These patients are referred to as having “vigorous” achalasia.
Achalasia is a rare disease of the muscle of the esophagus. Achalasia, also known as esophageal achalasia, achalasia cardiae, cardiospasm, dyssynergia esophagus, and esophageal aperistalsis. The most common form is primary achalasia, which has no known underlying cause. However, a small proportion occurs as a secondary result of other conditions, such as esophageal cancer or chagas disease. In achalasia there is an inability of the lower sphincter to relax and open to let food pass into the stomach. In at least half of the patients, the lower sphincter resting pressure also is abnormally high. A few patients with achalasia have high-pressure waves in the lower esophageal body following swallows, but these high-pressure waves are not effective in pushing food into the stomach.
Achalasia has effects on both the muscles and nerves of the esophagus; however, the effects on the nerves are believed to be the almost significant. Early in achalasia, inflammation can be seen under the microscope in the muscle of the lower esophagus, particularly around the nerves. In this disorder, the seamless muscle bed of the esophagus has impaired peristalsis, and the lower esophageal sphincter fails to loosen decently in reaction to swallowing. The almost popular symptom of achalasia is trouble swallowing. Patients typically identify nutrient sticking in the chest after it is swallowed. Dysphagia occurs with both strong and liquid nutrient. Sometimes, patients will identify simply a thick superstar in their chest after eating that may push them to halt eating. Occasionally, pain may be serious and mimic eye pain.
As the disease progresses, the nerves start to deteriorate and finally vanish, especially the nerves that induce the lower esophageal sphincter to loosen. Still later in the progression of the disease, muscle cells begin to degenerate, possibly because of the damage to the nerves. The result of these changes is a lower sphincter that cannot relax and muscle in the lower esophageal body that cannot support peristaltic waves. With time, the body of the esophagus stretches and becomes very enlarged. If the regurgitation happens at night while the patient is sleeping, food can enter the throat and cause coughing and choking. If the food enters the trachea and lung, it can lead to pneumonia. Because of the problem swallowing food, a large proportion of patients with achalasia lose weight. The complications of achalasia include weight loss and aspiration pneumonia.
The diagnosis of achalasia often is suspected on the basis of the history. Achalasia patients need to eat slowly, chew very well, drink plenty of water with meals, and avoid eating near bedtime. It is helpful to sleep with the head elevated by raising the head of the bed or using a wedge pillow. The approach to treatment is to reduce the pressure at the lower esophageal sphincter. This may be achieved by manipulating the lower esophagus sphincter with special instruments. Treatments for achalasia include oral medications, dilation or stretching of the lower esophageal sphincter, surgery to cut the sphincter, and the injection of botulinum toxin into the sphincter. Oral medications that help to relax the lower esophageal sphincter include groups of drugs called nitrates, e.g., isosorbide dinitrate and calcium-channel blockers, e.g., nifedipine and verapamil. Although some patients with achalasia, particularly early in the disease, have improvement of symptoms with medications, most do not.
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