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Esophageal achalasia is a disorder affecting the smooth muscle of the esophagus and the lower esophageal sphincter, causing difficulty swallowing. It can be difficult to diagnose and has no cure, but treatments include lifestyle changes, drugs, pneumatic dilation, and surgery. If these fail, Heller’s myotomy may be performed.
Esophageal achalasia, or simply achalasia, is an esophageal motility disorder. The smooth muscle of the esophagus and the circular muscle located at the junction between the stomach and the esophagus, called the lower esophageal sphincter, are involved. Primary esophageal achalasia has no known underlying cause, but some cases are attributed to Chagas disease and esophageal cancer. The most common symptom is difficulty swallowing or dysphagia. While there are several treatments for the symptoms, there is no proven cure for this condition.
As food flows from the mouth through the esophagus and then into the stomach, the lower esophageal sphincter (LES) relaxes to allow for smooth passage. In esophageal achalasia, passage is impeded because the LES has increased muscle tone and does not fully relax. Also, the smooth muscle of the esophagus is unable to push food towards the stomach, a condition known as aperistalsis. Both of these mechanisms are attributed to the inability of esophageal inhibitory neurons to fire in response to swallowing stimuli. As a result, an affected person has difficulty swallowing.
The difficulty swallowing caused by achalasia is usually progressive. Initially, an affected person is unable to swallow solids alone, but over time, liquids also present a swallowing challenge. Another common symptom of esophageal achalasia is regurgitation of undigested food, which frequently occurs when a person is lying down. Regurgitation increases the risk of aspiration, which could lead to pneumonia. Many patients also experience chest pain or heartburn, usually with meals, and this symptom explains why esophageal achalasia is also called achalasia cardiae or cardiospasm.
Diagnosing esophageal achalasia is sometimes difficult because its symptoms are similar to gastroesophageal reflux, hiatus hernia, and other functional disorders. Therefore, the doctor usually orders a variety of tests including baryta swallow, esophageal manometry, endoscopy, endoscopic ultrasonography, and computed tomography (CT) scan of the gastroesophageal junction. The last three tests are important to exclude gastroesophageal malignancy from the possible diagnosis. A baryta swallow is a procedure in which achalasia is indicated by a “bird’s beak” narrowing of the gastroesophageal junction. In manometry, findings suggestive of achalasia include both esophageal aperistalsis and increased or normal LES tone that fails to fully relax during swallowing.
Treatment of esophageal achalasia includes drugs that reduce the tone of the SLE, lifestyle changes, pneumatic dilation, and surgery. The first-line treatment for esophageal achalasia is pneumatic dilation, an operation that involves placing a balloon in the SLE. Injecting botulinum toxin and taking nifedipine can help reduce the pressure of the SLE. It is also recommended to avoid eating before bedtime and consuming both alcohol and products containing caffeine.
If these surgeries don’t work, a surgical procedure called Heller’s myotomy may be done. It consists of cutting along the esophagus, starting from the LES and up to the proximal part of the stomach. In this procedure, only the outer layers of the esophagus are cut. Fundoplication, or “wrapping” of the part of the stomach called the fundus around the distal esophagus, is done to minimize acid reflux.
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