Symptoms
Esophageal achalasia is a rare and chronic progressive motility disorder of the lower esophagus that manifests as dysphagia and retrosternal pain. Patients feel discomfort during and after eating. Possible accompanying symptoms include bad breath, cramping, and burning. Ingested food remains in the esophagus and cannot be adequately transported to the stomach. This can lead to sometimes severe complications such as weight loss, regurgitation of undigested food, aspiration pneumonia with chronic cough, and rarely esophageal cancer. Over time, the esophagus dilates enough to accommodate an entire meal.
Causes
Esophageal achalasia is a disorder of the lower esophageal sphincter and smooth muscle of the esophagus. The sphincter cannot relax sufficiently during swallowing, and the musculature cannot initiate the necessary peristalsis. This esophageal passenger disorder is caused by cellular degeneration of the myenteric plexus.
Diagnosis
Diagnosis is made by various methods under medical treatment. Esophageal manometry is considered the gold standard. Pain behind the sternum can be caused by a number of other conditions that must be ruled out at diagnosis. These include, for example, angina, myocardial infarction, inflammation of the pericardium, inflammation of the endocardium, gastric distention, GERD, nutcracker esophagus, and esophagitis. Diffuse esophageal spasm may occur concomitantly with achalasia.
Treatment
Treatment is usually by dilation and with surgical measures. Oral medical therapy with muscle-relaxing drugs such as nitrates, calcium channel blockers, or phosphodiesterase-5 inhibitors plays a minor role. Botulinum toxin injections, which relax the lower sphincter, are more effective for longer periods.