Diffuse esophageal spasm (DES), also known as distal esophageal spasm, is a condition characterized by uncoordinated contractions of the

esophagus The esophagus (American English), oesophagus (British English), or œsophagus (Œ, archaic spelling) (American and British English spelling differences#ae and oe, see spelling difference) all ; : ((o)e)(œ)sophagi or ((o)e)(œ)sophaguses), c ...

, which may cause difficulty swallowing (

dysphagia Dysphagia is difficulty in swallowing. Although classified under " symptoms and signs" in ICD-10, in some contexts it is classified as a condition in its own right. It may be a sensation that suggests difficulty in the passage of solids or l ...

) or regurgitation. In some cases, it may cause symptoms such as chest pain, similar to

heart disease Cardiovascular disease (CVD) is any disease involving the heart or blood vessels. CVDs constitute a class of diseases that includes: coronary artery diseases (e.g. angina pectoris, angina, myocardial infarction, heart attack), heart failure, ...

. In many cases, the cause of DES remains unknown. Certain abnormalities on x-ray imaging are commonly observed in DES, such as a "corkscrew esophagus" or "rosary bead esophagus", although these findings are not unique to this condition. Specialized testing called manometry can be performed to evaluate the motor function of the esophagus, which can help identify abnormal patterns of muscle contraction within the esophagus that are suggestive of DES. The treatment of DES consists primarily of medications, such as acid suppressing agents (like proton-pump inhibitors),

calcium channel blocker Calcium channel blockers (CCB), calcium channel antagonists or calcium antagonists are a group of medications that disrupt the movement of calcium () through calcium channels. Calcium channel blockers are used as antihypertensive drugs, i.e., as ...

s, hyoscine butylbromide, or nitrates. In only extremely rare cases, surgery may be considered. People with DES have higher incidences of gastroesophageal reflux disease (GERD), neuromuscular diseases, and degenerative neurological disorders.

Signs and symptoms

DES manifests as intermittent difficulty swallowing solid foods and liquids (

), and atypical chest pain. The chest pain may appear similar to cardiac chest pain ( angina pectoris), so investigating the possible existence of

is often indicated.

Causes

The causes of diffuse esophageal spasm is unclear. It is thought, however, that many cases are caused by uncontrolled brain signals running to nerve endings. Therefore, suppression medication is often the first-line therapy such as antidepressants and anti-epileptic medication are prescribed. It has also been reported that very cold or hot beverages can trigger an esophageal spasm. Avoidance therapy benefits some people, but it has not been medically proven. Food allergies or intolerances may also be a cause in which spasms may be triggered within hours or days from the offending foods.

Diagnosis

Several radiographic findings are suggestive of DES, such as a "corkscrew esophagus" or "rosary bead esophagus" appearance on barium swallow x-ray, although these findings are not unique to DES.

Treatment

Several drugs are used to treat DES, including

nitroglycerin Nitroglycerin (NG) (alternative spelling nitroglycerine), also known as trinitroglycerol (TNG), nitro, glyceryl trinitrate (GTN), or 1,2,3-trinitroxypropane, is a dense, colorless or pale yellow, oily, explosive liquid most commonly produced by ...

, hyoscine butylbromide,

s, hydralazine, and anti-anxiety medications. Acid suppression therapy, such as proton-pump inhibitors, are often the first-line therapy.

Procedures

Botulinum toxin Botulinum toxin, or botulinum neurotoxin (commonly called botox), is a neurotoxic protein produced by the bacterium ''Clostridium botulinum'' and related species. It prevents the release of the neurotransmitter acetylcholine from axon en ...

, which inhibits acetylcholine release from nerve endings, injected above the lower esophageal sphincter may also be used in the treatment of DES. Small studies have suggested benefit from endoscopic balloon dilation in certain patients, but all of the above have a low percentage of success in treating the condition; whilst the treatments work in some sufferers, it does not work for everyone. In extremely rare cases, surgery may be considered.

Epidemiology

It affects about 1 per 100,000 people per year.

References

External links

Barium swallow images of DES
{{Digestive system diseases Esophagus disorders Gastrointestinal motility disorders