Diffuse esophageal spasm (DES), also known as distal esophageal spasm, is a condition characterized by uncoordinated contractions of the
esophagus
The esophagus (American English) or oesophagus (British English; both ), non-technically known also as the food pipe or gullet, is an organ in vertebrates through which food passes, aided by peristaltic contractions, from the pharynx to the ...
, 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 disease#Terminology, condition in its own right.
It may be a sensation that suggests difficulty in the passag ...
) or
regurgitation. In some cases, it may cause symptoms such as
chest pain
Chest pain is pain or discomfort in the chest, typically the front of the chest. It may be described as sharp, dull, pressure, heaviness or squeezing. Associated symptoms may include pain in the shoulder, arm, upper abdomen, or jaw, along with n ...
, similar to
heart disease
Cardiovascular disease (CVD) is a class of diseases that involve the heart or blood vessels. CVD includes coronary artery diseases (CAD) such as angina and myocardial infarction (commonly known as a heart attack). Other CVDs include stroke, hea ...
. 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 inhibitor
Proton-pump inhibitors (PPIs) are a class of medications that cause a profound and prolonged reduction of stomach acid production. They do so by irreversibly inhibiting the stomach's H+/K+ ATPase proton pump.
They are the most potent inhibitors ...
s),
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
Hyoscine butylbromide, also known as scopolamine butylbromide and sold under the brandname Buscopan among others, is an anticholinergic medication used to treat crampy abdominal pain, esophageal spasms, renal colic, and bladder spasms. It is a ...
, or
nitrates
Nitrate is a polyatomic ion with the chemical formula . Salts containing this ion are called nitrates. Nitrates are common components of fertilizers and explosives. Almost all inorganic nitrates are soluble in water. An example of an insoluble ...
. In only extremely rare cases, surgery may be considered. People with DES have higher incidences of
gastroesophageal reflux disease
Gastroesophageal reflux disease (GERD) or gastro-oesophageal reflux disease (GORD) is one of the upper gastrointestinal chronic diseases where stomach content persistently and regularly flows up into the esophagus, resulting in symptoms and/ ...
(GERD), neuromuscular diseases, and degenerative neurological disorders.
Signs and symptoms
DES manifests as intermittent difficulty swallowing solid foods and liquids (dysphagia), and atypical chest pain. The chest pain may appear similar to cardiac chest pain (angina pectoris), so investigating the possible existence of heart disease 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
An upper gastrointestinal series, also called a barium swallow, barium study, or barium meal, is a series of radiographs used to examine the gastrointestinal tract for abnormalities. A contrast medium, usually a radiocontrast agent such as barium ...
x-ray, although these findings are not unique to DES.
Treatment
Several drugs are used to treat DES, including
nitroglycerin
Nitroglycerin (NG), (alternative spelling of nitroglycerine) also known as trinitroglycerin (TNG), nitro, glyceryl trinitrate (GTN), or 1,2,3-trinitroxypropane, is a dense, colorless, oily, explosive liquid most commonly produced by nitrating g ...
,
hyoscine butylbromide
Hyoscine butylbromide, also known as scopolamine butylbromide and sold under the brandname Buscopan among others, is an anticholinergic medication used to treat crampy abdominal pain, esophageal spasms, renal colic, and bladder spasms. It is a ...
,
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,
hydralazine
Hydralazine, sold under the brand name Apresoline among others, is a medication used to treat high blood pressure and heart failure. This includes high blood pressure in pregnancy and very high blood pressure resulting in symptoms. It has bee ...
, and anti-anxiety medications.
Acid suppression therapy, such as
proton-pump inhibitor
Proton-pump inhibitors (PPIs) are a class of medications that cause a profound and prolonged reduction of stomach acid production. They do so by irreversibly inhibiting the stomach's H+/K+ ATPase proton pump.
They are the most potent inhibitors ...
s, are often the first-line therapy.
Procedures
Botulinum toxin
Botulinum toxin, or botulinum neurotoxin (BoNT), is a neurotoxic protein produced by the bacterium ''Clostridium botulinum'' and related species. It prevents the release of the neurotransmitter acetylcholine from axon endings at the neuromusc ...
, 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.
[
]
See also
*Nutcracker esophagus
Nutcracker esophagus, Jackhammer esophagus, or hypercontractile peristalsis, is a disorder of the movement of the esophagus characterized by contractions in the smooth muscle of the esophagus in a normal sequence but at an excessive amplitude or ...
*Esophageal spasm
Esophageal spasm is a disorder of motility of the esophagus.
There are two types of esophageal spasm:
* Diffuse or distal esophageal spasm (DES), where there is uncoordinated esophageal contractions
* Nutcracker esophagus (NE) also known as hyp ...
References
External links
Barium swallow images of DES
{{Digestive system diseases
Esophagus disorders