Achalasia is a condition where the esophagus, the muscular tube that extends from the throat to the stomach, is unable to properly transport food to the stomach. The circular muscle structure at the end of the esophagus that serves as a valve between the esophagus and stomach, called the lower esophageal sphincter (LES), remains closed during swallowing, causing food to accumulate in the esophagus and difficulty in swallowing. Other symptoms include regurgitation of undigested food, chest pain, heartburn, and weight loss.
The main symptoms of achalasia include:

• Difficulty in swallowing
• Chest pain
• Painful burning sensation in the chest

Individuals with achalasia gradually experience increasing difficulty in consuming solid foods and liquids over a period of several years. If left untreated, achalasia can lead to significant weight loss and malnutrition. There is a slightly increased risk of developing esophageal cancer in individuals with achalasia, particularly if the obstruction has been present for a long time. Your doctor may recommend regular endoscopic screenings for the prevention and early detection of esophageal cancer.

Who is affected by Achalasia?

It is commonly diagnosed in adults but can also occur in children. There is no specific race or ethnic group that is more affected, and there is no familial predisposition.

What causes Achalasia?

The exact reason why the esophageal muscles fail to contract normally in individuals with achalasia is unknown. Researchers believe it could be due to a viral cause, and recent studies have shown that achalasia originates from nerve cells of the involuntary nervous system in the muscle layers of the esophagus. It is hypothesized that these cells are attacked by the individual’s own immune system and slowly degenerate for reasons that are currently not understood.

How is Achalasia-Related Swallowing Difficulty Diagnosed?

Three commonly used tests are:

Barium swallow:

The patient swallows a barium preparation (liquid or another form), and the movement of the substance through the esophagus is evaluated using X-rays.

Endoscopy:

An endoscope, a flexible narrow tube, is inserted into the esophagus to visualize its interior and display the images on a screen.

Manometry:

This test measures the timing and strength of contractions in the esophagus (pumping action) and relaxation of the lower esophageal sphincter (valve).

How is Achalasia treated?

If left untreated, achalasia can be debilitating, and patients may experience significant weight loss that can result in malnutrition. Several successful treatments are available:

Surgery:

The traditional surgical approach for treating achalasia has been Heller Myotomy since the early 20th century. In this procedure, the muscles that serve as the valve between the esophagus and stomach are cut. Traditional Heller Myotomy requires open surgery and typically necessitates hospitalization for up to a week for adequate recovery.

Minimally Invasive Surgery:

Today, selected patients with achalasia can be successfully treated with a minimally invasive surgical technique called Laparoscopic Esophagomyotomy or Heller Myotomy. A Heller Myotomy can be performed using five small incisions. It has been shown that adding a partial fundoplication (Dor) minimizes reflux and protects the esophagus from damage due to gastroesophageal reflux. This procedure typically requires a one-day hospital stay and offers faster recovery compared to traditional surgery.
Up to two-thirds of patients are successfully treated with surgery, but some patients may require repeat surgeries or balloon dilation to achieve satisfactory long-term outcomes.

Balloon Dilation:

Achalasia can rarely be treated non-surgically with balloon (pneumatic) dilation. Under mild sedation, a gastroenterologist inserts and inflates a specially designed balloon through the lower esophageal sphincter. The balloon disrupts the muscle of the esophagus and widens the opening for food to enter the stomach. Some patients may require repeated dilation treatments for symptom improvement, and the treatment may need to be repeated every few years to achieve long-term results.
On average, this procedure can alleviate symptoms for up to 75% of individuals for several years. However, there is a risk of esophageal perforation with this treatment.

Medical Therapy:

Patients who are not suitable candidates for balloon dilation or surgery may benefit from botox (botulinum toxin) injections. Botox is a protein produced by bacteria. When injected in very small amounts into the muscles, it relaxes spastic muscles. It works by preventing the nerves from sending contraction signals to the muscles. A smaller percentage of patients (up to 35%) may achieve good short-term results using Botox compared to balloon dilation. Additionally, injections need to be repeated frequently to maintain symptom relief.
Other medications such as nifedipine and nitroglycerin can help relax the spastic esophageal muscles. Patients taking nifedipine daily may experience satisfactory results for several years.

It should be noted that the treatment of achalasia does not correct the esophagus itself but aims to improve esophageal emptying. However, it should be kept in mind that this may result in the backflow of contents from the stomach to the esophagus (reflux).

Regardless of the treatment, long-term follow-up is necessary to ensure the preservation of the esophagus as a passive conduit. This requires adequate esophageal emptying after the preferred treatment and prevention of gastroesophageal reflux. If the obstructed esophagus or an esophagus exposed to reflux becomes dilated, it may eventually need to be replaced.