Achalasia

No one is sure how achalasia develops. Possible causes include:

• Degenerative – A small percentage of people, mostly older patients, seem to get it from the slow destruction of their nerves caused by an unknown neurological problem.
• Genetic – Very rare and accounts for only 1-2% of patients.
• Infection/autoimmune – The most common theory is that a person gets an infection that causes the immune system to “turn on” and fight it. When the immune system mistakes the nerves in the esophagus as the cause of the infection, it destroys the nerves.

People who experience achalasia have any or all of the symptoms listed below:

• Chest pain felt between the shoulder blades, neck or arms that worsens after a meal
• Dysphagia – Difficulty swallowing food or liquid
• Heartburn – Burning in the lower chest or the upper abdomen just beneath the breastbone
• Regurgitation – Backflow of food or liquid from the esophagus into the mouth
• Unplanned weight loss
• Vomiting – Ejection of undigested contents of the stomach and/or the esophagus

 

Food and liquid travel down the esophagus by the coordinated contraction of muscles in the esophageal wall (peristalsis). At the end of the esophagus, they reach a one-way muscular valve called the lower esophageal sphincter (LES). The LES senses the movement of food down the esophagus and opens it to let the contents pass into the stomach. The LES then closes to prevent a return of the food, liquid and digestive enzymes in the stomach to the esophagus.

 

People with achalasia have two problems:

1. Peristalsis does not happen.
2. The LES does not open and relax. Food and fluid remain stuck in the lower esophagus and pass slowly — or not at all — into the stomach.

 

People experience symptoms for an average of 4.6 years before achalasia is diagnosed. Symptoms take time to surface and are often confused with heartburn due to gastroesophageal reflux disease (GERD), which happens when your LES is loose and allows the acid to move back up the esophagus. Achalasia patients may have heartburn, but it is the result of pooling of food and fluid in the lower esophagus (above the LES) that irritates the esophageal lining.

Doctors rely on two tests to determine if a patient has achalasia:

1. Esophageal manometry – The best test for achalasia. It measures how well the esophagus squeezes, the pressure on the lower esophageal sphincter, and whether the LES relaxes when the patient swallows water. Patients must be awake to drink when asked. Manometry is not painful.
2. Upper endoscopy – This minimally invasive procedure allows a gastroenterologist or a surgical endoscopist to examine the patient using a long flexible tube with a light and camera on the end of it (endoscope). With this tool, the doctor looks for food or fluid in the esophagus, whether the esophagus has dilated because the wall muscles are weak, if the LES appears tight, and if there are any other causes behind the swallowing difficulties.

 

Other tests include:

1. Barium swallow – Patients drink a white liquid (barium) that radiologists see in an X-ray and use to trace the pathway from the esophagus into the stomach. Patients may also be given a barium pill to see if it passes.

 

Computer aided tomography (CAT) scan – A CAT scan shows something in the chest that pushes on the esophagus and causes symptoms similar to achalasia.

All treatments require a procedure or surgery and focus on one goal: to open the lower esophageal sphincter (LES) and allow passage of food and liquid. At the center, our experts treat achalasia in three ways:

1. Botox® injection – During an upper endoscopy, doctors inject Botox® (Botulinum toxin) into the LES to get it to relax. Botox® loses its effectiveness after 2-3 months. It is less likely to work each time it is re-injected. Botox® injections are a temporary solution for someone who is not fit to have a more permanent procedure.
2. Dilation – Specialists use X-ray guidance that passes a balloon into the esophagus and dilates the LES. The balloon stretches the muscle of the LES so dramatically that it permanently relaxes. Risks include:
   • The dilation balloon could rupture the esophagus, which causes a hole. This happens in 2-3% of cases and often requires urgent surgery.
   • More than one dilation is often required for a good result, which further increases the risk of esophageal rupture.
   • Because of these risks, many doctors recommend minimally invasive surgery or POEM.
3. Surgery – The most common method to treat achalasia. There are two layers to the esophageal wall. The first is an inner layer called the mucosa. The second is an outer, muscular layer called the submucosa. Surgery cuts the muscular layer (myotomy) not the inner layer. This “breaks” the ring of the LES muscle and allows it to open and relax. To perform a myotomy we use one of two methods:

• Laparoscopic Heller myotomy – With this minimally invasive laparoscopic surgery, the patient is under general anesthesia. The surgeon cuts into the muscular layer of the lower esophagus and upper stomach without damage to the underlying mucosa. 
• POEM – A new procedure where the surgeon or advanced endoscopist operates while inside the esophagus without creating incisions on the abdomen

Both the Heller myotomy and POEM surgeries take 2-3 hours. Heller myotomy patients will experience mild pain at their abdominal wall incisions and may experience mild chest pain. POEM patients tend to have less pain because there are no incisions. 

The day after surgery, surgeons order a barium swallow to look for leaks from the esophagus and ensure the liquid barium passes into the stomach. If the barium swallow looks okay, patients may start a liquid diet. Once patients consume a liquid diet without issues, they can go home. For most patients, this is the day after surgery.

Many surgeons tell patients to follow a liquid or soft diet and resume gentle activity until their follow-up visit in 1-2 weeks. Patients should not strain their abdominal muscles. After the first post-operative checkup, the surgeon will decide when the patient can eat regular food, often within 2-4 weeks.

Both the Heller myotomy and POEM procedure are intended to be permanent fixes for achalasia. There is a chance that the cut muscle of the esophageal wall could scar together, and swallowing difficulties may return. Long-term follow-up of patients who have had a myotomy shows that 80-90% of patients feel well more than two years after their surgery.