The main pathophysiologic mechanism of DES is the presence of multiple esophageal spasms which are uncoordinated and non-peristaltic. These contractions occur over multiple esophageal segments.
Given the uncoordinated spasms, food can get trapped in the esophagus causing dysphagia. It is important to note that while dysphagia is very common in these patients, regurgitation is not.
Chest pain is a common finding in these patients. The chest pain can sometimes radiate to the jaw, arms, and back. These features can sometimes make it difficult to distinguish the non-cardiac chest pain of DES from cardiac angina.
Esophageal manometry is an important diagnostic test for DES. The presence of multiple simultaneous esophageal contractions confirms the diagnosis. The spasms typically occur after swallowing food, are multiphasic, and repetitive. The lower esophageal sphincter response is normal in these patients.
The imaging findings on barium swallow resemble a corkscrew. This finding is only present in 50% of patients and cannot be used alone to confirm or exclude the diagnosis of DES.
In general, there is no completely effective therapy for DES and treatment failure is common. As such, identifying and reducing the triggers responsible for the esophageal spasms is important. Some triggers can include drinking very hot or very cold fluids, drinking red wine, anxiety, and depression.
Calcium channel blockers are often used in the management of DES. Calcium channel blockers are effective in reducing the amplitude of esophageal contractions.
Nitrates are another effective medication which can be used to treat diffuse esophageal spasm.
The treatment of last resort is esophagomyotomy. The efficacy of this procedure in treating DES is controversial and it is only recommended in cases when a patient is incapacitated by the symptoms.
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