Diffuse esophageal spasm (DES) is a rare disorder affecting the movement of the esophagus. It is characterized by simultaneous, uncoordinated, or rapidly spreading contractions of the smooth muscle in the esophagus, leading to difficulties in swallowing.
Traditionally, DES has been diagnosed using manometry, which measures the pressure in the esophagus during wet swallows. According to the previous criteria, DES was identified when over 20% of swallows showed simultaneous contractions with a strength greater than 30 mmHg alongside normal peristalsis.
However, with the advent of high-resolution esophageal pressure topography, the diagnostic criteria for DES have been revised. Currently, DES is diagnosed based on at least two premature contractions occurring within a time frame of under 4.5 seconds while ensuring that the relaxation of the esophagogastric junction is normal.
Epidemiology
The annual occurrence of diffuse esophageal spasm is estimated to be approximately 1 case per 100,000 individuals. Among different racial groups, individuals of White ethnicity appear to have a higher prevalence of esophageal spasms than other races. Furthermore, the condition tends to manifest more frequently in women than in men.
The incidence of diffuse esophageal spasms tends to rise with advancing age, and it is rarely observed in children. Referral center data suggests that the prevalence of this condition ranges from 4% to 7%, providing insights into its frequency within specific healthcare settings.
Anatomy
Pathophysiology
The journey of a food bolus from the mouth to the stomach typically takes around ten seconds, facilitated by the rhythmic contractions of the esophageal body known as peristalsis. However, in the case of diffuse esophageal spasm, there is a disruption in the proper propagation of peristaltic waves through the esophageal wall.
This results in simultaneous, uncoordinated contractions occurring in different esophagus segments, hindering the food bolus’s smooth progression. Consequently, DES is characterized by rapid wave progression that deviates from the normal peristaltic pattern during swallowing, referred to as non-peristaltic waves.
The exact cause of DES remains unclear, but it is believed that an abnormal release of the neurotransmitter acetylcholine plays a significant role. However, the factors triggering the release of acetylcholine have yet to be determined. Some hypotheses suggest a potential association between DES and conditions such as reflux, elevated body mass index (BMI), hyperlipidemia, and hyperglycemia.
Etiology
The exact cause of diffuse esophageal spasm (DES) remains unknown, and multiple theories have been proposed to explain its etiology. One theory suggests a disruption in the coordination of peristalsis, which may arise from an imbalance between the inhibitory and excitatory pathways in the postganglionic nervous system. In DES, muscular hypertrophy or hyperplasia is often observed in the distal part of the esophagus, which constitutes approximately two-thirds of its length.
While the triggering event for DES is still uncertain, increased release of acetylcholine is considered a potential contributing factor. Another theory proposes that impairment of inhibitory ganglion neuronal function mediated by nitric oxide could play a role. Additionally, gastric reflux, primary nerve abnormalities, or motor disorders have been suggested as potential mechanisms underlying the peristaltic abnormalities seen in DES.
Exposure to acid can induce esophageal spasms, and the presence of heartburn can lead to esophageal contractions. Some studies have indicated that total cholesterol and body mass index (BMI) could be predictive factors for esophageal contractility. Similarly, blood glucose levels and BMI have been associated with the functioning of the lower esophageal sphincter.
Genetics
Prognostic Factors
Clinical History
Clinical History
The patient may report experiencing difficulty or discomfort while swallowing. Dysphagia can be intermittent or persistent, depending on the severity and frequency of esophageal spasms. DES can cause episodic chest pain, often as squeezing or pressure-like sensations. The pain may mimic angina or heart attack symptoms, leading to concerns about cardiac issues.
Some individuals with DES may experience regurgitation of food or sour-tasting material, indicating the reflux of stomach contents into the esophagus. Patients may report a burning sensation in the chest or throat, commonly known as heartburn. This symptom can be associated with acid reflux resulting from abnormal esophageal contractions. The symptoms of DES are typically intermittent, with episodes occurring at varying frequencies.
The severity and duration of these episodes can vary from person to person. Patients may notice that certain foods, beverages, or activities worsen their symptoms. Common triggers include hot or cold foods, carbonated drinks, large meals, and stress. DES is more commonly observed in older individuals and tends to occur more frequently in women than men.
Physical Examination
Physical Examination
Diffuse esophageal spasm (DES) primarily presents with symptoms related to esophageal dysfunction, and there may not be specific physical findings directly associated with DES. However, certain observations or findings during a physical examination may be present, depending on the severity and associated complications of the condition. DES is sometimes associated with conditions like obesity, which may be evident during a physical examination.
In severe cases of DES with prolonged dysphagia or food avoidance, signs of malnutrition such as weight loss, muscle wasting, or vitamin deficiencies may be observed. If DES episodes lead to frequent regurgitation or vomiting, signs of dehydration, such as dry skin, decreased skin turgor, or dry mucous membranes, may be present. In some instances, individuals with DES may experience chest pain or discomfort during palpation, but this finding is non-specific and can also be associated with other conditions.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Differential Diagnoses
Angina pectoris
Chagas disease
Achalasia
Esophagitis
Gastroesophageal reflux
Myocardial infarction
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
Patients are advised to make lifestyle changes, such as avoiding trigger foods or beverages (e.g., hot or cold items, carbonated drinks), eating smaller and more frequent meals, and maintaining an upright posture after eating to minimize reflux and dysphagia symptoms. Medications like nifedipine or diltiazem can help relax the esophageal smooth muscles and reduce spasm frequency and severity. Proton pump inhibitors are prescribed to manage associated gastroesophageal reflux symptoms, which can exacerbate DES.
In some cases, low-dose tricyclic antidepressants like amitriptyline may be prescribed to help relieve symptoms and modulate pain perception. In severe cases of DES that do not respond to conservative measures, botulinum toxin injections can be administered directly into the esophageal muscle to paralyze the muscles and reduce spasm frequency temporarily.
However, the effects are temporary and may require repeat injections. Surgical intervention in the form of an esophageal myotomy may be considered for severe and refractory cases of DES. During this procedure, the muscular layer of the esophagus is partially or completely cut to reduce spasms and improve esophageal function. However, the decision for surgery is made on a case-by-case basis and is reserved for extreme cases.
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Diffuse esophageal spasm (DES) is a rare disorder affecting the movement of the esophagus. It is characterized by simultaneous, uncoordinated, or rapidly spreading contractions of the smooth muscle in the esophagus, leading to difficulties in swallowing.
Traditionally, DES has been diagnosed using manometry, which measures the pressure in the esophagus during wet swallows. According to the previous criteria, DES was identified when over 20% of swallows showed simultaneous contractions with a strength greater than 30 mmHg alongside normal peristalsis.
However, with the advent of high-resolution esophageal pressure topography, the diagnostic criteria for DES have been revised. Currently, DES is diagnosed based on at least two premature contractions occurring within a time frame of under 4.5 seconds while ensuring that the relaxation of the esophagogastric junction is normal.
The annual occurrence of diffuse esophageal spasm is estimated to be approximately 1 case per 100,000 individuals. Among different racial groups, individuals of White ethnicity appear to have a higher prevalence of esophageal spasms than other races. Furthermore, the condition tends to manifest more frequently in women than in men.
The incidence of diffuse esophageal spasms tends to rise with advancing age, and it is rarely observed in children. Referral center data suggests that the prevalence of this condition ranges from 4% to 7%, providing insights into its frequency within specific healthcare settings.
The journey of a food bolus from the mouth to the stomach typically takes around ten seconds, facilitated by the rhythmic contractions of the esophageal body known as peristalsis. However, in the case of diffuse esophageal spasm, there is a disruption in the proper propagation of peristaltic waves through the esophageal wall.
This results in simultaneous, uncoordinated contractions occurring in different esophagus segments, hindering the food bolus’s smooth progression. Consequently, DES is characterized by rapid wave progression that deviates from the normal peristaltic pattern during swallowing, referred to as non-peristaltic waves.
The exact cause of DES remains unclear, but it is believed that an abnormal release of the neurotransmitter acetylcholine plays a significant role. However, the factors triggering the release of acetylcholine have yet to be determined. Some hypotheses suggest a potential association between DES and conditions such as reflux, elevated body mass index (BMI), hyperlipidemia, and hyperglycemia.
The exact cause of diffuse esophageal spasm (DES) remains unknown, and multiple theories have been proposed to explain its etiology. One theory suggests a disruption in the coordination of peristalsis, which may arise from an imbalance between the inhibitory and excitatory pathways in the postganglionic nervous system. In DES, muscular hypertrophy or hyperplasia is often observed in the distal part of the esophagus, which constitutes approximately two-thirds of its length.
While the triggering event for DES is still uncertain, increased release of acetylcholine is considered a potential contributing factor. Another theory proposes that impairment of inhibitory ganglion neuronal function mediated by nitric oxide could play a role. Additionally, gastric reflux, primary nerve abnormalities, or motor disorders have been suggested as potential mechanisms underlying the peristaltic abnormalities seen in DES.
Exposure to acid can induce esophageal spasms, and the presence of heartburn can lead to esophageal contractions. Some studies have indicated that total cholesterol and body mass index (BMI) could be predictive factors for esophageal contractility. Similarly, blood glucose levels and BMI have been associated with the functioning of the lower esophageal sphincter.
Clinical History
The patient may report experiencing difficulty or discomfort while swallowing. Dysphagia can be intermittent or persistent, depending on the severity and frequency of esophageal spasms. DES can cause episodic chest pain, often as squeezing or pressure-like sensations. The pain may mimic angina or heart attack symptoms, leading to concerns about cardiac issues.
Some individuals with DES may experience regurgitation of food or sour-tasting material, indicating the reflux of stomach contents into the esophagus. Patients may report a burning sensation in the chest or throat, commonly known as heartburn. This symptom can be associated with acid reflux resulting from abnormal esophageal contractions. The symptoms of DES are typically intermittent, with episodes occurring at varying frequencies.
The severity and duration of these episodes can vary from person to person. Patients may notice that certain foods, beverages, or activities worsen their symptoms. Common triggers include hot or cold foods, carbonated drinks, large meals, and stress. DES is more commonly observed in older individuals and tends to occur more frequently in women than men.
Physical Examination
Diffuse esophageal spasm (DES) primarily presents with symptoms related to esophageal dysfunction, and there may not be specific physical findings directly associated with DES. However, certain observations or findings during a physical examination may be present, depending on the severity and associated complications of the condition. DES is sometimes associated with conditions like obesity, which may be evident during a physical examination.
In severe cases of DES with prolonged dysphagia or food avoidance, signs of malnutrition such as weight loss, muscle wasting, or vitamin deficiencies may be observed. If DES episodes lead to frequent regurgitation or vomiting, signs of dehydration, such as dry skin, decreased skin turgor, or dry mucous membranes, may be present. In some instances, individuals with DES may experience chest pain or discomfort during palpation, but this finding is non-specific and can also be associated with other conditions.
Differential Diagnoses
Angina pectoris
Chagas disease
Achalasia
Esophagitis
Gastroesophageal reflux
Myocardial infarction
Patients are advised to make lifestyle changes, such as avoiding trigger foods or beverages (e.g., hot or cold items, carbonated drinks), eating smaller and more frequent meals, and maintaining an upright posture after eating to minimize reflux and dysphagia symptoms. Medications like nifedipine or diltiazem can help relax the esophageal smooth muscles and reduce spasm frequency and severity. Proton pump inhibitors are prescribed to manage associated gastroesophageal reflux symptoms, which can exacerbate DES.
In some cases, low-dose tricyclic antidepressants like amitriptyline may be prescribed to help relieve symptoms and modulate pain perception. In severe cases of DES that do not respond to conservative measures, botulinum toxin injections can be administered directly into the esophageal muscle to paralyze the muscles and reduce spasm frequency temporarily.
However, the effects are temporary and may require repeat injections. Surgical intervention in the form of an esophageal myotomy may be considered for severe and refractory cases of DES. During this procedure, the muscular layer of the esophagus is partially or completely cut to reduce spasms and improve esophageal function. However, the decision for surgery is made on a case-by-case basis and is reserved for extreme cases.
medtigo
Dıffuse esophageal Spasm
Updated :
September 6, 2023
Diffuse esophageal spasm (DES) is a rare disorder affecting the movement of the esophagus. It is characterized by simultaneous, uncoordinated, or rapidly spreading contractions of the smooth muscle in the esophagus, leading to difficulties in swallowing.
Traditionally, DES has been diagnosed using manometry, which measures the pressure in the esophagus during wet swallows. According to the previous criteria, DES was identified when over 20% of swallows showed simultaneous contractions with a strength greater than 30 mmHg alongside normal peristalsis.
However, with the advent of high-resolution esophageal pressure topography, the diagnostic criteria for DES have been revised. Currently, DES is diagnosed based on at least two premature contractions occurring within a time frame of under 4.5 seconds while ensuring that the relaxation of the esophagogastric junction is normal.
The annual occurrence of diffuse esophageal spasm is estimated to be approximately 1 case per 100,000 individuals. Among different racial groups, individuals of White ethnicity appear to have a higher prevalence of esophageal spasms than other races. Furthermore, the condition tends to manifest more frequently in women than in men.
The incidence of diffuse esophageal spasms tends to rise with advancing age, and it is rarely observed in children. Referral center data suggests that the prevalence of this condition ranges from 4% to 7%, providing insights into its frequency within specific healthcare settings.
The journey of a food bolus from the mouth to the stomach typically takes around ten seconds, facilitated by the rhythmic contractions of the esophageal body known as peristalsis. However, in the case of diffuse esophageal spasm, there is a disruption in the proper propagation of peristaltic waves through the esophageal wall.
This results in simultaneous, uncoordinated contractions occurring in different esophagus segments, hindering the food bolus’s smooth progression. Consequently, DES is characterized by rapid wave progression that deviates from the normal peristaltic pattern during swallowing, referred to as non-peristaltic waves.
The exact cause of DES remains unclear, but it is believed that an abnormal release of the neurotransmitter acetylcholine plays a significant role. However, the factors triggering the release of acetylcholine have yet to be determined. Some hypotheses suggest a potential association between DES and conditions such as reflux, elevated body mass index (BMI), hyperlipidemia, and hyperglycemia.
The exact cause of diffuse esophageal spasm (DES) remains unknown, and multiple theories have been proposed to explain its etiology. One theory suggests a disruption in the coordination of peristalsis, which may arise from an imbalance between the inhibitory and excitatory pathways in the postganglionic nervous system. In DES, muscular hypertrophy or hyperplasia is often observed in the distal part of the esophagus, which constitutes approximately two-thirds of its length.
While the triggering event for DES is still uncertain, increased release of acetylcholine is considered a potential contributing factor. Another theory proposes that impairment of inhibitory ganglion neuronal function mediated by nitric oxide could play a role. Additionally, gastric reflux, primary nerve abnormalities, or motor disorders have been suggested as potential mechanisms underlying the peristaltic abnormalities seen in DES.
Exposure to acid can induce esophageal spasms, and the presence of heartburn can lead to esophageal contractions. Some studies have indicated that total cholesterol and body mass index (BMI) could be predictive factors for esophageal contractility. Similarly, blood glucose levels and BMI have been associated with the functioning of the lower esophageal sphincter.
Clinical History
The patient may report experiencing difficulty or discomfort while swallowing. Dysphagia can be intermittent or persistent, depending on the severity and frequency of esophageal spasms. DES can cause episodic chest pain, often as squeezing or pressure-like sensations. The pain may mimic angina or heart attack symptoms, leading to concerns about cardiac issues.
Some individuals with DES may experience regurgitation of food or sour-tasting material, indicating the reflux of stomach contents into the esophagus. Patients may report a burning sensation in the chest or throat, commonly known as heartburn. This symptom can be associated with acid reflux resulting from abnormal esophageal contractions. The symptoms of DES are typically intermittent, with episodes occurring at varying frequencies.
The severity and duration of these episodes can vary from person to person. Patients may notice that certain foods, beverages, or activities worsen their symptoms. Common triggers include hot or cold foods, carbonated drinks, large meals, and stress. DES is more commonly observed in older individuals and tends to occur more frequently in women than men.
Physical Examination
Diffuse esophageal spasm (DES) primarily presents with symptoms related to esophageal dysfunction, and there may not be specific physical findings directly associated with DES. However, certain observations or findings during a physical examination may be present, depending on the severity and associated complications of the condition. DES is sometimes associated with conditions like obesity, which may be evident during a physical examination.
In severe cases of DES with prolonged dysphagia or food avoidance, signs of malnutrition such as weight loss, muscle wasting, or vitamin deficiencies may be observed. If DES episodes lead to frequent regurgitation or vomiting, signs of dehydration, such as dry skin, decreased skin turgor, or dry mucous membranes, may be present. In some instances, individuals with DES may experience chest pain or discomfort during palpation, but this finding is non-specific and can also be associated with other conditions.
Differential Diagnoses
Angina pectoris
Chagas disease
Achalasia
Esophagitis
Gastroesophageal reflux
Myocardial infarction
Patients are advised to make lifestyle changes, such as avoiding trigger foods or beverages (e.g., hot or cold items, carbonated drinks), eating smaller and more frequent meals, and maintaining an upright posture after eating to minimize reflux and dysphagia symptoms. Medications like nifedipine or diltiazem can help relax the esophageal smooth muscles and reduce spasm frequency and severity. Proton pump inhibitors are prescribed to manage associated gastroesophageal reflux symptoms, which can exacerbate DES.
In some cases, low-dose tricyclic antidepressants like amitriptyline may be prescribed to help relieve symptoms and modulate pain perception. In severe cases of DES that do not respond to conservative measures, botulinum toxin injections can be administered directly into the esophageal muscle to paralyze the muscles and reduce spasm frequency temporarily.
However, the effects are temporary and may require repeat injections. Surgical intervention in the form of an esophageal myotomy may be considered for severe and refractory cases of DES. During this procedure, the muscular layer of the esophagus is partially or completely cut to reduce spasms and improve esophageal function. However, the decision for surgery is made on a case-by-case basis and is reserved for extreme cases.
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