DES is a movement disorder involving the type of muscle (called smooth muscle) of the esophagus. Because smooth muscle is found in the lower, or distal, esophagus, this condition is also called “distal esophageal spasm,” or even “esophageal spasm” for short.
The cause is unknown, but studies have suggested that it involves impaired nervous system input to the esophagus, using a neurotransmitter called nitric oxide (NO). Gastroesophageal reflux disease (GERD) might also be involved in a fraction of cases.
Typical symptoms include an intermittent sensation of food sticking inside the chest and chest pain.
DES is diagnosed by performing an esophageal manometry, which measures pressures generated by the esophagus at rest and during swallows. The typical pressure pattern in DES shows early contraction of the distal (lower) esophagus after the patient begins to swallow.
Several treatment options are available for DES, although the predictability of response is variable. Typically, proton pump inhibitors (PPIs) are tried first. Second-line options include medications that can have a relaxing effect on the esophageal smooth muscle, such as calcium channel blockers (a type of blood pressure medication), anti-spasmodic agents, and nitroglycerin (used commonly for blood pressure and chest pain related to the heart). Sometimes, an upper endoscopy can be performed to inject BoTox into the esophageal muscle, which causes it to relax. Longer-lasting options include a surgical myotomy or a special endoscopic procedure called POEM to cut the circular muscle within the esophagus, leaving it in a more relaxed state.