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Achalasia

Use this section to learn about achalasia and its:

  • Causes & Symptoms
  • Diagnosis
  • Treatment

What is Achalasia?

Achalasia is a disease of the myenteric plexus (the nerves around the esophagus) that affects the function of the esophageal body and the lower esophageal sphincter (the valve between the esophagus and stomach).

Achalasia is characterized by failure of the esophageal body peristalsis (the motion of the esophagus that pushes food toward the stomach) and by failure of relaxation of the lower esophageal sphincter (the valve between the esophagus and the stomach does not relax or open up).

When the esophagus cannot function well to push the food toward the stomach and the lower esophageal sphincter cannot relax or open, the food cannot pass form the esophagus to the stomach. Learn about the symptoms.

Who Suffers from Achalasia?

Achalasia is present in one to three per 100,000 persons in the Western world and most commonly affects individuals between the ages of 20 and 40 years.

What Happens if Achalasia is Untreated?

If left untreated, persistent achalasia may cause the esophagus to become dilated (enlarged) and eventually stop functioning. Patients with untreated achalasia have higher chances of developing esophageal cancer (squamous cell carcinoma).

How is Vigorous Achalasia Different?

In patients with vigorous achalasia, the esophageal contractions have very high pressure. These patients have simultaneous esophageal contractions (they all happen at the same time). Plus, the lower esophageal sphincter does not open.

Patients with vigorous achalasia have similar symptoms to patients with achalasia. In addition, they may have severe chest pain due to the elevated pressure in the esophageal body. The evaluation and treatment of these patients is the same as patients with achalasia.

Achalasia Causes & Symptoms

Causes

Causes of achalasia are not well understood. Factors such as viral or parasitic infections have been considered as potential causes.

Symptoms

  • Dysphagia (difficulty swallowing)
  • Regurgitation of food and fluid after eating or during the night
  • Chest pain and/or pressure after eating
  • Weight loss
  • Chronic cough due to aspiration of food or fluid from the esophagus into the trachea (windpipe)
  • Pneumonia due to aspiration of food or fluid from the esophagus into the trachea (windpipe)

Achalasia Diagnosis

Videoesophagram: This study shows the anatomy and the function of the esophagus and the gastroesophageal junction. In patients with achalasia, the esophagus is dilated, the lower esophageal sphincter does not open, and the gastroesophageal junction is tight and is seen as a narrow point.

A picture of a patient with a dilated esophagus on Barium esophagram is shown in the image below.

  • Esophageal motility study:This study is the most important study to diagnose achalasia. In patients with achalasia all the esophageal contractions are of low amplitude (pressure) and are simultaneous (happen at the same time), therefore, there is no peristalsis (coordinated function to push the food toward the stomach) in the esophageal body. The lower esophageal sphincter (the valve between the esophagus and the stomach) does not open.

The image below illustrates an esophageal motility study (high-resolution manometry) in two swallows in a patient with achalasia. Each color corresponds to a certain pressure in the esophagus.

Manometry in a Patient with Achalasia

Bravo Capsule
  • In patients with vigorous achalasia, in contrast to patients with achalasia, all the esophageal contractions are of high amplitude (pressure); but similar to patients with achalasia, all the contractions are simultaneous (happen at the same time). Therefore, there is no peristalsis (coordinated function to push food toward the stomach) in the esophageal body. The lower esophageal sphincter (the valve between the esophagus and the stomach) does not open.

The image below shows esophageal contractions on an esophageal motility study (high-resolution manometry) in a patient with vigorous achalasia. Each color corresponds to a certain pressure in the esophagus.

Manometry in a Patient with Vigorous Achalasia

Bravo Capsule

The image below is a simple schematic presentation of esophageal contractions on an esophageal motility study (high-resolution manometry) in a patient with vigorous achalasia.

Manometry in a Patient with Vigorous Achalasia

Bravo Capsule
  • Upper endoscopy: In patients with achalasia, upper endoscopy shows a dilated esophagus with a narrow gastroesophageal junction. It is mandatory to perform an upper endoscopy to confirm that the narrowing of the gastroesophageal junction is not caused by other diseases such as stricture or cancer.

  • Computed tomography (CT) scan: A CT scan uses X-rays to make detailed pictures of structures inside the body. This study is not routinely done for diagnosis of achalasia but many patients present with dilated esophagus, which is visible on a CT scan of the chest as shown in the images below.

Achalasia Treatment

The goal of therapy is to obtain relief of difficulty swallowing by opening the lower esophageal sphincter (the valve between the esophagus and the stomach) while preventing GERD.

Several treatments are available to improve the symptoms in patients with achalasia and can be chosen based on a patient’s overall health condition and preferences.

There is no specific therapy that can fix the underlying process (disease of the nerves around the esophagus). None of the treatment options can restore the normal muscle activity of the esophageal body and the lower esophageal sphincter.

All the treatment options are directed to improve the symptoms by relieving the obstruction at the junction between esophagus and stomach to allow the food to pass from the esophagus into the stomach.

Non-surgical treatments include:

Surgical Treatment

The most effective and durable treatment for achalasia is obtained by a myotomy: cutting the muscle of the lower esophageal sphincter (the valve between the esophagus and the stomach) and performing an anti-reflux procedure to prevent reflux following myotomy.

The surgical approaches for myotomy include:

  • open transthoracic (incision in the chest)
  • transabdominal (Incision in the abdomen)
  • thoracoscopic (minimally invasive procedure via small incisions in the chest)
  • laparoscopic (minimally invasive procedure via small incisions in the abdomen) techniques

The laparoscopic minimally invasive procedure called Heller myotomy, is the least invasive surgical procedure for treatment of achalasia and is shown to result in great symptomatic relief.

By Farzaneh Banki, M.D.

References

1. Lyass S, Thoman D, Steiner JP, et al. Current status of an antireflux procedure in laparoscopic Heller myotomy. Surg Endosc. 2003;17:554 –558.
2. Richards WO, Torquati A, Holzman MD, et al. Heller myotomy versus Heller myotomy with Dor fundoplication for achalasia: a prospective randomized double-blind clinical trial. Ann Surg. 2004;240:405– 412; discussion 412–415.
3. Zaninotto G, Annese V, Costantini M, et al. Randomized controlled trial of botulinum toxin versus laparoscopic Heller myotomy for esophageal achalasia. Ann Surg. 2004;239: 364 –370. 4. Ruffato A, Mattioli S, Lugaresi ML, et al. Long-term results after Heller-Dor operation for oesophageal achalasia. Eur J Cardiothorac Surg. 2006;29: 914–919.
5. Guilherme M. Campos, MD, PhD, Eric Vittinghoff, PhD, Charlotte Rabl, MD, Mark Takata, MD, Michael Gadenstatter, MD, Feng Lin, MS, and Ruxandra Ciovica, MD, Endoscopic and Surgical Treatments for Achalasia, A Systematic Review and Meta-Analysis. Ann Surg, Volume 249, Number 1, January 2009:45-57