Achalasia

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What is it?    

Achalasia is a rare disorder of swallowing in which the muscles of the esophagus (the tube connecting our mouth to stomach) contract inappropriately.  As a result, the esophagus fails to efficiently pass food or liquid from the esophagus to the stomach.  In addition, the lower esophageal sphincter (LES), a circular band of muscular tissue of the esophagus, located just above the stomach, stays closed and does not relax as it should to allow the food to enter the stomach.

Who gets it?

It is an uncommon disorder, affecting 1.6 out of 100,000 people.  Most people are between 25-60 years old when diagnosed. Both men and women are affected.

Why does it happen?

Om achalasia there is degeneration of the nerves to the esophagus.  The exact cause of this nerve degeneration in the esophageal wall is unknown.  It is thought that achalasia could be an autoimmune disorder in which the immune system attacks these nerves.  Achalasia has also been associated with infections with several viruses such as herpes zoster, measles virus, and HSV-1.

What are the symptoms?

  • Difficulty swallowing
  • Chest pain
  • Regurgitation of swallowed food and liquid
  • Heartburn
  • Difficulty burping
  • Fullness or lump in the throat
  • Hiccups
  • Weight loss

How is it diagnosed?

There are many disorders that can cause trouble swallowing.  To determine if the problem is due to achalasia, several tests may be performed:

  • Esophagram/Barium swallow:  x-ray that is taken during and after swallowing liquid barium.
  • Upper endoscopy:  a tube with light and camera is inserted through your mouth , into your throat, down to your stomach after you are sedated. Tissue samples can be taken during this procedure.
  • Esophageal manometry:  a thin mall tube placed through your nose or mouth, to obtain pressure readings in the esophagus as you sip small amounts of water.

How is it treated?

There is no cure to reverse the damage to the nerves and allow the muscles of the esophagus to function normally again.  Rather, the symptoms of the disorder are treated by attempting to relax the lower esophageal sphincter (LES), that muscular ring at the lower end of the esophagus that is inappropriately staying tightly closed instead of relaxing as it should.

  • Medicines:  Both nitrates and calcium channel blockers are used to relax the LES; typically they are taken 10-30 minutes before a meal. 
  • Balloon dilation:  A catheter with a balloon is placed at the LES.  The balloon is inflated, causing the LES to stretch so it will no longer be able to close so tightly.  People often require repeat dilation of the LES, but 60% of people are still symptoms free one year after the procedure and 25% of people five years later.
  • Botulinum injection:  This is performed during an upper endoscopy.  The Botulinum toxin is injected into the LES.  It causes temporary paralysis of the muscles of the LES so it does not stay so tightly contracted.  65-90% of people will have relief for 3 months to 1 year.
  • Surgery (myotomy):  A surgeon severs or cuts the muscle fibers of the LES.  This surgery is very effective and provides relief of symptoms for 70-90% of people with achalasia.

Are there complications of achalasia?

Because the LES does not relax properly and allow food to enter the stomach, food can back up or pool in the esophagus, leading to inflammation, ulcerations, or yeast (Candida) infection.  This back up of food in the esophagus can also lead to aspirating (breathing in) some of the food material into your lungs, causing other problems such as pneumonia.  There is an increased long-term risk of esophageal cancer so continued follow up is recommended.