Achalasia: Causes, Symptoms, Prevention, and Treatment

Overview

Achalasia is a rare disorder in which the damaged nerves in the esophagus prevent it from working correctly. Consequently, your esophagus becomes paralyzed and dilated over time and finally loses the ability to squeeze food down into your stomach.

This blog is a comprehensive guide to understanding achalasia, its causes, symptoms, prevention, and treatment.

What is achalasia?

Achalasia is a rare condition affecting the esophagus, a tube carrying food from the throat to the stomach. For people with achalasia, their lower esophageal sphincter (LES) fails to open during swallowing. This muscular ring closes the esophagus from the stomach most of the time, but it opens when food is swallowed, so that food can pass through.

The symptoms of this condition often appear gradually and may worsen as time goes on. Eventually, it can be challenging to swallow fluids or food, and treatment may be needed.

What are the symptoms of achalasia?

The symptoms of achalasia develop slowly, with symptoms lasting months or years. They include the following:

• Dysphagia (trouble swallowing and feeling as if food is stuck in the esophagus)  is the most common early sign of this condition.
• Regurgitation of the undigested food
• Pain or discomfort in the chest
• Heartburn
• Belching
• Chest pain that comes and goes
• Coughing at night
• Pneumonia (from aspiration of food into the lungs)
• Weight loss
• Vomiting

When should you call the doctor?

People must call the doctor when they:

• Have trouble swallowing or painful swallowing

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What are the causes of achalasia?

The exact cause of achalasia is unknown. Researchers believe that it can be due to a combination of various factors, including:

• Genetics, or family history
• An autoimmune condition in which the body’s immune system attacks healthy cells in the body. The degeneration of nerves in the esophagus often contributes to the symptoms of achalasia.
• Damage to the nerves in the esophagus or LES (lower esophageal sphincter)

Some researchers have theorised that viral infections can prompt autoimmune responses, particularly when people have a higher genetic risk of the condition.

What are the risk factors for achalasia?

Some of the potential risk factors for achalasia include:

• A possible spinal cord injury
• Experiencing a viral infection
• Having an autoimmune disease
• Age (it is more common in middle age and older adulthood)

What are the possible complications of achalasia?

The complications of achalasia may include:

• Regurgitation (backflow) of acid or food from esophagus (reflux)
• Pneumonia
• Lung infections (pulmonary infections)
• Tearing of the esophagus

How is achalasia treated?

The treatment for achalasia cannot completely cure the condition, but it can help:

• Improve people’s ability to swallow by opening the LES (lower esophageal sphincter)
• Reduce other symptoms such as pain and regurgitation
• Lower their chances of having an abnormally enlarged esophagus

Some of the other possible treatments options include:

• Surgery: Surgery is the most successful treatment option for achalasia because, with surgery, most people get long-term relief from symptoms. The Heller myotomy is the most common surgical option. 

• Heller myotomy: In this procedure, the surgeon cuts muscle at the lower end of esophageal sphincter allowing food to pass easily into the stomach. If the procedure is performed non-invasively it is called laparoscopic Heller myotomy. Some individuals who have undergone Heller myotomy may develop GERD (gastroesophageal reflux disease) later.

A procedure called fundoplication may also be done at the same time of Heller myotomy to avoid future problems with GERD. The fundoplication involves a surgeon to wrap the top of the stomach around lower esophagus to create an anti-reflux valve, thus preventing the acid to come back (GERD) into esophagus. Usually, fundoplication is performed laparoscopically, a minimally invasive procedure.

• Peroral endoscopic myotomy (POEM). The POEM procedure involves a surgeon using an endoscope inserted through mouth and down the throat to create a cut in the inside lining of the esophagus. Soon after this, the surgeon cuts the muscle at lower end of the esophageal sphincter, as in a Heller myotomy,.

Other Treatments

Some the non-surgical options include:

• Botox injections

In this procedure, the doctor injects Botox (botulinum toxin) into the tight esophagus muscles during an endoscopy. It helps relax the muscles temporarily so that people can swallow normally.

• Pneumatic dilation

In this procedure, the surgeon inserts a balloon into the centre of the esophageal sphincter so that the food can pass through. It is an outpatient procedure. However, it is essential to note that if the esophageal sphincter does not stay open, the patient needs to undergo the pneumatic dilation procedure again. Nearly 30 per cent of people treated with balloon dilation require repeat treatment within five years. 

• Medication

Before eating, the doctor may recommend muscle relaxants such as nitroglycerin or nifedipine. Since these medications have side effects, they are typically considered for patients who are not candidates for pneumatic dilation or surgery, and Botox has not helped. 

Conclusion

While more needs to be learnt about achalasia, around 90% of people see a long-term improvement in symptoms after treatment. People must get a diagnosis sooner rather than later because it may help them get treatment to improve their symptoms before the condition becomes severe. The patient with achalasia may require multiple treatments before the symptoms improve. It is essential to remember that if one treatment does not work, there may  be other options to consider. 

Frequently Asked Questions

1. How is achalasia diagnosed?

Typically, the doctor uses the following three tests to diagnose achalasia:

• Barium swallow
• Upper endoscopy
• Manometry

2. Is achalasia serious?

Yes, achalasia can be dangerous, mainly when it is left untreated. When people have achalasia, they gradually experience increased trouble eating solid foods and drinking liquids. This condition may lead to considerable weight loss and malnutrition. Patients with this condition also have a small risk of developing esophageal cancer, especially if the disease has been present for a long time. The doctor may recommend regular oesophagus screenings for early cancer detection if it develops.

3.  What are the lifestyle changes that people can make?

The helpful lifestyle changes that people can make include:

• Chop food into small, bite-size pieces and eat in an upright position. It will enable gravity to help move the food through their esophagus.
• Avoid eating solid foods during bedtime.
• It is essential never to sleep flat because it increases their risk of drawing food into their lungs. Therefore, they must sleep with their head elevated.