
  • Heartburn
  • Belching
  • Chest pain that comes and goes
  • Coughing at night
  • Pneumonia (from aspiration of food into the lungs)
  • Weight loss
  • Vomiting



What are the causes for esophageal aperistalsis?

The exact cause of achalasia is poorly understood. Researchers suspect it may be caused by a loss of nerve cells in the esophagus. There are theories about what causes this, but viral infection or autoimmune responses have been suspected. Very rarely, achalasia may be caused by an inherited genetic disorder or infection.



What are the treatments for esophageal aperistalsis?

Achalasia treatment focuses on relaxing or stretching open the lower esophageal sphincter so that food and liquid can move more easily through your digestive tract.

Specific treatment depends on your age, health condition and the severity of the achalasia.

Nonsurgical treatment

Nonsurgical options include:

  • Pneumatic dilation. A balloon is inserted by endoscopy into the center of the esophageal sphincter and inflated to enlarge the opening. This outpatient procedure may need to be repeated if the esophageal sphincter doesn't stay open. Nearly one-third of people treated with balloon dilation need repeat treatment within five years. This procedure requires sedation.
  • Botox (botulinum toxin type A). This muscle relaxant can be injected directly into the esophageal sphincter with an endoscopic needle. The injections may need to be repeated, and repeat injections may make it more difficult to perform surgery later if needed.

    Botox is generally recommended only for people who aren't good candidates for pneumatic dilation or surgery due to age or overall health. Botox injections typically do not last more than six months. A strong improvement from injection of Botox may help confirm a diagnosis of achalasia.

  • Medication. Your doctor might suggest muscle relaxants such as nitroglycerin (Nitrostat) or nifedipine (Procardia) before eating. These medications have limited treatment effect and severe side effects. Medications are generally considered only if you're not a candidate for pneumatic dilation or surgery, and Botox hasn't helped. This type of therapy is rarely indicated.

Surgery

Surgical options for treating achalasia include:

  • Heller myotomy. The surgeon cuts the muscle at the lower end of the esophageal sphincter to allow food to pass more easily into the stomach. The procedure can be done noninvasively (laparoscopic Heller myotomy). Some people who have a Heller myotomy may later develop gastroesophageal reflux disease (GERD).

    To avoid future problems with GERD, a procedure known as fundoplication might be performed at the same time as a Heller myotomy. In fundoplication, the surgeon wraps the top of your stomach around the lower esophagus to create an anti-reflux valve, preventing acid from coming back (GERD) into the esophagus. Fundoplication is usually done with a minimally invasive (laparoscopic) procedure.

  • Peroral endoscopic myotomy (POEM). In the POEM procedure, the surgeon uses an endoscope inserted through your mouth and down your throat to create an incision in the inside lining of your esophagus. Then, as in a Heller myotomy, the surgeon cuts the muscle at the lower end of the esophageal sphincter.

    POEM may also be combined with or followed by later fundoplication to help prevent GERD. Some patients who have a POEM and develop GERD after the procedure are treated with daily oral medication.

 



What are the risk factors for esophageal aperistalsis?

Theories on causation invoke infection, heredity or an abnormality of the immune system that causes the body itself to damage the esophagus (autoimmune disease).

The esophagus contains both muscles and nerves. The nerves coordinate the relaxation and opening of the sphincters as well as the peristaltic waves in the body of the esophagus. Achalasia has effects on both the muscles and nerves of the esophagus; however, the effects on the nerves are believed to be the most important. Early in achalasia, inflammation can be seen (when a medical professional examines esophageal tissue under the microscope) in the muscle of the lower esophagus, especially around the nerves. As the disease progresses, the nerves begin to degenerate and ultimately disappear, particularly the nerves that cause the lower esophageal sphincter to relax. Still later in the progression of the disease, muscle cells begin to degenerate, possibly because of the damage to the nerves. The result of these changes is a lower sphincter that cannot relax and muscle in the lower esophageal body that cannot support peristaltic waves. With time, the body of the esophagus stretches and becomes enlarged (dilated).



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