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Achalasia is a condition that affects the movement of food and liquids from the esophagus to the stomach. The esophagus is a muscular tube responsible for transporting food from the mouth to the stomach. At the lower end of the esophagus, there is a ring of muscle known as the lower esophageal sphincter (LES), which opens and closes to allow food to enter the stomach and prevent the stomach’s contents from backing up into the esophagus.
However, in achalasia, the nerves in the esophagus become damaged, resulting in paralysis and dilation of the esophagus over time. This causes difficulty swallowing, heartburn, and chest pain.
Achalasia can lead to significant weight loss and malnutrition, and people with the condition have a slightly higher risk of developing esophageal cancer. To reduce the risk of cancer, regular screenings of the esophagus may be recommended. Unfortunately, there is currently no cure for achalasia, and once the esophagus becomes paralyzed, it cannot regain its proper function. However, the symptoms can be managed through endoscopy, minimally invasive therapy, or surgery.
Symptoms
The symptoms of achalasia typically develop gradually and become more severe over time. Common signs and symptoms are:
- Dysphagia or difficulty swallowing
- Undigested food regurgitation
- Heartburn
- Belching
- Nighttime cough
- Intermittent chest discomfort
- Weight loss or malnutrition
- Vomiting
- Hiccups, trouble belching
- Pneumonia, due to food aspiration into the lungs
Causes
The exact cause of achalasia is not well understood, but it is thought to be related to a loss of nerve cells in the esophagus. This loss of nerve cells may result in the immune system attacking the nerve cells in the muscle layers of the esophageal walls, including the lower esophageal sphincter (LES). The degeneration of nerve cells that control muscle function can lead to excessive contractions in the LES. In rare cases, achalasia may be linked to an inherited genetic disorder or infection.
Diagnosis
The diagnosis of achalasia requires the following tests:
- Esophageal manometry: This is a swallowing test that can assess whether the esophagus can normally transfer food to the stomach. Specifically, this test analyzes the rhythmic muscular contractions in the esophagus, the coordination and force exerted by the esophageal muscles, and how well the lower esophageal sphincter relaxes or opens. Achalasia is diagnosed when the LES fails to relax in response to swallowing and there are no muscle contractions along the esophageal walls. This is the “gold standard” test for achalasia diagnosis.
- Barium swallow: The barium swallow will reveal an esophageal constriction at the LES. During the procedure, the patients are required swallow a barium preparation. When the substance coats and fills the interior lining of the digestive tract, X-rays are taken. The doctor may see a silhouette of the esophagus, stomach, and upper intestine thanks to the coating.
- Upper endoscopy: Endoscopy can be performed to diagnose a partial esophageal blockage if the symptoms or the findings of a barium study suggest it. This test is used to rule out carcinogenic (malignant) lesions and to screen for achalasia. During this procedure, a flexible, narrow tube with a camera on it, known as an endoscope, is passed down the throat. For evaluation, the camera shows photos of the inside of the esophagus onto a screen.
Treatment
Treatment options for achalasia include nonsurgical and surgical methods. The best treatment is determined based on the patient’s age, health, and the severity of the achalasia. The goal of the treatment is to alleviate the symptoms by relaxing the lower esophageal sphincter, allowing food and drink to pass more readily through the digestive system.
- Nonsurgical treatment: Nonsurgical options include:
- Pneumatic dilation: In this non-surgical treatment, patients will be sedated briefly while a specially constructed balloon is placed through the LES and inflated. The technique relaxes the sphincter muscle, allowing food to enter the stomach. If the esophageal sphincter does not stay open, this outpatient operation may need to be repeated. Over one-third of people treated with balloon dilation require repeat treatment.
- Botox (botulinum toxin type A): Botox is a protein produced by the bacteria responsible for botulism. Botox, when injected into muscles in extremely minute amounts, can relax spastic muscles. It acts by inhibiting the nerve signals that tell the sphincter muscles to constrict. Botox injections typically have a six-month lifespan. A significant improvement after Botox injection may assist confirming a diagnosis of achalasia.
If the patient is not a candidate for balloon dilation or surgery, or if he or she prefers not to have these operations, Botox injections may be beneficial. To keep symptoms under control, injections must be repeated, recurrent injections may make future surgery more challenging.
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- Medication: These medications are recommended only if the patient is not eligible for pneumatic dilation or surgery, or if Botox hasn’t helped. By reducing LES pressure, these medicines calm spastic esophageal muscles. These drugs only provide temporary relief from discomfort and has substantial adverse effects. Common muscle relaxants include nitroglycerin and nifedipine.
- Surgery: Surgical treatments for achalasia include:
- Heller myotomy. Laparoscopic Heller myotomy is a minimally invasive surgery performed to treat achalasia. A slender, telescopic-like equipment called an endoscope is inserted through a small incision. The LES muscle fibers are cut off during this procedure. It allows food to enter more freely into the stomach.
The addition of a second treatment known as a partial fundoplication aid in the prevention of gastroesophageal reflux disease (GERD), which is a side effect of the Heller myotomy procedure. The doctor ties the top of the stomach around the lower esophagus to form an anti-reflux valve, preventing acid reflux into the esophagus.
- Peroral endoscopic myotomy (POEM): During this procedure, the muscles on the esophageal side, the LES, and the upper region of the stomach are cut with a knife. This requires inserting an endoscope through the mouth and down the throat to make an incision in the lining of the esophagus. These cuts relax the muscles, allowing the esophagus to empty normally and send food into the stomach. POEM patients who develop GERD after the operation are given daily oral medication. In some cases, fundoplication may be necessary to help prevent GERD.
